Paediatric glaucoma in Scotland.

BACKGROUND: The primary aim was to estimate the incidence of primary and secondary childhood glaucoma in Scotland over a 2-year period. The secondary aim was to gauge the confidence and experience of ophthalmologists in Scotland in managing these patients. METHODS: A 7 question electronic survey was distributed to all consultant members of the Scottish Paediatric Club and Scottish Glaucoma Club. Respondents were asked to report the number of cases and types of childhood glaucoma they had managed in the last 2 years. Respondents were also asked about experience and confidence in a range of glaucoma procedures, number of patients requiring referral to specialist centres and interest in the development of a centre of excellence in Scotland. RESULTS: The survey returned a 56% response rate, reporting 85 new cases of paediatric glaucoma in Scotland over the preceding 2 years. 11 (12.9%) had primary glaucoma and 74 (87.1%) had secondary glaucoma. The most common subtype of secondary glaucoma was uveitic glaucoma (n = 29). None of the respondents declared confidence or experience in trabeculotomy or goniotomy procedures. Eleven children required referral to a specialist unit outside Scotland. 85.7% of respondents felt Scotland would benefit from a specialist unit for paediatric glaucoma. CONCLUSIONS: This survey reflects an appetite for a specialist service for paediatric glaucoma in Scotland. However, further consideration is needed to determine if there is sufficient patient load to maintain such a service.

PERIPHERAL RETINAL DEGENERATIONS AND RHEGMATOGENOUS DETACHMENT IN PRIMARY CONGENITAL GLAUCOMA.

BACKGROUND: To determine the prevalence of peripheral retinal degenerations (PRD) and rhegmatogenous retinal detachment in patients with primary congenital glaucoma (PCG). METHODS: Records of all patients with PCG operated from year 2000 onwards were evaluated to look for the prevalence of rhegmatogenous retinal detachment. Of these, those children who were old enough to cooperate and had sufficient medial clarity were screened with an indirect ophthalmoscopy in a cross-sectional evaluation from 2010 to 2014. Peripheral retina was examined, and prevalence of PRD was estimated in this subset. For statistical purposes, only one eye of each patient was considered in this cross-sectional analysis. RESULTS: Of the 310 eyes (180 patients with PCG) operated from the year 2000 onwards, a rhegmatogenous retinal detachment was noted in 13 eyes (4%). Mean axial length of these eyes was 26.3 ± 3.2 mm (range, 19.8-34.7 mm). Among the eyes screened for PRD (n = 60), prevalence of pathologic PRD (lattices with/without atrophic holes and isolated holes/tears) was 15%. The average follow-up between glaucoma filtering surgery and the date of last examination was 8.55 ± 3.98 years (range, 5-20 years) in this subset. Mean axial length was significantly greater in eyes with pathologic PRD than in those without (28.1 ± 3.3 mm vs. 25.8 ± 2.6 mm; P = 0.01). For axial length ≥ 26 mm, the odds of having a pathologic PRD were 14.4 times more than those with axial length < 26 mm (P < 0.001; 95% confidence interval, 1.7-120.5). CONCLUSION: Prevalence of PRD among eyes with PCG is high. Peripheral retinal screening should be performed in eyes with PCG, especially those with axial lengths ≥ 26 mm.

Co-existing lacrimal drainage anomalies in eyes with congenital Glaucoma.

AIM: To evaluate the occurrence of co-existing congenital nasolacrimal duct obstruction (CNLDO) and other lacrimal anomalies in eyes with congenital glaucoma (CG). METHODS: Retrospective analysis of children aged ≤1-year with co-existing CG and CNLDO seen between 1998 and 2019, treatment interventions and outcomes. RESULTS: During the study period, 1993 children had CG and 6203 children had CNLDO, among the children aged 1-year or less. Of these, 51 children (73 eyes) had co-existing CG and CNLDO. The prevalence of CNLDO in CG was 2.5% (51/1993) and the prevalence of CG among CNLDO was 0.8% (51/6203). Median age (Interquartile range, IQR) was 53 days (IQR: 8, 155), when they were diagnosed with CG. Among the children with CNLDO, 68 eyes (93.1%) had simple CNLDO, and 5 eyes (6.9%) had complex CNLDO. Associated lacrimal anomalies were present in 7/73 eyes, including congenital lacrimal fistula in two eyes, upper punctal agenesis and upper mid-canalicular obstruction in two eyes each, and upper single canalicular-wall hypoplasia in one eye. Lacrimal syringing and probing were successful in 69/72 eyes (95.8%), and failed in 3 eyes (4.2%). These 3 eyes had complex CNLDO and underwent Dacryocystorhinostomy (DCR) with Mitomycin-C and intubation. Sixty-two eyes underwent combined trabeculotomy and trabeculectomy, and two eyes underwent trabeculectomy. Median age at glaucoma surgery was 87 days (IQR: 54, 193). Median time delay for glaucoma surgery after probing was 18 days (IQR: 12, 35). CONCLUSION: Among children aged ≤1-year presenting with congenital glaucoma coexisting lacrimal drainage anomalies was noted in 2.5% and simple CNLDO was the commonest. Early intervention is of paramount importance to prevent sight-threatening intraocular infection with CNLDO, and to minimize the delay of glaucoma surgery. The outcomes of lacrimal probing were excellent, however complex CNLDO required DCR.

Surgical results in the management of advanced primary congenital glaucoma in a rural pediatric population.

OBJECTIVE: To present the anatomic and functional results of surgical treatment for advanced primary congenital glaucoma (PCG) in a rural setting. DESIGN: Retrospective, observational case series. PARTICIPANTS: Forty eyes of 22 consecutive patients diagnosed with PCG and who underwent surgical treatment. METHODS: All eyes underwent surgical treatment for PCG. Type of surgery and postoperative complications were noted. We examined anatomic and functional indices before and after the operation. MAIN OUTCOME MEASURES: Visual acuity (VA), intraocular pressure (IOP), refractive spherical error, cup-to-disc ratio (CDR), and horizontal corneal diameter (HCD). RESULTS: Average age at the time of surgery was 3.3 years (range, 0.4-10) and the mean follow-up was 6 months (range, 1-11). 15% showed marked lens dislocation owing to the severe buphthalmos at presentation. Preoperative IOP was 54 ± 2 mmHg, HCD was 15.1 ± 0.3 mm, and CDR (when visible) was 0.8 ± 0.02. Surgical intervention included 31 trabeculotomies, 6 Ahmed Glaucoma Valve implants, 1 goniotomy, and 2 eviscerations. Serious complications were noted in 4 eyes. Final postoperative IOP was 23 ± 2 mmHg (P < 0.0001). Patients were significantly more likely to have ambulatory VA (following objects or better) after operation (18% vs 64%; P < 0.0001). CONCLUSIONS: Our surgical outcomes in children with advanced PCG demonstrated moderate overall improvement in IOP and modest improvement of VA. Deep deprivation amblyopia, severe disease manifestation at presentation, opaque corneas, and frequent lens dislocation limited the possible success. These data further signify the need for effective, timely screening of children and prompt recognition by health care workers to reduce the rate of avoidable blindness in developing countries.

Outcomes of goniotomy for primary congenital glaucoma in East Africa.

OBJECTIVE: To evaluate the effectiveness of goniotomy to treat primary congenital glaucoma in an east African population. DESIGN: A retrospective series using case records. For patients who underwent goniotomies in both eyes, only the first was analyzed. PARTICIPANTS: Forty-seven children undergoing goniotomy (47/80 operated eyes analyzed). Thirty-six children (77%) were male. Mean age was 19 months (range 1-204 months, standard deviation [SD] = 35). INTERVENTION: First goniotomy was performed from the temporal side. Repeat surgery, if necessary, was performed via nasal side approach and 100% ethanol-soaked cotton-tipped swabs were used to clear the corneal epithelium when necessary for angle visualization. MAIN OUTCOME MEASURES: Postoperative intraocular pressure (IOP) <21 mmHg was considered success. Potential risk factors for failure were analyzed. Quantitative visual acuities were measured with Cardiff acuity cards and Lea tests in older children. RESULTS: A total of 36 of 47 children (77%) returned for follow-up. Mean follow-up interval was 12 months (range, 1-62 months; SD = 15). A total of 28 of 47 children (60%) had >3 months of follow-up, and 11 children had >1 year of follow-up. A total of 12 of 47 eyes (26%) required repeat goniotomy. Three of these required further trabeculectomy with mitomycin C, 1 of which required eventual enucleation after failure to control IOP, enlargement, and pain. A total of 11 of 28 children (39%) with >3 months follow-up required repeat surgery. A total of 24 of 28 children (86%) with follow-up intervals of at least 3 months achieved postoperative IOPs of <21 mmHg. Mean IOPs decreased (t = 6.1, P < 0.0005) from 23.4 mmHg (SD = 5.3 mmHg) to 14.4 mmHg (SD = 7.6 mmHg). Only 1 predictor of failure to achieve successful control of IOP was identified: female gender (relative risk = 5.3, 95% confidence interval, 1.1-26.3). Corneas were clear in 4 of 47 eyes (8.5%) preoperatively and 32 of 41 eyes (78%) postoperatively. Of 25 children with preoperative and postoperative acuities measured, 22 (88%) recorded improvement. CONCLUSIONS: Preliminary results of goniotomy in an African population with advanced disease and late presentation are reasonably encouraging. Overall results are comparable with historical drainage procedures, with the advantage of preserving conjunctiva. Further studies are justified.

Risk Factors for Blindness in Children With Primary Congenital Glaucoma-Follow-up of a Registry Cohort.

PURPOSE: To evaluate the baseline features associated with blindness in a cohort of children with primary congenital glaucoma (PCG) from a hospital registry. DESIGN: Retrospective clinical cohort study. METHODS: Setting: Observational cohort study. StudyPopulation: The registry included all children who presented to our tertiary care institute between 1995 and 2014 with a diagnosis of childhood glaucoma. ObservationProcedure: Baseline characteristics at initial presentation of children with PCG in the registry who were blind at the last follow-up were compared with those who were not blind, using bivariate and then multivariate regressions to account for potential confounders. MainOutcomeMeasures: Blindness was defined as a best-corrected visual acuity of 3/60 (20/400) or worse in the better eye at the final follow-up. RESULTS: The eligible sample consisted of 196 children with a mean age of 9.54 ± 22.44 months at presentation. After a mean ± standard deviation follow-up of 8.49 ± 3.85 years, 20 (10.2%) children were blind. The baseline demographic factors, intraocular pressure, horizontal corneal diameter, spherical equivalent, axial length, and corneal thickness, were similar for the "blind" and "not blind" groups (P > .05). In the multivariate regression, only the severity of corneal opacification remained significantly (P < .001) associated with the risk of blindness (odds ratio = 4.05; 95% confidence interval: 1.89-8.85). CONCLUSION: Corneal clouding is a predictor of future blindness in children with PCG. Children with severe corneal clouding may need more aggressive intraocular pressure control, closer follow-up, and earlier counseling.

Neonatal-Onset Congenital Ectropion Uveae: A Distinct Phenotype of Newborn Glaucoma.

PURPOSE: To describe neonatal-onset congenital ectropion uveae (N-CEU) as a distinct clinical entity of newborn glaucoma (NG) and to study its significance toward the severity and outcome of NG. DESIGN: Prospective clinical cohort study. METHODS: The study took place at a tertiary care postgraduate teaching institute. It included consecutive patients with NG who presented between July 1, 2016 and September 30, 2017, with a minimum postoperative follow-up of 1 year. Infants with any ocular anomaly apart from CEU were excluded. Patients with N-CEU were compared with those with neonatal-onset primary congenital glaucoma (N-PCG). All infants underwent goniotomy or trabeculotomy, with trabeculectomy depending on corneal clarity. Clinical features at presentation and outcome 1 year after surgery were defined as good or satisfactory if intraocular pressure was ≤16.0 mm Hg under anesthesia without or with topical medications, respectively, and poor if the infant required additional surgery. RESULTS: Twenty eyes of 10 patients with N-CEU were compared with 16 eyes of 9 patients with N-PCG. Infants with N-CEU had significantly worse corneal clarity (mean grade 2.0 ± 0.7 vs 1.4 ± 0.8; P = .026) and poorer outcomes compared with those with N-PCG. Seven of 16 (43.7%) eyes with N-PCG had a cornea clear enough at presentation for a goniotomy compared with only 2 of the 20 (10%) eyes with N-CEU (P = .026). Thirteen of 16 (81.2%) eyes with N-PCG had a good or satisfactory outcome compared with 6 of 20 (30%) eyes with N-CEU (P = .001). CONCLUSIONS: N-CEU appears to be distinct from the unilateral CEU in older patients described in the literature and may be considered a poorer prognosis phenotype of neonatal-onset glaucoma.

Primary Congenital Glaucoma: Trends in Presentation Over 3 Decades at a Tertiary Eye Care Center in India.

PRECIS: In this study, huge time delay was observed between first primary congenital glaucoma (PCG) symptom recognition by parents to ophthalmological diagnosis and this delay was unchanged over time. Bilateral affliction and mean presenting intraocular pressure (IOP) remained unaltered. PURPOSE: To compare the trends in presentation of patients with PCG over 3 different time periods at 10-year intervals (1998, 2008, and 2018) managed by a single surgeon at one of the largest tertiary eye care centers in South India. METHODS: Retrospective analysis of 313 eyes of 172 patients diagnosed to have PCG and treated at the L V Prasad Eye Institute (LVPEI) between January and December of the 3 specified years. Data collected included age at symptom recognition and at presentation, sex, affected eye, signs and symptoms, time between onset of symptoms and diagnosis (diagnostic delay), corneal clarity, and IOP. RESULTS: Of the 172 patients, 48 (27.9%), 76 (44.2%), and 48 (27.9%) belonged to 1998, 2008, and 2018, respectively. Bilateral affliction (79% to 84%) was common and there was no sex predilection. The median age at recognition of first symptoms by caregivers/parents was 1 day (interquartile range, 1 to 30), and at diagnosis was 120 days (interquartile range, 30 to 378). Overall, presentation to LVPEI was delayed by >3 months in 80 (46.5%) children, and there was no statistically significant difference in the duration of delay over time (P=0.13). There was no significant difference in the mean presenting IOP over time (P>0.05). CONCLUSIONS: We provide unique single center-based information of trends in the presentation of patients with PCG over 3 decades. Diagnostic delay was common, and a large number of characteristics of patients with PCG in South India have remained unchanged, particularly, age at onset and at diagnosis and laterality.

Primary congenital glaucoma in Denmark, 1977-2016.

PURPOSE: To perform an epidemiological survey of all patients in Denmark diagnosed with primary congenital glaucoma (PCG) from 1977 to 2016. METHODS: A retrospective, nationwide study based on a review of medical files of all children with PCG born in Denmark from 1977 to 2016. PCG was defined as glaucoma due to isolated angle dysgenesis affecting children from birth and including late onset/late recognized PCG, excluding glaucoma associated with other congenital abnormalities. Incidence and risk factors of PCG including gender, consanguinity, family history, ethnicity, comorbidity and prematurity were evaluated and stratified by decade (1977-1986, 1987-1996, 1997-2006 and 2007-2016). Age at first symptoms, age at diagnosis, age at time of first surgery and possible diagnostic delay were assessed. RESULTS: Of 118 patients were identified, annual incidence of PCG was 4.8 per 100 000 live born. 62.3% of cases were bilateral. The relative risk (RR) of PCG was significantly higher in boys (62.7% of patients, RR 1.60 (95% CI 1.10-2.32)). 13.6% had comorbidity. Median age at time of first symptoms was 75 days, median age at time of diagnosis was 150 days and median diagnostic delay was 75 days with no significant difference throughout the decades. Unilateral cases presented symptoms and were diagnosed significantly later than bilateral cases. CONCLUSION: We provide unique nation-based information on the incidence of PCG from a single country covering 40 years. Male gender was a risk factor. Diagnostic delay was unchanged throughout the 4 decades and a high percentage of comorbidity was revealed. Introducing a standardized paediatric screening of all PCG children should be considered.

Some clinical findings at presentation can predict high-risk pathology features in unilateral retinoblastoma.

AIMS: To identify clinical features at presentation to help in detecting patients with retinoblastoma and pathology risk factors (PRFs) preoperatively, and therefore selecting a high-risk population that could benefit from preoperative treatment. METHODS: A retrospective analysis of a prospectively filled form of 182 consecutive patients with unilateral retinoblastoma treated with initial enucleation from 1988 to 2006. Univariate and multivariate analyses were carried out. Major choroidal invasion and postlaminar optic nerve and scleral extension were considered PRFs. Within this subgroup, a higher-risk cohort (microscopical residual disease caused by trans-scleral invasion or invasion to the resection margin of the optic nerve) was analyzed separately. RESULTS: One hundred sixty-four patients had completely resected and 18 had microscopical residual disease. Seventy three had at least 1 PRF (massive invasion to the choroid in 25, to the postlaminar optic nerve in 41, intrascleral in 10, to the resection margin of the optic nerve in 12, and trans-scleral in 6). Seventy-one patients had glaucoma and 19 had buphthalmia. Intraocular pressure, glaucoma, and buphthalmia correlated significantly with the occurrence of both PRF and microscopical residual disease in multivariate analysis. Buphthalmia was the most specific factor but the sensitivity was lower. Glaucoma and buphthalmia had a high negative predictive value. CONCLUSIONS: Patients presenting with glaucoma and/or buphthalmia have a significantly higher risk for the occurrence of PRF, including those resulting in microscopically residual disease.

Congenital eye and adnexial anomalies in Kano, a five year review.

BACKGROUND: Knowledge of prevalence and types of congenital eye and adnexial anomalies is important as some of these anomalies can lead to childhood blindness. The aim of the study is to determine the types of congenital eye and adnexial anomalies seen in the eye clinic of Aminu Kano Teaching Hospital Kano, Nigeria between the years 2001 to 2005. To determine the proportion of children below the age of 10 years who presented with congenital eye and adnexial anomalies, the proportion of eye surgeries due to such anomalies, and proportion of these anomalies amenable to (surgical) intervention. METHODS: The clinic and theater registers were used to obtain the total number of children less than 10 years of age seen and those that had surgery during the review period. A list of those with congenital eye and adnexial anomalies was compiled. The case folders of patients with such anomalies were retrieved and information on age, sex, type of anomaly, laterality of the condition, and types of surgical intervention offered, was obtained. The data was manually analyzed. RESULTS: There were 4163 children seen in the eye clinic and 268 had surgery during the review period. There were 109 eyes of 69 patients with congenital eye and adnexial anomalies. The prevalence of such anomalies amongst children (<10 years of age) is 1.7% and surgery for congenital eye and adnexial anomalies accounted for 25.7% of eye surgeries in the study population. The male to female ratio is 2.3:1. In 40 patients, the anomaly was bilateral and it was unilateral in 29 patients. The commonest congenital anomalies are buphthalmos in 38%, cataracts in 35%, and naso lachrymal duct obstruction in 14%. Other less frequent anomalies are anophthalmia/micophthalmia, limbal dermoid cysts and aniridia. Eighty six percent of the patients had surgery to correct the anomaly. CONCLUSION: Most of the congenital anomalies seen in our hospital can lead to childhood blindness and vigilance by eye care providers is advised to ensure prompt identification and intervention.

Microcatheter-assisted Trabeculotomy Versus 2-site Trabeculotomy With the Rigid Probe Trabeculotome in Primary Congenital Glaucoma.

PURPOSE: The purpose of this study was to compare the outcomes of microcatheter-assisted trabeculotomy to circumferential trabeculotomy using the rigid probe trabeculotome in primary congenital glaucoma. METHODS: This retrospective study was performed in an institutional setting. The medical records of primary congenital glaucoma patients who underwent circumferential trabeculotomy (≥270 degrees incised) using Glaucolight-illuminated microcatheter or a rigid probe trabeculotome were reviewed. The primary outcomes were the percent reduction of intraocular pressure (IOP) and success rates. Complete success was defined as achieving an IOP<18 mm Hg without medications. Secondary outcomes were the postoperative IOP and glaucoma medications. RESULTS: The study included 92 eyes of 92 patients. Of these, 33 eyes of 33 patients aged 6.4±8.7 months underwent microcatheter-assisted trabeculotomy creating a 336±34-degree incision, with 19 eyes (58%) having a complete 360-degree incision. The other 59 eyes of 59 patients aged 8.2±13.1 months underwent 2-site trabeculotomy, using a rigid probe trabeculotome through a combined superonasal and inferotemporal approach, creating a 338±29-degree incision, with 33 eyes (56%) having a complete incision. After a follow-up of 21.2±8.9 months, there was a 42%±25% IOP reduction and a 73% rate of complete success in the microcatheter group, compared with 40%±22% IOP reduction and an 80% success rate in the rigid probe group (P=0.7 and 0.3, respectively). There was no significant difference in survival time in both groups (P=0.6). CONCLUSION: Circumferential trabeculotomy using either the illuminated microcatheter or rigid probe trabeculotome yielded comparable results; however, the added cost of the microcatheter should be considered.

A retrospective survey of childhood glaucoma prevalence according to Childhood Glaucoma Research Network classification.

PURPOSE: To evaluate the Childhood Glaucoma Research Network (CGRN) classification system and describe the prevalence of each subtype according to this classification. MATERIALS AND METHODS: Retrospectively, the medical records of 205 consecutive childhood glaucoma and glaucoma suspect patients at an urban tertiary care center were reviewed. The initial diagnosis and new diagnosis according to CGRN classification were recorded. RESULTS: All patients fit one of the seven categories of the new classification. Seventy-one percent of diagnoses were changed upon reclassification. Twenty-three percent of patients had primary glaucoma (juvenile open-angle glaucoma and primary congenital glaucoma [PCG]); 36% had secondary glaucoma (glaucoma associated with nonacquired ocular anomalies; glaucoma associated with nonacquired systemic disease or syndrome; glaucoma associated with acquired condition; and glaucoma following cataract surgery); and 39% were glaucoma suspect. Of the patients diagnosed with glaucoma, PCG was the most common diagnosis, seen in 32% of patients. CONCLUSION: The CGRN classification provides a useful method of classifying childhood glaucoma.

A review of primary congenital glaucoma in the developing world.

Primary congenital glaucoma is the most common type of infantile glaucoma, yet it remains a relatively rare disease. Treatment is principally surgical, and management lasts a lifetime. In the developing world, delayed diagnosis, limited treatment, and inadequate follow-up lead to a heavier burden on the patient and community. We highlight the epidemiology and genetics of the disease, its current management and prognosis, and the limitations facing both providers and patients in the developing world. Further efforts are necessary to provide effective, timely screening of children and allocate adequate resources to allow health care workers to reduce the rate of avoidable blindness in developing countries.