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Abstract Case Report Introduction Corneal foreign bodies (FBs) are a common form of ocular injuries presenting to the outpatient department in ophthalmology practice, the most common being metallic FBs. We report a rare case of solid detergent particle trauma to the eyeball in a middle-aged adult presenting as multiple intracorneal FBs. There have been only a few cases in literature with blunt objects causing intracorneal FBs. Case Report A 50-year-old male patient, a grocery storekeeper by occupation, presented with complaints of foreign body sensation and watering in the right eye following exposure to detergent powder 12 days back. While stacking detergent sachets (Surf Excel) on top rack of his shop at a height of approximately 8 feet, one of the sachets got accidentally torn by hitting a metallic nail adjacent to the rack following which the detergent granules spilled into the eye. Visual acuity in the right eye was 6/18 and 6/6 in the left eye. On slit-lamp biomicroscopy, there was a 2 mm × 2 mm epithelial defect seen inferiorly at the 6 o’clock position, around 3 mm from the limbus surrounded by a ring of multiple whitish deposits in the superficial cornea. Multiple mid‑stromal depositions were also identified at the 4 o’clock position, around 4 mm from the nasal limbus with no other signs of alkali injury such as limbal ischemia, vascularization, pannus formation, and conjunctival defects [Figure 1]. These findings were confirmed on anterior segment optical coherence tomography [Figure 2]. At the first visit, an attempt of debridement was made for particles Corneal foreign bodies (FBs) are a significant cause of vision loss and morbidity due to long‑term sequelae. The most common culprits are metal; others are glass, vegetative matter, and burns. We report a case of a 50-year-old male patient with multiple FBs embedded in the anterior stroma following blunt injury with detergent particles. Keywords: Blunt injury, foreign body, intrastromal detergent Address for correspondence: Dr. Vaishali Tomar, Guru Nanak Eye Center, Maharaja Ranjit Singh Marg, New Delhi ‑ 110 002, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Sharma S, Tomar V, Agarwal R, Dadeya S. A case report of intracorneal detergent particles deposits. Delhi J Ophthalmol 2023;33:322-4. A Case Report of Intracorneal Detergent Particles Deposits Shivani Sharma, Vaishali Tomar, Rinky Agarwal, Subhash Dadeya Guru Nanak Eye Centre, MAMC, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_147_23 Submitted: 09-Sep-2023 Revised: 06-Nov-2023 Accepted: 15-Nov-2023 Published: 15-Jan-2024 Figure 1: Slit‑lamp picture and anterior segment optical coherence tomography showing the level of mid‑stromal granules deposition 322 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Sharma, et al.: Intracorneal foreign body: Detergent particle deposits deposited near the inferior limbus; however, the attempt was unsuccessful since the detergent particles were very fine and most of them were embedded in the anterior to the mid-stroma. The patient was administered topical antibiotics, lubricants, and steroids in a tapering dose. At 1-week follow-up, the epithelial defect healed completely. At 1-month follow-up, the deposits decreased dramatically in size. Discussion To the best of our knowledge, this is the first time that corneal FBs with solid detergent granules are being reported. To date, chemical injury with liquid detergent has been reported.[1] Through this case report, we aim to highlight the mechanism of trauma by blunt objects like detergent particles which can lead to intracorneal FBs at various depths. Furthermore, we try to highlight the need for proper storage and use of these products with the adoption of prevention strategies in the form of protective eyewear. The geometry and energy of projectiles predict the type of injury and level of ocular penetration.[2] The energy transmitted by an intraocular FB (IOFB) is directly proportional to its mass and velocity. In addition to mass, the shape of IOFB has an effect on its penetration to the eye. Woodcock et al. [3] classified IOFBs into four different shape categories, blade (mean mass 30 mg), disc (mean mass 113 mg), cylinders (mean mass 12 mg), and spheres (mean mass 204 mg). We speculate that to penetrate the corneal layers, an FB must have either high velocity/momentum or sharp margins. In our case, it is possible that light-weight detergent granules with their irregular margins behaved like small projectiles after accidental rupture, acquiring momentum sufficient enough to penetrate the superficial cornea [Figure 3]. The variable size of granules must have produced variable effects with larger-sized granules causing mechanical irritation, subsequently epithelial defect, and smaller-sized granules penetrating cornea at differential levels. Modern detergents are usually composed of caustic soda (sodium hydroxide), caustic potash (potassium hydroxide), whitening agents, and other stabilizers containing hydroxyl ions which denature the collagen matrix of the cornea and facilitate further chemical penetration causing hazardous effects such as liquefaction necrosis and intense inflammatory reaction. Direct damage to the conjunctiva can cause scarring, symblepharon formation, destruction of conjunctival goblet cells, limbal stem cell deficiency, conjuctivalization of the cornea, anterior segment ischemia, and secondary glaucoma. Fortunately, no such signs of chemical injury were seen in this patient. Unlike other solid nonorganic FBs such as glass and metal, which remain unaltered, detergent particles get dissolved over time possibly due to localized inflammation which gradually absorbs them. Localized chemical injury to the corneal epithelium by the reaction of solid granules with tear film cannot be ruled out substantially. Conclusion To conclude, corneal FBs can be noted after accidental trauma with detergent granules. As long-term sequelae of this kind of corneal trauma remain unknown, it is advisable that storekeepers exert a level of precaution and care while storing these seemingly innocuous detergent sachets. Protective eyewear can also be worn to prevent such ophthalmic incidents. Figure 2: Slit‑lamp picture and anterior segment optical coherence tomography showing epithelial defect with deposited granules Figure 3: Picture of detergent powder granules of various sizes Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 323

Sharma, et al.: Intracorneal foreign body: Detergent particle deposits Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Haring RS, Sheffield ID, Frattaroli S. Detergent pod‑related eye injuries among preschool-aged children. JAMA Ophthalmol 2017;135:283-4. 2. Duma SM, Ng TP, Kennedy EA, Stitzel JD, Herring IP, Kuhn F. Determination of significant parameters for eye injury risk from projectiles. J Trauma 2005;59:960-4. 3. WoodcockMG, ScottRA, HuntbachJ, KirkbyGR. Mass and shape as factors in intraocular foreign body injuries. Ophthalmology 2006;113:2262-9. 324 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Abstract Case Report Introduction Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. Several etiologies have been recognized, including infections (such as syphilis and tuberculosis), autoimmune disorders (such as immunoglobulin (Ig) G4‑related disease, rheumatoid arthritis), vasculitis (such as Wegener’s granulomatosis), granulomatous inflammation (such as sarcoidosis), and neoplasms(such as lymphoma or metastasis).[1-4] When there is no identifiable cause, the condition is referred to “idiopathic HP.” Neuro-ophthalmic symptoms of IHP include visual field loss, complete blindness, optic neuropathy, and increased intracranial pressure with papilledema. The neurologic manifestations are attributed to compression of cranial nerves at the skull base by thickened dura mater resulting from the chronic inflammatory process. In this case report, we present a case of unilateral diminution of vision with papilledema in a young male patient who turned out to be a case of idiopathic HP (IHP) after extensive serological/ imaging workup and follow-up. Case Report A 19-year-old male patient presented to the ophthalmology outpatient department with complaints of diminution of vision in the right eye for 2months. On examination, his visual acquity in the right eye was 6/60. Fundoscopy revealed disc edema in the right eye. He was not a known case of diabetes mellitus, hypertension, or other systemic illness. The rest of ophthalmological examination was within normal limits. Contrast magnetic resonance imaging (MRI) of the brain revealed diffuse enhancing pachymeningeal thickening involving bilateral peri-mesencephalic/peri-pontine cistern and dural layer along bilateral anterior temporal and frontal convexities, more prominent on the right side [Figures 1 and 2]. The thickened dura along the right anterior temporal convexity extended up to the right cavernous sinus region and also involved the right orbital apex. Based on these findings, possibility of pachymeningitis was raised. His chest X‑ray was normal without evidence of tubercular involvement or features of sarcoidosis. His routine blood investigations liver and renal function tests were within normal limits except mildly elevated erythrocyte sedimentation rate. Cerebrospinal fluid (CSF) examination revealed mildly elevated Idiopathic hypertrophic pachymeningitis(IHP) is a rare condition characterized by a chronic fibrosing inflammatory process usually involving either the intracranial or spinal dura matter and rarely both. Neurological symptoms are mostly nonspecific. Patients commonly present with long‑standing headache and/or progressive neurologic deficits, multiple cranial nerve palsies or blindness. In this case report, we present a case of IHP in a 19-year-old male with unilateral diminution of vision and papilledema due to the involvement of intracranial duramater. Keywords: Brain, dural thickening, hypertrophic pachymeningitis, idiopathic hypertrophic pachymeningitis, magnetic resonance imaging, papilledema Address for correspondence: Dr. Rahul Sidheshwar Ranjan, Rama Medical College, Kanpur, Uttar Pradesh, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Namrata, Ranjan RS, Agarwal R, Singh AK, Varun K. Idiopathic hypertrophic pachymeningitis presenting with unilateral diminution of vision and papilloedema. Delhi J Ophthalmol 2023;33:325-7. Idiopathic Hypertrophic Pachymeningitis Presenting with Unilateral Diminution of Vision and Papilloedema Namrata1 , Rahul Sidheshwar Ranjan2 , Ruchika Agarwal1 , Anil Kumar Singh3 , Kumar Varun2 Departments of 1 Ophthalmology, 2 Radiodiagnosis, Rama Medical College, Kanpur, 3 Department of Radiodiagnosis, SGPGI, Lucknow, Uttar Pradesh, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_127_23 Submitted: 26-Jul-2023 Revised: 11-Oct-2023 Accepted: 02-Nov-2023 Published: 15-Jan-2024 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 325

Namrata, et al.: Idiopathic hypertrophic pachymeningitis causing reduced visual acuity CSF proteins with normal level of adenosine deaminase with sterile CSF culture. His venereal disease research laboratory test was negative. Workup for autoimmune disease was also negative. Serum IgG4 level was within normal limit. Based on these findings, the possibility of IHP was made, and the patient was offered the option of dural biopsy for confirmation, which he refused. Therefore, the patient was put on steroid therapy. There was a significant improvement in his symptoms at 1month follow-up with improved visual acquity in the right eye up to 6/12 with resolution of disc edema and therefore confirming the diagnosis of IHP. Discussion IHP is a rare disease caused by the thickening of either the intracranial or spinal dura mater or rarely both simultaneously. The diagnosis of IHP relies on the exclusion of other possible causes of pachymeningitis, from granulomatous diseases, such as neurosarcoidosis, neurosyphilis, tuberculosis, and rheumatoid pachymeningitis, and Wegener’s granulomatosis to primary tumor of the meninges, en plaque meningioma, or dural carcinomatosis. If an exhaustive work-up fails to identify the cause of the meningeal changes, a diagnosis of IHP is made. The confirmation of diagnosis is made by dural biopsy. The histopathology shows chronic inflammatory changes, lymphoplasmacytic cell infiltration, fibrous tissue hyperplasia, and hyaline degeneration.[5] Nonophthalmological manifestations of IHP include chronic headache, cranial nerve palsies (cranial nerves IV–VIII), cerebellar ataxia, and seizures. Other rare documented neurologic complications of IHP include venous sinus thrombosis, obstructive hydrocephalus, and cerebral edema. Cranial nerve II, III, IV, V, and VI neuropathy is well correlated with involvement of cavernous sinus to the optic canal and superior orbital fissure involvement. On the other hand, the thickening of falcotentorial dura was usually associated with cranial nerve V, VII, VIII, IX, and X palsies. Typically, IHP demonstrates smooth or nodular dural thickening that is isointense or hypointense on both T1- and T2-weighted sequences. It also shows avid enhancement after intravenous administration of contrast material. These signal intensity characteristics are due to the fibrosis and necrosis of the dura mater. Peripheral hyperintensity can be seen on T2-weighted images and is thought to represent active inflammation or increased vascularity of the dura mater and underlying neuroparenchyma. Warittikoon and Jakchairoongruang, in their study of MRI characteristics of idiopathic HP in 23 patients, concluded that T2 hypointensity or dark intensity and enhancement of the dural edge was more commonly associated with IHP than secondary HP due to other causes. Central T2 hyperintensity with hypointense rim (T2‑rim pattern) also turned out to be one 326 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 1: Axial fluid‑attenuated inversion recovery sequence showing pachymeningeal thickening along bilateral perimesencephalic and peripontine cistern (yellow arrow). Similar thickening is also seen along bilateral temporal convexity (orange arrow) reaching up to the right orbital apex

Namrata, et al.: Idiopathic hypertrophic pachymeningitis causing reduced visual acuity of the promising MRI findings in idiopathic HP.[6] Therefore, MRI plays an important role in suspecting IHP based on imaging findings and site of involvement. Corticosteroid therapy has shown effectiveness in alleviating symptoms and in the arrest of clinical progression of IHP. In refractory cases, immunomodulators such as azathioprine and cyclophosphamide have been used on the assumption that IHP is an autoimmune disease. Surgery may be required for cranial nerve decompression. Conclusion IHP is a chronic inflammatory disorder of the dura mater that causes neurologic deficits and may present with unilateral/ bilateral gradual diminution of vision/blindness with disc edema. MRI of the brain plays an important role in early diagnosis and also in the follow-up, as close observation for recurrence are necessary to ensure a good long-term outcome. Acknowledgment We are thankful to Mr. Mohan Chandra, Medical Transcriptionists, Rama Medical College for helping to prepare the Manuscript. The authors are also thankful to MRI technician Mr. Rajkumar Mishra. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Goyal M, Malik A, Mishra NK, Gaikwad SB. Idiopathic hypertrophic pachymeningitis: Spectrum of the disease. Neuroradiology 1997;39:619-23. 2. Yuh WT, Drew JM, Rizzo M, Ryals TJ, Sato Y, Bell WE. Evaluation of pachymeningitis by contrast-enhanced MR imaging in a patient with rheumatoid disease. AJNR Am J Neuroradiol 1990;11:1247-8. 3. Zelasko S, Hollingshead M, Castillo M, Bouldin TW. CT and MR imaging of progressive dural involvement by nephrogenic systemic fibrosis. AJNR Am J Neuroradiol 2008;29:1880‑2. 4. Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore) 2013;92:206‑16. 5. Zhu R, He Z, Ren Y. Idiopathic hypertrophic craniocervical pachymeningitis. Eur Spine J 2015;24 Suppl 4:S633-5. 6. Warittikoon S, Jakchairoongruang K. Distinguishing magnetic resonance imaging features between idiopathic hypertrophic pachymeningitis and secondary hypertrophic pachymeningitis. Asian Biomed (Res Rev News) 2019;13:113‑9. Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 327 Figure 2: Axial post contrast T1 weighted sequence showing pachymeningeal enhancement along bilateral perimesencephalic and peripontine cistern (yellow arrow). Similar enhancement is also seen along bilateral temporal convexity (orange arrow) reaching up to the right orbital apex

328 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow Abstract Case Report Introduction Cytomegalovirus (CMV) and varicella‑zoster virus (VZV) co-infection in an immunocompetent host are not only unheard of but also require an early and accurate diagnosis and management else it can lead to severe complications speedily. We present one such case which demonstrates the importance of early diagnosis and treatment in infectious retinitis. Case Report A 31-year-old healthy male presented with complaints of sudden diminution of vision in his right eye 3-day postonset of fever and rigors, for which he was treated locally. The best-corrected visual acuity in the right eye was 20/600 and the left eye was 20/20. Anterior segment examination was otherwise normal except for a relative afferent pupillary defect in the right eye. Fundus examination revealed minimal vitritis, disc edema, exudative retinal detachment, multiple superficial and deep hemorrhages, and sheathing of the vasculature [Figure 1a and b]. He tested negative for human immunodeficiency virus (HIV) (Elisa), syphilis (venereal disease research laboratory), tuberculosis(MT, QuantiFERON Gold), sarcoidosis (angiotensin‑converting enzyme and high‑resolution computed tomography), and autoimmune diseases (antinuclear antibodies panel) and was advised a polymerase chain reaction (PCR) analysis of aqueous humor for viral markers (CMV, herpes simplex virus [HSV], and VZV), which revealed positivity for both CMV (immunoglobulin [Ig] M >0.9 IU/mL) and VZV (IgM >1.4IU/mL). He was started on systemic (oral) valacyclovir (1000 mg TDS) and oral steroids (1 g/kg/day with weekly tapering). Post 1 week of therapy, severe vitritis was noted, which was an immune-mediated reaction commonly noted in immunocompetent patients. It gradually subsided in the next couple of days with ongoing treatment. After completion of 3 weeks of induction therapy of valacyclovir, the fundus picture revealed resolution of hemorrhages, sheathing, and exudative detachment, and the clog of exudates was noted to be elevated from the retinal surface along with an incomplete posterior vitreous detachment [Figure 2]. Avitrectomy with fluid‑air exchange was advised, under the cover of maintenance therapy of systemic valacyclovir and steroids. The vitreous tap was taken, but due to cost constraints, PCR analysis for viral titers could not be performed. Combined infection with cytomegalovirus (CMV) and varicella‑zoster virus (VZV) is a rare entity, especially in an immunocompetent person. Almost all cases with co-infection are either immunocompromised or on immunosuppressant. Here, we present to you one such case of a 31-year-old male who presented with complaints of sudden diminution of vision in his right eye after a bout of fever. After a battery of investigations, the patient was found to have raised blood titers of CMV and VZV. The patient was put on oral antivirals and steroids, followed by a vitrectomy, which yielded a good outcome. Keywords: Cytomegalovirus, exudative retinal detachment, valacyclovir, varicella-zoster virus, viral retinitis, vitrectomy Viral Retinitis with Co-Infection Cytomegalovirus and Varicella Zoster Dhaivat Shah, Ravin Punamia, Bennet Chacko Mathew, Rahul Singh, Milind Rokade Choithram Netralaya Comprehensive Eye Care Centre, Indore, Madhya Pradesh, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_120_23 Address for correspondence: Dr. Dhaivat Shah, Shriramtalawali, Dhar Road, Indore ‑ 453 001, Madhya Pradesh, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Shah D, Punamia R, Mathew BC, Singh R, Rokade M. Viral retinitis with co-infection cytomegalovirus and varicella zoster. Delhi J Ophthalmol 2023;33:328-30. Submitted: 19-Jul-2023 Revised: 06-Nov-2023 Accepted: 08-Nov-2023 Published: 15-Jan-2024

Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 329 Shah, et al.: Viral retinitis with co‑infection of Cytomegalovirus and varicella‑zoster virus in an immunocompetent young male One-month postoperative [Figure 3a], the patient was doing well with an improved visual acuity of 20/200 and a well-attached retina with retinal pigment epithelium atrophy over the macula and a secondary optic disc pallor. The optical coherence tomography line scan [Figure 3b] showed a loss of outer retinal layers at the macula, accounting for 20/200 visual acuity. The antiviral maintenance therapy was continued for 3-month postoperative. At 1-year follow-up, the patient’s ocular condition was stable. Discussion CMV is an enveloped, double-stranded DNA virus in the Herpesviridae family. It spreads through person-to-person transmission in bodily fluids, and may also be transmitted in donor organs from CMV-positive donors. It is mainly a disease of immunocompromised hosts, occurring in neonates, organ transplant recipients, and persons with acquired immunodeficiency syndrome from HIV. The drug of choice is oral valganciclovir.[1] VZV is a ubiquitous virus of the herpesvirus family with a double-stranded DNA genome. VZV is highly communicable and spreads by the airborne route, and can affect immunocompromised or competent hosts. Most of the virus is spread through the cutaneous vesicles, in which it is highly concentrated. The drug of choice is oral valacyclovir.[2] Simultaneous occurrence of CMV and VZV retinitis in an immunocompetent host is a very rare occurrence. Gupta et al. [3] reported in their study that the nine patients of CMV retinitis that were included were immunocompetent during the survey. Figure 1: (a) Is a fundus photo showing minimal vitritis, disc edema, multiple superficial and deep hemorrhages, sheathing of vasculature, and extensive exudation. (b) Fundus photo showing vitritis, posterior hypopyon, exudative retinal detachment, and sheathing of vasculature a b Figure 2: (a) Is the fundus photo post 6 weeks of treatment showing clog of exudates elevated from the retinal surface. Furthermore, superior clearing of retinal hemorrhages is noted. (b) The exudates floating inferiorly in the vitreous cavity a b

330 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Shah, et al.: Viral retinitis with co‑infection of Cytomegalovirus and varicella‑zoster virus in an immunocompetent young male Carlstroem G et al. [4] also reported two cases of CMV retinitis that were immunocompetent at the time of diagnosis. However, concurrent infection in a competent host, like in our case, is seldom reported in the literature. Valganciclovir is more effective orally as compared to valacyclovir with considerably fewer side effects in immunocompromised patients with CMV retinitis, but has minimal role in VZV retinitis.[5] Valacyclovir is the drug of choice in infection due to HSV and VZV, and it has also been found to be effective in CMV infections in a few cases.[6] In our patient, oral valacyclovir was selected due to immunocompetency of the patient, the concurrent occurrence of CMV and VZV, its established action against the Herpes family, and the cost-effectiveness of the drug. After the induction therapy, severe vitritis was noted due to immunocompetence. The exudative clump was eventually noted to be partially detached from the retinal surface, hence a vitrectomy was done to clear the vitreous and exudates. One-month postoperative, the patient improved both anatomically and functionally. Although the eye was quiet, the antiviral maintenance therapy was continued for around 3 months, and no recurrence was noted till a 1-year follow-up. Conclusion VZV retinitis is a disease usually related to immunocompetent individuals while CMV retinitis is noted in immunocompromised. Simultaneous occurrence of CMV and VZV retinitis in an immunocompetent patient is an extremely rare scenario. In this unique case report, we see a dramatic response to prompt therapy of systemic antiviral along with steroids, and no recurrence over a period of 1 year. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed. Acknowledgments We would like to thank our optometrists for their help in retinal imaging. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Port AD, Orlin A, Kiss S, Patel S, D’Amico DJ, Gupta MP. Cytomegalovirus retinitis: A review. J Ocul Pharmacol Ther 2017;33:224-34. 2. Gershon AA, Breuer J, Cohen JI, Cohrs RJ, Gershon MD, Gilden D, et al. Varicella zoster virus infection. Nat Rev Dis Primers 2015;1:15016. 3. Gupta S, Vemulakonda GA, Suhler EB, Yeh S, Albini TA, Mandelcorn E, et al. Cytomegalovirus retinitis in the absence of AIDS. Can J Ophthalmol 2013;48:126-9. 4. Carlstroem G. Virologic studies on cytomegalic inclusion disease. Acta Paediatr Scand 1965;54:17-23. 5. Freeman RB. Valganciclovir: Oral prevention and treatment of cytomegalovirus in the immunocompromised host. Expert Opin Pharmacother 2004;5:2007-16. 6. Ong SY, Truong HT, Diong CP, Linn YC, Ho AY, Goh YT, et al. Use of Valacyclovir for the treatment of cytomegalovirus antigenemia after hematopoietic stem cell transplantation. BMC Hematol 2015;15:8. Figure 3: (a) Is the fundus photo 1 month postoperative showing clear media with large scar over the macula. (b) Is the spectral‑domain optical coherence tomography line scan showing loss of outer retinal layers at the macula with intact inner retinal layers a b

Abstract Photo Essay Introduction Hypermature cataract is an advanced form of cataract. The prevalence of hypermature cataract in rural and urban India is found to be 8.62% and 0.97%, respectively.[1] Hypermature cataract has a potential to cause serious complications leading to severe visual impairment. A study by Pujari and Sharma has described the role of anterior segment optical coherence tomography (AS OCT) in cataract surgery.[2] Here, we report the intralenticular multiple subcapsular fluid pockets in an eye with hypermature cataract using AS OCT. Case Report A  63‑year‑old male patient visited to the outpatient department with a chief complaint of diminution of vision in the right eye. His best-corrected visual acuity in right and left eyes was hand movement close to the face and 20/20, respectively. Intraocular pressure in right and left eyes was 17 and 16 mm Hg. Slit-lamp examination revealed hypermature cataract in the right eye [Figure 1], whereas the left eye was pseudophakic. Left eye fundus was normal; however, fundus was not visible in the right eye. Hence, B-scan ultrasonography of the right eye was done which revealed normal optic nerve head shadow with attached retina and mild mobile hyperechos. AS OCT (Revo FC, Optopol technology, Zawiercie, Poland) of the right eye revealed multiple subcapsular fluid pockets [Figure 2]. Discussion The newer generation AS OCT has a potential to play a role in understanding hidden lenticular changes such as segregating mature cataracts with and without fluid pockets, retained intralenticular foreign bodies, lens dislocation, traumatic cataract, posterior capsular dehiscence, etc., which otherwise difficult to identify with slit lamp.[2-4] In the present report, we have described the hidden intralenticular liquefied material suggestive of raised intrabag pressure in hypermature cataract. These raised intralenticular bag pressure leads to capsule‑related complications such as Argentinian flag sign and difficulty in completing continuous curvilinear capsulorhexis. This report describes the intralenticular multiple subcapsular fluid pockets in an eye with hypermature cataract captured using the anterior segment optical coherence tomography in a 63-year-old male. Keywords: Anterior segment optical coherence tomography, hypermature cataract, subcapsular fluid pocket Address for correspondence: Mr. Onkar H. Pirdankar, Isha Netralaya, Radhakrishna Sankul, Opp Holy Cross Hospital, Karnik Road, Kalyan ‑ 421 301, Maharashtra, India. E‑mail: [email protected] How to cite this article: Sheth S, Kamble G, Shah S, Shah S, Zhende SD, Pirdankar OH. Intra-lenticular choppy wave in hypermature cataract. Delhi J Ophthalmol 2023;33:331-2. Intra-Lenticular Choppy Wave in Hypermature Cataract Siddharth Sheth1 , Gaurav Kamble2 , Smitesh Shah1 , Sonal Shah1 , Sushilkumar D. Zende2 , Onkar H. Pirdankar2 1 Department of Ophthalmology, Isha Netralaya, Kalyan, Maharashtra, India, 2 Department of Optometry, Isha Netralaya, Kalyan, Maharashtra, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_152_23 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Submitted: 15-Sep-2023 Revised: 07-Oct-2023 Accepted: 26-Oct-2023 Published: 15-Jan-2024 Figure 1: Illustrating right eye hypermature cataract © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 331

Sheth, et al.: Intralenticular choppy wave in hypermature cataract A study by Dhami et al. has reported that identifying the presence of fluid pockets could help strategized the surgery to avoid any intraoperative complications.[4] Although slit lamp is widely available to diagnose the hypermature morgagnian cataract, it will not help quantify the intra-lenticular vacuoles accurately. On the other hand, AS OCT can help in quantifying the liquefaction/vacuoles, thereby helpful in predicting the possible intra-operative capsular complications in patients who have fluid pockets. Thus, AS OCT could be useful in cases with hypermature cataracts where it is difficult to identify the intralenticular pockets using the slit lamp. In the present report, we did not evaluate the association between scan finding and severity since the primary purpose was to report the intra-lenticular pockets in hypermature cataract; however, future studies can be conducted to assess the same. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Singh S, Pardhan S, Kulothungan V, Swaminathan G, Ravichandran JS, Ganesan S, et al. The prevalence and risk factors for cataract in rural and urban India. Indian J Ophthalmol 2019;67:477-83. 2. Pujari A, Sharma N. The emerging role of anterior segment optical coherence tomography in cataract surgery: Current role and future perspectives. Clin Ophthalmol 2021;15:389-401. 3. Guan JY, Ma YC, Zhu YT, Xie LL, Aizezi M, Zhuo YH, et al. Lens nucleus dislocation in hypermature cataract: Case report and literature review. Medicine (Baltimore) 2022;101:e30428. 4. Dhami A, Dhami AS, Singh H, Dhami GS. Role of anterior segment optical coherence tomography for safer management of mature white cataracts. J Cataract Refract Surg 2019;45:480-4. Figure 2: Anterior segment optical coherence tomography image showing right eye intra‑lenticular multiple subcapsular fluid pockets with an appearance of the choppy wave 332 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Abstract Photo Essay An 18-year-old female presented with a diminution of vision in both eyes for 6 months. Her best-corrected visual acuity was 20/40 in both eyes. There was no history of any ocular trauma or use of local or systemic steroids. The patient had no known systemic illness. Slit-lamp examination revealed a triradiate pattern cataract in both her eyes, mimicking the Mercedes–Benz logo. Fundus examination was within normal limits for both eyes. Various types of developmental cataract can be present such as total cataract, lamellar cataract, blue dot cataract, sutural cataract, or posterior subcapsular cataract.[1-6] However, our patient presented with an unusual type of developmental cataract which we label as “Mercedes-Benz logo” cataract [Figure 1]. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Khokhar SK, Pillay G, Dhull C, Agarwal E, Mahabir M, Aggarwal P. Pediatric cataract. Indian J Ophthalmol 2017;65:1340-9. 2. Jinagal J, Gupta G, Thattaruthody F. Sutural cataract. Indian J Ophthalmol 2018;66:853. 3. Fard AM, Pourafkari L, Nader ND. Bilateral sutural cataract. QJM 2015;108:987. 4. Parikh RN, Kloek CE. Congenital lamellar and sutural cataract in a patient with type 2 diabetes. JAMA Ophthalmol 2022;140:e222158. 5. Ghadfan FE, Al‑Mesfer S, Khan AO. Cerulean (“blue‑dot”) cataract. J Pediatr Ophthalmol Strabismus 2009;46:190. 6. Gerth-Kahlert C, Michels R, Funk J, Gautschy U. Congenital lamellar cataract. JAMA Ophthalmol 2014;132:1122. Developmental cataracts are amongst the leading causes of treatable childhood blindness. There can be various morphologies of developmental cataract. We present one such unusual morphology of developmental cataract in a 18-year-old female. Keywords: Cataract, developmental cataract, lamellar cataract, sutural cataract Address for correspondence: Dr. Neiwete Lomi, Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi ‑ 110 029, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Sidhu N, Lomi N, Tandon R. Mercedes-benz logo cataract. Delhi J Ophthalmol 2023;33:333. Mercedes-Benz Logo Cataract Navneet Sidhu, Neiwete Lomi, Radhika Tandon Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_150_23 Submitted: 12-Sep-2023 Revised: 18-Sep-2023 Accepted: 20-Sep-2023 Published: 15-Jan-2024 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 333 Figure 1: Developmental cataract with the appearance of “Mercedes– Benz” logo

334 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow Photo Essay A 32-year-old man presented with a decrease in vision in his left eye for 1 month. The best-corrected visual acuity was 6/24. Fundus examination showed dilated and tortuous veins in the superotemporal quadrant [Figure 1a]. Widefield optical coherence tomography showed serous macular detachment with intraretinal fluid [Figure 1b]. Fundus fluorescein angiography showed early hyperfluorescence and leakage in the late phase [Figure 1c and d]. The size of Abstract The present report aims to describe the use of multimodal imaging in a rare case of Angiomatous Retinae which helps in its diagnosis, prognosis and plan management. Keywords: Angiomatous retinae, capillary hemangioma, tortuous veins Retinal imaging in Angiomatous Retinae Deeksha Singh1 , Anamika Nath1 , Divya Swamyraj1 1 Department of Retina and Vitreous Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Puducherry, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_137_23 Address for correspondence: Dr. Deeksha Singh, Aravind Eye Hospital, Cuddalore Road, Thavalakuppam, Puducherry ‑ 605 007, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Singh D, Nath A, Swamyraj D. Retinal imaging in angiomatous retinae. Delhi J Ophthalmol 2023;33:334-5. Submitted: 18-Aug-2023 Revised: 01-Oct-2023 Accepted: 30-Oct-2023 Published: 15-Jan-2024 the lesion was <2.5 Disc diameter (DD); hence, focal laser and antivascular endothelial growth factor were planned. At 2‑month follow‑up, a significant reduction was seen in optical coherence angiography, and the best-corrected visual acuity improved to 6/9. The reason for taking this picture is that it is a striking picture that we rarely see in the outpatient department (OPD). It is Figure 1: (a) Widefield fundus photograph shows dilated and tortuous vessels of angiomatous retinae. (b) Widefield optical coherence tomography shows neurosensory detachment with intraretinal fluid. (c) Early hyperfluorescence on fundus fluorescein angiography. (d) Leakage of dye in the late phase of angiogram a b c d

Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 335 Singh, et al.: Glimmering angiomatous retinae different from the bulk images such as diabetic retinopathy, hypertensive retinopathy, central serous chorioretinopathy, and age-related macular degeneration which we generally see in the retina OPD. This is a benign retinal hamartoma that may be associated with Von Hippel–Lindau (VHL) disease. The incidence of isolated retinal capillary hemangiomas is unknown; however, the birth incidence of VHL is approximately 1 in 36,000. This is usually detected by the second or third decade of life; however, it has been reported from birth to 80 years of age.[1,2] It can spontaneously regress in a few cases. Associated complications are – exudative retinal detachment, formation of hard exudates in the macula, vitreous hemorrhage, epiretinal membrane, and neovascular glaucoma. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Daniels AB, Chang EY, Chew EY. The New 2020 Guidelines for Retinal Surveillance of Patients with Von Hippel – Lindau Disease: New Approach, New Scope, New Guidelines. Presented at the Retina Society Annual Meeting, London; 2019. 2. Retinal Haemangioma. American Academy of Ophthalmology; 2017. Available from: www.AAO.org.

Letter to Editor Dear Editor, We read with keen interest the recently published study titled “A Study to Determine if the Incidence and Risk Factors of Retinopathy of Prematurity Have Changed During the COVID Period in a Pediatric Care Tertiary Hospital” by Wadhwani et al. [1] We want to congratulate the authors for their valuable contribution to the field of ophthalmology, particularly in illuminating the knowledge levels of retinopathy of prematurity (ROP) in preterm infants during the COVID-19 pandemic. While the study offers valuable insights into the impact of the pandemic on ROP cases, while we appreciate the significance of the study, we would like to provide some feedback and recommendations that we believe would enhance the comprehensiveness and impact of this research: The study indicates an increase in ROP incidence during the COVID‑19 period but does not provide specific data for comparison with the pre-COVID-19 period. Without pre-COVID-19 data for comparison, it is challenging to determine whether the observed increase in ROP cases during the pandemic is statistically significant or a continuation of existing trends.[2] The study identifies several risk factors associated with ROP, such as respiratory distress syndrome, anemia, and apnea of prematurity, but does not establish causation. While associations between risk factors and ROP are valuable, it is essential to investigate whether these factors directly contribute to ROP development or are merely correlated with it. Establishing causation can inform preventive strategies.[3] The study mentions systemic risk factors associated with ROP but lacks detailed information about these factors or their prevalence in the study population. Comprehensive data on systemic risk factors, their severity, and prevalence in the study population would enhance the understanding of their impact on ROP development.[3] Long-term follow-up data are crucial to assess the lasting impact of ROP and identify potential late-onset complications, which may not be evident in the short term.[4] The study suggests that delayed screening was due to COVID-19 restrictions, but it does not investigate specific reasons for these delays. Understanding the underlying causes of delayed screening is essential to developing targeted interventions and improving the timeliness of ROP diagnosis and treatment.[5] The study briefly mentions the use of telemedicine during the pandemic but does not explore its effectiveness in ROP screening. Analyzing the role and impact of telemedicine in ROP screening can provide valuable insights into its potential as an alternative or complementary approach to in-person evaluations. The study primarily focuses on medical aspects but does not consider the experiences and perspectives of parents and families of preterm infants during the pandemic. Qualitative data capturing the views and challenges faced by parents and families can offer a holistic understanding of the impact of COVID-19 on ROP care and inform patient-centered interventions. We believe that acknowledging and addressing this gap could not only enhance the quality and impact of research in this field but also benefit the broader scientific community and those who rely on this knowledge for decision-making and practice. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Mahendra Singh1 , Suraj Kumar Chaurasiya1 , Jayant Jha2 , Radhika Pushkar3 1 Department of Optometry and Vision Science, CL Gupta Eye Institute, Moradabad, Uttar Pradesh, 2 Department of Optometry Services, SJ Vision Foundation, Muzaffarpur, Bihar, 3 Department of Optometry and Vision Science, Uttaranchal (P.G.) College of Bio‑Medical Sciences and Hospital, Dehradun, Uttarakhand, India Address for correspondence: Prof. Mahendra Singh, Department of Optometry and Vision Science, CL Gupta Eye Institute, Ram Ganga Vihar Phase II (Extn.), Moradabad ‑ 244 001, Uttar Pradesh, India. E‑mail: [email protected] References 1. Wadhwani M, Kumar A, Jajoo M. A study to determine if the incidence and risk factors of retinopathy of prematurity have changed during the COVID period in a pediatric care tertiary hospital. Delhi J Ophthalmol 2023;33:87-92. 2. O’Connell NS, Dai L, Jiang Y, Speiser JL, Ward R, Wei W, et al. Methods for analysis of pre-post data in clinical research: A comparison of five common methods. J Biom Biostat 2017;8:1‑8. 3. Fortes Filho JB, Eckert GU, Valiatti FB, Dos Santos PG, da Costa MC, Procianoy RS. The influence of gestational age on the dynamic behavior of other risk factors associated with retinopathy of prematurity (ROP). Graefes Arch Clin Exp Ophthalmol 2010;248:893-900. 4. Stahl A, Bründer MC, Lagrèze WA, Molnár FE, Barth T, Eter N, et al. Ranibizumab in retinopathy of prematurity – One-year follow-up of ophthalmic outcomes and two-year follow-up of neurodevelopmental Incidence and Risk Factors of Retinopathy of Prematurity Have Post COVID Period 336 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Letter to Editor outcomes from the CARE-ROP study. Acta Ophthalmol 2022;100:e91-9. 5. Zepeda-Romero LC, Meza-Anguiano A, Barrera-de León JC, Angulo-Castellanos E, Ramirez-Ortiz MA, Gutiérrez-Padilla JA, et al. Case series of infants presenting with end stage retinopathy of prematurity to two tertiary eye care facilities in Mexico: Underlying reasons for late presentation. Matern Child Health J 2015;19:1417-25. This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. How to cite this article: Singh M, Chaurasiya SK, Jha J, Pushkar R. Incidence and risk factors of retinopathy of prematurity have post COVID period. Delhi J Ophthalmol 2023;33:336-7. © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_165_23 Submitted: 07-Oct-2023 Accepted: 30-Oct-2023 Published: 15-Jan-2024 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 337

Omnibus Humans Way back in 1972 as a house officer in Ophthalmology at Rohtak Medical College, I was permitted to operate on my very first cataract. The Registrar (Senior resident) supervised me as for this Intracapsular Cataract Extraction of bilateral hypermature cataracts I operated on both eyes. Postoperatively, during ward rounds of 70 beds in eye ward (septic and nonseptic zones) conducted by the head of department, trailed by an army of 20 doctors. And nurses, I cut away from the caravan at the exit of the ward. I was beside myself with excitement and anticipation as I fetched both a +9D and +10D lens and a trial frame to check the vision of my first operated patient. In that era for an aphakic patient, the vision of finger counting at 1 m was considered a good outcome. Lo and behold! With a +10D lens, he could see all around and recognized his 20-year-old daughter. He was perplexed, getting his sight back after almost 1.5 years of blindness and started laughing and crying at the same time and wept holding the face of his daughter. The daughter pointed at me as the operating surgeon who had given him his sight back and he fell on the floor of the corridor and touched my feet with respect and gratitude. I too cried tears-of happiness, excitement, and satisfaction. This moment changed my life-I decided right there that I must become an ace eye surgeon in order to repeatedly experience the gratifying sight of restored vision in the blind and semi-blind. I subsequently did postgraduation (DOMS) in Sitapur, U. P., which was then one of the largest eye hospitals with 2000 beds. It had a teaching branch named National Institute of Ophthalmic Research. We students used to stand around consultants examining 600 patients in a single day. We learned retinoscopy with planoconcave mirror and light bulb (streak was unavailable). The pupils of all the patients were dilated and we students had to write retinoscopy findings and fundus findings of all patients. By the age of 23 years, I was considered an expert in retinoscopy and direct ophthalmoscopy with a hand‑held pocket ophthalmoscope (Heine, German made costing Rs. 600 or 800). In the evening, not as mandated duty, but as a student keen to learn, I used to visit indoor patients of cataract to be operated next day. I used to talk to the patients inquiring after them and their families, emphasizing the value of education for their children. Since I was myself a farmer’s son, I became a role model for these villagers. Next, I would do a complete ophthalmic examination from lids to retina using a +13 D lens and loupe (44 D) and made my diagrams. I used to make a diagram of the eye in my copy explaining the patient and his/her attendant the anatomy of the eye and how his lens which had become opaque shall be removed in toto and then postoperative a +10 D glass will help them see. Many villagers were very scared of eye surgery. In fact, one patient in our neighboring village aged 105 years very healthy and doing 50 sit-ups a day refused to get operated on because of the fear of injection and died blind before my next visit to the village. To allay fears, I used to explain to the patients by bringing the patient’s finger to palpate the area near the eye where the first anesthetic injection would be given. I would also explain the entire procedure from retrobulbar injection, superior rectus suture (no speculum was used) then the opening of eye incision, removal of cataractous lens (Safed motia), and eye suturing. Postoperative rounds had a special flavor. Around 80–100 patients were inspected daily by the unit head/consultant, followed by DOMS students, nurses, a ward boy carrying drugs in a trolley slung from his neck (much like a snack seller in a stadium) and an attendant running ahead opening the bandages. Patients were kept in ward for 9 days and discharged with postoperative meds explained to the attendants. Many patients on the 1st postoperative day 3%–4% had iris prolapse, which had to be reposited, adding to the OT list. On my request, the CMO started giving 2 additional corneal sutures around at 10 and 2 o clock. I had the satisfaction of noting that Iris prolapses reduced to <1%. Over the years, many patients or their attendants came to see me at hostel for their problems if any. I was ever ready to offer counselling, visit their patients, hold their hands, and reassure them. I believed “High Touch Ophthalmic Care was as important as High‑Tech Ophthalmic Care.” After returning from an instructive WHO fellowship in 1993 at Moorfield Eye Hospital London (U. K) I worked at Nath Sect Muth outside Rohtak city. I earned a lot of affection from the patients and their families because of my soft skills and “high touch” method of dealing with the patients. As a registrar, I did retinoscopy, refraction, and direct ophthalmoscopy. All registrars at Medical College Rohtak were so good in retinoscopy that we would do retinoscopy even with slightest of red reflex/glow seen in immature cataract patients. All our teachers and students were down to earth with no high-tech gadget purchased as we along with ear, nose, and throat were considered minor departments. I believe General Ophthalmologists who can conduct cataract, glaucoma, squint, ptosis, and other basic surgeries are required at Tehsils and District levels. Today, I find that super-specialization/Balkanization of medical education and practice has reduced the standing of many fine comprehensive ophthalmologists in India. Retina, orbit, and oncology can Art of Ophthalmology - Glimpses From a Journey of 51 years 338 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Malik: The Art of Ophthalmology – Glimpses from a Journey of 51 Years certainly be put in the category of super specialty or any other course defined by National Medical Council (NMC). Right now, we do not have any formally recognized super specialty course or degree in ophthalmology. As head of department at Safdarjung Hospital we got one of the first phaco machines of the country. The Phaco machine (Oertli made in Switzerland) was the size of Maruti 800 engine, gifted to us by the WHO. When we were just about getting good enough in phacoemulsification, Professor Blumenthal of Israel demonstrated his MINI-NUC technique of Small incision cataract surgery (SICS) with anterior chamber maintainer at the clinic of Dr. Arun and Dr. Reena Sethi. I was so impressed with this technique which operated cataracts efficiently without needing an expensive phaco machine like the one we had. It fitted in strongly with my ideal of a “high touch and not high tech” way of life suited to a country like ours where the majority did not have access to expensive resources. Ignoring our phaco machine somewhat, we started doing SICS in large numbers, delivered lectures at various conferences advocating SICS as a very good surgery giving not only comparable but better results as compared to phaco in certain cases. Istill recollect our first SICS instruction course at AIOS Conference in Kolkata. The hall was full to the brim with delegates who were spilling over each over and filling every inch of the hall including floor, aisles, and even the floor around the podium. I was truly an unforgettable sight and the speakers included Dr. Ruchi Goel, Dr. Seema Agarwal, Dr Neeraj Awasthi, and myself. My Personalized Art of Ophthalmology Along with building surgical skills, I have always worked on building my “touch skills.” As in being able to touch the very heart of my patients. I did this in three chief ways: • High-quality communication: talking, patiently repeating, really listening, and taking care of each complaint of the patient and looking after them holistically paying attention to their systemic diseases, their mental health and at times even escorting them to other doctors • Distributing and sharing sweets, chocolates, toffees kept in the pocket of my apron with my patients and attendants. This was my way of cheering up many a tired or grumpy soul and exhibiting warmth and hospitality • Giving my time generously: I would talk to lonely or anxious patients about their village or locality, local food, health of patient’s family members, and inquire after the health of most patients that passed me by in the corridors. Conclusion Remember the famous quote by George Bernard Shaw – “The greatest problem in communication is the illusion that it has been accomplished.” Treatment these days is high-tech and low-touch, leading to costly treatment and an increasing threat of malpractice suits. The need of the hour is to have active and greater involvement of patients in decision-making. Important additions to our “high tech” practice can include important “touches” such as: • A warm connection – Established by smiling, greeting, shaking hands with a patient • A calming environment – Created for the patient by truly listening, understanding, reassuring, and caring • Easing the patient’s comprehension–By having conversations and giving patients leaflets in their vernacular language. We have interpreters for “medical tourism” for foreign patients, then why not for local patients? A generous use of models and colorful posters facilitates easy understanding. Last but not least: try and end every appointment and interaction on a positive note if possible. Remember, a good doctor treats the disease, but a great doctor treats the patient! K. P. S. Malik Department Ophthalmology, Ayushman Multispecialty Hospital, Dwarka, New Delhi, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. How to cite this article: Malik KP. Art of ophthalmology – Glimpses from a journey of 51 years. Delhi J Ophthalmol 2023;33:338-9. Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_194_23 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 339

Abstract Theme Section History Chief complaints Whitish opacity, not able to visualize objects, failure to make eye-to-eye contact, wandering eye movements, poor school performance, and presence of squint or nystagmus have to be elicited. History of polyopia or diplopia has to be taken in elder children as this may be the presenting symptom in cases of subluxated lens. Age of onset of symptoms The earlier the age of onset of complaints, the more will be the amblyopia for diseases like cataracts/squint/ptosis or any other diseases resulting in stimulus deprivation. Patients with developmental cataracts and patients who develop squint at a later age have a better prognosis. Duration of symptoms The longer the duration of symptoms, the poorer the prognosis. Laterality Certain diseases such as persistent hyperplastic primary vitreous (PHPV) and coats’ disease are more commonly unilateral. Diseases such as cataracts, congenital glaucoma, microspherophakia, and keratoconus are commonly bilateral. Prognosis is usually worse in unilateral cataracts when compared to bilateral cataracts due to late detection, development, and irreversible deprivation syndrome. Association with nystagmus The presence of nystagmus is suggestive of poor fixation and therefore poor visual prognosis. In a patient with a squint, it is important to ask if the child freely alternates eyes or squint is present in one eye as this will affect the prognosis of the case. In patients with intermittent divergent squint, the duration of deviation of eyes through the day should be noted. Antenatal history Antenatal history regarding any rashes/fever during pregnancy is important to rule out toxoplasmosis, other diseases(syphilis and hepatitis B), rubella, cytomegalovirus, and herpes simplex (TORCH) infections.[1] Natal history It includes whether the child was born at full term/preterm, mode of delivery (normal vaginal delivery/lower‑segment cesarean section), whether the child cried immediately after birth or not, the immunization status of the child, birth weight, any history of oxygen administration, and history of convulsions. The comprehensive pediatric eye and vision examination helps in the evaluation of the structure, function, and health of the eyes and visual system. The parent/caregiver should accompany the child into the examination room for making the examination of a child easier. During the examination, information that is obtained is collated to explain symptoms reported by the patient and/or parent/caregiver and diagnose their cause. A comprehensive pediatric eye examination provides the means to identify the presence of other ocular or systemic conditions that may exist with or without symptoms. Keywords: Childhood blindness, congenital cataract, examination under anesthesia, pediatric eye examination Address for correspondence: Dr. Siddharth Madan, Assistant Professor, Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi -110002, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Baindur S, Madan S, Soman A, Sharma U, Sethi M, Garg K, et al. Pediatric eye examination: An overview. Delhi J Ophthalmol 2023;33:340-8. Pediatric Eye Examination: An Overview Siddharth Baindur, Siddharth Madan, Aparna Soman, Umesh Sharma, Meghna Sethi, Kashish Garg, Chitra Ogio, Anju Rastogi Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_188_23 Submitted: 08-Dec-2023 Revised: 18-Dec-2023 Accepted: 26-Dec-2023 Published: 15-Jan-2024 340 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Baindur, et al.: Pediatric eye examination Postnatal history The history of neonatal intensive care unit stay is relevant. Developmental history It includes whether the child has attained the milestones as per his/her age or it was delayed. Delayed milestones can mean delayed visual development too. A history of delayed developmental milestones is suggestive of an underlying systemic disorder, and it makes contact lens or spectacle tolerance difficult thus impairing visual prognosis. Family history includes history of consanguinity and history of ocular illness in parents or siblings. Other history including history of any known allergy, any medication use, immunization status, and previous consults should be noted. Systemic history including history of neuropsychological symptoms, history of seizures, and congenital abnormalities like cerebral palsy and Down syndrome needs to be elicited. General Examination A full-body examination should be done to rule out any association with a syndrome resulting or associated with the ocular complaints. Listed below are some common syndromes and their systemic features: Features of Down syndrome include distinctive facial features, such as a small head, flattened face, short neck, protruding tongue, and palpebral fissures in the form of upward slanting eyelids. Features of Marfan syndrome include a tall and slender build, disproportionately long arms, legs, and fingers, a breastbone that protrudes outward or dips inward, a high, arched palate and crowded teeth, an abnormally curved spine, and flat feet. Ocular examination To conduct a comprehensive examination of a child, the minimum required tools include a torch, a direct ophthalmoscope, and an indirect ophthalmoscope. It is ideal to assess the child when they are in a comfortable, awake, and alert state, especially for small children who can be positioned on their mother’s lap or shoulder. Utilizing diffuse lighting is essential during the examination, as intense light can startle the child, making further evaluation challenging. To engage the child and maintain their cooperation during the examination, items such as toys, pictures, candies, mobile phones, or any captivating objects can be employed. For a thorough assessment of the fundus and anterior segment, it is recommended to perform these examinations when the child is asleep or sedated. In specific cases, an examination under anesthesia may be required to achieve a comprehensive evaluation. A comprehensive eye examination should include: 1. Visual acuity 2. Ocular motility and squint examination 3. Slit-lamp examination 4. Pupils 5. Intraocular pressure (IOP) 6. Cycloplegic refraction 7. Dilated fundus examination. Visual acuity assessment Both uniocular and binocular Visual acuity assessment is performed by age-appropriate vision screening methods.Vision screening stands as the foremost priority in pediatric eye examinations, yet it can pose a challenge when dealing with preverbal children. Ophthalmologists often resort to indirect methods for assessing vision in such cases. These indirect methods involve observing fixation patterns, pupillary light reflexes, and the child’s general response to their surroundings. Fixation reflex typically emerges shortly after birth in most infants and becomes established in all by the age of 3 months. When a child demonstrates central, steady, and sustained fixation with the ability to freely alternate between both eyes, it suggests good and equal vision in both eyes. Conversely, if a child exhibits a preference for fixing with one eye or resists occluding one eye, it implies that the fellow eye may have poor vision. In infants, the Bruckner test, optokinetic nystagmus[optokinetic drum; Figure 1], cover test, Tellers Acuity Cards[Figure 2], and preferential looking test are useful methods of assessing visual acuity. Between the age group of 1 and 2 years, Worth’s ivory ball test[Figure3], screening test for young children, Retarded test [Figure 4], and Cardiff Acuity Cards [Figure 5] are used. LEA Symbols® Test [Figure 6] is useful in the age group between 2 and 3 years. Allen’s picture cards [Figure 7] and Lippman’s HOTVtest are used in children aged 3–5 years. In older children over 5 years, Tumbling E, Landolt’s broken ring, Snellen’s chart [Figure 8], and logMAR visual acuity testing [Figure 9] methods are used to document visual acuity. • External examination should include looking for head posture and oculofacial anomalies. Torch light examination of the external ocular structures should be done, such as the lids, lacrimal system, cornea, conjunctiva, sclera, and iris. Head posture can be best assessed by observing a child while he/she is interacting with parents • Ocular motility can be assessed using objects or toys that will grab a child’s attention. The Hirschberg test or a cover Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 341 Figure 1: Optokinetic drum

Baindur, et al.: Pediatric eye examination test should be performed in suspected cases of ocular misalignment. It is important to differentiate pseudostrabismus from true strabismus. The presence or absence of nystagmus should be noted as this is an indicator of poor vision possibly due to some congenital ocular pathology • The pupillary examination is a must and should include an assessment of size, shape, color of the iris, pupillary reactions (direct and consensual light reflex and relative afferent pupillary defect [RAPD]), and inter‑eye asymmetry in pupil size. Abnormal pupillary reflexes or asymmetry in pupil size may be suggestive of an underlying central nervous system or ocular pathology, thus necessitating urgent imaging and referral to the neurologist. The presence of RAPD implies a posterior segment pathology. Abnormal pupillary reflexes or asymmetry in pupil size may be suggestive of an underlying central nervous system or ocular pathology. The abnormal shape of the pupil is suggestive of underlying ocular pathology. For example, a keyhole pupil in iridofundal coloboma. In cases of cataracts, maximum pupillary dilatation should be checked • Ocular motility and squint examination: Strabismus and nystagmus should be specifically looked for. Strabismus examination involves the Hirschberg test, cover uncover test, alternate cover test, and extraocular muscle movements. The degree of squint and fixation patterns (central, steady, maintained, or not) has to be checked. Central fixation suggests that the fovea is the fixing point. Asteady fixation suggests that there is no component of nystagmus, and a fixation that is maintained suggests that there is no squint or that the eye has a reasonably good visual acuity. Strabismus can be seen in children when they have poor vision in one eye due to any cause. In patients with cataracts, the presence of either strabismus or nystagmus indicates that the cataract is visually significant • Lacrimal apparatus examination: Regurgitation on pressure over the lacrimal sac area has to be carried out in all cases to rule out nasolacrimal duct obstruction • Red reflex (Bruckner’s reflex) should be assessed by distance direct ophthalmoscopy in a darkened room or in ambient light. Amarkedly diminished reflex, absence of red reflex, or presence of a white or yellow reflex (leukocoria), or asymmetry of the red reflexes is abnormal and indicates some underlying pathologies.[2] A markedly diminished 342 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 2: Teller Acuity Cards Figure 3: Worth’s ivory ball test Figure 4: Screening test for young children and retarded test Figure 5: Cardiff Acuity Test

Baindur, et al.: Pediatric eye examination reflex, absence of red reflex, or presence of a white or yellow reflex (leukocoria), or asymmetry of the red reflexes, is abnormal and indicates some underlying pathologies. Causes of leukocoria include: • Abnormalities in the cornea (corneal opacity) • Vitreous causes (vitreous hemorrhage and PHPV) • Retinal diseases (Coats’ disease, retinoblastoma, familial exudative vitreoretinopathy, retinal detachment, retinopathy of prematurity [ROP], and coloboma) • Tumors such as medulloepithelioma and retinal astrocytoma. Distant direct ophthalmoscopy is a very useful bedside technique of pediatric eye examination as it provides a lot of clinically relevant information regarding media opacities, media clarity, assessment of a visually significant pediatric cataract, and posterior capsular opacification requiring surgery. • Detailed ocular examination should be performed by slit‑lamp biomicroscopy (whenever applicable and possible). Slit‑lamp biomicroscopy is useful to assess lens and cataract morphology in cases of congenital/ developmental cataracts (zonular, blue dot, lenticonus, and polar cataracts). If the child is unable to sit at the slip lamp, a direct ophthalmoscope should be used to asses details as much as possible. When a child is not cooperative for slit-lamp examination, an examination under general anesthesia should be done. However, the same will not be possible in the examination by the resident but can be mentioned during viva • Examination of the cornea: The following points have to be noted: 1. Microcornea (Nance–Horan syndrome) 2. Increased corneal diameter may be seen in megalocornea or buphthalmos 3. Central corneal opacity (Peters’ anomaly and Alport syndrome) 4. Haab’s striae (congenital glaucoma) 5. Posterior embryotoxon (Peters’ anomaly) 6. Perforation and scarring (traumatic cataract). • Examination of the anterior chamber 1. AC depth 2. Cells or flare (uveitic cataract) 3. Presence of hyphema (posttrauma or in cases of juvenile xanthogranuloma) 4. Presence of hypopyon (seen in cases of endophthalmitis, corneal ulcers, or some cases of retinoblastoma) 5. Neovascularization of the iris Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 343 Figure 6: LEA Symbols Test Figure 7: Allen’s picture cards Figure 8: Snellen chart Figure 9: LogMAR chart

Baindur, et al.: Pediatric eye examination 6. Presence of vitreous in AC (traumatic cataract). • Examination of the Iris 1. Iridodonesis can be seen in cases of subluxated lens or aphakia 2. Posterior synechiae (seen in cases of trauma or uveitis) 3. Iris dystrophy (TORCH infection) 4. Iridohyaloid blood vessels (persistent fetal vasculature [PFV]) 5. Aniridia 6. Persistent pupillary membrane. • Examination of the lens 1. Morphology of cataract 2. Subluxation/dislocation 3. Phacodonesis 4. Microspherophakia (Weill–Marchesani syndrome). • Examination of the posterior capsule 1. Capsule PC tear (traumatic cataract) 2. Preexisting posterior capsular defect(PFV, lenticonus, lentiglobus, and posterior polar cataract) 3. Posterior capsular calcification. • A dilated fundus examination should be done for every child to evaluate the posterior segment. It is usually done with the help of an Indirect Ophthalmoscope. If the child is not cooperative for examination, retractors can be used after putting a drop of proparacaine in both eyes. Retinal anomalies in primary care practice include a swollen optic disc, which may be physiological or pathological swelling. The optic nerve drusen gives a swollen appearance to the optic disc (pseudopapilledema). Tilted disc and situs inversus usually occur in patients with high myopia. Bergmeister’s papilla consists of remnants of hyaloid vessels and glial supporting structures and can be confused with raised neural tissue on the nerve head. “Choked disc” may be caused by papilloedema, which is due to a passive swelling of the optic nerve head or papillitis‑active inflammation of the optic nerve head. Due to the similar ophthalmoscopic appearance of the two entities, investigations provide a definite diagnosis. Vision will usually be normal in papilledema. Peripheral and other retinal conditions include choroidal nevus, congenital hypertrophy of the retinal pigment epithelium, coloboma, Toxocara, toxoplasma chorioretinitis, and an operculated tear. All these conditions need to be identified after a comprehensive examination of the retina using scleral indentation wherever indicated • Age-appropriate cycloplegic refraction using retinoscopy should be performed in all cases. The refractive error beyond 4D of myopia, 5D of hyperopia, and 2D of astigmatism can lead to amblyopia. Spherophakia and developmental cataracts can present with progressively increasing myopia. Subluxation can present with high hyperopia if the lens is shifted away from the pupillary area and the child is essentially aphakic. If one eye is pseudophakic, then the refractive status can help in the calculation of IOP power for the other eye • Stereoacuity assessment (optional) may be done at least once in cooperative patients[3,4] • Assessing IOP is not required in children unless glaucoma is suspected (e.g., presence of buphthalmos, epiphora, photosensitivity, and corneal clouding) or steroid therapy is given. Depending on the age and cooperation of a child, it may be done using the noncontact tonometer or rebound tonometer or under sedation/anesthesia with the help of a Perkins tonometer or Tono-Pen.[5] Applanation tonometry can be done if the patient is cooperative. Digital tensions have to be taken if the child is not cooperative. IOP can be raised in cases of traumatic cataracts. IOP also needs to be assessed to rule out congenital glaucoma • Examination under anesthesia (EUA): This type of examination is required for younger children who cannot be seen at the slip lamp or are uncooperative for examination. EUA allows for detailed eye examinations and allows doctors to perform investigations. EUA is commonly done: • For congenital glaucoma, the following parameters are evaluated 1. IOP measurement using the Schiotz tonometer and Perkins tonometer 2. Retinoscopy for refraction 3. Corneal diameter using calipers 4. Central corneal thickness measurement by pachymetry 5. Axial length (AL) measurement using A‑scan 6. Direct gonioscopy using Koeppe lens or Swan–Jacob lens 7. Indirect/direct ophthalmoscopy for optic disc evaluation and complete fundus examination 8. Microscopic examination for corneal opacities, Haab’s striae, or breaks in Descemet’s membrane 9. In children, postglaucoma surgery attention should be paid to bleb health, tube position, corneal clarity, and myopic shift. • In trauma/chemical injury cases, staining of the cornea and conjunctiva (ocular surface) can be done using a sterilized 0.1% sodium fluorescein strip to look for associated epithelial defects. Eversion of the lids, including double eversion of the upper lid must be done in cases of suspected foreign bodies or chemical injuries[6] Other indications include examination for anterior segment dysgenesis, congenital corneal opacities, retinoblastoma, and posterior segment examination, including dilated fundus evaluation (children with high myopia for peripheral retinal degenerations/treatment of retinal holes/lattices/ROP using laser indirect ophthalmoscopy) and other ocular tumors. • Ocular investigations. Ultrasonography B-scan: Rule out posterior segment pathologies such as retinal detachment, fundal coloboma, and retinoblastoma in case of traumatic cataracts to rule out intraocular foreign body. Ultrasound biomicroscopy: Noninvasive imaging technique that helps in identifying anterior PFV, posterior capsular 344 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Baindur, et al.: Pediatric eye examination defect, and posterior polar cataract preoperatively. Pediatric cataract is a common PG case given, and detailed information regarding biometry is a must. The following investigations are carried out. Keratometry It is done using handheld keratometers or automated keratometers. In the case of an uncooperative child, standard K values of 43.00 D can be used; K values steeply reduce in the first 6 months (−0.40 D/month), −0.14 D/month in the next 6 months, and −0.08 D/month in the 2nd year, reaching the adult range at about 3 years of age. Axial length AL measurement is better estimated with immersion A-scan than indentation A-scan. Predicting AL growth and hence the myopic shift is difficult after pediatric cataract surgery. AL increases rapidly in the first 6 months (0.46 mm/ month), then has a relatively slower (infantile phase) growth (0.15 mm/month) till 18 months, followed by a slow (juvenile phase) growth (0.10 mm/month). Intraocular lens power calculation Intraocular lens (IOL) can be implanted in eyes with Axial Length >17 mm and corneal diameter >10 mm. The initial desired refractive outcome after IOL implantation is moderate hypermetropia to avoid an undesired myopic shift in adulthood. Dahan and Drusedau suggested an undercorrection of 20% in children <2 years and 10% in children between 2 and 8 years. Enyedi suggested postoperative target refraction to be used for IOL power calculation according to age (age + postoperative refraction = 7). SRK/T and the Holladay 2 formulae have been shown to have the least predictive error. For a comprehensive pediatric eye examination at a specialized facility, various indications for referral to a pediatric ophthalmologist are summarized in Table 1. [7] Age-appropriate methods for pediatric vision screening and criteria for referral (Table 1) are adapted from the American Academy of Ophthalmology Preferred Practice Patterns 2017.[7] Timing of examination and screening Pediatric vision screening and eye examination should be timed to maximize the detection of ocular morbidities and be able to provide timely intervention. Table 2 gives the guidelines for vision screening and comprehensive eye examination in children. Table 2: Guidelines for vision screening and comprehensive eye examination in children Age Timing Neonates high-risk cases (premature, low birth weight, and Down syndrome) At discharge and at 1 month of age Birth–3 years Vision screening by an ophthalmologist at least once 3–5 years Comprehensive eye examination by an ophthalmologist at least once 5–8 years Comprehensive eye examination by an ophthalmologist at least once Table 1: Age-appropriate methods for pediatric vision screening and criteria for referral Method Indications for referral Recommended Age Newborn-6 months 6-12 months 1-3 years 3-4 years 4-5 years Every 1-2 years after age 5 years Red reflex test Absent, white, dull, opacified, or asymmetric • • • • • • External inspection Structural abnormality (e.g., ptosis) • • • • • • Pupillary examination Irregular shape, unequal size, poor or unequal reaction to light • • • • • • Fix and follow Failure to fix and follow Cooperative infant ≥3 months • • Corneal light reflection Asymmetric or displaced • • • • • Instrument based screening Failure to meet screening criteria • • • • Cover test Refixation movement • • • Distance visual acuity (monocular) Worse than 20/50 either eye or 2 lines of differences between the eyes • • • Worse than 20/40 either eye • • Worse than 3 of 5 optotypes on 20/30 line, or 2 lines of difference between the eyes • Source: Hagan JF, Duncan PM, eds. 2017, Bright Futures: Guidelines for Health Supervision of Infants, Children and Adolescents. 4th ed.. Elk Grove Village, IL: American Academy of Pediatrics; 2017 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 345

Baindur, et al.: Pediatric eye examination Guidelines for vision screening and comprehensive eye examination in children are adapted from the National Consensus Statement regarding pediatric eye examination, refraction, and amblyopia management.[8] Special examination/tests Color vision Color vision screening is not mandatory; however, given an opportunity, it should be done in children at least once during their school years. Visual fields Routine visual field testing is typically not necessary. In the case of suspected visual field defects in children, confrontation visual field testing can be conducted. To assess the peripheral visual field in younger children, one can observe their response to the presentation of an object of interest in a specific gaze direction. If possible, the visual fields of children should be assessed with the method that is recommended for adults, i.e., automated static perimetry. For children who are too young to be tested with standard adult perimetry procedures, there are no widely available, quantitative perimetry techniques and, therefore, no standardized methods for evaluating disability related to restricted visual fields.[9] Imaging Imaging in the pediatric age group is indicated only in certain special situations. • Face photography helps to document and follow changes of facial or ocular structural abnormalities. They can be taken with a flash so that corneal reflexes are seen that can help to check for ocular alignment and differentiate pseudo from true strabismus • Family album tomography scan of old photographs may demonstrate a preexisting anomalous head or face tilting or turning that might reflect underlying well‑controlled strabismus • Anterior segment photography through a slit lamp for cataracts and other anomalies • Corneal topography to detect early changes related to keratoconus • Fundus photography may be needed for ROP screening and evaluation of torsion • Fundus photography can be done to assess objective ocular torsion[10] • Smartphone photography is a type of noncontact fundus photography. Newer generation smartphones are equipped with a high-quality optical system and a coaxial light source, which can be used to capture high-quality retinal images. The camera‘s coaxial flashlight and a handheld high-plus power lens create an indirect ophthalmoscopy-like optical system that is able to record high-resolution digital retinal images. The quick data transfer capability in smartphones can be utilized as an effective telemedicine tool to share and discuss cases in remote places, for example, screening for pediatric eye diseases such as ROP and diabetic retinopathy in children and adolescents[11] • Rarely, optical coherence tomography (OCT)‑based image analysis may be needed for optic nerve head assessment or abnormal retinal pathology • One limitation to the widespread use of retinal nerve fiber layer measurements in pediatric patients is the lack of a normative database that can be used for comparison, as the databases currently in use represent only white patients of at least 18 years of age • Flying baby spectral-domain-OCT provided a rapid and widely accessible imaging approach that overruled clinical findings and altered the classification and management of infants with advanced ROP. The methodology was suitable for outpatient settings with no risks associated with systemic anesthesia [Figure 10][12] • Fundus fluorescein angiography (FFA): FFA is useful in other pediatric retinal diseases such as retinopathy in type 1 diabetes, incontinentia pigmenti, Familial Exudative Vitreoretinopathy (FEVR), dyskeratosis congenital, and muscular dystrophy, which have a common presentation of peripheral retinal avascularity, progressive neovascularization, and early development of tractional retinal detachment. In Coats’ disease, FFA documents occurrence/progression of characteristic early telangiectasia, capillary nonperfusion areas, and leakage leading to subretinal exudation – all of which are difficult to detect and assess on indirect ophthalmoscopy. Visual Evoked Response The visual evoked response (VER) is a valuable tool for vision screening in infants and children facing developmental delays, but its interpretation requires careful consideration. Typically, this test is administered after sedation, involving the use of flash stimuli through VER goggles. It is particularly beneficial when dealing with cases 346 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 10: Flying baby Heidelberg Spectralis spectral‑domain optical coherence tomography. Images were acquired with the infant held in the “flying baby” position with the lid speculum under topical anesthesia only. The head was stabilized by gripping the angles of the mandible and additional support to the arms, and back was provided by an assistant

Baindur, et al.: Pediatric eye examination that present normal ocular findings but raise suspicions of cortical visual impairment. However, it is important to note that VER testing is not widely accessible and cannot be regarded as a standard component of a comprehensive pediatric eye examination.[13] Pediatric refraction Pediatric refraction is challenging since children have strong accommodation, are often uncooperative, and have a dynamic ocular system due to growth.[14] Timing of refraction It is recommended that all children undergo a vision assessment, including refractive testing if there are concerns about subnormal vision during preschool years, typically around the age of 3–4 before starting formal schooling. Early refraction evaluation is essential in cases where symptoms such as difficulty recognizing faces from a distance, a history of frequent falls, close-up activities like watching television or mobile devices, or specific clinical conditions are present. These clinical conditions may include a history of ocular surgery, especially for congenital cataracts, premature birth, perinatal complications, developmental delays, strabismus, or a family history of significant refractive errors. Pediatricians should be aware of the importance of timely referrals to an ophthalmologist in such cases, as high refractive errors can potentially lead to conditions such as squint or amblyopia and have a negative impact on the child’s normal growth and development.[15] Method of refraction The most reliable method for pediatric refraction is a thorough retinoscopy. Although there are handheld and portable autorefractors available nowadays, they can be useful only for initial screening purposes. However, their accuracy is often less reliable when dealing with toddlers and infants. When it comes to school-age children who can cooperate effectively, autorefractors can be employed, especially when combined with appropriate cycloplegia to ensure accurate results.[16] While noncycloplegic refraction may be used for screening purposes, it is not recommended for the final prescription due to the variability in refractive errors.[17] Cycloplegia, which involves temporarily paralyzing the eye’s focusing muscles, is essential to obtain a more accurate and stable prescription. Use of cycloplegia Atropine, homatropine, and cyclopentolate are commonly used cycloplegic agents in pediatric eye care, and they are Table 4: Guidelines for refractive correction in infants and young children Refractive errors (dioptres) Condition Age <1 year Age 1 to <2 years Age 2 to <3 years Age 3 to <4 years Isometropia (similar refractive error in both eyes) Myopia 5.00 or more 4.00 or more 3.00 or more 2.50 or more Hyperopia (no manifest deviation) 6.00 or more 5.00 or more 4.50 or more 3.50 or more Hyperopia with esotropia 2.00 or more 2.00 or more 1.50 or more 1.50 or more Astigmatism 3.00 or more 2.50 or more 2.00 or more 1.50 or more Anisometropia (without strabismus) Myopia 4.00 or more 3.00 or more 3.00 or more 2.50 or more Hyperopia 2.50 or more 2.00 or more 1.50 or more 1.50 or more Astigmatism 2.50 or more 2.00 or more 2.00 or more 1.50 or more Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 347 Table 3: Various drugs used for inducing cycloplegia in children Name of drug Age indicated Dose of instillation Ocular side effects[19] Systemic side effects[19] Atropine sulfate (1% ointment) <5 years TDS 3 days* Lid contact dermatitis, allergic conjunctivitis, and keratitis Dryness of secretions, fever, skin rash, irritability, tachycardia, and convulsions Homatropine hydrobromide (2% drops) 5–8 years One drop every 10 min for 3–4 times Similar to atropine but less severe Cyclopentolate hydrochloride (1% drops) 8–20 years One drop every 15 min for 3 times Tearing, allergic blepharoconjunctivitis, conjunctival redness, and increased intraocular pressure Irritability, drowsiness, ataxia, disorientation, incoherent speech, restlessness, and visual hallucinations Tropicamide (1% drops) Primarily a cycloplegic agent with a mydriatic action One drop every 15 min Allergic reactions, drowsiness, and irritation Phenylephrine (5%, 10% drops) Only used as mydriatic alone or in combination with tropicamide. Concentration reduced to half (2.5%) in neonates and young infants One drop every 15 min for 3–4 times Systemic hypertension and tachycardia if applied at 10% concentration or higher dosages TDS: Ter die sumendum

Baindur, et al.: Pediatric eye examination sometimes used interchangeably. Studies conducted in Indian populations have revealed variations in the cycloplegic efficacy of these drugs, with atropine and cyclopentolate being the most potent, followed by homatropine.[18] It is crucial to ensure effective cycloplegia, which can be verified by performing dynamic retinoscopy, while the child focuses on distant and near objects to obtain a consistent measurement. In cases of esotropia in children under 5 years of age, atropine is the preferred cycloplegic because it is necessary to neutralize the accommodative component of esotropia. The commonly used cycloplegic agents in the pediatric population are elaborated in Table 3. Prescribing spectacles There are different practices in different parts of the country about the minimum refractive error to be prescribed. Table 4 provides the guidelines for minimum refractive correction in infants and young children. Guidelines for refractive correction in infants and young children are adapted from the American Academy of Ophthalmology Preferred Practice Patterns 2017. Prescription in special conditions: • Prescription in pseudophakic and aphakic patients: Refractive error as assessed by retinoscopy should be prescribed immediately irrespective of age in pseudophakic and aphakic patients. Glasses should be prescribed for intermediate vision in children up to 2 years, and thereafter with the added demand for distance and near vision, bifocals are to be prescribed. Occlusion/ patching is needed in unilateral pseudophakia/aphakia or bilateral cases with unequal vision • Gross developmental delay, Down syndrome, or mental retardation: Refraction and prescription according to retinoscopy should be given as early as 6 months of age • ROP: Myopia tends to progress in cases of ROP, and prescription can be given according to retinoscopy as early as 6 months and repeated every 6 months • Esotropia–hyperopia of ≥+1.5D must be prescribed in children with esotropia. Overcorrection is permissible as long as the vision stays above 6/12. Bifocals are needed in cases with a high AC/A ratio. Weaning of glasses should start at the age of 7 years depending on the retinoscopy but a close watch on esotropia is needed, and weaning is stopped or reversed if esotropia recurs with glasses on weaning. Myopic children should be advised to reduce screen time and time spent on near activities (like reading) and increase outdoor activities.[20] Follow-up of a case of refractive error For children <3 years of age, refraction needs to be repeated at least once every 6 months, while for older children, it should be done on a yearly basis. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Vijayalakshmi P, Kakkar G, Samprathi A, Banushree R. Ocular manifestations of congenital rubella syndrome in a developing country. Indian J Ophthalmol 2002;50:307-11. 2. Khokhar S, Pillay G, Agarwal E. Pediatric cataract – Importance of early detection and management. Indian J Pediatr 2018;85:209-16. 3. Hussaindeen JR, Rakshit A, Singh NK, George R, Swaminathan M, Kapur S, et al. Prevalence of non-strabismic anomalies of binocular vision in Tamil Nadu: Report 2 of BAND study. Clin Exp Optom 2017;100:642-8. 4. Hussaindeen JR, Rakshit A, Singh NK, Swaminathan M, George R, Kapur S, et al. The minimum test battery to screen for binocular vision anomalies: Report 3 of the BAND study. Clin Exp Optom 2018;101:281-7. 5. Jethani J. Using the rebound tonometer to measure intraocular pressure in an anesthetized patient. Indian J Ophthalmol 2014;62:832. 6. Dhillon HK, Agarkar S, Vijaya L, Bhende M, Baskaran M, Jaichandran VV. Examination under anesthesia: Preferred practice. Indian J Ophthalmol 2023;71:3438-45. 7. Wallace DK, Morse CL, Melia M, Sprunger DT, Repka MX, Lee KA, et al. Pediatric eye evaluations preferred practice pattern®: I. Vision screening in the primary care and community setting; II. Comprehensive ophthalmic examination. Ophthalmology 2018;125:P184-227. 8. Saxena R, Sharma P, Pediatric Ophthalmology Expert Group. National consensus statement regarding pediatric eye examination, refraction, and amblyopia management. Indian J Ophthalmol 2020;68:325-32. 9. RashtriyaBalSwasthyaKaryakarm. Guidelines for Universal Eye Screening in Newborns Including Retinopathy of Prematurity. Ministry of Health and Family Welfare, Government of India; 2017. p. 37. Available from: https://rbsk.gov.in/RBSKLive/ 10. National Research Council (US) Committee on Disability Determination for Individuals with Visual Impairments; Lennie P, Van Hemel SB, editors. Visual Impairments: Determining Eligibility for Social Security Benefits. Washington (DC): National Academies Press (US); 2002. 4, ASSESSMENT OF VISION IN INFANTS AND CHILDREN. Available from: https://www.ncbi.nlm.nih.gov/books/NBK207548/ 11. Khanna RK, Pasco J, Santallier M, Pisella PJ, Arsene S. Objective ocular torsion outcomes after unilateral horizontal rectus surgery in infantile esotropia. Graefes Arch Clin Exp Ophthalmol 2018;256:1783-8. 12. Agarwal K, Vinekar A, Chandra P, Padhi TR, Nayak S, Jayanna S, et al. Imaging the pediatric retina: An overview. Indian J Ophthalmol 2021;69:812-23. 13. Cehajic‑Kapetanovic J, Xue K, Purohit R, Patel CK. Flying baby optical coherence tomography alters the staging and management of advanced retinopathy of prematurity. Acta Ophthalmol 2021;99:441-7. 14. Zheng X, Xu G, Zhang K, Liang R, Yan W, Tian P, et al. Assessment of human visual acuity using visual evoked potential: A review. Sensors (Basel) 2020;20:5542. 15. Monga S, Dave P. Spectacle prescription in children: Understanding practical approach of Indian ophthalmologists. Indian J Ophthalmol 2018;66:647-50. 16. Khurana R, Tibrewal S, Ganesh S, Tarkar R, Nguyen PT, Siddiqui Z, et al. Accuracy of noncycloplegic refraction performed at school screening camps. Indian J Ophthalmol 2018;66:806-11. 17. Guha S, Shah S, Shah K, Hurakadli P, Majee D, Gandhi S. A comparison of cycloplegic autorefraction and retinoscopy in Indian children. Clin Exp Optom 2017;100:73-8. 18. Khurana AK, Ahluwalia BK, Rajan C. Status of cyclopentolate as a cycloplegic in children: A comparison with atropine and homatropine. Acta Ophthalmol (Copenh) 1988;66:721‑4. 19. Major E, Dutson T, Moshirfar M. Cycloplegia in children: An optometrist’s perspective. Clin Optom (Auckl) 2020;12:129‑33. 20. Saxena R, Vashist P, Tandon R, Pandey RM, Bhardawaj A, Gupta V, et al. Incidence and progression of myopia and associated factors in urban school children in Delhi: The North India Myopia study (NIM Study). PLoS One 2017;12:e0189774. 348 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Abstract Theme Section Introduction Corneal cases in MS examinations can be kept for long cases/ short cases/spot images. It can be often daunting for the candidate to tackle corneal examination if one is not used to it. Practice makes a man perfect; a habit of presenting cases in front of seniors can be very helpful. This article aims to provide a case-by-case approach to corneal examinations. The list of cases that should be at the tip of the fingers as a postgraduate are: • Infective keratitis • Keratoconus/other ectatic disorders • Dystrophies/degenerations • Keratoplasty • Chemical injury. The candidate should be well versed in the cases and also the associated theory. Around stencil, colored pencils, fluorescein strip, cotton wisp, direct ophthalmoscope, pen torch, indirect ophthalmoscope, 20D, and 78D/90D are the basic requirements which must be kept handy to avoid last minute beg borrow steal. A routine sequence must be followed to avoid getting confused. History taking A detailed history can give easy to grasp clues and help achieve a provisional diagnosis. History of any previous ocular disease, past surgeries, history of laser refractive surgery, or use of glasses/contact lenses should be taken in every case. A detailed negative history as described below is important and depends on each case. Examination The corneal examination can never be complete without an examination of the components of the ocular surface which includes (lids, conjunctiva, tear film, and the cornea). The sequence of examination remains the same for any ophthalmological case presentation. It includes the following[1]: Vision Uncorrected visual acuity/best-corrected visual acuity distance and near vision should be taken for all cases. Refraction is particularly important in a complaint of keratoconus. Lids/adnexa Look for entropion/ectropion, frequency of blinking (can be reduced in viral keratitis, neurotrophic keratitis with fifth cranial nerve pathology, postcorneal transplant patients, and one-eyed patients), lagophthalmos (exposure keratopathy), trichiasis, distichiasis, signs of anterior/posterior blepharitis, and punctual stenosis/atresia. Eversion of lids is important for all cornea cases It is said that to study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all. Anterior segment examination may appear to be easy at the outset to a post graduate, however it is more intriguing than it seems. To arrive at differentials based on age old examination techniques without the ancillary investigations is a skill that can be acquired by practice. This article aims to briefly describe the basic examination skills that are required for during post-graduation. Keywords: Cornea, examination, postgraduates, training Address for correspondence: Dr. Avani Hariani, Guru Nanak Eye Centre, Maharaja Ranjit Singh Marg, New Delhi ‑ 110 002, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Hariani A, Gupta I, Jain P. Demystifying corneal examination: A guide for post graduates. Delhi J Ophthalmol 2023;33:349-55. Demystifying Corneal Examination: A Guide for Post Graduates Avani Hariani, Isha Gupta, Parul Jain Department of Ophthalmology, Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_178_23 Submitted: 19-Nov-2023 Revised: 15-Dec-2023 Accepted: 26-Dec-2023 Published: 15-Jan-2024 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 349

Hariani, et al.: Corneal examination for post graduates to look for papillae, concretions, formation of pseudomembrane, foreign body (FB), and retained chemical particles. Conjunctiva Look for the normal sheen of conjunctiva, abnormal vascularity, and any lesions/growths. In dry eyes, the conjunctiva has loss of sheen, with associated frothy discharge from meibomian glands, bitot’s spots, and keratinization. In case of chemical injury, there can be a presence of conjunctival epithelial defects and signs of limbal stem cell deficiency with conjunctivalization of the cornea. Tear film assessment It includes assessing tear secretion and stability. Various bedside tests that can be performed include: Schirmer’s test It is done using Whatman‑41’ paper 35 mm × 5 mm. The Schirmer strip is placed between the inner 2/3rd and outer 1/3rd of the lid. 1. Schirmers 1 (without anesthesia): Basal and reflex secretion 2. Schirmers 2 (with anesthesia): Basal secretion 3. Schirmers 3 (stimulation of nasal mucosa): Done in patients with a borderline score on Schirmers 1. Tear film breakup time Invasive tear breakup time (TBUT): Done with the instillation of fluorescein dye. Atear layer is observed on a slit lamp while the eye is held open for several seconds. Eventually, the tear 350 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 1: Slit Lamp techniques. (a) Diffuse illumination, (b) Slit illumination/ Optical section, (c) Sclerotic scatter, (d) Retro-illumination, (e) Parallelepiped beam d c b a e Figure 2: Color coding in schematic corneal diagrams Figure 3: Staining pattern. (a) Fluorescein staining of dendritic ulcer, (b) Fluorescein staining of an ulcer, (c) Rose Bengal dye staining the margin of dendritic ulcer a b c

Hariani, et al.: Corneal examination for post graduates Figure 5: (a) Granular dystrophy, (b) Macular dystrophy, (c) Lattice dystrophy (retro illumination), (d) fuchs endothelial corneal dystrophy (retro illumination) c d a b Figure 6: Symblepharon grading (a) IA, (b) IIB, (c) IIIC a b c Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 351 Figure 4: (a) Oil droplet reflex, (b) Rizuttis sign, (c) Munsons sign a b c

Hariani, et al.: Corneal examination for post graduates A detailed history is important as it can suggest the etiology of the ulcer. Time of onset Sudden onset and rapid progression are generally associated with bacterial corneal ulcers such as Staphylococcus aureus, Pseudomonas aeruginosa, and Pneumococcus species. Gradual onset and an indolent course are commonly seen in ulcers caused by fungi and parasites (Acanthamoeba) and a few bacteria such as Moraxella, coagulase-negative Staphylococcus, Nocardia species, and atypical Mycobacteria. Associated pain Acanthamoeba keratitis usually presents with excruciating pain due to associated radial keratoneuritis. The pain is usually out of proportion to the objective clinical findings. In contrast reverse is true for fungal ulcers where pain may be completely absent in spite of an advanced corneal ulcer. Past history History of trauma, contact lens use, allergic eye disorders, recurrent episodes of redness (viral etiology), topical medication use (steroids), prior ocular surgery, or systemic disease have to be noted carefully. Examination • Diffuse illumination: The following must be noted • Location: The location of the ulcer gives an indication about the probable cause, visual prognosis, and the initial choice of antibiotics. The location can be: • Central: Staphylococcus aureus, Pseudomonas, and Fusarium • Peripheral: Coagulase‑negative S. aureus, Mycobacterium tuberculosis, Herpes simplex • Superior: Ulcer associated with vernal keratoconjunctivitis (VKC) (Shield ulcer) or FB in sulcus subtarsalis • Inferior: Ulcers associated with exposure keratopathy • Margins: Well‑defined: Seen in healing infectious ulcers or sterile ulcers. • Punched out: In cases of neurotrophic ulcer • Indistinct: Seen in cases of progressive ulcer • Hyphae or feathery: Characteristic of fungal corneal ulcer • Overhanging: Mooren’s ulcer. Optical section • The size (height and width) of the ulcer should be measured with the reticule on the slit lamp and be noted in the documentation on a corneal grid immediately to Table 1: R.P Center grading of Graft Clarity Graft clarity Description 4 Graft is absolutely clear and all details of iris and AC are visible 3 Graft is clear but some details of iris and AC obscured 2 Graft hazy, iris, and AC visible but no details discernible 1 Graft is very hazy, iris and AC are only visible 0 Graft opaque AC: Anterior chamber Table 2: Symblepharon grading Length Width Inflammation I: Equal to or more than the normal palpebral conjunctiva. A: 1/3 eyelid length is covered by the symblepharon 0: no conjunctival hyperemia II: shorter than the normal palpebral conjunctiva, but equal in length to the normal tarsus B: 2/3 eyelid length is covered 1: Mild III: shorter than the normal tarsus C: greater than 2/3 is covered 2: Moderate IV: close to zero in length 3: Severe film is displaced and dry spots form. The length of time, in seconds, for this to happen is the TBUT. Noninvasive TBUT: Does not involve instillation of fluorescein. Noninvasive breakup time can be measured by corneal topography, interferometry, aberrometer, anterior segment optical coherence tomography, and confocal microscopy. Cornea A detailed description of the corneal examination in various case scenarios has been described below. One must be well-versed in the techniques of slit-lamp examinations. Adiffuse slit-free illumination is used for gross pathologies of the lid margin, conjunctiva, and cornea [Figure 1a] A direct slit illumination passes a narrow slit of light through the transparent tissues and helps in the cross-sectional study of opaque lesions like corneal and lens opacities[Figure 1b]. The light directed at the sclera glows the cornea through total internal reflection and indirectly highlights subtle corneal pathologies [Figure 1c]. Light is made to reflect from the iris (iris retro illumination) or fundus (fundus retro illumination) to visualize corneal and lens opacities better [Figure 1d]. A parallelepiped beam of light focused at an angle over the cornea delineates enface architecture [Figure 1e]. The rest of the examination remains the same for any other ophthalmological case. Case 1: Infective Keratitis (Corneal Ulcer) In the case of an ulcer, the candidate must also know about the scraping techniques, types of stains, various culture media, and management of ulcers along with the various clinical trials. History The patient will present with chief complaints of pain, diminution of vision, redness, watering, and photophobia. 352 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Hariani, et al.: Corneal examination for post graduates avoid missing any findings. Color coding in corneal diagrams [Figure 2].[2-4] • The depth of the ulcer, area of thinning, descemetocele formation, iris plugging, depth of the anterior chamber, and associated hypopyon should be noted. Indirect illumination Helps to look at the surrounding cornea to look for the presence of satellite lesions (characteristic of fungal ulcer), corneal scars (ghost scars in recurrent viral keratitis), and vascularization. The appearance of new vessels is a sign of healing keratitis. Superficial or deep corneal vascularization of varying extents may be seen in cases of infectious keratitis. A quadrant-wise record of corneal vascularization should be made. Staining Corneal staining is the most reliable clinical way to assess corneal integrity. It must not be done after instilling a drop of topical anesthetic as it will hamper checking the corneal sensations. Apositive Seidel’s test is suggestive of a perforated corneal ulcer. Characteristic corneal staining patterns may be seen with fluorescein staining in different types of infectious keratitis [Figure 3a and b]. Rose Bengal dye may be used to stain the margins of epithelial defects, particularly in the case of herpetic keratitis which stains with this dye as it is loaded with the virus particles [Figure 3c]. Corneal sensations This should be assessed in every case of corneal ulcer as it can be a cause of nonhealing ulcer/neurotrophic ulcer. Corneal sensations are markedly reduced in the case of herpetic keratitis. It can be tested qualitatively with the help of a cotton wisp and quantitatively with the help of a handheld esthesiometer (Cochet‑Bonnet). Case 2: Keratoconus/Other Ectatic Disorders In a case presentation of corneal ectatic disorders, the candidate may be provided with corneal topography maps of the patient; hence one should be well-versed with reading the tests and interpreting them. In addition, the following topics should be read: Corneal collagen crosslinking (CXL), contact lenses, intracorneal ring segments, and Lamellar keratoplasty. History The patient will have the following chief complaints: progressive visual blurring and/or distortion, photophobia, glare, ghosting of images, monocular diplopia, and frequent change of glasses. May be associated with VKC and chief complaints of intense itching and h/o eye rubbing. Past history A history of redness/whitish opacity/photophobia to rule out previous hydrops. History of use of contact lenses, allergic eye disease, VKC, and history of any procedure done in the past for example, CXL and refractive surgery. Associated conditions History to rule out the following conditions: Ocular associations: floppy eyelid syndrome, Leber’s congenital hereditary optic neuropathy, cone-rod dystrophy, VKC; Systemic associations: Down syndrome, atopy-bronchial asthma, angioneurotic edema, Marfan syndrome. Examination Distant direct ophthalmoscopy: Oil droplet reflex, Rizutti sign, Munson sign [Figure 4a-c]. Slit-lamp examination: 1. Corneal thinning: The thinnest part of the cornea is usually located outside the visual axis, and corneal thinning is a common sign preceding ectasia 2. Corneal ectasia: An eccentrically located ectatic protrusion of the cornea is noted in keratoconus. Three types of cones can be seen in advanced keratoconus: round or nipple-shaped cone; oval or sagging cone; globus type of cone when the ectasia involves >75% of the cornea. In the case of keratoconus thinning and ectasia are at the same level, in the case of pellucid marginal corneal degeneration, the area of ectasia lies above the area of thinning giving a bean pot appearance 3. Indirect illumination: To visualize corneal scarring, Vogt’s striae, and prominent corneal nerves 4. Cobalt blue filter to look for Fleischer’s ring. Case 3: Corneal Dystrophies/Degenerations The common cases in exams include lattice/granular/ macular dystrophy + Operated keratoplasty in the other eye. Fuchs endothelial corneal dystrophy (FECD) + PBK/ Operated DSAEK in the other eye. CHED, BSK, Spheroidal degeneration. History A detailed personal and family history must be taken. Onset Blurring of vision is rare before the fifth decade of life in the case of granular corneal dystrophy (GCD), lattice corneal dystrophy (LCD) may present as progressive loss of vision in the first decade and between 3 and 9 years of age in the case of macular corneal dystrophy (MCD). The blurring of vision in the 5th–7th decade which is more in the morning and gradually improves over the day is suggestive of FECD. Photophobia Recurrent erosions can occur in all types of stromal dystrophy but it is most commonly seen in LCD in the first to second decades, in cases of GCD, it can occur in early stages, but episodes are usually mild and rare. MCD usually presents with recurrent erosions which occurs in second to third decades. Progression The progression is relatively faster in MCD followed by LCD when compared to GCD. Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 353

Hariani, et al.: Corneal examination for post graduates Examination Granular dystrophy The deposits can resemble crushed breadcrumbs, snowflakes, popcorn, or Christmas trees. The granules are primarily located in the central cornea. Initially, the stroma between the opacities remains clear [Figure 5a]. Macular dystrophy Pleomorphic opacities with hazy intervening areas between the opacities give a ground glass appearance with full thickness involvement [Figure 5b]. Lattice dystrophy The lattice lines are typically refractile with a double contour and a clear core on retro illumination [Figure 5c]. Fuchs endothelial corneal dystrophy Corneal guttae can be visualized as dropout areas on specular reflection on a slit lamp. As the disease progresses, guttae spread peripherally and coalesce centrally along with pigment dusting on the endothelium. This characteristically gives the appearance of “beaten metal appearance.” The guttae are well appreciated on retro illumination [Figure 5d]. Case 4: Keratoplasty The candidate must be aware of indications of keratoplasty, types of keratoplasty, newer advances, and graft rejection. History Detailed history of the primary condition for which the surgery was done, multiple keratoplasties, full-thickness/ lamellar keratoplasty, and family history might suggest a corneal dystrophy. In case of graft rejection, history of recurrent redness (herpetic disease), trauma, infection, use of antiglaucoma medications, and surgeries done postkeratoplasty must be taken. Examination In the case of penetrating keratoplasty, the following details must be seen on the slit lamp • Graft clarity: Graded according to the RP center grading as follows:[Table 1] • Graft host junction: Well apposed/gaping/wound leak/ seidels test • Sutures: Number of sutures, tight/loose sutures, and note of exposed knots to be made • Anterior chamber details/status of the lens • Intraocular pressure (IOP): If possible, with a noncontact tonometer or a tono-pen. In the case of lamellar keratoplasty: The status of graft/lenticule apposition needs to be mentioned. In case of graft rejection • Cellular infiltration of the cornea as discrete subepithelial infiltrates can be seen in the donor tissue. An epithelial rejection line, also known as Krachmer’s line, in the graft from the host graft junction without edema and keratic precipitates/infiltrate can be seen in a case of epithelial rejection • Keratic precipitates: They can appear as scattered deposits or can form a distinct line known as the Khodadoust line. Endothelial rejection is often associated with stromal edema overlying the areas that have been traversed by the endothelial rejection line while the areas ahead of the line are clear. Case 5: Chemical Injury History Regarding the type of chemical/thermal injury will help understand the mechanism of injury. The place of injury also needs to be asked in a medicolegal case. Knowledge about the classification system is important to prognosticate the patients. Examination • Lids/adnexa: Double eversion of lids has to be done in all cases where possible to rule out retained chemical particle. Lid margin/cilia abnormalities are to be noted • Symblepharon: This is graded from 0 to 4. A score of 1 for every 3 clock hours involvement. Alternatively, based on length, defined as the shortest distance between the lid margin to the limbus, Grade I symblephara have a length equal to or more than the normal palpebral conjunctiva. Grade II symblephara are shorter than the normal palpebral conjunctiva but equal in length to the normal tarsus. Grade III are shorter than the normal tarsus, and Grade IV are close to zero in length as described by Kheirkhah et al.[5] Based on width, defined as the longest horizontal distance compared to the length, the gradation is “a” if 1/3 of eyelid length is covered by the symblephara, “b” if 2/3 of eyelid length is covered, and “c” if >2/3 is covered. Based on inflammatory activity, symblephara are graded 0 if conjunctival hyperemia is absent, 1+ if mild, 2+ if moderate, and 3+ if severe [Table 2] [Figure 6] • Conjunctival involvement: The conjunctiva needs to be examined for the areas of necrosis and underlying scleral necrosis/melt. Staining the conjunctiva with fluorescein stain will help to evaluate the area of epithelial defect • Limbal involvement: In a case of acute chemical injury, the area of limbal ischemia (loss of palisades of Vogt + white appearance at limbus due to blanching of vessels) needs to be evaluated. In case of chronic injury, the clock hours of conjunctivalization of cornea/pannus formation need to be assessed • Corneal involvement: Corneal clarity is graded according to Roper Hall classification. The cornea is stained to assess the epithelial defect • IOP assessment with the help of Ton‑open, if available. Take home message As the famous saying goes, “The eyes will not see what the mind does not know,” this article is just the tip of the iceberg, nothing can replace a well-read textbook along with the 354 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Hariani, et al.: Corneal examination for post graduates habit of presenting cases and documenting them. A detailed understanding of corneal examination in a systematic manner must be known by every aspiring ophthalmologist. In this new era of artificial intelligence, let us not forget the age-old basic clinical skills that can decode any clinical case presented to us. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Waring GO, Laibson PR. A systematic method of drawing corneal pathologic conditions. Arch Ophthalmol 1977;95:1540-2. 2. MishraD, KaurK, GurnaniB, HedaA, DwivediK. Clinical and diagnostic color-coding in ophthalmology – An indispensable educational tool for ophthalmologists. Indian J Ophthalmol 2022;70:3191-7. 3. Salmon JF. Kanski’s Clinical Ophthalmology. 9th ed. Amsterdam: Elsevier; 2019. 4. Bron AJ. A simple scheme for documenting corneal disease. Br J Ophthalmol 1973;57:629-34. 5. Kheirkhah A, Blanco G, Casas V, Hayashida Y, Raju VK, Tseng SC. Surgical strategies for fornix reconstruction based on symblepharon severity. Am J Ophthalmol 2008;146:266-75. Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 355

Abstract Theme Section Introduction Your examination of a patient starts the moment you see the patient, especially the way he/she walks. A patient with glaucomatous damage will walk slowly, with minimal sideways eye movements, unlike a patient with cataracts or macular changes. She/he may have an accompanying attendant. What History is to be Taken – Both Positive and Negative? Tailor history taking according to patient age and presenting complaints. For age ≥50–60 years, primary glaucoma is the first diagnosis, and for those below 40 years, traumatic, steroid induced followed by pigment dispersion and juvenile glaucoma should be thought of. Presenting complaint Painless progressive diminution of vision is an invariable complaint, rarely interspersed with episodes of red eye and headache, in case of angle-closure episodes. Angle-closure glaucoma (ACG) is more common in females and those of Asian origin. History of presenting illness In the elderly, difficulty in dim light, contrast issues, and rarely inability to perceive objects in the peripheral field can be elicited. Specifically ask for the history of falls and fractures. In the young, history of trauma, use of steroids topical/nasal/ systemic, and previous incisional or laser surgeries must be taken. Ahistory of colored haloes or headache and ocular pain triggered in dim light, especially during the evening, can be elicited for angle closure. Concomitant eye diseases such as uveitis, cataracts, and macular degeneration have to be ruled out. This is required to ascertain the cause of glaucoma, management to be adopted, and prognostication. Visual acuity and visual field both can be affected by cataracts and macular and retinal pathologies. Disease symmetry and asymmetry indicate primary glaucoma as the etiology, whereas unilateral disease invariably indicates secondary glaucoma. Ask about the history of eye drop usage and frequency (antiglaucoma medications [AGM] or steroids) and prior eye surgery (cataract, glaucoma, retina, and trauma repair). Specifically, ask for a prior laser procedure in the affected or fellow eye. Patients often do not consider laser to be a surgery and will not remember unless specifically asked for. History of systemic diseases affecting the cardiovascular system like hyper/hypotension and migraine/blood loss during parturition/hemorrhoids/accidents should be taken and documented or ruled out in glaucoma cases especially Glaucoma patient is a common case scenario in postgraduate examinations; glaucoma examination involves a sequence with certain “must-do” procedures. This article details the relevant history, including negative history, and examination with emphasis on what all needs to be documented. Diagrams are both descriptive and scoring in examinations, and their representative samples have been depicted in this article. Keywords: Glaucoma I examination, gonioscopy, optic disc evaluation Address for correspondence: Dr. Arshi Singh, House No. 11, Type VI MAMC Faculty Campus, Maulana Azad Medical College, Kotla Road, New Delhi ‑ 110 002, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Singh A, Singh K, Jain D. Examination and presentation of a glaucoma case. Delhi J Ophthalmol 2023;33:356-9. Examination and Presentation of a Glaucoma Case Arshi Singh, Kirti Singh, Divya Jain1 Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, 1Department of Ophthalmology, Postgraduate Institute of Child Health, Noida, Uttar Pradesh, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_199_23 Submitted: 26-Dec-2023 Revised: 29-Dec-2023 Accepted: 31-Dec-2023 Published: 15-Jan-2024 356 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Singh, et al.: How to examine and present a glaucoma case in exam for normal tension glaucoma. Diabetes, thyroid disorder, asthma, chronic obstructive lung disease, and sleep apnea also need to be ruled out. Mention positive or negative history of hypotensive episodes as these are important for ocular blood flow aspects of glaucoma etiopathogenesis. Systemic medications can affect glaucoma patients like systemic medications like steroids being the cause or systemic beta blockers masking the increased intraocular pressure. AGMs such as timolol, brimonidine, and acetazolamide affect systemic diseases. Eye Examination Sequence Always examine both eyes and draw a diagram of both eyes unless specified by the examiner. Carry these examination tools – 78/90D lens, gonioscope, colored pencils to draw diagrams, and alcohol swabs for disinfection. This prevents dependency on the examination center. Always document the must-know areas. Vision Central vision and projection of rays are to be documented. Document the functional near vision, as the use of pilocarpine can affect that. Orbit/extraocular movements Rule out any restrictions in extraocular movements, since that would affect the IOP. Inferior rectus muscle restriction in thyroid eye disease, could result in erroneous measurement of IOP. Periocular fat atrophy/ sunken globe in younger age, may signify longterm use of prostaglandin analogs. Eyelashes and periocular tissues Hypertrichosis and pigmentation may provide a clue of the longterm use of prostaglandin analogs. Lids/conjunctiva (do elevate lid and do evert lid) In young cases, a pale watery conjunctiva would imply allergic conditions. Always evert the upper lid to r/o vernal keratoconjunctivitis. Elevate the lid in all cases to look for any prior glaucoma filtering surgery. The patient may not give the history. If a bleb is seen, then grade it by either IBAGS (Indiana Bleb appearance grading scale)/ Moorfields Bleb Grading. Mention if it is functional or not. Look for the surgical peripheral iridectomy (PI), and if not seen or not patent, write in your sheet that it is not visible or not done as the case may be. Cornea Look for pigmentation on endothelium (Krukenberg spindle or diffuse pigmentation postsurgery/PI). R/o healed herpetic keratouveitis, keratorefractive surgery (laserassisted in situ keratomileuses). Anterior chamber depth Measure AC depth by van Herick grading and draw the same [Figure 1]. Pupils Ensure that a relative afferent pupillary defect (RAPD) is not missed. A RAPD defect would imply asymmetrical disease. Examine pupil ruff to rule out ruff atrophy/ entropion uvea /sphincteric atrophy. Sphincteric tears may indicate prior trauma/surgery. Fixed dilated pupil signposts advanced glaucoma, especially angle closure. Examine for corectopia, polycoria, neovascularization, pseudoexfoliative material, and pupillary dilation. Iris Iris has to be examined all 360°. Iris atrophy ranging from pupil ruff loss, sphincteric atrophy, whorling, and iridoschisis indicates the glaucoma subtype. Thick peripheral iris roll and convex iris configuration may imply angle‑closure glaucoma. Always look for any peripheral iridotomy and mention if patent or not. [1] Iridocorneal endothelia/Axenfeld–Rieger/pigment dispersion syndrome (PDS)/pseudoexfoliation (PXF) and Fuchs’ heterochromic iridocyclitis are all diagnosed by iris examination. Any altered iris configuration – queer/concave or steep/convex confirm on gonioscopy. Give negative findings of the Krukenberg spindle, Sampaolesi line, and transillumination defect (not easy to see in pigmented races with thick iris) in case diagnosis of PDS/PXF is being made. Lens In addition to lens shape, transparency r/o evidence of PXF, pigmentation on the lens. Look out for a lens bulge/ volcano configuration signifying exaggerated lens vault/ glaukomflecken (gray–white epithelial and anterior cortical lenticular opacities, signifying past acute attack with high IOP). Check for zonule integrity. Optic nerve head examination (undilated first) with 90/78 D lens: Do a dilated examination only once your gonioscopy has r/o angle closure. Define disc by following aspects: • Disc size (normal dimensions vertical 1.5–2 mm/ Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 357 Figure 1: Van Herick grade 2 = to ¼ of peripheral corneal thickness

Singh, et al.: How to examine and present a glaucoma case in exam horizontal 1.5–1.7 mm, shape (normal vertically oval). Larger than 2 mm size is considered large size disc • Color of the neuroretinal rim (NRR) – Health pink, hyperemic (immediately after/during acute ACG), diffuse pallor, and localized pallor • Cupping dimensions – Cup disc: C: D ratio and shape – concentric, focal ischemic (notched with localized loss of NRR width), and senile sclerotic bean pot. Mention if the ISNT rule is followed The Disc Damage Likelihood Scale classifies glaucomatous structural changes in the optic disc based on the radial width of NRR at its thinnest location, or if no rim is present, the extent of absence of it. It takes disc size into account.[2] Vasculature signs – nasalization/centrifugal displacement of blood vessels away from the center, bayonetting, overpass phenomenon, collaterals, and disc hemorrhage (flame/splinter‑shaped in nerve fiber layer at NRR level/immediately adjacent to optic disc margin). Mention the presence or absence of these findings. Draw diagram [Figure 2] • Peripapillary atrophy – Also seen in myopes. Alpha-peripheral atrophy with irregular hyper/ hypopigmentation; beta-central atrophy of choroid and retina revealing the underlying choroidal vessels and sclera. Gamma, previously called myopic temporal crescent, is a white-colored area interspersed between the beta zone and optic disc border. The gamma zone differs from the beta zone in lacking a Bruch’s membrane, which is identified in optical coherence tomography[3] • Lacrimal apparatus testing – ROPLAS. Diagnosis Complete with management plan: For example, a. Advanced primary angleclosure glaucoma right eye, primary angleclosure suspects left eye with early immature senile cataract both eyes (IMSC OU) • Plan: Phacotrabeculectomy Right eye (as high lens vault with early cataract) • Laser iridotomy left eye with close followup. b. Juvenile open angle glaucoma (JOAG) both eyes with functional filtering blebs OU with high IOP. • Plan: Maintain target pressure with supplemental AGM use, can consider bleb revision by needling. Must‑do Procedures: Examination Gonioscopy Optic nerve head examination Undilated fundus → dilated of time permits and angle is open. Intraocular pressure measurement By Goldmann applanation tonometer. Ask for or use your personal sterilizing wipes for tonometer tips. Dry the tip and wash off residue with saline or lubricant eye drop, IOP is written as depicted in Figure 3. Mention corrected IOP (underestimation of IOP in thin corneas and overestimation in thicker corneas), if known. Otherwise, discuss during the presentation of the case. Corneal hysteresis is measured by the Reichert Ocular Response Analyzer has an additional benefit. Higher corneal hysteresis has increased optic nerve surface compliance during artificially induced IOP elevations, suggesting better tolerance to IOP, and reduced corneal hysteresis is associated with progressive visual field loss in glaucoma patients.[4] Gonioscopy Indentation gonioscopy with 4‑mirror (Zeiss/Posner/Sussman) or static gonioscopy with single or 2-mirror Goldmann. 358 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 2: Disc damage as per the Disc damage Likelihood Scale in the right and left eyes Figure 3: Applanation tonometry depiction with time of tonometry Figure 4: Goniogram of the right and left eyes

Singh, et al.: How to examine and present a glaucoma case in exam Draw the goniogram, with the labeling of angle structures visible, pigmentation grade, synechiae, iris processes, iris configuration, and blood vessels/PXF material if visible, iris insertion (high in juvenile Open angle glaucoma) [Figure 4]. Tips Use the shortest off‑center slit beam (2 mm wide) at high magnification. If trabecular meshwork is not visible, differentiate appositional from synechial closure. Do dynamic gonioscopy with Goldmann gonioscope (ask the patient to look in the mirror or tilt the mirror toward the angle to be viewed).[5] Preferably, do an indentation gonioscopy with 4 mirror. Label the angle as open or closed using the ISGEO classification. Do mention pigmentation grade and pattern. Blotchy pigment on visible TM indicates an episode of a prior attack.[5] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Singh K, Singh A, Bhattacharyya M. Iris clues in ACG. Int Ophthalmol 2021;41:1959-64. 2. Spaeth GL, Henderer J, Liu C, Kesen M, Altangerel U, Bayer A, et al. The disc damage likelihood scale: Reproducibility of a new method of estimating the amount of optic nerve damage caused by glaucoma. Trans Am Ophthalmol Soc 2002;100:181-5. 3. Singh K. Challenges in optical coherence tomography interpretation for diagnosing glaucoma in myopic eyes. Delhi J Ophthalmol 2020;31:9-17. 4. Wells AP, Garway-Heath DF, Poostchi A, Wong T, Chan KC, Sachdev N. Corneal hysteresis but not corneal thickness correlates with optic nerve surface compliance in glaucoma patients. Invest Ophthalmol Vis Sci 2008;49:3262-8. 5. Singh K, Singh A. Gonioscopy: An Essential Skill to Combat Glaucoma Blindness. DOS Times; 2018. pg. 5868 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 359

Abstract Theme Section Chief Complaints and Their Relevance in History Taking Diminution of vision should be characterized into sudden or gradual. Sudden diminution of vision – Causes include vitreous hemorrhage, rhegmatogenous retinal detachment (RRD), central retinal artery (CRAO)/branch retinal artery occlusion (BRAO), central retinal vein occlusion (CRVO)/branch retinal vein occlusion (BRVO), arteritic anterior ischemic optic neuropathy (AAION), nonarteritic ischemic optic neuropathy (NAAION), and polypoidal choroidal vasculopathy (PCV) (by sudden submacular hemorrhage or large break‑through vitreous hemorrhage). Traction retinal detachment(TRD) due to proliferative diabetic retinopathy (PDR)(TRD) can cause sudden loss of vision when the TRD involves the macula. TRD can also cause a combined retinal detachment due to the development of a break, in which case there will be sudden loss of vision. CRAO and BRAO – Sudden and instantaneous “blackening of vision” (akin to switching off a light bulb). AAION due to giant cell arteritis causes painful vision loss (temporal headache). CRVO and BRVO – “Dulling of vision” (akin to dimming of a light bulb) may be noticed on waking up in the morning, due to nocturnal hypoperfusion. Gradual diminution of vision Gradual diminution of vision causes include diabetic macular edema (DME), macular edema due to retinal vein occlusions, age‑related macular degeneration (dry or exudative), and epiretinal membrane (ERM). Diminution of vision for distance/near/both Pathologies affecting the retina predominantly cause distance vision abnormalities and those affecting the macula cause near-vision abnormalities. Pro-tip – In a diabetic patient, sudden vision loss in case of a posterior vitreous detachment (PVD) causing a dense vitreous hemorrhage can be differentiated from a vitreous hemorrhage caused by PDR by the sole history of floaters preceding the vision loss in the former. Flashes and floaters Causes of flashes include PVD, retinal breaks, retinal detachment, and retinal dialysis. Flashes are caused by vitreoretinal traction on the retinal surface. Floaters – causes include vitreous hemorrhage, vitritis, PVD, and retinal breaks. Trivia – Another name for floaters is myodesopsia. Pro‑tip – Asteroid hyalosis almost never causes floaters. Pathologies affecting the retina can cause a range of symptoms and signs. Postgraduates will be assigned two long cases and 2–3 short cases in their qualifying examinations. This article intends to be a ready reckoner that can be used as pro forma for history taking and examination. Acomprehensive history taking and well-rounded examination will go a long way, both for post-graduate exit examinations and future practice. Keywords: Colour coding, history taking, retina case, retinal examination Address for correspondence: Dr. Apoorva Ayachit, Department of Vitreoretina, M M Joshi Eye Institute, Hubballi, Karnataka, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Ayachit A, Mishra A, Salvi RS, Joshi S, Ayachit G. History taking and examination in a retina case. Delhi J Ophthalmol 2023;33:360-4. History Taking and Examination in a Retina Case Apoorva Ayachit, Akshay Mishra, Rajashree Satish Salvi, Shrinivas Joshi, Guruprasad Ayachit Department of Vitreoretina, M M Joshi Eye Institute, Hubballi, Karnataka, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_201_23 Submitted: 28-Dec-2023 Accepted: 29-Dec-2023 Published: 15-Jan-2024 360 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

Ayachit, et al.: Retinal history- taking and examination Metamorphopsia – This is caused by pathologies affecting the macula and is caused due to misalignment of photoreceptors due to fluid accumulation or loss of foveal contour either due to macular swelling or due to traction. Causes include central serous chorioretinopathy (CSC), ERM, exudative/neovascular AMD (nAMD), and macular edema (vascular or inflammatory). Other symptoms – Nyctalopia (rod dystrophies and rod cone dystrophies), difficulty in day‑time (cone dystrophies), color vision (optic neuritis and macular dystrophies), and miscellaneous symptoms‑flashes, waviness, and visual snow-can be noted in retinal dystrophies. The congruent decrease in visual acuity and visual field is seen in retinal dystrophies (especially rod‑cone dystrophies). History of Past Illness • Similar complaints in the past – same eye or other eye: Relevant in DME, Vitreous hemorrhage (VH), floaters and flashes (RRD), CSC, nAMD, or dry AMD Figure 1: (1) Horseshoe tear (HST), (2) Detached retina, (3) Retinal corrugations, (4) Attached retina Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 361 Figure 3: (1) Fibrovascular proliferation- red indicates vascular component and green indicates fibrous component, (2) Intraretinal haemorrhage, (3) Venous looping, (4) Sclerosed vessels, (5) Dot and blot haemorrhages, (6) Hard exudate Figure 2: (1) Subretinal haemorrhage at superior border of CNVM lesion, (2) Greenish CNVM lesion, (3) Pigments, (4) Right angled venules

Ayachit, et al.: Retinal history- taking and examination • History of trauma – relevant in vitreous hemorrhage/retinal dialysis/retinal detachment/sympathetic ophthalmia • History of ocular surgery such as cataract/refractive surgeries/glaucoma/retinal surgeries • History of intravitreal injections (DME, nAMD, RVO, and PCV) or laser treatments for (PDR, RVO, vasculitis, and peripheral retinal lesions i.e. for lattices and holes. Medical History Diabetes mellitus (DM) – type I or II, duration, control (last known fasting blood sugar, post prandial blood sugar, and HbA1c), use of oral hypoglycemic agents and exact prescription if available (glitazones can cause macular edema), insulin dosage, general compliance with treatment, and follow-ups with treating physicians are to be noted in detail. History of neuropathy diabetic ulcers, cellulitis, history of amputations, and last known renal parameters should be documnented. Pro-tip – Creating awareness about diabetes causing retinopathy, nephropathy and neuropathy should be reiterated by the retinal physician at this point in history taking. Hypertension – exact prescription of anti-hypertensives if available and compliance with treatment should be asked for. Night-time hypertensive medication can cause nocturnal hypotension which is a risk factor for RVO and NAAION. History of stroke, transient ischemic attacks, transient vision loss, is important in case of CRAO/BRAO was noted. The • History of glasses use/contact lens use/refractive surgeries-relevant in myopia, retinal breaks, and RRD 362 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 4: (1) Neurosensory detachment, (2) White without pressure, (3) HST with flap, (4) Operculated hole, (5) Lattice with hole, (6) Cystoid spaces, (7) Subretinal gliotic band, (8) Focal star fold, (9) Retinal dialysis, (10) Diffuse fold- Upper case X, (11) Anterior PVR- circumferential retinal fold (lower case x), (12) Hard exudate, (13) Cotton wool spot, (14) Asteroid hyalosis, (15) Epiretinal membrane, (16) Fresh laser marks, (17) Choroidal detachment, (18) Old laser marks, (19) Retinoschisis, (20) Retinal Thinning Figure 5: (1) Neurosensory detachment, (2) Arteritis, (3) Phlebitis, (4) Choroidal granuloma, (5) Area of extensive retinitis, (6) Multiple neurosensory detachments, (7) Vitreous haze, (8) Active choroiditis lesion, (9) Snowballs, (10) Snowbanking, (11) Paravascular healed choroiditis lesion Figure 6: (1) Giant retinal tear, (2) Rolled posterior edge of GRT, (3) Silicon oil meniscus, (4) Buckle indentation effect, (5)HST, (6) Cryo mark around HST, (7) Bare RPE after retinectomy, (8) Lasered retinectomy edge, (9) Indentation from encircling belt, (10) Lattice with hole, (11) Old laser marks

Ayachit, et al.: Retinal history- taking and examination use of blood thinners is a risk factor for vitreous hemorrhage, subretinal hemorrhage, and uncontrolled intra-operative and postoperative ocular hemorrhage. Intravitreal anti-vascular endothelial growth factors (VEGFs) are contraindicated in patients who have suffered cerebrovascular or cardiovascular events in the past 3–6 months. History of Cardiovascular diseases and carotid occlusive disease are important causes of CRAO, BRAO. Diabetic nephropathy, renal hypertension cause higher grades of hypertensive and diabetic retinopathy, Autoimmune disorders such as systemic lupus erythematosus (SLE), Polyarteritis nodosa (PAN), granulomatosis polyangiitis (GPA), Susac syndrome are important causes of vasculitis and vascular occlusions in young patients. Complement mediated glomerulopathies can cause drusen, Alport syndrome can cause retinal thinning and dot-and-fleck retinopathy. Peptic ulcer disease includes CSC. Steroid use from any route-oral , intravenous , intramuscular, topical, inhalational, suppositories is important in CSC. History relevant to tuberculosis, sarcoidosis, and autoimmune conditions in cases of intermediate and posterior uveitis should be obtained. Systemic malignancy can cause choroidal metastasis, cancer-associated retinopathy, and melanoma-associated retinopathy. In cases without known malignancy, a work-up for systemic malignancy should be carried out by an oncologist if there is new-onset night blindness or there are posterior segment findings of choroidal mass with extensive subretinal fluid, leopard mottling, or pigmentary changes. Family History History of consanguineous marriage (retinal dystrophies and RRD) pedigree chart should be made including at least three generations. Personal History Diet‑inadequate nutrition: Vitamin A deficiency can cause nyctalopia. History of substance abuse – amount, duration, and type (smoking/tobacco use in any form, alcohol intake, and drug abuse). Allergies – to be noted before doing fluorescein and/or indocyanine green angiography. Sleep hygiene – poor sleep habits can trigger the episodes of CSC. Obstetric and menstrual history – last menstrual period to be noted if fluorescein angiography, intravitreal anti‑VEGFs are planned. Birth History Especially important for retinopathy of prematurity – gestational age, postmenstrual age, birth weight, whether vaginal delivery/ caesarean section, cry immediately after birth, neonatal intensive care unit admission: Number of days, oxygen therapy: Number of days and mode of oxygen delivery, stormy perinatal period due to meconium aspiration, hyaline membrane disease, septicemia, seizures, intracranial hemorrhage, whether there is appropriate weight gain. TORCH infection: Congenital toxoplasmosis, rubella retinopathy, cytomegalovirus retinitis, and HSV 1/2 (viral retinitis). Table 1: Color coding for fundus drawings Red solid Red cross - lined Blue Blue cross - lined Green Green dots Brown Yellow Black Attached retina Open portion of GRT and dialyses Detached retina Inner leaflet of retinoschisis Vitreous opacities Asteroid hyalosis Outline of ora serrata Intraretinal and subretinal hard exudate Edge of buckle Retinal arterioles Thin areas of retina Retinal veins White with pressure and without pressure Vitreous hemorrhage Bare RPE Retinitis lesions Old laser and cryo marks Attached macula (plus mark) Outlines of retinal holes and breaks Rolled posterior edges of retinal tears and GRTs Weiss ring Choroidal melanoma Fibrin in CSC Retinal and choroidal pigmentation Hemorrhages - intraretinal and preretinal Retinal folds Outline of fibrovascular proliferation Choroidal detachment Fresh laser marks, fresh cryomarks Demarcation lines in chronic RRD Inside of a retinal hole/tear Silicon oil Edge of buckle under detached retina Cotton wool spots Flat neovascularization CSC: Central serous chorioretinopathy, RRD: Rhegmatogenous retinal detachment, RPE: Retinal pigment epithelial, GRTs: Giant retinal tears Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 363

Ayachit, et al.: Retinal history- taking and examination Immunization History Measles-subacute sclerosing pan encephalitis-associated retinitis. General Physical Examination Pulse rate, blood pressure examination, and respiratory rate for baseline documentation. Patients with orthopnea could have breathlessness intra‑operatively due to flash pulmonary edema. Such patients should be specially evaluated for cardiorespiratory status. Pallor – anemic patients with diabetic TRD can have worse traction and worse intra-operative bleeding. Anemia could also be due to indolent myeloid leukemias. All diabetic patients should undergo complete blood count and peripheral smear to rule out blood dyscrasias which can adversely affect the surgery and postsurgery outcomes. Examination of lower extremities for diabetic ulcers, cellulitis, and gangrene should not be ignored. In these cases, Surgery should be deferred to avoid postoperative endophthalmitis in diabetic patients. Ocular Examination Head posture, facial symmetry, and ocular movements. Visual acuity: • Distance vision: Snellen chart/ETDRS chart • Near vision. Uncorrected visual acuity (UCVA) and best‑corrected visual acuity (aided as well as subjective refraction). Projection of rays. Color vision (optic nerve pathology and macular dystrophy). Intraocular pressure ( p r e f e r a b l y A p p l a n a t i o n tonometry) – high pressures in neovascular glaucoma and low pressures in RRD. Anterior Segment • Adnexa: Recurrent stye (uncontrolled DM) • Conjunctiva: Congestion (anterior uveitis and endophthalmitis) • Cornea: Reduced corneal sensation (diabetic neuropathy) • Anterior chamber: Cells, flare (ocular ischemic syndrome [OIS]), and hypopyon (uveitis, endophthalmitis) • Anterior chamber angle: NVA (PDR, CRVO, and OIS) • Iris: Neovascularization of the iris-CRVO, PDR, and OIS • Pupil: Relative afferent pupillary defect grading – CRVO, RRD, and TRD. Lens: Phakic, pseudophakic, or aphakic. Scleral buckling is preferred in young, phakic patients with RRD. In aphakic and pseudophakic patients, due to possibility of missing flap tears near the ora serrata, vitrectomy procedures are preferred. Combined phaco + vitrectomy is planned in PDR/TRD patients if the lens is cataractous. Anterior vitreous – Pigments (tobacco dusting or Shaffer’s sign) in eyes with RRD. Dilated fundus examination condition: Dark room, sitting position (+90/+78 D) slit‑lamp examination, and supine position (peripheral indented examination). Instrumentation: DO, IDO, +90/+78/+20 D lens, scleral indenter, and dilating drop. Optic disc: Size, color, cup-disc ratio, neuroretinal rim, disc margins, and peripapillary retinal nerve fiber layer. Retinal blood vessels: Normally A:V ratio is 2:3. • If arteries are attenuated and veins are of normal caliber A:V ratio is 1:3 • If arteries are normal and veins are dilated A:V ratio is 2:4 • If both arteries and veins are narrowed A:V ratio is 1:2. A-V crossing changes such as Gunn sign and Salu sign must be noted. Vascular sheathing, cuffing, and paravascular choroiditis lesions or pigmentation must be noted. Macula (+90D/+78D) – Foveolar reflex must be noted. Retina (+20D): Peripheral indented examination. Retinal drawings are made on an Amsler-Dubois chart. There are three concentric circles on a fundus chart: the inner-most circle represents the equator; the second circle denotes the ora serrata; and the outer-most circle denotes the pars plana. The radial lines denoted by roman numerals indicate the clock hours to describe the location and extent of lesions. Posterior segment – The following is a ready reckoner for color-coding used in fundus drawings [Table 1 and Figures 1-6]. The color coding may vary slightly in various retinal practices. However, the color coding described here largely represents the standard practice across institutions. Any variations can be explained simply by labeling the findings as shown in the figures. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. 364 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Theme Section Oculoplasty Evaluation for Postgraduates Rwituja Thomas, Aman Vaishya Vision Eye Centres, New Delhi, India Contd... Proptosis Evaluation History Onset, duration, progression of symptoms, associated symptoms, past history of any ocular/neuro/sinus surgery, comorbidities, family history, and systemic complaints [Table 1]. Table 1: Recording findings of Proptosis evaluation Finding Right eye Left eye Inspection Vision (BCVA) Color vision (Ishihara) Contrast sensitivity (Pelli‑Robson) or Red desaturation Intraocular pressure (Goldmann applanation tonometry) Pupillary reaction Facial asymmetry Type of proptosis (axial/abaxial) Exophthalmometry (Hertel’s/ Luedde’s) [Figure 1a and b] with base value e.g., 22 mm at base 104 Eyelid skin (erythema/excoriation/ telangiectasias) Ocular motility (with mention of diplopia in any version) Lid malposition (ptosis, lid retraction/ectropion/ entropion) Lagophthalmos (mm) Abstract Clinical examination and evaluation of Oculoplastics patients as a postgraduate can be daunting, either due to a paucity of patients with oculoplastic conditions or lack of exposure to the sub-specialty. This article aims to concisely provide an examination template for postgraduates along with clinical images of examination findings and techniques, in order to simplify this process. We have divided the evaluation into proptosis, eyelids, lacrimal, trauma, socket and ocular surface examination. Keywords: Oculoplastic Evaluation, Proptosis, Ptosis, Anophthalmic, Lacrimal Address for correspondence: Dr. Rwituja Thomas, Vision Eye Centres, New Delhi, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. How to cite this article: Thomas R, Vaishya A. Oculoplasty evaluation for postgraduates. Delhi J Ophthalmol 2023;33:365-8. Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_190_23 Submitted: 12-Dec-2023 Accepted: 24-Dec-2023 Published: 15-Jan-2024 Conjunctiva (congestion/tortuous vessels/ chemosis/SLK) Cornea status (exposure keratopathy) Fundus examination (ILM/choroidal folds/optic disc changes) Valsalva Palpation of mass (if any) Location Extent Margins if distinguishable Consistency Tenderness Finger insinuation Pulsatility/thrill Local rise of temperature Resistance to retropulsion Bony margins of orbit Sinus tenderness Lymph nodes (preauricular, postauricular, submandibular, and cervical) Nasal cavity examination (with endoscope if available) Auscultation (bruit) FAT BCVA: Best-corrected visual acuity, FAT: Familial album tomogram, ILM: Internal limiting membrane, SLK: Superior limbic keratoconjunctivitis © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 365

Thomas and Vaishya: Oculoplasty examination and reporting of findings Confrontational fields to be done or visual fields to be advised if any disturbance in vision/color vision/pupillary reaction. This is followed by imaging as needed – computed tomography scan with contrast (after renal function tests) or magnetic resonance imaging with contrast(for better delineated soft tissue). Clinical activity score to be used to evaluate and grade activity for thyroid eye disease [Figure 2 and Table 2]: Table 2: CAS (clinical activity score) for Thyroid eye disease Symptoms Score (1 for each) Pain or pressure in a periorbital or retroorbital distribution Gaze-evoked pain Signs Swelling of eyelids Redness of eyelids Conjunctival injection Chemosis Inflammation of caruncle/plica Total score on first visit Changes (in subsequent visits) Increase in measured proptosis >2 mm in 1–3 months Decrease in eye movement >8° over 1–3 months Decrease in visual acuity (2 Snellen lines) over 1–3 months Total score ≥3/7 ‑ active disease ≥4/10 ‑ active disease Eyelids Workup (Ptosis/Lid Retraction /Entropion/Ectropion) History Table 3: Findings of Eyelid evaluation Examination findings Right eye Left eye Vision (BCVA) and refraction Facial asymmetry Head tilt/chin lift/face turn (congenital ptosis) Laterality Frontalis overaction Lid details 1) Lid crease (present/absent/distant) 2) Scars (prior Sx/trauma) Hirschberg test Cover/uncover test (rule out pseudoptosis) PFH in primary gaze in mm MRD1: Upper lid margin to corneal reflex [Figure 3] MRD2: Lower lid margin to corneal reflex [Figure 3] MRD3: Upper lid margin to ocular reflex in extreme up gaze Contd... Contd... Levator action (Burke’s method ‑ lid excursion) Poor: ≤4 mm Fair: 5–7 mm Good: 8–14 mm Normal: ≥15 mm MCD in partial downgaze TPS in primary gaze MGJW 366 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 3: Short double arrow represents MRD1, long double arrow represents MRD2 Figure 2: Demonstrates findings seen in active thyroid eye disease Figure 1: (a) Placement of Hertel’s exophthalmometer on lateral orbital rims just adjacent to the lateral canthi with the examiner sitting at the same eye level, (b) The red lines overlapping to avoid parallax error (bold yellow arrow) and the location of the corneal apex to be measured (yellow arrow) a b

Thomas and Vaishya: Oculoplasty examination and reporting of findings Snap back test Grade 0 - normal Grade 1 - return in 2–3 s Grade 2- return in 4–5 s Grade 3 - return in >5 s Grade 4 - never return spontaneously Medial canthal laxity/dystopia+/− Lateral canthal laxity/dystopia+/− Eyelashes (madarosis, trichiasis/distichiasis) Lid margin keratinization Ectropion grading Grade 1 - only punctum everted Grade 2 - lid margin everted Grade 3 - fornix visible Grade 4 - tarsus everted Entropion grading Grade 1 - only posterior lid border inrolled Grade 2 - inturning of intermarginal strip Grade 3 - whole lid margin inturned Esthetic evaluation Skin - elasticity, pigmentation, and rhytids Tear trough deformity Dermatochalasis UL/LL fat prolapse PFH: Palpebral fissure height, MCD: Margin crease distance, TPS: Tarsal platform show, MGPW: Marcus gun jaw winking, BCVA: Best-corrected visual acuity, FAT: Familial album tomogram, BPES: Blepharophimosis, ptosis, epicanthus inversus syndrome, UL/LL: Upper lid/lower lid, Contd... MRD: Margin reflex distance Hering’s phenomenon Fatigue test Ice pack test Phenylephrine test for mild ptosis (1–2 mm) Neostigmine test (under anesthetist monitoring) Ocular motility Bell’s Phenomenon (upward and outward) [Figure 4] Good/fair/poor Inverse/reverse/perverse Lagophthalmos (mm) Lid lag on downgaze (present in operated/congenital ptosis) [Figure 5] Corneal sensations and surface Pupillary examination Associated features (narrowed horizontal palpebral fissure, telecanthus, and epicanthus – BPES) FAT scan for older photographs Palpate for levator dehiscence Pinch test/lid distraction (for lid laxity in entropion/ ectropion) Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 367 Figure 5: Demonstrates right‑sided lid lag on downgaze after ptosis surgery Figure 4: Demonstrates a good Bell’s phenomenon Figure 6: Sac syringing with a straight cannula Figure 7:Demonstrates left‑sided enophthalmos following orbital floor fracture

Thomas and Vaishya: Oculoplasty examination and reporting of findings Onset, duration, progression of lid malposition, associated symptoms, previous surgeries, current medications [Table 3]. Lacrimal System Examination History Duration of symptoms, frequency of discharge, nature of discharge (clear/mucoid/bloody), previous acute sac infection, previous sac surgery/incision drainage, previous sinusitis/ sinus surgery/trauma, nasal septum surgery. Criggler massage attempted earlier and to be demonstrated by a parent in the clinic [Table 4]. Table 4: Findings of Lacrimal evaluation Examination findings Right eye Left eye Tear film height Lacrimal gland evaluation S‑shaped ptosis+/− Palpebral lobe prolapse Conjunctivochalasis Puncta Opening Globe apposition Position (punctal ectropion+/−) Number (supernumary puncta) ROPLAS Type of fluid (clear/mucoid/mucopurulent) Probing Hard stop (mm) Soft stop (mm) Sac Syringing [Figure 6] Freely patent Site (opposite/same puncta/cutaneous fistula) Clear/mucoid fluid Palpation of lacrimal sac area (mucocele/tumor) Lid laxity Nasal endoscopy bedside ROPLAS: Regurgitation on pressure on lacrimal sac Ideal to have an ENT opinion on nasal status before surgery. Trauma [Table 5] Table 5: Findings of trauma evaluation Examination findings Right eye Left eye Vision (BCVA) Color vision (Ishihara) IOP Pupil Motility and double vision Orbital margins Crepitus Lid laceration/canalicular involvement Infraorbital sensations Hertel’s for enophthalmos [Figure 7] Forced duction test under monitoring of vitals Jaw opening (TMJ fracture) Diplopia/Hess charting General: ENT examination, pulse, BP BCVA: Best-corrected visual acuity, BP: Blood pressure, TMJ: Temporomandibular joint, IOP: Intraocular pressure, ENT: Ear nose throat Socket [Table 6] History Cause for anophthalmos, previous surgery, previous history of tumor. Table 6: Findings of Socket evaluation Examination findings Right eye Left eye Palpebral aperture (with prosthesis) Vertical (mm) Horizontal (mm) Volume (enophthalmos/proptosis) Surface (congestion, keratinization, papillae, granuloma, thinning, and visibility of implant) Fornix Adequate/shortening Shelving (+/−) Lids Entropion Ectropion Sulcus deformity [Figure 8] Implant Present/absent/migrated Extrusion (+/−) Prosthesis Surface Edges Sheen Color match Retainability Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. 368 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 Figure 8: Right‑sided anophthalmic socket with ill‑fitting prosthesis, enophthalmos, and superior sulcus deformity

Abstract Theme Section Introduction Optic nerve and visual pathway or central nervous system diseases often present with nonspecific complaints and subtle signs, which can often be missed in the outpatient department (OPD). A good clinician should know when to investigate and intervene in such cases. We present a basic overview of examination pro forma, which can come in handy during the OPD. Talking to the Patient Clinical history is an integral part of the workup and diagnosis in neuro-ophthalmology. The most commonly encountered complaint is diminished vision. The clinician should make sure that there is a blurring of the fine details, loss of central vision, loss of peripheral field of vision, distortion of images, or diminution of near vision. The associated ocular complaints vary from eye pain, headache, deviation of eyes, nystagmus, or diplopia. The chronology and speed of onset of the symptoms give essential clues to the diagnosis. The occurrence of visual loss before pain suggests an optic pathway lesion, whereas pain before visual loss could be an inflammation or external compression. There can be additional systemic symptoms like neurological deficits (hemiplegia and deviation of the face) or complaints specific to systemic diseases such as diabetes mellitus, hypertension, tuberculosis, or hormonal imbalance. Any history of use of systemic medications such as antitubercular therapy, antipsychotic medications, or antiepileptic medications, must be elicited. The clinical examination can thus be targeted toward the differentials established with the history. Back to Clinical Basics The preliminary examination starts with assessing the visual status of the patient. Vision must be assessed under the following headings: 1. Distance vision: This assesses the foveal function. Uncorrected distance vision can be assessed using the Snellens’ alphabet charts, Landolt C-charts, or Illiterate E-charts. The accepted ideal distance is six meters. The possibility of refractive errors and media opacities must be considered and the best possible visual acuity be ascertained. The available refractive aids with the patient may not correlate with the actual refractive status, and Neuro‑ophthalmology patients present with nonspecific complaints and are often missed in the outpatient department (OPD). This review systematically outlines the basics of clinical examination essential for picking up subtle clinical signs and corroborating the findings to arrive at a provisional diagnosis. These patients require a multidisciplinary approach to management. Ahigh degree of clinical suspicion, appropriate clinical history, and examination, imaging, or systemic workup if needed, can aid in early diagnosis of neuro-ophthalmology cases. We also add a note about commonly seen neuro-ophthalmology cases in the OPD. Keywords: Examination, neuro-ophthalmology, techniques Address for correspondence: Dr. Shalin Shah, Department of Ophthalmology, Guru Nanak Eye Centre and Maulana Azad Medical College, Bahadur Shah Zafar Marg, New Delhi ‑ 110 002, India. E‑mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] How to cite this article: Shah S, Dutta P. Neuro-ophthalmology – An approach to common cases in the outpatient department. Delhi J Ophthalmol 2023;33:369-72. Neuro-Ophthalmology – An Approach to Common Cases in the Outpatient Department Shalin Shah, Paromita Dutta Department of Ophthalmology, Maulana Azad Medical College, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/DLJO.DLJO_198_23 Submitted: 20-Dec-2023 Accepted: 28-Dec-2023 Published: 15-Jan-2024 © 2024 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 369

Shah and Dutta: Approach to a neuro‑ophthalmology case fresh refraction should be attempted for all cases. The use of a pinhole helps a fair estimate of the best possible visual acuity. Assessment of distance vision is challenging in children and requires the assistance of picture charts 2. Near vision: The charts with preprinted texts at a handheld distance (33 cm) are classically used for near vision assessment. The correction of presbyopia is an essential step. Any loss of accommodation inconsistent with presbyopia or cycloplegia should raise a suspicion of ciliary ganglion lesions 3. Projection of light rays: It assesses the peripheral retina and optic nerve. Inaccuracy in the projection of rays, with a healthy-looking retina, is suggestive of neuro-ophthalmic lesions 4. Color vision: The pseudoisochromatic plates of Ishihara are classically used to evaluate the cone function. The optic nerve and visual pathway lesions predominantly demonstrate a red-green defect with the exception of glaucomatous optic atrophy which shows blue-yellow changes in early disease. The Hardy-Rand-Rittler plates and Farnsworth-Munsell 100 hue test offer a more accurate color vision assessment. The red color desaturation test elicits an early sign of unilateral optic neuritis, where the red color is perceived as greyish in the affected eye 5. Contrast sensitivity: The function of foveal cones is assessed by higher order gratings, while lower gratings of contrast are for peripheral macular function. The contrast sensitivity can be assessed with Pelli-Robson or FACT charts. It is often the earliest visual function to be affected in optic neuritis and visual pathway lesions 6. Field of vision: Clinical examination is incomplete without confrontational field assessment. The visual pathway lesions present with characteristic field loss, which should not be missed in the OPD. The neuro‑ophthalmology cases have field loss respecting the vertical midline, as opposed to glaucomatous loss which respects the horizontal midline. The altitudinal field loss in anterior ischemic optic neuropathy and hemianopias in visual pathway lesions are commonly seen 7. Stereopsis: This assesses the binocularity along with the foveal function. Stereo acuity cards at a handheld distance are commonly used 8. Photo stress test: Light adaptation and recovery period can be tested by exposing the eyes to bright light for 60 s and waiting for a return to baseline visual acuity. This macular function test can be impaired in cases of internal carotid artery obstruction. The external examination involves the assessment of facial symmetry and eye-lid examination. Facial nerve involvement can present with loss of forehead creases, lagophthalmos, loss of nasolabial fold, and drooping of the angle of the mouth. Ptosis can be seen ipsilaterally in Horner’s syndrome and oculomotor nerve lesion. Bilateral ptosis can occur in cases of oculomotor nucleus and midbrain lesions. Variable ptosis during the day is suspicious for ocular myasthenia. The association of proptosis may point toward an orbital lesion compressing the optic nerve. A retropulsion test should be performed in any suspected case to rule out orbital space-occupying lesions. Extraocular muscle movement examination also adds valuable information for the diagnosis of neuro-ophthalmology cases. The examination should include: 1. Head position: Any abnormal head position, compensatory posture, or mannerisms should be noted. The face turn or head tilt is toward the direction of action of the paralyzed muscle. Paradoxical head tilt can also be seen, like cases of acquired unilateral superior oblique or trochlear nerve palsy, where the head is tilted ipsilateral to the underacting muscle (Bielchowsky phenomenon) 2. Range of movements: The movements are assessed mono‑ocularly (ductions) and binocularly (versions) 3. Hirschberg test: A simple torch light test to gauge the type and angle of heterotropia. Any change in the amount of deviation in a particular gaze should be noted to diagnose incomitant squints. Patients with raised intracranial pressures often present with esotropia, due to bilateral abducent nerve palsy 4. Cover tests: The direct cover test helps assess the fixation, type of heterotopia, and angle of primary deviation. The alternate cover breaks the binocularity and unmasks the heterophoria component 5. Park–Bielschowsky test: The cases of vertical strabismus should be assessed with the three-step test if a single muscle palsy is suspected. This test is very useful for the diagnosis of superior oblique palsy 6. Measuring angle of deviation: The prism bar cover test helps measure the angle of deviation. Using the Krimsky test, the angle of primary and secondary deviation should be meticulously documented in cases of paralytic strabismus, presenting to neuro-ophthalmology OPD. Serial measurements at follow-up visits help monitor the recovery of nerve palsy cases 7. Diplopia assessment: The cases of acquired strabismus-like paralytic lesions present with troublesome diplopia. Mono-ocular diplopia should be ruled out. The severity of diplopia should be graded as per the Bahn–Gorman system. The diplopia must be checked in all gazes, as worsening is expected in the direction of action of the paralytic muscle. Diplopia charting should be repeated at each visit to monitor the recovery of paretic strabismus. Torsional diplopia can be assessed with the double Maddox rod method. The Hess/Lee charts also aid in the diagnosis and monitoring of paralytic strabismus cases. Long-standing paretic lesions can present with restrictive squint. Variable diplopia can be associated with ocular myasthenia cases 8. Nystagmus assessment: The presence of nystagmus should be assessed in binocular state and all gazes. The cover test helps unmask the latent component of nystagmus, which is commonly associated with childhood 370 Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023

Shah and Dutta: Approach to a neuro‑ophthalmology case lesions. Assessment of saccades, pursuits, and refixation movements using an optokinetic drum is essential. The cases of supranuclear palsy show the decreased speed of vertical saccades, dementia has increased latency to saccades, cerebellar disorders characteristically show saccadic hypermetropia (over‑shoot), while internuclear ophthalmoplegia has slowed of adduction saccades 9. Vergence assessment: Assessment of convergence and miosis during near reflexes is essential. Prisms can be used to calculate convergence and divergence amplitude 10. Vestibular assessment: Any occurrence or exacerbation in nystagmus with vestibular stimuli should be ruled out in all neuro-ophthalmology cases. The assessment of pupils is the next important step in the OPD. Pupil examination is performed under the following parameters: 1. Size: The pupil size should be measured with distance fixation under ambient illumination. Anisocoria should be assessed for worsening in bright or dim illumination. Pupil abnormalities should raise suspicions of drug use or an efferent pathway defect – Either in sympathetic or parasympathetic (oculomotor nerve) pathways. Physiological anisocoria can occur, with a difference in the size of <1 mm 2. Light reflex: Direct and consensual light reflexes should be assessed for each side. A brisk pupil reaction with well-sustained response suggests a healthy pupillary innervation. A swing flashlight test should always be done to confirm relative afferent pupillary defect (RAPD) 3. Near reflex: Loss of miosis with preserved convergence and accommodation suggests a light-near dissociation. This is seen in mid‑brain lesions such as neurosyphilis (Argyll Robertson pupil) and Holmes‑Adies syndrome 4. Pharmacological testing involves the instillation of diluted concentrations of miotic or mydriatic agents in cases of anisocoria. In cases of denervation supersensitivity, the anisocoria improves with topical diluted drugs. The assessment remains incomplete without a detailed examination of the retina and optic nerve head. Disc pallor is a common finding in neuro-ophthalmology patients. Primary optic atrophy should be carefully distinguished from secondary and consecutive optic atrophy. The use of slit-lamp biomicroscopy helps in eliciting finer vascular details such as optociliary shunts in optic nerve sheath meningioma and Paton’s lines in disc edema. Pseudo-disc edema due to causes such as hypermetropia, disc drusen, small crowded disc, and myelinated retinal nerve fibers must be ruled out. The retinal and disc findings must be correlated to associated field defects. A comprehensive clinical assessment yields a provisional diagnosis, which would require further investigation. Ophthalmology Investigations The ophthalmology workup beyond the clinical examination includes: 1. Intraocular pressure (IOP): IOP assessment using Goldmann applanation is necessary to rule out any associated pressure effects on the optic nerve head, besides the neuro-ophthalmology diseases 2. Automated perimetry: To document and diagnose visual field changes. Subtle visual field defects may be missed on confrontational perimetry, which can be picked up with standard automated perimetries 3. Electrophysiology testing: Visual evoked potential (VEP) and electroretinogram (ERG) can be useful adjuncts in selected cases. Flash VEP can provide a basic idea about visual potential in preverbal children. Pattern VEP and ERG can supplement the findings of clinical examination in cases of optic neuritis, cone-rod disease, etc. These investigations should, however, be interpreted with caution, and not be used as a standalone tool for diagnosis. Imaging and Systemic Workup A detailed neurology examination for higher functions, cranial nerve examination, cerebellar and vestibular evaluation are an integral part of patient workup. Assessment of vitals, blood sugar control, liver and renal functions, hemogram, and serum lipid profile form a necessary basis for further management. Neuroimaging is often required in the form of magnetic resonance imaging (MRI) of the brain, with contrast‑enhanced or specific sequences for definite indications. The use of magnetic resonance (MR) venography for sagittal sinus thrombosis, MR angiography, and color Doppler of the carotid artery for suspected arterial occlusions are the valuable specialized tools. Let us take a look at some of the common neuro-ophthalmology cases presenting in the OPD. Optic neuritis Optic neuritis patients can present with the first episode or recurrent attacks of diminished vision, with retrobulbar pain on eye movements. A detailed chronological history of any associated focal neurological deficits should be elicited, to look for dissemination of lesions with time. Optic neuritis can often be the presenting symptom in multiple sclerosis (MS) cases, and further episodes may be essential to satisfy McDonald’s criteria for MS diagnosis. The clinical examination proceeds as per the previously described format. Special attention should be paid to the following key points. 1. RAPD may be the earliest finding with preserved Snellen visual acuity 2. Extraocular movements, especially adduction, and elevation, may elicit pain due to the close proximity of the optic nerve to the origin of medial and superior rectus muscle fibers 3. Optic nerve head swelling (disc edema) can be present. However, cases of retrobulbar neuritis(RBN) present with a normal-looking disc in the early stages. Long-standing cases tend to develop disc pallor with the onset of optic atrophy Delhi Journal of Ophthalmology ¦ Volume 33 ¦ Issue 4 ¦ October-December 2023 371