Regional Institute of Ophthalmology, PGIMS, Rohtak

reduce the risk of systemic complications in posterior hyaloid, thereby worsening tractional retinal
premature infants.12,13 Aflibercept, a fusion protein detachment. A similar phenomena of “crunch” after
that inhibits all isoforms of VEGF-A, has high anti-VEGF therapy is observed in proliferative
binding affinity and longer intraocular half-life, diabetic retinopathy (PDR) where anti-VEGF therapy
which may result in a longer duration of clinical may worsen traction on the retina.17
action intraocularly than either ranibizumab or
bevacizumab.13 The combination of a smaller size and impaired
blood–retinal barrier in premature infants leads to
Bevacizumab is a monoclonal antibody that was increased serum concentrations of bevacizumab
originally developed for the treatment of metastatic following intravitreal injections. Serum bevacizumab
colorectal cancer and has been used with good results levels 8 weeks after intravitreal injection still prevents
in treating many retinopathies. Currently, VEGF from acting in preterm infants at a stage when
bevacizumab is the most commonly studied VEGF is needed for the development of kidneys,
intravitreal anti-VEGF therapy for the treatment of lungs, brain, and other organs.18
severe ROP; it has thus far demonstrated promising
results, particularly in severe cases of posterior One of the most important barriers to widespread anti-
disease.14,15 VEGF use in ROP is lack of certainty regarding the
systemic long-term sequelae of anti-VEGF therapy in
Dosage and systemic absorption of VEGF neonates. Although 56% of pediatric and retinal
inhibitors subspecialists self-reported having used antiVEGF
agents to treat ROP, 66% of those surveyed reported a
The optimal dosages for anti-VEGF agents remain lack of certainty in systemic effects.7 Intravitreal anti-
controversial. The adult dose of intravitreal VEGF agents in the most commonly used dosages
bevacizumab (IVB) is 1.25 mg/0.05 mL and of seem to enter systemic circulation and affect serum
intravitreal ranibizumab (IVR) is 0.5/0.05 mL. The VEGF levels. Bevacizumab is detected in serum after
most commonly used dose of anti-VEGF in neonates intravitreal injection in animal models, and serum
is half the adult dose: 0.625 mg IVB (range, 0.03 VEGF levels decrease after intravitreal anti-VEGF
mg17 to 1.25 mg19) and 0.25 mg IVR (range, 0.15 injections in infants.19,20
mg20 to 0.3 mg21).
Ocular outcomes of VEGF inhibition in the
The neonate eye is estimated to be less than one third treatment of ROP
the normal volume of an adult eye; therefore,
evidence suggests that lower doses of anti-VEGF may Unlike laser, treatment that inhibits VEGF does not
be more appropriate.14 Benefits of lower dosing involve the destruction of the peripheral retina. It
include reduced risk of intraocular toxicity, systemic allows for continued vessel growth into the peripheral
absorption, and potential adverse effects and lesser retina, thus avoiding some of the complications of
volume delivered. Drawbacks of lower dosing ablative treatment. The BEAT-ROP (bevacizumab
include inadequate treatment, possible higher rates of eliminates the angiogenic threat of ROP) trial results
recurrence, and special compounding necessary for published in 2011 were the first data from a
lower doses, thereby increasing the risk of multicenter randomized trial on anti-VEGF treatment
contamination and dilutional errors. in ROP. This study included 300 eyes of 150 infants;
they were randomly assigned to receive bevacizumab
Effective dosing of anti-VEGF therapy seems not or laser therapy. The rate of recurrence for zone I and
only to be quantity dependent but also time posterior zone II combined was significantly higher
dependent. There is an optimal window, at the first with conventional laser therapy than with intravitreal
sign of plus disease or neovascularization but before bevacizumab (26% versus 6% of infants, p = 0.002).
the formation of extensive fibrovascular membranes, The rate of recurrence for zone I ROP alone was
during which anti-VEGF has the best benefit to risk significantly higher with conventional laser therapy
profile. Given in stage 3, plus disease, early APROP, than with intravitreal bevacizumab (42% versus 6%
and even early stage 4, IVB and IVR induce of infants, p = 0.003), but for posterior zone II ROP the
regression of ROP.16 However, when administered in recurrence rate did not differ significantly between
late stage 4 or 5 disease, anti-VEGF may cause the two treatment modalities (12% versus 5% of
contraction of the fibrovascular membranes and

49

infants, p = 0.27). Furthermore, the rate of ROP between the 2 groups, but reported a significantly
recurrence in the laser group was high compared to higher chance of high myopia (more than 5.00
other studies. One of the limitations of this study was diopters) in the IVB group.24 The potential decreased
short follow-up (20 weeks after treatment or up to 54 incidence of early unfavorable refractive outcomes in
weeks of PMA). This study has introduced anti- the anti-VEGF groups compared with the laser group
VEGF treatment as a potential treatment option for may represent a potential benefit of anti-VEGF
ROP in infants with stage 3+ ROP in zone I.9 Lepore therapy, but consistent supporting data need to be
et al. compared the structural outcomes at 9 months of substantiated.
eyes treated with intravitreal injection of
bevacizumab with fellow eyes treated with Recurrence
conventional laser photoablation. Abnormalities at
the periphery (large avascular area, abnormal Recurrence remains an ever important and
vascular branching, presence of shunt vessels) and the challenging topic. The clinical definition of
posterior pole (hyperfluorescent lesion, absence of recurrence varies. Additionally, dosing, treatment,
foveal avascular zone) were noted in all eyes treated zones, stages, and duration of follow-up vary, making
with intravitreal bevacizumab, but were not observed direct comparison between series difficult.
in the majority of the laser-treated eyes.21 Tahija et al Recurrence has been defined as reappearance of plus
did a similar study to evaluate vascularization of the disease, neovascularization, extraretinal
peripheral retina using fluorescein angiography (FA) fibrovascular proliferation, new ridge after prior
for infants who had been treated with intravitreal initial regression, or progression of disease despite
bevacizumab for zone I and posterior zone II ROP and prior treatment. By FA, eyes with peripheral
found that 11 of 20 eyes had not achieved normal avascularity (eg, persistent retinal ischemia greater
retinal vascularization. The time interval between than 2 disc diameters from the ora after intravitreal
treatment with intravitreal bevacizumab and FA anti-VEGF treatment) and leakage at the peripheral
ranged from 27 to 224 weeks.15 There are fewer vascular-avascular junction have been described as
studies reporting on the ocular outcomes after “treatment-requiring” to prevent retinal detachment.25
ranibizumab intravitreal injections for ROP. By convention, most studies generally define
recurrence as treatment-requiring progression of
Refractive outcomes disease after prior improvement. In 317 infants, the
ETROP reported that 13.9% needed retreatment after
One of the major benefits of anti-VEGF therapy over laser therapy.6 Hwang et al14 showed, in 54 eyes with
laser therapy is that it offers a decreased incidence of type 1 ROP, 3% recurrence after laser [2.6 weeks after
myopia; however data on refractive outcomes after treatment, postmenstrual age (PMA) 35.3 weeks]
anti-VEGF treatment are mixed. Kuo et al. compared compared with 14% recurrence after IVB (9 weeks
patients who were treated with laser therapy or IVB to after treatment, PMA 45 weeks). Karkhaneh et al26
those with nontype 1 ROP under conservative follow- showed, in 86 eyes with zone II, stage 2‒3+ ROP,
up and premature babies without ROP. Patients who 1.4% recurrence after laser (3 weeks after treatment)
received treatment for ROP were more prone to compared with 10.5% recurrence after IVB (5 weeks
myopia, regardless of treatment modality. The results after treatment). Zhang et al27 showed, in 100 eyes
suggest that treatment-demanding ROP eyes are with zone II, stage 2 or 3+ disease, 4% recurrence after
susceptible to more severe myopia with age compared laser compared with 52% recurrence after IVR. Thus,
with eyes without ROP or those with spontaneously it can be inferred that recurrence after laser therapy is
regressed ROP.22 In contrast, Gunay et al who observed in a small but significant percentage of
examined 48 eyes treated with IVB compared with 30 patients and generally occurs within the first 9 weeks
eyes treated with traditional laser therapy, found that after treatment. In comparison with conventional
refractive errors were significantly less myopic with laser ablative therapy, recurrence after IVB is
lower rates of anisometropia and strabismus at 2 years observed more frequently and later after initial
in those treated with IVB compared with laser.23 In treatment. Yetik et al28 showed, in 122 patients with
prethreshold, threshold, and APROP disease treated
comparing IVB with IVR, Chen et al found no with IVB monotherapy, 4.6% required additional
significant differences in mean refractive errors injections by 89 weeks PMA. In the original BEAT
ROP study, time to recurrence was 19.2 weeks after

50

IVB compared with 6.4 weeks after laser treatment in Retinopathy of prematurity in the United States.
zone I. Thus, though both laser and anti-VEGF Br J Ophthalmol. 2008;92:320‒325.
therapy induce initial regression of ROP, eyes treated
with IVB require longer follow-up for recurrence. In 4. Smith BT, Tasman WS. Retinopathy of
comparison with IVB and conventional laser ablative prematurity: late complications in the baby
therapy, recurrence after IVR is observed more boomer generation (1946-1964). Trans Am
frequently than either IVB or laser and earlier after Ophthalmol Soc. 2005; 103:225‒234.
treatment than IVB. Li et al29 demonstrated, in 32 eyes
treated with IVR monotherapy for APROP, 22% 5. Multicenter trial of cryotherapy for retinopathy of
recurrence at 2‒8 weeks after injection. Erol et al30 prematurity. Preliminary results. Cryotherapy for
showed, in 36 eyes with type 1 ROP, 27% of eyes Retinopathy of Prematurity Cooperative Group.
treated with IVR monotherapy recurred at a mean of Arch Ophthalmol. 1988;106:471‒479.
41 weeks PMA.
6. Early Treatment for Retinopathy of Prematurity
Recurrence after the combination of laser and anti- Cooperative Group. Revised indications for the
VEGF agents as primary treatment has not been treatment of retinopathy of prematurity: results of
extensively studied. Kim et al31 demonstrated no the Early Treatment for Retinopathy of
recurrence at a mean of 83.6 weeks PMA with a Prematurity randomized trial. Arch Ophthalmol.
combination of IVB and laser in 18 eyes with zone I 2003;121:1684‒1694.
ROP. A case of later recurrence after combined IVR
and laser treatment in 1 patient with zone I, stage 3+ 7. Madan A, Penn JS. Animal models of oxygen-
disease was reported at 4 months after treatment. induced retinopathy. Front Biosci.
Regardless of treatment modality, patients with initial 2003;8:1030‒1043.
regression of ROP should be monitored closely for
recurrence, with laser expected to recur earlier and 8. Goldberg MA, Schneider TJ. Similarities between
IVR and IVB expected to recur more frequently and the oxygen-sensing mechanisms regulating the
later. expression of vascular endothelial growth factor
anderythropoietin.JBiolChem.1994;269:4355‒4359.
Conclusion
9. Mintz-Hittner HA, Kennedy KA, Chuang AZ;
Recent studies consistently report that anti-VEGF BEAT-ROP Cooperative Group. Efficacy of
injections work well in stopping ROP progression, intravitreal bevacizumab for stage 3+ retinopathy
and can be performed quickly without the need for the of prematurity. N Engl J Med. 2011;364:603‒615.
general anaesthesia as is the case with conventional
laser therapy. The rate of reccurrence and need for 10. Smith LE. Through the eyes of a child:
repeat injections and thus longer, more frequent understanding retinopathy through ROP. The
follow-ups are definite obstacles to widespread Friedenwald Lecture. Invest Ophthalmol Vis Sci.
implementation 2008;49: 5177‒5182.

References 11. Hartnett ME. Studies on the pathogenesis of
avascular retina and neovascularization into the
1. Ludwig CA, Chen TA, Hernandez-Boussard T, et vitreous in peripheral severe retinopathy of
al. The epidemiology of retinopathy of prematurity (an American Ophthalmological
prematurity in the United States. Ophthalmic Surg Society thesis). Trans Am Ophthalmol Soc
Lasers Imaging Retina. 2017;48:553‒562. 2010;108:96–119.

2. Blencowe H, Lawn JE, Vazquez T, et al. Preterm- 12. Menke MN, Framme C, Nelle M, et al. Intravitreal
associated visual impairment and estimates of ranibizumab monotherapy to treat retinopathy of
retinopathy of prematurity at regional and global prematurity zone II, stage 3 with plus disease.
levels for 2010. Pediatr Res. 2013;74(Suppl BMC Ophthalmol. 2015;15:20.
1):35‒49.
13. Stewart MW, Rosenfeld PJ. Predicted biological
3. Lad EM, Nguyen TC, Morton JM, et al. activity of intravitreal VEGF trap. Br J
Ophthalmol. 2008;92(5):667–678.

14. Hwang CK, Hubbard GB, Hutchinson AK, et al.
Outcomes after intravitreal bevacizumab versus
laser photocoagulation for retinopathy of
prematurity: a 5-year retrospective analysis.

51

Ophthalmology. 2015;122:1008‒1015. 25. Toy BC, Schachar IH, Tan GS, et al. Chronic
vascular arrest as a predictor of bevacizumab
15. Tahija SG, Hersetyati R, Lam GC, et al. treatment failure in retinopathy of prematurity.
Fluorescein angiographic observations of Ophthalmology. 2016;123:2166‒2175.
peripheral retinal vessel growth in infants after
intravitreal injection of bevacizumab as sole 26. Karkhaneh R, Khodabande A, Riazi-Eafahani M,
therapy for zone I and posterior zone II et al. Efficacy of intravitreal bevacizumab for
retinopathy of prematurity. Br J Ophthalmol. zone-II retinopathy of prematurity. Acta
2014;98:507‒512. Ophthalmol. 2016;94:417‒420.

16. Cheng HC, Lee SM, Hsieh YT, et al. Efficacy of 27. Zhang G, Yang M, Zeng J, et al. Comparison of
intravitreal injection of anti-vascular endothelial intravitreal injection of ranibizumab versus laser
growth factor agents for stage 4 retinopathy of therapy for zone II treatment-requiring
prematurity. Retina. 2015;35:660‒666. retinopathy of prematurity. Retina.
2017;37:710‒717.
17. Wong RK, Hubschman S, Tsui I. Reactivation of
retinopathy of prematurity after ranibizumab 28. Yetik H, Gunay M, Sirop S, et al. Intravitreal
treatment. Retina. 2015;35:675‒680. bevacizumab monotherapy for type-1
prethreshold, threshold, and aggressive posterior
18. Qian J, Lu Q, Tao Y, Jiang YR. Vitreous and retinopathy of prematurity - 27 month follow-up
plasma concentrations of apelin and vascular results from Turkey. Graefes Arch Clin Exp
endothelial growth factor after intravitreal Ophthalmol. 2015;253:1677‒1683.
bevacizumab in eyes with proliferative diabetic
retinopathy. Retina. 2011;31:161–168. 29. Li XJ, Yang XP, Sun S, et al. Intravitreal
ranibizumab for aggressive posterior retinopathy
19. Bakri SJ, Snyder MR, Reid JM, et al. of prematurity. Chin Med J (Engl).
Pharmacokinetics of intravitreal bevacizumab 2016;129:2879‒2881.
(Avastin). Ophthalmology. 2007;114:855‒859.
30. Erol MK, Coban DT, Sari ES, et al. Comparison of
20. Miki K, Miki A, Matsuoka M, et al. Effects of intravitreal ranibizumab and bevacizumab
intraocular ranibizumab and bevacizumab in treatment for retinopathy of prematurity. Arq Bras
transgenic mice expressing human vascular Oftalmol. 2015;78:340‒343.
endothelial growth factor. Ophthalmology.
2009;116:1748‒1754. 31. Kim J, Kim SJ, Chang YS, et al. Combined
intravitreal bevacizumab injection and zone I
21. Lepore D, Quinn GE, Molle F, et al. Intravitreal sparing laser photocoagulation in patients with
bevacizumab versus laser treatment in type 1 zone I retinopathy of prematurity. Retina.
retinopathy of prematurity: report on fluorescein 2014;34:77‒82.
angiographic findings. Ophthalmology.
2014;121:2212–2219. Address for Correspondence :

22. Kuo HK, Sun IT, Chung MY, et al. Refractive Dr. Ritesh Verma
error in patients with retinopathy of prematurity Sr. Resident,
after laser photocoagulation or bevacizumab RIO, PGIMS,
monotherapy. Ophthalmologica. 2015; Rohtak-124001 (Haryana)
234:211–217. E-mail : [email protected]
Mobile : 9914432104
23. Gunay M, Celik G, Gunay BO, et al. Evaluation of
2-year outcomes following intravitreal
bevacizumab (IVB) for aggressive posterior
retinopathy of prematurity. Arq Bras Oftalmol.
2015; 78:300–304.

24. Chen SN, Lian I, Hwang YC, et al. Intravitreal
antivascular endothelial growth factor treatment
for retinopathy of prematurity: comparison
between ranibizumab and bevacizumab. Retina.
2015; 35:667.

52

Limbal Stem Cell Deficiency :
Etiology, Diagnosis and Management

Monika MS, J P Chugh MS, Latika Pandey MBBS,
Ester MBBS, Aakash Sharma MBBS, Sakshi Lochab MBBS,

Regional Institute of Ophthalmology, PGIMS, Rohtak

Abstract

Severe ocular surface disease can result in limbal stem cell deficiency (LSCD), a condition leading to ocular
pain, photophobia and decreased visual acuity. First step in the management of limbal stem cell deficiency is early
control of the ocular surface inflammation and elimination of the causative factor. To restore the ocular surface in
advanced stem cell deficient corneas, an autologous or allogenic limbal stem cell transplantation is performed.
Successful limbal stem cell transplantation can achieve rapid surface healing, stable ocular surface, regression of
corneal vascularization, and restoration of a smooth and optically improved ocular surface, resulting in improved
visual acuity and increased success for subsequent keratoplasty.

Introduction Secondary causes 
Thermal or chemical burns
Cornea is highly organised transparent tissue Contact lens wear
consisting of multiple cellular and noncellular layers. Inflammatory eye disease:  Stevens-Johnson
The corneal epithelium covers the corneal surface and Syndrome
plays a major role in protection and transparency. Toxic Epidermal Necrolysis
Epithelial cells are shed regularly and replaced by Ocular Cicatricial Pemphigoid
stem cell sources located at the limbus, a rim of tissue Chronic Limbitis
located at corneoscleral junction. The limbal
epithelial stem cells (LESCs) reside in specific
regions at the limbus known as the limbal stem cell
niches or Palisades of Vogt. Damage to the stem cells
or disruption of the niches may lead to Limbal Stem
Cell Deficiency (LSCD). In the absence of a healthy
corneal epithelium, the conjunctiva proliferates over
the cornea resulting in opacification and
vascularization of cornea, which in turn may lead to
reduced vision, pain, and photophobia.1-4 LSCD can
be caused by a wide variety of primary and secondary
causes but is most frequently seen associated with
severe chemical or thermal burns.5

Etiology of limbal stem cell deficiency Diagnosis of LSCD
It is often on the bases of history and clinical findings,
Primary causes which include loss of limbal anatomy, corneal
Aniridia conjunctivalization, persistent epithelial defects, and
Multiple endocrine deficiency scar formation. In partial LSCD clinical signs are
Epidermal dysplasia Ectrodactyly-ectodermal- present but limited to specific regions, which may be
dysplasia-clefting syndrome  quantified by the number of limbal clock hours
Congenital eryhrokeratodermia involved. The diagnosis is confirmed by impression
Dyskeratosis congenital cytology illustrating the presence of goblet cells,

53

increased cytokeratin 19 (CK19) expression, and circumferential migration of dividing epithelial cells.
reduced CK3/12 expression.6 More recently CK7, However, in cases of sectoral LSCD, often,
mucin1, and mucin5AC have been reported as more conjunctival cells intrude the adjacent area of cornea
specific than CK19 for diagnostic purposes.7 thereby preventing expansion of the remaining
In vivo confocal microscopy (IVCM) and anterior nearby stem cells in the affected area. In these
optical coherence tomography (OCT) are promising circumstances, SSCE is performed where repeated
techniques that may assist in diagnosing and removal of sectoral conjunctiva covering the cornea
quantifying LSCD and guiding therapeutic or bulbar conjunctiva adjacent to the affected area
management. IVCM provides high-resolution images prevents conjunctiva from reaching the limbus and
of anatomical structures at the cellular level. A allow healing of defect with epithelial cells of corneal
number of practical factors limit its use; firstly there is phenotype.14
no consensus on the definitive morphological
appearance of LESCs, surrounding niche cells or Dua et al. reported a series of 10 patients with
goblet cells on IVCM. Secondly, in the presence of a conjunctivalization of cornea which were followed
hazy cornea, the technique is less effective in defining for an average of 7.5 months. Five patients in this
structures due to high degree of backscatter, and group had their conjunctival epithelium removed
finally it requires the prolonged cooperation of the from the corneal surface and allowed to heal from the
patient 8-10. Anterior OCT, and in particular Fourier remaining intact corneal epithelium. In another 4
Domain OCT (FD-OCT), is a more rapid and patients with corneal epithelial defect, the
convenient method of imaging limbal, scleral, and conjunctival epithelium was actively prevented from
conjunctival structures, though, with significantly crossing the limbus by mechanically scraping it off.
lower resolution than IVCM. 3D guided Removal of conjunctival epithelium from the cornea
reconstructions of the limbus can be made and may allowed cells of corneal epithelial phenotype to cover
assist guided limbal biopsy.11 Furthermore, FD-OCT the denuded area with improvement of symptoms and
can be applied in imaging hazy corneas and facilitates vision. 14
intraoperative dissection of fibrovascular pannus.12,13
Conjunctival-limbal autograft (CLAU)
Figure 2: Total LSCD following Alkali burn
In this procedure, 3-4 mm of conjunctiva along with
Management of LSCD limbal epithelium is harvested from fellow eye of the
Currently no medical treatment is available to restore patient. It is imperative to exclude LSCD in the donor
limbal stem cells. After the acute inflammation and eye especially in bilateral chemical injuries where
inciting event is controlled, stem cell transplantation other eye may be subclinically involved. Basti et al.
is the definitive treatment to correct LSCD. noted progressive conjunctivalization in three cases
following conjunctival limbal autograft, probably due
Sectoral sequential conjunctival epitheliectomy to attenuation of limbal stem cell function with time.
(SSCE) Careful case selection is very important aspect of this
It is well-known that any corneal epithelial defect procedure. In their series, pseudopterygium
involving the limbus heals by centripetal as well as formation was seen in one donor eye of a patient with
history of bilateral chemical injury but without
clinical evidence of LSCD. The amount of harvested
tissue should not exceed 6 clock hours for fear of
precipitating LSCD in donor eye. Care is taken to
avoid damage to limbus while dissection.
Buttonholing and dessication of donor tissue should
be avoided.15

.

Keratolimbal allograft (KLAL)

In this technique, the cadaveric donor tissue is
incorporated as part of cornea along with limbal
tissue. Tissue from the youngest possible donor with

54

an upper limit of 50 years is recommended. Surgery Cultivated limbal epithelial transplantation
should be performed within 72 hours as the cells are (CLET)
expected to be more active and vital. 16,17 While
harvesting the donor tissue damage to epithelium In this procedure autologous limbal epithelial stem
should be avoided and a peripheral skirt of adjoining cells are expanded over a carrier tissue (AM mostly)
conjunctiva and partial thickness sclera of about 3-4 in a special explants cell culture system for 2 weeks
mm should be included. If the whole globe is used, a and then transplanted onto the diseased ocular
vaccum trephine may be used centrally, set to a depth surface. Various animal derived products are used in
of 150 microns and the dissection of peripheral cornea xenogenic explants culture system, eg., murine feeder
and limbus may be carried out with a diamond knife. cells, fetal bovine serum, which theoretically carries a
Amniotic membrane has been used as an adjunct to risk of rejection or transmission of prion diseases. To
limbal transplantation as it helps to reconstruct avoid this autologous human serum has been
perilimbal stroma and reduces inflammation and proposed as an alternative constituent in explant
vascularization.18 cultures.

Amniotic membrane transplantation (AMT) Simple limbal epithelial transplantation (SLET)

Role of amniotic membrane (AM) in cases of recent In this technique limbal tissue is divided into small
chemical and thermal injuries is well-established. It pieces and transplanted over amniotic membrane
not only controls active inflammation that is harmful sutured over recipient cornea followed by bandage
for surviving limbal stem cells but also provides contact lens application. SLET enjoys advantage over
stable and quiet ocular surface for stem cell CLET in being a single staged procedure without any
transplantation. Tseng et al. have successfully used extra requirements of stringent laboratory conditions
AMT and mechanical debridement and advocate its to propagate and maintain cellular growth. Sangwan
use to treat patients with partial stem cell deficiency. et al. has reported series of six patients having total
Their study showed that with partial or focal limbal LSCD secondary to ocular burns who underwent
stem cell deficiency, AMT improves both the corneal SLET. All their cases maintained stable corneal
surface and the vision.19 Both fresh and preserved AM epithelial surface by 6 weeks post-operatively and
have been found to function equally well when maintained so during follow-up visits. Visual acuity
transplanted onto the ocular surface. Advantage with improved from worse than 20/200 in all cases to 20/60
preserved AM is that it is well-processed and or better in 66.6% cases without any complications.
thoroughly screened against transmissible diseases They concluded that SLET is an effective modality for
thereby eliminating the slightest risk of disease treating total LSCD secondary to ocular burns.20
transmission that may be associated with fresh AM. In
addition to this 30 preserved grafts can be prepared Keratoprosthesis
from one placenta unlike tissue wastage while using
fresh AM. Dry preserved AM is easily available in Patients having Steven Johnson Syndrome,
market and can be stored at room temperature for 2 to Oculocutaneous Pemphigus, or other autoimmune
5 years unlike cryopreserved AM which requires diseases are poor candidates for stem cell therapy and
stringent storage conditions. corneal transplantation as the severe and long
standing ocular surface inflammation hinders the
AM as a carrier in this instance has several distinct survival of stem cells. Such candidates can be given
advantages over other substrates that have been used. visual outcome with the help of keratoprosthesis.
The basement membrane of AM contains Type IV Boston type I keratoprosthesis and Osteo-odonto
collagen and laminin which plays an important role in Keratoprosthesis have shown satisfactory visual
cell adhesion. Further, it acts as a natural substrate for outcomes in such cases.21
the cell growth and when transplanted gets integrated
onto the corneal surface. It also enables easier
handling during transplantation.

55

REFERENCES woundhealing.BrJOphthalmol.1998;82:1407-1411.
15. Basti S, Mathur U. Unusual intermediate-term
1. Van Buskirk EM. The anatomy of the limbus.
Eye (Lond.). 1989;3:101–108. outcome in 3 cases of limbal autograft
transplantation. Ophthalmology. 1999;106:958-963.
2. Ramos T, Scott D, Ahmad S. An update on ocular 16. Kenyon KR, Tseng SC. Limbal autograft
surface epithelial stem cells: cornea and transplantation for ocular surface disorders.
conjunctiva. Stem Cells Int. 2015;2015:601731. Ophthalmology. 1989; 96:709-722.
17. Croasdale CK, Schwartz GS, Malling JV,
3. Tseng SC. Concept and application of limbal Holland EJ. Keratolimbal allograft:
stem cells. Eye (Lond.). 1989;3:141–157. Recommendations for tissue procurement and
preparation by eye banks and standard surgical
4. Joe AW, Yeung SN. Concise review: identifying technique. Cornea 1999;18:52-58.
limbal stem cells: classical concepts and new 18. Dua HS, Azuara-Blanco A. Allo-limbal
challenges. Stem Cells Transl Med. transplantation in patients with limbal stem cell
2014;3:318–322. deficiency. Br J Ophthalmol. 1999;83:414-419.
19. Tseng SC, Prabhasawat P, Barton K, Gray T,
5. Sangwan VS. Limbal stem cells in health and Meller D. Amniotic membrane transplantation
disease. Biosci Rep. 2001;21:385–405. with or without limbal allografts for corneal
surface reconstruction in patients with limbal
6. Ordonez P, Di Girolamo N. Limbal epithelial stem cell deficiency. Arch Ophthalmol.
stem cells: role of the niche microenvironment. 1998;116:431-441.
Stem Cells. 2012;30:100–107. 20. Sangwan VS, Basu S, MacNeil S,
Balasubramanian D. Simple limbal epithelial
7. Di Girolamo N. Moving epithelia: Tracking the transplantation: A novel surgical technique for
fate of mammalian limbal epithelial stem cells. the treatment of unilateral limbal stem cell
Prog Retin Eye Res. 2015;48:203–225. deficiency. Br J Ophthalmol. 2012;96:931-934.
21. Falcinelli G, Falcinelli B, Taloni M, Rigby H,
8. Zhao J, Mo V, Nagasaki T. Distribution of label- Fullwood NJ, Kinoshita S. Modified osteo-
retaining cells in the limbal epithelium of a odonto keratoprosthesis for treatment of
mouse eye. J Histochem Cytochem. cornealblindness: Long term anatomical and
2009;57:177–185. functional outcomes in 181 cases. Arch
Ophthalmol. 2005;123:1319-1329.
9. Dziasko MA, Daniels JT. Anatomical features
and cell-cell interactions in the human limbal Address for Correspondence :
epithelial stem cell niche. Ocul Surf.
2016;14:322–330. Dr. Monika
Sr. Resident,
10. Castro-Munozledo F. Review: Corneal epithelial RIO, PGIMS,
stem cells, their niche and wound healing. Mol Rohtak-124001(Haryana)
Vis. 2013;19:1600–1613. E-mail : [email protected]
Mobile : 8813023464
11. Funderburgh JL, Funderburgh ML, Du Y. Stem
cells in the limbal stroma. Ocul Surf.
2016;14:113–120.

12. Yamada K, Young RD, Lewis PN, Shinomiya K,
Meek KM, Kinoshita S, et al. Mesenchymal-
epithelial cell interactions and proteoglycan
matrix composition in the presumptive stem cell
niche of the rabbit corneal limbus. Mol Vis.
2015;21:1328–1339.

13. Shortt AJ, Secker GA, Munro PM, Khaw PT,
Tuft SJ, Daniels JT. Characterization of the
limbal epithelial stem cell niche: novel imaging
techniques permit in vivo observation and
targeted biopsy of limbal epithelial stem cells.
Stem Cells. 2007;25:1402–1409.

14. Dua HS. The conjunctiva in corneal epithelial

56

An overview of specular microscopy

Apoorva Goel MBBS, R S Chauhan MS, Ashok Rathi MS,
J P Chugh MS, Vandana MS, Sandhya J MS

Regional Institute of Ophthalmology, PGIMS, Rohtak

Abstract

Specular microscopy can provide a non-invasive morphological analysis of the corneal endothelial cell layer. It is
used to examine the endothelial cell changes from aging, ocular surgical procedures, pharmaceutical exposure
and can also be used to examine the general health of the corneal endothelium. The purpose of this review article
is to discuss the normal and stressed endothelial cell morphology and the clinical applications of specular
microscopy.

Keywords: Specular microscope, cornea, endothelium.

Introduction edges between the flat surfaces are irregular, causing
the light to be reflected in many directions and
Specular microscopy is a non-invasive technique to therefore giving the edges a dark appearance. This is
access the structure and function of the corneal depicted in Figure 1.
endothelium. It can capture the cell images at
different magnifications and calibrations. It enables Fig. 1 : A. Incident light is diffracted through
the study of changes occurring in the cornea under pigment cells, leaving a shadow. B. Reflection from
living conditions at a higher magnification than is
possible with the slit lamp. Primarily, the tissue pigment is mirror-like, giving a bright reflective
examined is corneal endothelium but other structures pattern. C. Reflection from flat, mirror-like
like corneal epithelium, corneal stroma and the endothelium.
crystalline lens can also be examined and evaluated
using specular microscope. Clinical specular Specular microscopy indications
microscopy is a practical tool not only for the cornea 1. Endothelial abnormality on slit lamp : corneal
subspecialist in evaluating donor corneas and corneal
dystrophies but also for all the ophthalmic surgeon in guttae, keratic precipitates, pigmented and
identifying subtle as well as macroscopic changes in inflammatory cells
the cornea prior to surgery. With the development of 2. Any history of: corneal dystrophy, trauma, acute
newer refractive procedures, such as phakic narrow-angle or chronic open-angle glaucoma,
intraocular lenses, intraocular telescopes, new topical uveitis, keratitis, graft rejection
and intraocular drugs and solutions, there is a need for 3. Previous ocular surgery: secondary intraocular
standardization and validation of corneal endothelial lens implantation, corneal transplantation
specular microscopy.1-7 Types of specular microscopes8
Non- contact specular microscope: It uses internal
Optical principle fixation points to provide a more standardized
approach to image central endothelium, mid-
Light striking a surface can be reflected, transmitted, periphery and periphery. Some commercially
or absorbed. Clinical specular microscopy is based on
the principle that the light is reflected specularly
(mirror like), where the angle of reflection is equal to
the angle of incidence. This light is captured by the
specular microscope and the image of interest is
formed. For example, the flat surface of the
endothelial cells are directly reflective, while the

57

available non- contact specular microscopes are: equal side lengths. Polymorphism or
Topcon SP-200P, Konan: ROBO Pachy SP-9000 and pleomorphism refers to a various number of cell
Konan: ROBO CA SP-8800. sides, length and cell shapes. With age the average
Contact specular microscope: Local anesthetic should be cell size distribution area increases and the
used prior to its use and the beam of light is directed cellular pattern becomes pleomorphic. Shapes
through the pupil to ensure the placement of the cone on other than hexagonal confrigation may be seen
the most central portion of the cornea. Some like stretched, scalloped, large round, small
commercially available contact specular microscopes round, square and triangular cells (Figure 3). It is
are:HAIlabs:HAICL-100xyz andTOMEY: EM-1000. important to know that alterations in shape have
There are some commercially available microscopes not been directly related to the physiologic
that can be used as either contact or non- contact by function of cells.
changing the objective lenses like Bio- optics LSM -
2000C and Keeler- Konan 580.

Fig. 2: Specular microscopy machine Fig. 3: Morphological changes in endothelial cells.
TOPCON SP-3000P Arrows indicate the features mentioned.

Corneal morphology on specular microscopy A. Stretched; B. Scalloped; C. Large round; D. Small
round; E. Square; F. Triangular
Endothelium: The normal specular micrograph of
a young person should show a regular endothelial There can be various miscellaneous endothelial
mosaic of hexagonal cells of approximately the structures which are mentioned below and shown
same size with well defined cell boundaries. With in figure 4.
age, endothelial cells become pleomorphic. l Corneal guttae: they are excrescences of
1. Cell borders : in the endothelial cell- aqueous descemet's membrane which can be seen with
humor interface, the light incident on cell borders specular microscope much earlier than with the
is scattered in such a way that it is not taken up by slit lamp. They are small, dark structures, but with
the image forming optical system. As a result, cell time they become larger than individual
borders appear as dark, thin and straight lines. endothelial cells. They can also be seen in the far
With pathology cell borders may appear curved or periphery of young individuals where they are
doubled which is secondary to swelling of called Hassall-Henle warts. The incidence of
individual cells resulting in slight differences in corneal guttae increases significantly with age.
the height of cell borders causing various degrees l Endothelial cilia: it appears as a small intracellular
of incident light scatter. At a common intersection, dark structure with sharp borders on specular
three adjacent cells usually meet at an angle of micrographs.
about 1200. An endothelial cell is normally l Intracellular vacuoles or blebs: they are larger
surrounded by six other cells. Any variation intracellular dark structure which are less distinct
usually represents aging, reparative changes after and is located within the cell.
injury or specific pathology. l Pigment deposits on the endothelial surface:
2. Cell shape: the central endothelial cells of young These are bright structures spanning several
people with normal eyes are hexagonal and endothelial cells with sharp borders indicating
approximately the same size with roughly the that they are very near to the endothelial– stromal
interface .

58

· Cell nucleus: these appear like Intracellular bright periphery and periphery because a regional disparity
structures, the size of which corresponds to the inECDandmorphologyin hasbeenreported
size of the endothelial cell and is associated with
stressed cells especially after corneal Clinical findings on specular microscopy
transplantation.
l Aging- ECD decreases throughout life and Cell
loss is most rapid from birth to the first few years
of life. This decrease in ECD may be due to the
normal enlargement of the globe during early
childhood. After the age of 60 years, ECD
decreases with an average age-related cell loss is
approximately 0.5% per year.9 Polymegathism
and pleomorphism also increase with increasing
age which is depicted in figure 5.

Fig. 4: Miscellaneous endothelial structures. Arrows Fig. 5: Changes in endothelial cell as a function of
indicate features mentioned. age. Note the decrease in cell density as well as
increased cell polymorphism and pleomorphism.
A. Corneal guttae- isolated smooth excrescences.
B. ultiple coalesced excrescences. l Fuchs' endothelial corneal dystrophy – Fuch's
C. and D. intracellular bright structures representing dystrophy is the most common corneal dystrophy
causing vision loss. It's key features are guttae, folds
cell nuclei. in descemet's membrane, stromal edema and
E. Pigmented endothelial deposits. microcystic epithelial edema as shown in figure 6.
F. Dark structures representing endothelial cilia. Corneal guttae are focal accumulations of collagen
G. Intracellular dark structures representing on the posterior surface of descemet's membrane
that are formed by stress or abnormal endothelial
intracellular vacuoles. cells. The evolution of guttae leads to corneal
H. Intercellular dark structures representing invading edema and vision loss secondary to reduced corneal
transparency. Primary guttae have to be
inflammatory cells. distinguished from secondary guttae which are
formed after trauma, inflammation and other
Quantitative interpretation with corneal dystrophies. Specular microscopy reveals
specular microscopy that an individual excrescence (guttae) begins as a
very small structure, much smaller than an
l Corneal edema occurs in between 300 and 700 individual endothelial cell with adjacent endothelial
cells per mm2 cells being normal. Five stages of endothelial guttae
have been described and all five stages can exist in
l Any given intraocular surgical event is associated the cornea at the same time that too in a cornea with
with a cell loss in the range of 0–30% no clinically significant corneal edema. With time,
the excrescence destroy borders of the adjacent and
l Patient should have at least 1000 to 1200 cells per overlying cells making their borders indistinct.10
mm2 to safely undergo most anterior segment Guttae may affect the physiology of cornea in
surgery

l There should be no significant difference in the
ECD between eyes. A difference of more than 280
cells per mm2 is significant

l Cornea with a coefficient of variation greater than
0.40 or the presence of less than 50% hexagonal
cells is abnormal and at increased risk for
postoperative edema

l When evaluating postoperative corneas, it is
important to use multiple images in the central, mid-

59

multiple ways. Their density correlates directly with l Posterior polymorphous corneal dystrophy- it is a
decreased endothelial density and function. Their rare, bilateral and autosomal dominant inherited
presence adds an irregular interface to the visual dystrophy. Specular microscopy show vesicles
pathway, producing glare. Visual degradation due to which have a thick, dark border giving a
guttae may be subjectively noticeable to patients doughnut-like appearance. This structure appears
despity normal snellen acuity measurements and to lie anterior to undistorted endothelial cells,
may precede progressive edematous change. which are more recognizable toward the center of
the lesion and the endothelial cells adjacent to the
structure are distorted and commonly smaller than
normal (Figure 8,9)

Fig. 6: The guttae appear as dark spots Fig. 8: Characteristic 'snail- tracking' seen in
with bright central reflections. posterior polymorphous corneal dystrophy.
Fig. 9: Specular microscopy of posterior
l Penetrating or endothelial keratoplasty along with polymorphous corneal dystrophy.
anterior segment surgery : If there are a significant
number of peripheral endothelial cells and the l Keratoconus: there is elongation of endothelial
guttae are primarily located centrally penetrating cell near the site of rupture. The endothelial cells
or endothelial keratoplasty can be avoided but If are of a more normal size and morphologic
there is complete confluence of guttae in the appearance away from the site of rupture. There
peripheral cornea associated with increased can be presence of dark bodies within an
corneal thickness of over 0.68 microns (µm), a otherwise normal-appearing cell. These dark
combined procedure is indicated. bodies occur less frequently in larger endothelial
cells and are consistent in appearance with blebs
l Iridocorneal endothelial syndrome: it is a group of or vacuoles seen with the electron microscope.11
disorders characterized by abnormal corneal
endothelium that is responsible for variable degree l Glaucoma: Persistently elevated intraocular
of iris atrophy, secondary angle- closure glaucoma pressure results in gradual loss of endothelial cells
in association with peripheral anterior synechiae and progressive loss of endothelial function,
and corneal edema. Slit lamp examination reveals however no morphologic changes despite
the endothelium appearing as fine, hammered elevated pressures has been noted. Endothelial
silver metal. Specular microscopy shows rounding cell loss is not a direct result of increased pressure,
off of cell angle, loss of cellular definition, but rather some other disturbance such as
increased granularity of the intracellular details prolonged low oxygen concentration in the
and hexagonal shape (Figure 7). As the disease aqueous humor as if the pressure is medically
progresses, the endothelial monolayer may no controlled, cell loss is reduced. Studies have
longer be recognizable as a mosaic of cells and a shown varying cell loss associated with various
'reversal appearance' may develop, with black glaucoma procedures like trabeculotomy and tube
central areas and white borders. shunts.

Fig. 7: Specular microscopy of ICE syndrome l Intraocular inflammation: During an acute
showing notice the 'rounded- up' appearance of the episode of anterior uveitis, mononuclear
cells and the small, centric and dark areas of each cell. inflammatory cells penetrate apical junctional
complexes and infiltrate both between endothelial
cells and between endothelial cells and descemet's

60

membrane. Endothelial cells are not generally Fig. 10: During graft rejection episodes
harmed by this process, but in severe cases can intercellular bright bodies, black
become dislodged and float free in the aqueous
humor.12 inflammatory cells, and keratic precipitates
l Cataract extraction with intraocular lens on the endothelial surface can be seen.
implantation: Endothelial cell loss following
uncomplicated phacoemulsification and l Endothelial keratoplasty (EK): Studies have
posterior chamber intraocular lens implantation shown significantly greater cell loss in the first 6
using viscoelastic and modern small-incision months after EK compared to PKP. Although
techniques is quite low, ranging from 6-17 %. there is greater loss at 1 year when compared to
Suture fixation of PCIOL or placement of an IOL PKP, the rate of cell loss begins to level off around
in sulcus appears to be equally traumatic. The 6 months. In regards to wound size, a smaller-
implantation of an anterior chamber lens has sized incision for EK surgery results in greater
been shown to result in higher endothelial cell acute endothelial area damage than larger size (5
loss due to the procedure itself with continued mm) incisions.
endothelial loss which is greater than that of
posterior chamber intraocular lenses.13 l Donor corneas: The minimum donor ECD is
l Refractive surgery: neither LASIK nor PRK established for keratoplasty, for most eye banks is
results in a decreased endothelial density. Laser at 2000 cells/mm2. Studies have reported that
ablation of the stroma within 200 µm of the baseline donor ECD does not predict graft failure
corneal endothelium will result in endothelial 5 years after PKP; however, the 6-month ECD was
structural changes and the formation of the predictive of subsequent failure.
amorphous deposits on descemet's membrane.
Studies have described an increase in central · Intraocular irrigating solutions: Endothelial
endothelial cell density after PRK and LASIK structure and function are best maintained when
due to endothelial migration initiated by the solutions resemble aqueous humor in
discontinuation of contact lens wear and not as a composition. Irrigating solutions not satisfying
result of refractive surgery. the basal metabolic requirements of the corneal
l Penetrating keratoplasty (PKP): 5 to 10 years endothelium demonstrate endothelial changes
after successful PKP for a variety of indications and resultant corneal swelling .
including keratoconus and endothelial
dysfunction disorders, endothelial cell loss l Vitreocorneal contact : in this case some endothelial
progresses at a rate 7 times faster than normal. cells are markedly enlarged and grossly abnormal in
However, between 10 and 15 years, this rate shape while Others contain abnormal bright or dark
substantially slows and approaches the rate of structures within their cell boundaries. Central guttae
loss of endothelial cells in normal aging. Phakic are often seen. Despite the seeming irreversibility of
patients lose significantly more endothelial cells the endothelial changes, clinical reversal of corneal
during PKP than aphakic patients. This finding edema may occur in cases of moderately prolonged
was attributed to the deeper anterior chamber vitreous contact.
and the absence of endothelial trauma from the
lens–iris diaphragm in the aphakic eye. Specular l Blunt trauma: The impact of small,
microscopy can detect early evidence of graft nonpenetrating foreign bodies on the cornea may
rejection not detectable by clinical examination give rise to posterior annular keratopathy which
in high-risk patients. presents as grey rings on the corneal endothelium.
Specular microscopy demonstrate that posterior
annular keratopathy occurring after blunt corneal
trauma consists of disrupted and swollen

61

endothelial cells and the degree of endothelial cell 3. Sperling S. Endothelial cell density in donor
loss appears to be related to the severity of the cornea.Acta Ophthalmol. 1980;58:278-282.
injury.
l Contact lens wear: Both acute and chronic 4. Laing RA, Sandstrom MM, Berrospi AR,
endothelial changes are seen following contact Leibowitz HM. Morphological changes in corneal
lens wear. Within minutes of application of a endothelial cells after penetrating keratoplasty.
contact lens, small dark endothelial blebs occur Am J Ophthalmol. 1976;82:459-464.
that disappear quickly if the lens is removed.
These endothelial blebs reach a maximum size in 5. Cheng H, Sturrock GD, Rubenstein B, Bulpitt CJ.
20–30 minutes from the time the contact lens is Endothelial cell loss and corneal thickness after
placed on the cornea and then gradually decrease intracapsular extraction plus iris clip lens
in size due to the effects of hypoxia or lactate implantations-a randomised controlled trial. Br J
accumulation. Long-term wear of either hard or Ophthalmol. 1977;61:785-790.
soft contact lenses results in increased
polymegathism which is not reversed upon 6. Sugar J, Mitchelson J, Kraff M. The effect of
cessation of lens wear.14 phacoemulsification on corneal endothelial cell
density.Arch Ophthalmol. 1978;96:446-448.
Fig. 11: A. Specular microscopy of a 59 year old male
with no history of contact lens wear (coefficient of 7. Sato T. Studies on the endothelium of the corneal
variation= 22% and hexagonality=73). graft. Jpn J Ophthalmol. 1978;22:114-126.

B. Specular microscopy of a 54 year old male with 8. Olsen T. Non-contact specular microscopy of the
a history of contact lens wear (PMMA) for 34 human corneal endothelium. Acta Ophthalmol.
years (coefficient of variation= 44% and 1979;57:986-998.
hexagonality=43)
9. Laing RA, Sandstrom MM, Berrospi R, Leibowitz
l Diabetes: In diabetes the cell density significantly HM. Changes in corneal endothelium as a
decreases with age. However, no difference in function of age. Exp Eye Res. 1976;22:587-594.
corneal thickness or endothelial permeability has
been described in studies. Diabetic corneas also 10. Laing RA, Leibowitz HM. Oak SS, Chang R,
exhibit increased polymegathism and Benospi AR, Theodore JA. Endothelial mosaic in
pleomorphism and a decreased percentage of Fuchs' dystrophy. Arch Ophthalmol. 1981;99:83-
hexagonality. 84.

References 11. Laing RA, Sandstrom MM, Berrospi R, Leibowitz
1. Bourne WM. Examination and photography of HM. The human corneal endothelium in
keratoconus. Arch Ophthalmol. 1980;58:250-
donor corneal endothelium. Arch Ophthalmol. 256.
1976;96:1799-1800.
2. Bigar F, Schimmelpfennig B, Hurzeler R. Cornea 12. Olsen T. Changes in the corneal endothelium after
guttata in donor material. Arch Ophthalmol. acute anterior uveitis as seen with the specular
1978;96:653-655. microscope. Acta Ophthalmnol. 1980;58:250-
256.

13. Bourne, W. M., and Kaufman, H. E. Cataract
extraction and the corneal endothelium. Am J
Ophthalmol. 1976;82,44-47.

14. Zantos SG, Holden BA. Transient endothelial
changes soon after wearing soft contact lenses.
Am J Optom Physiol Opt. 1977;54:856-858.

Address for correspondence:

Dr.Apoorva Goel
Jr. Resident,
RIO, PGIMS, Rohtak
E- mail: [email protected]
Mobile: 8395975985

62

Floppy Eyelid Syndrome: An Overview

Nisha Bura MBBS, Urmil Chawla MS, DNB, Akanksha Rani MBBS,
Richa Sharma MBBS, Reena Gupta MS, Neebha Anand MS

Regional Institute of Ophthalmology, PGIMS, Rohtak

Abstract

Floppy eyelid syndrome (FES) usually affects middle-aged obese men, presenting as a unilateral or bilateral
chronic papillary conjunctivitis. The upper eyelid is lax, floppy, and easily everted. The laterality corresponds to
the side the patient sleeps on.The diagnosis of FES is made clinically and defined broadly as rubbery, malleable,
and easily everted upper eyelids. It is often associated with patients with high body mass index and obstructive
sleep apnoea.Clinical findings include obesity or eye rubbing, lash ptosis, and, less commonly, blepharoptosis.
Treatment includes eyelid lubrication and taping, lid shields and surgical horizontal eyelid shortening.

Keywords: Foppy eyelid syndrome, papillary conjunctivitis, obstructive sleep apnoea, eye shields.

Introduction direct mechanical irritation.
l Meibomian gland dysfunction and atrophy:found
Floppy eyelid syndrome is a disease whose most
prominent features often include floppy upper eyelids in association with FES5.
that can be easily everted, as well as papillary
conjunctivitis.1 It is typically found in overweight, Clinical Features
middle aged men, but has been described in a broad
range of patients. Presenting symptoms
Floppy eyelid syndrome is thought to revolve around l Unilateral or bilateral chronic eye irritation and
the upregulation of elastin degrading enzymes, as well
as mechanical factors. These can cause instability of burning
the eyelid scaffold, resulting in the malposition of the l Tearing
eyelid.2 l Ropy, mucoid discharge (usually worse in the

Etiopathogenesis morning)
l Decreased vision (if there is an associated
Mechanical Injury: Patients who sleep on one side
more than the other side tend to have more severe keratopathy)
changes on that side. This suggests mechanical injury l Daytime somnolence
as the primary cause of the papillary conjunctivitis. l Morning headaches
l History of loud snoring or a diagnosis of OSA:
Sleep History
this requires the patient to sleep on one side or in a
prone position with the face in the pillow. Use of l Usual sleep position on one side or prone with the
an eye shield to protect the eyelids during sleep face in the pillow
often can improve the patient's signs and
symptoms. l Frequent episodes of waking up during the night
l Keratoconus: association with keratoconus
suggests mechanical irritation from eye rubbing Past ocular history
as a contributing factor 3.
l Chronic conjunctivitis: Due to poor apposition of l Chronic papillary conjunctivitis
the lax upper eyelid to the globe with inadequate l Chalazia or hordeola
spreading of the tear film4. This condition leads to l Keratoconus
corneal and conjunctival compromise, rather than l Contact lens use
l Intermittent symptoms, which may be seasonal

due to associated allergy

Past medical history

l Acne rosacea
l Psoriasis
l Hypertension
l Congestive heart failure (CHF)

63

l Obstructive sleep apnea (OSA) Histopathology
External ophthalmic examination
l Lax upper eyelid (Figure 1) that is easily everted l Light microscopy of the surgical specimens show
chronic conjunctival inflammation, papillary
when pulled superiorly toward the eyebrow conjunctivitis, and meibomian gland
(Figure 2) abnormalities, including granuloma formation.
l Soft and rubbery tarsal plate that can be folded
upon itself l Verhoeff's modified elastin stain demonstrates a
l Laxity that can be quantified through measurement marked decrease in the amount of elastin fibers in
of anterior eyelid distraction 6. tarsus from patients with floppy eyelid syndrome
l Atrophic tarsal plate compared with controls.
l Stringy, mucoid conjunctival discharge
l Punctate corneal epitheliopathy and mucous l Immunohistochemical staining for elastin also
strands in the tear film and fornices (possibly) show a marked decrease of tarsal elastin in floppy
l Eyelash ptosis (Figure 3) with loss of lash eyelid patients.
parallelism (ie, lashes point downward toward
the cornea and curve in different directions) 7. l Electron microscopy demonstrates a reduced
amount of tarsal elastin in floppy eyelid
Fig.1: Lax upper eyelid syndrome.

Fig. 2 : Lax upper eyelid that is easily everted Differential Diagnosis for Floppy Lid Syndrome

Fig. 3: Eyelash ptosis with loss of lash parallelism l Chronic conjunctivitis, Vernal conjuncitivitis,
Diagnostic criteria have been proposed and include giant papillary conjunctivitis, superior limbic
objective measurements of clinical findings such as keratoconjunctivits, toxic conjunctivitis
horizontal distraction, snapback, and vertical lid pull.
Regardless of the specific criteria considered, the l Canaliculitis, Involutional ectropion
diagnosis of FES should be considered when a patient l Nasolacrimal duct obstruction
presents with any of the symptoms in the setting of l Sebaceous cell carcinoma
loose, easily everted upper eyelids.
Management

The treatment of FES depends on both the severity of
the patient's symptoms and, if present, the treatment
of patients' underlying OSA.

Conservative Medical Therapy

l Topical application of a lubricating or antibiotic
ophthalmic ointment in the affected eye is
indicated for mild corneal or conjunctival
abnormalities. Erythromycin ophthalmic
ointment may be applied 2-4 times daily for
superior punctate keratitis. Lubricating
ophthalmic ointment may be applied at bedtime.

l If meibomian gland dysfunction is suspected,
trial of an oral tetracycline (eg, such as
doxycycline 100 mg once or twice daily for 6-12
wks) may be appropriate.

l Eye shields prevent nocturnal eversion of the
lids and often results in a rapid resolution of the
symptoms. Patient should be instructed to tape
the eyelids closed and wear an eye shield while
asleep to protect the conjunctiva and the eye
from rubbing on the pillow.

64

Surgical Intervention l Special shields or a mask may have to be fitted to
shield the eye from mechanical irritation.
l Upper and lower eyelids can be tightened at the
lateral canthus by lateral tarsal strip procedure8. Conclusion

l Horizontal shortening of the lateral upper eyelid More complete understanding of the pathogenesis of
can be achieved by performing a full-thickness floppy eyelid syndrome will lead to improved medical
resection of the lateral one fourth to one third of therapies, better surgical outcomes, and prevention of
the eyelid margin.9 This can be accomplished by the condition. The exact pathogenesis of the disorder,
means of a vertical full-thickness resection up to however, remains unknown. Ocular surface
an eyelid crease incision. Ptosis repair or lubrication and surgery to address the horizontal
blepharoplasty can be performed at the same eyelid laxity remain the mainstay of treatment.
time. The disparity in skin length can be
managed with a vertical Burrow triangle References
directed toward the brow at the lateral extent of 1. Pham, Thu T, Perry, Julian D. Floppy eyelid
the eyelid crease incision.
syndrome. Current Opinion in Ophthalmology.
l A modified curvilinear back-tapered full- 2007;18(5):430–433.
thickness resection with an advancement flap at 2. Ezra DG, Beaconsfield M, Collin R. Floppy
the lateral upper eyelid. eyelid syndrome stretching the limits. Survey of
Ophthalmology.2010;55:35-46.
l In cases with more medial laxity, horizontal 3. Goldberg R, Seiff S, McFarland J, et al. Floppy
shortening of the medial upper eyelid can be eyelid syndrome and blepharochalasis. Am J
achieved by performing a laterally displaced Ophthalmol. 1986;102(3):376-381.
pentagonal full-thickness resection in the medial 4. Gonnering RS, Sonneland PR. Meibomian
one third of the eyelid, lateral to the superior gland dysfunction in floppy eyelid syndrome.
punctum. Any brow ptosis, dermatochalasis, Ophthal Plast Reconstr Surg. 1987; 3(2):93-99.
blepharoptosis, or ectropion can be repaired at 5. Jordan DR, Anderson RL. The lateral tarsal strip
the same time. revisited. The enhanced tarsal strip. Arch
Ophthalmol. 1989;107(4):604-606.
l In repairing ptosis of a lax upper eyelid, the 6. Iyengar SS, Khan JA. Quantifying upper eyelid
eyelid often must be tightened to achieve the laxity in symptomatic floppy eyelid syndrome
desired contour. by measurement of anterior eyelid distraction.
Ophthal Plast Reconstr Surg. 2007;23(3):255.
Complications of surgical treatment of FES include : 7. Dutton J. Surgical management of floppy eyelid
Ÿ Poor wound healing syndrome. Am J Ophthalmol. 1985;99(5):557-
Ÿ Unacceptable eyelid height or contour 560.
Ÿ Undercorrection or overcorrection 8. Paciuc, Miguel, Maria Elena Mier. A woman
with the floppy eyelid syndrome. Am J of
Monitoring Ophthalmology. 2013; 32(9):1232-1236.
9. Moore MB, Harrington J, McCulley JP. Floppy
Patients treated for FES should be observed every 3-7 eyelid syndrome Management including
days initially until any keratitis is resolved; after the surgery. Ophthalmology. 1986; 93(2):184-188.
first week, they may be observed every 2-6 weeks, as
necessary. Address for correspondence:
l Antibiotic ophthalmic ointment (eg, erythromycin)
Dr. Nisha Bura
is prescribed postoperatively 2-4 times a day along House no.958,Sector -7,Kurukshetra-136118, Haryana
suturesandin theeyefor 1 week. E-mail : [email protected]
l Lubricating ophthalmic ointment in the eye at Mobile: 8572014958
bedtime can be continued, as needed.
l Patients with FES should be encouraged to
refrain from sleeping with the face in the pillow,
to avoid rubbing the eyes, and to lose weight if
obese.

65

A rare case of typical Devic's disease

Sahebaan Sethi MS, DNB, Aditya Sethi DOMS,
Vaibhav Sethi DNB

Arunodaya Deseret Eye Hospital, Gurugram

Abstract

We report a rare case of a 26 years old patient with sudden drop in vision and lower limb weakness. He had left eye
disc edema and abnormal CNS function. On investigation, he was diagnosed to have optic neuritis with associated
transverse myelitis- a syndrome referred to as Device's disease also called as Neuromyelitis Optica. Patient was
successfully managed with corticosteroids.

Keywords: Neuromyelitis optica, devic's disease, optic neuritis, transverse myelitis.

Introduction distention in the abdomen since a day before.
On General Examination, patient was conscious and
Neuromyelitis optica (NMO) is an immune-mediated oriented. He had no pallor, icterus, clubbing,
inflammatory disorder of the central nervous system. cyanosis, lymphadenopathy or pedal edema. His
It is characterized by concurrent inflammation and vitals were normal. Systemic Examination of
demyelination of the optic nerve (optic neuritis [ON] cardiovascular system had no murmurs. S1 and S2
and the spinal cord (myelitis).1 It occurs when the were heard. He had normal vesicular breath sounds on
body's immune system reacts against its own cells in examination of respiratory system. On per abdomen
the central nervous system, mainly in the optic nerves examination, he had suprapubic distention and
and spinal cord, but sometimes in the brain. tenderness with mild splenomegaly. Bowel sounds
The cause of neuromyelitis optica is usually heard and normal.
unknown, although it may sometimes occur after an Examination of the central nervous system-
infection, or it may be associated with another GCS- 15/15
autoimmune condition. Neuromyelitis optica is often Power : Right lower limb- 3+/5
misdiagnosed as multiple sclerosis (MS) or perceived
as a type of MS, but NMO is a distinct condition.2 Left lower limb-4+/5
The condition is extremely rare, affecting between Both upper limbs- 4+/5
0.05 and 4.4 in every 100,000 people worldwide.3 It is Sensation : Bilaterally reduced below the sternum.
more common in people above 40 years of age. Altered sensation D10-D11.
Neuromyelitis optica may cause blindness in one or Plantar reflex : Right–exaggerated;left-reduced.
both eyes, weakness or paralysis in the legs or arms. Deep tendon reflexes :Brisk more in lower limb.
Neuromyelitis optica episodes may be reversible, but Tone : both upper & lower limb-normal
can be severe enough to cause permanent visual loss
and problems with walking. OCULAR EXAMINATION
Here we discuss a typical case of Devic's disease, a

rare occurrence and how it can be tackled.

Case Report
A 26 year old male presented with complaints of

sudden drop in vision & pain on movement in left eye

for few hours. He also gave history of weakness in

right lower limb since 3 days followed one day later

by weakness in left lower limb. In addition, he

suffered from fever with chills & rigors and loss of

bladder control for 2 days associated with pain and

66

Fig. 1 Left eye disc edema and right color vision, loss of spinal cord function leading to
eye normal on fundus photography. muscle weakness, spastic paraparesis or tetraparesis,
reduced sensation, loss of bladder/bowel control.
Fig. 2 MRI brain suggestive of demyelination at Optic nerve involvement can be unilateral or bilateral 4
T7-T9 level with left optic neuritis. and is a painful condition worsened by movement with
decreased visual acuity in the affected eye and is the
CSF oligoclonal bands were positive. NMO most common initial symptom.5 Half of the patients
antibodies were positive. lose functional vision within 5 years of onset.6
On the basis of clinical examination and Myelitis involvement classically involve more than
investigations, a diagnosis of Acute transverse three spinal cord segments. 4
myelitis with left optic neuritis also known as Devic's Currently three supposed causes of the disease are:
disease was made. 1. A first subset of patients is anti-AQP4+. In them,
The patient was immediately treated with high dose
intravenous steroids intravenous methylprednisolone the cause of the neuromyelitis optica
1gm/day for 3 days followed by tapering dose of oral (understood as the syndrome) is an autoimmune
steroids 1mg/kg body weight over 3 weeks. The aquaporin-4 channelopathy, due to these specific
patient's visual function recovered fully at 1 month autoantibodies.7 In these cases, astrocytes are the
follow up. victims of the autoimmune attack.
Discussion 2. A second subset of patients is anti-MOG+. In
Devic's disease is an autoimmune inflammatory them the cause is an anti-MOG associated
disorder wherein patient's own immune system- encephalomyelitis.8
antibodies called NMO-IgG attack protein of nervous 3. In a third subset of patients, NMO is due to the
system cells called aquaporins in the cell membrane presence of anti-neurofascin (anti-NF) auto-
of astrocytes. This produces optic neuritis and antibodies which damage the Ranvier nodes of
myelitis. Symptoms include loss of vision or the neurones.9 These antibodies are more related
decreased visual acuity, visual field defect, loss of to the peripheral nervous demyelination, but they
were also found in NMO.10
The etiology is unknown. It is said to have been
associated with other autoimmune diseases like
collagen vascular disease, autoantibody syndrome,
infections like varicella zoster ,Ebstein barr virus,
human immunodeficiency virus and exposure to
clioquinol & antituberculosis drugs.
The Mayo Clinic proposed a revised set of criteria for
diagnosis of Devic's disease in 2006. Those new
guidelines require two absolute criteria plus at least
two of the three supportive criteria.11
l Absolute criteria: 1) Optic neuritis 2) Acute
myelitis
l Supportive criteria: 1) Brain MRI not meeting
criteria for MS at disease onset. 2) Spinal cord
MRI with continuous T2-weighted signal
abnormality extending over three or more
vertebral segments, indicating a relatively large
lesion in the spinal cord. 3) NMO-IgG
seropositive status (The NMO-IgG test checks
the existence of antibodies against the aquaporin
4 antigen.)

67

Treatment Conclusion
Currently, there is no cure for Devic's disease, but Devic's disease is a rare encounter which when
symptoms can be treated. Some patients recover, but diagnosed correctly and intervened timely can have a
many are left with impairment of vision and limbs, good prognosis. This case also emphasizes the need
which can be severe. for good history taking and thorough examination
including general and systemic.
Attacks
Attacks are treated with short courses of high dosage References
intravenous corticosteroids such as methylprednisolone 1. Zarei, Sara et al. “Comprehensive review of
IV. Plasmapheresis can be an effective treatment12
when attacks progress or do not respond to neuromyelitis optica and clinical characteristics
corticosteroid treatment. Clinical trials for these of neuromyelitis optica patients in Puerto Rico.”
treatments are very limited. Surgical neurology international (Vol. 9) 242. 3
Dec. 2018
Secondary prevention 2. Wingerchuk DM, Lennon VA, Lucchinetti CF, et
al. The spectrum of neuromyelitisoptica. Lancet
Treatment in order to prevent relapses within Neurol. 2007;6:805–815.
neuromyelitis optica is generally employed, though 3. Etemadifar M, Nasr Z, Khalili B, Taherioun M,
the exact duration of prophylaxis is still debated.13 In Vosoughi R. Epidemiology of neuromyelitis
2019, eculizumab became the first and only optica in the world: A systematic review and
medication to be approved by the FDA for the meta-analysis. Mult Scler Int . 2015:1-8.
treatment of neuromyelitis optica spectrum disorder 4. Masters-Israilov A, Robbins MS ;Headache in
in adults who are AQP4 antibody positive.14 Other Neuromyelitis Optica.; Curr Pain Headache Rep.
immunosuppressant treatments that are used include 2017Apr; 21(4):20.
azathioprine (Imuran) plus prednisone, mycophenolate 5. Morrow MJ, Wingerchuk D ;Neuromyelitis
mofetil plus prednisone, mitoxantrone, intravenous optica.; J Neuroophthalmol. 2012 Jun; 32(2):154-
immunoglobulin (IV IG), and cyclophosphamide.15 166.
B-cell depletion, which indirectly reduces the 6. Wingerchuk DM, Hogancamp WF, O'Brien PC,
antibody levels, has been tried16 with the monoclonal Weinshenker BG; The clinical course of
antibody rituximab, showing good results.17 neuromyelitis optica (Devic's syndrome).;
Several other disease modifying therapies are being Neurology. 1999 Sep 22; 53(5):1107-1114
tried. In 2007, Devic's disease was reported to be 7. Pittock SJ, Lucchinetti CF. "Neuromyelitis optica
responsive to glatiramer acetate18 and to low-dose and the evolving spectrum of autoimmune
corticosteroids.19 Use of Mycophenolate mofetil is aquaporin-4 channelopathies: a decade later".
also currently under research.20 Annals of the New York Academy of Sciences.
Hematopoietic stem cell transplantation (HSCT) is 1366 (1): 20–39.
sometimes used in severe cases of NMO. Currently 8. Spadaro M, Gerdes LA, Mayer MC, Ertl-Wagner
available data suggest that this procedure can reduce B, Laurent S, Krumbholz M, Breithaupt C, Högen
inflammatory activity in to short term, but a clear T, Straube A, Giese A, Hohlfeld R, Lassmann H,
majority of the patients will relapse within 5 years.21 Meinl E, Kümpfel T. "Histopathology and clinical
Prognosis of this disease is fairly good. In course of MOG-antibody-associated
Monophasic devic's disease, there is a single attack encephalomyelitis". Annals of Clinical and
and permanent remission. On the other hand, 85% Translational Neurology. 2 (3): 295–301
patients have a relapsing form of disease with 9. Jun-ichi Kira, Ryo Yamasaki, Hidenori Ogata,
repeated attacks. Relapses usually occur early, with Anti-neurofascin autoantibody and demyelination,
about 55% of patients having a relapse in the first year Dec 2018, Neurochemistry International, doi :
and 90% in the first five years.22 https://doi.org/10.1016/j.neuint.2018.12.011

68

10. Kun Jia et al., Anti-neurofascin-155 antibody- of the Neurological Sciences. 372: 92–96.
positive neuromyelitis optica spectrum disorders, 18. Gartzen K, Limmroth V, Putzki N . "Relapsing
Journal of Neurological Sciences, January 16,
2019. neuromyelitis optica responsive to glatiramer
acetate treatment". European Journal of
11. Wingerchuk DM, Lennon VA, Pittock SJ, Neurology. 14 (6): e12–3.
Lucchinetti CF, Weinshenker BG (May 2006). 19. Watanabe S, Misu T, Miyazawa I, Nakashima I,
"Revised diagnostic criteria for neuromyelitis Shiga Y, Fujihara K, Itoyama Y. "Low-dose
optica". Neurology. 66 (10): 1485–1489. corticosteroids reduce relapses in neuromyelitis
optica: a retrospective analysis". Multiple
12. Wingerchuk, Dean (2006). "Neuromyelitis Sclerosis. 13 (8): 968–974.
Optica (Devic's Syndrome)" . 2006 Rare 20. Montcuquet A, et al. "Effectiveness of
Neuroimmunologic Disorders Symposium. mycophenolate mofetil as first-line therapy in
Archived from the original (PDF) on 2006-09-25. AQP4-IgG, MOG-IgG, and seronegative
neuromyelitis optica spectrum disorders".
13. Kimbrough, Dorlan J et al: "Treatment of Multiple Sclerosis. 23 (10): 1377–1384.
neuromyelitis optica: Review and 21. Burman J, Tolf A, Hägglund H, Askmark H
recommendations". Multiple Sclerosis and (February 2018). "Autologous haematopoietic
Related Disorders. 1 (4): 180–187. stem cell transplantation for neurological
diseases". Journal of Neurology, Neurosurgery,
14. Commissioner, Office of the (27 June 2019). and Psychiatry. 89 (2): 147–155.
"FDA approves first treatment for neuromyelitis 22. Wingerchuk DM (May 2006). "Neuromyelitis
optica spectrum disorder, a rare autoimmune optica". International MS Journal. 13 (2): 42–50.
disease of the central nervous system". FDA.
FDA. Retrieved 28th June, 2019. Address for correspondence:

15. Weinstock-Guttman B, Ramanathan M, Lincoff Dr. Sahebaan Sethi
N, Napoli SQ, Sharma J, Feichter J, Bakshi R Arunodaya Deseret Eye Hospital,
(July 2006). "Study of mitoxantrone for the Sector 55, Gurgaon.
treatment of recurrent neuromyelitis optica E-mail:[email protected]
(Devic disease)". Archives of Neurology. 63 (7): Mobile: 9600175053
957–963.

16. Matiello M, Jacob A, Wingerchuk DM,
Weinshenker BG (June 2007). "Neuromyelitis
optica". Current Opinion in Neurology. 20 (3):
255–60.

17. Evangelopoulos ME, et al: "Treatment of
neuromyelitis optica and neuromyelitis optica
spectrum disorders with rituximab using a
maintenance treatment regimen and close CD19
B cell monitoring. A six-year follow-up". Journal

69

Welder's Maculopathy- A Case Report

Nikita Arora MS, Praveen Arora MS

Dr. Praveen Eye Hospital & Research Centre,Sirsa, Haryana

Abstract

Electrical welding arcs emit an intense and extended spectrum of radiation ranging from Infrared (IR)to
Ultraviolet (UV) radiation. UV and far-IR radiation are absorbed by the cornea and the lens, whereas VL, near-IR
radiation and intensive near-UV emissions penetrate deep into the retina. If sufficient in intensity, they may cause
thermal or photochemical retinal damage, which may be permanent and sight threatening. High resolution
spectral – domain Optical Coherence Tomography (SD-OCT) provides a fast, simple and noninvasive method to
assess retinal structures at microscopic level.A case of welder's maculopathy with fundus findings and OCT is
reported .

Keywords: Welder's maculopathy, electrical welding, photo-ophthalmia, phototoxic maculopathy.

Introduction radiation and the availability of protective measures.6
As these retinal changes are typically small, they are
Electromagnetic radiation ranges between 100 nm difficult to identify and document clinically. The use
and 1 mm of wavelength and is commonly known as of Optical Coherence Tomography, Multifocal
“Optical Radiation” 1. Optical radiation is further Electroretinography and Micro-perimetry have been
divided into Ultraviolet light (UV), Visible light (VL) reported as effective methods for documenting retinal
and Infrared light (IR), which again is subdivided in layer changes seen with this type of injury.7 High
IR-A, IR-B and IR-C. 2 resolution spectral – domain Optical Coherence
Tomography (SD-OCT) provides a fast, simple and
Phototoxic macular degeneration is seen in 0.14% of noninvasive method to assess retinal structures at
ophthalmologic patients. As many as 15% of patients microscopic level.
with phototoxic maculopathy reportedly are welders.3
Photic retinopathy (Retinal phototoxicity) can occur Case Report
when prolonged light exposure overwhelms retinal
defenses against oxidative damage. Retinal A 35-year-old male job hopper came with a chief
phototoxicity is the primary cause of solar as well as complaint of reduced vision in both eyes for past 1
welder's maculopathy.4 Such damage largely occurs in year. Diminution of vision was insidious in onset and
wavelength range of 400–500 nm.3 progressed gradually.
Welding arc maculopathy seems to be rather a sequel There was no past history of any ocular disease,
of occupational accidents and negligence of safety trauma, drug intake, systemic illness or gazing
regulations.3 Most reported cases of welder's solar/lunar eclipse with naked eye. Patient was an
maculopathy are caused by inadequate eye protective occasional drinker and denied smoking. There was no
filters, or by novice welders temporarily lifting or significant family history of any ocular disorders. On
looking around a shaded visor while welding.5 The further enquiry about patient's occupation, it was
prevalence and seriousness of the illness depend both found that patient had been working as a welder for
on the intensity and characteristics of emitted past 7 years and did not use eye protection multiple
times. He changed his profession several times as he
could not focus while welding due to black spot
(scotoma) in his central field. At the time of
presentation, he was working as a painter.
On systemic examination his vitals were stable, On

70

ocular examination, his best corrected visual acuity in following-
right eye was 6/9, N8 and in left eye 6/12, N8. l Photo-ophthalmia (Welder's Flash).
Anterior segment examination was unremarkable. l Keratoconjunctivitis.
Intraocular pressure was 10.2 mm of Hg in right eye & l Cataracts caused by UV radiation.
9.4 mm of Hg in left eye. Fundus examination showed l Phototoxic maculopathy (Welder's maculopathy)
a round dark reddish foveal lesion with yellow spots The retina is susceptible to at least 3 different forms of
and obscure boundary, the remaining retina and disc
appeared normal. SD-OCT of both eyes showed a light damage—thermal, mechanical, and
distinct discontinuity and defect of Ellipsoid Zone
(EZ) and Interdigitation Zone (IZ) with an intact photochemical. The type of retinal damage depends
External Limiting Membrane (ELM) and Retinal
Pigment Epithelial (RPE) layer characteristic of on wavelength, energy level, duration of exposure,
Welder's Maculopathy. and degree of pigmentation.10
Retinal lesions induced by arc welding have been
Fig.1: Fundus picture (BE) showing round dark
reddish foveal lesion with yellow spots and called phototoxic maculopathy', 'retinitis photoelectrica',
obscure boundary
“photic maculopathy” and “macular photoinjury', all
Fig.2: OCT of both eyes showing a distinct these definitions refer to identical pathology.6
discontinuity and defect of EZ and IZ Welder's maculopathy usually occurs in young

characteristic of Welder's Maculopathy apprentice welders who are at increased risk of retinal
Discussion
Electrical welding arcs emit an intense and extended injury because of their presumably clear ocular media
spectrum of radiation ranging from Infrared (IR)to and occupational inexperience.2,4,12
Ultraviolet (UV) radiation. UV and far-IR radiation The possible theories for the mechanism underlying
are absorbed by the cornea and the lens, whereas VL,
near-IR radiation and intensive near-UV emissions welder's retinal damage include a phototoxic reaction
penetrate deep into the retina. If sufficient in intensity,
they may cause thermal or photochemical retinal between visible light and the phospholipids of retinal
damage, which may be permanent and sight
threatening.8,9,10,11 pigments which generates reactive oxygen
There are a variety of ocular changes produced by an intermediates.13
electric arc, apparently dependent on wavelength and Acute presentation of phototoxic trauma includes de-
exposure dose .12
Occupational welders may present with the pigmentation of the RPE and oedematous greying of

the macula, while there is secondary involvement of
the other retinal layers.3
Paracentral or central scotoma, and macular edema

have been found in cases of relatively mild welder's

maculopathy, but severe cases are characterized by

sub retinal scarring, hemorrhage, neovascularisation,

and macular detachment. Most welders'

maculopathies were reported to be reversible or

recover in a few months to a year, but some cases show

persistent retinal lesions that evolve to retinal atrophy
and visual impairment.14 As these retinal changes are

typically small, they are difficult to identify and

document clinically. The use of Optical Coherence

Tomography, Multifocal Electroretinography and

micro-perimetry have been reported as effective

methods for documenting the retinal layer changes
seen with this type of injury7
Despite the low incidence and small anatomical

defect associated with this injury type, the impact on
visual function should not be underestimated .7

71

Preventive strategies should include good welding 7. Vu k i c e v i c M , H e r i o t W. P h o t o t o x i c
equipment, environmental background lighting, eye maculopathy associated with arc welding:
protection and training of workers. Workers should be clinical findings and associated functional vision
adequately informed about the danger of welding too impairment. Clin Exp Ophthalmol.
close to the eyes and of looking round the side of the 2008;36:695-697.
visor, even for a very short period. Workers should be
advised to avoid ingestion of photosensitizing drugs, 8. Mahindrakar A, Toshniwal S, Doongerwala MI,
such as hydrochlorothiazide, furosemide, allopurinol, Anthony H. Spectralis optical coherence
benzodiazepines and fluphenazine, while welding. tomography findings in Welder's maculopathy.
Early diagnosis of radiation retinopathy may be useful Ind J Ophthalmol. 2013;61(5):238–240.
in reducing macular oedema by photocoagulation
treatment, so improving vision in eyes with welders' 9. Jabbarpoor Bonyadi MH. Early and late spectral
maculopathy.6 domain optical coherence tomography features
of acute welding maculopathy. J Ophthalmic Vis
Conclusion Res. 2013; 8(4):391–392.

Welder's maculopathy is an important occupational 10. Yang X, Shao D, Ding X, Liang X, Yang J, Li.
health hazard of arc welding, and this should be Chronic phototoxic maculopathy caused by
brought to the notice of all workers by Health and welding arc in occupational welders. Can J
Safety Inspectors under Occupational Health and Ophthalmol. 2012 : 47(1):45–50.
Safety Act.
11. Naidoff M A & Sliney D H. Retinal injury from a
References welding arc.Am J Ophthalmol 1974;77: 663-68
1. Leccese, F & Salvadori, G & Casini, M &
12. Britain G P H. Retinal burns caused by exposure
Bertozzi, M. Artificial Optical Radiation (AOR) to MIG-welding arcs: report of two cases. Br J
due to lighting sources in indoor workplaces. Ophthal. 1988;172: 570-575.
14th CIRIAF National Congress 2014 ,Vol 4 (1-
5). 13. Ambati J, Ambati BK, Yoo SH, Ianchulev S,
2. Fich M, Dahl H, Fledelius H, Tinning S. Adamis AP. Age related macular degeneration:
Maculopathy caused by welding arcs. A report etiology, pathogenesis and therapeutic
of3 cases.ActaOphthalmol. 1993;71(3):402–404. stratagies. Surv Ophthalmology. 2003; 48, 257-
3. Maier R, Heilig P, Winker R, Neudorfor B, 293.
Hoeranter R. Hugo Ruediger Welder's
maculopathy? Int Arch Occup Environ Health. 14. Kim EA, Kim BG, Yi CH, Kim IG, Chae CH,
2005;78: 681–685. Kang SK. Macular degeneration in an arc
4. Mainster MA, Turner PL. Retinal injuries from welder. Ind Health. 2007;45:371-373
light: mechanisms, hazards and prevention. In:
Ryan SJ, Hinton DR, Schachat AP, Wilkinson P, Address for correspondence:
eds. Retina. 4th ed, Vol 2. London: Elsevier
Publishers; 2005. Dr. NikitaArora
5. LucasR, HarperA, McCombeM, MainsterM, Dr. Praveen Eye Hospital & Research Centre,
SilneyD, Zamir E. Optical coherence Sirsa-125055, Haryana.
tomography findings in welder's maculopathy. E-mail: [email protected]
Retin Cases & Brief Rep. 2007; 1: 169–171. Mobile: 9729439223
6. Magnavita N. Photoretinitis: an underestimated
occupational injury? Occup Med (Lond.).
202;52:223-235.

72

Congenital lacrimal fistula: a peculiar case

AK Khurana MS*, Sonakshi Sehrawat MBBS*,
Urmil Chawla MS, DNB**, Saumya Sharma MBBS*

*Department of Ophthalmology, SGT Medical College, Hospital and Research, Budhera, Gurugram
**Regional Institute of Ophthalmology, Pt. B.D.Sharma, PGIMS, Rohtak

Abstract

Congenital lacrimal fistula is a rare developmental abnormality of the lacrimal system. They are usually
unilateral but bilateral cases have been reported. Typical location of congenital lacrimal fistula is inferonasal to
the medial canthal angle. Patient is generally asymptomatic, however some cases are symptomatic and the
clinical presentation is in the form of epiphora and mucus discharge from the punctum or fistula orifice. We report
an unusual case of congenital lacrimal fistula that was managed successfully by surgical intervention.

Keywords: nasolacrimal duct, fistula, epiphora, regurgitation.

Introduction also nasolacrimal obstruction. Recommended course
of treatment is surgical if fistula is symptomatic.
Congenital lacrimal system anomalies other than Dacryocystorhinostomy with common canalicular
nasolacrimal duct obstruction are rare. Congenital dissection, fistula excision, for all patients with
lacrimal fistula being one of them, has an estimated symptomatic congenital lacrimal fistulas to facilitate
incidence of one in 2,000 live births.1 Congenital outflow and to avoid common canalicular
lacrimal fistula was first described by Rasor2 in 1675. obstruction is treatment of choice.If needed in
The fistula is an epithelial lined tract that connects the certain cases canalicular intubation can also be
skin to the common canaliculus, lacrimal sac or done.5
nasolacrimal duct.(Figure1) In some cases the tract
ends blindly in the sub-cutaneous tissue near the Case Report
lacrimal sac. Most are unilateral and are located A 25 years old female presented in the OPD with
inferonasal to the medial canthus.2-4 complaint of watering from right eye which was
intermittent (on and off throughout the day) and was
Fig.1: Diagrammatic representation of normal associated with presence of mucopurulent discharge
anatomy (left), fistula arising from the common from an orifice close to the right medial canthus since
canaliculus (centre) and from the lacrimal sac early childhood. No history of pain, redness,
photophobia, blurring of vision, swelling on eyelids
(right) was reported. There was no history of trauma or
previous ocular surgery. Ophthalmic examination
These fistulae may be asymptomatic and therefore revealed uncorrected visual acuity of 6/6 in both eyes.
overlooked for some time after birth. In some cases it External inspection showed an orifice of about 1mm
may be difficult to ascertain whether a fistula has been on the skin 2mm medial and below the lower puncta.
present since birth and remained unnoticed or has (Figure 2) Slit lamp examination of the anterior and
arisen later as a result of infection or surgical posterior segment were unremarkable. ROPLAS (
intervention. Symptoms may be of tearing or regurgitation on pressure over lacrimal sac area) was
mucopurulent discharge from the ostium of the fistula positive with mucopurulent discharge from opposite
or from the eye or both depending on whether there is puncta and from fistula.

Fig. 2: Presence of orifce of fistula medial and
below the lower puncta

73

Syringing of right eye showed regurgitation of Discussion
mucopurulent discharge from the external orifice of
fistula as well as from opposite puncta in right Congenital lacrimal fistula are uncommon
eye.Syringing of left eye was patent. Fluorescein dye developmental anomalies which are usually unilateral
disappearance test was grade +3 (wide bright but familial cases are reported to be bilateral.6-8 It is
fluoroscein strip retained)indicating inadequate more likely that such fistulae represent an outgrowth
drainage of dye. Dacryocystography (DCG) from the common canaliculus rather than an ingrowth
confirmed blockage in nasolacrimal duct (Figure 3). from the surface. These findings are in contrast to
No pathology was seen on endoscopic nasal those from patients with either a history of infection or
examination. On the basis of history and examination inflammation, of previous (often multiple) surgical
patient was diagnosed to be a case of congenital intervention or both. In such cases the fistula
lacrimal fistula. invariably arises from the lacrimal sac rather than
from the common canaliculus, is not lined by
Fig.3: DCG- showing Nasolacrimal duct obstruction stratified squamous epithelium and is rarely
associated with other canalicular abnormalities.
The patient had been on conservative treatment in Various authors have commented upon the hereditary
the form of topical antibiotic drops since long but pattern of lacrimal fistulas. Jones and Wobig7
with no relief. Surgical management was planned. presented a family in which bilateral lacrimal fistulae
Dacryocystorhinostomy with fistulectomy was done were present in four generations and followed an
(Figure 4a, 4b). autosomal dominant inheritance pattern. Other
authors since have also reported a high incidence of
Fig. 4a , 4b: Intraoperative picture showing DCR bilaterality of fistulae and have supported the view of
with fistulectomy an autosomal dominant inheritance pattern.4,8,9
The mode of treatment of lacrimal fistula remains
After 1week ( Figure 5) sutures were removed and the controversial. In 1929 Levinel0 reported cauterising a
passage was found to be patent on syringing with no lacrimal fistula in a seven year old boy. Unfortunately
discharge from fistulous orifice. Follow up was done whilst this closed the fistula it resulted in considerable
at 1 month, 3 months and at 6 months. Repeat tearing which was considered surprising because the
syringing done at 1yr also has been patent and the nasolacrimal duct had been found to be patent at the
patient remained symptom free. time of surgery. It is clear that since almost all
congenital lacrimal fistulae arise from the common
Fig. 5: Follow up after 1 week canaliculus simple probing, cauterising or closed
excision may result in damage to the lacrimal
drainage system. It has been hence proposed that
dacryocystorhinostomy, fistula excision and
canalicular intubation offers safe and effective
treatment for symptomatic congenital lacrimal
fistulas.5
Opening the lacrimal sac facilitates surgical
dissection and accurate ligation and removal of the
fistula from its origin, minimising the risk of damage
to the common canaliculus. In addition the incidence
of recurrence of the fistula should be reduced because
of the decreased resistance to outflow of tears effected
by the dacryocystorhinostomy (DCR). Tears should
therefore preferentially flow through the DCR rather
than through the fistula. Intubation of the common
canaliculus may be recommended in all cases in
which the common canaliculus has been manipulated
and thus is much simpler and safer through a DCR.11-12

74

An important differential diagnosis to be kept in mind Management of congenital lacrimal sac fistula.
while dealing with such cases is supernumerary Arch Ophthalmol. 1990;108:38890
punta.Supernumerary puncta were noted by 5. Toda C, Imai K, Tsujiguchi K, Komune H, Enoki
Mackenzie13 in 1854.The incidence of supernumerary E, Nomachi T. Three different types of
puncta and canaliculi is estimated to be 1 in 60,000. congenital lacrimal sac fistulas. Ann Plast Surg.
They are more common in lower lid as compared to 2000;45:6513
upper lid. Exact etiopathogenesis is unknown but it is 6. Chaung JQ, Sundar G, Ali MJ. Congenital
believed to be an accessory outbudding of solid lacrimal fistula: A major review. Orbit.
epithelial cord from the lacrimal drainage system 2016;35:212-20.
during embryogenesis. These are known to be 7. Jones LT and Wobig JL. Surgery of the eyelids
associated with lacrimal anomalies like nasolacrimal and lacrimal system. Birmingham, Ala.
duct obstruction, lacrimal fistula, lacrimal sac Aesculapius Publishing Co, 1976: 67,167-173.
diverticulum, and monocanalicular agenesis. 8. Altun A, Kurna SA Sengor T, Altun G, Oflaz A.
Although usually asymptomatic, supernumerary Unilateral congenital lacrimal fistula with renal
puncta have been reported to cause dry eye or agenesis and pelvic kidner: a case report and
epiphora. For the management of supernumerary review of the literature. Case Rep Opthalmol
puncta without symptoms, observation is a reasonable Med 2015 : 368950.
approach. In cases of dry eye due to increased 9. Lokesh HM, Bindurani MK. Hereditary bilateral
evacuation tears in the affected eye occlusion of the lacrimal fistula in twins. J Pharm Biomed Sci 25
puncta has been effective treatment. (25): 152-155.
Dacryocystorhinostomy with removal of 10. Levine J: Congenital fistula of the lacrimal sac.
supernumerary system is the choice of treatment Am J Ophthalmol1929, 12: 745-6.
when tearing may be due to associated congenital 11. Ozyol E, Ozyol P. Mirror image of unilateral
anomalies of the nasolacrimal system.14-15 lacrimal fistula in monozygotic twins : a case
In our case patient had unilateral congenital lacrimal report and review of the literature. J Clin Exp
fistula and was symptomatic. Patient was managed Opthalmol 3: 223.
surgically and the results were satisfactory. Due to 12. Birchansky LD, Nerad JA, Kersten RC, Kulwin
rarity of this condition we are hereby reporting this DR. Management of congenital lacrimal sac
case which was managed successfully. fistula.Arch Ophthalmol 108 (3) : 388-390.
13. Mackenze w. Diseases of the Eye, 4th ed.
Conclusion Longman, Brown, Green, and Longman,
Congenital lacrimal fistula are generally London.
asymptomatic. Symptomatic patients require 14. Ugurbas SK, Zilelioglu G. Congenital lacrimal
intervention as sometimes they are associated with fistula. Eur J Ophthalmol 10 (1) : 22-26.
nasolacrimal duct obstruction. The prognosis after 15. Zhuang L, Sylvester CL, Simons JP. Bilateral
surgical intervention in the form of DCR with congenital lacrimal fistulae : a case report. Ear
fistulectomy is quite good. Nose Throat J 91 (5) : 208-209.

References Address for Correspondence:
1. Francois J, Bacskulin J. External congenital
Prof.AK Khurana
fistulae of the lacrimal sac.Ophthalmologica. Dept. of Ophthalmology
1969;159:24961 SGT Medical College,
2. Rasor C, Schirmer R.Graefe-Saemisch Hospital and Research Institute, Budhera, Gurugram.
Handbuch der Augenheilkunde. Leipzig, E-mail :[email protected]
Germany, Engelman, 1877 vol 8 1-58. Mobile: 9255583523
3. Missner S, Kauffman C. Congenital lacrimal sac
fistula: a case report and review. Cutis.
2001;67:1213
4. Birchansky L, Nerad J, Kersten R, Kulwin D.

75

Frontal sinus osteoma presenting with proptosis

Raman Wadhera MS, Keshav Gupta MBBS, Manisha Nada MS, DNB*

Department of Otorhinolaryngology, Pt. BD Sharma PGIMS, Rohtak
*Regional Institute of Ophthalmology, Pt. BD Sharma PGIMS, Rohtak

Abstract

Only 3% of all sinonasal tumours arise in the sphenoid and frontal sinuses. Osteoma is the most common benign
tumour of the paranasal sinuses, with a prevalence of 3%. These slow growing tumours occur most often in
ethmoid followed by frontal sinuses. Most osteomas are asymptomatic. The commonest reported clinical
symptoms are headache or a pressure sensation. We present a case of a 27 years old male who presented with
supraorbital swelling and proptosis of 6 months duration. A huge osteoma of size approximately 5X4 cm was
surgically excised by an external approach. The patient is on regular follow up and asymptomatic for the last two
years.

Keywords: Osteoma, paranasal sinus, proptosis.

Introduction headache, pain in eyes or eyebrows, diminished
vision or double vision. On examination, there was
As most of the osteomas are asymptomatic, most of round, hard, non-tender swelling in left supraorbital
them go unnoticed, or are incidentally detected at region with normal looking overlying skin. There
radiological examination. Sixty percent of sinonasal were no limitation of ocular movements and visual
tumours arise in the maxillary sinus and only 3% of acuity was normal. Patient was sent to ENT
sinonasal tumours arise in sphenoid and frontal department for supraorbital swelling.
sinuses.
Osteomas are the slow growing tumours that occur Fig.1 : Left supraorbital swelling and proptosis
most often in the ethmoid followed by the frontal NCCT PNS showed a well-defined lobulated lesion
sinuses. Based on etiology, osteoma is classified as
developmental, traumatic and infectious. in left frontal sinus, extending into superomedial
Histologically, these may be ivory or compact, mature part of left orbit (extraconal compartment) with
or cancellous. When symptomatic, they usually inferior extension into left ethmoid air cells with
present as headache or a pressure sensation in the subtle extension into left frontal region (extraaxial),
involved region. However, a high degree of clinical
suspicion is required to attribute these symptoms to an with minimal left proptosis- suggestive of
osteoma. NCCT nose and paranasal sinuses is the osteoma(Figure 2). It also showed bilateral
preferred investigation which reveals a homogenous, ethmoidal sinusitis with minimal mucosal
dense and well-circumscribed lesion. MRI is done for thickening in right frontal and sphenoid sinuses.
extra sinus involvement.
Small asymptomatic osteomas do not require surgery. Fig. 2: NCCT PNS shows
Symptomatic patients and large osteomas may be a large frontal sinus
removed endoscopically, by an external approach or
by a combined approach. osteoma extending into
left orbit
Case Report

A 27 years old male presented to the Ophthalmology
Outpatient Department of PGIMS, Rohtak with
swelling left supraorbital region and proptosis left eye
of six months duration (Figure1). There was no
history of trauma, nasal discharge, nasal stuffiness,

76

After routine investigations, he was posted for based on location of attachment of the base, the
excision under general anesthesia. relationship of the lesion to a virtual sagittal plane
through the lamina papyracea and the anteroposterior
Fig. 3 : A large osteoma filling more than half of width of the frontal recess.
the frontal sinus. Grade I- Base of attachment is posterior-inferior
along the frontal recess.
Lynch Howarth incision was given just below the left Tumour is medial to a virtual sagittal plane through
eyebrows on medial aspect and it was extended the lamina papyracea.
further laterally. Skin and periosteal flap was raised. Anterior-posterior diameter of the lesion is less than
Bony osteoma was found protruding out of anterior 75% of the anterior-posterior dimension of the frontal
table of frontal sinus (Figure 3) and filling more than recess.
half of the frontal sinus and upper part of left orbit, Grade II- Base of attachment is posterior-inferior
which was removed intact with drill and burr. An along the frontal recess.
approximately 5X4 cm piece of bony osteoma was Tumour is medial to a virtual sagittal plane through
removed and sent for histopathological evaluation the lamina papyracea.
(Figure 4). Orbital roof was dehiscent however orbital Anterior-posterior diameter of the lesion is greater
periosteum was intact. than 75% of the anterior-posterior dimension of the
frontal recess.
Fig. 4: The excised frontal sinus osteoma. Grade III- Base of attachment is anterior or
Flaps reposited back and incision was sutured in two superiorly located within the frontal sinus and/or
layers. The swelling and proptosis disappeared in Tumour extends lateral to a virtual sagittal plane
postoperative period. No complications were through the lamina papyracea
recorded. The patient is on regular follow up and Grade IV- Tumour fills the entire frontal sinus.
asymptomatic for the last two years. Surgery is indicated for symptomatic patients, for
Discussion tumour occupying more than half the volume of sinus,
Osteomas are slow growing, benign osteogenic bony for a growing osteoma, for an osteoma obstructing the
tumours encountered most frequently in the frontal recess and for involvement beyond the
craniofacial skeleton and the paranasal sinuses. Non- paranasal sinuses. Surgical approaches can be
contrast CT of nose and paranasal sinuses is the classified as external, endoscopic or a combination of
preferred radiological investigation. MRI may be both. External approaches include external
helpful in cases of beyond sinus involvement. Frontal frontoethmoidal approach, osteoplastic frontal
sinus osteoma has been graded into four grades on the sinusectomy and craniofacial approaches.
Endoscopic approaches such as Draft IIb and
modified endoscopic Lothrop procedure
(MELP/Draft III) are preferred nowadays by some
surgeons.

References
1. Erdogan N, Demir U, Songu M, et al. A

prospective study of paranasal sinus osteomas in
1,889 cases: changing patterns of localization.
Laryngoscope. 2009;119(12): 2355–2359.
2. Lund VJ, Stammberger H, Nicolai P, et al.
European position paper on endoscopic
management of tumours of the nose, paranasal
sinuses and skull base. Rhinol Suppl. 2010; 22:
1–143.

77

3. Jones NS. Sinus headaches: avoiding over and 7. Nair S. The Frontal Sinus. In: Watkinson JC,
mis-diagnosis. Expert Rev Neurother. 2009; Clarke RW, Clark LJ, Donne AJ, James R,
9(4): 439–444. Mehanna HM, et al, editors. Scott-Brown's
Otorhinolaryngol Head and Neck Surgery(Vol
4. SeidenAM, el HefnyYI. Endoscopic trephination 1). 8th ed. NewYork: CRC Press. 2019.p.1081.
for the removal of frontal sinus osteoma.
Otolaryngol Head Neck Surg. 1995; 112(4): Address for correspondence:
607–611.
Dr. Raman Wadhera
5. Castelnuovo P, Giovannetti F, Bignami M, et al. Senior Professor, ENT,
Open surgery versus endoscopic surgery in Pt. B.D Sharma PGIMS, Rohtak.
benign neoplasm involving the frontal sinus. J E-mail: [email protected]
Craniofac Surg. 2009; 20(1):180–183. Mobile: 9416243616

6. Bignami M, Dallan I, Terranova P, et al. Frontal
sinus osteomas: the window of endonasal
endoscopic approach. Rhinology. 2007; 45(4):
315–320.

78

A giant inclusion cyst following strabismus surgery

Malay Verma MS, FMRF (Chennai)

Vision plus, HB road, Tharpakhna, Ranchi

Abstract

Inclusion cyst formation following strabismus surgery is quite rare. A rare case of an inclusion cyst following
strabismus surgery and its management is presented.

Keywords: Inclusion cyst, strabismus,surgical excision.

Introduction Fig1: Sub-conjunctival cyst

Advances in the field of strabismus as well as better Fig 2: CT Scan demonstrating large cystic mass
understanding of the surgical principles behind the The muscle as such was free from the cyst. There was
strabismus surgery have improved the outcome. no contrast enhancement, foreign body or scolex so
Because of better instrumentation and suture myocysticercosis was ruled out. Cyst excision was
materials complications have reduced to 0.7%.1 We done under local anaesthesia by transconjunctival
report one rare complication associated with the approach. The cyst was found adherent to the sclera
strabismus surgery. and tenons near the medial rectus insertion. It was
adherent to the muscle posteriorly. Complete excision
Case report of the cyst was done (Figure 3).
A 16 year old girl presented to our clinic with history
of reddish swelling in the inner corner of the right eye Fig.3: Excised cyst
since past 3 months. She was on topical antibiotic
treatment from elsewhere but there was no response to
the treatment. She had a history of right exotropia for
which a strabismus surgery had been done elsewhere
6 months back. She did not have any other significant
history pertaining to trauma, foreign body or family
history of strabismus. On examination, her best
corrected visual acuity was 6/6; N6 in both the eyes.
On cover tests the eyes were orthophoric. Hirschberg
reflex was central. Ocular movements were limited in
adduction in the right eye and full in other direction of
gazes. In the left eye the ocular movement was full
free and painless. In the right eye a smooth, reddish,
nontender sub-conjunctival cystic mass was noted
medially (Figure 1). The mass became prominent on
abduction. Conjunctiva could be moved marginally
over the mass lesion. Few telengiectatic vessels were
also seen over the mass. A provisional diagnosis of
myocysticercosis or inclusion cyst was made. CT scan
of the orbit revealed the presence of a large cystic
lesion involving the medial rectus insertion (Figure
2).

79

Histopathological examination of the cyst revealed References
clear fluid containing few macrophages inside the 1. Kampanartsanyakorn S, Surachatkumtonekul T,
cyst. The cyst wall showed non-specific
inflammation. Few muscle fibres were also noted in Dulayajinda D, Jumroendararasmee M, Tongsae
the wall. Diagnosis of inclusion cyst was made. The S. The outcomes of horizontal strabismus surgery
postoperative period was uneventful. At six weeks and influencing factors of the surgical success. J
follow up there was no conjunctival congestion and MedAssoc Thai. 2005 Nov;88 Suppl 9: 94-99.
the eyes were orthophoric as before. The ocular 2. Metz HS, Searl S, Rosenberg P, Sterns G. Giant
movement had improved though not normalized. orbital cyst after strabismus surgery. J AAPOS.
1999 Jun;3(3):185-187.
Discussion 3. Song JJ, Finger PT, Kurli M, Wisnicki HJ, Jacob
Inclusion cyst formation following strabismus CE. Giant secondary conjunctival inclusion cysts:
surgery is quite rare. There are incidental case reports a late complication of strabismus surgery.
or small case series only.2,3,4 The most likely cause for Ophthalmology. 2006 Jun; 113(6):1049.e1-2.
cyst formation is supposed to be inadvertent inclusion Epub 2006 May 2.
of epithelial remnant under the conjunctiva while 4. Hawkins AS, Hamming NA. Thermal cautery as a
suturing.2 Usually these cysts present months to treatment for conjunctival inclusion cyst after
decades after squint surgery.3 In our case the cyst had strabismus surgery. J AAPOS. 2001 Feb;5(1):48-
become noticeable within 3 months itself. Tumor size, 49.
cystic nature, and involvement of underlying
structures can be determined by ultrasonography or Address for correspondence:
radiographic imaging.3 The cyst in our case was quite
anterior so simple ultrasonography would not have Dr. Malay Verma
given help. In such cases an immersion Vision plus(Above nayansukh care),
ultrasonography or ultrasound biomicroscopy can be HB road, Tharpakhna, Ranchi [JH]
used. CT scan was able to delineate the lesion and the E-mail : [email protected]
status of medial rectus. It was also able to rule out Mobile : 9234401278
myocysticercosis.
Some small cysts may disappear on their own
however surgery might be required for most of them.4
Complete surgical removal can be curative though
another technique of cauterization under slit-lamp
visualization has also been advocated.4 We were able
to surgically remove the cyst in toto. Meticulous care
was taken to avoid any injury to the muscle. The bed of
the cyst was also cauterized to remove any residual
epithelial remnant.
To summarize, in case of any cystic swelling under the
conjunctiva in a patient who has a history of squint
surgery months or years before, possibility of
inclusion cyst should be kept in mind. Radiological
investigations can help in making the diagnosis as
well as delineating the extent of involvement.
Meticulous surgical excision with cauterization of the
base can prove curative and prevent recurrence.

80

Traumatic Phacocele - An unusual case

Nikunj Bhat MS

Mahim, Mumbai

Abstract

Blunt ocular trauma can lead to scleral rupture with dislocation of natural crystalline lens in sub-tenon or sub-
conjunctival space. Such a dislocation of natural crystalline lens is termed as Phacocele. Most cases of phacocele
are associated with prior intra-ocular surgery. Here, we report a case of traumatic phacocele in a 57 year old
female following blunt trauma with handle of the sickle. Traumatic phacocele is itself a very rare clinical
condition and to the best of our knowledge this is the first reported case of phacocele following blunt trauma with
the handle of the sickle.

Key-words: Blunt trauma, Phacocele, Scleral rupture.

Introduction cells . Intraocular pressure was unrecordably low in
right eye and 16 mmHg in left eye with Goldman's
The term phacocele is derived from a Greek word, applanation tonometer. Fundus examination was
where 'phaco' denotes lens and 'cele' means obscured due to media haze. Ultrasound B-scan
herniation. It is an unusual and very rare clinical revealed some degree of vitreous haemorrhage but
condition.1 Globe rupture with lens dislocation is rare there was no evidence of retinal detachment. There
complication of blunt trauma to the eye.2 Blunt force was absence of lens spike on axial scan of right eye.
in anteroposterior direction results in equatorial Patient was scheduled for wound exploration under
expansion, which disrupts the zonular fibres and lens general anesthesia. The conjunctival peritomy was
dislocation occurs. Generally impacts are absorbed by made along the limbal margin from 10 o'clock to 2 o'
the eye-globe, rupture may occur where the impact clock and lens was removed. Underlying scleral
took place (Direct rupture) or at a certain distance rupture was sutured with 8-0 vicryl sutures. Anterior
(Indirect rupture).3,4 Here we report a case of traumatic chamber washed and automated anterior virectomy
phacocele in a 57 year old female following blunt was performed. Patient was left aphakik.
trauma with handle of the sickle with no previous Postoperatively patient was kept on topical and
history of any ocular surgery. systemic steroids, topical antibiotic and cycloplegic
Case Report eyedrops. Patient was scheduled for secondary scleral
A 57 year old lady presented to us with chief fixated IOL after the resolution of inflammation but
complaints of sudden diminution of vision in right eye patient denied any further intervention.
since 3 days. She had suffered blunt trauma to her
right eye with wooden handle of the sickle. There was Fig. 1: Superonasal conjunctival swelling with
no significant ocular or systemic history. Her visual congestion and hyphema
acuity was perception of light positive with projection
of rays accurate in right eye and 6/6 in left eye. No Discussion
abrasion, laceration or ecchymosis was seen in the Traumatic phacocele usually occurs due to indirect
periorbital region. On slit-lamp examination of the
affected eye diffuse sub-conjunctival haemorrhage
was noted. A well-circumscribed sub-conjunctival
mass was noted superiorly about 2 mm from the
limbus beneath the upper eyelid measuring about
9x10 mm (Figure 1). Cornea was clear. Grade 2
hyphema was present in anterior chamber with 4+

81

impact on the globe following blunt trauma. The References
superonasal quadrant near the limbus is the most 1. Allen RC, Gupta RR, Poblete R, et al. Traumatic
common location of phacocele followed by
superotemporal quadrant. The inferotemporal phacocele. J Cataract Refract Surg.
quadrant is the most common site of impact which 2001;27:1333–1334.
compresses the globe against the trochlea and 2. Jarrett WH. Dislocation of the lens: a study of 166
rupturing it in the superonasal quadrant. The scleral hospitalized cases. Arch Ophthalmol.
rupture frequently occurs between limbus and Spiral 1967;78(3):289–296.
of Tillaux. Spiral of Tillaux is the imaginary line 3. Kramar PO, Brandt DE. Subconjuntival
joining the site of insertion of four recti muscle on dislocation of the lens. Arch Ophthalmol. 1976;
sclera. 94: 110-111.
It is seen more commonly in elderly people with rigid 4. Andonegui J, Ferro J. Subconjuntival cataract
sclera rather than in children with elastic ocular coats. after trauma. Photo Essay. Arch Ophthalmol.
Predisposing factors include previous surgical or 2002; 120: 1102.
traumatic scleral scar, recurrent episodes of scleritis, 5. Santos-Bueso E, Sáenz-Francés F, Díaz-Valle D,
and others.5,6 et al. [Ocular rupture associated with lens
Phacocele has been reported to comprise 13% of all dislocation to the subconjunctival space]. Arch
lens subluxation. It was first reported by Fejir in 1928. Soc Esp Oftalmol. 2007;82:641–644.
Blunt trauma can lead to scleral rupture with 6. Bhattacharjee K, Bhattacharjee H, Deka A, et al.
phacocele. Diagnosis is based on history and slit-lamp Traumatic phacocele: review of eight cases.
clinical examination such as sub-conjunctival mass, Indian J Ophthalmol 2007;55:466–468.Fejér AJ.
intact overlying conjunctiva, normal cornea and Subconjunctival lens dislocation. Am J
absence of lens spike on ultrasound axial scan. Ophthalmol. 1928;11:254-256.
Surgical intervention includes exploration of wound,
removal of lens, repair of the scleral wound, anterior Address for correspondence:
vitrectomy and placement of secondary IOL. Timely
intervention can ensure good visual recovery. Dr. Nikunj Bhat
A/12 , Dignity, Mogallane,
Mahim, Mumbai-400016
E-mail :[email protected]
Mobile:8607173082

82

Upper lid cicatricial ectropion : A rare case

Sonakshi Sehrawat MBBS, Saumya Sharma MBBS,
Neeraj Sharma MS, Raman Sethi MS,Mch

SGT Medical College, Hospital and Research Institute,
Budhera, Gurugram

Abstract

Cicatricial ectropion is the second common type of ectropion which occurs as a result of shortening of anterior
lamella of the eyelid. It usually involve lower eyelid. Various causes of cicatricial ectropion include thermal or
chemical burns, mechanical or surgical trauma/scars, medications, sun damage and chronic inflammation.
Surgical correction of upper lid cicatricial ectropion is quite challenging but is essential to prevent further
complications.

Keywords : Cicatricial ectropion , trauma, anterior lamella.

Introduction surgery, facial palsy, skin allergy. Personal and family
history were unremarkable. On examination, her
Ectropion is an outward turning of the eyelid margin. general physical and systemic examination were
Types of ectropion: congenital, acquired (involutional, normal. On ocular examination her unaided Visual
cicatricial, paralytic, and mechanical).1The most acuity was 6/24 in Right eye and 6/6 in Left eye. There
common type is involutional followed by cicatricial, was no improvement with pin hole. Extra ocular
typically involving the lower eyelid.2 Cicatricial movements were full and free in both eyes. Right eye
ectropion is characterized by vertical shortening examination revealed that medial half of the eyebrow
and/or scarring of the anterior lamella of eyelid and was absent. Scar mark was present on medial side of
can be the result of thermal or chemical burns, the eyebrow, near the medial canthus and bridge of the
mechanical or surgical trauma/scars, medications, nose. There was outward turning of upper eyelid -
sun damage and chronic inflammation.1,3 Periocular Grade2 Ectropion, Grade3 (House Brackman)
area and lids are frequently affected with facial burns, lagophthalmos was present. (Figure 1a,1b,1c) . Slit
ranging from 7.5 to 20% of all burn cases.4 lamp examination revealed oval, nebular corneal
Approximately 60% of eyelid burns develop eyelid
contractures and eyelid ectropion.5 Cicatricial opacity of about 4´3mm on centre of the cornea in
ectropion surgery following burn injuries is difficult right eye which was fluorescein stain negative. On
and is sometimes unsuccessful .Various techniques the basis of above findings we made diagnosis of right
have been described for correcting cicatricial eye upper eye lid cicatricial ectropion. Patient under
ectropion by lengthening the anterior lamella with went stricture removal with the help of unipolar
transposition flaps or with full-thickness free skin cautery and ectropion correction by the use of full
grafts.5-9 We are reporting a case upper lid cicatricial thickness skin graft from the post auricular
ectropion which was successfully managed by using region.(Figure 2).
full thickness skin graft.
ab c
Case report
A 45 years old female patient presented to OPD of Fig. 1(a,b,c) showing right upper eyelid ectropion,
SGT Hospital with complaints of-Outward turning of scar mark near medial half of the eyebrows and along
right upper eyelid for the last 2years.There was
history of Blurring of vision in right eye for past 6
months which was gradual in onset and progressive in
nature, associated with on and off watering . There
was history of thermal burn (gas stove) on right side of
face 2years back. There was no history of periocular

83

bridge of the nose from the post auricular region we successfully managed
the case.
a b cd
References
e f gh
1. Vallabhanath P and Carter S .Ectropion and
Fig. 2 a - Horizontal incision given along the lid crease, entropion. Curr Opin Ophthalmol. 2000; 11:
b - Strictures released using unipolar cautery, c- Margins 345–351.
of the wound marked and a template of the area is drawn,
d- Full thickness skin graft taken from post auricular area, 2. Ousterhout DK. and Weil RB.The role of the
e- Suturing of the graft area done with 4-0 silk suture, lateral canthal tendon in lower eyelid laxity. Plast
f- Positioning of graft on wound, g- Interrupted suturing Reconstr Surg. 1982; 69: 620–623.
done, h- Anchoring sutures used for better apposition of
graft 3. Menezes Bedran EG, Correia Pereira MV and
Discussion Bernarde TF.Ectropion.Seminars in
Cicatricial ectropion is characterized by vertical Ophthalmol. 2010: 59–65.
shortening and/or scarring of the anterior lamella of
eyelid. The most common etiologic agents of eyelid 4. Stern JD, Goldfarb IW, Slater H. Ophthalmological
burns include local thermal and chemical burns and complicationsasa manifestation of burn injury.
systemic burns.9,10 Stern et al found that 15% of their Burns. 1996;22:135–136.
burn patients had ocular pathology with eyelid burns
and lid contractures being the most common injuries.4 5. Litwin AS , Kalantzis G , Drimtzias E , Hamada
We also found similar findings in our case. Although S, Chang B, and Malhotra R Nonsurgical
lower lid ectropion is more common but in our case treatment of congenital ichthyosis cicatricial
upper lid was involved. Various types of skin grafts ectropion and eyelid retraction using Restylane
like split thickness, full thickness and sliding have hyaluronic acid. Br J Dermat. 2015:601–603.
been advocated by Stallard for the repair of such
lesions.11 Donor sites for skin grafts included upper 6. Choi CJ, Bauza A.,Yoon MK. Sobel RK, and
eyelid, supraclavicular, preauricular, postauricular, Freitag SK. Full-thickness skin g r a f t a s a n
inner brachial skin and axilla .6 Our case illustrates independent or adjunctive technique for repair of
successful management of upper eyelid cicatricial cicatricial lower eyelid ectropion secondary to
ectropion by the use of full thickness skin graft from actinic skin changes. Ophthal Plastic & Reconst
the post auricular region. Surgery. 2015; 6: 474–477.
Conclusion
Postburn cicatricial ectropion of the upper eye lid often 7. Kim HJ, Hayek B, Nasser Q, and Esmaeli
lead to severe exposure keratitis, corneal ulceration and B.Viability of full-thickness skin grafts u s e d
blindness. Therefore early management of upper lid for correction of cicatricial ectropion of lower
ectropion is necessary.Various surgical techniques like eyelid in previously irradiated f i e l d i n t h e
myocutaneous skin flaps (Z plasty) and skin grafting periocular region. Head & Neck.2013;
have been advocated for correction of cicatricial 35:103–108.
ectropion. The major issues when addressing cicatricial
ectropion followed by severe burns are: the right donor 8. Kostakolu N and Özcan G.Orbicularis oculi
site selection, appropriate graft thickness, followed by myocutaneous flap in reconstruction of
immediate grafting after debridement of scaring and postburn lower eyelid ectropion. Burns.1999; 25:
unhealthy tissue, with generous preservation of the 553–557.
orbicularis muscle. Although the patient presented to
us very late ,so the early management was not possible . 9. Tuft SJ, Shortt AJ. Surgical rehabilitation
By releasing the contractures and lengthening of following severe ocular burns. Eye.
anterior lamella with full thickness free skin graft taken 2009;23:1966–1971.

10. Malhotra R, Sheikh I, Dheansa B. The
management of eyelid burns. Surv Ophthalmol.
2009;54:356–371.

11. Stallard, HB. Eye Surgery Edition III John
Wright and Sons London.1958: 221-223.

Address for correspondence:

Dr. Sonakshi Sehrawat

Resident Ophthalmology,

SGT Medical College,

Hospital and Research Institute,

Budhera, Gurugram.

E-mail : [email protected]

Mobile : 9991646232

84

Intraocular lens calcification- A Nightmare

Preeti Yadav MBBS, Manisha Nada MS, DNB, Aakash Sharma MBBS,
Sunil Kumar Verma MS, Monika MS, Satvir Singh MS.

Regional Institute of Ophthalmology, PGIMS, Rohtak

Abstract

Cataract is the leading cause of reversible blindness and visual impairment worldwide. Cataract surgery using
phacoemulsification and intraocular lens (IOL) implantation is one of the commonest surgical procedures.
Although the material and manufacturing techniques of IOLs are constantly improving, IOL calcification remains
a rare potential complication that can affect the visual outcome of the cataract surgery. Multiple factors are
involved in the pathogenesis of IOL calcification. Hydrophobic acrylic IOLs have very low rate of postoperative
posterior capsular opacification, lens opacification and calcification compared to hydrophilic lenses.

Keywords: IOL calcification, calcium deposits, lens opacification, cataract.

Introduction period was uneventful. There was no history of
systemic illness or long term medication.
The cataract surgery technique has evolved into a safe At the time of presentation BCVA in left eye was 2/60.
Slit lamp examination revealed clear cornea, a quiet
and successful procedure for visual rehabilitation of anterior chamber, an opacified IOL and an intact
those who are blind from cataracts.1 Even after an posterior capsule( Figure 1).
Intraocular lens exchange was performed under local
uneventful cataract surgery, complications can reduce anaesthesia after the patient's written informed
consent. The opacified IOL was explanted via a
the visual outcome. Intraocular lens (IOL) sclerocorneal tunnel and +20 D hydrophobic IOL was
implanted in the bag (Figure 2 and 3) . The explanted
calcification is an uncommon complication following IOL was preserved in formalin and sent for
cataract surgery.2 This phenomenon has also been histopathology and culture. Topical antibiotics and
corticosteroids were started immediately in
observed in silicone IOLs and multifocal IOLs in postoperative period.
association with asteroid hyalosis. 3,4 Calcification of After 1 week follow up the BCVA was 6/12 in left eye.
The histopathology showed a positive Von Kossa stain
ophthalmic devices is considered rare but has been for calcium deposited on lens surface of IOL and the
culture was negative.
observed in silicone scleral buckles, the intracameral
portion of Molteno implants and IOLs.5 Multiple

factors including IOL material, host environment,

packaging and surgical instruments such as forceps-

related are involved in the pathogenesis of this
phenomenon.2,6

Fig. 1:Showing left eye intraocular lens calcification Fig. 2 : Calcified IOL Fig. 3 : Exchange of IOL

Case Report Discussion
A 42 year old woman presented with decrease of vision Various pathologic processes lead to clinically
in left eye for last 2 years after phacoemulsification significant opacification or discoloration of the optic
cataract surgery with implantation of a hydrophilic +20 component of intraocular lenses (IOLs) manufactured
dioptres (D) under local anaesthesia. The postoperative

85

from different biomaterials and in different designs. during surgery. In the late postoperative period, most
Factors such as patient's associated conditions, reports are related to silicone and hydrophilic acrylic
manufacturing process, method of IOL storage, IOLs.10 Laboratory tests showed that the opacification
surgical technique and adjuvants or a combination of of explanted hydrophilic acrylic IOLs was mostly
these may be involved. Following type of processes caused by precipitation of calcium and phosphate on
are identified: formation of deposits/precipitates on and/or within the IOL.11
the IOL surface or within the IOL substance, Mak et al concluded in their case report of a 72-year-
opacification by excess influx of water in old man who had phacoemulsification with
hydrophobic materials, direct discoloration by implantation of an Akreos Adapt Advanced Optics
capsular dyes or medications, coating by substances (AO) IOL in the left eye complained of blurring vision
such as ophthalmic ointment and silicone oil and a 4 months postoperatively. They found multiple fine
slow progressive degradation of the IOL biomaterial. white granules within the IOL. Intraocular lens
In March 2004, Werner et al analysed 106 Memory exchange was performed at 7 months, and the
Lens IOLs explanted because of late postoperative explanted IOL was sent for histopathological
opacification. The mean interval between the initial analysis. Diffuse fine white granules were seen within
cataract surgery and the diagnosis of IOL the explanted IOL material just beneath the surface,
opacification was 25.8 ± 11.9 months. The stained positive by alzarine red and the von Kossa
manufacturer correlated the opacification of this method. Scanning electron microscopy confirmed the
design with a change in the polishing process in 1999. presence of calcium deposits in the IOL material.
The modified manufacturing method used a Blood and aqueous were drawn from the patient for
phosphate buffer in the tumbling process, which biochemical analysis, and the results were normal. It
would attract more protein. The process would then was the first clinicopathological report of
continue, with the deposition of minerals, most likely calcification of theAkreosAdaptAO IOL.12
calcium, on top of the protein film.7 Werner et al concluded an association between
Altaie et al concluded calcification as a primary asteroid hyalosis and dystrophic calcification of IOLs
reason for IOL opacification in their study. The only with silicone plate haptic designs in their study.
formation of deposits containing calcium phosphate The material opacifying the 3-piece silicone lens
due to excessive saturation of the aqueous humour probably was derived from the asteroid bodies or from
with calcium crystals was a possible mechanism.8 the process that results in this vitreous condition.
Izak et al observed two types of deposits using Clinical evaluation of other pseudophakic patients
electron and optical microscopy: granular deposits on with asteroid hyalosis confirmed that IOL
the external optical surface and fine seed-like deposits calcification was restricted to silicone IOLs.3
inside the lens optics. Both were observed as parallel Neuhann et al classified IOL calcification as-
lines on the anterior and posterior surfaces.9 1. Primary calcification
Jorge et al conducted a study and concluded that 2. Secondary calcification
opacification can occur in different ways depending 3. False-positive calcification or
on the time period and the IOL materials. According
to their study, opacification was usually caused by pseudocalcification.
precipitation of crystalline deposits on the IOL's
surface during surgery which can be related to the Primary Calcification- The primary form refers to
viscoelastic substance or the balanced saline solution calcification that is inherent in the IOL, based on
used in the surgery. In the immediate postoperative possible inadequate formulation of the polymer,
period there are reports of silicone IOL opacification fabrication of the IOL or issues with its packaging
due to hydration and also due to the interaction of process. The calcification occurs in otherwise normal
hydrogel material with dyes or the viscoelastic used eyes and generally is not associated with pre-existing
diseases.

86

Secondary Calcification- The secondary form refers subjective complaints, including glare in various
to deposition of calcium onto the surface of the IOL illuminating conditions, halos around bright lights,
most likely the result of environmental circumstances colour and contrast loss, as well as hazy vision. These
( changes in the aqueous surrounding the implanted symptoms may prompt IOL explantation even in the
IOL associated with pre-existing or concurrent absence of measurable decrease in visual acuity in
diseases or any condition that has disrupted the blood cases of IOL calcification.20
aqueous barrier).It is not related to any problem with
the IOL itself hence it is referred to as secondary References
calcification. 1. Reddy MK. Complications of cataract surgery.

False-positive or Pseudocalcification- When other Ind J Ophthalmol. 1995;43(1):201-209.
pathology is mistaken for calcification or false- 2. Rezaei Kanavi M, Javadi A, Mirbabaei Ghafghazi
positive staining for calcium occurs.6
Histological studies and structure of IOLs reveal that F. Intraocular lens calcification: a clinicopathologic
the opacities occur due to the formation of organic report. J OphthalmicVis Res. 2009;4:122-124.
deposits on the IOL components or the presence of 3. Werner L, Kollarits CR, Mamalis N, Olson RJ.
impurities in the polymer.13 In some cases, the lenses Surface calcification of a 3-piece silicone
required removal and replacement due to various intraocular lens in a patient with asteroid hyalosis;
levels of vision loss or high intensity glare; while in a clinicopathologic case report. Ophthalmol.
others delayed opacity due to calcification was 2005;112:447-452.
reported after surgery.14 4. Bucher PJM, Buchi ER, Daicker BC. Dystrophic
It is most frequently reported in acrylic hydrophilic calcification of an implanted hydroxyethylmethacrylate
IOLs. The role of the blood-aqueous barrier changes intraocularlens.ArchOphthalmol.1995;113:1431-1435.
in the eye as a factor in triggering inflammation and 5. Trivedi RH, Werner L, Pandey SK, Peng Q,Arthur
subsequent opacity of the hydrophilic lens S, Izak AM et al. Intraocular lens-related
components remains unclear.15,16 The calcium content opacifications: in front of, on, within, between,
of the natural aqueous humour is low and half of the and behind the intraocular lens. In: Steinert RF,
serum calcium. Any local increase in calcium ed, Cataract Surgery; Technique, Complications,
phosphate may lead to dystrophic calcification, which and Management, Philadelphia, PA, Saunders.
may by caused by inadequate cleaning of the cortex 2004;2:443-467.
during cataract surgery and the residues of the 6. Neuhann IM, Kleinmann G, Apple DJ. A new
“crystalline lens” after cataract surgery. The classification of calcification of intraocular
saturation of the aqueous humour is the primary lenses. Ophthalmology. 2008;115:73-79.
mechanism of the delayed calcification of crystalline 7. Werner L. Causes of intraocular lens opacification
lenses.17 or discoloration. J Cataract Refract Surg.
2007;33(4):713-726.
Postoperative calcification represents the most 8. Altaie R, Loane E, O'Sullivan K, Beatty S.
important indication for explantation of hydrophilic Surgical and visual outcomes following exchange
acrylic IOLs.18 Clinical studies on opacified IOLs of opacified Hydroview intraocular lenses. Br J
demonstrated that they may be more associated with Ophthalmol. 2007;91:299-302.
complaints of cloudy vision, while visual acuity is not 9. Izak AM, Werner L, Pandey SK, Apple DJ.
drastically reduced.19 The quality of vision loss in Calcification of modern foldable hydrogel
affected patients appears to be predominantly related intraocular lens designs. Eye. 2003;17:393-396.
to increased forward light scattering and subsequently 10. Jorge P de A, Jorge D, Ventura CV, Ventura BV,
retinal stray light, which can give rise to a variety of Lira W, Ventura MC, Werner L, Kara N Jr. Late
opacification in hydrophilic acrylic intraocular
lenses: analysis of 87 eyes in a random sample of

87

102 patients. J Cataract Refract Surg. Ophthalmology. 2004;111:2094–3001.
2013;39(3):403-407. 17. Altaie R, Loane E, O'Sullivan K, Beatty S.
11. Werner L, Apple DJ, Escobar-Gomez M,
Ohrström A, Crayford BB, Bianchi R et al. Surgical and visual outcomes following exchange
Postoperative deposition of calcium on the of opacified Hydroview intraocular lenses. Br J
surfaces of a hydrogel intraocular lens. Ophthalmol. 2007;91:299–302.
Ophthalmology.2000;107(12):2179-2185. 18. Mamalis N, Brubaker J, Davis D, Espandar L,
12. Mak ST, Wong AC, Tsui WM, Tse RK. Werner L. Complications of foldable intraocular
Calcification of a hydrophilic acrylic intraocular lenses requiring explantation or secondary
lens: clinicopathological report. J Cataract intervention–2007 survey update.J Cataract
Refract Surg. 2008;34(12):2166–2169. Refract Surg. 2008;34:1584-1591.
13. Abela-Formanek C, Amon M, Schauersberger J, 19. Blundell MSJ, Mayer EJ, Knox Cartwright NE,
Schild G, Kolodjaschna J, Barisani Asenbauer T et al. Hunt LP, Tole DM, Dick AD. The effect on visual
Uveal and capsular biocompatibility of 2 foldable function of Hydroview intraocular lens
acrylic intraocular lenses in patients with uveitis or opacification: a cross-sectional study. Eye.
pseudoexfoliation syndrome; comparison to a control 2010;24:1590-1598.
group. J Cataract Refract Surg. 2002;28:1160–1172. 20. Dagres E, Khan MA, Kyle GM, Clark D.
14. Werner L, Apple DJ, Kaskaloglu M, Pandey SK. Perioperative complications of intraocular lens
Dense opacification of the optical component of a exchange in patients with opacified Aqua-Sense
hydrophilic acrylic intraocular lens; a lenses. J Cataract Refract Surg.2004;30: 2569-
clinicopathological analysis of 9 explanted lenses. 2573.
J Cataract Refract Surg. 2001;27:1485–1492.
15. Izak AM, Werner L, Pandey SK, Apple DJ. Address for correspondence:
Calcification of modern foldable hydrogel
intraocular lens designs. Eye. 2003;17:393-396. Dr. PreetiYadav
16. Neuhann IM, Werner L, Izak AM, Pandey SK, Jr. Resident,
Kleinmann G, Mamalis N, et al. Late RIO, PGIMS,
postoperative opacification of a hydrophilic Rohtak-124001(Haryana)
acrylic (hydrogel) intraocular lens. A E-mail : [email protected]
clinicopathological analysis of 106 explants. Mobile: 8930688130

88

Resident's Corner

Eye Care during COVID 19 Pandemic

Omesh Kumar Bharti M.B.B.S.,D.H.M.,M.A.E.(ICMR)

Padma Shri awardee, State Epidemiologist, State Institute of Health &
Family Welfare, Shimla, Himachal Pradesh, India

Brief communication virus from an asymptomatic COVID patient having
acute angle-closure glaucoma in early January, 2020.
Corona pandemic caused by SARS-CoV-2 virus has Another doctor got the infection in Wuhan through
devastated lives and economies all over the word. inoculation of the virus through his eyes. He wore an
COVID disease caused by SARS-CoV-2 has killed N95 mask but did not wear anything to protect his
thousands of people across nations and crippled day eyes. Respiratory viruses are capable of inducing
to day lives of millions. Basically it's a respiratory ocular complications in infected patients, which then
illness caused by spread of the virus through nose and leads to respiratory infection.
mouth of the infected person through droplets to
healthy ones. The usual symptoms are fever, mostly Weneedtopostponeelectivesurgeriesinthelightofongoing
dry cough, sore throat and difficulty in breathing. pandemic and attend only urgent eye care procedures after
People with severe infections can develop pneumonia taking all precautions including full Personnel Protective
and die from varied complications of the illness. Equipment (PPE) along with eye glasses. General public
also need to be made aware of this uncommon route of virus
Eye care during COVID pandemic appears to be a transmission and to report to COVID clinic in case they
neglected subject. When an infected person talks or develop red eye or conjunctivitis. Routine washing of eyes
sneezes, s/he may inadvertently infect the other by general public and health care professionals should also
person by spraying the virus leaden droplets that are be recommended to save them from infection from unlikely
either inhaled by the other through nose or mouth or source especially from an asymptomatic carrier of SARS-
virus may enter through eyes. You can also become CoV-2.
infected by touching something that has the virus on it
and then touching your eyes. Therefore appropriate References
precautions are advocated to be taken by everyone to
prevent COVID from spreading from one person to 1 . Seah, Ivan Yu Jun et al. Assessing Viral
another especially through eyes. Shedding and Infectivity of Tears in
Coronavirus DiseaseDOI:
Though, it is unlikely that COVID patients spread
virus (SARS-CoV-2) through their tears and just https://doi.org/10.1016/j.ophtha.2020.03.026
single patient out of 17 developed conjunctival
injection and chemosis i.e. pink eye. Two out of 12 2 . Ping Wu,Fang Duan, Chunhua Luo et al;
patients with ocular abnormalities (16.7%) had Characteristics of Ocular Findings of Patients
positive results for SARS-CoV-2 on RT-PCR from With Coronavirus Disease 2019 (COVID-19)
both conjunctival and nasopharyngeal swabs. in Hubei Province, China; JAMA
Another study showed positive SARS-CoV-2 RT- Ophthalmol. Published online March 31, 2020.
PCR results from a patient's tears, but isolation of the doi:10.1001/jamaophthalmol.2020.1291
virus was unsuccessful. Therefore there is no
evidence of spread of virus from infected patients 3 . Xia J, Tong J, Liu M, et al. Evaluation of
through tears however low abundance of the virus in coronavirus in tears and conjunctival
tear and conjunctival secretions does not eliminate the secretions of patients with SARS-CoV-2 infection. J
risk of transmission through conjunctival tissue that MedVirol.2020; DOI:10.1002/jmv.25725
may also have implications in corneal transplant.
However health care workers can get infected through 4 . By Keng Jin Lee; Coronavirus kills Chinese
droplet exposure to their eyes. At least there is a report whistleblower ophthalmologist.
of death of an ophthalmologist in Wuhan in early
stages of the pandemic following exposure to the BBC, CNN 2020; Feb.10.

https://www.aao.org/headline/coronavirus-
kills-chinese-whistleblower-ophthalmol

89

5 . Lu CW, Liu XF, Jia ZF. 2019-nCoV Address for Correspondence :
transmission through the ocular surface must
not be ignored. Lancet (London, England). Dr. Omesh Kumar Bharti
2020;395:e39. (Padma ShriAwardee)
M.B.B.S.,D.H.M.,M.A.E.(ICMR),
6 . Belser JA, Rota PA, Tumpey TM. Ocular State Epidemiologist,
tropism of respiratory viruses. Microbiol Mol State Institute of Health and Family Welfare,
Biol Rev. 2013;77:144–156. Department of Health & Family Welfare,
Government of Himachal Pradesh,
Parimahal, Kasumpti, Shimla- India-171009,
Mobile: +91-9418120302
E-mail: [email protected]

90

Congenital nasolacrimal duct obstruction -
what we need to know

Saurabh Kamal, MS, FLVPEI

Eye Hub, Faridabad

Abstract

Congenital nasolacrimal duct obstruction (CNLDO) is a definite clinical entity which requires specialist
approach in management. There is requirement of understanding the correct approach to a child with CNLDO so
that desired results can be achieved. This question/ answer write up aims to clear the myths and reality
pertaining to CNLDO, based on clinical facts, available literature and advances happening in the lacrimal
diseases.

Keywords: Epiphora, Children, Probing, Endoscopy, Nasolacrimal duct obstruction, Watering, Nasal
Endoscopic probing.

Introduction taken up for probing - First attempt should be the best
attempt.
Congenital nasolacrimal duct obstruction (CNLDO) is
a clinical entity managed by multiple specialists Myself being an Oculoplasty specialist with special
including general physician, paediatricians, general interest in Dacryology, hereby try to clear some of the
ophthalmologist, otolaryngologists, paediatric myths and reality pertaining to CNLDO, based on
ophthalmologist and Oculoplasty specialist and very clinical facts, available literature and advances
recently Dacryologist. Confused what I wrote, happening in the lacrimal diseases.
confused is what child's parent become. Nasal
endoscopy for CNLDO is similar to phacoemulsification Q 1: What is the role of topical antibiotics ?
probe for cataract. From being a simple two minute
procedure under restraint (where child cries helplessly) A 1: None, no evidence exist that topical antibiotics
to being a more ideal 15 minute procedure conducted in help in resolution of obstruction. They are indicated
general anaesthesia with nasal endoscopy and only in cases with conjunctival inflammation, eyelid
dacryoendoscopy, it has come a lot way forward. Like edema, and acute dacryocystitis. Even the frank
cataract surgery in ophthalmology, probing is a discharge in conjunctival fornix is not an indication
procedure performed by many and when procedure of topical antibiotics.
didn't succeed, dacryocystorhinostomy (DCR) was
performed. Forget about the small child who is Downside: Many parents continue to use them for
undergoing his first surgery of life, be it probing, be it many months. Some clinicians often use fortified
DCR. Not only that, parent education, method of topical antibiotics which are purely unnecessary.
Criggler's massage (massage??), blind use of Systemic absorption of drug can occur. Also it gives
antibiotics, mis-management of dacryoceles, some parents false belief that obstruction will get treated
doing early probing even at two months, and some with the use of drops. Parents put more importance to
doing probing even at two years. No uniformity! drops rather than sac compression (drops are easy,
Simple and complex types of CNLDO can only be doing sac compression is a challenge to them).
diagnosed intra-operatively with the use of nasal
endoscopy. Many failed cases of probing are being Practice tips: Give topical antibiotics only when
taken up for DCR unfortunately. Older children above 4 indicated and clearly specify the short duration.
years are taken up for DCR straightaway unnecessarily. Stress the importance of sac compression. For
A stepwise approach of first probing, second probing, discharge simply ask the parents to clean the eye
inferior turbinate fracture, intubation and then DCR - is frequently with cotton soaked in warm water.
obsolete and not recommended at all. If the child is

91

Q 2: When to begin sac compression and if in bony canal and it will not compress over the
symptoms resolves till what time it should be nasolacrimal duct canal).
continued ?
Practice tips: Follow 3 STEP Demonstration. First,
A 2: There are no guidelines for this. Sac compression demonstrate yourself the technique over the child
can be safely done as soon as symptoms and signs of explaining the anatomical landmarks. Second
CNLDO occurs. There is no harm in doing sac demonstrate and palpate the tear trough area of the
compression till the symptoms and signs resolve even mother (or person who will do sac compression) and
if required for entire one year of age. Sac compression let them feel the exact location and pressure over
has been thought to hasten the resolution of CNLDO lacrimal sac. Third, ask mother to finally demonstrate
symptoms. and conduct the sac compression in front of you over
her child.
Downside: Many parents either under or over do the
sac compression. Parents often have fear that Q 4: How to clean the eye and discharge ?
compression may put pressure over eye or nose and
cause harm. Some parents often tend to continue A 4: Most appropriate and simple method of cleaning
compression till 3-4 years of age, with the belief that an eye discharge or water is to boil few cotton balls in
symptoms will improve and that it will cure the water, leave it to cool afterwards, and then use
condition. squeezed cotton balls to clean the eye and
accumulated discharge. Cleaning can be done 2-3
Practice tips: We should clearly explain the duration times a day but depending upon discharge may be
of sac compressions, exact location, and proper done more frequently.
technique. Parents should be inform about the need
and duration of sac compression. Sac compression Downside: Many parents are reluctant to clean the
should be continued further for 3 months after eye and for them instillation of antibiotic eyedrop is
CNLDO resolves or till 9-12 months of age, easier.
whichever is later.
Practice tips: Educate parents that discharge happens
Q 3: What is the technique of sac compression ? because of accumulation in lacrimal sac and if they do
lacrimal sac compression and cleaning of eye
A 3: Do not complicate simple technique of sac regularly, discharge will decrease with time and there
compression. It is basically asking the parents to do inclination towards unneeded eyedrops will gradually
ROPLAS (regurgitation on pressure over lacrimal decrease.
sac) them-self. Purpose of compression is to put
pressure directly on lacrimal sac with tip or side of Q 5: How to confirm the diagnosis of CNLDO ?
little/index finger. It is important to properly
demonstrate the technique to mother or grandparents A 5: Diagnosis of CNLDO is confirmed by history
(who are likely to them at home). For right eye, head and clinical examination. Onset of symptoms, triad of
of the child should be placed on the right arm of the watering, discharge and matting of eyelashes in
mother, mother should use left little/index finger morning is typically present. Discharge may be
compressing over the lacrimal sac fossa (tear trough) variable. On examination again triad of increased tear
region just above the anterior lacrimal crest, below the meniscus height, positive regurgitation on pressure
eye and side of the nose. Once the area is compressed over lacrimal sac (ROPLAS) and positive Fluorescein
with the finger, it should be moved in sufficiently so as dye disappearance test (FDDT) confirms CNLDO.
to evacuate any sac contents. Finger should not be FDDT is performed by instilling topical Paracaine
moved upward, downwards or circular manner. drops first followed application of Fluorescein
stained strip to the lower conjunctival fornix and
Downside: As mentioned earlier, all different observe any residual dye or tear meniscus at 5
specialist involved in the management, advise minutes. It is important to make child involved in
different methods. With some methods pressure over some activity and avoid the hand of child rubbing their
the desired area is never achieved, and it may be given own eyes. Positive FDDT is when the tear meniscus
over the eye or nose. There is no role of downward height remain raised or residual fluorescein is seen at
movement of finger along the side of nose (as duct is

92

the end of 5 minutes. Always do FDDT on both eyes why success rate decreases with increasing age.
simultaneously. CNLDO has been divided into simple and complex
types with many rare variants coming up. Simple
Downside: Many other paediatric ophthalmic CNLDO cases tends to resolve early or resolves by
diseases should be ruled out before labelling it as sac compression, but CNLDO cases which persist
CNLDO. If any doubt regarding the paediatric beyond 1 year are likely to be of complex types which
glaucoma, examination under anaesthesia can be are unlikely to resolve spontaneously. Another
performed after obtaining informed consent from the important thing that is usually never considered is the
parents. occurrence of sac atonicity with increasing age, which
is likely to result in functional failure.
Practice tips: Check ROPLAS and do FDDT
yourself. Often clinical checking ROPLAS by doctor, Downside: Many parents never understands the
can create enough hydrostatic pressure inside the duct concept of simple and complex types. This is because
so that the valve of Hasner's can give away during of the fact that resolution of CNLDO occurs in about
assessment and / or demonstration of the sac 90% of cases. Second thing is child can never express
compression to parents. their concerns like adults do.

Q 6:At what age probing should be considered ? Practice tips: Explain to the parents about the types
of CNLDO. Inform them that they can only be
A 6: Routinely at one year of age. Early probing is diagnosed at the time of probing with nasal
only indicated for large dacryoceles, acute endoscopy. Also chances of sac atonicity increases
dacryocystitis, need for intraocular surgery and atonic with increasing age resulting in functional failure of
sac/mucoceles. Early probing can be done as early as anatomical success and need of DCR.
1-2 months or even few days old child . Bilateral
dacryoceles needs urgent treatment. Even unilateral Q 8: What is simple and complex CNLDO ?
dacryoceles require early intervention because of
chances of developing atonicity over the time. By A 8: Simple CNLDO is the presence of soft
enlarge its not good to have dacryoceles persisting membranous obstruction at lower end of nasolacrimal
beyond 6 months because of the risk of developing duct at the level of valve of Hasner. Complex CNLDO
atonicity. encompasses all other obstructions with anatomical
variations at lower nasolacrimal duct such as cyst,
Downside: Counsel parents. Many parents go against buried probe, impacted inferior turbinate, nasal
whatever you decide. Some insists on early probing, hypoplasia and syndromic CNLDO (e.g. craniofacial
so explain the chances of self resolution of CNLDO at synostosis, downs syndrome etc). Simple CNLDO
that particular age. While others inspite of all dreaded has high tendency for self resolution with higher
complications like dacryoceles, acute dacryocystitis success of probing. Complex CNLDO has lower
will never get probing done. Often many parents seek resolution rate, likely to persist beyond one year with
second opinion, where some other specialty doctor lower success rate of probing. It is important to
gives some other opinion and they become confuse. remember that the diagnosis of simple or complex
type can only be made intraoperatively and with the
Practice tips: Explain that the chances of self use of nasal endoscopy.
resolution is high but probing should not be delayed
beyond 1 year of age. In case of acute dacryocystitis, Q 9: What is procedure of choice for probing ?
dacryoceles or atonic sac, early probing is indicated.
A 9: Any CNLDO can be associated with nasal
Q 7: Why age is important factor in deciding the abnormalities and type of CNLDO is not known
probing ? beforehand. Therefore, unless nasal endoscopy is
done properly and thoroughly, a simple probing
A 7: Many new concepts have come into literature. without endoscopy is not recommended.
All studies have one thing common, that the success Furthermore, child should be properly under sedation
rate of probing decreases as the child grows older and or general anaesthesia, to have the probing done
that the chances of self resolution decreases with carefully and completely. Procedure of choice no
increasing age. Now it is important to understand that

93

doubt is Nasal endoscopic guided (NEG) probing Practice tips: Before advising DCR, diagnosis of the
under general anaesthesia. type of CNLDO must be known to the surgeon. Only
then child can be saved from DCR.
Downside: It is important to be aware that since the
child is undergoing a surgical procedure, it is most Q 13: How to manage congenital dacryoceles ?
important to have all the necessary equipments and
expertise, so as to maximise the outcome and prevent A 13: Dacryoceles are often associated with
failures. intranasal cysts, which can be very large and almost
obstructs the entire nasal cavity. Since dacryoceles are
Practice tips: Learn Nasal endoscopy. present since birth, early treatment in form of sac
compression should be initiated as early as possible.
Q 10: For whom Nasal endoscopic guided probing Bilateral dacyoceles although rare, must be treated
is recommended ? urgently. Dacryoceles can cause respiratory distress
in bilateral cases as the infants are nasal breathers,
A 10: Any child with CNLDO, irrespective of the age. even unilateral cases of dacryoceles are tricky
because nasal cavity can be compressed during breast
Downside: It is often believed that the nasal feeding resulting in choking. Dacryoceles can
endoscopy is to be done for the failed cases and older complicate into acute dacryocystitis which becomes
children. However, one should give whatever the best emergency because of the possibility of sepsis in new
chance available to child at first attempt only. born and long standing dacryceles can result in atonic
sac and mucocele formation, where only option of
Q 11: What to do for child with previous failed DCR remains. Therefore for any unilateral
probing ? dacryocele, start sac compression for few weeks and
if it not resolving NEG with cyst marsupialization is
A 11: Nasal endoscopy if not done at first attempt, performed. For bilateral cases, early and urgent
should be done in all failed cases. There are many marsupialization is performed.
variants of CNDLO which can be missed when nasal
endoscopy is not performed. And many previously Q 14: How to manage acute dacryocystitis in
failed cases either done once, twice or thrice can be infants or child with CNLDO ?
well managed by NEG probing. Straight-forward
Dacryocystorhinostomy (DCR) is not recommended A 14: Systemic antibiotics preferably intravenous
for failed cases. antibiotics should be initiated as soon as the diagnosis
is confirmed. Because the infection and sepsis can
Downside: For many years stepwise approach to spread very early in neonates and infants. Paediatric
probing has been practiced, where a first attempt acute dacryocystitis is well recognised clinical entity
followed by second attempt if it fails, blind inferior now. NEG probing is the procedure of choice, after
turbinate fracture, stents and them finally if 24-48 hours of intravenous antibiotics. Microbiology
everything fails, DCR was done at 4 years of age. and culture sensitivity of the pus is recommended.
Also, for CNLDO cases presenting after 4 years, DCR
was being done. This approach has now become Downside: Acute dacryocystitis in children is often
obsolete. managed conservatively with oral antibiotics.

Q 12: What is the true indication for DCR in Practice tips: Because the aetiology of paediatric
CNLDO? acute dacryocystitis is CNLDO and small children are
prone to sepsis, intravenous antibiotics are preferable
A 12: Bony obstruction, complete absence of bony and early surgical management of CNLDO with NEG
nasolacrimal duct, failure of previous nasal is recommended.
endoscopy probing with use of adjunctive such as
intubation / balloon dacryoplasty. Age is not a Conclusion
consideration for doing DCR. Older children must be
given a trial of NEG probing before performing DCR Recent understanding about aetiology, anatomy,
in them. In author experience, some children up to age physiology and pathology of CNLDO has changed
of 10 years can be treated with NEG probing alone.

94

the entire clinical approach and management. 10. Paediatric Eye Disease Investigator Group. A
Standard protocol now has streamlined to variant randomized trial comparing the cost-
based approach where with the use of endoscopy, effectiveness of two approaches for treating
intraoperative diagnosis of particular variant is first unilateral nasolacrimal duct obstruction. Arch
established followed by its one to one approach. Ophthalmol. 2012;130:1525–1533.
Adjuncts such as balloon dacryoplasty and silicone
intubation are useful for failed probings. Dacryoceles 11. PEDIG. Primary treatment of nasolacrimal duct
need early management. With the use of nasal obstruction with probing in children less than four
endoscopy guided probing, DCR is rarely utilised in years. Ophthalmology. 2008;115:577–584.
selective cases of CNLDO. Further suggested
reading: Kamal S, Ali MJ, Gauba V. Congenital 12. Ali MJ, Kamal S, Gupta A, et al. Simple vs
Nasolacrimal Duct Obstructions. In 'Principles and complex congenital nasolacrimal duct
Practice of Lacrimal Surgery”, 2nd edition, Ali MJ obstruction: etiology, management and
(ed). Springer, Singapore, 2018, pp 147–161. outcomes. Int Forum Allergy Rhinol.
2015;5:174–177.
References
13. Gupta A, Kamal S, Javed Ali M, et al. Buried
1. Kamal S, Ali MJ, Gauba V. Congenital probe in complex congenital nasolacrimal duct
Nasolacrimal Duct Obstructions. In 'Principles obstruction: clinical profiles and outcomes.
and Practice of Lacrimal Surgery”, 2nd edition, Ophthal Plast Reconstr Surg. 2015;31:318–320.
Ali MJ (ed). Springer, Singapore. 2018, pp
147–161. 14. Kamal S, Ali MJ, Gupta A, et al. Lacrimal and
nasal masquerades of congenital nasolacrimal
2. Macewen CJ, Young JDH. Epiphora during the duct obstruction: etiology, management and
first year of life. Eye. 1991;5:596–600. outcomes. Int Ophthalmol. 2015;35:807–810.

3. Wallace EJ, Cox A, White P, et al. Endoscopic- 15. Kashkouli MB, Beigi B, Parvaresh MM, et al.
assisted probing for congenital nasolacrimal duct Late and very late initial probing for congenital
obstruction. Eye. 2006;20:998–1003. nasolacrimal duct obstruction: what is the cause
of failure? Br J Ophthalmol. 2003;87:1151–1153.
4. Jones LT, Wobig JL. Surgery of the eyelids and
lacrimal system. Birmingham, Alabama: 16. Paediatric Eye Disease Investigator Group.
Aesculapius; 1976. p. 162–164. Repeat probing for treatment of persistent
nasolacrimal duct obstruction. JAAPOS.
5. Kushner BJ. The management of nasolacrimal 2009;13:306–307.
duct obstruction in children aged between 18
months and 4 years. JAAPOS. 1998;2:57–60. Address for Correspondence :

6. MacEwen CJ, Young JD, Barras CW, et al. Value Dr. Saurabh Kamal
of nasal endoscopy and probing in the diagnosis Director, Eye HubVision Care,
and management of children with congenital #41, Link Road, Sector-28,
epiphora. Br J Ophthalmol. 2001;85:314–318. Faridabad, Haryana
E-mail: [email protected]
7. Ali MJ, Psaltis AJ, Brunworth J, et al. Congenital Mobile : 9953715616
dacryocele with large intranasal cysts. Efficacy of
cruciate marsupialization, adjunctive procedures
and outcomes. Ophthal Plast Reconstr Surg.
2014;30:346–351.

8. Paediatric Eye Disease Investigator Group.
Resolution of congenital nasolacrimal duct
obstruction with nonsurgical management. Arch
Ophthalmol. 2012;130:730–734.

9. Schnall BM. Paediatric nasolacrimal duct obstruction.
CurrOpinOphthalmol. 2013;24:421–424.

95

Informed Consent: Need and the Execution

Parveen Monga MS, DNB

Jindal Institue of Medical Sciences, Hisar

Introduction incorporated in each informed consent:

General consent is an umbrella consent taken for a) Name of the procedure
conducting those patient care processes which do not
pose any significant risk of harm to patient like b) Purpose of the procedure or expected
collection of blood sample. It can be implied or benefits of it
expressed. However, for conducting clinical
processes that can have significant risk of patient c) Nature of anesthesia and risks
harm such as surgery, blood transfusion, radiation
therapy etc., informed consent specific to the clinical d) Probability of expected result
process is required.
e) Name of the person who will perform the
What constitutes an ideal consent is still elusive, nor is procedure
it possible to frame it for all cases as a prototype
template. The ambiguity in the consent document f) Possible complications
leads to variable interpretations that have resulted in
damages to medical fraternity at large. The Supreme g) Alternative treatments and their probable
court of India in a judgment in case of Samira Kohli effects
vs. Dr. Prabha Manchanda & ANR 1(2008) CPJ 56
(SC) has elaborated various aspects of consent taking h) Prognosis if procedure is not performed
in an attempt to stream line consent process. The
judgment was related to a Gynecological case where i) Risks involved in the procedure
initial consent was taken for diagnostic laparoscopy,
however hysterectomy with bilateral salpingo j) Requirement of any implant or device
ophorectomy was done as additional procedure in the
same sitting under general anesthesia. The consent for k) Any other relevant information
hysterectomy was taken from the mother of the
patient. The Supreme Court held the doctor liable for 4. Patient and family should be explained in a
malpractice with opinion that additional surgery language and manner they can understand.
however beneficial to the patient in saving time,
expenses, pain and suffering is not acceptable in 5. Consent has to be signed by the patient unless
circumstances not threatening to life without her minor, unconscious or insane. In these
consent. There is a need to understand the fine points exceptions consent would be taken from next of
while taking consent for a surgical procedure. kin/legal guardian in order of preference of
spouse, son/daughter, parents, brother/sister. For
Consent taking process life threatening situations when a patient is
incapable and next of kin is unavailable, the
1. Ensure that the patient is in right frame of mind to treating doctor and another clinician can take a
understand and interpret the information and able decision to safeguard the patient's life.
to make decision.
6. Consent should be taken by the person who is
2. Ask if he would like to incorporate family performing the procedure or a doctor of his
members in decision making. This is the right of surgical team and he shall sign the consent
every patient. document.

3. From the guidelines of the above mentioned case, 7. Consent should be taken before procedure within
certain essential ingredients should be a reasonable time (either on the same day or
previous day). If for some reason, the procedure
is postponed to a later date, a fresh consent
should be taken.

8. In case patient has to undergo a procedure
repeatedly (eg. Blood transfusion in Thalassemia
patient, Laser procedure), an informed consent is
taken at the first instance with a defined validity

96

period but not more than one month. The patient 2. Supriya Subramani. Patient autonomy within real
endorses the consent verbally at each treatment. or valid consent: Samira Kohli's case. Indian
However, if there is a change in the treatment Journal of Medical Ethics. Published online:
modality or an additional modality, fresh consent January 18, 2017.
shall be obtained.
9. At least one independent witness should sign the 3. Samira Kohli vs Dr Prabha Manchanda and
consent document however if the patient is Another . [(2008) 2 Supreme Court Cases 1].
illiterate, his thumb impression may be taken
along with signature of two independent 4. Guidebook for Accreditation Standards for HCO,
witnesses. 4th edition, National Accreditation Board for
In the end, besides your clinical skills, things that Hospitals and Healthcare Providers. December
protect you from medico legal problems: 2015.
1) Communication
2) Documentation 5. Omprakash V Nandimath. Consent and medical
3) Communication of documentation. treatment: The legal paradigm in India. Ind J Urol.
4) Documentation of communication 2009 Jul-Sep; 25(3): 343–347.
5) Preservation of the document.
Dr. Parveen Monga
References MS, DNB, MNAMS, FRCS(I), MHA
1. Daljit Singh. Informed vs. Valid Consent: Senior Consultant Eye Surgeon &
Assessor for NABH,
Legislation and Responsibilities Indian Journal of Jindal Institute of Medical Sciences,
Neurotrauma (IJNT). 2008 : 5 (2) ; 05- 108. Hisar, Haryana, India. 125001
E-mail : [email protected]
Mobile : 9996119245

Then and Now

Use your PPE judiciously

97

HOS QUIZ- MAY 2020
OPHTHALMIC CROSSWORD

4 1,11 14
32 4
2 8 12
3 6 9

5 6

7 13
8

9 10
1,10 5

11
12 7

13

14 Vertical Columns:
1. Basal iridectomy done in aphakic eyes treated with
Horizontal Rows:
1. Sign of infrequent blinking in patients of Grave's silicone oil
2. Autosomal recessive disorder characterised by cone-
disease
2. A study done on VEGF Trap eye in diabetic macular rod dystrophy, hearing loss, childhood obesity and
type 2 diabetes mellitus
edema 3. A classification system for chemical injuries
3. Father of Vitrectomy surgery 4. Termination of descemet's membrane
4. Visual acuity chart for diabetic retinopathy patients 5. A type of corneal opacity
5. A test done in dry eye disease 6. Identify this type of corneal dystrophy
6. A layer of the cornea
7. Thickening of lid margin 7. Light sensitive visual protein
8. ……ophthalmos – a congenital pathology in which 8. Procedure of making hole in iris
9. A planoconcave lens of power -58.6D
eye is reduced in volume without any gross ocular 10. Identify this ruler

abnormality
9. A syndrome in which subluxation of lens is seen
10. A test to diagnose macular hole
11. Identify this disease ……… branch angitis

12. A type of restrictive squint 11. Grading system to grade hypertensive changes in
13. A syndrome characterised by limbal dermoid in eye, retina.

pre auricular skin tags and vertebral abnormalities 12. Retinal haemorrhage with white centre seen in
14. Anti-VEGF used for treatment ofARMD leukemia

Instructions : 13. …….. retraction syndrome – a cranial dysinnervation
Ÿ Exclusively for members of HOS. syndrome
Ÿ Mail your answers to [email protected].
14. A drug used in the treatment of exophthalmos
Please write your HOS membership number along with
your answers. Quiz Compiled By:
Dr. Jyoti Deswal, Dr. Manisha Nada

98