Contents
Editorial Oculoplasty
04 Revolution in Techniques and Technology in 38 Orbital Cellulitis Post Uneventful
Cataract Surgery Phacoemulsification
Beyond Ophthalmology 41 Evaluation of Acquired Ptosis
05 COVID-19 Related Conjunctivitis: A Short Review Basics
10 COVID-19 and its Repercussions on Ophthalmology
46 Importance of Complete Histopathology and
What’s New Immunohistochemistry Work up in Ocular
Pathology to Diagnose a Rare case
14 An Overview of Artificial Intelligence in
Ophthalmology PG Corner
Subspecialities 49 Filamentary Keratitis
Cornea Systemic Diseases and Eye
19 Congenital Fibrosis of Extra-ocular Muscles 52 Ocular Sarcoidosis
Pediatric Ophthalmology Monthly Meeting Update
22 Paediatric Ocular Biometry and IOL Power 55 Advances and Newer Trends in Cataract Surgery
Calculation 67 Bilateral Simultaneous Loss of Vision: A Clinical
24 “When all else fails” - Secondary Episcleral Plaque Dilemma
Brachytherapy in Retinoblastoma 72 Optic Atrophy - Easy to Catch, Difficult to Diagnose
75 Optimizing Outcomes in Uveitic Cataract
27 Congenital Corneal Opacities
DOS Quiz
Glaucoma
Tearsheet
32 Anterior Segment Dysgenesis
84 Types of Lacrimal Stents or Intubation Systems
Retina
36 Choroidal Melanoma
02 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
DOS Executive Members 2019-21
DOS Office Bearers
Dr. Subhash C Dadeya Dr. Pawan Goyal Dr. Namrata Sharma Dr. Hardeep Singh
President Vice President Secretary Joint Secretary
Dr. Jatinder S Bhalla Dr. Vinod Kumar Dr. Manav Deep Singh
Treasurer Editor Library Officer
Executive Members
Dr. Dewang Angmo Dr. Jatinder Bali Dr. Shantanu Gupta Dr. C. P. Khandelwal
Dr. Rahul Mayor Dr. Vipul Nayar Dr. Rajendra Prasad Dr. Kirti Singh
DOS Representative to AIOS Ex-Officio Members
Dr. Jeewan S. Titiyal Dr. M. Vanathi Dr. Rakesh Mahajan Dr. Subhash C Dadeya Dr. Arun Baweja
Volume 25 No. 6, May-June, 2020
Editor In Chief DOS Times Editorial Board
Namrata Sharma
Editorial Board National Board
Editor Dr. Parul Icchpujani
Prafulla Kumar Maharana Dr. Atul Kumar Dr. Ronnie George
Dr. Aniruddha Maiti Dr. Sushmita Kaushik
Assistant Editors Dr. Apporva Ayachit Dr. Gopal Pillai
Dr. Jitendra Jethani Dr. Usha Singh
Ritu Nagpal Sahil Agarwal Dr. Mita Joshi Dr. Subhendu Boral
Dr. P. Dutta Majumdar Dr. Meena Chakrabarti
Dr. Noopur Gupta Dr. Raksha Rao
Dr. Brijesh Kakkar Dr. Kumudini Verma
Dr. Digvijay Singh Dr. Rashmin Gandhi
Dr. Ritika Sachdev Dr. Siddharth Kesarwani
Dr. Dewang Angmo Dr. Chaitra Jayadev
Dr. Rebika Dr. Bibhuti P. Sinha
Dr. Saurabh Sawhney Dr. Amit Porwal
Dr. Reena Sharma Dr. Prashant Bawankule
Dr. Rajat Jain Dr. Arvind Kumar Morya
Dr. Jaya Gupta
Deepali Singhal Rahul Kumar Bafna Dr. Anita Ganger
Dr. Umang Mathur
Mohamed Ibrahime Asif Gunjan Saluja Dr. Neera Agarwal
Dr. Poonam Jain
Dr. Manisha Agarwal
Dr. Hardeep Singh
Dr. Anita Sethi
Dr. Tushar Agarwal
Dr. Rohit Saxena
Dr. Swati Phuljhele
Dr. Vivek Dave
Dr. Mohita Sharma
Dr. Rajesh Sinha
Dr. Ritu Arora
Dr. P.K. Pandey
Dr. H.K. Yaduvanshi
Dr. O.P. Anand
Sohini Mandal
04 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Editorial
From the
Editor’s Desk
Revolution in Techniques and Technology in Cataract Surgery
Respected Seniors and Friends,
The field of cataract surgery has been an ever-evolving branch. With days passing by,
scrupulous work is being done to improve the accuracy and outcome of cataract surgeries.
Although cataracts are one of the most common causes of vision loss and blindness across the
world, advances in technology and innovations have allowed the treatment of this pathology
to be very safe and efficacious. Thanks to many of the innovators, we have come a long way
from the practice of couching.
What started with couching has now evolved into a robotic surgery with availability of modular
IOLs which help to improve the vision post-surgically too. Cataract surgery has progressed with
ICCE, ECCE, SICS, Phacoemulsification and FLACS as a few of the pathbreaking milestones.
Through all these times, there have been various advances in phacoemulsification technology
as well such as Femtosecond Laser Assisted Cataract Surgery, Zepto cataract surgery, Capsulo-
Laser, intra-operative use of OCT in cataract surgery, intra-operative aberrometry, heads
up cataract surgery and robot assisted simulated cataract surgery. But unfortunately, many Prof. (Dr.) Namrata Sharma
people do not have access to modern cataract surgery juxtaposed with an aging population, the
incidence and prevalence of cataracts will therefore continue to rise. (MD, DNB, MNAMS)
Femtosecond Laser-Assisted Cataract Surgery (FLACS), approved by the FDA in 2010 has an Hony. General Secretary
imaging software to image the cornea, capsule, lens and the anterior chamber. It can perform Delhi Ophthalmological Society
corneal incisions for entry into the eye and for astigmatism correction, capsulotomy, and
fragmentation. Surgeons can use this technology for corneal astigmatism correction or in cases Cornea, Cataract & Refractive Surgery Services
where advanced-technology lenses, such as astigmatism correcting (toric) lenses are inserted. Dr. R.P. Centre for Ophthalmic Sciences,
Zepto Cataract surgery is the latest advancement in automated cataract surgery. “Zepto All India Institute of Medical Sciences (AIIMS)
New Delhi
Capsulotomy Device” was developed around a decade ago. The device is disposable, handheld, and attached to a state of the art console. It is
made of Nitinol alloy, which can be inserted through a 2.2mm corneal incision. It uses a built-in vacuum and fast, low-energy pulses of direct
current to perform a perfectly centered and circular capsulotomy.
Intraoperative optical coherence tomography (iOCT) enables real-time visualization of ocular structures during surgery and enhances our
understanding of intraoperative dynamics. It also finds an application in phacoemulsification wherein it helps to assess corneal incisions,
intralenticular pressure, and posterior capsule integrity during nuclear emulsification. iOCT aids in decision-making during various anterior
segment surgeries, and its efficacy and feasibility in anterior lamellar keratoplasty and endothelial keratoplasty is well established. In addition,
it aids in establishing diagnosis, and provides intraoperative guidance in pediatric patients who are uncooperative for examination.
The heads-up three-dimensional visualization is an evolving technology demonstrating comparable efficacy to the standard operating
microscope for cataract surgery. The practice of 3D heads-up display systems in the COVID era aids in maintaining an adequate distance
between surgeon and patient and also in improving ergonomics. Moreover, personal protective equipment are better tolerated by the surgeon
using 3D heads- up viewing system. It provides five times extended depth of field and helps in maintaining the sharp focus across the expanded
surgical space.
In future, we desire to see our patients as well as surgeons completely satisfied with the post-surgical outcomes of cataract surgery.
With best wishes.
Prof. (Dr.) Namrata Sharma
(MD, DNB, MNAMS)
Hony. General Secretary
Delhi Ophthalmological Society
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 05
Beyond Ophthalmology
COVID-19 Related Conjunctivitis: A
Short Review
Rinky Agarwal MD, DNB, MNAMS, Namrata Sharma MD, DNB, MNAMS
Dr. Rajendra Prasad Centre for Ophthalmic Sciences. AIIMS, New Delhi, India.
Severe Acute Respiratory Syndrome While early studies failed to isolate the conjunctivitis as the first symptom
CoronaVirus 2 (SARS-CoV- 2) is a viral RNA from tears and ocular surface, of the disease18. Rarely, it can be the
novel virus responsible for ongoing other studies provided contrary results, sole manifestation of the disease. For
COVID-19 pandemic. Its emergence albeit with a low incidence9-13. example, Scalinci et al reported 5 cases
in China at the end of 2019 has caused of acute conjunctivitis as the primary
a large global outbreak affecting over Although the concept of ocular surface and sole manifestation of COVID-19
200 countries1,2,3. The SARS-CoV-2 is as a source of viral transmission has infection.19 As per recent reports,
an enveloped, single-stranded RNA always been speculative, the role of conjunctivitis along with anosmia and
virus and characteristically affects the lacrimal drainage and interaction of hyposmia may be a hidden indicator of
respiratory system. Although the virus virus with CD147 (which is present early SARS CoV-2 infection.
appears not quite as likely to cause in tear and human ocular tissues, e.g.,
mortalities as the SARS coronavirus corneal epithelium, endothelium, Other ocular complaints of COVID-19
or MERS coronavirus, it is highly keratocytes, conjunctiva, and retinal infection include15.
transmissible and has a significant pigment epithelium) in its pathogenesis
fatality rate, especially in the elderly and in newer studies showed contrary • Conjunctival congestion,
those with systemic comorbidities such results13-16. Even an animal study • Increased conjunctival secretion,
as immune suppression, respiratory conducted by Deng et al on macaque’s • Epiphora,
disease and diabetes mellitus. Patients conjunctiva showed that inoculation • Blurring of vision,
typically present with respiratory of conjunctiva with SARS-CoV-2 lead • Ocular pain,
illness, including fever, cough and to the development of mild interstitial • Foreign body sensation,
shortness of breath. Ocular complaints pneumonia, implying that ocular • Epithelial defect,
are infrequent manifestation of the tissues and fluid may be a potential • Pseudodendrite formation and
disease with conjunctivitis as the most alternative mode of SARS-CoV-2 • Subepithelial infiltrates.
common presentation. transmission17. Correlation with systemic disease
According to Loffredo’s meta-analysis,
Pathogenesis Clinical presentation the incidence of conjunctivitis may be
The virus, after gaining access into The incidence of COVID-19 associated directly proportional to the severity
body, interacts with cells having conjunctivitis is 1.1% which can range of the systemic disease20. They also
Angiotensin converting enzyme 2 from as low as 0.8% to as high as 31.6%. suggested that ocular symptoms
(ACE2) on their surface following Symptoms may appear as early as 2 commonly appear in patients with
which ceaseless viral multiplication days or may be delayed till 14 days after severe pneumonia and the overall rate
undertakes resulting in severe cytokine contracting systemic infection. It is of conjunctivitis was 1.1%; it was 3%
storm and inevitable organ damage3,4. usually bilateral but unilateral cases and 0.7% in severe and non-severe
Based on this theory, the virus has have also been reported. Both virus COVID19 patients, respectively. The
been suggested to have aerodigestive induced and secondary superinfections metaanalysis also demonstrated that
spread and its ocular transmission has have been implicated as the cause of patients with severe COVID19 infection
been debatable with studies doubting COVID-19 related conjunctivitis. had, at admission to the hospital,
the presence and action of ACE-2 increased incidence of conjunctivitis.
receptors on conjunctival and corneal According to a recent metanalysis by This implies that patients with
epithelium5-8. The receptor is mainly Bhattacharyya (total 854 patients), conjunctivitis consequently may need
found in the posterior tissues of the eye reported pooled prevalence of
which are rarely affected in the disease. conjunctivitis was 3.175%, however
only 0.703% patients reported
06 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Beyond Ophthalmology
more frequent systemic monitoring. positive in 3/43 cases compared to ventilation24. These individuals are also
Liang et al. discovered that the viral load confirmatory nasopharynageal swab in prone to conjunctival hyperemia and
of conjunctival sac secretion was directly 30/43 cases22. Supporting this, Chen et mucopurulent discharge as cocktail
proportional to the severity of the al also showed low expression of virus of medications (sedatives, inotropes
disease21. Wu et al. noted an interesting in ocular secretions when compared to and muscle relaxants) employed
relationship between conjunctivitis respiratory specimens.16 According to for successful ventilation interfere
and severity of COVID-19 and blood a recent metanalysis by Bhattacharyya, with blink reflex and tear secretion.
parameters. They reported that patients the pooled proportion of COVID-19 Therefore, all ventilated patients need
with ocular symptoms were more likely patients that showed tear positivity is close ocular monitoring with special
to have higher white blood cell and 1.949%18. attention towards lid closure and blink
neutrophil counts, and higher levels of reflex for timely management of this
procalcitonin, C-reactive protein, and Unfortunately, all these studies have condition.
lactate dehydrogenase than patients been conducted outside India. Genetic
without ocular symptoms. Their differences may make its presentation Management
investigation revealed that patients variable in Indian sub-population. In In Table 1, we have a tabulated
with positive ocular signs were noted a prospective study by Kumar et al chronological review of important
to have more severe manifestations who recruited 45 confirmed COVID studies on this topic is added to help the
of systemic COVID-19 infection or positive cases, only one (2.23%) patient clinicians diagnose and manage similar
abnormal findings on laboratory blood had detectable levels of SARS CoV 2 in situations (Table 1)1-36.
investigations. conjunctival swabs using RT PCR23. As all cases reported till date have been
self-resolving and benign in nature,
Diagnosis However, the tear positivity may conjunctivitis is-alone afflicted patients
The diagnosis of COVID related be affected by the timing of sample can be advocated self-quarantine
conjunctivitis is commonly established collection, time for maximum and followed-up telephonically until
on slit-lamp examination but replication of the virus, possibly complete recovery to avoid unnecessary
telemedicine can also be utilized less secretion of the virus through communication of the virus. The
for such purposes to prevent cross- conjunctival secretion, and also low management is commonly conservative
infections. sensitivity of RT‑PCR. Sometimes, and a trial of frequent preservative-free
Although the Coronavirus RNA has in patients with conjunctivitis as a lubricants and topical antibiotics are
been detected in tears and ocular sole exhibition of active infection, frequently sufficient to prevent or treat
surface, the conjunctival sac titres conjunctival swab evaluation may be bacterial superinfection. The use of
as well as the infectivity have been necessary for diagnosis. In these cases, topical ribavirin and ganciclovir for its
reported to progressively decrease over swabs are obtained by putting sterile treatment mandates larger comparative
time. The ocular samples, both tear synthetic fibre swabs into the lower trials for their safety and efficacy
secretions and conjunctival swabs have fornix of each eye without topical considering their cost and availability.
a low positivity rate (around 1.949%) anaesthesia which are then inserted Few clinicians have also advocated
and therefore, are not suitable for into a viral transport medium for 1% Povidone Iodine for treatment of
diagnosis. Wu et al studied 38 COVID- detecting SARS-CoV-2. COVID associated conjunctivitis37. The
19-positive patients in Hubei Province, same drug has also been speculated to
China and demonstrated that only 2 out Differential diagnosis play a protective role for postexposure
of 12 patients with ocular complaints Numerous differential diagnosis of prophylaxis with the virus. However,
had positive conjunctival swabs. While COVID-19 associated conjunctivitis the present literature in this regard is
Deng et al concluded that no SARS- include limited38.
CoV-2 could be detected by RT-PCR in
114 conjunctival swabs samples from • Other viral and bacterial Precautions
patients with COVID-19 pneumonia, Xia conjunctivitis, The nature and proximity of the
et al reported a positivity rate of 0.33% ophthalmic examination renders
(1/30 patients)10,15. A study by Zhou et • Allergic conjunctivitis, eye-care providers at a higher danger
al identified 67 laboratory-confirmed • Foreign body in lids, of acquiring the infection. The virus
or suspected COVID-19-positive • dry eye syndrome, has also been detected in normal
patients, out of which 3 had positive • Exposure keratopathy in an conjunctiva of COVID-19 affected
RT-PCR results from conjunctival swab. subjects. Considering the decreasing
Karimi et al showed tear RT-PCR was intubated patient. viral loads over time, maximum
Ofoneparticularinterestisconjunctivitis transmission rates are expected during
in critically ill patients admitted in
intensive care units for mechanical
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 07
Table 1: Chronologically tabulated current literature on ocular findings in COVID-19 patients
Author, country Publication Type of Sample Mean age Ocular Timing of Conjunctivitis Specimen positivity
date study size Specimen collection
Bilateral www.dosonline.org/dos-times
Chen, China16 Feb 6th 2020 Case report 1 30year Conj swab 14days 1/67 (1.49%) Ocular Nasopharyx Management
1/30 (3.33%) Positive Positive Topical
3/37 (8.11%) Ribavirin
Zhou, China25 Feb 12th Retro- 67 35.70± Conj swab Not specified 1/17 (5.88%) 3/67 (4.48%) 94.02% ---
Xia, China15 2020 spective 30 10. 6yr 12/38 (31.58%)
Liang, China21 Feb 19th Prospective 37 54.50± Tear+conj 7.33±3.82 2/12 (16.66%) 2/60 (3.33%) 100% --
Seah, Singapore11 2020 17 14. 17yr secretions days Bilateral
Ping Wu, China13 Mar 1st 2020 Prospective 38 --- Conj swab Not specified Unilateral 1/37 (2.70%) --- ---
Salducci, Italy26 Mar 24th Prospective 1 -- Tear 3 to 20days Bilateral none All positive --
2020 samples Follicular
Mar 31st Prospective 65.8± 16. Conj swab Not specified conjunctivitis 2/38 (5.26%) or 73.7% ---
2020 60yr Unilateral
Mar 2020 Case report 72yrs Not sent Not applied 5/5 Not applied Positive Ganciclovir
Conjunctivitis + gel, lubricants
Cheema, Canada27 Apr 2nd, Case report 1 29yr Conj swab 6days dermatitis Positive Positive Oral
Colavita, Italy28 2020 65yr Bilateral valcyclovir
Khavandi, Iran29 Case report 1 65yrs Conj swab Daily from 3 to Positive Positive Self-resolving
Daruich, France30 Apr 17th 27yr 21days Unilateral
Scalinci, Italy19 2020 Case report 1 46.8yr Conj --- Bilateral Positive Positive Antibiotic-
Wu, China31 2.5yrs secretion Hemorrhagic, steroid
Xie, China32 Apr 21st Case report 1 57.6yr Not sent Not applied pseudo- Not applied Positive Topical
2020 membranes Moxifloxacin
Case series 5 48yr Not sent Not applied 2/43 (4.65%) --- 100% ---
Apr 21st 54yr None
2020 Case report 1 63yrs Not sent Not applied 4/15 (15%) Not applied Positive --- 08 DOS Times - Volume 25, Number 6, May-June 2020
Apr 24th Retro- 33 Ocular 4 to 5days 2/33 (6.06%) 100% Tobramycin-
2020 spective surface Not applied steroid
swab ---
Apr 24th
2020 Not sent Lubricants,
serum,
Apr 26th azithromycin
2020 ---
Beyond Ophthalmology Casalino, Italy33 Apr 28th Case report 1 Not applied Positive ---
Ying, Malaysia34 2020 Not applied Positive
Navel, France35 Case report 1 Not sent Not applied
Apr 30th
2020 Case report 1 Tear+conj 17th and 20th
secretion day
May 4th
2020
Karimi, Iran22 May 18th Prospective 43 56±13yr Tear 1 to 7days 3/43 (6.97%) 69.76%
Kumar, India23 2020 Prospective 45 samples 2 to 5days
Valente, Italy36 May 25th Prospective 27 31.26± Conj swab --- 1/45 (2.33%) Positive
2020 12.81yr
Jun 9th 2020 84mon Conj swab 3/27 (11%) or 100%
1/4 (25%)
Conj- Conjunctival: All studies used RT-PCR, reverse transcriptase polymerase chain reaction, for diagnosis of COVID-19
Beyond Ophthalmology
the early phase of infection. It is, 1. James Chodosh, Gary N. Holland, Steven 12. Mungmungpuntipantip R, Wiwanitkit
therefore, imperative to wear protective Yeh. Important coronavirus updates V. Ocular manifestation, eye protection,
equipment (N95 mask, gloves, gown, for ophthalmologists. AAO headlines: and COVID-19. Graefes Arch Clin Exp
cap, and face shield) while subjecting March 30, 2020. Ophthalmol. 2020;4662.
any individual to routine ophthalmic
investigations such as slit-lamp 2. Guan W, Ni Z, Hu Y, et al. Clinical 13. Wu P, Duan F, Luo C et al. Characteristics
examination, non-contact tonometry Characteristics of Coronavirus Disease of ocular findings of patients with
and fundoscopy to eradicate the risk of 2019 in China. New Engl J Med 2020. coronavirus disease 2019 (COVID
aerosol spread. 2020;382(18):1708-1720. 19) in Hubei Province, China. JAMA
Ophthalmol 2019; 2020: 10– 13.
Routine appointments and elective 3. Zhou P, Yang XL, Wang XG, et al. A
surgeries can be postponed till complete pneumonia outbreak associated with a 14. Zhou Y, Duan C, Zeng Y, Tong Y,
resolution of signs and symptoms. newcoronavirus of probable bat origin. Nie Y, Yang Y, et al. Ocular Findings
Tele-ophthalmology can be employed Nature. 2020;579(7798):270-273. and Proportion with Conjunctival
actively to triage these patients and SARS-COV-2 in COVID- 19 Patients.
only those mandating emergent care 4. Wan Y, Shang J, Graham R, et al. Receptor Ophthalmology. 2020 Apr 21.
be called for personal review. Patients recognition by novel coronavirus from
can be advised to actively practice Wuhan:An analysis based on decade- 15. Xia J, Tong J, Liu M, et al. Evaluation of
home‑based monitoring of vision using long structural studies of SARS. J Virol. coronavirus in tears and conjunctival
Snellen’s charts, Amsler’s grid and 2020;94(7):e00127-20. Published 2020 secretions of patients with SARS-CoV-2
smartphone apps. The patients called Mar 17. infection. J Med Virol 2020.
for an out-patient review should be
allowed inside only after ensuring masks 5. Savaskan E, Löffler KU, Meier F, 16. Chen L, Liu M, Zhang Z, et al. Ocular
and hand hygiene/gloves. To avoid Müller-Spahn F, Flammer J, Meyer P. manifestations of a hospitalised
overcrowding, physical distancing must Immunohistochemical localization patient with confirmed 2019 novel
be adopted. The examination should be of angiotensin-converting enzyme, coronavirus disease. Br J Ophthalmol.
functioned in a ventilated place (Open angiotensin II and AT1 receptor in 2020;104(6):748 751.
door ventilation with no internal human ocular tissues. Ophthalmic
recirculation of air) and air disinfection Res.2004;36(6):312-320. 17. Wei Deng, Linlin Bao, Hong Gao et
should also be employed regularly. Any al. Ocular conjunctival inoculation
contact procedure should be planned 6. Sun Y, Pan X, Liu L, Ni RX. Expression of of SARSCoV- 2 can cause mild
judiciously and strict sterilization SARS coronavirus S protein functional COVID-19 in Rhesus macaques. bioRxiv
practices are mandatory for shared receptorACE2 in human and rabbit 2020.03.13.990036.
clinical equipment. cornea and conjunctiva. Rec Adv
Ophthalmol. 2004:24(5):332-336. 18. Bhattacharyya J, Prajapat M, Shekhar
Conclusion N, et al. Ocular manifestations and tear
To conclude, although the present 7. Liu L, Sun Y, Pan X, Shen W, Liu or conjunctival swab PCR positivity for
literature suggests low risk of ocular 2019-ncov in patients with covid-19: a
transmission, non-occurrence of ZY, Liu YP. Expression of SARS systematic review and meta-analysis.
ophthalmic symptoms doesn’t equate to coronavirus S proteinfunction1al Soc sci res netw.
absence of COVID-19 virus in the ocular receptor- angiotensin-converting
fluids. Therefore, ocular manifestation enzyme 2 in human cornea and 19. ScalinciSZ,BattagliolaET.Conjunctivitis
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not be overlooked both by general symptom of COVID-19. IDCases.
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ophthalmic healthcare personnel 8. Sun Y, Liu L, Pan X, JingM.Mechanism Tiscione, G., Oliva, A. and Violi, F.
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10 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Beyond Ophthalmology
COVID-19 and its Repercussions on
Ophthalmology
Rinky Agarwal MD, DNB, MNAMS, Namrata Sharma MD, DNB, MNAMS
Dr. Rajendra Prasad Centre for Ophthalmic Sciences. AIIMS, New Delhi, India.
The ongoing COVID-19 pandemic • Green Zones: Districts having zero government institutes in certain areas
instigated by the SARS – CoV-2 confirmed cases of coronavirus in and multiple centres shrunk to offering
virus has befuddled the entire globe, the last 21 days. only emergency services. In a survey
forcing multiple nations into a self- assessing the effect of lockdown on
imposed quarantine to contain viral • Red Zones/Hotspot district: Before ophthalmic practice in India, Nair and
transmission1. As indicated by its April 15, the ministry classified a colleagues highlighted a near-total
name, the virus primarily affects the district as red zone if it reported cessation (72.5%) of elective surgeries,
respiratory system leading to severe more than 15 cases. However, an overwhelming deterioration
respiratory distress. The disease is presently districts with several in clinical practice and rise of
inevitably fatal in the elderly and active cases and high doubling rates future unpredictability in majority
systemically comorbid individuals are labelled Red Zone. ophthalmologists of the country4.
and has slayed more than 600,000 All these parameters have significantly
individuals worldwide with the • Orange Zones: Those zones which affected the epidemiology of cases
numbers still rising. When it comes to are neither red nor green are called experienced by the ophthalmologists.
India, the virus has infected more than Orange Zones. For instance, according to a recent study
two Million population to which nearly by Das and Narayan, the maximum
30,000 have succumbed. The health- • Red Zones Containment areas: number of patients presenting to
care sector has been continuously These areas are demarcated within emergency department were adult
battling the virus with perseverance, red and orange zones by the district (77.85%) males (62.16%) with a
dedication and commitment, administration based on mapping median age of 35years.5 Around 65%
constantly guiding mankind through and geographical dispersion of of these were emergency related with
these tough times. However, the present cases and contacts. These zones microbial keratitis (23.74%), followed
pandemic has imposed multiple have more than 4 positive corona by corneal trauma (16.39%) as most
unprecedented challenges on existing cases. common presentations. Around
health-infrastructure and the field of 1/3rd of these patients underwent
ophthalmology is not immune to it. We Local human movement was guided by surgical intervention and the most
presently discuss the repercussions of this data and according to guidelines commonly performed procedures were
unexpected COVID-19 pandemic and from the Home Ministry, GOI, vitreo‑retinal procedures (32.86%)
subsequent human interventions on outpatient departments, medical clinics followed by trauma related (31.43%)
the field of ophthalmology in India. etc., were permitted to operate in all surgeries.
zones except containment areas with
Epidemiological Impact social distancing norms. With passage Clinical Impact
The Government of India (GOI) of time, levied restrictions were slowly Out of fear of contracting and
mandated a nation-wide lockdown of all and systematically eased partially circulating infection credited to the
non-essential services beginning from and routine life-based activities were proximal nature of ocular examination,
25th March 2020 onwards2,3. Complying encompassed depending on incidence all clinicians have shifted to more
to this, the citizens witnessed a complete of new cases. non-contact procedures for routine
shutdown of services not pertaining to patient assessment. Rigid protection
emergency life-saving measures. Entire In the wake of these unparalleled and disinfection measures are being
country was divided into multiple measures, country-wide clinical followed regardless of patients’clinical
zones described below based on the practice went downhill for the first presentation to minimise chances of
prevalence of the disease; time in the history of ophthalmology. cross-infection.
Routine eye-care and elective surgeries
were suspended indefinitely by
various private practitioners and
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 11
Beyond Ophthalmology
Conjunctivitis has been described as retinopathy are expected to follow their institutes have also suffered adversely
a rare manifestation of SARS CoV- natural course to end-stage disorders from lockdown. Classroom lectures,
2 infection and both virus induced thereby worsening prognosis. In hands-on clinical demonstrations, and
and secondary superinfections have adults and children, digital revolution surgical training are crucial components
been implicated as the cause of and excessive use of electronic media of medical education. Restricted patient
COVID-19 related conjunctivitis. It due to mandatory indoor stay has inflow, postponement of elective
can start anytime between 7-14 days predisposed them to risk of acquiring surgeries and fear of contracting
of contracting the systemic infection dry eye disorders and myopia9-10. virus from academic gatherings has
and infrequently can also be a primary With rampant use of chloroquine or radically incapacitated standard
presentation of the disease6-8. The hydroxychloroquine as a prophylactic curriculum of residents and trainees
overall rate is reported to be 1.1%; measure for COVID-19 infection, in thereby sidelining their learning for an
ranging from 0.7% in non-severe the future, ophthalmologists could indefinite time. In a survey undertaken
disease to 3% in severe disease. Recent play a decisive role in the screening by Mishra et al, 80.7% ophthalmology
meta analysis showed that patients of maculopathies associated with trainees across the country perceived
with severe systemic infection have an these agents. Another potential ocular that the COVID-19 related-lockdown
increased chance of manifesting with problem is sanitiser associated ocular has negatively impacted their learning,
conjunctivitis and may, therefore, need surface disease. Alcohol, a dehydrating especially surgical, due to disruption
more frequent systemic monitoring. agent, due to its desiccating and of training program schedules11. The
Various differential diagnoses such as irritant action on corneal epithelial impact of these deficient experiences
other viral and bacterial conjunctivitis, cells can lead to their inflammation correlated inversely with the length
allergic conjunctivitis, foreign body in by perpetuating eye rubbing. With of the training program and was
lids, dry eye syndrome and exposure cumulative short exposures of high greatest on the residents/fellows in
keratopathy in an intubated patient concentrations of alcohol associated their ultimate or penultimate years of
must be ruled out before ascribing any with recurrent sanitizer usage, harm training.
conjunctivitis to SARS CoV-2 virus. on ocular surface is highly anticipatory.
Conjunctival and tear secretions have It is therefore imperative to educate Various mitigation measures have been
a low positivity rate (16.7%) compared the general public about safe and adopted by teaching hospitals to ensure
to naso-pharyngeal (91.7%) specimens responsible use of hand sanitizers. smooth delivery of medical teaching to
and are therefore not recommended students without compromising their
routinely. However, sometimes when Financial Impact safety. Rotational clinical postings
needed, the swabs are obtained by Beyond the devastating humanitarian for acquiring skills pertaining to non-
placing sterile synthetic fibre swabs impact inflicted by the COVID-19 operative patient care, regular online
into the lower fornix in unanaesthetised pandemic, its financial repercussion classes by means of virtual classrooms,
eye which are then inserted into a viral on healthcare economy is profound. digital replacement of conferences by
transport medium and sent for RT- Decreased health-expenditure from the webinars and simulation based surgical
PCR. Fortunately, it is a self-resolving part of the GOI due to national economic training arranged by numerous medical
condition and conjunctivitis-alone backlash has shattered government- fraternities have been perceived as
afflicted patients can be followed-up aided hospitals and declined monetary useful stop-gap arrangement by 75.7%
telephonically till its resolution to avoid collections from interrupted elective trainees for staying up-to-date with the
unnecessary viral transmission. While services has vandalized private health- current knowledge.
commonly suggested management sector. Added cost of safety equipment,
strategy includes a trial of frequent drug shortage due to fractured Psychosocial Impact
preservative-free lubricants and topical pharmaceutical supply chains and
antibiotics, few authors have also tried wage, labor and capital costs have COVID-19 pandemic and lockdown
topical Ribavirin and ganciclovir for its caused huge revenue losses to multiple have disbursed uncertainty, anxiety and
resumption. small-scale clinics and hospitals forcing stress levels among ophthalmologists
their temporary closure succeeded across India. A sense of insecurity
Besides affecting the demographics by an uncertain future. This negative prevails among all due to an anticipated
and epidemiology of emergency cases, impact on overall health-infrastructure diminution in footfalls for availing eye
COVID-19 pandemic has also affected can culminate to inflations in cost of care services. Consequent financial
the natural pathogenesis of routine health-care in the coming times. burden could be a major cause for
ocular disorders. Due to non-availability depression in this sub-group. According
of ophthalmic services particularly Educational Impact to Khanna et al, a strikingly high
to the elderly and visually disabled, Mirroring abrupt closure of schools and proportion of ophthalmologists were
diseases like cataract and diabetic colleges all-over the country, medical psychologically affected during this
situation with 32.6% having some
12 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Beyond Ophthalmology
degree of depression, which was mild, remained 12 to 25ug/m3 and 28 to 62ug/ a safe future. At the same time,
moderate and severe in 21.4%, 6.9%, m3 respectively, a substantial decrease caution is being maintained on
and 4.3% cases respectively12. The by 40-50% compared to previous medical and medicolegal limitations
levels of depression were significantly years. Subsequent improvement in of teleophthalmology in diagnosis
higher in younger ophthalmologists air quality has been directly linked to of ocular disorders in children,
and the odds lessened by 3% with provisional shutdown of coal-based underprivileged and patients with
every one-year increase in age. It was power production units and decreased posterior segment disorders.
also detected to be higher in non- electricity consumption level in
practicing ophthalmologists, who industrial states of India. Nevertheless, Conclusion
were considerably worried about the optimistic effect of this on human To conclude, health-care workers
their training or professional growth, ophthalmological health, although must achieve a stable professional and
and experienced hitches in meeting expected, remains yet undetermined. ethical balance between becoming
living expenses due to dearth of hotspots for viral transmission and
new employment opportunities. Administrative Impact providing ophthalmic care during this
According to Mishra et al, 54.8% of potentially long-winding pandemic.
ophthalmic trainees witnessed an All the afore-mentioned repercussions Tele‑Ophthalmology holds great
increase in stress levels during present have guided a dramatic administrative promise in providing care to patients
situation and 77.4% reported that their drive in nation-wide hospitals and remotely through the use of digital
family members had expressed an institutions, which have now adopting infrastructure. It has to be remembered
increased concern for their safety and to safer methods of patient care and that the current situation will take years
wellbeing. Frontline corona warriors student teaching14-24. Patients seeking to stabilize and till then, humanity must
are witnessing an overwhelming ophthalmic care are being screened for be prepared for the worst. Nevertheless,
workload, depletion of personal signs and symptoms of COVID-19 for with every human enlightening each
protection equipment, feared contagion timely help. The health-care workers other in darkness, the dawn is near.
acquirement and transmission, societal are now adapting to personal and
stigmatization, and loneliness all of public safety by wearing protective References
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14 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
What’s New
An Overview of Artificial
Intelligence in Ophthalmology
Ekta Singla, Parul Ichhpujani, Suresh Kumar
Deptt. of Ophthalmology, Government Medical College and Hospital, Chandigarh, India.
Abstract: Increase in the life expectancy has added to increased burden of providing adequate health facilities on the health
care professionals all over the world. To overcome this problem, Artificial intelligence(AI),a computer based program which
aims to stimulate human behavior offers promising solution. AI is a broad term encompassing multiple components of which
machine learning (ML) and deep learning (DL) are the two major components. At present,AI is being used in diagnosis of
diabetic retinopathy, age related macular degeneration and glaucoma. The other areas being explored includes retinopathy
of prematurity and cataract. This articles gives an overview of the artificial intelligence, its components, its applicability in
ophthalmology and its limitation.
With the advancement in technology, learning (ML) and deep learning (DL) for the next layer. The analysis from
human race has advanced as well are the two major components. The use each layer is transmitted to the network
leading to an increase in the life of AI in ophthalmology began with the until final outcome is produced. For
expectancy. This multiplying aging traditional ML technique also referred an image-based diagnosis, a CNN
population have added to the load of all to as the “supervised learning,” as algorithm teaches itself by analyzing
physicians including ophthalmologists described by Ruamviboonsuk et al.2 In pixel or voxel intensities in a labeled
across the world to provide adequate ML technique, the health care provider training set of expert-graded images
health services at the population level. labels the individual clinical features and then provides a diagnostic output
Limited availability of infrastructure and severity in the images to help AI at the top layer. This process is repeated
(eye clinics and hospitals) and eye develop an algorithm which can be several times for every image. Once the
care providers (Ophthalmologists and used to classify images based on those algorithm optimizes itself, it can work
optometrists) results in long waiting labeled clinical features. on unknown images. However, the
time to see an ophthalmologists. DL, also referred to as the “unsupervised convolutional operations are carried on
This results in irreversible visual learning” is the new AI technique within the network in CNN, whereas in
loss especially in resource deprived, which bypasses the need for labeling by MTANN they are outside the network3.
developing nations. The need of the the health care provider. DL works on
hour is to look for solutions to increase the principle of deep neural networks How can AI be Implemented for
the accessibility and availability of eye modeled after human brain which Ophthalmology Diagnosis and
care services. Artificial Intelligence (AI) are used to recognize a pattern. DL Screening?
offers a plausible solution. “self-learns” the pointing features to With increasing burden of the common
classify a disease or its severity by using ophthalmological diseases such as
What is Artifical Intelligence? the entire images with their clinical diabetic retinopathy (DR), age related
The phrase “Artificial intelligence” diagnosis. There are mainly two types macular degeneration (ARMD), etc., AI
was first coined by John McCarthy in of DL programs: convolutional neural based DL can be used for narrowing the
19551. The simplified meaning of AI is network (CNN) and massive-training gap between health care system and
to accomplish tasks using advanced artificial neural network (MTANN). the clinical demand. For the real-world
computers with minimal human CNN and MTANN, just like human implementation two models have
involvement. Its aims to simulate the brain, consist of multiple interconnected been suggested: “the fully-automated”
human behavior. layer of neurons designed to simulate and the “semi-automated”. The fully
“thinking”. An input is fed into first automated model would itself triage
AI is a broad term encompassing layer whose output serves as an input the patient whether they need to
multiple components of which machine see an ophthalmologist or continue
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 15
What’s New
to major ophthalmic diseases such as
DR, ARMD, glaucoma and ROP. Role of
AI for various ophthalmic disorders is
being addressed underneath:
a) AI in DR
By the year 2040, nearly 600 million
people will have diabetes mellitus
with about one-third of them having
DR7 screening. Screening for DR with
timely referral and management is the
key to prevent blindness. DR screening
across the world is done by many
health care professionals including
ophthalmologists, optometrists,
general physicians using direct as well
as indirect ophthalmoscopy, slit lamp
examination, fundus cameras and OCT.
AI has revolutionized the technique of
DR thus aiding in increased accessibility
and availability of care for the patients.
Figure 1: IDxDR Machine. Table 1 shows the specificity and
sensitivity of various DL algorithms
community-based monitoring without used worldwide for diagnosing
any human involvement. On the other referable DR (> moderate NPDR or
hand,the semi-automated model would worse including diabetic macular
require the help of a human assessor to edema) based on fundus photographs.
augment DL to triage the patients4.
This semi-automated model of DL has b) AI in ARMD
successfully been used by Xie et al.5 in
Singapore for screening DR whereby fundus photographs (CFP) and optical By the year 2040, the world is expected
the AI triaged the patients using colored coherence tomography (OCT) images in to have 288 million patients of ARMD
fundus photographs and those flagged ophthalmology.2 DL have been applied of which 100% may have intermediate
as abnormal were then graded by expert
ophthalmologists. Table 1: Specificity and sensitivity of various DL algorithms for Diabetic
Applications of AI in Ophthalmology Retinopathy
Advanced ophthalmology practice
involves use of multimodal imaging DL System Authored by Senstivity (%) Specificity (%)
devices and hence is the perfect platform
to implement AI for improving the Abràmoff et al8 (2016) 96.80 87
patient care. This is reflected by the fact
that in 2018, FDA approved the first Gulshan et al9 (2016) 87 98.5
ophthalmic AI machine, IDx-DR, for the
screening of DR (Figure 1)6. Gargeya and Leng10 (2017) 96.3 92.1
AI mainly works by utilizing colored
Ting et al11 (2017) 100 81.3
Abràmoff et al6 2018(IDx-DR) 87.2 90.7
Natarajan et al12 (2019) 100 84
Varadarajan13 (2020) 85 80
Table 2: Specificity and sensitivity of various DL algorithms for ARMD
DL System Authored By Senstivity (%) Specificity (%)
Ting et al11 (2017) 93.20 88.70
Burlina et al.15 (2017) 90.3 89.2
Grassmen et al.16 (2018) 84.20 94.30
16 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
What’s New
ARMD or worse14. Hence these patients predicting pre-plus or worse forms of grade cataract into four categories (non
need to be screened timely for adequate ROP24. It is expected that this system cataract, mild, moderate and severe
referral to tertiary care centres. Various could also develop a severity scoring cataract). The system achieved an AUC
DL algorithms based on fovea centered system to monitor the progression, (area under curve) of 97.2% for cataract
images are being used to screen regression and treatment protocols. detection, and 87.7% for severity
ARMD with acceptable sensitivity and e) AI in Cataract grading.
specificity (Table 2). AI in the field of cataract has been
c) AI in Glaucoma relatively much less explored compared Likewise, for IOL power calculation
Glaucoma is expected to affect 112 to other counterparts. Till date cataract AI based formula “The Ladas Super”
million people globally by the year is diagnosed by the ophthalmologists Formula was derived by extracting
202017. Timely diagnosis, monitoring using slit lamp and graded according features from respective “ideal
structural as well as functional to classifications such as the Lens portions” of existing formulas (namely,
progression and titration of the opacity classification system (LOCS Hoffer Q, Holladay-1, Holladay-1
treatment at various time points III)25. Firstly, this process requires with Koch adjustment, Haigis, and
can help prevent permanent visual clinical expertise and secondly, it is an SRK/T formulas) and plotted into a
disability. subjective assessment. 3-D surface30. Apart from optimizing
Using DL algorithm, optic nerve head Similarly, intraocular lens (IOL) formula selection process, AI has also
cupping has been estimated by Li et al18 power calculation for cataract surgery been used to develop algorithms for IOL
and Ting et al.11 with sensitivity and is mainly based on axial length and power calculation. One such method
specificity ranging from 92-96%. ML keratometry catering to the eyes with is the Hill-Radial Basis Function (RBF)
based algorithms have been applied normal values, without considering method which has been obtained on
successfully to predict structural and the abnormal profiles (post-LASIK, algorithm based on measurements
functional damage in glaucoma19. keratoconus, etc.). To overcome these obtained from the Haag-Streit
Yousefi et al. have developed a ML short comings, AI algorithms using LENSTAR optical biometer31. Similarly
based algorithm which predicts visual both ML and DL have been developed26. another formula is Kane formula. Both
field progression earlier than the Cataract grading using AI algorithms these formulas have shown promising
conventional strategies. takes into consideration either slit lamp- results32.
Intraocular pressure (IOP) lowering based photographs or colored fundus
is considered to the only effective photographs. Wu et al.27 in a large-scale f) Miscellaneous
treatment in slowing down the study in China have recently developed
disease progression20. Kazieman et al a 3-step sequential algorithm based on Other major areas where applicability
have developed an algorithm based DL for diagnosis and referral of cataract of AI is being researched includes
on tonometry and visual field data using slit lampbased photographs. amblyopia, retinal vascular occlusions,
to predict the rate of progression of First step involves recognition of retinal detachment and corneal ectasias
glaucoma at different IOP values21. mydriatic versus non-mydriatric and such as keratoconus.
d) AI in Retinopathy of Prematurity optical section versus diffuse slit lamp
(ROP) based photographs. The second step Challenges with AI
ROP is the leading cause of childhood comprises categorizing as normal (no Despite the high level of accuracy of
blindness in the world. Early screening cataract), cataractous or postoperative AI based algorithms, there are certain
can help prevent blindness from IOL. And finally, if cataract is detected, challenges which needs to be addressed.
the disease22. The main barriers in the type and severity of cataract using Foremost the data used is not from a
screening is its subjective nature and LOCS II is done28. Accordingly, the heterogeneous group of population.
secondly the limited availability of ROP patient is referred to the tertiary eye care Applying the same set of images to
trained professionals. DL together with centre. The sensitivity and specificity the population can result in erroneous
tele-ophthalmology can offer novel of this algorithm for cataract detection results. Hence, diversifying the data
solutions. Brown et al23 developed an has been reported to be 92% and 83%, set in terms of ethnicities may help in
automated DL system to diagnose PLUS respectively27. resolving the issue11. Secondly, disorders
disease of ROP with promising results. Li et al29 developed a DL based like ROP and Glaucoma have large
Similarly, the iROP DL algorithm algorithm for diagnosing the cataract inter-observer variations. Too little data
reported a sensitivity and specificity using colored fundus photographs. The is available for certain common diseases
of 100% and 94%, respectively, in visibility of fundus images was used to (cataract) and certain uncommon
diseases (ocular tumors). The AI tool
learns from what is presented to it.
Hence if the dataset is small or not
representative of real time population
it is likely to produce inaccurate results.
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 17
What’s New
Figure 2: Black Box. on the black box nature of AI, its al. Improved automated detection of
transparency, liability, issues related to diabetic retinopathy on a publicly
The complex CNN which makes AI a cyber security, clinical deployment and available dataset through integration of
powerful tool fails to explain how the the cost effectiveness may help in better deep learning.Invest Ophthalmol Vis Sci
system reaches the conclusions it does- understanding and implementation. AI 2016;57:5200–6.
something referred to as “black box” is on the brink of bringing paradigm
phenomenon. Black-box is basically an shift in the ongoing clinical practice 9. Gulshan V, Peng L, Coram M, et al.
opaque object which converts input and services in the upcoming future. Development and validation of a deep
into output without being aware of Last but not the least, remember that AI learning algorithm for detection of
the underlying processing mechanism does not intend on replacing the human diabetic retinopathy in retinal fundus
(Figure 2). Researches are discovering race. photographs. JAMA 2016;316:2402–10.
how to peer into this black box. To
untangle the mystery, researchers have References 10. Gargeya R, Leng T. Automated
used the black masks to shield parts of 1. McCarthy J, Minsky ML, Rochester N, identification of diabetic retinopathy
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to discover how such masking affected et al. A proposal for the Dartmouth 2017;124:962–9.
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help them in looking for areas in AI Intelligence, August 31, 1955. For: AI 11. Ting DSW, Cheung CY, Lim G, et al.
accounting for the process of decision Magazine2006;2712–14 Development and validation of a deep
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the physicians in decision making ophthalmology: Evolutions in Asia. Asia images from multiethnic populations
and forming opinions in the future. AI Pac J Ophthalmol (Phila) 2020; 9:78–84. with diabetes. JAMA 2017;318:2211–23.
doesn’t consider the psychological and 3. Kappor R, Walters SP, Al-Aswad
social aspects of human nature which LA, The Current State of Artificial 12. Natarajan S, Jain A, Krishnan R, et al.
a skilled physician would normally Intelligence in Ophthalmology, Survey Diagnostic accuracy of community-
account for. Moreover, most currently of Ophthalmology.2018;64(2): 233-40. based diabetic retinopathy screening
used AI machines are capable of 4. Gunasekeran, Dinesh Visva et al with an offline artificial intelligence
monitoring only a single disease at a Artificial Intelligence in Ophthalmology system on a smartphone. JAMA
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Lastly, the lack of infrastructure 5. Xie Y, Nguyen Q, Bellemo V, et al. Cost- optical coherence tomography-derived
for implementing the AI system is effectiveness analysis of an artificial diabetic macular edema grades from
major concerning issue especially in intelligence-assisted deep learning fundus photographs using deep
developing countries where internet system implemented in the national tele- learning. accepted for publication in
supply is limited. medicine diabetic retinopathy screening Nature Communications. Nat Commun
Conclusion in Singapore. Invest Ophthalmol Vis Sci 2020; 11:130.
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avoidable blindness. Future research 7. Ting DSW, Pasquale LR, Peng L, et al. Br J Lancet Glob Health 2014;2:e106–16.
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8. Abràmoff MD, Lou Y, Erginay A, et 15. Burlina PM, Joshi N, Pekala M, et al.
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16. Grassmann F, Mengelkamp J, Brandl
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2018;125:1410–20.
17. Tham YC, Li X, Wong TY, et al. Global
prevalence of glaucoma and projections
of glaucoma burden through 2040: a
systematic review and meta-analysis.
18 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
What’s New
Ophthalmology 2014;121:2081–90. 24. Brown JM, Campbell JP, Beers A. intraocular lens formulas to generate a
Fully automated disease severity “super formula” and maximize accuracy.
18. Li Z, He Y, Keel S, et al. Efficacy of a assessment and treatment monitoring JAMA Ophthalmol 2015; 133:1431–
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the Collaborative Initial Glaucoma Government Medical College and Hospital,
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Ophthalmol 2018;136:803–10. A 3-D “super surface” combining modern
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 19
Subspeciality-Cornea
Congenital Fibrosis of Extra-ocular
Muscles
Gunjan Saluja, Asha Samdani
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India.
Congenital fibrosis of extraocular fixed in a down and out position mutation in the PHOX2A gene which is
muscles (CFEOM) historically was approximately 200 to 300 below the located on Chromosome 11q137,8.
believed to be an anomaly primarily of horizontal midline with marked
the extraocular muscles resulting due to restriction of ocular motility4. Ptosis
their fibrosis. However, with the advent Patients usually have ptosis of varying
of better imaging studies, CFEOM Head Posture severity with a large angle exotropia,
was further re-classified as congenital The patients typically assume a with a chin up head position.
cranial dysinnervation syndrome compensatory “chin up” head posture
(CCDS)1. The classification of CCDS to fixate on objects. Extra-ocular movements
has been summarized in Table1, and as CFEOM 2 also has severe restriction
the name suggests it results from the Extra-ocular movements of extraocular movements. There can
abnormal development of innervations Horizontal movements are severely be varied presentations of vertical
to the extra-ocular muscles with fibrosis restricted and horizontal strabismus strabismus, which includes either
occurring as a secondary process. may be present with exotropia. Presence absence of vertical misalignment,
CFEOM has been further classified into of severe ptosis with limitation of bilateral supraducted or infraducted
3 types, (CFEOM-1,2 and 3) depending movements of extra-ocular muscles eyes9.
on the gene involved. Each of the sub- can lead to strabismic and stimulus
category of CFEOM has been discussed deprivation amblyopia. Pupil
in detail below. Pupillary abnormalities in CFEOM
Aberrant regeneration appear to have structural atrophy of the
CFEOM-1 iris.
CFEOM-1 is the most common type Abnormal “jerky convergent
and has an estimated prevalence of movements” in attempted upgaze Imaging
1/2,30,000 population2. can be observed. There can also be High resolution magnetic resonance
CFEOM1 has an autosomal dominant simultaneous abduction on attempted imaging of brain has demonstrated
inheritance and results from the mis horizontal gaze known as synergistic anatomically absent third and fourth
sense mutations in the KIF21A gene divergence. Marcus Gunn jaw-winking cranial nerves. Extra-ocular muscles
located on Chromosome 12. KIF21A have been observed in CFEOM1 innervated by both cranial nerve III (i.e.
codes for the kinesin microtubule patients. The synkinetic movements levator palpebrae superioris, superior
associated motor protein which are believed to be originating from rectus, inferior rectus, medial rectus,
is responsible for the anterograde aberrant axonal routing. Thus, inferior oblique) and cranial nerve IV
organelle transport of neuronal cells3. supporting the neuropathic etiology (superior oblique) were found to be
for the pathogenesis of CFEOM15. small or absent.
Clinical features Neuro-radiological evaluation further
Ptosis supports the neurogenic hypothesis as CFEOM-3
The ptosis is classically bilateral and hypoplasia of the superior rectus and CFEOM-3 is rare with autosomal
severe. levator palpebrae superioris, hypoplasia dominant inheritance, incomplete
of third cranial nerve superior is penetrance, variable expressivity.
Total ophthalmoplegia commonly associated with CFEOM6. CFEOM-3 can have a variable
Ophthalmoplegia in CFEOM is phenotypic presentation, with
characterized with the eyes being CFEOM2 unilateral or bilateral disorder. Ptosis
CFEOM2 is an autosomal recessively can be of variable degrees, with mild to
inherited disorder, resulting from a
20 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Cornea
Figure 1: Image of a patient with classic CFEOM, note the position of eyes being fixed in with congenital fibrosis of extraocular
down and out position with severe ptosis. muscles, which has been hypothesised
to result due to the proximity of genes
moderate ophthalmoplegia. Synergistic CFEOM3 with oligosyndactyly. causing CFEOM and Prader Willi
and synkinetic eye movements are syndrome12.
not always present and pupillary Systemic associations of CFEOM
abnormalities are not always present10. Joubert’s syndrome Mental retardation,spina bifida,
CFEOM-3 can be further sub-classified Joubert’s syndrome is a rare disorder syringomyelia and craniofacial
into CFEOM-3A, CFEOM-3B and characterised by panting utachypnea, dimorphism are some of the other
CFEOM-3C. CFEOM-3A results from jerky eye movements, ataxia and systemic associations of CFEOM.
mutations in TUBB3 gene, which can be a genesis of vermis of cerebellum.
associated with systemic features like Joubert’s syndrome has also been Differential Diagnosis
intellectual disability, facial weakness described to be associated with The main differential diagnosis of
and progressive atonal polyneuropathy. CFEOM, which can be explained by the CFEOM include
Dysplasia of corpus callosum, basal malformed brain stem which harbours
ganglia,cortico spinal tracts are some the nuclei of 3rd,4th and 6th nerve11. Double elevator palsy
of the abnormalities of central nervous The double elevator palsy can result
system anomalies associated with Prader Willi syndrome from inferior rectus restriction, superior
CFEOM3A. Prader-Willi syndrome results from rectus palsy or due to supra nuclear
CFEOM3B results from mutations in interstitial deletions of the long arm of causes and usually is associated with
KIF21A gene with variable phenotypic chromosome 15 and has typical features pseudoptosis. Forced duction test will
presentation. like mental retardation, hypotonia, be tight specifically for inferior rectus.
Tukel syndrome is an autosomal short stature, hypogonadism, and
recessive disorder associated with obesity. Parder Willi syndrome also Brown’s syndrome
has been reported to be associated Brown’s syndrome is characterized by
limitation of elevation in adduction
with divergence is upgaze and normal
depression in adduction, differentiating
it from CFEOM.
Ocular myasthenia gravis
Ocular myasthenia results from
formation of auto-antibodies against
Ach-R and is associated with typical
diurnal variation and the progressive
weakness of extraocular.
Partial or complete third nerve
palsy
In a complete third nerve palsy the eye
is fixed in a down and out position,
however forced duction test, history
of birth trauma and negative family
history helps to differentiate.
Management
Non surgical management
Non-surgical management involves
optical correction of refractive errors
and management of anisometropic
amblyopia
Surgical management
Strabismus surgery
The goal of strabismus surgery in
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 21
Subspeciality-Cornea
Table 1: Cassification of Congenital Cranial Dysinnervation Syndrome extraocular muscles. Ann Neurol 1997;
41:314–25.
Cranial nerve nucleus Syndrome Gene 5. Brodsky MC. Hereditary external
ophthalmoplegia, synergistic divergence,
3rd nerve CFEOM-1 KIF21A jaw winking, and oculocutaneous
hypopigmentation. Ophthalmology 1998;
CFEOM-3 TUBB3 105:717–25.
6. Demer JL, Clark RA, Engle EC. Magnetic
4th nerve Brown’s syndrome resonance imaging evidence for
widespread orbital dysinnervation in
3rd +4th nerve CFEOM-2 POHXA2 congenital fibrosis of extraocular muscles
due to mutations in KIF21A. Invest
6th nerve DRS CH1N1 Ophthalmol Vis Sci. 2005; 46:530–9.
7. Wang SM, Zwaan J, Mullaney PB, et al.
DRRS SALL4 Congenital fibrosis of the extraocular
muscles type 2, an inherited exotropic
HGPPS ROBO3 strabismus fixus, maps to distal 11q13. Am
J Hum Genet 1998; 63:517–25.
6th+7th nerve Mobius syndrome 8. Nakano M, Yamada K, Fain J, et al.
Homozygous mutations in ARIX(PHOX2A)
DRS:Duane’s retraction syndrome result in congenital fibrosis of the
DRRS:Duane radial ray syndrome extraocular muscles type 2. Nat Genet
HGPPS: Horizontal gaze palsy with progressive scoliosis 2001; 29:315–20.
9. Bosley TM, Oystreck DT, Robertson RL,
CFEOM is to Ptosis surgery et al. Neurological features of congenital
Poor Bell’s in patients with CFEOM is fibrosis of the extraocular muscles type 2
• Remove obstacles from the visual a contra-indication for ptosis surgery, with mutations in PHOX2A. Brain 2006;
axis and crutch glasses can be helpful. 129:2363–74.
Strabismus surgery ideally should 10. Gutowksi NJ, Bosley TM, Engle EC. 110th
• Eliminate the chin up head posture be performed first followed by ptosis ENMC International Workshop:The
• Align eyes in primary position surgery. Surgery can be avoided in congenital cranial dysinnervation
The choice of strabismus surgery patients with no significant head disorders (CCDDs) Naarden,The
depends upon the ocular alignment in posture and no occlusion of visual axis. Netherlands, 25-27 October, 2002.
primary position, ocular rotation and Sling surgery either with autologous Neuromuscul Disord 2003;13:573–8.
forced duction test results. fascia lata or silicon sling is the ptosis 11. Appleton RE, Chitayat D, Jan JE, Kennedy
surgery of choice in CFEOM patients R, Hall JG. Joubert’s syndrome associated
Individual consideration must be given and should be cautiously done to with congenital ocular fibrosis and
to the muscle to be operated. Due to prevent the dreadful complication of histidinemia. Archives of Neurology.
involvement of orbital and conjunctival exposure keratopathy. 1989;46:579-582.
tissue involvement conjunctival 12. Kalpakian B, Bateman JB, Sparkes RS,
recession can be helpful. Forced duction References Wood GK. Congenital ocular fibrosis
test is usually positive for inferor syndrome associated with the Prader-
rectus and lateral rectus, and require 1. Hanisch F, Bau V, Zierz S. Die Willi syndrome. J Pediatr Ophthalmol
large recessions or hang back suture kongenitale Fibrose der äusseren Strabismus. 1986;23:170-173.
technique. Placing two muscle hooks 13. Velez FG, Thacker N, Britt MT, et al. Rectus
to pre-place the absorbable sutures is Augenmuskeln (CFEOM) und andere muscle orbital wall fixation: a reversible
helpful in patients with tight muscles13. Phänotypen der kongenitalen kranialen profound weakening procedure. J AAPOS
2004; 8:473–80.
Resection Dysinnervationssyndrome (CCDD)
Resection can be done for superior [Congenital fibrosis of extraocular Corresponding Author:
rectus and medial rectus, but should be
carefully done to avoid the worsening muscles (CFEOM) and other phenotypes Dr. Gunjan Saluja
of enophthalmos. Dr. Rajendra Prasad Centre for Ophthalmic
of congenital cranial dysinnervation Sciences, AIIMS, New Delhi, India.
Surgery on more than 3 muscles should syndromes (CCDD)]. Nervenarzt.
be avoided to lower the risk of anterior
segment ischemia. Two or more sittings 2005;76:395-402.
are required in most of the cases. Patients
must be explained about the prognosis 2. Reck AC, Manners R, Hatchwell E.
to avoid unrealistic expectations. Phenotypic heterogeneity may occur
in congenital fibrosis of the extraocular
muscles. Br J Ophthalmol.1998; 82:676–9.
3. Yamada K, Andrews C, Chan WM, et al.
Heterozygous mutations of the kinesin
KIF21A in congenital fibrosis of the
extraocular muscles type1 (CFEOM1). Nat
Genet 2003; 35:318–21.
4. Engle EC, Goumnerov BC, McKeown
CA, et al. Oculomotor nerve and muscle
abnormalities in congenital fibrosis of the
22 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Pediatric Ophthalmology
Paediatric Ocular Biometry and
IOL Power Calculation
Mousumi Banerjee, Sudarshan Khokhar
Dr. Rajendra Prasad Centre for Ophthalmic Sciences. AIIMS, New Delhi, India.
As the axial length of the eye grows in proportion with the lens and cornea, the refraction of a normal infant’s eye stays close to emmetropia.
Gordon and Donzis’s cross sectional biometric study of 148 normal eyes found on average that the axial length increased from 16.8 to 23.6
mm from birth to adulthood, whereas the refraction changed minimally, from +0.4 to – 0.5 D. This is primarily because the power of the
crystalline lens declined from +34.4 to +18.8 D. In contrast, aphakic eyes have a decline in hyperopia of 10D over the same period. As there
is no proportionally changing lens to compensate for the ocular growth; aphakic eyes have a large myopic shift.
Childhood blindness is a public health • Keratometry steeply reduces in without the speculum as there may be
concern across the world due to its deep the first 6 months (-0.40 D/month), erroneous readings due to deforming
impact on psychological, emotional, -0.14 D/month in the next 6 months, effect of the speculum on the globe.
and socioeconomic growth of the and -0.08 D/month in the second
family. Paediatric cataract is a treatable year, reaching the adult range at Axial length can be measured with both
leading cause of childhood blindness. It about 3 years of age6. Thus , mean immersion and applanation A scan,
accounts for 7.4%–15.3% of paediatric corneal power at birth is 51.2D however , immersion method is more
blindness and a significant avertable which reduces to a mean of 43.5D accurate as it avoids compression of the
disability-adjusted life years1,2. by around 3 years of age7. anterior corneal surface. Applanation
An accurate refractive outcome after method gives average 0.27 mm (0.24mm
primary IOL implantation is crucial to • Lenticular power decreases from -0.32 mm) lesser AL measurements
avoid a large myopic shift in later life. a mean of 34 D at birth to 28 D by than immersion technique. Hence,
Numerous studies have found that 6 months, gradually reaching the if immersion scan is not possible, an
IOL formulas for adult cataract surgery adult value of 20 D8. average of ten readings with maximum
are less accurate in children. In adults, anterior chamber depth should be taken
modern theoretical formulas are Biometry into account.
accurate within approximately 0.5 1. Conventional ultrasound biometry
diopters (D); in children various studies Keratometry should be performed
have found mean absolute errors of • Applanation A scan before applanation A scan since
between 1.08 and 1.4 D3,4,5. • Immersion A scan vice versa can lead to erroneous
2. Optical Biometry keratometry measurements.
Concept of emmetropization
Emmetropization involves interplay of • Iol Master 500 (Based on partial IOL formula
3 major variables (axial length , corneal coherence interferometry). Theoretical formulas (SRK-T, Holladay
and lenticular power). 1&2, Hoffer Q and Haigis) preferred
• Mean AL at birth is 16.6 –17.0 mm • Lenstar (based on optical low over regression formula like SRK.
coherence reflectometry). [10] Vasavada et al11 compared the
AL increases rapidly in the first 6 accuracy of modern intraocular
months (0.62 mm/month), then • Swept source IOL master 700. lens power calculation formulae in
has a relatively slower (infantile Paediatric patients are usually not paediatric population and concluded
phase) growth (0.19 mm/month) cooperative for optical biometry, and that in pediatric eyes, SRK/T and the
till 18 months, followed by a slow examination under anesthesia (EUA) Holladay 2 formulae had the least
(juvenile phase) growth (0.10 mm/ is required for calculation of axial predictive error (PE). Personalizing the
month)6. length and keratometry. Keratometry lens formula constant did reduce the
using handheld keratometer (Nidek, PE significantly for all formulae except
Alcon autokeratometer) is performed Hoffer Q. In extremely short eyes (AL
under EUA in paediatric age group.
Keratometry readings should be taken
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 23
Subspeciality-Pediatric Ophthalmology
<20 mm),SRK/T and Holladay 2 gave management of paediatric cataract over 9. McClatchey SK, Parks MM. Myopic shift
the best PE. the years. A comprehensive eye care after cataract removal in childhood. J
approach involving early detection, Pediatr Ophthalmol Strabismus. 1997;
IOL power calculation management and visual rehabilitation 34: 88-95.
An accurate refractive outcome after can help to tackle childhood blindness
primary IOL implantation is crucial to due to cataract with favourable 10. Eibschitz- Tsimhoni, et al. Discrepancies
avoid a large myopic shift in later life. outcomes. between Intraocular Lens Implant
Thus, initial desired refractive outcome Power Prediction Formulas in Pediatric
is therefore hypermetropia. To conclude, words of Ellis17 are worth Patients Ophthalmology,2007;114:383-6.
remembering, “Young children with
• Dahan and Drusedau suggested an IOL in place are a unique clinical 11. Vasavada V, Shah SK, Vasavada VA, et
undercorrection of -20% in <2 years responsibility of an ophthalmologist. al. Comparison of IOL power calculation
and 10% in children between 2 and Long-term follow-up is especially formulae for pediatric eyes. Eye (Lond).
8 years12. important.” 2016;30(9):1242-50.
• Prost suggested undercorrection of References 12. Dahan E, Drusedau MU. Choice of
-20% between 1 and 2 years of age, 1. Gilbert C, Foster A. Childhood blindness lens and dioptric power in pediatric
15% undercorrection between 2 pseudophakia. J Cataract Refract Surg
and 4 years, and 10% between 4 and in the context of VISION 2020 – The 1997;23 Suppl 1:618-23.
8 years of age13. right to sight. Bull World Health Organ
2001;79:227-32. 13. Prost ME. IOL calculations in cataract
• Enyedi suggested postoperative 2. Rahi JS, Sripathi S, Gilbert CE, Foster A. operations in children. Klin Oczna
target refraction to be used for IOL Childhood blindness in India: Causes 2004;106:691-4.
power calculation according to age in 1318 blind school students in nine
(age + postoperative refraction = states. Eye (Lond) 1995;9 (Pt 5):545-50. 14. Enyedi LB, Peterseim MW, Freedman
7)14. 3. Hoffer KJ. The Hoffer Q formula: a SF, Buckley EG. Refractive changes after
comparison of theoretic and regression pediatric intraocular lens implantation.
However, Khokhar et al study on formulas. J Cataract Refract Surg. 1993; Am J Ophthalmol 1998;126:772-81.
biometric changes in Indian paediatric 19: 700-12.
cataract and post operative refractive 4. Andreo LK, Wilson ME, Saunders RA. 15. Khokhar SK, Tomar A, Pillay G,
outcomes noted a lower rate of axial Predictive value of regression and Agarwal E. Biometric changes in Indian
length growth and keratometry theoretical IOL formulas in pediatric pediatric cataract and postoperative
changes in Indian eyes as compared to intraocular lens implantation. J Pediatr refractive status. Indian J Ophthalmol.
western eyes implying the need for less Ophthalmol Strabismus. 1997; 34: 240-3. 2019;67(7):1068-72.
undercorrection in emmetropic IOL 5. Moore DB, Ben Zion I, Neely DE, Plager
power for Indian eyes15. DA, Ofner S, Sprunger DT, et al. Accuracy 16. Ness PJ, Werner L, Maddula S, Davis D,
of biometry in pediatric cataract Zaugg B, Stringham J, et al. Pathology of
Hence, we prefer an undercorrection of extraction with primary intraocular lens 219 human cadaver eyes with 1-piece or
– 20 % for children <3months, 10 % at implantation. J Cataract Refract Surg. 3-piece hydrophobic acrylic intraocular
1 year, 5 % at 2 years and 2 % at 5 years. 2008; 34: 1940-7 lenses: Capsular bag opacification and
6. Capozzi P, Morini C, Piga S, Cuttini sites of square-edged barrier breach. J
Criteria for IOL implantation M, Vadalà P. Corneal curvature and Cataract Refract Surg 2011;37:923-30.
1. AL >17 mm axial length values in children with
2. Corneal diameter >10mm congenital/infantile cataract in the first 17. Bradford GM, Keech RV, Scott WE:
Choice of IOL 42 months of life. Invest Ophthalmol Vis Factors affecting visual outcome after
Hydrophobic acrylic lenses with square Sci 2008;49:4774-8. surgery for bilateral congenital cataracts.
edges inhibits lens epithelial cell (LEC) 7. Gordon RA, Donzis PB Refractive Am J Ophthalmol 1994; 117: 58-64.
migration and posterior capsular development of the human eye. Arch
opacification formation and are hence Opthalmol1985 Jun;103(6):785-9. Corresponding Author:
preferred in pediatric age group16. 8. Larsen JS. The sagittal growth of the eye.
IV. Ultrasonic measurement of the axial Dr. Mousumi Banerjee
Conclusions length of the eye from birth to puberty. Dr Rajendra Prasad Centre for Ophthalmic
A paradigm shift has been noted in the Acta Ophthalmol. 1971;49(6):873– 86. Sciences. AIIMS, New Delhi, India.
24 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Pediatric Ophthalmology
“When all else fails” - Secondary
Episcleral Plaque Brachytherapy in
Retinoblastoma
Harika Regani, Santosh Honavar
Centre for Sight, Banjara Hills, Hyderabad, India
Retinoblastoma is the most common thickness, <16mm base. it with Co60. Subsequently, various
primary malignant intraocular tumor 3. Residual/recurrent irrespective of radioisotopes were used in the
in children. Trends in the management
of retinoblastoma currently focus location, >3mm thickness which treatment of retinoblastoma, and the
on eye and vision salvage. Mortality are not amenable to other focal advantages and the disadvantages of
rate is reduced to 2-5% with earlier treatments. which are discussed in Table 15. Modern
detection and improved treatment. 4. Vitreous seeding confined to <2mm plaques currently include assemblies
The management is individualized, of tumor surface. of gold shells with low energy photon
considering the age, laterality, number, s(Reeud1s06(Io12r5, SPr9d010)3,plCasq1u31e)s.oCr osmolpidarabtievtae
size, location and associated vitreous Contraindications dosimetry revealed that the lower
seeds. Intraarterial or intravenous 1. Anterior segment involvement. weneerregmy oprheortaopnisdlfyroambsoPrdb1e0d3 irradiation
chemotherapy along with appropriate 2. Diffuse vitreous seeds. within the
focal therapy is the current standard target volume with less irradiation to
of care. Although retinoblastoma is Methods of tumor localization normal ocular structures.
highly radiosensitive, external beam 1. Direct view using an indirect
radiotherapy is not currently used for The radiation dose to the tumor apex
primary or secondary management of ophthalmoscope. is important in terms of regression
intraocular retinoblastoma because of 2. Posterior point source illumination and has been evolving over the years
several complications associated with it, after noticing poor response and
including second malignant neoplasm1. - Fiber optic or He-Ne light sources recurrences due to suboptimal doses
In patients with tumors refractory to or scleral depression combined to the tumor apex6. Currently, a dose of
chemotherapy, residual tumors and with indirect ophthalmoscopy. approximately 4500-5000 cGy is usually
recurrences, plaque brachytherapy has 3. Intraoperative ultrasonography. followed.
emerged as a reasonable management 4. Morphometric (Ultrasonography
option. combined with fundus photo). Plaque design
5. Plaque simulator software-guided. The radioisotopes are loaded on an
Episcleral brachytherapy involves applicator (gold or silver). Ru106 is
placement of the radiation source in the Types of plaques commonly used by the ophthalmic
form of a plaque adjacent to the tumor, The various plaques used in the surgeons in India due to its ergonomic
and the radiation is delivered through treatment of retinoblastoma can be design. It is extremely easy to handle
the sclera, thus reducing the damage to broadly classified as: with a thickness of only 1mm. The
the surrounding ocular structures2. It radioactive core of the Ru106 eye
has been used both as a primary and a 1. Photon emitters: I125, Pd103, Cs131 applicator consists of a foil coated
secondary treatment modality. 2. Beta emitters: Ru106, Sr90 with Ru106 (0.2mm). This core is safely
HB Stallard pioneered in the first encapsulated within pure silver sheets
Indications3,4 with <8mm interstitial procedure for treatment (A silver window of 0.1mm and a silver
1. Unilateral cases. of retinoblastoma in 1929, and later backing of 0.7mm). The silver backing
2. Unifocal tumors developed the first radioactive plaque absorbs almost 95% of the beta radiation
applicator. He used Radium in his and acts as an effective radiation shield7.
first ten patients but later replaced
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 25
Subspeciality-Pediatric Ophthalmology
Isotope Half life Radiation Energy Advantages Disadvantages
Cobalt 60 5.26 years type 1. Long half life
Gamma 1.17,1.33 Mev 2. Good tissue penetration 1. Difficult shielding
2. Less focused target
Iridium 192 74.2 days Gamma 0.38 Mev (avg) 1. Good tissue penetration dose
2. Easily customized 3. High energy, increases
the exposure to
Iodine 125 59.4 days Gamma 27-35 Mev 1. Easy to shield personnel
2. Appropriate tissue penetration Personal exposure higher
Palladium 103 16.99 days Gamma 21 Kev 3. Easily customized than I 125, Pd 103, Ru
Ruthenium-106 371.8 days Beta 3.5 Mev (max) Similar to Iodine 125 106.
1. Easy for shielding Shorter half-life
2. Larger therapeutic window compared to Co 60
3. Steep dose depth gradient
4. Less complications Very short half life
Limited tissue
penetration
The round plaques were used for many should be taken to minimize any regression pattern should be closely
years. The Notch design is extremely delay between the evaluation and observed
helpful when the perimeter of the the assessment since the doses
tumor overlaps or lies adjacent to the might require a revision in case the Complications
optic disc where the plaque provides tumor dimensions change. The most common complication after
a more conformal dosimetry coverage 2. All surgeries are performed under the plaque brachytherapy is vitreous
of the tumor base and its surrounding general anesthesia hemorrhage secondary to necrotic
margin8. 3. After a localized peritomy and tumor. The hemorrhage usually resolves
separation of the tenon’s fascia, in about 90% cases and the unresolving
Manufacture the plaque is placed on the sclera cases might need enucleation.
Currently, the plaques are being by suturing with nonabsorbable
delivered by BEBIG from Germany. sutures through the plaque eyelets 1. Radiation keratopathy.
The Indian plaques are manufactured 4. The plaque is inserted and left 2. Radiation cataract (2.5% at 1 year to
by Bhabha Atomic Research Centre in position, keeping the patient
(BARC)9 and the modified designs are admitted under the standard plaque 54.3% at 10 years) .
under development. protocol adhering to the radiation 3. Radiation retinopathy or
safety guidelines of Atomic
Surgical Procedure for Plaque Energy Regulatory Board (AERB), papillopathy (15.4% at 1 year to
brachytherapy in Retinoblastoma: Government of India. 21.6% at 5 years).
5. The plaque removal is also done 4. Radiation optic neuropathy (8.6%
Pre-operative: under general anesthesia. at 1 year to 21.2% at 5 years).
Regression Patterns after Plaque 5. Radiation Vasculopathy.
1. Evaluation of the tumor by indirect Brachytherapy: 6. Scleral necrosis.
ophthalmoscopy with scleral
depression for the approximate Different regression patterns have Results
distance of the tumor from the been observed after the plaque Tumor control of 79% has been
optic disc and fovea. brachytherapy. reported in a 5 year follow up study.
Young patients without associated
2. Estimation of tumor base, height, 1. Flat scar seeding showed a good response.
width by ultrasonography. 2. Partial or complete fish flesh Tumor recurrence of 4.2% at 1 year
and 7.1% at 5 years have been reported
Surgery regression. Cases with such in a study. The clinical risk factors for
1. Reassessment of the tumor size tumor recurrence have been predicted
as the older patient age and subretinal
before starting the surgery. Care
26 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Pediatric Ophthalmology
Figure 1: A,C,E are the fundus images of recurrent Retinoblastoma patients. B,D,F are the retinoblastoma. Trans Ophthalmol Soc
fundus images showing excellent response to Plaque Brachytherapy with Ruthenium-106. UK 1962;82:473-535.
3. American Brachytherapy Society -
or vitreous seeds10. followed to monitor the complications Ophthalmic Oncology Task Force.
of radiotherapy and predicted to occur Electronic address: paulfinger@
Conclusion in patients with large tumors. eyecancer.com; ABS – OOTF Committee.
Secondary Plaque brachytherapy The American Brachytherapy Society
for Retinoblastoma has shown good References consensus guidelines for plaque
results and an eye salvage of about 1. Shields CL, Shields JA, Cater J, Othman I, brachytherapy of uveal melanoma
90%. However, an appropriate case and retinoblastoma. Brachytherapy.
selection, accurate tumor dimensions Singh AD, Micaily B. Plaque radiotherapy 2014;13(1):1-14.
and a dedicated team approach can for retinoblastoma: Long-term tumor 4. Raksha Rao, Santosh G Honavar Recent
greatly improve the chances of eye control and treatment complications Developments in Retinoblastoma. DJO
salvage especially in the refractory in 208 tumors. Ophthalmology. 2016;27:50-61.
cases and one-eyed patients [Figure 2001;108:2116–21. 5. Hernandez JC, Brady LW, Shields CL,
1]. These patients should be closely 2. Stallard HB. Doyne Memorial Lecture, Shields JA, DePotter P. Conservative
1962. The conservative treatment of treatment of retinoblastoma. The use of
plaque brachytherapy. Am J Clin Oncol.
1993;16(5):397-401.
6. Schueler AO, Flühs D, Anastassiou G, et
al. Beta-ray brachytherapy with 106Ru
plaques for retinoblastoma. Int J Radiat
Oncol Biol Phys. 2006;65(4):1212-1221.
7. h t t p s : / / w w w . b e b i g . c o m / f i l e a d m i n /
bebig_neu/user_uploads/Products/
Ophthalmic_Brachytherapy/
Ophthalmic_Brochure__Rev._06__
English.pdf
8. https://www.eyephysics.com/PS/PS5/
UserGuide/NotchedPlaques.html
9. Suganeswari G. Update on Intraocular
Oncology, Sci J Med & Vis Res Foun
2015;XXXIII: 88–92.
10. Shields CL, Honavar SG, Shields JA, et al.
Factors predictive of recurrence of retinal
tumors, vitreous seeds, and subretinal
seeds following chemoreduction for
retinoblastoma. Arch Ophthalmol
2002;120:460 – 4.
Corresponding Author:
Dr. Harika Regani
Centre for Sight, Banjara Hills, Hyderabad,
India
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 27
Subspeciality-Pediatric Ophthalmology
Congenital Corneal Opacities
Mohamed Ibrahime Asif, Rahul Kumar Bafna, Namrata Sharma
Dr. Rajendra Prasad Centre for Ophthalmic Sciences. AIIMS, New Delhi, India.
Congenital corneal opacities could be Classification Primary corneal disease
hereditary, developmental or infectious 1. STUMPED classification1 (From
causes. It can be unilateral/ bilateral; Waring GO, Rodrigues MM. Congenital i) Corneal dystrophies
isolated/associated with ocular or and neonatal corneal abnormalities)- • CHED
systemic abnormalities. Though rare, Table 1 • PPCD
diagnosing them is very important • CHSD
to predict the natural course of the S Sclerocornea • XECD
disorder, take appropriate medical and T Tears in Descemet membrane
surgical therapy as required considering ii) Corneal dermoid
the amblyopic age group, to provide (congenital glaucoma, birth iii) Peripheral sclerocornea
genetic counseling to the parents and to trauma) iv) CYP1B1 cytopathy
diagnose associated systemic disorders U Ulcers (viral keratitis, bacterial Secondary corneal disease
and treat them. keratitis)
M Metabolic (mucopolysaccharidoses, 1. Congenital
History and Examination mucolipidoses) • Kerato - irido - lenticular
A detailed history and examination P Posterior corneal defect (Peters dysgenesis
would be helpful in diagnosing the anomaly, Posterior keratoconus) • Irido-trabecular dysgenesis
condition. E Endothelial dystrophy (CHED, • Primary congenital glaucoma
Obstetric history- regarding any drug PPCD) • Intracorneal cyst
intake or any gestationally acquired D Dermoid
infections like rubella, varicella. History 2. New classification of neonatal corneal 2. Acquired
of any vaginal or cervical infections opacities (Nichal KK2) • Trauma
for the mother especially herpetic/
gonococcal infections. Family history Table 2: Differentiating features between birth trauma and congenital
of any ocular disorder in paternal/ glaucoma
maternal side/ any other previous
delivery. Birth trauma Congenital glaucoma
Examination could be performed at bed
side as slit lamp examination would not Tears in the DM are vertical/oblique- Tears in the DM are usually horizontal/
be possible in many cases. Handheld slit due to instrumental delivery (forceps concentric to limbus (Haab striae)
lamp/ torchlight could be used for bed delivery)
side examination. However, a complete
examination is accomplished under Corneal diameter is normal Corneal diameter could be enlarged
general anesthesia (EUA). During EUA, (buphthalmos)
ultrasonography (A scan and B scan)
and ultrasound biomicroscopy (UBM) Intraocular pressure normal Elevated IOP
should be performed. Corneal diameter,
intraocular pressure, gonioscopy Corneal edema appears in the Corneal edema appears few weeks after
(if possible) should be performed. immediate post-partum period birth
Corneal examination with fluorescein
staining and cobalt blue light should be Optic nerve head is unaffected Optic nerve head cupping could be
performed. present
Left eye more frequently affected due Either/ both eyes could be affected
to common head crowning position
Usually less incidence of photophobia Photophobia commonly present
28 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Pediatric Ophthalmology
Table 3: Differentiating features between various mucopolysaccharidosis
Hurler/ Gragoylism Maroteaux- Sheie Morquio Hunter
Lamy
Hurler/ Gragoylism Maroteaux-Lamy Sheie Morquio Hunter
Inheritance AR AR AR AR XLR
Deficiency Alpha-1-iduronidase Arylsulfatase B Alpha-1- Galactosamine-6- Iduronate sulphatase
iduronidase sulphate sulphatase/
beta galactosidase
Onset Within first few years Within first few From birth Usually after 10 Doesn’t present as
years but decrease years old corneal opacity
vision by 2nd
decade
Ocular Diffuse punctate stromal Punctate corneal Thickened or Corneal opacities Corneal opacity
opacities not involving opacities almost edematous seen in few. (10%) might occur later in
epithelium and always present cornea, life in mild form
endothelium. Significant at birth, narrow prominent in
corneal clouding angle glaucoma the periphery.
Associated
glaucoma
Systemic Mental retardation, large Mild facial Claw hand Dwarfism, joint Clinically appears
head with abnormal abnormalities, deformities, laxity, valvular like Hurler but
facies, dwarfism, joint skeletal changes valvular (aortic valve) heart deafness and cardiac
contractures, hepato- like kyphosis, (aortic valve) diseases defects are common
splenomegaly, deafness, genu valgum, heart diseases.
hirsutism, cardiac and protuberant They have
CNS defects sternum, normal
hydrocephalus intelligence,
height and life
expectancy
Table 4: Ocular features of various mucolipidosis is flat with keratometry usually less
than 43D. Ocular associations include
MLS-I These three types do not cause any severe corneal microcornea, microphthalmos,
MLS-II (I cell disease) diseases and do not usually require any ocular glaucoma, coloboma, aniridia,
treatment. Mild opacification seen in less than 20% in
type I, 40% in type II and almost all cases of type III blue sclera, ectopia lentis, cataract
and systemic associations include
MLS-III This type is always associated with significant cerebellar anomalies, Dandy-Walker
MLS-IV congenital corneal opacity. Episodic eye pain is seen cyst, cryptorchidism, ear deformities,
due to accumulation of abnormal material in the
epithelium leading to surface irregularities. Other polydactyly, osteogenesis imperfecta
ocular findings include retinal degeneration, optic and various other syndromes.
atrophy, cataract, attenuated vessels. They have
profound psychomotor retardation without skeletal/ Histologically, the corneal stroma
facial deformity. appears like stroma due to irregular
arrangement of thickened collagen
• Infection asymmetric scleralization of the fibers, decrease in the diameter of
• Metabolic peripheral or entire cornea which may collagen fibrils in the posterior stroma
occur alone or in association with other in contrast to the normal cornea and
Disorders ocular anomalies. Opacification is attenuated or absent DM.
1. Sclerocornea (cornea plana): It is smooth, white, vascular involving full
non-progressive, sporadic (can be thickness appearing similar to sclera UBM and ASOCT could be useful to
familial/ AD/ AR form- mutation in and it is prominent in the peripheral look for any associated structural
KERA gene), usually bilateral and cornea than the central. The cornea abnormalities, identification of pupil
and thereby facilitating surgical
planning. Surgery should be planned
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Subspeciality-Pediatric Ophthalmology
Table 5: Types of Peter anomaly Type 2 Peter treated with systemic acyclovir
Type 1 Peter Type 1+ lens abnormalities 30mg/kg/day for 10 days apart from
Corneal opacity along with (keratolenticular adherence, topical antivirals and cycloplegics.
iridocorneal adhesions (lens is usually cataractous lens) Topical should be given till re-
clear) epithelialization but no longer than
Predominantly unilateral involvement Predominantly bilateral with denser 14 days.
with less dense opacity opacity
Associated ocular anomalies- • Bacterial- rare to appear at birth.
microcornea, sclerocornea, infantile Associated ocular anomalies-
glaucoma, vitreoretinal abnormalities microphthalmia with PHPV, Neisseria gonorrhoeae can produce
rare microcornea, sclerocornea, cornea serious ocular and systemic
Systemic associations- rare plana, coloboma, aniridia, optic
atrophy complications. They present as
Good visual potential Systemic associations- poly/ severe unilateral conjunctivitis
syndactyly, Wilms tumor, craniofacial within few hours to 2-3 days
dysplasia, cardiac defects, CNS with associated lid edema, severe
anomalies, pulmonary hypoplasia, ear chemosis, serosanguineous
abnormalities discharge and pseudo membrane
formation. Systemic treatment
Poorer compared to type 1 is necessary to avoid systemic
complications. Previously i.v
aqueous penicillin G 100000 units/
kg/day in divided doses for 7 days,
however, due to resistance a single
Table 6: Differentiating features between Peter and Sclerocornea i.m dose of 125mg ceftriaxone
Peter Sclerocornea became the recommended
Opacity involves the central cornea Opacity more pronounced peripherally treatment. Cefotaxime 25mg/kg
and its localized than the central cornea. Variant can i.v or i.m every 8-12 hours for 7
involve the total cornea
days can also be given. Oral dose of
fluoroquinolones is also effective
Iris strands attach to the posterior Generally absent. Could be seen if but have potential disadvantage
corneal defect/ leukoma associated with anterior chamber of inducing antibiotic resistance.
cleavage anomalies
In addition to systemic treatment,
Mostly occurrence is sporadic. AR/AD sporadic (can be familial/ AD/ AR form) hourly irrigation of the eyes with
have been observed. Gene involved is KERA gene saline, topical fluoroquinolones are
Genes involved PITX2, FOXC1,
CYP1B1, PAX6. used in the early treatment.
Better prognosis with PK compared to Worst prognosis than Peter8 4. Metabolic diseases
Sclerocornea
• Mucopolysaccharidosis
if significant central vision of both form) • Mucolipidosis
eyes is affected with relatively normal Penetrating grafts carry very poor visual
other ocular structures as the outcomes outcomes in these cases. • Tyrosinemia- AR disorder due to
of the surgery are very poor. Usually 2. Tears in endothelium and Descemet defect in soluble hepatic cytosol
penetrating grafts are required, however membrane tyrosine aminotransferase
the prognosis for a clear graft poorer Left eye more frequently affected due (chromosome 16). Only type II
than Peter anomaly. to common head crowning position (Richner-Hanhart syndrome) is
Either/ both eyes could be affected associated with corneal opacity. It
Waring and Rodrigues classified Usually less incidence of photophobia is characterized by a triad of mental
sclerocornea into 4 groups 3: Photophobia commonly present retardation, dendriform lesions of
3. Ulcers the cornea, hyperkeratotic lesions
• Isolated peripheral sclerocornea; • Viral- all infections of herpes in of palms and soles. It usually
• Sclerocornea plana; appears in first few months of age
• Sclerocornea associated with the neonatal period should be and it is important to distinguish
this from herpes keratitis. Bilateral
anterior chamber cleavage involvement, morphology (lack of
anomalies; terminal bulbs and poor staining
• Total sclerocornea (most common of the lesion, consistent infero-
central location), normal corneal
30 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Pediatric Ophthalmology
Table 7: Differentiating features between types of endothelial dystrophies
Congenital Hereditary Endothelial Posterior Polymorphous Corneal Congenital Stromal Corneal Dystrophy
Dystrophy Dystrophy Corneal opacity- central with feathery
appearance. Corneal thickness is normal
Diffuse, non-bullous severe corneal Lesser corneal edema with usually Corneal changes are present at birth and
edema (thickness increased by 2-3 normal corneal thickness are stationary
times) leading to bluish-gray ground
glass appearance Autosomal dominant
Corneal clouding present at birth or Onset in first or second decade with
shortly after slow progression of corneal clouding
over a decade
No associated symptoms like epiphora/ Usually present with epiphora
photophobia and photophobia with subsequent
developing clouding of cornea
Autosomal recessive Autosomal dominant
Table 8: Differential diagnosis of dermoids
Dermoid Keloid Salzmann nodules
They are multiple, bluish-white
They are yellowish-white elevated They are chalky white solid masses superficial corneal nodules which are
nodule containing hair follicles, with glistening appearance and the usually seen in mid periphery.
sebaceous glands, sweat glands, bones, texture is usually gelatinous. They are not present at birth and are
cartilages or teeth, situated in the Can be central with often associated usually related to associated ocular
inferotemporal limbus usually. satellite lesions. inflammation like VKC, trachoma,
Definitive diagnosis made with corneal keratoconus, EBMD or corneal surgery)
biopsy
sensitivity, lack of response to Lubricating eye drops and as cornea verticillata. They are not
anti-viral treatment (persisting therapeutic bandage contact present at birth but can occur as
longer than 6 weeks), exacerbation lenses could be useful in providing early as six months of age. Enzyme
with increased protein intake and symptomatic relief. Topical replacement therapy is available for
systemic associations should rise cysteamine solution 0.55% Fabry disease.
the suspicion of tyrosinemia4. administered every 1-2 hourly could • Gangliosidoses
Child should be advised tyrosine and be useful in dissolving the cystine 5. Posterior corneal defect
phenylamine restricted diet which accumulated. It is also useful post-
would resolve corneal lesions. PK to prevent re-deposition. • Peter anomaly- congenital disorder
with a central leukomatous
• Cystinosis- AR disorder caused • Fabry disease- XLR disorder due corneal opacity with associated
by mutation in CTNS gene in to lack of the enzyme alpha defect in posterior stroma, DM and
chromosome 17, leading to defect galactosidase A leading to endothelium. It is usually bilateral
in the cystine transport with deposition of sphingolipids within (80%) and asymmetrical. There
intracellular accumulation of lysosomes. It is characterized by would be associated defects in angle
free cystine, thereby resulting in angiokeratomas of skin along with development leading to increased
crystalline deposition in various multisystem involvement. Ocular incidence of glaucoma (50-80%).
tissues (eye, kidneys, bone manifestations include corneal
marrow, lymph nodes). These opacities (90%), conjunctival vessel Peter-plus syndrome- Peter +
needle shaped crystals are seen in tortuosity (60%), retinal vessel mental retardation, short stature,
cornea (epithelium, stroma and tortuosity, cataract (Fabry cataract- brachymorphy, ear abnormalities, cleft
endothelium) and conjunctiva. spoke like posterior capsular lip and palate.
They start anteriorly and in cataract).6 Corneal changes are
periphery and later involve the earliest and most consistent ocular Krause-Kivlin syndrome- Peter +
entire cornea5, leading to irritation, changes seen. In advanced cases, developmental delay, retarded skeletal
pain and photophobia. fine, curvy or whorl-like superficial maturity with disproportionate short
corneal opacity are seen, known stature and facial abnormalities
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 31
Subspeciality-Pediatric Ophthalmology
Hence, patients of Peter should be (choristoma). Three grades of dermoid 4. Charlton KH, Binder PS, Wozniak L,
screened for systemic malformations choristoma have been described based Digby DJ. Pseudodendritic keratitis and
especially midline body structures on the extent of involvement (Mann systemic tyrosinemia. Ophthalmology.
like heart and pituitary gland.7 grading)10: 1981 Apr 1;88(4):355-60.
Definite ocular management includes
penetrating keratoplasty though in Grade1: most frequent type; it is single, 5. Cotran PR, Bajart AM. Congenital corneal
few cases with small localized opacity small usually less than 5mm diameter opacities. International ophthalmology
attempt of optical iridectomy could be in the inferotemporal limbus generally clinics. 1992 Jan 1;32(1):93-105.
done. Outcomes of PK are better in type involving superficial structures. It is
1 Peter than in type 2.8 present at birth and may enlarge during 6. Fledelius HC, Sandfeld L, Rasmussen
puberty. One-third cases could have ÅK, Madsen CV, Feldt‐Rasmussen
• Posterior keratoconus- Uncommon, associated syndrome complex such as U. Ophthalmic experience over 10
mostly unilateral, sporadic and Goldenhar syndrome (triad of epibulbar years in an observational nationwide
non-progressive characterized by dermoid, preauricular appendages and Danish cohort of Fabry patients with
local conical, internal protrusion pre-tragal fistulas) access to enzyme replacement. Acta
of the posterior corneal curvature ophthalmologica. 2015 May;93(3):258-
with associated stromal thinning. Grade 2: larger covering a part or entire 64.
It could be mildest variant of Peter cornea but does not involve DM or
anomaly with no relationship to endothelium 7. Traboulsi EI, Maumenee IH.
anterior keratoconus Peters’ anomaly and associated
Grade 3: most severe type; rarest; congenital malformations. Archives
• Congenital anterior staphyloma- anterior segment is involved in this type of Ophthalmology. 1992 Dec
it is characterized by protruding, 1;110(12):1739-42.
ectatic congenital corneal opacity Management- Grade 1 dermoid-
of one or both eyes. The lesion can conservatively managed (except 8. Kim YW, Choi HJ, Kim MK, Wee WR,
be vascularized and the posterior when induced irregular astigmatism, Yu YS, Oh JY. Clinical outcome of
portions of these ectatic thin corneas amblyopia unresponsive to penetrating keratoplasty in patients
are lined by pigment epithelium of conservative management, progressive 5 years or younger: peters anomaly
the atrophic iris. Pathogenesis is dellen, inadequate lid closure, aesthesis). versus sclerocornea. Cornea. 2013 Nov
unknown though it is thought to be Surgical intervention from simple 1;32(11):1432-6.
secondary to intrauterine infections excision to lamellar/ penetrating grafts
or developmental abnormality. might be required for larger dermoids. 9. Busin M, Beltz J, Scorcia V. Descemet-
stripping automated endothelial
6. Endothelial dystrophy References keratoplasty for congenital hereditary
1. Waring GO, Rodrigues MM. Congenital endothelial dystrophy. Archives
Management- DSAEK could be of ophthalmology. 2011 Sep
performed in cases of CHED/ PPCD if and neonatal corneal abnormalities. 12;129(9):1140-6.
there is no significant stromal scarring Tasman W, Jaeger E. Duane’s
in the visual axis. Performing surgery ophthalmology, CD-Rom. Lippincott 10. Mann I. Developmental abnormalities
at early age could improve visual Williams & Wilkins: Philadelphia; 2002 of the eye. CUP Archive; 1957.
development and potentially avoid 2. Nischal KK. A new approach to the
amblyopia9. If significant stromal classification of neonatal corneal Corresponding Author:
scarring is present in visual axis, full opacities. Current opinion in
thickness graft is preferred. ophthalmology. 2012 Sep 1;23(5):344-54. Dr. Mohamed Ibrahime Asif
3. Waring 3rd GO, Rodrigues MM. Dr. Rajendra Prasad Centre for Ophthalmic
7. Dermoid Ultrastructure and successful Sciences. AIIMS, New Delhi, India.
keratoplasty of sclerocornea in Mietens’
They are congenital overgrowth of syndrome. American journal of
normal tissues in an abnormal location ophthalmology. 1980 Oct;90(4):469.
32 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Glaucoma
Anterior Segment Dysgenesis
Aafreen Bari MD, Dewang Angmo MD, DNB, FRCS, FICO
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
Abstract: Anterior segment dysgenesis is failure of normal development of tissues of anterior segment of the eye leading to
anomalies of cornea, angle, iris and lens associated with increased risk of glaucoma and corneal opacity. Various genes like
PITX2, FOXC1 and PAX6 in relation to the neural crest cells play role to produce a spectrum of disorders with phenotypic
variability and genetic heterogenicity. Timely diagnosis and management can help to visually rehabilitate the individual and
decrease the risk of amblyopia.
Anterior segment dysgenesis (ASD) Figure 1: Showing the three waves by neural crest cells at 4th, 5th and 7th week gestational
is a spectrum of disorders involving age.
congenital anomalies of the cornea,
angle, iris and lens. Earlier it was Failure of proper interaction due eye anomalies together with dental
termed as ‘Anterior chamber cleavage to various recognized transcription anomalies (OMIM 180500). Since
syndrome’ or ‘Mesodermal Dysgenesis’ factors like PAX6 (chromosome then, studies reporting similar cases
but later it was recognized that the 11p13), PITX2 (chromosome 4q25), in different families lead to a series of
affected tissues had their origin from FOXC1 (chromosome 6p25), FOXE3 clinical classifications, for example,
the neural crest-derived mesenchymal (chromosome 1p23) and CYP1B1 iridogoniodysgenesis, iris hypoplasia,
progenitor cells, thus giving the (chromosome 2p22) results in a Rieger anomaly, Axenfeld anomaly,
spectrum preferable name as ‘anterior complex spectrum of entities included Peter’s Anomaly and Axenfeld–Rieger
segment dysgenesis’. in anterior segment dysgenesis anomaly4.
syndrome3.
Relevant Embryology The Step Ladder Classification5 divides
The eye begins to develop during the Classification of ASDS the anomalies into three groups:
4th week of gestation as an outgrowth In 1925 a German ophthalmologist,
from the neuroectoderm. At 5 weeks, Karl Axenfeld described congenital a. Peripheral (prominent Schwalbe’s
the invagination of optic vesicle angle anomalies with iris strands, and line, prominent iris processes,
forms double layered optic cup of the in 1935 an Austrian ophthalmologist, hypoplasia of anterior iris stroma).
neuroectoderm. The surface ectoderm Herwigh Rieger characterised Rieger
forms the lens placode and gives rise to syndrome as a dominantly inherited b. Central (central posterior corneal
the corneal and conjunctival epithelium condition involving abovementioned defect, central irido-corneal
and the eyelid epidermis. At the same adhesions, corneo-lenticular
time, the first wave of mesenchymal
cells from neural crest cells of surface
ectoderm extends under epithelium
from limbus to begin forming corneal
endothelium. A subsequent second
wave of mesenchymal cells at the 7th
week forms the corneal stroma and
sclera1. Finally, the third wave migrates
into the space adjacent to the anterior
rim of the developing optic cup,
contributing to the stroma of the ciliary
body and iris, as well as the trabecular
meshwork2 (Figure 1).
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Subspeciality-Glaucoma
Table 1: Showing spectrum of ASD associated with the genes and embryotoxon (Figure 2) with bands
overlapping clinical features of iris tissue extending to anterior
chamber angle and Schwalbe’s line6
(Figure 3).
The disorder is mostly bilateral with
variable extent of anomaly in both the
eyes.
Rieger’s anomaly has mid-peripheral
iridocorneal adhesions in addition
to significant iris abnormalities like
iris hypoplasia, pupillary distortion,
polycoria, and corectopia (Figure 4).
It may be associated with other ocular
abnormalities like cataract, strabismus,
retinal detachment, macular
degeneration, optic nerve hypoplasia,
and chorioretinal coloboma. It has
also been reported with lenticular
abnormalities like microspherophakia7.
Figure 2: Arrow showing anteriorly approximation). Clinical features like dental anomalies
displaced schwalbe’s line, known as c. Combination of central and (hypodentia, microdentia, anodentia),
posterior embryotoxon. facial anomalies (hypertelorism, malar
peripheral components. hypoplasia, hypospadias, redundant
In 2007, Sowden et al established the periumbilical skin) and other
spectrum of ASD based on clinical systemic features like cardiovascular,
symptoms and associated genes4 as in neurological, hearing defects, short
(Table 1). stature and mental deficiency may be
associated. In such cases it is called the
Axenfeld Rieger Anomaly Rieger’s Syndrome (Figure 5).
Axenfeld’s anomaly is characterized
by prominent anteriorly displaced There have been many cases of
Schwalbe’s line known as posterior overlapping phenotypes between
Figure 3: Gonioscopic image of band of iris tissue extending to Figure 4: Slit shaped pupil with patchy iris defects with corectopia
Schwalbe’s line. and polycoria.
34 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Glaucoma
Figure 5: Features of systemic involvement – oligodontia and hypodontia (5a); peg-shaped This spectrum has been divided in
teeth (5b); malar hypoplasia (5c); and redundant periumbilical skin (5d). following two types:
Figure 6: paracentral corneal opacity (6a); and UBM with irido-lenticular band (6b). a. Type I: It has iris attachments
from collarette adhering to
Axenfeld’s Syndrome and Rieger’s or drainage devices can be tried. corneal stroma. There is absence of
Syndrome in the persons from same Cycloablation should be the last resort. Descemet’s membrane in this area
family, so they have been together Mandal et al have reported high with normal lens.
termed as the Axenfeld Rieger’s success rates of primary combined
Syndrome (ARS). trabeculotomy- trabeculectomy with a This localized defect in the central
ARS is mostly autosomal dominant mean drop in IOP from 27 to 14.9 mm or paracentral portion of the
with bilateral involvement. Glaucoma Hg post-operatively8. Descemet membrane, known as
is associated in 50% cases. They usually internal ulcer of von Hippel.
present in first or second decade of life. Peter’s Anomaly
Medical management with control of Peter’s Anomaly presents as bilateral b. Type II: Lens may be adherent to
intraocular pressure should always be central corneal opacity with adhesions corneal endothelium and cataract
tried first. If target IOP is not reached of central iris to posterior surface of may be present.
using medical management, surgery cornea.
It is important to first ascertain the
extent of ocular involvement by
ultrasound biomicroscopy (UBM),
B scan, or anterior segment optical
coherence tomography (AS OCT),
if it is not obvious clinically (Figure
6).
Nearly 50% patients of Peters anomaly
may have associated ocular findings
like myopia, microphthalmos, aniridia,
cataract and retinal detachment.
Systemic findings may also be present
like developmental delay, congenital
heart and ear disease, hearing and
spinal defects and cleft palate. If
systemic findings are present in a case
of Peter’s anomaly, it is called Peter’s
Plus Syndrome, also known as Kivlin
syndrome. Unlike the ARS, it is mostly
sporadic and autosomal recessive
disorder.
Glaucoma is present in 50% cases.
when the presentation is in infancy,
trabeculodysgenesis may be the cause
and surgery is preferred over medical
management.
Trabeculectomy with anti-scarring
agents may be considered in mild
Peters anomaly, and primary GDD
surgery may be indicated in the
moderate to severe forms9. Transscleral
cyclophotocoagulation is useful in
cases of refractory glaucoma9.
Conclusion
A wide spectrum of disorders come
under the term ‘Anterior Segment
Dysgenesis.’ Defective migration of
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Subspeciality-Glaucoma
neural crest cells results in dysgenesis 26: 374-386. 9. Yang LL, Lambert SR, Lynn MJ, Stulting
of trabecular meshwork, iris, cornea RD. Surgical management of glaucoma
and in some cases even the lens. Early 4. Sowden, J. Molecular and developmental in infants and children with Peters’
diagnosis, intervention and visual mechanisms of anterior segment anomaly: long-term structural and
rehabilitation are necessary to avert dysgenesis. Eye 21, 1310–1318 (2007). functional outcome. Ophthalmology,
amblyopia and development and 2004; 111:112-117.
progression of glaucoma. 5. Reese AB, Ellsworth RM. The anterior
chamber cleavage syndrome. Arch Corresponding Author:
References Ophthalmol 1966; 75:307-318.
1. BCSC Section 2, Fundamentals and Dr. Dewang Angmo MD, DNB, FRCS,
6. Stamper, Robert L, Marc F. Lieberman, FICO, MNAMS
Principles of Ophthalmology. Michael V. Drake, and Bernard Becker. Dr. R.P.Centre for Ophthalmic Sciences,
Becker-shaffer’s Diagnosis and Therapy All India Institute of Medical Sciences,
2. Akula, Monica et al. “Relationship of the Glaucomas. Edinburgh: Mosby/ New Delhi, India
between neural crest cell specification Elsevier, 2009.
and rare ocular diseases.” Journal of
neuroscience research vol. 97,1 (2019): 7. Shakrawal J, Selvan H, Sharma A, Angmo
7-15. D. Double trouble: Microspherophakia
with Axenfeld-Rieger anomaly. Indian J
3. Cvekl, A. and Tamm, E.R. (2004), Ophthalmol 2019;67:394-5.
Anterior eye development and ocular
mesenchyme: new insights from mouse 8. Mandal, A., Pehere, N. Early-onset
models and human diseases. Bioessays, glaucoma in Axenfeld–Rieger anomaly:
long-term surgical results and visual
outcome. Eye 30, 936–942 (2016).
36 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Retina
Choroidal Melanoma
Mushriff Supriya, Goynka Yashaswi
Index Medical College Hospital and Research Centre, Indore
Choroidal melanoma is the most Figure 1: USG B scan showing collar stud appearance in the right eye, pathognomonic of Choroidal
common primary intraocular melanoma.
malignancy in adults1. Choroidal
melanoma accounts for 80% of all limits. Right eye USG showed low to is mostly diagnosed in the 6th decade
uveal melanomas and usually occurs moderate hyperechoic opacity in the of life. Exposure to sunlight, fair skin
in adults of between 40 and 60 years of vitreous cavity of mushroom-shaped complexion, light iris colour, pre-
age. Males have a 30% greater incidence configuration arising from choroid existing melanocytic nevi is the main
than females. In the US, the incidence suggestive of choroidal melanoma. etiological factors. Genetic factors and
is approximately 5 cases per million Computed tomography gave the numerous mutations also play a role.
individuals, with a significantly higher impression of a semi-circular shape soft It usually presents with the blurring of
incidence in non-Hispanic whites (6.02 tissue mass lesion of approximate size vision if the lesion is near the fovea and
per million) when compared with 1.1 *1.3 cm which had a broad base and optic nerve or due to retinal detachment,
blacks and Asians (0.31 and 0.39 per was located in the medial wall of right field defects, floaters, photopsia, pain
million respectively). We herein report globe. On post-contrast, the mass lesion (due to secondary glaucoma).
a case of a 45-year-old female patient showed subtle enhancement. Right Diagnosis can be made by
diagnosed with choroidal melanoma. optic nerve appeared to be thickened as ultrasonography which reveals
compared to the left optic nerve. Liver “collar button” or “mushroom” like
Case History function test was within normal limits. appearance. The melanoma breaks
A 45-year-old female presented with the overlying Bruch’s membrane
chief complaints of sudden painful Discussion reaching the subretinal space, thus
diminution of vision and redness in the The incidence of choroidal melanoma achieving the shape of a“collar button”
right eye for 10 days. There was no history is 5-10 cases per 10 lakhs people,2 and
of trauma and a similar complaint in the
past. There was no significant family
history. On examination, best-corrected
visual acuity (BCVA) of her right eye
was perception of light with accurate
projection of rays in all quadrants.
Anterior segment examination of the
right eye revealed hyperpigmentation
of iris which was confined to the inferior
half of iris suggestive of the proliferation
of melanocytes on anterior iris stroma.
The pupil was mid-dilated. Intraocular
pressure (IOP) was 60 mmHg. BCVA
of the left eye was 6/9 and the anterior
segment was within normal limits. Left
eye IOP was 18mmHg. On examining
the fundus, right eye fundus revealed
a solid mass in posterior segment
occupying vitreous cavity with
overlying retinal detachment in media
and rest fundus was not appreciable.
Left eye fundus was within normal
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Subspeciality-Retina
Figure 2: Low to moderate hyperechoic opacity in the vitreous Figure 3: Right eye Anterior segment showing hyperpigmentation of iris and ectropion
cavity of mushroom-shaped configuration arising from choroid uvea.
suggestive of Choroidal melanoma.
or “mushroom”, which are highly Indications for enucleation include of uveal melanoma and prediction of
characteristic of, even pathognomic larger tumour size, optic disc invasion patient survival after enucleation and
of choroidal melanoma3. Fluorescein extensive involvement of the ciliary brachytherapy. Am J Ophthalmol.
angiography shows “Double body and irreversible vision loss. 1991;112:682–688.
circulation” consisting of choroidal 6. Munk P, Downey D, Nicolle D, Vellet
and tumour vessels that are leaky in References AD, Rankin R, Lin DT. The role of colour
late phases of a fluorescein fundus 1. Scotto J, Fraumeni JF, Jr, Lee JA. flow Doppler ultrasonography in the
angiography is characteristic of uveal investigation of disease in the eye and
melanoma4. MRI and CT are also found Melanomas of the eye and other orbit. Can J Ophthalmol. 1993;28:171–
to be very helpful. Ultrasonography has noncutaneous sites: epidemiologic 176.
been reported to have an accuracy of aspects. J Natl Cancer Inst. 1976;56:489–
>95%5,6. 491. Corresponding Author:
2. Singh AD, Topham A. Incidence of uveal
Treatment depends on the size of the melanoma in the United States: 1973– Dr. Mushriff Supriya
tumour. A pigmented lesion with a 1997. Ophthalmology. 2003;110:956– Index Medical College Hospital and Research
diameter larger than 5DD (7.5mm) 961. Centre, Indore, M.P. India.
should be considered a malignant 3. Zimmerman, L. E. Malignant melanoma.
melanoma until proven otherwise. If In: Spencer, W. H., (ed). Ophthalmic
tumour size <2mm then conservative Pathology. 3rd Ed. Philadelphia: W.B.
management is advised. Tumour size Saunders Company; 1986:2072-2139.
<10mm across and up to 2mm thick then 4. Shields, JA, Shields, CL, Donoso,
one can go for plaque brachytherapy. If LA. Management of posterior uveal
tumour size is 10-15mm in diameter melanoma. Surv Ophthalmol.
and 3-5mm in height, plaque or external 1991;36:161-95.
proton beam radiation can be opted. 5. Coleman DJ, Silverman RH, Rondeau MJ,
et al. Ultrasonic tissue characterization
38 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Oculoplasty
Orbital Cellulitis Post Uneventful
Phacoemulsification
Avantika Verma MBBS, DNB, ICO (UK)
Shekar Eye Hospital, Bangalore, Karnataka, India
Abstract: The aim of this study was to report a case of orbital cellulitis that occurred after routine uneventful cataract operation
(Phacoemusification) under peribulbar anaesthesia in southern region of India. A complete ophthalmic evaluation, systemic
evaluation, investigations, treatment and follow up of a 60 year old male patient was done. Orbital cellulits following cataract
surgery is extremely rare. A timely diagnosis was made and the patient was treated with intravenous antibiotics. Patient did
not have any predisposing risk factors, therefore most likely cause of cellulitis was surgical trauma during administration
of the peribulbar block. This case illustrates the need for adequate skin preparation before the administration of peribulbar
anaesthesia and minimal tissue trauma during the procedure.
Keywords: orbital cellulitis, peribulbar anaesthesia complications, phacoemulsification.
Orbital cellulitis is a clinical condition Medical history and Family history hydrochloride, 0.75% bupivacaine
arising usually from a complication of were non significant. hydrochloride and hyaluronidase 1500
paranasal sinus infection and affecting units was administered. A total of 4 ml
soft tissues of the orbit, posterior to Pre Op Examination was injected at the junction of the middle
the orbital septum1,2. It is a relatively On examination, his vision in right and outer thirds of the lower lid and 2.5
common disease of developing eye was 2/60, PH No improvement, ml at the medial canthus via a 3/8-inch,
countries, with a frequency ranging Left eye was 6/12, PH 6/9. Extraocular 25-gauge needle. Patient underwent
between 21-90%3. It has various causes movements were full and normal. uneventful phacoemulsification and
and may be associated with serious Anterior Segment showed right eye posterior chamber intraocular lens
complications, cavernous sinus senile immature cataract, grade 3 implantation in the right eye.
thrombosis being the most dangerous, with posterior subcapsular cataract, Right eye Post Op Examination
resulting in visual loss and death. Prompt left eye had pseudophakia. Both the Day 1
diagnosis and treatment are essential for pupils were reactive to light, no APD On examination right eye vision
treatment of this condition. However, was noted. Posterior segment showed was 6/36, PH No improvement, color
it is an uncommon complication of CDR 0.3, macula normal, retina on. vision was normal. Severe restriction
ophthalmic surgery. Cases have been Intraocular pressure was 14 mm Hg
reported after strabismus surgery, for the right eye, and 16 mm Hg for the Figure 1: Post OP Day 1 OD.
blepharoplasty, radial keratomy and left eye. Keratometry readings for the
retinal surgery4,5. Various case reports right eye were K1 44.50, K2 45.00. A
on orbital cellulitis following peribulbar scan Biometry showed right eye AXL
anaesthesia have been described before, 21.7 mm, A Constant 118.20, IOL Power
alcohol preparation thought to be a 21.50 D.
contributory factor.
Treatment Plan
Case Report Phacoemulsification and posterior
A 60 year old male was admitted to our chamber intraocular lens implantation
hospital with a history of diminishing in the right eye under local anesthesia
of vision in right eye since 6 months. He was planned for him. Skin preparation
had underwent phacoemulsification with 5% povidone iodine was done
with posterior intraocular lens prior to peribulbar block. A peribulbar
implantation in his left eye 1 year ago. injection consisting of 2% lignocaine
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 39
Subspeciality-Oculoplasty of ocular movements was noted.
Figure 2: MRI 1. Adenexa showed 5mm of axial
proptosis, periorbital swelling, lid was
tense and difficult to open. Right eye
anterior segment showed conjunctival
chemosis, Anterior chamber showed
a trace of cells and flare, PCIOL in bag.
Pupil was reactive to light, no APD.
Posterior segment was CDR 0.3 with
macula normal, retina on. Intraocular
pressure in right eye was15 mm Hg
(Figure 1).
Diagnosis
A diagnosis of right eye orbital cellulitis
was made.
Management
The patient was admitted to hospital.
Intravenous antibiotics in addition
to antibiotic-steroid combination
eyedrops were started. Intravenous
cefotaxim 1gm BD, metronidazole 1
Figure 3: MRI 2. Figure 4: MRI 3.
40 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Oculoplasty
Figure 5: On Discharge. cataract extraction with intraocular References
lens insertion and trabeculectomy 1. Jack Kanski, Brad Bowling. Clinical
vial BD and gentamicin 80 mg BD were has been reported by Hofbauer et al.6
administered to provide Gram-positive They felt that skin preparation with Ophthalmology: A Systematic
and Gram-negative coverage. CT scan alcohol prior to injection of the local Approach, 7th Edition, 2010, Page 175-
orbit and paranasal sinuses showed the anaesthetic instead of povidone iodine 177.
right eye to be proptosed, with extensive resulted in incomplete asepsis, thus 2. Douglas JR, Marck FP. The Wilms Eye
periorbital soft-tissue swelling leading to access of the skin flora into Manual, Office and Emergency Room,
consistent with orbital cellulitis the orbit. Redmill et al7 reported orbital Diagnosis and Treatment of Eye Disease,
(Figure2,3,4). The adjacent sinuses were cellulitis following corneal gluing 3rd Edition, YEAR, Page 174-177.
clear and without evidence of sinusitis. under subtenon’s local anaesthesia in 3. M. R. Shaikh, M. Baqir, N. Zakir. Orbital
Culture of conjunctiva, nose and an immunocompromised patient. Here Cellulitis, Masquerading as Cavernous
pharynx was negative. Blood cultures altered conjunctival flora due to intake Sinus Thrombosis, Journal of Pakistan
were also negative. The leucocyte of systemic immunosuppressives had Medical Association, 2004, Page 280-282.
count showed mild leucocytosis. Renal been the predisposing factor. In our 4. Weakley DR. Orbital cellulitis
function tests and blood gentamicin patient, the short postoperative time complicating strabismus surgery: a case
levels were regularly monitored to course (less than 48 h), absence of sinus report and review of the literature. Ann
prevent renal toxicity. disease and occurrence of skin trauma Ophthalmol 1991; 23(12): 454-457.
Post Op Examinations during injection in an otherwise 5. McLeod SD, Flowers CW, Lopez PF, Marx
On continous post op examinations immunocompetent patient indicate J, McDonnell PJ. Endophthalmitis and
dramatic improvement in the proptosis peribulbar injection as the possible orbital cellulitis after radial keratomy.
and ocular inflammation were seen over cause of periorbital cellulitis. However, Ophthalmology 1995; 102: 1902–1907.
the next 24 hours. The ocular motility in spite of skin preparation with 5% 6. Hofbauer JD, Gordan LK, Palmer J.
gradually improved with reduction povidone iodine it is likely that trauma Orbital cellulitis after peribulbar
in periorbital swelling. At the time of during injection resulted in the access injection. Am J Ophthalmol 1994; 118:
discharge, right eye uncorrected visual of the skin flora to the orbit through 391–392.
acuity was 6/9 and there was complete the needle track causing cellulitis. 7. Redmill B, Sandy C, Rose GE. Orbital
recovery within a week (Figure 5). The ecchymosis facilitated the spread cellulitis following corneal gluing under
Discussion of infection. Prompt recognition and subtenon’s local anaesthesia. Eye 2001;
Orbital cellulitis following peribulbar treatment of cellulitis resulted in a 15(4): 554–556.
anaesthesia for combined extracapsular favorable outcome.
Corresponding Author:
Conclusion
Orbital cellulitis is usually treated with Dr. Avantika Verma MBBS, DNB, ICO (UK)
parenteral broad spectrum antibiotics Shekar Eye Hospital,
such as third or fourth generation Bangalore, Karnataka, India
cephalosporins and metronidazole to
cover most organisms. However, blood
gentamicin levels and renal function
test need to be closely monitored to
prevent renal toxicity. It is suggested
that an aseptic technique should be
used with minimal soft tissue trauma
during peribulbar anaesthesia.
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Subspeciality-Oculoplasty
Evaluation of Acquired Ptosis
(Lt Col) Neeraj Sharma MS
Command Hospital, Kolkata, India
Definition- A ptosis which is acquired or injury to branch of 3rd nerve as seen in road traffic accidents
later in life and not present since birth supplying LPS. is usually present in Traumatic
is leveled as acquired ptosis. Acquired ptosis work up include ptosis (direct laceration/dehiscence
diligent history taking and detailed of levator palpebralis superioris
Causes:- can be broadly categorized as ocular and systemic examination along muscle or involvement of branch of
follows with appropriate investigation to help 3rd nerve).
identify the cause and hence plan
(a) Aponeurotic (b) Neurogenic appropriate management. - Painful ptosis associated with a
(c) Myogenic (d) Mechanical and vertical squint is suggestive of
Traumatic. History:- Following important clues an inflammatory cause such as
from history will help identify most myositis or myocysticercosis2.
(a) Aponeurotic ptosis- occurs because likely cause.
of thinning/dehiscence of levator - A history of diurnal variation in the
palpebrae superioris aponeurosis Onset- an acute onset of ptosis may level of ptosis along with history of
(LPS). Mostly seen in senile ptosis, be manifestation of life threatening diplopia, improving in the morning
various inflammatory conditions of condition -the posterior communicating and after rest is typically seen in
lids like blepharochalasis, chronic artery aneurysm pressing upon the third myasthenia gravis.
conjunctivitis and as a part of nerve, and hence needs to be evaluated
traumatic ptosis. with a sense of urgency1. - Associated systemic complaints like
difficulty in walking, in swallowing
(b) Neurogenic ptosis-related to - Progression-A bilateral ptosis and speech hint towards a systemic
involvement of 3rd nerve supplying which started in early childhood, disorder like muscular dystrophy or
LPS or interruption in sympathetic progressive in nature and myasthenia gravis.
outflow to Muller’s muscle. 3rd associated with limitation of
nerve can get affected because of ocular movements may hints - Any history of previous eye surgery
various vascular, neoplastic or towards myogenic ptosis like (cataract or Trabeculectomy- eye
inflammatory lesions at any point chronic progressive external speculum/bridle suture induced
in its course from its nucleus ophthalmoplegia (CPEO). injury to superior rectus and levator
through cranial cavity and into palpebralis superioris muscle
orbit. - Senile ptosis seen in older complex)
population is because of lax lid
(c) Myogenic ptosis-include systemic structures as a part of ageing results - Use of Botox injection to treat
disorders like Myasthenia gravis, in Aponeurotic type of ptosis. for essential blepharospasm-
chronic progressive external Peudoptosis
ophthalmoplegia (CPEO) and - In a young individual with
various muscular dystrophies. recurrent episodes of swelling - History of contact lens use- causing
along with redness in the lid may be microtrauma.
(d) Mechanical ptosis-gravity related related to Blepharochalasis (don’t
drooping that occurs in condition operate in inflamed lid). - Examination- A baseline ocular
like upper eye lid odema or examination must be performed in
hematoma post trauma or surgery, - History of frequent episodes of all cases.
upper eyelid mass lesions (benign redness, itching and rubbing of
/ malignant lid tumors) and in eyes is presents in chronic allergic • Specific examination that are
neurofibromatosis (NF). conjunctivitis causing Aponeurotic essential to workup of acquired
type of ptosis. ptosis must include:
(e) Traumatic ptosis-seen in traumatic
injuries due to direct laceration/ - A history of direct trauma to the lid 1. Eyelid eversion- Examination of
stretching of LPS aponeurosis or a history of closed head injury palpebral conjunctiva and fornices
to look for mechanical causes of
ptosis such as symblepharon or
large papillae.
42 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Oculoplasty
2. Examination of the Pupil - Figure 1: Senile ptosis : Note bilateral frontalis muscle over action, along with brow ptosis,
a. a miotic pupil in case with mild dermatochalasis and lower lid entropion right side.
ptosis is suggestive of Horner’s Figure 2: Blepharochalasis-loose fold of skin with altered skin texture.
syndrome
b. dilated pupil with limitation Figure 3: Mechanical ptosis in NF type 1.
of extra ocular movement
and squint and severe ptosis trauma or any surgical procedure. along with redundant skin folds
is suggestive of 3rd nerve are characteristic feature of
involvement 2. A loose lax skin fold Blepharochalasis (Figure 2).
c. Traumatic ptosis may be (Dermatochalasis) is a part of senile B. Mechanical ptosis - as seen in
associated with traumatic ptosis. There can be associated brow Neurofibromatosis (NF) (Figure 3).
mydriasis (sphincter injury). ptosis (Figure 1).
C. Aponeurotic ptosis- is characterized
3. Fundus examination to look for 3. A Cigarette paper feel of lid skin
pigmentary retinopathy is essential
to rule out Kearns-Sayre syndrome,
in a case of CPEO.
4. Squint work is very important to
pick up cases of ptosis secondary to
a. Partially recovered 3rd nerve
palsy: apart from mild limitation
of movement adduction,
depression and elevation,
it may be associated with
misinnervation of the eyelid.
b. Acquired monocular elevation
defect (MED) where hypotropia
is seen in association with ptosis-
Pseudoptosis
c. Inflammatory lesion of the SR-
LPS complex- may cause painful
ptosis and vertical squint.
d. Intermittent squint- Myasthenia
gravis
e. Traumatic ptosis may also be
associated with squint
Abnormal Eyelid Movements -
Misinnervation of eyelid may be seen
in partially recovered 3rd nerve palsy,
causing abberrant movement of eyelid
and variable amounts of ptosis in
different gazes.
Rule out Enophthalmos/
Microphthalmos in the same eye or lid
retraction in other eye that may case
pseudoptosis.
Some other consideration during
workup of acquired ptosis is:-
A. Eyelid skin
1. Look for any scar mark of previous
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Subspeciality-Oculoplasty
Table 1: A Generalized Ptosis Work up includes
Parameter Measured Significance Remark
Head Posture /Any Seen In Bilateral Severe B/L Surgical Intervention
Chin Lift Ptosis if Surgical Cause Present
Frontalis Overaction Compensatory Mechanism No Frontalis Action-
-Poor Prognosis for
Frontalis Sling Surgery Figure 4: High lid crease and fold seen in
-Crutch Glasses for Poor Aponeurotic ptosis.
Lps Action
Phenyelphrine test for mild ptosis:-
Presence and Height Helps Plan Incision for High Eyelid Crease is this test is done in mild ptotic eye
of Eyelid Crease and Ptosis Surgery With an Seen in Aponeurotic with good levator action to check
Eyelid Fold Aim to Provide Eyelid Ptosis for the suitability for a Conjunctival
Crease Symmetry Muller resection. One drop of 2.5% or
preferably 10% Phenylephrine drop
Mrd1 and Mrd2 To Determine Amount of Mild Ptosis Present in is instilled in eye after an interval of 5
Interpalpebral Fissure Ptosis and Severity Horner’s Syndrome minutes and MRD1 is noted after 10
Height Mrd1 + Mrd2 = Severe Ptosis - Seen minutes ,an improvement of 2mm in lid
Interpalpebral Fissure in 3rd Nerve Palsy, height is taken as positive test3 (Figure
Height (Measurement Traumatic or 5).
Done with Patient And Aponeurotic Ptosis Horner’s syndrome- the diagnosis of a
Examiner at Same Level suspected Horner’s syndrome is based
-Block Frontalis While on demonstration of denervation
Taking Mrd1) hypersensitivity of pupil and levator
to sympathetic stimulus and can be
Levator Palpebralis (A) Type of Surgery Severe Ptosis with Fair confirmed by pharmacological testing
Superioris (Lps) Muscle Depends Upon Lps Lps Action - Suggestive with apraclonidine 0.5% or 1%4.
Action Action-(Lps Resection, Lps Of Aponeurotic Ptosis Common etiologies include: carotid artery
Advancement/Plication) dissection, aortic dissection, trauma,
(B) Poor Lps Action- demyelinating disease such as multiple
Frontalis Sling Surgery sclerosis (MS), tuberculosis, sarcoidosis,
(Block Frontalis Muscle cluster headache, Pancoast syndrome,
Before Measuring Lps herpes zoster, giant cell arteritis (GCA)
Action) and malignancy. The imaging is of
utmost help in clinching a cause based
Tbut And Schirmer’s Determine Ocular Surface Poor Ocular Surface upon clinical evaluation5.
Test Wetting Wetting -Relative
Contraindication For Myasthenia gravis-
Surgical Intervention Icepack test is a simple bedside test
to rule out Myasthenia gravis in a
Bell’s Phenomenon To Look For Post Surgical Poor /Reverse suspected case of fluctuating ptosis
Corneal Exposure Bell’s Phenomenon with squint. Here MRD1 and ocular
-Contraindication for alignment is noted before and after
Surgery applying icepack over lids for a period
of 5 minutes. An improvement in ptosis
Eversion of Eyelid To Look for Upper Look for Papillae/ or ocular alignment signifies positive
Palapebral and Forniceal Follicles-for Chronic test (Figure 6).
Conjunctiva Conjunctivits and Cogan’s lid twitch sign seen in
Symblepharon myasthenia gravis and is related to easy
fatigability of lids on eye movements.
by Severe ptosis with fair to good LPS appreciated clinically as prominence of
action along with high lid crease - sharp upper border of tarsus (feels like
seen in senile ptosis, bleharochalasis a step). At times through the overlying
and post traumatic ptosis because skin at the thinned out aponeurosis iris
of lid hematoma stretching levator color can be appreciated in a closed lid.
aponeurosis (Figure 4). Other relevant tests for acquired ptosis
Thinning of LPS aponeurosis can be work up:-
44 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Subspeciality-Oculoplasty
(a) (b)
Figure 5: (a) before Phenylephrine test (mild ptosis left eye) (b) after response.
(a) (b)
Figure 7: CT scan (orbit) cyst with scolex in
extra ocular muscle.
Figure 6: (a) Before Icepack test (b) after test-Improved eye alignment and lid position. Other systemic work up:-
For Pupil sparing 3rd nerve palsy
Table 2: Role of Imaging in cases of Acquired ptosis having ischemic (microvasculopathy)
as underlying mechanism work up
CE MRA/CTA Acute pupil Rule out posterior communicating for systemic disease like Diabetic
involving 3rd nerve artery aneurysm pressing upon 3rd mellitus, Hypertension or Toxic and
palsy nerve Demyelinating disorders should be
done in consultation with treating
CE CT/MRI Adult onset Horner’s Rule out any neoplastic, clinician.
syndrome inflammatory / vascular lesion
lesions affecting course of 1st Apart from Imaging, serology test like
order, preganglionic 2nd order or indirect immunofluorescence and
postganglionic 3rd order sympathetic immune electrophoresis such as ELISA
neurons at the level of Hypothalmus, and western blot may aid in diagnosis of
spinal cord, apex of lung, neck and suspected myocystiercosis8.
cavernous sinus 5.
Myocysticercosis of LPS SR complex7 For myogenic ptosis test like muscle
(Figure 7) biopsy, genetic testing, EMG, blood tests
for low magnesium level, increased
Myasthenia gravis Rule out Thymoma lactic acid, increased pyruvic acid,
increased CPK are warranted to rule out
Neurofibromatosis Rule out sphenoid wing dysplasia muscular dystrophy9.
Orbital lesion Neoplastic, inflammatory / vascular References
lesion affecting branches of 3rd nerve 1. Fang C, Leavitt JA, Hodge DO, Holmes
in orbit at superior orbital fissure,
cavernous sinus JM, Mohney BG, Chen JJ. Incidence
and Etiologies of Acquired Third Nerve
USG Orbit Rule out myocysticercosis Palsy Using a Population-Based Method.
JAMA Ophthalmol. 2017;135(1):23-28.
Here patient is asked to look in up gaze Other important tests for diagnosing 2. Sharma V, Saxena R, Betharia SM,
and down gaze several times by showing myasthenia are: Elevated antibody Sharma P. Myocysticercosis: a cause of
him a target like a pencil until patient against Ach receptors (AChR-Ab titers) acquired ptosis in children simulating
is asked to stop suddenly in primary seen in approx. 85% of patients, anti congenital ptosis with lid lag (report of
position of gaze; while in normal person MuSK antibodies (positive in 10-15%), two cases). J AAPOS. 2004;8(4):390-392.
upper eyelid comes to rest immediately, Tensilon test with IV Edrophonium 3. Ben Simon GJ, Lee S, Schwarcz RM,
in patients with myasthenia gravis the drug, repetitive nerve stimulation test McCann JD, Goldberg RA. Muller’s
lid overshoots the primary position and CT thorax to look for associated muscle-conjunctival resection for
with a twitch before it comes back Thymus enlargement6. correction of upper eyelid ptosis:
slowly to resting position. relationship between phenylephrine
testing and the amount of tissue resected
with final eyelid position. Arch Facial
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 45
Subspeciality-Oculoplasty
Plast Surg 2007;9(6). 7. Pushker, N, Bajaj, MS, Chandra, M. Corresponding Author:
Ocular and orbital cysticercosis. Acta
4. Mughal M, Longmuir R. Current Ophthalmol Scand 2001; 79: 408–413. Dr. (Lt Col) Neeraj Sharma MS
pharmacologic testing for Horner Command Hospital,
syndrome. Curr Neurol Neurosci Rep. 8. Rodriguez S, Wilkins P, Dorny P. Kolkata, India
2009;9(5):384-9. Immunological and molecular diagnosis
of cysticercosis. Pathog Glob Health.
5. Almog Y, Gepstein R, Kesler A. 2012;106(5):286-298.
Diagnostic value of imaging in horner
syndrome in adults. J Neuroophthalmol. 9. Sinha R, Sarkar S, Khaitan T, Dutta S.
2010;30(1):7-11. Duchenne muscular dystrophy: Case
report and review. J Family Med Prim
6. Akshay Gopinathan Nair, Preeti Patil- Care. 2017;6(3):654-656.
Chhablani, Devendra V Venkatramani,
Ocular myasthenia gravis: A review
Indian J Ophthalmol. 2014 Oct; 62(10):
985–991.
46 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Basics
Importance of Complete
Histopathology and
Immunohistochemistry Work up
in Ocular Pathology to Diagnose a
Rare case
Arpan Gandhi
Head Ocular Pathology, Dr Shroff’s Charity Eye Hospital, New Delhi, India.
Abstract: Germ cell tumor can affect extragonadal sites. Teratoma in literature is a well-recognized extragonadal tumor in the
orbit. Primary yolk sac tumor (YST) of the Orbit is A rare Clinico histological entity in literature too Its clinical presentation
and an early diagnosis improves ocular morbidity and mortality. In. We would like to describe a case of primary orbital YST and
aim to describe the clinical presentation, imaging features, histopathological and immunohistochemistry characteristics, and
management of orbital YST.
Germ cell tumors usually arise from Figure 1 A&B: Meshwork of myxoid intervening spaces lined by flat to cuboial cells. These
the primordial cells in gonads and cells showed a high degree of anaplasia and scattered miotic activity. Figure 1c: These
cases with extragonadal involvement tumour cells are arranged in various patterns - microsystic, macrocystic reticular, alveolo-
is rare. Of the extragonadal germ cell gladular and myxomatous. Figure 1D: Tumour cells positive of SALL-4.
tumors Teratoma is the commonest one
and which typically involves midline
structures and called as primary orbital
teratoma or combined teratoma. The
primary orbital presentation of germ
cell tumor is rare and postulated to
result from either ectopic primitive
germ cell or defect in embryogenesis
.8,12 It is known that the germ cells could
migrate through the midline dorsal
mesentery before reaching gonadal
ridge. Therefore, this can lead to germ
cell tumor. Another proposed theory
for the same is the presence of ectopic
extra-embryonic germ cells which reach
the circulation during embryogenesis
These ectopic cells probably arise from
endodermal cells of the yolk sac, and
therefore, they are called ‘‘yolk sac
carcinoma/tumor’’.
Case Report
A 6-year-old female child presented
with a history of gradual progressive
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 47
Basics
forward protrusion of the right eye SALL-4 and AFP was Immunoreactive, rare neoplasms. In our case, the 6-year-
since 4 months. On examination, the score 4+ in neoplastic cells, CD56 old child presented with proptosis and a
child had Protrusion of the right eye, Immunoreactive, score 1+ in neoplastic well-defined mass in the superior orbit.
bleeding, swelling, ocular pain OD since cells,CD99 Dim Immunoreactive, Clinical differential diagnoses in such
3-4 months sudden proptosis started at score 1+ in neoplastic cells. a case are rhabdomyosarcoma, Ewings
the time of lockdown started – about 3 Synaptophysin Non-immunoreactive, sarcoma, leukemia and eosinophilic
months ago .The anterior and posterior score 0 in neoplastic cells. S-100 Non- granuloma. Because of rarity of orbital
segment examination of both eyes was immunoreactive, score 0 in neoplastic YST, clinical diagnosis is difficult and
unremarkable. cellsCD117 Immunoreactive, score diagnosis solely rests upon detailed
2+ in neoplastic cells,GLYPICAN-3 histopathological examination and a
MRI orbits showed a Heterogenous, Immunoreactive, score 3+ in neoplastic complete immunohistochemistry panel
hyperintense lesion filling right orbit cells, CD30 Non - immunoreactive, by an experienced Ocular pathologist.
reaching till orbital apex. score 0 in neoplastic cells, MPO Non- Of the 13 published cases in English
immunoreactive, score 0 in neoplastic language initial histopathological
Globe distorted, no definite intraocular cells. CD34 Non-immunoreactive, misdiagnosis have been made in 5/14
mass.Previous CT (3 months back) = score 0 in neoplastic cells, PLAP (35%) reported cases. It is possible that
isodense mass medial intraconal space Immunoreactive, score 1+ in neoplastic orbital YST may be under-reported in
pushing optic nerve laterally. MR not cells, Beta-HCG Non-immunoreactive, the literature due to lack of awareness
seen separately. CE MRI multilobulated score 0 in neoplastic cells, EMA Non- and experience. The histopathological
intraconal mass seen - heterogenously immunoreactive, score 0 in neoplastic picture with the selection of a detailed
enhancing involving optic nerve and cells, Ki-67 Proliferative index 40-50%. Immunohistochemistry Panel is critical
MR - not seen separately isodense to for such rare neoplasms. The salient
EOM. Serum Alpha Feto Protein was markedly points of a cellular lesion mixed with
increased 7200 ng/ml normal range is myxomatous areas, with tumor cells
With a clinical diagnosis of right less than 10 ng/ml. showing positivity to AFP and hyaline
orbital rhabdomyosarcoma, complete globules staining with PAS. AFP and
excision biopsy of the orbital mass Management SALL -4 stain is a useful marker for
through transcutaneous (upper eyelid After the surgical excision, immunohistochemical staining of
incision) approach was performed. chemotherapy, and radiotherapy YST and raised serum AFP levels are
Postoperatively, at 2 weeks, there was were started and the there was an useful for diagnosis, prognosis and
significant reduction with no palpable improvement in the ocular prognosis management AFP is a glycoprotein
mass/proptosis/dystopia. A repeat CT and was able to limit the morbidity and produced by yolk sac and liver during
orbit showed the complete absence of mortality for such rare neoplasms. fetal development Serum AFP levels are
the mass. also useful to monitor the response to
Discussion chemotherapy and identify ANY tumor
Histopathological sections of the tissue Orbital YST are rare tumors seen in recurrence. The most common clinical
sent showed a cellular lesion with infants and young children. The need presentation of orbital YST is proptosis.
meshwork of myxoid intervening spaces to diagnose them early is because they and is related to the obvious mass effect.
lined by flat to cuboidal cells. These cells have a rapid progressive course with
showed a high degree of anaplasia and a tendency to invade surrounding References
scattered mitotic activity. These tumor structures including brain. The
cells are arranged in various patterns- other sites apart from what we have 1. Ahmad Z, Khurshid A, Qureshi A (2009)
microcystic, macrocystic, reticular, reported are - extragonadal sites - pelvis, Primary orbital yolk sac tumour in a
alveolar-glandular and myxomatous. retroperitoneum, and mediastinum 14-year-old girl. BMJ Case Rep.
Scattered areas of hemorrhage are seen. which are more commonly reported in
Pseudopapillary pattern with organoid the literature. On literature search we 2. Appignani BA, Jones KM, Barnes PD
structures resembling glomeruli called could not find well-defined guidelines (1992) Primary endodermal sinus tumor
Schiller–Duval bodies can be seen on for the management of orbital YST of the orbit: MR findings. AJR Am J
histopathology. Myxomatous stroma due to rarity of the disease. Based on Roentgenol 159:399–401.
consists of hyaline globules are seen. our published literature, an early
histological diagnosis and multimodal 3. Bresters D, Zwaan ChM, Veerman AJP et
Complete IHC Panel was CD45 Non- treatment with surgical excision, al (2003) A threeyear-old girl with a yolk
immunoreactive, score 0 in neoplastic chemotherapy, and radiotherapy may sac tumor in the orbit/maxillarysinus.
cells, DESMIN Non-immunoreactive, improve the ocular prognosis and limit Med Pediatr Oncol 40:70–71.
score 0 in neoplastic cells, CK the morbidity and mortality for such
Immunoreactive, score 4+ in neoplastic 4. Crandall BF (1981) Alpha-fetoprotein: a
cells. review. Crit Rev Clin Lab Sci 15:127–185.
5. Devaney KO, Ferlito A (1997) Yolk sac
tumors (endodermal sinus tumors) of
48 DOS Times - Volume 25, Number 6, May-June 2020 www.dosonline.org/dos-times
Basics
the extra cranial head and neck regions. tumors in the head and neck region. Med structures of the rat’s placenta. Cancer
Ann Otol Rhinol Laryngol 106:254–260. Pediatr Oncol 29:303–307. 25:644–658.
16. Yih JP, Sullivan P, Taylor D (1995)
6. Dragan LR, Aghaian E, Vora R, Kim GE, 11. Margo CE, Folberg R, Zimmerman LE, A-fetoprotein as marker for a case of
Seiff SR (2004) Orbital, middle cranial Sesterhenn IA (1983) Endodermal sinus orbital yolk sac tumor. Br J Ophthalmol
fossa, and pterygopalatine fossa yolk tumor (yolk sac tumor) of the orbit. 79:787–798.
sac tumor in an infant. Ophthal Plast Ophthalmology 90:1426–1432. 17. Kim JH, Oh DE, Kim YD (2007) Primary
Reconstr Surg 20:469–471. yolk sac tumor of the orbit. J Korean
12. Mogaddam AS, Memar B, Aledavood Ophthalmol Soc 8:604–610.
7. Katz NNK, Ruymann FB, Margo A, Eslampoor A (2007) Isolated orbital
CE, Popejoy LA, La Piana FG (1982) endodermal sinus tumor. Ophthal Plast Corresponding Author:
Endodermal sinus tumor (yolk sac Reconstr Surg 23:477–479.
carcinoma) of the orbit. J Pediatr Dr. Arpan Gandhi
Ophthalmol Strabismus 19:270–274. 13. Pierce BG, Bullock WK, Huntington RW Head Ocular Pathology,
Jr (1970) Yolk sac tumors of the testis. Dr Shroff’s Charity Eye Hospital,
8. Kiratli H, Erkan Balci K, Gu¨ler G (2008) Cancer 25:644–658. New Delhi, India
Primary orbital endodermal sinus tumor
(yolk sac tumor). JAAPOS 12:623–625. 14. Shields JA, Sheilds CL (2003)
Rhabdomyosarcoma: review for the
9. Kivela T, Tarkkanen A (1994) ophthalmologist. Surv Ophthalmol
Orbital germ cell tumors revisited: 48:39–57.
a clinicopathological approach to
classification. Surv Opthalmol 38:541– 15. Telium G (1970) Endodermal sinus
554. tumors of the ovary and testis:
comparative morphogenesis of the so-
10. Kusumakumari P, Geetha N, Chellam called mesonephroma ovarii (Schiller)
VG, Nair MK (1997) Endodermal sinus and extraembryonic (yolk sacallantoic)
www.dosonline.org/dos-times DOS Times - Volume 25, Number 6, May-June 2020 49
PG Corner
Filamentary Keratitis
Deepali Singhal1, Prafulla K. Maharana2 MD
1. Institute of Vision and Optics, VEIC, University of Crete, Greece.
2. Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
Filamentary keratitis (FK) is a form Table 1: Ocular and systemic associations with filamentary keratitis
of chronic and recurrent disorder of
the ocular surface mainly involving Ocular Systemic Ophthalmic Medication
the cornea and rarely, conjunctiva. It surgery
is characterized by the deposition of
mucin filaments on the corneal surface. Dry eye disease Rheumatoid Penetrating Diphenhydramine
FK has been reported to be associated arthritis keratoplasty hydrochloride
with several ocular surface disorders like
dry eye, keratoconjunctivitis,exposure Severe Meibomian Sjögren Vitreo-Retinal
keratitis, allergic conjunctivitis, Gland Dysunction syndrome surgeries
postcataract surgery,brain stem injury,
penetrating keratoplasty (PKP), ptosis, Recurrent corneal Psoriasis Cataract
recurrent erosion, prolonged eye patch erosions extraction
use, and large-angle strabismus.1 FK
is diagnosed clinically on slit lamp Superior limbic Atopic Photorefractive
biomicroscopic examination. keratoconjunctivitis dermatitis keratectomy
Pathophysiology Herpes simplex Brainstem LASIK
The majorly accepted composition keratitis injury
of these filaments is a combination
of degenerated epithelial cells with Neurotrophic Cranial nerve
mucus. The composition of these keratopathy palsy
filaments has been described by
Tanioka et al.2 Light microscopic Prolonged patching
analysis has shown that filaments
contain a core of eosinophillic cells However, it has been shown earlier that raised epithelium acts as a receptor
(corneal or conjunctival epithelium) these filaments are primarily composed for the mucus and the debris
with spindle - shaped cytoplasm of mucus with epithelial squamous produced due to irregular corneal
and nuclei which is surrounded by cells, lipids, and foreign matter taken surface.4
basophilic fibers and areas including up secondarily.3 • Maudgal theory: According
basophilic segments and polymorphic to this theory, there are areas
nucleic cells. Immunostaining Several authors have described the of focal degeneration of the
demonstrated a central core of CK12- theories leading to formation of the corneal epithelial cells along with
positive corneal epithelium surrounded filaments in FK. deposition of overlying mucus.
by CK4- and CK13-positive conjunctival This is followed by migration
epithelium, peripheral mucin, DNA, • Zaidman theory: The authors of surrounding epithelial cells
and inflammatory cells.The mucin is used transmission electron into that area with focal defect.
positive for MUC1-, MUC4-, MUC5AC-, microscopy in a patient with These cells are also covered by
and MUC16- and inflammatory cellsare brain stem disease to formulate mucus, leading to the formation
positiveHLA-DR and neutrophil this theory. It was seen that there of a protuberance. This further
elastase. is accumulation of inflammatory gets elevated with the continued
cells and fibroblasts below the basal migration of epithelial cells
epithelium with disruption of the forming a filament.5
epithelial basement membrane
and Bowman’s layer. This leads to Risk factors/ Associations
epithelial basement membrane Various ocularand systemic associations
detachment due to the shearing have been reported with FK (Table 1).
force of eyelids. Then the area of The major ocular risk factors include
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