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Corneal endothelial dystrophy is a progressive disease with gradual loss of vision and characterized by degeneration and dysfunction of corneal endothelial cells. Mutations in SLC4A11, a Na+ dependent OH− transporter, cause congenital hereditary endothelial dystrophy (CHED) and Fuchs’ endothelial corneal dystrophy (FECD), the two most common forms of endothelial degeneration. Along with genetic factors, oxidative stress plays a role in pathogenesis of several corneal diseases. In this study we looked into the role of SLC4A11 in antioxidant stress response in human corneal endothelial cells (HCEnC). We found increased expression of SLC4A11 in presence of oxidative stress. Depletion of SLC4A11 using targeted siRNA, caused an increase in reactive oxygen species, cytochrome c, lowered mitochondrial membrane potential, and reduced cell viability during oxidative stress. Moreover, SLC4A11 was found to be necessary for NRF2 mediated antioxidant gene expression in HCEnC. On the other hand, over expression of SLC4A11 reduces reactive oxygen species levels and increases cell viability. Lastly, CHED tissue specimens show evidence of oxidative stress and reduced expression of NRF2. In conclusion, our data suggests a possible role of SLC4A11 in regulating oxidative stress, and might be responsible for both the etiology and treatment of corneal endothelial dystrophy.

To characterize the clinical presentation of posterior polymorphous corneal dystrophy (PPCD) in eyes of Indian ethnicity. Retrospective cohort study from January 1995 to December 2015. Patients with the diagnosis of posterior polymorphous corneal dystrophy. Medical records of the patients were reviewed for clinical presentation. Histology of corneal specimens of those that underwent keratoplasty was assessed. Descriptive analysis of clinical condition. Mean age at first evaluation was 32.5 years (range 1–73 years), male:female = 35:18. Majority (44/53; 83 %) of the patients had bilateral involvement. 5/9 (44 %) patients with unilateral presentation were amblyopic in the affected eye. The clinical features documented were vesicles in 94 eyes, band-like pattern in 32 eyes, edema of varying degree in 23 eyes (12 patients, 1 patient was one eyed), and anterior segment changes in 1 eye. 8/45 (17 %) eyes had a regular astigmatism with steep axis >47 D (range 47.2–56.2 D). 16 eyes of 12 patients who had clinically evident corneal edema underwent keratoplasty. Mean age at keratoplasty was 58 years (range 1–73 years). 8 patients had penetrating keratoplasty (PK) and 8 had Descemet stripping endothelial keratoplasty (DSEK). Mean follow-up after keratoplasty was 4.2 years (1 month to 13 years). Except one, all grafts remained clear till the last follow-up. In all specimens, the Descemet membrane was grossly thickened. In our study, 12/53 (22.6 %) patients required keratoplasty for visually significant corneal edema. Except one, all were older adults. The patients who needed keratoplasty were bilaterally afflicted and had visually significant cornea edema in both eyes. With a mean follow-up duration of 4.2 years after keratoplasty, no recurrences were noted.

Aims To study various types of morphological patterns of the air bubble and their relation to a peripheral paracentesis after air injection in corneal stroma in vitro experiment. Methods Air was injected into the donor corneas from the endothelial side and pattern was noted. Four different scenarios were created, namely (a) air injection into the deep stroma (n=11), (b) air injection into the superficial stroma (n=3), (c) air injection into the deep stroma after making a peripheral incision internal to the trabecular meshwork region that simulated an anteriorly placed paracentesis incision, with the site of air injection within a clock hour of the peripheral incision (n=7) and (d) air injection into the deep stroma after making a peripheral incision, the site of air injection being 180° away from the peripheral incision site (n=3). Results Air injection at deep posterior stroma resulted in the formation of type-1 and type-2 bubbles, type 2 began from the periphery and followed the type-1 bubble pattern in majority of the donor corneas. The type-1 pattern was noted as a bubble in the central part of the donor disc that did not reach the peripheral extent of the cornea. The type-2 pattern was a bubble that started at the peripheral cornea and expanded but was limited by the limbus circumferentially. With a full-thickness peripheral incision and air injection in the same clock hour of the incision, only a type-1 bubble pattern was noted with air leakage from the site of the incision. Conclusions The results of the study corroborate with the clinical observations made during deep lamellar keratoplasty (DLK). The placement of the paracentesis has a bearing on the pattern of the air bubble and can be used to an advantage during DLK surgery.

A 5-month-old boy presented with a congenital whitish raised lesion in the central cornea of the left eye. The child had a tendency to develop bullous eruptions on the skin with trivial trauma. The patient's father had a similar history of skin lesions. Because the lesion was in the central visual axis, a superficial anterior keratectomy with an amniotic membrane grafting was performed. The lesion healed well, restoring the corneal transparency and resulting in good visual acuity. When the boy was 4 years of age, his cornea was clear, and best-corrected visual acuity in the left eye was 20/60. There was no recurrence of the lesion.

Purpose To report a case of spontaneous Descemet-membrane detachment in a patient with anterior megalophthalmos managed by intracameral perflouropropane (C3F8) gas instillation. Methods Retrospective case review. Results A 12-year-old boy presented with spontaneous corneal edema in the left eye. The anterior segment findings were suggestive of anterior megalophthalmos. Slit-lamp examination of the left eye revealed a detached Descemet membrane superiorly. Anterior segment optical coherence tomography delineated the detached Descemet membrane. Descemetopexy with nonexpansile 14% perflouropropane (C3F8) gas resulted in rapid and complete resolution of corneal edema. Conclusions Patients with anterior megalophthalmos can develop spontaneous detachment of Descemet membrane, which can be effectively managed by intracameral gas instillation.

Management of endothelial dysfunction in phakic patients is sometimes a dilemma for corneal surgeons. Phakic patients with visually significant cataract and endothelial dysfunction are preferably managed by performing combined cataract surgery with endothelial keratoplasty. However, combined surgery may be deferred in eyes with early incipient cataract, younger age and where anterior chamber is poorly visualized. As cataract formation may be accelerated after endothelial keratoplasty, these eyes may need cataract surgery subsequently. Surgical intervention in eyes with endothelial keratoplasty is of concern as this may affect the graft adversely and threaten graft survival. In this report, we describe the intraoperative surgical details and postoperative clinical course of a patient who underwent phacoemulsification with intraocular lens implantation after Descemet stripping automated endothelial keratoplasty (DSAEK).

Cataract surgery in eyes with coloboma can present intraoperative challenges and pose greater risk of complications because of the associated ocular malformations.1 2 There may be associated scleral weakness in the fetal cleft region, zonular deficiency, poor pupillary dilatation and accompanied microphthalmos. In addition, these eyes are to be handled with great caution due to the increased risk of retinal detachment, association of maculopathy and poor surgical results. There are very few reports on the outcomes of cataract surgery in eyes with congenital coloboma owing to its relatively infrequent occurrence. The aim of this study is to describe our experience of cataract surgery in 26 eyes of 25 patients with congenital iridolenticular choroidal coloboma. From January 2009 to December 2010, 25 patients (26 eyes) with congenital iridolenticular choroidal coloboma underwent cataract surgery through the anterior segment route. The charts were reviewed for demographics, extent of coloboma, type and density of cataract, surgical approach, intraoperative surgical details and postoperative outcomes. Biometry was performed using the Ocuscan RxP ophthalmic ultrasound system (Alcon laboratories, Forth Worth, Texas, USA) and a manual keratometer (Bausch and Lomb Keratometer). Axial length measurement was done by locating the patient's preferred fixation point and taking an average of the 10 most accurate axial length readings. …

Purpose: To report the results of Descemet’s stripping endothelial keratoplasty (DSEK) for failed therapeutic penetrating keratoplasty (PK). Methods: Twenty-seven eyes of 27 patients undergoing DSEK for failed therapeutic PK were analysed. Results: The mean age of the patients was 36 ± 13.9 years (range: 14–70 years). The median size of the therapeutic graft was 10 mm (inter-quartile range; IQR 9.5–11 mm). Descemet’s membrane stripping was performed in all eyes. Graft clarity was achieved in 20/27 eyes. Six eyes had primary graft failure, and one had interface keratitis in the early postoperative period. In all eyes with primary graft failure, there was progressive stromal vascularization, which led to haziness in the graft. Late postoperative complications were rejection in four eyes, infection in two eyes, secondary graft failure in one eye and vortex keratopathy in one eye. Graft size was found to be a significant risk factor for graft rejection in this series. At the last follow-up visit, the best-corrected visual acuity was ≥20/40 in 5/27 eyes (18.5%), 20/60–20/40 in 12/27 eyes (44.4%), 20/100–20/60 in 3/27 eyes (1.5%) and ≤20/200 in 7/27 eyes (25.9%). Conclusions: Considering the limited success of repeat PK in failed large therapeutic keratoplasty, DSEK is a viable option for visual rehabilitation in these eyes, however; visual acuity may be limited due to sub-epithelial/stromal or interface scarring.

PURPOSE The aim of this study was to describe a previously unreported characteristic corneal epitheliopathy in congenital methemoglobinemia (type 1). METHODS This was an observational case series of ocular findings in congenital methemoglobinemia. RESULTS Siblings of 2 different affected families (age range, 12-14 years; 3 girls, 1 boy) affected with congenital methemoglobinemia had similar ocular complaints and clinical presentation. All of them had recurrent episodes of irritation and tearing in both eyes. Slit lamp biomicroscopy examination in all of them revealed the presence of dark colored corkscrew conjunctival vessels (prominently seen on the tarsal and forniceal region) and grayish-white raised corneal epithelial lesions. Diagnostic corneal scrapings in 2 of the patients showed the presence of epithelial cells with nonspecific inflammatory cells, and the results were negative for microbes. None of the patients had any abnormality in the lid adnexa, blink response, corneal sensation, or in the tear film. The corneal lesions resolved in 2 to 3 weeks time while on supportive therapy alone and on vitamin C supplements. CONCLUSIONS Dark colored conjunctival vessels and recurrent corneal epitheliopathy causing irritation and tearing may be ocular features associated with congenital methemoglobinemia. It is pertinent for ophthalmologists, hematologists, and pediatricians to be aware of this association.

The aim of this study was to evaluate the anterior corneal surface regularity and light scattering before and after Descemet membrane endothelial keratoplasty (DMEK) with epithelial debridement and application of mitomycin C in Fuchs dystrophy-affected eyes with preoperative subepithelial fibrosis or anterior basement membrane dystrophy.In this case-control study, a chart review identified 37 eyes with Fuchs dystrophy and anterior corneal changes evident on preoperative slit lamp examination and that underwent DMEK combined with epithelial removal plus mitomycin-C application. These cases were compared with 83 contemporaneous DMEK procedures performed in eyes with Fuchs dystrophy without clinically evident anterior surface problems (controls). The outcome measures were corrected distance visual acuity (CDVA), corneal surface regularity assessed by topography, anterior corneal light scattering by densitometry, endothelial cell loss, and complications.Cases and controls had comparable demographics. Preoperatively, the cases had significantly poorer corneal surface regularity and transparency than did controls (P0.0001). Six months postoperatively, the cases achieved comparable CDVA, corneal surface regularity, and transparency as did controls (all P0.05), and 77% had a CDVA ≥ 20/25, excluding 2 eyes with preexisting retinal problems. The median 6-month endothelial cell loss was 25% and did not differ significantly between cases and controls (P = 0.31). In 1 case, there was delayed epithelial healing.Even patients with endothelial dysfunction with anterior stromal changes can realize a significant improvement in corneal surface topography and transparency by undergoing combined epithelial removal with DMEK, and this can preclude the need for a subsequent procedure to address residual corneal surface problems or the need for a penetrating keratoplasty.

Bone involvement in cysticercosis is a rare occurrence. A case of cysticercosis of the frontal orbital bone in a thirty-year-old female is reported. The diagnosis was established by computerized tomographic scanning of the orbit and histopathological examination of the cyst, surgically removed from the cavity in the orbital plate of the frontal bone. To our knowledge, this is the first case report of cysticercosis of the bony orbit.

Purpose To report the various clinical presentations of congenital corneal anesthesia. Methods The medical records of children diagnosed with congenital corneal anesthesia between January 2006 and June 2012 at the L.V. Prasad Eye Institute, Hyderabad, India, were retrospectively reviewed for demographics and clinical characteristics. Results A total of 40 patients (29 males) were included. Median age at presentation was 12 months (interquartile range, 6-60 months). Bilateral involvement was noted in 28 cases (70%). Generalized pain insensitivity with anhidrosis was documented in 10 cases (25%). The spectrum of corneal lesions included geographic epithelial defects (32 eyes), sterile ulcers (15 eyes), sterile ulcers with hypopyon (11 eyes), secondary bacterial keratitis (2 eyes), superficial/ deep corneal vascularisation (5 eyes). Generalized anesthesia with self-mutilating behavior was recognized in 10 cases. In all cases, sterile corneal ulcers resolved on average 30 days after permanent 2/3-width paramedian tarsorrhapy. One eye progressed to the stage of anterior staphyloma and subsequently underwent auto evisceration. Four eyes that underwent keratoplasty for visual rehabilitation had poor outcomes. Conclusions Accurate diagnosis, recognition of risk factors, and thorough neurological assessment is important for lessening long-term sequelae of congenital corneal anesthesia. Judicious use of tear substitutes and expedited tarsorrhaphy is crucial to restoration of useful vision in these children.

To compare the outcomes of triple Descemet's membrane endothelial keratoplasty (DMEK) versus DMEK alone in pseudophakic eyes.Retrospective, comparative, interventional case series.Patients with Fuchs' endothelial dystrophy, secondary corneal edema, and prior failed endothelial keratoplasty with or without prior cataract extraction.Outcomes of 492 DMEK procedures performed between April 2010 and August 2012 were reviewed; 292 pseudophakic eyes underwent DMEK (group 1) and 200 eyes had concurrent cataract surgery with DMEK (group 2).Corrected distance visual acuity, endothelial cell loss, immediate and early postoperative complications.The mean age at the time of surgery was 70 years (range, 47-94 years) in group 1 and 64 years (range, 46-90 years) in group 2 (P0.0001). At 6 months, the median corrected distance visual acuity was 20/25 (range, 20/16-20/80; n = 164) in group 1 and 20/20 (range, 20/16-20/100; n = 121) in group 2 (P0.0001), excluding 21 eyes with retinal or optic nerve problems. The DMEK graft failed to clear in 9 eyes (3.1%) in group 1 and 7 eyes (3.5%) in group 2 (P = 0.34); all were regrafted successfully with DMEK. No further graft failures occurred during the follow-up period. The air reinjection rate was 30% in group 1 and 29% in group 2 (P = 0.69). The air reinjection rate dropped significantly in both groups, from 45% to 16%, after use of viscoelastic was eliminated during the tissue insertion step. The median endothelial cell loss at 3 to 6 months did not differ significantly between groups (26% in both).Triple DMEK was not associated with any higher risk of complications than DMEK alone. Compared with sequential management of patients with concomitant cataract and endothelial dysfunction, triple DMEK is an effective strategy in rapid visual rehabilitation and offers the advantage of a 1-stage procedure, with reduced risks and costs.

Purpose: To study the various clinical manifestations associated with congenital aniridia in an Indian population. Methods: In this retrospective, consecutive, observational case series, all patients with the diagnosis of congenital aniridia seen at the institute from January 2005 to December 2010 were reviewed. In all patients, the demographic profile, visual acuity, and associated systemic and ocular manifestations were studied. Results: The study included 262 eyes of 131 patients with congenital aniridia. Median patient age at the time of initial visit was 8 years (range: 1 day to 73 years). Most cases were sporadic and none of the patients had parents afflicted with aniridia. The most common anterior segment abnormality identified was lenticular changes. Cataract was the predominant lens finding, observed in 93 of 231 (40.3%) phakic eyes. Other lens abnormalities were subluxation, coloboma, posterior lenticonus, and microspherophakia. Corneal involvement of varying degrees was seen in 157 of 262 (59.9%) eyes, glaucoma was identified in 95 of 262 (36.3%) eyes, and foveal hypoplasia could be assessed in 230 of 262 (87.7%) eyes. Median age when glaucoma and cataract were noted was 7 and 14 years, respectively. None of the patients had Wilm’s tumor. Conclusions: Congenital aniridia was commonly associated with classically described ocular features. However, systemic associations were characteristically absent in this population. Notably, cataract and glaucoma were seen at an early age. This warrants a careful evaluation and periodic follow-up in these patients for timely identification and appropriate management. [J Pediatr Ophthalmol Strabismus 2014;51(1):59–62.]

To describe the results of endothelial keratoplasty (EK) in the management of irido-corneal endothelial (ICE) syndrome. Retrospective, consecutive, interventional case series. Eight eyes of seven patients underwent EK for corneal edema owing to ICE syndrome. Simultaneous cataract surgery was performed in five eyes. The charts were reviewed for demographics, clinical findings, and outcomes after EK. Mean age was 50.4 years (range 35–59 years). Mean follow-up was 12.5 months (range 6–24 months). Corneal clarity was restored in all eyes and was maintained till the last follow-up. Four patients had acute rise in the intraocular pressure in the post-operative period, which was well controlled on medical treatment. One patient who was non-compliant with medications and regular follow-up advice had progression of glaucomatous disc changes and visual field loss. The best-corrected visual acuity ranged from 20/50–20/30. EK is a viable option in ICE syndrome. Adequate control of glaucoma is the paramount factor in maintaining good visual acuity in these eyes.

Purpose To evaluate the outcomes of deep anterior lamellar keratoplasty (DALK) in children. Design Retrospective interventional case series. Methods setting: Institutional, L.V. Prasad Eye Institute, a tertiary care center in south India. study population and intervention: All children less than 16 years of age undergoing DALK from January 2003 to January 2011. main outcome measure: Visual outcome and complications. Results Twenty-six eyes of 26 children (13 male and 13 female) with a mean age of 7.82 ± 4.64 years underwent DALK for keratoconus (8), microbial keratitis (6), corneal scar (6), corneal keloid (3), chemical injury with limbal stem cell deficiency (2), and dermoid (1). Big bubble was achieved in 5 eyes, while manual dissection was done in 21. Follow-up ranged from 1 week to 7.3 years. Seventeen patients with a minimal follow-up of 6 months were evaluated for visual outcomes. Final vision varied from counting fingers to 20/20 (mean sphere 2.32 diopters, mean cylinder -2.5 diopters). Complications encountered were suture-related graft infiltrate (3), graft dehiscence (3), and Descemet membrane detachment (2). Conclusions DALK is a feasible option in children with stromal corneal pathology. It offers advantages in the form of lower risk of graft rejection. However, the risk of complications such as suture-related infections and graft dehiscence persists even in these cases.

The transcription factor gene FOXC1 has been implicated with Axenfeld–Rieger syndrome (ARS) in humans. Mutations in this gene have been suggested to contribute to the mechanism leading to developmental defects in the anterior chamber of the eye. In PNAS, Seo et al. (1) demonstrated that mutations in FOXC1 led to corneal angiogenesis in mice and in anterior segment anomalies like ARS in humans. Although the data on mice are very convincing, we provide a different perspective based on our observations in two different patient cohorts with FOXC1 mutations. Our screening of patients (n = 10) with Axenfeld–Rieger anomaly (ARA) for FOXC1 mutations did not reveal any form of corneal neovascularization among the heterozygous mutation carriers (2). We also screened the FOXC1 gene in a large cohort (n = 210) of another related disease, primary congenital glaucoma (PCG) without any anterior segment anomalies, and, similarly, the mutations carriers were devoid of any corneal neovascularization as observed in our ARA cohort (3). Interestingly, the results were consistent across PCG cases that harbored heterozygous alleles of FOXC1 alone or in combination with an accompanying allele of CYP1B1.

A significant proportion of paediatric keratoplasties are performed for endothelial dysfunction due to failed graft, congenital hereditary endothelial dystrophy1 and pseudophakic corneal oedema. Descemet's stripping endothelial keratoplasty (DSEK) is an evolving procedure for isolated endothelial dysfunction with encouraging results in adults. The application and outcome of this procedure in the paediatric population has not been well studied with few reports being published so far.2–5 This study reports the indications, surgical technique and early outcomes of DSEK in children

Cataract formation may be accelerated after Descemet stripping automated endothelial keratoplasty (DSAEK) and these eyes may need cataract surgery subsequently. The reasons cited for cataract development are: intraoperative manipulation during surgery, use of air tamponade, multiple surgeries and postoperative long-term steroid use. Therefore, phakic patients with clinically significant cataract and endothelial dysfunction are preferably better managed by performing simultaneous cataract surgery with endothelial keratoplasty. However, in pediatric patients with clear lens or early incipient cataract, combined surgery is avoided in order to preserve accommodation. Cataract surgery in eyes undergone DSAEK previously is of concern as this may affect the graft adversely and threaten graft survival. Herein, we describe the intraoperative surgical details and postoperative clinical course of a 6-year-old child who underwent lens aspiration with intraocular lens implantation following cataract development 6 months after DSAEK.

To describe the results of non-Descemet stripping automated endothelial keratoplasty (non-DSAEK). Retrospective, interventional, consecutive clinical case series. Twenty-three patients underwent non-DSAEK from January 2010 to February 2011. The various indications were pseudophakic corneal edema, aphakic corneal edema, failed graft, iridocorneal endothelial syndrome and congenital glaucoma. Corneal edema cleared in all patients within 1–4 weeks. Mean follow-up duration was 7.6 months. Post-operative best corrected visual acuity ranged from 20/400 to 20/40. Fourteen patients had co-morbid factors affecting the vision. No patient had interface haze. Immediate post-operative complications were partial graft detachment (1 patient) and secondary angle closure (1 patient). Corneal clarity was restored following secondary interventions. One late post-operative complication was graft rejection at 6 months, leading to graft failure. Non-DSAEK is a safe and viable option in cases of corneal decompensation when the Descemet membrane is non-pathological.

A 54-year-old man presented with irritation and progressive blurring of the vision in the right eye during the preceding 2 months. He was an unfortunate victim of a terrorist bomb blast attack, which happened in a shopping mall complex 2 years previously. As he had received a severe ocular injury, the left eye had been enucleated. At this presentation, the visual acuity in the right eye was 20/160, improving to 20/20 with correction. Slit lamp examination of the right eye showed a deep stromal foreign body in the nasal mid-peripheral area near the 3 o' clock location (figure 1). The epithelium and stroma showed a reddish-brown hue in a 3–4 mm zone around the foreign body. The Descemet membrane and the endothelium showed a golden-brown metallic sheen along with guttate changes and radiating folds arising from the site of the foreign body (see online video). The anterior chamber was quiet, the pupil was round, regular and reacting normally, and the lens was clear. The fundus examination did not reveal any abnormality. The left eye socket was healthy, with a well-fitting prosthesis in place. Intraocular pressure was 10 mm Hg in the right eye. The patient's examination records at the time of the blast injury and later follow-up visits revealed a visual acuity of 20/20 and a retained deep stromal corneal foreign body in the right …

Purpose To compare outcomes of non–Descemet's stripping endothelial keratoplasty (nDSEK) with Descemet's stripping endothelial keratoplasty (DSEK) for the surgical management of congenital hereditary endothelial dystrophy (CHED). Methods Patients with CHED who were treated with DSEK or nDSEK were analyzed and compared with respect to graft adhesion, graft clarity, visual acuity, refraction, and complications. Results A total of 6 patients were included in the series. Of these, 3 (mean age, 5.6 ± 1.52 years; 1 male) underwent DSEK and 3 (mean age, 5.6 ± 0.57 years; 2 males) nDSEK. Preoperative best-corrected visual acuity of patients who underwent DSEK was 1.3 logMAR units compared with 1.13 logMAR units for nDSEK patients. There were no intraoperative complications. During the early postoperative period, 1 patient who had n DSEK had lenticule detachment and was successfully managed by repeat air injection. The average time for resolution of graft edema was 30 days. The postoperative best-corrected visual acuity was 0.13 ± 0 .10 in DSEK patients and 0.13 ± 0 .10 in nDSEK patients ( P = 1). Mean refraction was comparable in both groups of patients. Conclusions The outcomes of nDSEK were similar to DSEK in CHED in this small series. The specific advantage of nDSEK is the decreased surgical time and intraocular tissue manipulation.

Purpose To analyze the outcomes of post-cataract surgery descemetopexy for Descemet membrane detachment using intracameral air injection. Setting Cornea Department, LV Prasad Eye Institute, Hyderabad, India. Design Interventional case series. Methods The clinical data of patients who had descemetopexy from August 2010 to February 2011 for Descemet membrane detachment after cataract surgery using intracameral (100%) air injection were reviewed after institutional review board and ethics committee approval was obtained. Results Of the 14 patients, 9 had manual small-incision cataract surgery using the Blumenthal and Moisseiev technique, 4 had phacoemulsification, and 1 had combined phacoemulsification with trabeculectomy. The mean duration between cataract surgery and descemetopexy was 19.5 days (range 2 to 49 days). Successful reattachment of Descemet membrane and resolution of corneal edema occurred in all except 1 patient. Two patients had elevated intraocular pressure 1 day postoperatively due to appositional angle closure in 1 and pupillary block in the other. Of the 13 patients with successful Descemet membrane reattachment, the corrected distance visual acuity was 20/40 or better in 11 cases, 20/80 in 1 case, and 20/160 in 1 case. Three of the 13 patients had comorbidity factors affecting vision. Conclusion Air descemetopexy was a safe option in the management of Descemet detachment after cataract surgery. Financial Disclosure No author has a financial or proprietary interest in any material or method mentioned.

Persistent pupillary membrane is a congenital, incomplete involution of the tunica vasculosa lentis. These membranes are usually tenacious, with fine fibrils attached to the iris collarette on one side, with the other end either free floating or attached to the lens or iris focally on the opposite side. This condition is thought to represent ectopic iris tissue on the lens with abnormal iris stroma caused by aberrant involutional changes in the primitive embryological vascular system of anterior segment. Dense and thick membranes, particularly within the pupil, may result in deprivation amblyopia. We report clinical and histopathological findings in a case of a 12-year-old boy with bilateral extensive hyperplastic persistent pupillary membrane that was surgically removed without injury to the crystalline lens.

To review the clinical profile of graft detachment following Descemet's stripping endothelial keratoplasty (DSEK) and analyse the outcomes of rebubbling for graft detachment.Retrospective review.27 of a total of 309 eyes that underwent DSEK (8.7%) underwent rebubbling with air injection between January 2009 and February 2010. Eighteen (66.6%) of these eyes had complete and 9 (33.3%) had partial detachment of the transplanted lenticule. Successful attachment was observed in 20/27 (74%) eyes (12/18 with total and 8/9 with partial detachments), and clear grafts were achieved in 13 (65%) of these 20 eyes, while 7 (35%) eyes had primary graft failure. Visual acuity was 20/60 or better in 6 of these 20 eyes (35%), between 20/100 and 20/60 in 4 eyes (25%), and20/200 in 10 eyes. Three of the 10 lenticules that failed to attach with the first rebubbling procedure underwent a second attempt of rebubbling, four underwent a repeat DSEK with good outcomes, and three underwent PK. Univariate analysis showed additional vitrectomy at the time of DSEK to be a statistically significant risk factor for persistent graft detachment following rebubbling (p0.04).Rebubbling following detachment of the donor lenticule was successful in achieving graft adhesion in the majority of patients (74%), but 35% had a primary graft failure. The second attempt at rebubbling (in three patients) led to successful attachment, but the oedema failed to recover. Those who underwent repeat DSEK for unsuccessful rebubbling did well with no complications.

To compare astigmatism with clear corneal incision versus scleral tunnel incision following paediatric cataract surgery with primary intraocular lens implantation.Retrospective, comparative case series of 218 eyes of 138 children12 years of age undergoing cataract extraction with intraocular lens implantation. The study cohorts were grouped into two categories based on incision location: group 1 comprising 108 eyes from 65 children with scleral incision; group 2 comprising 110 eyes from 73 children with clear corneal incision. Cycloplegic refraction was performed at 1, 3 and 6 months postoperatively.The mean age in group 1 was 61 ± 39 months and in group 2, 51 ± 41 months. The mean postoperative astigmatism in group 1 was 1.28 ± 0.97 D, 1.42 ± 1.00 D and 1.38 ± 0.98 D at 1, 3 and 6 months respectively. The change in astigmatism between 1 and 6 months was non-significant (p=0.26). The mean astigmatism in group 2 was 1.34 ± 1.20 D, 1.13 ± 0.88 D and 1.03 ± 0.89 D at 1, 3 and 6 months respectively. Astigmatism in group 2 decreased significantly with time (p0.001). The amount of astigmatism was comparable between the two groups at 1 month postsurgery (p=0.90), while it was significantly lower in the corneal incision group at 3 (p=0.03) and 6 months (p=0.01).Postoperative astigmatism after paediatric cataract surgery by clear corneal incision was lower compared with scleral incision. However, the difference was small and clinically insignificant.

To report the outcomes of ipsilateral rotational autokeratoplasty (RAK) for nonprogressive paracentral corneal opacities in children16 years of age.Retrospective, consecutive, interventional case series.Thirty-three eyes of 33 children aged16 years undergoing RAK for nonprogressive paracentral scars.Medical records were retrospectively reviewed for the primary etiology of corneal opacity, time of onset, duration of opacity, preoperative visual acuity, formula used for calculation of trephine size, size of the trephine used, and duration of follow-up. Any intraoperative and early and late postoperative complications were noted for all patients. Postoperative visual acuity and astigmatism were noted. Visual acuity was converted to logarithm of the minimum angle of resolution units for analysis.Primary outcome was postoperative visual acuity. Graft clarity and complications were analyzed as secondary outcomes.The mean age at surgery was 7.2 ± 3 .9 months. The mean follow-up duration was 27 ± 37 months. The commonest etiology of corneal opacity was trauma (62.5%), followed by resolved microbial keratitis (21.9%). Postoperative visual acuity (1.25 ± 0.84) was significantly better (P0.001) than preoperative visual acuity (2.05 ± 0.96). The mean astigmatism at last visit was 4.04 ± 2.21 diopters. Postoperative visual acuity was better in older children (β = -0.01; P = 0.03) and had a shorter delay in presentation (β = 0.02; P = 0.05). At the last follow-up, the graft was clear in 27 cases (81.25%). The cumulative probability of graft survival was 85% at 2 years and 65% at 5 years. Complications included wound leak in 4 eyes, secondary glaucoma in 2 eyes, graft infiltrate and traumatic dehiscence in 1 eye each.Rotational is a autokeratoplasty viable alternative surgical option to allogenic keratoplasty. Graft survival at 2 years seems to be better than allogenic keratoplasty. Younger age and delay in presentation contribute to poorer visual outcomes after surgery.The authors have no proprietary or commercial interest in any of the materials discussed in this article.

PURPOSE: To report cases of microbial keratitis after LASIK from a tropical climatic zone. METHODS: This retrospective case series included 17 eyes of 15 patients managed at our center from January 1999 to December 2007. All patients had a detailed clinical evaluation followed by corneal scrapings. The material obtained on scraping was subjected to detailed microbiology evaluation. Initial medical treatment was based on microbiology results and included irrigation of the interface with appropriate antimicrobial solutions. The flap was amputated in patients who developed flap necrosis. N-butyl cyanoacrylate tissue adhesive was applied for gross thinning and penetrating keratoplasty (PK) was performed for advanced cases of keratitis. RESULTS: Mean patient age was 25.8 years (range: 18 to 44 years). Two patients had undergone unilateral surgery. Thirteen patients presented with infection in 1 eye and 2 patients had bilateral infection. The microorganisms isolated were filamentous fungi (4 eyes), Nocardia asteroides (5 eyes), atypical mycobacteria (4 eyes), Acanthamoeba (2 eyes), Corynebacterium (1 eye), and Staphylococcus epidermidis (1 eye). The infection resolved with medical treatment in 6 eyes, medical treatment and flap amputation in 4 eyes, and PK in 4 eyes. Two patients were lost to follow-up. Five eyes of 4 patients referred to us within 10 days of symptom onset resolved on medical treatment with final visual acuity ranging from 20/20 to 20/80. CONCLUSIONS: In a tropical region, it is important to consider uncommon organisms such as fungi, Nocardia , and Acanthamoeba as possible etiological agents in keratitis after LASIK. A detailed microbiology work-up will help in definitive diagnosis and appropriate treatment. [ J Refract Surg . 2010;26:209–216.]

We report an unusual case of bilateral anterior capsule rupture in a healthy 8-year-old child.

Context: This study was carried out as a part of an internal audit and is the largest series of patients having keratoglobus, published in the literature. Poor visual acuity of the patients indicates the blinding nature of the disease. Aims: We report our experience with patients having keratoglobus at a tertiary eye care center in India. Settings and Design : Retrospective study. Materials and Methods: We analyzed adults and pediatric patients (20/40 in 6/42 (14.3%) pediatric eyes and 15/53 (28.30%) adults. Visual acuity ranging from counting of fingers to no light perception was noted in 20/53 (37.74%) adults and 21/42 (50%) pediatric patients; 13/20 (65%) with blue sclera and 8/22 eyes (36.37%) without blue sclera. Vernal keratoconjunctivitis was present in one pediatric patient. Choroidal osteoma, retinitis pigmentosa, and retinal detachment were present in adults. Surgeries performed were corneal tear repair (5 eyes), tissue adhesive application (2 eyes), descematopexy (4 eyes) and penetrating keratoplasty (PK - 8 eyes: Three had post-PK glaucoma, graft failure-one eye, 4 patients wore scleral lens - prosthetic replacement of the ocular surface ecosystem). Conclusions: About 50% of pediatric eyes (65% having blue sclera) had no functional vision. Trivial trauma was responsible for corneal rupture indicating need for protective glasses. About 50% patients had post-PK glaucoma though grafts were clear.

Xeroderma pigmentosum (XP) is a rare autosomal recessive genetic disorder characterized by solar injury to exposed skin and ocular tissues due to an enzymatic defect in the ability to repair DNA damage caused by ultraviolet (UV) light [1]. The cornea in XP may show dryness, haze, exposure keratitis, vascularization, and opacification [2]. Most patients present to the cornea clinic with a significantly scarred cornea which requires corneal transplantation for visual rehabilitation. In such cases, corneal surgeons are faced with the dilemma of performing penetrating keratoplasty (PK) versus lamellar keratoplasty (LK). Anterior LK is preferred over PK for management of corneal opacity as it eliminates the risk of endothelial rejection. The few reports on the outcomes of PK in XP are encouraging, but the feasibility and results of anterior LK in XP patients is not available in the literature. According to the only reported in vivo study of corneal endothelium in XP, the patients were found to have a decreased cell density, and an increased coefficient of variation in the cell area, compared to that of normal persons [3]. Histopathological reports of corneal buttons in XP have shown a moderate to severe decrease in endothelial cells [4]. Here we describe unusual clinical observations in two XP patients which indirectly corroborate and support the evidence that endothelial cell density may be poor in these patients. This has an implication in deciding the surgical plan (PK vs LK) for corneal opacification in XP patients.

A 5-year-old boy who had undergone deep anterior lamellar keratoplasty (DALK) in the right eye 8 months earlier presented with a full-thickness graft-host junction dehiscence and iris prolapse following an injury with a rubber ball. The junction was resutured and the graft became clear within 5 weeks. To our knowledge, this is the first reported case of wound dehiscence after DALK in a child. Although Descemet's membrane might be considered a protective barrier following DALK, the graft-host junction remains vulnerable and can rupture with blunt trauma.

Conventional endothelial keratoplasty (EK) entails stripping off the Descemet's membrane (DM) and transplantation of the posterior lamellar graft. However, isolated cases published in the literature support the feasibility of EK without stripping the DM in patients with failed grafts.1 2 Based on our observation of favourable outcomes after EK without stripping the DM in patients with no clinical evidence of DM pathology, we hypothesise that Descemet's stripping may be an avoidable intraoperative step in EK for the indications where it is expected to be normal and optically clear. We report our clinical observations in four patients who underwent EK without DM removal for indications other than failed grafts. Four cases where EK was performed without DM stripping are described (table 1). DM removal was deferred in these eyes as there were no clinical features suggestive of altered/pathological DM based on slit-lamp examination and intraoperative evaluation. View this table: Table 1 Clinical information ### Surgical technique The donor graft dissection was done using the Moria microkeratome (Moria/Microtek Inc., Doylestown, Pennsylvania, USA). A 4–5 mm scleral or limbal incision was made and the posterior lamellar graft was inserted using sheets of intraocular lens (IOL) glide …

Purpose To evaluate the lenticular changes associated with congenital iridolenticular choroidal coloboma. Design Retrospective, observational case series. Methods setting : Tertiary eye care center in south India. study population : Total of 145 eyes of 98 patients. observation procedure : Medical records of all patients with the diagnosis of congenital iridolenticular choroidal coloboma between January 2011 and December 2012 were reviewed retrospectively for demographic profile, extent of coloboma, and associated lenticular changes. Results Median age of patients at the time of initial visit was 23 years (interquartile range, 13–38 years). The male-to-female ratio was ∼ 1:1. Forty-eight percent had bilateral involvement. Lens showed cataract changes in 68 eyes (48.9%). The most common type of cataract was nuclear sclerosis, which was noted in 51% of cases. A distinct type of cataract, called “coloboma cataract” (characterized by linear opacity in the region of the coloboma), was observed in 29% of cases. Other associated findings were phacodonesis in 3 eyes, dislocation in 3 eyes, and subluxation in 5 eyes. Disc and/or macular involvement in 57 eyes (39.3%) did not influence the type or density of cataract (P > .05). Conclusions Congenital iridolenticular choroidal coloboma is associated with early cataractous changes. The most common type of cataract is nuclear sclerosis. The type and density of cataract do not seem to be related to the extent of the choroidal coloboma. We suggest a distinct description with the nomenclature “coloboma cataract” to be considered in the clinical grading of cataracts in patients with this condition.

A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8–10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome.

A 12-year-old boy with Hurler-Scheie syndrome (H/S syndrome) reported with reduced vision in both the eyes for past few years. Deep anterior lamellar keratoplasty (DALK) was performed for visual rehabilitation in his left eye. During surgery, the predescemet's plane was reached by meticulously dissecting the lamellar fibres using a manual technique. Histopathology of the dissected cornea showed the presence of numerous alcian blue positive deposits corroborating with the diagnosis of mucopolysaccharidosis (MPS). Postoperative course was uneventful. One year following surgery, the graft was clear and had a visual acuity of 20/50 with +1.00 170° - 0.75 refractive correction. Endothelial cell count, as measured by non-contact specular microscopy, was 2473.4 cells/mm(2). This case report highlights the application of DALK in a case of MPS-related corneal stromal opacification.

Purpose To report the clinicopathologic features of corneal involvement in patients with xeroderma pigmentosum. Design Retrospective review of corneal histopathology. Methods Thirteen corneal specimens of 11 patients with xeroderma pigmentosum who underwent keratoplasty (lamellar/full-thickness) for corneal involvement were studied. Five-micrometer-thick sections were made from all the samples and stained using hematoxylin-eosin and periodic acid–Schiff stains. A light microscopic examination was performed to study the histopathologic changes. Results The corneal findings on clinical examination were haze, scarring, vascularization, stromal edema, pigment clumps on endothelial surface, and corneal thinning. The histopathologic evaluation revealed changes in all layers of cornea. Epithelial changes seen were intraepithelial edema, fibrosis, epithelial downgrowths, and pannus formation. The Bowman membrane was fragmented or absent. Stroma was characterized by alteration in the lamellar pattern, scarring, edema, loss of keratocytic nuclei, and calcification. The Descemet membrane was thickened to a variable extent in most specimens and there was marked loss of endothelial cells in all. Conclusion Most histologic features are consistent with the previous few reports. The remarkable finding in all corneal specimens was a moderate to severe degree of loss of the endothelial cells. This noteworthy finding supports the ultraviolet (UV) radiation–induced endothelial cell damage in these patients. This has an important clinical implication when planning for anterior lamellar keratoplasty, as endothelial cell density may be subnormal in these patients.

The Burkholderia cepacia complex is a group of genetically distinct, phenotypically similar, Gram-negative bacteria noted for their environmental versatility. Burkholderia species are implicated in a wide array of disease processes such as pneumonia, sepsis, urinary-tract infection, meningitis and endocarditis. It is intrinsically resistant to aminoglycosides, and first- and second-generation cephalosporins.1 We present the clinical picture and treatment outcomes of Burkholderia keratitis based on a series of four cases. This is a retrospective case series of four patients with culture-positive Burkholderia keratitis seen at a tertiary eye-care centre in India, between January 2006 and January 2008. All patients presented with chief complaints of pain, redness and watering. They …