A Service Coverage Analysis of Primary Congenital Glaucoma Care Across the United States.

PURPOSE: To assess the number of infants at risk of delayed primary congenital glaucoma (PCG) evaluation due to long travel times to specialists. DESIGN: Cross-sectional geospatial service coverage analysis. METHODS: All American Glaucoma Society (AGS) and American Association for Pediatric Ophthalmology and Strabismus (AAPOS) provider locations were geocoded using each organization's member directory. Sixty-minute drive time regions to providers were generated using ArcGIS Pro (Esri). The geographic intersection of AGS and AAPOS service areas was computed because patients typically require visits to both types of specialists. American Community Survey data were then overlaid to estimate the number of infants within and beyond the AGS/AAPOS service areas. RESULTS: One thousand twenty-nine AGS and 1,040 AAPOS provider locations were geocoded. The analysis yielded 944,047 infants age 0-1 year (23.6%) who live beyond the AGS/AAPOS service areas. Therefore, approximately 14-94 new PCG cases/year may be at risk of delayed diagnosis as a result of living in a potential service desert. Compared with children living within the AGS/AAPOS service areas, children aged <6 years in these potential service deserts were more likely to live in households earning below the US federal poverty level, lack health insurance, and live in a single-parent home. These communities are disproportionately likely to experience other rural health disparities and are more prevalent across the Great Plains. CONCLUSION: Service coverage analysis is a useful tool for identifying underserved regions for PCG referrals and evaluation. These data may assist in targeting screening programs in low access areas for pediatric glaucoma care.

Follow-up Adherence and Barriers to Care for Pediatric Glaucomas at a Tertiary Care Center.

PURPOSE: To determine the percent adherence to follow-up for patients with pediatric glaucomas seen at a tertiary care center and to elucidate risk factors. DESIGN: Retrospective cohort study. METHODS: Patients with pediatric glaucomas seen at the University of Minnesota over 8.5 years were classified as adherent, nonadherent, or lost to tertiary follow-up if they followed up within 0-30 days, between 31 and 180 days, or later than 180 days of the recommended appointment time or never, respectively. RESULTS: Of 176 patients analyzed, 95 (54%) were adherent (51% male; mean [standard deviation (SD)] age: 56.1 [59.8] months), 5 (3%) were nonadherent (20% male; mean [SD] age: 25.0 [35.8] months), and 76 (43%) were lost to tertiary follow-up (55% male; mean [SD] age: 58.9 [53.1] months). Multiple logistic regression analysis of variables that were significant in isolation revealed that only race (white: odds ratio, 3.58; 95% confidence interval, 1.42-9.05; P = .007) and distance from the eye clinic (per 50 miles: odds ratio, 0.79; 95% confidence interval, 0.67-0.92; P = .003) significantly impacted adherence. CONCLUSIONS: This is the first study of adherence to follow-up recommendations for patients with pediatric glaucomas. Percent adherence to follow-up appointments was alarmingly low, and decreased adherence was observed with non-white race and increased distance to the eye clinic. Physicians should consider these risk factors when risk-stratifying patients with pediatric glaucomas for nonadherence to follow-up. Additional studies to improve adherence through interventions that reduce biases and barriers to follow-up are needed.

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.

Clinical Presentation and Management Outcomes of Coexistent Congenital Glaucoma and Retinopathy of Prematurity.

PURPOSE: The purpose of this study was to report presentation and outcomes of coexisting congenital glaucoma with retinopathy of prematurity (ROP) in "Indian Twin cities ROP study (ITCROPS)." METHODS: Children with ROP and congenital glaucoma were identified from ITCROPS (prospective digital database) between 1997 and 2016. The presentation, interventions, and outcomes for glaucoma and ROP were evaluated. RESULTS: Out of 15,000 premature-infants in the database, 3000 children had ROP, and of these 87 eyes of 57 premature children had glaucoma. Five eyes (5.7%) of 3 children in the entire ROP cohort had coexisting congenital glaucoma (before any ROP intervention), 3 eyes of 2 children had primary congenital glaucoma and 2 eyes of one child had glaucoma with microspherophakia. The mean age at surgery was 2.7±0.6 months. At presentation, all eyes had corneal edema, mean corneal diameter was 10.3±0.75 mm and IOP was 20.4±1.67 mm Hg. Three eyes had stage-2 ROP and two eyes had stage-3 ROP. Four eyes received intravitreal Bevacizumab. All eyes underwent combined trabeculotomy with trabeculectomy. Post glaucoma surgery, 2 eyes underwent laser for ROP and 1 eye with stage-2 ROP was observed with no treatment. ROP regressed without any sequalae in all 5 eyes. Two eyes with microspherophakia and one eye with lens coloboma underwent lensectomy. One eye postlensectomy needed Ahmed glaucoma valve implant for IOP control. The mean IOP at last follow-up was 13.6±1.67 mm Hg and all eyes needed topical antiglaucoma medications for IOP control. CONCLUSIONS: Congenital glaucoma coexisting with ROP is a medical emergency. Intravitreal therapy can help tide over the vision-threatening fulminant phase of ROP while awaiting the effect of glaucoma surgery on corneal clarity. The need for medications even after glaucoma surgery suggests severe glaucoma phenotype. Close coordination between subspecialties is needed for best outcomes.

Early Predictors of Long-term Outcomes in Childhood Glaucoma.

PURPOSE: A data-based staging system for childhood glaucoma is likely to improve the prediction of visual acuity and intraocular pressure (IOP) control outcomes. We investigated early clinical factors associated with poor long-term visual, and IOP control outcomes in early-onset glaucoma as the initial steps to constructing a severity staging system. DESIGN: Statistical modeling of retrospective case series data. PARTICIPANTS: Glaucoma patients younger than 3 years of age who presented to Bascom Palmer Eye Institute between 1990 and 2010 with at least 5 years of follow up. METHODS: Statistical modeling of retrospective case series data from first, second, third, 3-year, 5-year, and final visits. MAIN OUTCOME MEASURES: Association of early clinical characteristics to final IOP-control outcomes and visual acuities. RESULTS: A total of 26 eyes of 15 children were included. Nine of 15 (60%) of patients were male. Mean age at initial presentation: 9.98±10.55 months. Mean duration between initial and final visits: 11.13±3.55 years. By the third visit (mean 6.69 mo after presentation), presence of nystagmus, anterior segment dysgenesis (ASD) or having failed angle surgery increased the final LogMAR visual acuity by 0.76 (P=0.0516), 0.64 (P=0.0618), and 0.58 (P=0.0159), respectively. At year 3, failed amblyopia therapy, failed angle surgery, nystagmus, media opacity (MO), or ASD increased the final LogMAR by 1.30, 1.34, 1.21, 0.85, 0.64, respectively (all P<0.02). Failed angle surgery or MO increased the chance of uncontrolled IOP at the final visit (proportional odds ratio of 6.77 and 12.88, respectively). CONCLUSIONS: In this pilot study of a modest size cohort, the presence of nystagmus, ASD or failed angle surgery early in the course of infantile-onset glaucoma management predicted poor final visual outcome, whereas failed angle surgery and presence of MO predicted poor final IOP control. These predictors of poor outcomes will serve as the initial steps in constructing a severity staging system.

Long-Term Clinical Course in Eyes With Peters Anomaly.

PURPOSE: Peters anomaly (PA) is known to be a primary cause of congenital corneal opacity. The aim of this study is to report the long-term clinical course and visual achievement of patients with PA who did not undergo keratoplasty. METHODS: This retrospective study involved 15 eyes of 9 infants with PA less than 5 months of age at initial presentation at the Kyoto Prefectural University of Medicine who were followed up without keratoplasty for more than 6 years after presentation. In each subject, visual acuity, intraocular pressure (IOP), presence of congenital glaucoma, and change of corneal opacity were retrospectively investigated. RESULTS: At initial presentation, the mean patient age was 2.3 months (SD: 1.3; range: 0-4 mo), and the mean follow-up period was 7.9 years (SD: 1.8; range: 6.5-11.3 yrs). Of the 9 patients, 6 were affected bilaterally. Thirteen eyes of 7 patients were found to have glaucoma. Over the follow-up period, corneal opacity gradually decreased in 4 eyes, 1 eye each of 2 unilateral patients without glaucoma and both eyes of 1 bilaterally affected patient with good IOP control during the follow-up period. Visual acuity in the unilateral case with intensive amblyopia treatment gradually increased along with decreasing corneal opacity. CONCLUSIONS: The findings of this study show that a natural decrease of corneal opacity can occur in eyes with PA. In cases of unilateral PA without IOP elevation and no enlargement of the corneal diameter, strict amblyopia treatment is vital to obtain a favorable visual prognosis.

Comparison of EX-PRESS Shunt and Trabeculectomy With Mitomycin-C in Congenital and Juvenile Glaucoma.

PURPOSE: To compare efficacy and safety of EX-PRESS mini glaucoma shunt (Alcon) and trabeculectomy with mitomycin-C (MMC) in the management of pediatric glaucoma. METHODS: The authors retrospectively reviewed patients from a single pediatric ophthalmology practice with either congenital or juvenile glaucoma who underwent surgical treatment with either trabeculectomy or EX-PRESS shunt insertion with MMC. Postoperative intraocular pressure at 6, 18, and 36 months, medication requirements, visual acuities, postoperative interventions, complications, and noncompliance with medications or follow-up was reviewed. RESULTS: A total of 42 cases of glaucoma with either trabeculectomy or EX-PRESS shunt were identified for review (n trabeculectomy=25, n EX-PRESS=17). At 6 months, 62.5% of trabeculectomies and 94.1% of EX-PRESS shunts met criteria for success (P=0.0281). At 18 months, 38.1% of trabeculectomies and 81.3% of EX-PRESS shunts met criteria for success (P=0.0178). At 36 months, trabeculectomies had a success rate of 33.3% versus 66.7% for EX-PRESS shunts (P=0.321). The average decrease in medications postoperatively was 0.375 and 1.63 for trabeculectomy and EX-PRESS shunt, respectively (P=0.06). Preoperative visual acuities compared with postoperative best-corrected visual acuities before additional surgery demonstrated a slight average worsening of 0.11 logMAR in trabeculectomy patients and an average improvement of 0.56 logMAR in EX-PRESS shunt patients (P=0.0037). CONCLUSIONS: In this review, patients with pediatric glaucoma managed with EX-PRESS shunt with MMC compared with trabeculectomy with MMC appear to have better intraocular pressure control, better visual acuities, and fewer complications and reoperations.

Congenital Glaucoma and CHARGE Syndrome: A Case Report.

PURPOSE: To report a rare case of congenital glaucoma in a patient with CHARGE syndrome, present gonioscopic photographs, and explore mechanisms of disease that may account for this association. PATIENTS AND METHODS: We describe a 35-week-old girl with previously diagnosed CHARGE syndrome who presented with corneal edema, buphthalmos, and elevated intraocular pressure in the left eye. She was subsequently diagnosed with congenital glaucoma and started on topical and oral therapy. RESULTS: Examination under anesthesia confirmed the above findings as well as bilateral abnormal angles with an anterior iris insertion at the level of the posterior trabecular meshwork, prominent iris vasculature and stromal strands, and nonvisible scleral spur and ciliary body bands. Trabeculotomy and trabeculectomy were performed in the left eye with a poor outcome. CHARGE syndrome is a complex neurocristopathy, and we propose that the abnormal angle findings and associated asymmetric glaucoma in our patient share a common mechanism of neural crest cell dysfunction. CONCLUSIONS: CHARGE syndrome can be associated with congenital glaucoma and we emphasize the importance of a thorough ophthalmic examination to detect glaucoma with surgical management as deemed appropriate.

Persistent Corneal Edema Associated With Subconjunctival 5-fluorouracil in an Infant With Primary Congenital Glaucoma.

A 3-month-old boy with primary congenital glaucoma developed a transient corneal endothelial opacity after needle bleb revision with adjunctive 5-fluorouracil. This case presents a rare toxicity that, although transient, is concerning due to the potential of amblyopia. The authors review prior cases of corneal opacity associated with 5-fluorouracil. [J Pediatr Ophthalmol Strabismus. 2016;53:e54-e57.].

Evaluation the adjunctive use of combined bevacizumab and mitomycinc to trabeculectomy in management of recurrent pediatric glaucoma.

PURPOSETO evaluate the efficacy and safety of combined bevacizumab-mitomycin c (MMC) in recurrent cases of pediatric glaucoma.METHODSA prospective non-masked controlled study that included bilateral cases of 12 patients (24 eyes) with recurrent (had previous glaucoma surgery before) pediatric glaucoma. One eye in each patient (12 eyes) was assigned to trabeculectomy operation with combined application of MMC (0.4 mg/ml for 3 min) under and around the scleral flap before trabeculectomy and bevacizumab (avastin) (2.5 mg in 0.2 ml) injected subconjunctivally around the bleb after completing the surgery (group I). The other eye of each patient (12 eyes) was assigned to trabeculectomy operation with application of MMC (0.4 mg/ml for 3 min) only (group II). The mean follow-up period was 13±1 months.ResultsThe mean age was 2.16±1.5 (range 7 months to 4.1 years). No significant difference in preoperative intraoperative pressure (IOP) was observed between the groups (P>0.05). Recurrent primary congenital glaucoma represents 66.7% of the cases. Other cases included were recurrent aphakic and pseudophakic glaucoma 25% and recurrent post uveitic glaucoma 8.3%. The mean IOP was 12.1±4.2, 12.6±5.4, and 12.8±5.2 mm Hg in group I at 3, 6, and 12 months, respectively, and was 12.8±5.3, 13.7±6.7 and 15.6±5.9 mm Hg in group II at 3, 6, and 12 months, respectively. There was a statistically significant difference in the mean IOP between the studied groups at the 1-year follow-up visit (P<0.05). In addition, group I showed a higher statistically significant difference in absolute and total success (75 and 91.7%, respectively) compared with group II (58.3 and 75%, respectively) (P<0.05). The encountered complications included mild hyphema, which occurred in 8.33% in group 1, wound leakage, which occurred in 8.33% in each group, and shallow anterior chamber (AC), which occurred in 16.7% in each group and was the most common encountered complication in the study. One case of shallow AC in group I led to choroidal effusion (8.33%). One case in group II developed late bleb-related endophthalmitis after 3 months, which resulted in phthisis bulbi (8.33%).ConclusionThe additive effect of subconjunctival bevacizumab to MMC-augmented trabeculectomy in the case of recurrent pediatric glaucoma was beneficial in improving the success rate. Better IOP control and prolonging the bleb survivalvia reducing the long-term need of using anti-glaucoma drugs postoperatively without adding complications had also been achieved with this technique. This offers a promising alternative for the treatment of this type of glaucoma.

A rare case of primary congenital glaucoma in combination with neurofibromatosis 1: a case report.

BACKGROUND: Neurofibromatosis 1 (NF1) is a common disease that mainly affects the skin and peripheral nervous system, and is characterized by bony dysplasia. Primary congenital glaucoma (PCG) is a sight-threatening disease that can manifest as a prodrome of NF1, especially in newborn babies. We report a case of PCG with NF 1. CASE PRESENTATION: A 1-month-old boy presented with an enlarged right eyeball. An increased IOP and typical glaucomatous optic neuropathy were found, on the initial physical examination, a clinical diagnosis of primary congenital glaucoma (PCG) was made and a trabeculectomy with mitomycin C (MMC) therapy was subsequently performed. Three year later, the boy again presented with an even larger right eye and a gradually expanding left one. In addition to typical glaucomatous optic neuropathy, the boy also had multiple café au lait patches all over his body, megacephaly (head circumference = 60 cm; body weight = 14 kg; height = 93 cm) and remarkable facial features included swollen, soft upper eyelids and a flat, broad nose sphenoid wing dysplasia, eyelid thickening, bony orbit enlargement were found. CONCLUSIONS: It is rare have both PCG and NF1, and PCG may be a prelude to NF1. Continuous follow-up should be advised and we should raise our awareness of the combined condition and to improve chances for an early diagnosis.

Effect of intraocular pressure control on central corneal thickness, horizontal corneal diameter, and axial length in primary congenital glaucoma.

PURPOSE: To assess the effect of intraocular pressure (IOP) control on central corneal thickness (CCT), horizontal corneal diameter (HCD), and axial length (AL) in primary congenital glaucoma (PCG). METHODS: The medical records of children under 10 years of age surgically treated for PCG from 2003 to 2012 were retrospectively reviewed to identify patients with IOP of ≤12 mm Hg with a minimum of 6 months' follow-up. Pre- and post-treatment IOP, CCT, HCD, and AL values were correlated and compared with those of healthy age-matched controls. RESULTS: A total of 131 controls and 20 patients (12 bilateral; 8 unilateral) were included. Mean follow-up was 32.4 months. In 24 eyes IOP was controlled by 1 trabeculotomy, with 6 eyes requiring antiglaucoma medication; in 5 eyes, by 2 trabeculotomies, without medication; and in 3 eyes, by 2 trabeculotomies plus 1 with mitomycin-C and 2 eyes requiring antiglaucomatous medication. The mean IOP was 15.69 ± 5.31 mm Hg preoperatively and 6.16 ± 2.42 mm Hg at final follow-up. Mean pre- and postoperative CCT values were, respectively, 614.38 ± 89.41 µm and 548.56 ± 63.12 µm; HCD, 13.45 ± 1.00 mm and 13.98 ± 1.01 mm, respectively; AL, 24.57 ± 2.71 mm and 25.37 ± 2.66 mm, respectively. All differences were statistically significant (P ≤ 0.002). In 40% of patients, post-treatment CCT values were comparable to those of controls; in 6.2%, HCD measurements; and in 26%, AL measurements. Pre- and post-treatment measurements were positively correlated (P ≤ 0.009). CONCLUSIONS: Controlling IOP had a positive effect on the CCT, HCD, and AL in patients with congenital glaucoma.

Medical and surgical outcomes in childhood glaucoma: a population-based study.

INTRODUCTION: To describe the clinical and surgical outcomes among patients younger than 20 years of age diagnosed with glaucoma in a defined population during a 40-year period. METHODS: The medical records of all patients (<20 years) diagnosed with glaucoma in Olmsted County, Minnesota, from January 1, 1965, through December 31, 2004, were retrospectively reviewed. RESULTS: Thirty children (45 eyes) were diagnosed with various forms of glaucoma during the 40-year study period. During a mean follow-up of 12.5 years (range, 7 days to 32 years), 18 (60%) of the 30 children underwent a mean of 2.7 surgeries (range, 1 to 10), including 6 (20%) patients whose sole surgery consisted of enucleation or evisceration for a blind, painful eye. Twenty-eight (93%) of the 30 children required medical management during the follow-up period, including 14 (47%) treated before their first surgery. At the final follow-up examination, 11 (37%) had a visual acuity of 20/200 or worse. The 10-year Kaplan-Meier risk of vision decreasing less than 20/200 in all glaucoma patients was 22.7% (95% CI, 0-40.9), and patients requiring any glaucoma surgery was 68.3% (95% CI, 42.4-82.6). CONCLUSIONS: In this population-based study of children diagnosed with glaucoma during a 40-year period, most patients required surgery, with few being successfully controlled by medications alone. A poor visual outcome or the loss of an eye was relatively common.

CYP1B1-related anterior segment developmental anomalies novel mutations for infantile glaucoma and von Hippel's ulcer revisited.

PURPOSE: To determine the prevalence of CYP1B1 mutations in a cohort of patients with congenital corneal opacification (CCO), infantile glaucoma, or both and to describe a developmental CCO associated with CYP1B1 mutation that may explain von Hippel's original description of an internal ulcer. DESIGN: Retrospective genotyping of a cohort of patients with infantile glaucoma and CCO. PARTICIPANTS: Thirty-three patients with CCO, infantile glaucoma, or both. METHODS: All patients underwent a full clinical evaluation with or without examination under anesthetic including anterior segment photography, ultrasound biomicroscopy (for CCO patients; n = 22), and histopathologic analysis in patients in whom penetrating keratoplasty (PK) was performed (n = 10). Patient DNA and DNA from 50 normal control individuals who had undergone a full ophthalmologic examination were screened for CYP1B1 mutations. MAIN OUTCOME MEASURES: Classification of the developmental corneal opacity phenotype in infantile glaucoma patients with CYP1B1 mutations. RESULTS: Nine distinct pathogenic recessive CYP1B1 mutations were found in 11 patients from 6 unrelated families, including 1 patient with an entire deletion of the CYP1B1 gene. Two of these patients, including the patient with the deletion, had isolated infantile congenital glaucoma with no other abnormalities. No CYP1B1 mutations were found in another 13 patients (7 of whom underwent PK in at least 1 eye) who had CCO with iridocorneal or keratolenticular adhesions (Peters' anomaly types I and II, respectively). Eight further children with CYP1B1 mutations who had CCO from birth and glaucoma underwent successful glaucoma treatment but had persistent diffuse CCO without iridocorneal or keratolenticular adhesions. Three of these underwent bilateral PK, and the histologic results were not consistent with any hitherto recognized congenital corneal dystrophy and showed abnormalities of the central corneal endothelium. CONCLUSIONS: Both severe CCO and isolated infantile glaucoma are associated with CYP1B1 mutations. The severe CCO phenotype reported herein often requires PK and has typical histopathologic changes. The mutations associated with this phenotype have not been reported previously. This phenotype may explain the patient described by Von Hippel in 1897.

Adjunctive use of bevacizumab versus mitomycin C with Ahmed valve implantation in treatment of pediatric glaucoma.

PURPOSE: Evaluate the use of bevacizumab (avastin) injected around valve body intraoperatively versus the use of mitomycin C as a single intraoperative application at the site of Ahmed valve implantation to enhance the survival of Ahmed valve in cases of pediatric glaucoma's as regarding the efficacy and complications. METHOD: Twenty eyes of 16 patients with pediatric glaucoma underwent Ahmed valve implantation with bevacizumab (avastin) (1.25 mg in 0.05 mL) injected around the valve body after completing the surgery compared with 20 eyes of 14 patients with pediatric glaucoma who underwent Ahmed valve implantation with application of mitomycin C (0.4 mg/mL for 3 min) before valve implantation and 20 eyes of 18 patients managed by Ahmed valve only (control group). The follow-up period was 12 months. RESULTS: The results revealed that 80% total success (70% complete success and 10% qualified success) in first group in which avastin augmented Ahmed valve was performed compared with group II in which mitomycin C was used during Ahmed valve implantation 90% total success (80% complete success and 10% qualified success) and this difference between the 2 groups was nonsignificant (P>0.05), but in group III complete success occurred in (60%) only with no cases of qualified success (P>0.05). CONCLUSIONS: The adjunctive use of bevacizumab or mitomycin C during Ahmed valve implantation significantly enhances the valve survival and the first drug seems to be much safer with no visually devastating complications.

Combined trabeculotomy-trabeculectomy augmented with 5-fluorouracil in paediatric glaucoma.

BACKGROUND: To describe our experience of combined trabeculotomy-trabeculectomy in paediatric glaucomas with a special emphasis on the use of 5-fluorouracil and releasable sutures. DESIGN: Retrospective review carried out at Manchester Royal Eye Hospital, UK, a tertiary referral centre. PARTICIPANTS: Twenty-nine eyes of 21 consecutive patients with congenital glaucoma undergoing combined trabeculotomy-trabeculectomy augmented with 5-fluorouracil. METHODS: 5-Fluorouracil augmented combined trabeculotomy-trabeculectomy was carried out with intense postoperative management and suture adjustment of releasable sutures within the first 3 weeks after surgery. Peribleb 5-fluorouracil injections were given repeatedly if there were signs of aggressive bleb scarring. MAIN OUTCOME MEASURES: Absolute success was defined as intraocular pressure of 21 mmHg or less, clear cornea and absence of progressive glaucomatous optic disc changes at last follow up, whereas qualified success was defined as these endpoints with anti-glaucoma medication. RESULTS: Absolute success was achieved in 19 out of 29 eyes (65.5%), and a further 4 (13.8%) had qualified success. There was no difference in the surgical outcomes of primary infantile glaucoma and secondary causes of paediatric glaucoma such as anterior segment dysgenesis. Combined trabeculotomy-trabeculectomy had a significantly greater success rate as a secondary procedure rather than as a primary procedure. CONCLUSION: 5-Fluorouracil-enhanced combined trabeculotomy-trabeculectomy with releasable sutures appears to be an effective procedure for congenital glaucoma refractory to goniotomy. It is less effective as a primary procedure when severe corneal haze prevents goniotomy in newborn congenital glaucoma. Intense postoperative monitoring including active bleb manipulation with needling and 5-fluorouracil injections may increase the success of the procedure.