Case Report: Pediatric Ocular Ischemia and Neovascular Glaucoma in Neurofibromatosis Type 1.

SIGNIFICANCE: Neovascular glaucoma is an important subset of secondary glaucoma in neurofibromatosis patients. Vasculopathy of the ophthalmic circulation needs to be borne in mind while evaluating their etiology. PURPOSE: This study aimed to report the presentation, diagnostic work-up and management of an unusual case of neovascular glaucoma in a child. CASE REPORT: A 7-year-old boy presented with uniocular ischemic fundus and secondary neovascular glaucoma. Detailed family history and evaluation led to a diagnosis of familial neurofibromatosis type 1. Fundus fluorescein angiography revealed compromised retinal and choroidal circulations in the affected eye. Ocular ultrasound B scan and neuroimaging did not show any contributory lesions. Cardiovascular evaluation was within normal limits. Ophthalmic Doppler imaging revealed normal proximal ophthalmic arteries in both eyes; however, the central retinal artery of the affected eye showed low flow in its proximal part and absent flow in the distal part, as compared with the fellow eye showing regular flow until the optic disc margin. Corroborating the clinical, fundus fluorescein angiography and Doppler findings, a diagnosis of neurofibromatosis type 1-related vasculopathy of the distal ophthalmic artery was made. Poor visual prognosis for the affected eye was explained, and anterior retinal cryopexy along with cyclocryotherapy was performed to treat the neovascular glaucoma. CONCLUSIONS: Vasculopathy of the ophthalmic circulation is an important cause of neovascular glaucoma in neurofibromatosis patients. The morphology of Lisch nodules may be altered in an ischemic eye, and therefore, careful examination of the other eye and systemic evaluation is vital in such unusual scenarios.

Clinically useful smartphone ophthalmic imaging techniques.

Imaging devices in ophthalmology are numerous, and most of them are sophisticated and specialized for specific regions of the eye. In addition, these are fixed and involve close interaction of the patient and the examiner; therefore, simple, portable and tele facility-imbibed imaging tools can be considered optimal alternatives to routine exercises. In the last 10 years, utility of smartphones in ophthalmology is being continuously explored to unearth their potential benefits. In this direction, a smartphone device with/without simple attachments has been noted to aid in detailed, high-quality imaging of the ocular adnexa, cornea, angle, iris, lens, optic disc, and the retina including its periphery. In addition, such utility has also been extended in strabismology workup and intraocular pressure measurements. Hence, using these clinician friendly tools and techniques or by devising newer and more comprehensive tool kits, ophthalmic care can be well-managed with apt use of technology. Also, the smartphone companies are encouraged to collaborate with the medical experts to endeavor more, and help and serve the people better.

Improving long-term intraocular pressure and visual outcomes in eyes with aniridic glaucoma.

PURPOSE: To study the long-term intraocular pressure (IOP) and visual outcomes in treated aniridic glaucoma. METHODS: A retrospective chart review of patients with aniridia and glaucoma, with ≥ 2-year follow-up, was performed. Eyes in early glaucomatous stages were medically managed, while moderate-severe stage eyes underwent a trabeculectomy with mitomycin-c (MMC). Success was termed 'complete' when average final IOP was ≤ 18 mmHg without usage of glaucoma medications, and 'qualified' when with/without topical glaucoma therapy. A significant change in vision was defined as > 2-line change on Snellen vision chart or > 0.2 change in logMAR units in in either direction (better or worse). RESULTS: Thirty-five eyes of 20 patients were included. The mean duration of follow-up was 7.29 ± 5.75 years. Associated ocular anomalies were present in 19 eyes (54.29%). Twelve eyes (34.28%) were maintained on medical management, while 23 eyes (65.71%) had undergone a trabeculectomy with MMC. The mean baseline IOP was 31.46 ± 6.34 mmHg, and mean IOP on last follow-up was 13.25 ± 5.82 mmHg, p < 0.001. Seventy-five percent of the medically managed eyes achieved an IOP ≤ 18 mmHg. 52.17% and 95.65% of the surgically treated eyes achieved 'complete' and 'qualified' success respectively. The median best corrected visual acuity (BCVA) at baseline was 1.48(0.6-2) logMAR units and on final follow-up was 1.3 (0.48-5) logMAR units, p = 0.21. Fifty percent of the eyes remained stable, 35.71% showed an improvement and 14.29% a deterioration of > 0.2 logMAR units. Patients with a longer follow-up (> 10 years) and those who had undergone a trabeculectomy with MMC were more likely to show good IOP control (p = 0.003; p = 0.004 respectively). CONCLUSION: Aniridic glaucoma can be managed efficiently by medications in early glaucomatous neuropathy, and with trabeculectomy augmented with mitomycin-C and releasable sutures for more advanced glaucomas, offering favourable long-term IOP control, visual stability and safety.

Myopia and glaucoma progression among patients with juvenile onset open angle glaucoma: A retrospective follow up study.

OBJECTIVE: To identify the risk factors for glaucoma progression, especially the association with myopia, among treated juvenile open angle glaucoma (JOAG) patients. METHODS: Glaucomatous progression was analysed in the eyes of JOAG patients with at least 5-years of follow up in this retrospective study. Baseline variables such as age, inheritance pattern, baseline intraocular pressure (IOP), baseline central corneal thickness, visual acuity, baseline refractive error, spherical equivalent (SE) and duration of follow-up were noted. Stereoparametric global trend analysis and Moorfields Regression Analysis on confocal scanning laser ophthalmoscopy were used to detect progression. Variables associated with glaucoma progression, with respect to progressors (PG) and non-progressors (NPG) were analysed. Since both eyes of a patient were taken for analysis, a generalised estimating equation method was used to correct the bias. RESULTS: Among 74 eyes (37 subjects), glaucoma progression was noted in 11 eyes (14.9%) of 8 patients, with a median time to progression of 7.4 years (range 5-15.5 years). For myopes (SE ≤ -1.00 D), glaucoma progression was 18 times more likely than mild and no myopes (>-1DS) (p = 0.03, 95% CI: 1.14, 217.44). The prevalence of myopia in the JOAG, PG and NPG cohorts was 70.3%, 87.5%, and 65.5%, respectively. Myopia progression was noted at follow up in 70% patients. One-unit increase in baseline vertical cup disc ratio, 1 mmHg increase in IOP fluctuations and 1 dB year-1 depression of visual field were associated with 0.44, 0.06 and 0.07 D year-1 increases in the rate of myopia progression, respectively. CONCLUSIONS: JOAG progressors had a greater baseline myopic refraction and a faster myopia development over time. The development of myopia in JOAG eyes could be an indicator of glaucoma progression, and hence progressing myopic (≤-1 D) JOAG patients should be followed up more rigorously.

Severity of visual field defects in primary congenital glaucoma and their risk factors.

PURPOSE: The primary objective of the study was to assess the frequency and severity of visual field defects (VFD) in primary congenital glaucoma (PCG). The secondary objective was to ascertain any associated risk factors. METHODS: An ambispective review of patients with PCG on follow-up with a 'target' intraocular pressure (IOP) of ≤ 15 mmHg. Age, sex, laterality, duration of follow-up, baseline IOP, baseline cup-disc ratio (CDR), central corneal thickness (CCT), age during filtering surgery, second surgery if any, yearly IOP, glaucoma medications and best corrected visual acuity from 2013 (year 1) to the final review and final CDR were noted down. Children ≥ 5 years of age with best corrected visual acuity ≥ 6/60 were subjected to manual kinetic Goldmann perimetry, and visual field defects (VFD) were identified. RESULTS: Seventy-one of 90 eyes completed a reliable kinetic perimetry. The mean age of children was 12.34 ± 4.86 years, and the mean follow-up duration was 10.77 ± 4.69 years. Baseline IOP and CDR were 29.07 ± 8.83 mmHg and 0.66 ± 0.22, respectively. 86.67% of eyes underwent a trabeculotomy + trabeculectomy with mitomycin-C. Thirty-one eyes (34.44%) required a second surgery, 25 of which were bleb revisions and 3 trabeculectomies. Mean IOP and CDR during last visit were 10.23 ± 2.76 mmHg and 0.52 ± 0.25, p < 0.001 as compared with baseline. On Goldmann perimetry, 19 eyes, 26.76%, had defects, arcuate scotoma being most frequent. On the Fisher exact test, a baseline/final CDR > 0.8, undergoing just a trabeculectomy with MMC, needing ≥ 2 glaucoma medications on review or a repeat trabeculectomy was associated with greater severity of VFD. On univariate logistic regression, eyes that needed a bleb revision [OR, 95% CI 9.75 (2.66-35.67), p = 0.001], a repeat trabeculectomy with mitomycin-C [OR (CI) 18 (1.31-245.58), p = 0.03] and final CDR of > 0.8 [OR (CI) 23.1 (3.7-144.21), p = 0.001] were associated with VFD. On multivariable regression analysis, female sex [OR (CI) 18 (2.01-161.04), p = 0.01] was identified as the single most important risk factor for development of a VFD. CONCLUSION: At a 'target' IOP of ≤ 15 mmHg, 26.76% of PCG eyes manifested a VFD over 10 years. Baseline and/or final CDR > 0.8, necessity for ≥2 medications or a repeat glaucoma surgery, and female sex were identified as risk factors for development and greater severity of glaucomatous VFD.

Clinical and ASOCT evaluations of 'bleb-sparing epithelial exchange' in paediatric and adult dysfunctional blebs over 5 years.

PURPOSE: To evaluate the long-term outcome of 'bleb-sparing epithelial exchange' surgery for dysfunctional filtering blebs in paediatric and adult eyes. METHODS: Patients who had undergone bleb revision ≥ 5 years back and were on regular follow-up were included. Age, ocular diagnosis, details of primary filtering surgery including mitomycin-C (MMC) usage, indication and year of bleb revision were recorded. After bleb revision, the mean intraocular pressure (IOP), glaucoma medications and best corrected visual acuity (BCVA) up to 5 years were noted. On last review, clinical details, bleb characteristics and swept source anterior segment tomographic (SSOCT) assessment of bleb were recorded. Comparative analysis of paediatric and adult eyes was performed. MAIN OUTCOME MEASURES: Change in IOP and BCVA. 'Complete success' was defined as IOP ≥ 6 to ≤ 18mmHg without use of any topical glaucoma medications and 'Qualified success' when ≥ 1 topical glaucoma medication(s) was required. RESULTS: A total of 51 eyes of 51 consecutive patients were studied, among which 22 were children. The mean duration between filtering surgery and bleb revision was 4.54 ± 1.53 years in paediatric and 6.48 ± 3.5 years in the adult group, p = 0.04. Children underwent trabeculotomy + trabeculectomy with 0.04% MMC, while adults underwent trabeculectomy with 0.02% MMC. The mean pre-revision IOP was 6.38 ± 2.80 and 6.51 ± 2.78 mmHg in the paediatric and adult group respectively, p = 0.86. At 3 months post-revision, it increased to 11.81 ± 3.48 and 12.75 ± 3.52 mmHg respectively (p < 0.001). At final review, mean IOP of paediatric group was 10.90 ± 2.59 and adult group was 11.86 ± 2.66 mmHg, p = 0.20. At 5 years, complete success was 68.18% and 72.41%, and qualified success was 31.87% and 27.59% in the former and latter group respectively, p = 0.49. No failures were seen. Kaplan-Meier probability at 5 years for IOP target ≤ 18, ≤ 15 and ≤ 12 in children was 95.45%, 63.64% and 50% and in adults 93.10%, 65.52% and 41.38% respectively. BCVA improved up to 1 year in paediatric group, with continued improvement in adults up to 3 years. SSOCT measured bleb height was 0.88 ± 0.37 and 1.32 ± 0.49mm in children versus adults (p = 0.006) and wall thickness, 0.35 ± 0.22 and 0.58 ± 0.24mm respectively, p = 0.008. CONCLUSION: Bleb-sparing epithelial exchange is an equally safe and effective technique with good long-term success in both paediatric and adult dysfunctional blebs.

COMPARATIVE EVALUATION OF ANTERIOR SEGMENT OPTICAL COHERENCE TOMOGRAPHY, ULTRASOUND BIOMICROSCOPY, AND INTRAOCULAR PRESSURE CHANGES AFTER PANRETINAL PHOTOCOAGULATION BY PASCAL AND CONVENTIONAL LASER.

PURPOSE: To compare intraocular pressure, anterior segment optical coherence tomography, and ultrasound biomicroscopy parameters over 3 months after panretinal photocoagulation (PRP) for proliferative diabetic retinopathy after 1 of 2 sittings by conventional laser (half PRP) and a single sitting of Pattern Scan Laser (PASCAL) PRP. METHODS: This was a prospective, randomized, interventional study. All tests were performed at baseline, and at 1, 6, and 24 hours, and 1, 4, 8, and 12 weeks after PRP. RESULTS: The intraocular pressure at 1 hour and 6 hours after PRP was significantly raised in both groups. Mean intraocular pressure was 21.17 ± 4.01 mmHg after PASCAL and 17.48 ± 3 mmHg after conventional laser at 1 hour, P < 0.001. On anterior segment optical coherence tomography, conventional laser PRP caused a more significant narrowing of angle-opening distance (AOD750) and trabecular-iris space area (TISA 500), P = 0.03 and 0.04, respectively, on Day 1. Ultrasound biomicroscopy showed a significantly narrow angle in both groups on Day 1. A significant increase in ciliary body thickness was observed in both groups, with 57.1% of PASCAL and 100% of conventionally treated eyes showing ciliary effusion on Day 1 that decreased but persisted for the next 3 months. CONCLUSION: Performing PRP in sittings, prescribing previous glaucoma medications in patients at risk, and recording intraocular pressure an hour after the PRP could decrease complications.

Case Report: Cyclodialysis Cleft in a Case of Open-globe Injury and Role of Swept-source Anterior Segment Optical Coherence Tomography in Diagnosis.

SIGNIFICANCE: Cyclodialysis clefts can potentially develop secondary to open globe injury. The swept-source anterior segment optical coherence tomography (SS-AS-OCT) may be a valuable diagnostic tool for the identification and estimation of the circumferential extent of cyclodialysis clefts. It could be considered an alternative when ultrasound biomicroscopy cannot be performed successfully. PURPOSE: The purpose of this study was to report a case of open-globe injury associated with cyclodialysis cleft and the utility of SS-AS-OCT in its diagnosis. CASE REPORT: A 12-year-old boy presented to the clinic because of penetrating ocular trauma to his left eye with a projectile stone. He was diagnosed with limbal perforation with uveal tissue prolapse and cataract. He underwent limbal repair with cataract extraction and posterior chamber intraocular lens implantation. However, even at the 6 weeks' post-operative period, he did not gain vision and had persistent hypotony with hypotonic maculopathy. Gonioscopy showed a 2-clock-hour superonasal cyclodialysis cleft. However, on SS-AS-OCT, it was discovered that the cleft extended along 5 clock hours, involving both superonasal and inferonasal quadrants. Recognizing the large extent of the cleft, endocyclopexy by modified sewing-machine technique was planned and performed. An IOP spike and improvement in vision were noted on the next post-operative day. The SS-AS-OCT confirmed cleft closure. CONCLUSIONS: Although rare, cyclodialysis can occur in cases of open globe injury. The SS-AS-OCT is a useful diagnostic tool to study the circumferential extent of cyclodialysis and may unravel detachments hidden behind intact anterior ciliary body face.

Deep blue dot corneal degeneration: confocal characteristics.

PURPOSE: To discuss the clinical features, differential diagnosis and the novel confocal microscopic findings noted in the rare 'deep blue dot corneal degeneration'. METHODS: Observational case report. RESULTS: Slit-lamp biomicroscopic examination revealed bilateral, numerous, circular to oval discrete blue opacities at the level of deep stroma and fine grey linear opacities at the level of mid to deep stroma. Confocal microscopy demonstrated two types of corresponding hyper-reflective extracellular lesions: oval deposit-like, most concentrated at a depth of 430-480 µ and needle-like at the depth 330-370 µ. CONCLUSIONS: Deep blue dot corneal degeneration is a rare entity where blue deposits of amyloid are seen in the deep corneal stroma. It should be considered as a differential diagnosis when an old-aged person presents with good vision and the above mentioned findings.

Hook and flip technique: for phacoemulsification in non-rotating nuclei and posterior polar cataracts.

PURPOSE: We put forward a physical levitation method to hook and flip the chopped nuclear fragments that could not be solely drawn by vacuum during phacoemulsification, due to various reasons such as a non-rotating nuclei or posterior polar cataracts where hydrodissection was unsuccessful or contra-indicated, respectively. METHOD: A Sinskey hook is insinuated through the crack of the divided nuclei into a plane behind the nuclear pie to 'hook and flip' the chopped piece, heading it towards the phacoemulsification probe. This simple step disassembles the nuclear chunk, thereby creating space to facilitate the dismantling of the rest of the fragments. The remnant epinuclear cushion guards the posterior capsule, mitigating the chances of serious intra-operative complications. RESULT: We have employed this technique in 17 eyes during similar situations. No specific intra-operative complications were observed; all surgeries were uneventful. A Sinskey hook utilised for this step ensures safety and familiarity, none encountered posterior capsular rent. This technique not only eases the surgery, but also decreases the anticipated intra-operative and post-operative complications. CONCLUSION: 'Hook and flip technique' thus proves useful whenever dismantling difficulties are encountered during phacoemulsification.

Long-term evaluation of ocular hypertension with primary angle closure and primary open angles.

PURPOSE: To evaluate the long-term course of primary angle-closure ocular hypertension and primary open-angle ocular hypertension and possible risk factors for progression to glaucoma. METHODS: A total of 109 eyes of 109 ocular hypertension (OHT) patients with a minimum follow-up period of 5 years having complete ocular/medical records were evaluated. They were classified into primary angle closure or primary open angle based on gonioscopy at baseline. Baseline and review data of Humphrey field analyser, HFA, and Heidelberg retinal tomography, HRT, were recorded. Guided progression analysis (GPA) and univariate Cox regression were used for time to event analysis in identifying progression to glaucoma. RESULTS: Over a mean follow-up of 12.18 ± 4.8 years, progression to glaucoma was 17.43% (19 eyes), out of whom 5.5% (6 eyes) showed ≥ 3 loci on GPA. Sub-classifying them, progression to primary angle-closure glaucoma was 19.72%, and that of primary open-angle glaucoma was 13.16%. The mean time to progression was 9.34 ± 3.6 years. Significant risk factors included small disc area (≤ 1.99 sq.mm on HRT), requirement of ≥ 2 drugs to maintain target IOP and those engaged in activities yielding a Valsalva effect in daily life. Coronary artery disease (CAD) and systemic use of steroids were associated with increased severity. CONCLUSION: Overall progression of OHT to glaucoma was 17.43% over a mean of 9 years, with target IOP of ≤ 18 mm Hg. Patients with smaller discs, CAD, exercising Valsalva type activities and using ≥ 2 glaucoma medications or systemic steroids should be closely monitored.

Triple chamber: a clinical rarity after deep anterior lamellar keratoplasty and role of optical coherence tomography in management.

PURPOSE: To report a case demonstrating triple chamber following deep anterior lamellar keratoplasty (DALK) and its successful intra-operative optical coherence tomography-guided management. METHOD: Case report of a young male with macular corneal dystrophy, who underwent DALK in his left eye by Big-Bubble technique. The surgery was uneventful. On the first post-operative day, triple chamber was observed and followed-up with serial clinical photography and anterior segment optical coherence tomography. Due to decrease in graft clarity and increase in volume of the two extra chambers, interface drainage along with descemetopexy was undertaken 4 days later. RESULT: The compartments constituting the triple chamber were those in-between the donor tissue and host pre-Descemet layer (Dua's layer), the latter and host Descemet membrane and the true anterior chamber. Presence of viscoelastic in the interface was identified as the cause. Microscope integrated optical coherence tomography (MiOCT) guided drainage followed by intracameral air tamponade ensured near total disappearance of the two extra chambers at the end of surgery. Examination on the next day confirmed complete apposition of the graft and host. CONCLUSION: To the best of our knowledge, this is a unique demonstration of Dua's layer in vivo by slit lamp biomicroscopy and description of MiOCT guided management of triple chamber.

Vitreous Amyloidosis: Ocular, Systemic, and Genetic Insights.

PURPOSE: To report the unique clinical and surgical characteristics encountered in eyes with vitreous amyloidosis. Systemic evaluation and visual outcome after vitrectomy are discussed. A novel mutation in the transthyretin gene (TTR) in Indian patients with familial amyloid polyneuropathy (FAP) is described. DESIGN: Retrospective, observational study. PARTICIPANTS: Ten eyes of 5 patients from 2 pedigrees with a diagnosis of vitreous amyloidosis. METHODS: Detailed history, pedigree charting, systemic and ocular examination of 10 eyes (5 patients from 2 pedigrees) were carried out. Tests were performed to rule out vitreitis, retinal vasculitis, vitreous hemorrhage, and systemic amyloidosis. Genetic analysis to identify the mutation was performed in 1 patient. Vitreous biopsy, followed by 25-gauge pars plana vitrectomy, was performed in the same sitting in all cases. Samples were sent for Congo red staining and polarized microscopy. Patients were followed up on days 1, 7, and 28 and then every 2 months. Visual acuity assessment, intraocular pressure measurement, and fundus examination were performed each time. MAIN OUTCOME MEASURES: Mutations in TTR and postoperative visual acuity. RESULTS: Mean age at presentation was 32 years, with a 3:2 male-to-female distribution. Family history was positive in all patients. Nine eyes had pseudopodia lentis, whereas all 10 had glass wool-like vitreous. Glaucoma developed in 1 patient (2 eyes). Waxy paper-like vitreous with firm vitreous adhesions beyond major arcades and along retinal vessels was noted during surgery in all eyes. Congo red staining and apple green birefringence demonstrated vitreous amyloidosis. The mean preoperative best-corrected visual acuity (BCVA) was 1.39±0.64 logarithm of the minimum angle of resolution (logMAR), whereas the postoperative BCVA improved to 0.17±0.07 logMAR (P = 0.004). Gene sequencing revealed a phenylalanine→isoleucine mutation in the 33rd position of exon 2 of TTR in 1 patient of 1 pedigree, confirming the diagnosis of FAP. Two patients subsequently were found to have sensorimotor autonomic neuropathy, whereas 2 others had subclinical autonomic dysfunction. CONCLUSIONS: The clinical clues, management strategy, surgical characteristics, vitrectomy outcomes, and significance of systemic evaluation in vitreous amyloidosis are highlighted. A novel single mutation (Phe33Ile) in a case of FAP with vitreous amyloidosis from India is reported.

Bilateral Aggressive Mooren Ulcer in the Setting of Bilateral Pterygia and Pregnancy: A Unique Case.

PURPOSE: To report an unusual case of bilateral aggressive Mooren ulcer that occurred in the setting of bilateral pterygia and showed a relentless course during pregnancy. METHODS: A 39-year-old woman of Black African ethnicity, 36-week pregnant, presented to the eye casualty with bilateral nasal corneal ulcer and associated melt around preexisting pterygia. A detailed workup including microbial evaluation, culture and sensitivity, polymerase chain reaction for herpes simplex virus, varicella zoster virus, and cytomegalovirus, inflammatory blood profile, autoimmune markers, and human leucocyte antigen (HLA) screening was undertaken. Treatment was initiated in a stepwise approach. RESULTS: Infections and systemic autoimmune and rheumatologic conditions were ruled out. A diagnosis of bilateral Mooren ulcer was made by exclusion. The peripheral blood was positive for HLA DQ2. As the condition seemed refractory to medical management (topical steroids and intravenous pulse methylprednisolone followed by oral prednisolone and topical cyclosporine), urgent bilateral conjunctival resection with multilayered amniotic membrane transplantation was performed to reduce the inflammatory stimulus and keratolysis. Stabilization of the condition warranted the need for systemic immunosuppressive agents. Using a multidisciplinary approach, in liaison with Obstetricians and Rheumatologists, the patient was planned for an earlier elective Cesarean section and commencement of oral mycophenolate mofetil postpartum, which aided in successful control of the disease. CONCLUSIONS: Mooren ulcer could follow an aggressive course during pregnancy, especially in the setting of preexisting pterygium. The complex hormonal and immunological changes during pregnancy and the delivery of inflammatory mediators directly onto the cornea by pterygium could contribute to the severity. A well-planned, stepwise, and multidisciplinary management is pivotal for the treatment of this condition.

Baerveldt Aqueous Shunt with or without Mitomycin C Augmentation: A Retrospective Comparison Study.

OBJECTIVE: To evaluate if intraoperative mitomycin C (MMC) influences the success of Baerveldt aqueous shunts. DESIGN: Retrospective comparative case series. PARTICIPANTS: The study population consisted of 88 patients. Fifty-five received intraoperative MMC and 33 did not (controls). METHODS: The medical records of consecutive patients who underwent standalone Baerveldt aqueous shunts at Birmingham Midland Eye Centre, United Kingdom, were retrospectively reviewed. Patients in the MMC group received 0.2 to 0.4 mg/mL of MMC intraoperatively whereas controls did not. MAIN OUTCOME MEASURES: Primary outcome was survival, which was defined as an intraocular pressure (IOP) > 6 mmHg and ≤ 21 mmHg or ≤ 18 mmHg and > 20% IOP reduction from baseline. Further analysis of patients who required medications (qualified) or no medications (complete) was undertaken. Secondary outcomes were IOP, number of glaucoma medications, complications, intraluminal ripcord removal (IRR), and interventions. RESULTS: Average follow-up was 4.7 ± 1.4 years. At year 5, complete success with the ≤ 21 mmHg threshold was significantly higher in MMC vs controls (39.3% vs 17.8%; log rank P = 0.016). Final complete success with the ≤ 18 mmHg threshold was higher in patients with MMC shunts vs controls (38% vs 15.6%; log rank P = 0.0042). Qualified success was not different between patients with MMC shunts and controls with ≤ 21 mmHg (82% vs 93%; log rank P = 0.29) and ≤ 18 mmHg thresholds (70.3% vs 79.3%; log rank P = 0.44). Uveitic patients were also more likely to achieve complete success at both 21 and 18 mmHg thresholds among the patients receiving MMC compared with controls. Mitomycin C was correlated with lower number of medications between month 3 and year 2 post operatively (P < 0.001) and with a lower rate of IRR at all timepoints (P < 0.001). There were no significant differences in the incidence of prolonged hypotony, although MMC cases had higher transient hypotony at year 1 (P = 0.049). CONCLUSIONS: Mitomycin C provides a significant advantage in Baerveldt aqueous shunt survival when considering medication-free success but not in qualified success. Control patients required more medications to control IOP. This study suggests that intraoperative MMC augmentation of Baerveldt aqueous shunt surgery may be advantageous in achieving IOP control without the need for medication but that it may be associated with more transient hypotony episodes. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

The high-pressure bleb.

A 45-year-old male presented with a slowly progressive, painless swelling in his right eye for the past 6 months. He had undergone an open globe injury repair 10 years back and an eventful cataract surgery 5 years back. The presenting visual acuity in the affected eye was perception of light with inaccurate projection of rays. The intraocular pressure was 44 mm Hg with advanced glaucomatous cupping. The swelling was identified to be a communicating sub-tenon cyst secondary to scleral wound dehiscence from secondary angle closure glaucoma. Wound re-suturing, cyst excision and diode laser cyclophotocoagulation was performed in a single sitting, with explained poor visual prognosis.

Juvenile-onset open-angle glaucoma - A clinical and genetic update.

Juvenile-onset open-angle glaucoma (JOAG) is a subset of primary open-angle glaucoma that is diagnosed before 40 years of age. The disease may be familial or non-familial, with proportions varying among different populations. Myocilin mutations are the most commonly associated. JOAG is characterized by high intraocular pressures (IOP), with many patients needing surgery. The mean age at diagnosis is in the 3rd decade, with a male preponderance. Myopia is a common association. The pathophysiology underlying the disease is immaturity of the conventional outflow pathways, which may or may not be observed on gonioscopy and anterior segment optical coherence tomography. The unique optic nerve head features include large discs with deep, steep cupping associated with high IOP-induced damage. Progression rates among JOAG patients are comparable to adult primary glaucomas, but as the disease affects younger patients, the projected disability from this disease is higher. Early diagnosis, prompt management, and life-long monitoring play an important role in preventing disease progression. Gene-based therapies currently under investigation offer future hope.