Strategies and Application of Compression Sutures With a Modified Technique for Rapid Resolution of Large (Grade III) Hydrops: A Prospective Interventional Study.

PURPOSE: The aim of this study was to describe the strategies and outcomes, with a modified technique, of compression sutures in large acute hydrops. METHODS: This was a prospective interventional study in 29 eyes of 28 patients who underwent compression sutures using a modified technique. The degree of resolution after surgical intervention was quantified by 3 masked observers, using the slit-lamp photographic documentation and optical coherence tomography. The degree of resolution, best-corrected visual acuity, and complications were analyzed. RESULTS: The mean age was 20.89 (7-46) years. Five patients had pellucid marginal degeneration (pellucid marginal corneal degeneration), 23 had keratoconus, and 1 had posttrauma hydrops. The preoperative visual acuity was hand motions in all eyes. The reduction in edema was 76% on day 1, 91.6% on 6 ± 2 days, 98.6% on 18 ± 3 days from surgical intervention. The baseline pachymetry assessed on optical coherence tomography was beyond measurement in 26 eyes. The mean pachymetry at day 1 was 704 µm (range 480-950) which reduced to 607 (range 422-850) microns at 6 ± 2 days and 518 (range 415-718) microns at 18 ± 3 days postintervention ( P < 0.0001). The mean duration between the surgery and complete suture removal was 48 (35-68) days. No suture-related complications were noted. Twenty eyes could attain good visual rehabilitation with glasses/contact lenses (vision could not be assessed in 5 eyes). Penetrating keratoplasty was performed on 1 patient. CONCLUSIONS: Compression sutures using the modified technique was safe and effective in the rapid resolution of acute hydrops in keratoconus and pellucid marginal corneal degeneration. No complications were noted, and visual restoration with spectacles and contact lenses was satisfactory.

Characteristics of Corneal Endothelium in Axenfeld Rieger Spectrum.

PURPOSE: The purpose of this study was to compare the corneal endothelial characteristics in Axenfeld anomaly (AXA), Rieger anomaly (RGA), and Axenfeld-Rieger anomaly/syndrome with age-matched healthy controls. METHODS: This is a retrospective, comparative case-control study of 52 eyes of 30 patients with AXA/RGA and AXA/S and 36 controls. RESULTS: Median age at endothelial imaging was 21.5 years (interquartile range, 13.8-33.3 years). In the study group, the mean endothelial cell density (ECD) was 2112.4 ± 78.5 cells/mm 2 , the mean cell area (MCA) was 526.9 ± 28.5 µm 2 , and the coefficient of variation of cell size was 41.2 ± 1.8%. The ECD was significantly (all, P < 0.0001) lower than controls, while MCA ( P < 0.0001), SD of cell size ( P < 0.0001), and maximum cell area ( P = 0.0007) were significantly higher than controls. Four eyes of 3 patients had guttae on slitlamp evaluation and endothelial imaging. There were no differences in the corneal endothelial characteristics among the clinical subtypes. CONCLUSIONS: Patients with AXA, RGA, and Axenfeld-Rieger anomaly/syndrome have lower ECD and increased MCA compared with normal eyes. The reduced ECD associated with inherent anterior segment alterations can predispose to the risk of postcataract surgery endothelial decompensation in these eyes. The association of guttae in some eyes needs further investigational studies.

Outcomes after surgical removal of anterior epi-capsular plaque associated with persistent pupillary membranes in children.

INTRODUCTION: Anteriorepi-capsular plaque (ACP) is usually eccentric and is associated with persistent iris strands arising from the collarette. Outcomes of lens preserving ACP peeling along with removal of persistent pupillary membrane (PPM) strands have seldom been reported with only a few cases within some case series appearing in the literature. Herein we aim to report outcome after surgical removal of ACP associated with PPM in children. METHODS: Research was done in three different Institutes. Charts were reviewed for all children who had undergone ACP removal surgery from 2010. Age at surgery, gender, preoperative best corrected visual acuity (BCVA), preoperative refraction, morphologic details of ACP, intraoperative complications, ophthalmic viscosurgical device use, age at last follow-up, postoperative complications, and postoperative BCVA were reviewed. RESULTS: A total of 16 patients, 10 male and 6 female, were included in study. Age at surgery ranged from 2 to 86 months old. Age at last follow up visit ranged from 2 to 14 years old. Postoperative complications were not observed in any of the 16 patients. Specifically, in all patients the crystalline lens remained clear at the last follow up visit. CONCLUSIONS: Congenital ACP of the lens associated with PPM and clear lens can be safely surgically removed with a very low risk of iatrogenic cataract in the early period after surgery.

Outcomes of Ahmed Glaucoma Valve Implantation in Eyes with Pediatric Keratoplasty.

PURPOSE: This study aimed to assess the efficacy and safety of Ahmed glaucoma valve (AGV) (New World Medical Inc.) implantation in eyes with pediatric keratoplasty and glaucoma. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-eight eyes of 25 children who underwent AGV after penetrating keratoplasty (PK) in 22 eyes and Descemet's stripping endothelial keratoplasty (DSEK) in 6 eyes were included. METHODS: Children (age ≤ 16 years) treated with AGV implant after keratoplasty from 2008 to 2019 with documented 6 months follow-up operated by a single surgeon were included. MAIN OUTCOME MEASURES: The primary outcome measure was AGV survival (intraocular pressure [IOP] between 6 and 21 mmHg with or without topical anti-glaucoma medications [AGMs]), and the secondary outcome measure was corneal graft survival. RESULTS: The mean age (± standard deviation) of patients at AGV surgery was 4.8 ± 0.7 years. The mean follow-up after AGV was 3.4 ± 0.5 years. The mean IOP decreased significantly from 31.5 ± 2.0 mmHg to 21.1 ± 2.3 mmHg at 6 months postsurgery (P < 0.001). The mean number of AGM decreased significantly (P < 0.001), and there was no change in mean visual acuity (P > 0.04 with Bonferroni correction). The cumulative survival probability of AGV was 92.9% ± 4.9% at 1 year and 81.3% ± 11.7% at 5 years. The AGV success was comparable between PK and DSEK eyes (P = 0.73). The cumulative probability of graft survival was 96.2% ± 3.8% at 1 year and 77.8% ± 9.1% at 5 years. The graft survival was also comparable between PK and DSEK eyes (P = 0.18). One eye needed tube trimming; none had tube or implant exposure. The major complication noted was corneal graft infection in 8 eyes (28.5%). CONCLUSIONS: In this cohort, the long-term AGV success and graft survival outcomes are satisfactory considering that AGV was performed in complex eyes with keratoplasty.

Toric intraocular lenses: Expanding indications and preoperative and surgical considerations to improve outcomes.

Since the introduction of the first toric intraocular lens (IOLs) in the early 1990s, these lenses have become the preferred choice for surgeons across the globe to correct corneal astigmatism during cataract surgery. These lenses allow patients to enjoy distortion-free distance vision with excellent outcomes. They also have their own set of challenges. Inappropriate keratometry measurement, underestimating the posterior corneal astigmatism, intraoperative IOL misalignment, postoperative rotation of these lenses, and IOL decentration after YAG-laser capsulotomy may result in residual cylindrical errors and poor uncorrected visual acuity resulting in patient dissatisfaction. This review provides a broad overview of a few important considerations, which include appropriate patient selection, precise biometry, understanding the design and science behind these lenses, knowledge of intraoperative surgical technique with emphasis on how to achieve proper alignment manually and with image-recognition devices, and successful management of postoperative complications.

Descemet Stripping Automated Endothelial Keratoplasty in Pediatric Age Group: A Decade of Our Experience.

PURPOSE: This study was to report the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) in managing corneal endothelial disorders in children less than 14 years of age. METHODS: Medical records of 180 DSAEK performed, between 2008 and 2020, on 167 eyes of 111 children who had a visually significant endothelial dysfunction were retrospectively reviewed for the primary etiology of corneal decompensation, time of the onset and duration of cloudiness, preoperative visual acuity, the technique of endothelial keratoplasty, surgical modifications, and reintervention. RESULTS: Median age at surgical intervention was 7.9 years (interquartile range, 5.2-11.2 years). At a median postinterventional follow-up of 2.5 years (interquartile range, 0.9-3.4 years), 86.2% (144 of 167 eyes, 95% confidence interval 79.9%-90.1%) maintained a clear graft. Median best-corrected visual acuity (logarithm of the minimum angle of resolution) improved from 1.45 ± 0.70 preoperatively to 0.90 ± 0.06 at the last follow-up visit (P < 0.0001). The cumulative overall long-term graft survival was 92.7%, 86.5%, and 77.7% at 1, 3, and 7 years, respectively. Cox proportional hazards regression analysis showed that the indication for DSAEK (P = 0.007; hazards ratio: 2.17 ± 0.62), age at surgery (P = 0.02; hazards ratio: 0.87 ± 0.05), and any subsequent intervention after DSAEK (P = 0.003; hazards ratio: 0.11 ± 0.08) were significant risk factors for DSAEK failure. The endothelial cell loss was 40.1% at 6 months, 45.4% at 1 year, 55.2% at 5 years, and 61.9% at 7 years. CONCLUSIONS: DSAEK is a safe and effective surgical strategy in managing endothelial disorders among the pediatric age group.

Outcomes of keratoplasty in a cohort of Indian patients with xeroderma pigmentosum.

Purpose: To evaluate the outcomes of keratoplasty for xeroderma pigmentosum (XP) performed at a tertiary eye care center. Methods: A retrospective review of medical records of those patients who were clinically diagnosed to have XP (54 eyes of 36 patients) and underwent keratoplasty; either deep anterior lamellar keratoplasty (DALK, four eyes), endothelial keratoplasty (EK, eight eyes), or penetrating keratoplasty (PK, 42 eyes) from 1994 to 2018. Results: The median age at surgery was 20.6 years (interquartile range [IQR], 14.6-27.6 years) and 20 (55.6%) were males. Graft failure occurred in 15 eyes (35.7%) in the PK group and two eyes (50%) in the DALK group; none failed in the EK group. The probability of graft survival in the PK group was 97.2% ± 2.7% at 1 year, 74.0% ± 8.0% at 2 years, and 54.8% ± 11.7% at 5 years. In the PK group, 13 eyes needed antiglaucoma medications, 11 eyes developed graft infiltrate, and 13 eyes needed secondary interventions (cataract surgery, excision biopsy, and tarsorrhaphy). In the EK group, three eyes needed secondary interventions (excision biopsy). Median postoperative endothelial cell density at the last follow-up in the PK group was 1214 cells/mm2 (IQR, 623-2277 cells/mm2). Conclusion: Despite the complexities of the ocular surface and adnexal issues in XP, keratoplasty had reasonably good outcomes. More than half of the PK grafts survived 5 years with no failures in the EK group. Regular follow-up and timely management of suture-related infections raised intraocular pressure, and suspicious ocular surface lesions, in addition to solar protection, are important for the success of keratoplasty in these eyes.

Ocular Features in a Large Cohort of Indians With Xeroderma Pigmentosum.

PURPOSE: Xeroderma pigmentosum (XP) is an extreme hypersensitivity to sunlight causing skin freckling and pigmentary changes because of defective DNA repair mechanisms. The purpose of this article is to evaluate the spectrum of ocular and systemic features in XP at a tertiary eye care center in India over 32 years. METHODS: Data from 418 eyes of 209 patients diagnosed with XP from 1987 to 2018 were reviewed retrospectively for demographics, complaints, ocular features, systemic associations, and their management. RESULTS: Median age at diagnosis was 2 years (interquartile range, 0.5-5 years). A total of 124 patients (59.3%) were men. There was parental consanguinity in 74.4% cases. Common ocular complaints were photophobia (47.1%), ocular discomfort (45%), defective vision (36.6%), redness (13.4%), tissue growth (12%), white spot (11.2%), and pain (10.5%). At presentation, 43.5% had corneal scars (45.5% were located inferiorly and 70.9% covered visual axis). Corneal vascularization and limbal stem cell deficiency were noted in 37.4%. A total of 56% of patients had at least 1 tumor, and 6.7% had neurological abnormalities. At least 1 ocular surgery was performed in 37.8% of patients. Ophthalmic surgical interventions included tumor excision (23%), keratoplasty (13.4%), and nontumor ocular surface surgery (3.4%). CONCLUSIONS: XP is a disorder that has high ocular morbidity in Indian patients. The recognition of common signs and symptoms and relative frequency of various ocular complications with time trends will help in managing and reducing the sequelae of this otherwise untreatable and progressive disease.

Determination of Oxidative Stress Markers in the Aqueous Humor and Corneal Tissues of Patients With Congenital Hereditary Endothelial Dystrophy.

PURPOSE: The aim of this study is to determine the presence of oxidative stress markers in the aqueous humor (AH) and corneal tissues of patients with congenital hereditary endothelial dystrophy (CHED). METHODS: Interventional prospective study was undertaken to quantify levels of ascorbic acid and glutathione in the AH of patients with CHED. AH was collected from patients undergoing keratoplasty and levels of ascorbic acid and glutathione were determined using biochemical assays and measured spectrophotometrically. AH collected from pediatric patients with cataract were used as control. Corneal sections of patients who underwent penetrating keratoplasty were obtained, and presence of glutathione peroxidase 1, catalase, and superoxide dismutase was determined by immunohistochemistry. Tissue sections obtained from cadaveric corneas unsuitable for clinical transplant were used as control. RESULTS: Significantly increased ascorbic acid levels were determined in patients with CHED (605.6 ± 158.9 µM) compared with those in controls (190.5 ± 74.72 µM). However, a trend toward reduced level of glutathione was detected in patients with CHED compared with that in the controls. Increased glutathione peroxidase 1 staining and reduced expression of catalase was detected in corneal tissues of patients with CHED compared with those in control corneal tissues. There was no apparent changes observed in the expression of superoxide dismutase in the corneal sections obtained from patients with CHED. CONCLUSIONS: To the best of our knowledge, this is the first study to determine the levels of ascorbic acid and glutathione in AH of patients with CHED. Our data suggest the presence of oxidative stress in CHED that might be responsible for the pathological changes in patients with CHED.

Acute endophthalmitis after penetrating and endothelial keratoplasty at a tertiary eye care center over a 13-year period.

PURPOSE: To evaluate the clinico-microbiological profile, donor cornea risk factors, and outcomes of postkeratoplasty endophthalmitis at a tertiary care center. METHODS: Retrospective analysis of charts of 28 consecutive patients (28 eyes) of acute endophthalmitis following either an endothelial keratoplasty (EK) or an optical penetrating keratoplasty (PK) surgery, performed between 2006 and 2018 (13-year period). Positive microbiology, identification and classification of predisposing factors, surveillance of utilized paired donors, treatment outcomes, and differences in the rate and severity of the event between optical penetrating and endothelial keratoplasty. RESULTS: The estimated incidence of endophthalmitis was 0.23% in the entire cohort; it was 0.34% and 0.15%, after EK and PK, respectively (P = 0.049). The median time of endophthalmitis was 4.5 days postsurgery. Donor-related endophthalmitis was recognized in 7/28 (25%) eyes. Culture positivity was 68% (n = 19 of 28). Bacteria was isolated in 84% (n = 16 of 19) instances; Gram-negative bacilli were more common (87.5%; 14 of 16), and Pseudomonas species (50%; 7 of 14) was the most common Gram-negative bacterium. Majority (>75%) of the Gram-negative bacteria were resistant to all fluoroquinolones, aminoglycosides, 3rd generation cephalosporins, and meropenam; 1/3rd were resistant to imipenem; and 90% were sensitive to colistin. Treatment included intraocular antibiotic injections (96.4%), vitrectomy (42.9%), and therapeutic keratoplasty (50%). In 85.7% (24 of 28), globe was salvaged. The final vision was 20/200 or better in 39.1% (9 of 23) eyes. CONCLUSION: EK carried a higher risk of endophthalmitis than PK in this cohort. Bacterial infection was more common in this series, with Gram negative bacilli being the commonest organisms. Multidrug resistance was common (~75%) in Gram negative isolates.

Bilateral retinal detachment in Goldenhar syndrome.

The ocular features of Goldenhar syndrome (GS) are typically external, like surface dermoids and lid coloboma. Retinal detachment (RD) is rare and has not been reported in absence of other concomitant predisposing congenital ocular disorders. We present a unique case of bilateral rhegmatogenous retinal detachment (RRD) with GS. To the best of our knowledge, this association of GS with RRD is novel and has not been reported earlier in ophthalmic and systemic literature on RRD.

Surgical management of acquired implantation iris cysts: indications, surgical challenges and outcomes.

PURPOSE: To describe the clinical spectrum, clinicopathological correlation and outcomes of different surgical strategies in the management of acquired implantation iris cysts. METHODS: From 1 January 1989 to 31 December 2015, 27 patients (27 eyes) with acquired implantation iris cysts underwent surgery. The charts were reviewed for demographics, preoperative characteristics, surgical approach, histopathological records of excised cyst and postoperative outcomes. RESULTS: The median age at presentation was 5 years (IQR: 1.3-14 years). Out of 27 patients, 21 (78%) were aged≤18 years. Almost two-third (17/27, 63%) patients had history of penetrating ocular trauma prior to surgery. All patients underwent cyst aspiration combined with complete cyst excision with additional surgical procedures when necessary. Along with complete cyst excision, sector iridectomy was performed in 20/27 (74%) eyes. At a median postoperative follow-up period of 8 months (range: 1-72 months), recurrence was noted in 3/27 (11%) cases at a mean follow-up period of 2.3±1.5 months postsurgery. Eyes in which sector iridectomy was performed had lower incidence of recurrence, and this was statistically significant (p=0.03). However, the improvement in best-corrected visual acuity postoperatively was not statistically significant (p=0.15). CONCLUSION: Acquired implantation iris cysts are associated with significant ocular morbidity. Complete excision of the cyst with sector iridectomy is an effective treatment option if other less invasive surgical approaches fail. Visual acuity can be significantly improved but is typically limited due to associated comorbidities.

Ligneous conjunctivitis in a Dandy-Walker syndrome: A rare case report.

Ligneous conjunctivitis (LC) is a rare form of pseudomembranous conjunctivitis seen in children, perhaps due to plasminogen deficiency, which manifest as a chronic refractory pseudomembranous conjunctivitis. LC cases are incapable in maintaining their fibrinolytic activity due to plasminogen deficiency; consequently, transudates of plasma assume as a thick, gelatinous, woody membranes over the mucosal surfaces. This is a short case report on a child with a LC, who presented with recurrent pseudomembranous conjunctivitis in conjunction with progressive congenital hydrocephalus due to aqueductal stenosis (Dandy-Walker syndrome). This rare association was clinically confirmed and prompt corrective surgical measures were instituted.

Outcomes of keratoplasty in lattice corneal dystrophy in a large cohort of Indian eyes.

Purpose: The purpose of this study is to evaluate the outcomes of keratoplasty for lattice corneal dystrophy (LCD) performed at a tertiary eye care center. Methods: A retrospective review of medical records of those patients who were clinically diagnosed to have LCD (72 eyes of 57 patients) and underwent either penetrating keratoplasty (PK, 58 eyes of 46 patients) or deep anterior lamellar keratoplasty (DALK, 14 eyes of 13 patients) between the years 1987 and 2014 was performed. The main outcome measures included demographics, clinical features, and outcomes of keratoplasty. Results: The median follow-up after keratoplasty was 3.1 years (interquartile range [IQR], 9 months to 9 years). The median best-corrected visual acuity (BCVA) was 0.18 (IQR, 0.10-0.48) (Snellen equivalent 20/30 [IQR, 20/25-20/60]) at 4 years postoperatively and 0.65 (IQR, 0.18-0.95) (Snellen equivalent 20/89 [IQR, 20/30-20/178]) at 10 years following surgery. DALK eyes had a significantly better BCVA than PK eyes at 2 years following keratoplasty. The median overall survival of grafts was 15.8 years. Late complications included recurrence of LCD (14 eyes), graft infiltrate (23 eyes), graft rejection (15 eyes), graft failure (16 eyes), and glaucoma (14 eyes). Conclusion: The outcomes of graft are similar following PK and deep anterior lamellar keratoplasty; however, the latter appears to provide slightly better visual outcome. Recurrence of dystrophy in the graft and graft infiltrates limit the overall graft survival in both the groups.

Angiopoietin receptor TEK interacts with CYP1B1 in primary congenital glaucoma.

Primary congenital glaucoma (PCG) is a severe autosomal recessive ocular disorder associated with considerable clinical and genetic heterogeneity. Recently, rare heterozygous alleles in the angiopoietin receptor-encoding gene TEK were implicated in PCG. We undertook this study to ascertain the second mutant allele in a large cohort (n = 337) of autosomal recessive PCG cases that carried heterozygous TEK mutations. Our investigations revealed 12 rare heterozygous missense mutations in TEK by targeted sequencing. Interestingly, four of these TEK mutations (p.E103D, p.I148T, p.Q214P, and p.G743A) co-occurred with three heterozygous mutations in another major PCG gene CYP1B1 (p.A115P, p.E229K, and p.R368H) in five families. The parents of these probands harbored either of the heterozygous TEK or CYP1B1 alleles and were asymptomatic, indicating a potential digenic mode of inheritance. Furthermore, we ascertained the interactions of TEK and CYP1B1 by co-transfection and pull-down assays in HEK293 cells. Ligand responsiveness of the wild-type and mutant TEK proteins was assessed in HUVECs using immunofluorescence analysis. We observed that recombinant TEK and CYP1B1 proteins interact with each other, while the disease-associated allelic combinations of TEK (p.E103D)::CYP1B1 (p.A115P), TEK (p.Q214P)::CYP1B1 (p.E229K), and TEK (p.I148T)::CYP1B1 (p.R368H) exhibit perturbed interaction. The mutations also diminished the ability of TEK to respond to ligand stimulation, indicating perturbed TEK signaling. Overall, our data suggest that interaction of TEK and CYP1B1 contributes to PCG pathogenesis and argue that TEK-CYP1B1 may perform overlapping as well as distinct functions in manifesting the disease etiology.

In vitro study of air bubble dynamics following pneumodissection of donor corneas and relationship of air bubble pattern with a peripheral paracentesis incision.

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.

Outcomes of endothelial keratoplasty in pseudophakic corneal oedema: with or without Descemet's membrane stripping.

AIMS: To evaluate and compare outcomes of Descemet's stripping endothelial keratoplasty (DSEK) with non-DSEK (nDSEK) in pseudophakic corneal oedema (PCE). METHODS: The study was a prospective, non-consecutive, comparative, interventional case series. Twenty-six patients with PCE were randomised into two groups. Group 1 comprised of those cases that had undergone conventional DSEK, and group 2 consisted of those that had nDSEK for PCE between 2010 and 2012. The data on demographics and clinical characteristics were collected from all patients. Any intraoperative and early and late postoperative complications were also noted. The primary outcome measure was postoperative functional success. Graft survival and complications were analysed as secondary outcomes. RESULTS: The median best-corrected visual acuities at 6 months postoperatively in groups 1 and 2 were 0.18 (IQR 0.10-0.30) and 0.44 (IQR 0.18-0.51), respectively (p=0.11). Corneal oedema cleared in all patients. During the early postoperative period, a patient in group 2 had lenticule detachment managed by rebubbling. CONCLUSIONS: nDSEK has outcomes similar to DSEK in PCE with advantages of decreased surgical time and avoidance of inadvertent pull on the posterior stromal fibres that may occur during Descemet's stripping.

Corneal endothelium in xeroderma pigmentosum: clinical specular microscopy study.

AIMS: Xeroderma pigmentosum is a condition caused due to a defective DNA repair mechanism when exposed to ultraviolet radiation. Many of the patients with this disorder develop severely oedematous cornea with varying degrees of anterior corneal haze, which necessitates a full-thickness keratoplasty or selective endothelial keratoplasty. Presence of corneal oedema suggests that these patients have a dysfunctional endothelium. The purpose of this study is to evaluate the corneal endothelium in the patients with xeroderma pigmentosum when clinical specular microscopy was feasible. METHODS: Thirteen patients with classic skin changes of xeroderma pigmentosum were included in the study conducted during January 2010-December 2012. An age-matched group of 13 volunteers were included as controls who were emmetropes without any history of ocular or systemic illness. Corneal endothelium was assessed using specular microscopy from the central clear area of cornea. RESULTS: The mean age of the patients with xeroderma pigmentosum was 16.6±7.2 years and that of the controls was 17.4±6.9 years (p=0.78). The number of analysed cells and endothelial cell density were significantly higher in controls (p<0.001), whereas the average cell area, coefficient of variation of cell area and maximum cell area were significantly higher in xeroderma pigmentosum (p≤0.007). CONCLUSIONS: The specular microscopic findings in patients with xeroderma pigmentosum are suggestive of an accelerated endothelial cell loss. It is pertinent that the treating physicians must be involved in emphasising proper ocular protection from ultraviolet radiation to prevent avoidable blindness from xeroderma pigmentosum.

Descemet Membrane Detachment in a Child With Anterior Megalophthalmos Managed Using Intracameral Perflouropropane (C3F8) Gas Injection.

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.