Encapsulated bleb with scleral fistula - A diagnostic dilemma treated by a simple procedure

We present a case of post-trabeculectomy encapsulated dysesthetic bleb with scleral fistula, managed successfully with autograft. The child was operated on twice before for trabeculectomy, and intraocular pressure (IOP) recorded was in the normal range for the initial few years. This time child presented with a large encapsulated dysesthetic bleb with borderline IOP. As the IOP was on the lower side, an underlying scleral fistula was suspected and planned for bleb revision with a donor patch graft. We describe the novel technique of bleb revision along with the repair of the scleral fistula with an autologous free fibrotic Tenon’s tissue graft instead of a donor patch graft with a successful outcome.

Evaluation of visual field changes with retinal nerve fiber layer thickness in primary congenital glaucoma

Purpose: To evaluate visual field changes in primary congenital glaucoma (PCG) with retinal nerve fiber layer thickness on optical coherence tomography. Methods: In this cross?sectional, observational study, consecutive PCG children who underwent combined trabeculotomy with trabeculectomy and on regular follow?up were enrolled. All patients were aged over four years and co?operative for RNFL OCT and visual field examination. Perimetry was done on Humphrey visual field (HVF) analyzer using 30?2 and 10?2 SITA standard algorithms as appropriate. If a reliable automated perimetry was not feasible, kinetic perimetry was done. The following were noted at baseline and every follow?up: age, sex, visual acuity, intraocular pressure (IOP), cup–disc ratio (CDR), corneal diameters, refraction, any topical antiglaucoma medications, surgeries underwent, age at surgery and duration between surgery and final examination. Results: Forty?eight eyes of 34 children operated for PCG and 19 eyes of 17 controls were analyzed. A statistically significant thinner average RNFL thickness of 87.2 ± 28 ?m was noted in PCG eyes as compared to controls with 100.6 ± 7.2 ?m (P = 0.04). The mean cup–disc area ratio on OCT in PCG eyes was 0.43 ± 0.2 (0.02–0.93) and in control eyes was 0.23 ± 0.07 (0.1–0.4) (P < 0.001). On RNFL OCT, there was significant focal RNFL loss in temporal superior (P = 0.003), nasal inferior (P = 0.037) and temporal inferior (P < 0.001) quadrants compared to controls. Among PCG eyes, 20/48 eyes (41.7%), had definitive, reproducible glaucomatous VF defects. Mean baseline IOP in PCG eyes with VF defect was 28.7 ± 5.7 mmHg and in eyes with normal VF was 24.6 ± 5.9 mmHg (P = 0.03). On univariate regression analysis, higher baseline IOP was significantly associated with both RNFL loss (odds ratio (OR): ?2.17) and VF defects (OR: 3.35). Fluctuation in follow?up IOP (OR: 3.33) was also significantly associated with the presence of VF defects. On multivariable regression analysis maximum, IOP was significantly associated with RNFL loss and VF defects. Conclusion: Peripapillary RNFL thickness could be used to identify PCG eyes having visual field loss and possibly poor visual function from PCG eyes without visual field defects. Baseline and follow?up IOP, significantly correlated with RNFL thickness in PCG eyes

Impact of initial topical medical therapy on short‑term quality of life in newly diagnosed patients with primary glaucoma.

Purpose: To evaluate the impact of initial topical medical therapy on newly diagnosed glaucoma patients using the Indian Vision Function Questionnaire (IND-VFQ33). Patients and Methods: The IND-VFQ33 was used to evaluate the quality of life (QoL) in 62 newly diagnosed patients with moderate to severe primary glaucoma and 60 healthy controls. IND-VFQ33 is a 33 item QoL assessment tool with three domains: General functioning, psychosocial impact and visual symptoms. The glaucoma patients were started on medical therapy and the QoL assessment was repeated after 3 months. Results: Glaucoma patients (mean age: 55.6 ± 9.6 years, range 40–77 years) and controls (mean age: 54.9 ± 6.7 years, 42–73 years) were matched with respect to age (P = 0.72), gender (P = 0.91) and literacy (P = 0.18). Glaucoma patients had significantly worse QoL as compared to controls at baseline across all the three domains (P < 0.001). 3 months after initiation of treatment, the overall QoL life significantly worsened from baseline with a decrease in general functioning (P < 0.001) and psychosocial impact (P = 0.041). Visual acuity in better eye significantly co-related to poor QoL at baseline (P < 0.001) and at 3 months (P = 0.04). In addition, the use of >2 topical medications significantly co-related to poor QoL at 3 months (P = 0.01). Conclusions: Evaluation using the IND-VFQ33 revealed that newly diagnosed glaucoma patients have a significant worsening of QoL after initiation of topical ocular hypotensive therapy. This should be an important consideration when educating patients about the disease and its therapy.

Evaluation of ExPress glaucoma filtration device in Indian patients with advanced glaucoma.

ExPress glaucoma filtration device (GFD) has recently become available in India as a surgical option for glaucoma patients. We retrospectively evaluated the outcome of ExPress GFD in 12 eyes with advanced glaucoma with intraocular pressures (IOPs) not controlled on maximal tolerable medical therapy. The mean preoperative IOP of 29.58 ± 7.13 mmHg decreased to 17.0 ± 2.67 and 17.40 ± 0.89 mmHg at 6 and 12 months after surgery. Absolute success (IOP ≤ 18 mmHg, with no additional glaucoma medications) was achieved in eight cases (66.7%) and qualified success (IOP ≤ 18 mmHg, with additional glaucoma medications) in two cases (16.7%) at 1‑year after surgery. Early intervention was needed in 4 patients; two underwent anterior chamber reformation while the other two required needling. Two patients required resurgery. There was no significant change in the best corrected visual acuity postoperatively (P = 0.37). ExPress GFD does not seem to offer a benefit over standard trabeculectomy in patients with advanced glaucomatous disease in terms of IOP control or complication rate. However, due to the small sample size with a heterogeneous mixture of primary and secondary glaucoma’s, we await further studies with a larger sample size and long‑term follow‑up, to see how the device performs.

Spinocerebellar ataxia 7 (SCA7) in Indian population: predilection of ATXN7-CAG expansion mutation in an ethnic population.

Background & objectives: spinocerebellar ataxia 7 (SCA7) is a rare form of neurodegenerative disorder with the clinical manifestation of cerebellar ataxia and retinal degeneration. In this study we describe the clinico-genetic characteristics of nine SCA7 families of Indian origin and cross compare these with other available worldwide studies. Methods: Thirty five individuals from nine SCA7 families were clinico-genetically characterized and CAG repeat distribution analysis was carried out in 382 control DNA samples from healthy controls (derived from 21 diverse Indian populations based on ethnic and linguistic and geographical location). Results: Of the nine families studied, 22 affected individuals and one asymptomatic carrier were identified. The average age at disease onset was 23.4±12.6 yr. The length of expanded CAG ranged from 40-94 with mean value of 53.2±13.9. The main clinical findings in affecteds individuals included cerebellar ataxia, and retinal degeneration along with hyper-reflexia (95%), slow saccades (85%) and spasticity (45%). Analysis of the association of number of CAG repeats with disease onset revealed that <49 repeats were associated with earlier age at onset in South East Asians compared to European populations. Further analysis of CAG repeats from 21 diverse Indian populations showed pre-mutable repeats (28-34) alleles in the IE-N-LP2 population. Six of the nine families identified in this study belonged to the same ethnic population. Interpretations & conclusion: Our results show that presenece of SCA7 is relatively rare and confined to one ethnic group from Haryana region of India. We observed a homogeneous phenotypic expression of SCA7 mutation as described earlier and an earlier age of onset in our patients with CAG <49. The identification of pre-mutable allele in IE-N-LP2 suggests this population to be at the risk of SCA7.

Socioeconomics of long‑term glaucoma therapy in India.

Purpose: To determine the socioeconomic impact of long‑term glaucoma therapy. Materials and Methods: One hundred and fifty consecutive glaucoma patients on medical therapy, following up at our glaucoma service for at least 6 months were recruited. A questionnaire regarding monthly income, cost of glaucoma medications prescribed, availability of medications, travel time, time spent in review clinics, compliance, education status, medical insurance and systemic or local side‑effects was administered. Results: The patients seen at the tertiary government hospital had an average monthly income of Rs. 10,912/‑ (range: Rs. 500/‑ to Rs. 50,000/‑) with approximately 56% of the patients having an income of less than Rs. 5000/month. The expenditure on anti‑glaucoma medications ranged from 0.3% in high income group to 123% of their monthly gross income in low income group (P < 0.0001). The total expenditure including travel, stay, and loss of wages of patients and accompanying persons ranged from 1.6% in high income group to 137% of the monthly income in low income group (P < 0.0001). Mean time required for a glaucoma clinic visit was 15.66 h, (range: 6–96 h/month). About 2.7% experienced systemic side‑effects and 21.3% had complaints of ocular adverse effects. About 90% of the patients were compliant. 92% were not covered by any insurance plan/government reimbursement for their treatment. Conclusions: Medical therapy for glaucoma is an economic burden to many patients and should be individualized, according to the socioeconomic status, availability of drugs and the required distance to travel to reach the specialist clinics.

Scanning laser polarimetry in glaucoma.

Glaucoma is an acquired progressive optic neuropathy which is characterized by changes in the optic nerve head and retinal nerve fiber layer (RNFL). White‑on‑white perimetry is the gold standard for the diagnosis of glaucoma. However, it can detect defects in the visual field only after the loss of as many as 40% of the ganglion cells. Hence, the measurement of RNFL thickness has come up. Optical coherence tomography and scanning laser polarimetry (SLP) are the techniques that utilize the evaluation of RNFL for the evaluation of glaucoma. SLP provides RNFL thickness measurements based upon the birefringence of the retinal ganglion cell axons. We have reviewed the published literature on the use of SLP in glaucoma. This review elucidates the technological principles, recent developments and the role of SLP in the diagnosis and monitoring of glaucomatous optic neuropathy, in the light of scientific evidence so far.

Novel occurrence of Axenfeld: Rieger syndrome in a patient with blepharophimosis ptosis epicanthus inversus syndrome.

is a complex eyelid malformation characterized by the classical tetrad of blepharophimosis, telecanthus, ptosis, and epicanthus inversus. It has been reported to be associated with other ocular anomalies such as euryblepharon, strabismus, nystagmus, amblyopia, microphthalmos, lacrimal drainage apparatus abnormality, extra ocular muscle abnormalities, microcornea, trabecular dysgenesis, optic nerve hypoplasias, and colobomas of the optic disk. We describe a case of BPES with Axenfeld–Rieger syndrome, a neurocristopathy characterized by maldevelopment of the anterior segment with predisposition to development of glaucoma. Interestingly, both syndromes are caused by mutations in the same class of genes, namely the winged‑helix/ forked transcription factors (FOX) involved in a variety of developmental processes.

Test retest variability of TonoPen AVIA.

The study was conducted to evaluate the intra-session repeatability of Tonopen AVIA (TPA). 180 eyes of 180 patients (50 eyes with glaucoma, 130 eyes of controls) were recruited for this observational study. The mean age of patients enrolled in the study was 43.9 ± 16.7 yrs (84 males, 96 females). Mean IOP recorded with Tonopen AVIA was 19.5 ± 9.5 mmHg, 19.4 ± 9.6 mmHg and 19.3 ± 9.2 mmHg, respectively in the first, second and third instances (P = 0.656). The intraclass correlation coefficient (ICC) ranged from 0.996 (95% CI: 0.956 - 0.998) for glaucoma subjects to 0.958 (95% CI: 0.934 - 0.975) for controls. The coefficient of variation in the study population ranged from 3.47% (glaucoma patients) to 8.10% (healthy controls), being 6.07% overall. The coefficient of repeatability varied between 2.96 (glaucoma patients), 3.35 (healthy controls) to 3.24 (overall). Thus, the Tonopen Avia shows good intrasessional repeatability of IOP in both glaucomatous patients and healthy subjects.

Prevalence of plateau iris configuration in primary angle closure glaucoma using ultrasound biomicroscopy in the Indian population.

Purpose: To report the prevalence of plateau iris in patients with primary angle closure glaucoma (PACG), in North India. Materials and Methods: The patients with PACG, attending the glaucoma services at a tertiary care center in North India were included in the study. All patients had undergone Nd-YAG laser peripheral iridotomy at least four weeks prior to inclusion in the study. Four weeks prior to inclusion in the study, none of the patients had used pilocarpine. Ultrasound Biomicroscopy (UBM) images were qualitatively evaluated and plateau iris configuration was defined in an eye if the following criteria were fulfilled in two or more quadrants: anteriorly directed ciliary process supporting the peripheral iris, steep rise of iris root from its point of insertion followed by a downward angulation from the corneoscleral wall, absent ciliary sulcus, and iridotrabecular contact in the same quadrant. Results: One hundred and one eyes were included in the study. There were 63 (62.4%) females and 38 (37.6%) males. The mean age of the patients was 57.8 ± 9.5 years (range: 42 to 78 years). The mean axial length in the study population was 22.2 ± 1.1 mm. The mean spherical equivalent refraction was 0.06 ± 1.12 D. The mean intraocular pressure was 18.5 ± 4.7 mmHg (range: 12 – 24 mmHg). Twenty-nine (28.7%) subjects were diagnosed with plateau iris on the basis of above-defined criteria. Of the 29 eyes, 18 (62.1%) subjects had plateau iris in two quadrants, nine (31.03%) in three quadrants, and two (6.8%) had this configuration in all the four quadrants. Conclusions: Approximately 30% of the eyes with PACG had plateau iris on UBM. Plateau iris was very often the cause for residual angle closure following laser peripheral iridotomy in Indian eyes with PACG.

Ahmed glaucoma valve in post-penetrating-keratoplasty glaucoma: A critically evaluated prospective clinical study.

Aim: The aim was to evaluate the outcome of Ahmed glaucoma valve (AGV) in post-penetrating-keratoplasty glaucoma (PKPG). Materials and Methods: In this prospective study, 20 eyes of 20 adult patients with post-PKPG with intraocular pressure (IOP) >21 mmHg, on two or more antiglaucoma medications, underwent AG (model FP7) implantation and were followed up for a minimum of 6 months. Absolute success was defined as 5 <IOP <21 mmHg and qualified success as 5 <IOP <21 mmHg with medications or minor procedures. Results: The mean IOP decreased from 42.95 ± 10.24 to 17.69 ± 3.64 mmHg (P <0.001) and the use of medications dropped from 2.92 to 0.39 (P <0.001) after AGV implantation. The absolute success was achieved in 11 eyes and qualified success in 9. There was no significant change in best corrected visual acuity, graft clarity, or graft thickness. Six device-related complications occurred after AGV implantations which were successfully managed with medical or minor surgical therapy. Conclusions: Postkeratoplasty refractory glaucoma managed by AGV implantation revealed a satisfactory outcome up to 6 months of follow-up.

Comparison of the diagnostic ability of Moorfield’s regression analysis and glaucoma probability score using Heidelberg retinal tomograph III in eyes with primary open angle glaucoma.

Purpose: To compare the diagnostic performance of the Heidelberg retinal tomograph (HRT) glaucoma probability score (GPS) with that of Moorfield’s regression analysis (MRA). Materials and Methods: The study included 50 eyes of normal subjects and 50 eyes of subjects with early-to-moderate primary open angle glaucoma. Images were obtained by using HRT version 3.0. Results: The agreement coefficient (weighted k) for the overall MRA and GPS classification was 0.216 (95% CI: 0.119 – 0.315). The sensitivity and specificity were evaluated using the most specific (borderline results included as test negatives) and least specific criteria (borderline results included as test positives). The MRA sensitivity and specificity were 30.61 and 98% (most specific) and 57.14 and 98% (least specific). The GPS sensitivity and specificity were 81.63 and 73.47% (most specific) and 95.92 and 34.69% (least specific). The MRA gave a higher positive likelihood ratio (28.57 vs. 3.08) and the GPS gave a higher negative likelihood ratio (0.25 vs. 0.44).The sensitivity increased with increasing disc size for both MRA and GPS. Conclusions: There was a poor agreement between the overall MRA and GPS classifications. GPS tended to have higher sensitivities, lower specificities, and lower likelihood ratios than the MRA. The disc size should be taken into consideration when interpreting the results of HRT, as both the GPS and MRA showed decreased sensitivity for smaller discs and the GPS showed decreased specificity for larger discs.

Pars plana Ahmed glaucoma valve implantation with triamcinolone-assisted vitrectomy in refractory glaucomas.

Glaucoma drainage devices are an option in refractory glaucomas for control of intraocular pressure (IOP). We evaluated the outcome of pars plana Ahmed glaucoma valve along with triamcinolone-assisted vitrectomy in 11 eyes with uncontrolled IOP on maximum tolerable antiglaucoma therapy. The mean preoperative IOP of 33.64 ± 5.99 (range 26 to 44 mmHg) decreased to 17.09 ± 2.26 (range 14 to 20 mmHg) and 17.45 ± 1.81mm of Hg (range 14 to 24 mmHg) at 6 and 12 months following surgery. The mean number of antiglaucoma medications decreased from 3.27 ± 0.05 to 0.64 ± 0.67 and 0.55 ± 0.6 at 6 and 12 months following surgery.

Effect of cataract surgery on retinal nerve fiber layer thickness parameters using scanning laser polarimetry (GDxVCC).

Purpose: To study the effect of cataract extraction on the retinal nerve fiber layer (RNFL) thickness, and assessment by scanning laser polarimetry (SLP), with variable corneal compensation (GDx VCC), at the glaucoma service of a tertiary care center in North India. Materials and Methods: Thirty-two eyes of 32 subjects were enrolled in the study. The subjects underwent RNFL analysis by SLP (GDx VCC) before undergoing phacoemulsification cataract extraction with intraocular lens (IOL) implantation (Acrysof SA 60 AT) four weeks following cataract surgery. The RNFL thickness parameters evaluated both before and after surgery included temporal, superior, nasal, inferior, temporal (TSNIT) average, superior average, inferior average, and nerve fiber index (NFI). Results: The mean age of subjects was 57.6 ± 11.7 years (18 males, 14 females). Mean TSNIT average thickness (μm) pre- and post-cataract surgery was 49.2 ± 14.1 and 56.5 ± 7.6 (P = 0.001). There was a statistically significant increase in RNFL thickness parameters (TSNIT average, superior average, and inferior average) and decrease in NFI post-cataract surgery as compared to the baseline values. Mean NFI pre- and post-cataract surgery was 41.3 ± 15.3 and 21.6 ± 11.8 (P = 0.001). Conclusions: Measurement of RNFL thickness parameters by scanning laser polarimetry is significantly altered following cataract surgery. Post the cataract surgery, a new baseline needs to be established for assessing the longitudinal follow-up of a glaucoma patient. The presence of cataract may lead to an underestimation of the RNFL thickness, and this should be taken into account when analyzing progression in a glaucoma patient.

Post-penetrating keratoplasty glaucoma.

Post-penetrating keratoplasty (post-PK) glaucoma is an important cause of irreversible visual loss and graft failure. The etiology for this disorder is multifactorial, and with the use of new diagnostic equipment, it is now possible to elucidate the exact pathophysiology of this condition. A clear understanding of the various mechanisms that operate during different time frames following PK is essential to chalk out the appropriate management algorithms. The various issues with regard to its management, including the putative risk factors, intraocular pressure (IOP) assessment post-PK, difficulties in monitoring with regard to the visual fields and optic nerve evaluation, are discussed. A step-wise approach to management starting from the medical management to surgery with and without metabolites and the various cycloablative procedures in cases of failed filtering procedures and excessive perilimbal scarring is presented. Finally, the important issue of minimizing the incidence of glaucoma following PK, especially through the use of oversized grafts and iris tightening procedures in the form of concomitant iridoplasty are emphasized. It is important to weigh the risk-benefit ratio of any modality used in the treatment of this condition as procedures aimed at IOP reduction, namely trabeculectomy with antimetabolites, and glaucoma drainage devices can trigger graft rejection, whereas cyclodestructive procedures can not only cause graft failure but also precipitate phthisis bulbi. Watchful expectancy and optimal time of intervention can salvage both graft and vision in this challenging condition.

Current profile of secondary glaucomas.

PURPOSE: To study the current profile of secondary glaucomas for their incidence and to identify risk factors. Materials and Methods: In this retrospective chart review, 2997 patients newly diagnosed and referred with glaucoma to our tertiary glaucoma center in the year 2005 were included. Evaluation of all cases was done on the basis of a detailed history and recorded examination including vision, intraocular pressure (IOP), anterior segment examination, gonioscopy and fundus evaluation by glaucoma specialists. Demographic data, etiology of secondary glaucoma, and any other significant findings were noted. RESULTS: Of 2997 referred patients, 2650 had glaucoma or were glaucoma suspects. Of all glaucoma patients or glaucoma suspects, 579 patients (21.84%) had secondary glaucoma. Age distribution was as follows: 25% were between 0-20 years; 27% were between 21-40 years; 30% were between 41-60 years and 18% were > 60 years. The male female ratio was 2.2. Frequent causes of secondary glaucoma were post - vitrectomy 14%, trauma 13%, corneo-iridic scar 12%, aphakia 11%, neovascular glaucoma 9%. Post-vitrectomy glaucoma eyes had vitreous substitutes in 83% cases of which 66% eyes had retained silicone oil for more than three months. Vision <or=20/200 was present in 63% eyes, 57% eyes had baseline IOP > 30 mm Hg. Of all traumatic glaucoma patients, 71% cases were < 30 years of age. Fifty per cent had baseline IOP of> 30 mm Hg and vision <or=20/200. CONCLUSIONS: Most patients with secondary glaucoma have poor vision (<or=20/200) with high IOP and advanced fundus changes at presentation.

Evaluation of retinal nerve fiber layer thickness measurement following laser in situ keratomileusis using scanning laser polarimetry.

AIM: To evaluate the effect of laser-assisted in situ keratomileusis (LASIK) on the measurement of retinal nerve fiber layer thickness by scanning laser polarimetry using customized corneal compensation in myopes. MATERIALS AND METHODS: Scanning laser polarimetry was performed on 54 eyes of 54 healthy patients with myopia using the glaucoma diagnostics variable corneal compensation (GDx VCC) instrument (Laser Diagnostic Technologies, San Diego, California) before and a week after LASIK. The various parameters were compared using the Student's t test. RESULTS: No statistically significant change was observed in any of the retinal nerve fiber layer parameters before and after LASIK. CONCLUSIONS: While the measurement of retinal nerve fiber layer thickness by scanning laser polarimetry is affected by anterior segment birefringent properties and LASIK would be expected to produce changes in the same, customized corneal compensation using the GDx VCC seems to adequately compensate for these changes.

Polymicrobial keratitis in an HIV-positive patient.

We describe a case with non-responding polymicrobial spontaneous corneal ulceration in an HIV-positive patient. Acanthamoeba was among the microorganisms isolated.