Secondary Congenital Glaucoma Associated With Retro-orbital Infantile Hemangioma: A Masquerade Syndrome.

PURPOSE: The main purpose of this study was to describe a unique case of retro-orbital infantile hemangioma (IH) simulating a congenital glaucoma requiring prompt management with systemic propranolol. STUDY DESIGN: This was a case report. RESULTS: A 2-month-old white female was referred to our department of ophthalmology for progressive, unilateral protrusion of the left eye associated with epiphora. Clinical examination was suggestive of congenital glaucoma, as evidenced by high intraocular pressure, optic disc cup, increased corneal diameter, and axial length. The left eye proptosis was measured at 4 mm with the Hertel exophthalmometer. Cycloplegic refraction discloses high degree of astigmatism in the left eye. A cerebral and orbital magnetic resonance imaging revealed an intraconal vascular mass evocative of orbital IH.Oral propranolol was initiated and resulted in a rapid radiologic reduction of the IH. Concomitantly, the notable finding was the reversal of congenital glaucoma parameters without the need of surgery. CONCLUSIONS: To the best of our knowledge, this is the first observation of a secondary congenital glaucoma reversal with oral propranolol used for orbital IH management.

Microcatheter-assisted Trabeculotomy for Primary Congenital Glaucoma After Failed Glaucoma Surgeries.

PURPOSE: To evaluate the effectiveness of microcatheter-assisted trabeculotomy (MAT) to treat primary congenital glaucoma after failed previous glaucoma surgeries. MATERIALS AND METHODS: Retrospective, noncomparative, interventional case series conducted at Beijing Tongren Eye Center, China. Outcome measures were compared between 3 groups: successful and complete (≥330 degrees) MAT; successful and partial (<330 degrees) MAT; or cases converted to traditional trabeculotomy when the Schlemm's canal could not be catheterized >180 degrees. Success was defined as final intraocular pressure ≤21 mm Hg, with (qualified success) or without (complete success) glaucoma medications. RESULTS: In total, 74 eyes of 63 consecutive patients were included. MAT was performed in 50 eyes (67.6%). Postoperative intraocular pressure and number of glaucoma drops (17.7±8.6 mm Hg, 0.6±1.2 medications) was significantly less than the preoperative values (35.3±7.2 mm Hg, 2.7±0.8 medications; P<0.001). Cumulative probabilities of qualified and complete success were 84.0% and 80.0% at 3-year follow-up with no difference between complete and partial trabeculotomies. MAT was not successfully performed in 24 eyes (32.4%), requiring conversion to traditional trabeculotomy and associated with greater incidence of previous surgeries (P<0.001), earlier age of disease onset (P=0.024) and worse corneal transparency (P=0.010). Cumulative probabilities of qualified and complete success were 37.0% and 29.2% at 3-year follow-up. CONCLUSIONS: Both complete and partial MAT achieved significant pressure reduction in cases of primary congenital glaucoma with previous failed glaucoma surgeries in intermediate term.

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.].

Manifestações oftalmológicas de Síndrome de Klippel Trenaunay / Ophthalmological manifestation of the Klippel Trenaunay Syndrome

RESUMO A Síndrome de Klippel-Trenaunay (SKT) é uma doença congênita rara, com maior prevalência no sexo masculino e incidência de 2-5:100.000. Apresenta-se, na forma clássica, como a tríade de manchas vinho porto, hipertrofia de membros e malformação venosa e/ou linfática. O diagnóstico é essencialmente clínico e devido à complexidade da síndrome, de natureza progressiva e ampla variedade de apresentações clínicas, os pacientes devem ser tratados de forma individualizada por uma equipe multidisciplinar. Alterações oftalmológicas associadas à SKT incluem anormalidades vasculares da órbita, íris, retina, coroide e nervo óptico. Relato de caso: Paciente de 23 anos, sexo feminino, portadora de SKT, em acompanhamento no Centro da Visão - Universidade Federal do Paraná, com queixa de diminuição da acuidade visual em olho direito. A paciente apresentava manchas vinho porto em dimidio direito e hipertrofia de membros ipsilateral. Foi diagnosticado glaucoma e realizados exames complementares oftalmológicos a fim de avaliar o grau de comprometimento dos campos visuais e o fundo de olho. A visão com a melhor correção foi de 20/100 OD e foi de 20/20 OE. À fundoscopia, constatou-se aumento da escavação do nervo óptico à direita - 0,75 x 0,90 mm. Optou-se por tratamento clínico com Cloridrato de Dorzolamida, Latanoprosta, Brimonidina e Timolol, com bons resultados a longo prazo - a tonometria de aplanação mostrou 19 mmHg OD e 15 mmHg OE, apesar da dificuldade na estabilização da doença. Conclusão: Relatos demonstram que os resultados dos tratamentos clínico e cirúrgico do glaucoma em associação à SKT são insatisfatórios quando comparados a outros tipos de glaucoma - o controle clínico não é possível em cerca de 1/3 dos pacientes, e o manejo cirúrgico tem alto índice de complicações. São necessários estudos mais expressivos que estabeleçam a correlação entre glaucoma e SKT e embasem o tratamento de escolha.

ABSTRACT The Klippel-Trenaunay Syndrome (KTS) is a rare congenital disease, which the prevalence is higher in males, and its incidence of 25:100,000. It is presented in its classic form as the triad of port-wine stains, enlarged limbs and venous and / or lymphatic malformation. The diagnosis is essentially clinical and due to the complexity of the syndrome, the progressive characteristic and the wide variety of clinical presentations, a multidisciplinary team should treat patients individually. The ocular changes associated with KTS include vascular, orbit, iris, retina, choroid and optic nerve abnormalities. Case report: A 23-year-old female patient, carrier KTS, being followed at Vision Center - Federal University of Paraná, complaining of decreased visual acuity in the right eye. The patient had port-wine stains in right hemibody and hypertrophy of ipsilateral members. Glaucoma was diagnosed and eye exams were performed to assess the degree of impairment of visual fields and fundus. The best correction was checked at 20/100 OD and 20/20 OS. At fundoscopy, there was increased excavation of the optic nerve right - 0.75 x 0.90 mm. Clinical treatment was chosen with Dorzolamide Hydrochloride, Latanoprost, Brimonidine and Timolol, presenting good long-term results - the tonometry showed 19 mmHg OD and 15 mmHg OS, despite the difficulty in stabilizing the disease. Conclusion: Reports have shown that the results of clinical and surgical treatments of glaucoma in association with KTS are unsatisfactory compared to other types of glaucoma - clinical control is not possible in about 1/ 3 of patients and the surgical management has a high rate of complications. Significant studies are needed to establish the correlation between glaucoma and KTS, and base the treatment of choice.

Supra-Tenon Capsule Implantation of the Ahmed Glaucoma Valve in Refractory Pediatric Glaucoma.

PURPOSE: To evaluate the efficacy of supra-Tenon capsule implantation of an Ahmed glaucoma valve (AGV) as a measure to decrease the fibrotic potential of the Tenon capsule on bleb formation and its subsequent effect on intraocular pressure (IOP) control in children with refractory glaucoma. SETTING: Mansoura Ophthalmic Centre, Faculty of Medicine, Mansoura University, Egypt. DESIGN: A prospective interventional study. PATIENTS AND METHODS: Twenty-two eyes of 12 children with refractory glaucoma underwent supra-Tenon capsule implantation of AGV. Ophthalmic examinations under general anesthesia including measurement of the corneal diameter and the IOP with Perkin's tonometer were performed preoperatively, on the first postoperative day, the first postoperative week, weekly for the first month, 2-weekly for the following 3 months, and monthly for at least 18 months. Postoperative complications and the number of glaucoma medications used preoperatively and postoperatively were recorded. The paired Student t test was used to compare preoperative and postoperative data. RESULTS: There were 12 eyes (54.6%) with refractory congenital glaucoma, 7 eyes (31.8%) with refractory pseudophakic glaucoma, and 3 eyes (13.6%) with refractory aphakic glaucoma. Patients included 10 male (83.3%) and 2 female (16.7%) children with a mean age of 16.3±9.7 months. The mean follow-up duration was 24.1±4.3 months. There was a statistically significant difference between the mean preoperative IOP (30.7±2.88 mm Hg) and the mean postoperative IOP (16.1±3.60 mm Hg) (t=16.22 and P=0.000, with a mean decrease in the IOP by 47.6%). The difference between the mean number of antiglaucoma medications before surgery (1.86±0.4) and after surgery (1.0±0.9) was also statistically significant (t=4.31 and P=0.000). Total success was achieved in 18 eyes (81.9%). Postoperative complications included tube exposure and slippage (10%), hypotony (10%), and hyphema (5%). CONCLUSIONS: Supra-Tenon capsule implantation of the AGV was successful in controlling the IOP with few postoperative complications in the management of children with refractory glaucoma.

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.

Impact of age, diagnosis, and history of glaucoma surgery on outcomes in pediatric patients treated with latanoprost.

PURPOSE: To evaluate the impact of age, glaucoma-specific diagnosis, and history of prior glaucoma surgery on outcomes in pediatric patients treated with latanoprost monotherapy. PATIENTS AND METHODS: Prospective, randomized, double-masked 12-week, multicenter study included individuals 18 years or younger with glaucoma. Subjects stratified by age (0 to <3, 3 to <12, 12 to 18 y), diagnosis [primary congenital glaucoma (PCG) vs. non-PCG], and baseline intraocular pressure (IOP; 22 to <27, 27 to 31, >31 mm Hg), and randomized (1:1) to latanoprost vehicle (8 AM) and latanoprost 0.005% (8 PM) or timolol 0.5% (or 0.25% for those less than 3 y old; 8 AM/8 PM). IOP and safety assessments performed and adverse events recorded at baseline, weeks 1, 4, 12. Post hoc analyses in age-specific and diagnosis-specific groups of latanoprost-treated subjects were conducted (intent-to-treat population). RESULTS: Sixty-eight subjects were treated with latanoprost (0 to <3, n=17; 3 to <12, n=26; 12 to 18, n=25); 82%, 42%, and 24%, respectively, had a primary diagnosis of PCG. Among Non-PCG subjects, 0% (0/3), 47% (7/15), and 63% (12/19) had a primary diagnosis of juvenile open-angle glaucoma in the 0 to <3, 3 to <12, and 12 to 18 year cohorts, respectively. Mean percent IOP reductions from baseline at week 12 were 22%, 24%, and 30% in the youngest through oldest age groups, respectively (P=0.3600). At week 12, a higher responder rate (≥15% IOP reduction) was observed in the non-PCG than in the PCG group (70% vs. 45%, respectively; P=0.0361). Latanoprost was well tolerated. CONCLUSION: All age and diagnosis subgroups showed clinically relevant (>20%) mean IOP reduction at week 12 with latanoprost monotherapy.

Congenital glaucoma with acquired peripheral circumferential iris degeneration.

Iris anomalies associated with congenital or early-childhood glaucoma include stable primary developmental abnormalities such as those associated with the Axenfeld-Rieger spectrum and aniridia. Secondary generalized iris atrophy from uncontrolled intraocular pressure is another potential iris finding in pediatric glaucoma. We document an unusual pattern of acquired peripheral circumferential iris degeneration in 2 unrelated children with otherwise-controlled congenital glaucoma. Genetic testing revealed a common homozygous CYP1B1 mutation in one (p.Gly61Glu) and a novel heterozygous FOXC1 deletion in the other (p.Tyr81_Pro95del).

Biomechanical profile of the cornea in primary congenital glaucoma.

PURPOSE: The aim of our study was to investigate the biomechanical properties of the cornea in primary congenital glaucoma (PCG) and to identify the potential ocular determinants, which affect the corneal biomechanical metrics. METHODS: Corneal hysteresis (CH), corneal resistance factor (CRF) and central corneal thickness (CCT) were measured in 26 patients with PCG (40 eyes) with the aid of ocular response analyser. In vivo laser-scanning confocal microscopy was used for the estimation of stromal keratocyte density (KD) and the evaluation of corneal endothelium. Twenty normal subjects (40 eyes) served as controls. Student's t-test and Pearson's correlation coefficients were used for statistical analysis. p Values <0.05 were considered statistically significant. RESULTS: Corneal hysteresis, CRF and CCT were significantly reduced in patients with PCG (all p < 0.05). Corneal hysteresis and CRF negatively correlated with the corneal diameter in both groups (r(1) = -0.53, r(2) = -0.66, p < 0.001 for CH and r(1) = -0.61, r(2) = -0.69, p < 0.001 for CRF). Moreover, we identified a significant correlation between CH and CRF with CCT in both groups (r(1) = 0.51, r(2) = 0.48, p < 0.001 for CH and r(1) = 0.45, r(2) = 0.44, p < 0.001 for CRF). Mean KD was significantly reduced both in the anterior and posterior corneal stroma in patients with PCG (764 ± 162 and 362 ± 112 cells/mm(2) , respectively) compared with controls (979 ± 208 and 581 ± 131 cells/mm(2) , respectively) (p < 0.001). There was no significant correlation between the keratocyte density in anterior and/or posterior stroma and CH or CRF in any group (r(1) = 0.29, r(2) = 0.31, p < 0.06). Mean endothelial cell density was also significantly reduced in PCG group (2920 ± 443 cells/mm(2) ) compared with control group (3421 ± 360 cells/mm(2) ) (p < 0.001). Pleomorphism and polymegalism were significantly increased in corneal endothelium of patients with PCG. CONCLUSIONS: Our results showed a significant reduction in CH and CRF in PCG. Both CH and CRF were negatively correlated with corneal diameter. A significant correlation of CH and CRF with CCT was identified in both groups. Keratocyte density was decreased in PCG, but did not have a significant impact on CH and CRF. Mean endothelial density was also decreased in PCG. Our results suggest that reduced CCT and increased corneal diameter are major ocular determinants for the modified corneal biomechanical profile in PCG, while cellular alterations in corneal stroma and endothelium have no significant biomechanical impact.

Infantile bilateral glaucoma in a child with ectodermal dysplasia.

Ectodermal dysplasia is a rare disease which affects at least two ectodermal-derived structures such as hair, nails, skin, sweat glands and teeth. Approximately 200 different conditions have been classified as an ectodermal dysplasia and X-linked hypohidrotic ectodermal dysplasia (XHED) represents the commonest form. Clinically, XHED is characterized by hypotrichosis, hypohidrosis and hypodontia. A variety of ocular manifestations have been reported in XHED, the most common being dryness of eyes due to tear deficiency and instability of the film secondary to the absence of meibomian gland function. Here we report a child with the distinctive clinical features of XHED confirmed with molecular diagnosis who presented with infantile bilateral glaucoma, in addition to the classical ocular involvement in XHED.

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.

Comparison of latanoprost and timolol in pediatric glaucoma: a phase 3, 12-week, randomized, double-masked multicenter study.

OBJECTIVE: To compare the efficacy and safety of latanoprost versus timolol in pediatric patients with glaucoma. DESIGN: Prospective, randomized, double-masked, 12-week, multicenter study. PARTICIPANTS: Individuals aged ≤18 years with glaucoma. METHODS: Stratified by age, diagnosis, and intraocular pressure (IOP) level, subjects were randomized (1:1) to latanoprost vehicle at 8 am and latanoprost 0.005% at 8 pm or timolol 0.5% (0.25% for those aged <3 years) twice daily (8 am, 8 pm). At baseline and weeks 1, 4, and 12, IOP and ocular safety were assessed and adverse events were recorded. Therapy was switched to open-label latanoprost pm and timolol am and pm for uncontrolled IOP. MAIN OUTCOME MEASURES: Mean IOP reduction from baseline to week 12. Latanoprost was considered noninferior to timolol if the lower limit of the 95% confidence interval (CI) of the difference was >-3 mmHg. A proportion of responders (subjects with ≥15% IOP reduction at weeks 4 and 12) were evaluated. Analyses were performed in diagnosis subgroups: primary congenital glaucoma (PCG) and non-PCG. RESULTS: In total, 137 subjects were treated (safety population; 12-18 years, n=48; 3-<12 years, n=55; 0-<3 years, n=34). Mean age was 8.8±5.5 years, and mean baseline IOP was 27.7±6.17 mmHg; 125 subjects completed the study, and 107 subjects were in the per protocol population. Mean IOP reductions for latanoprost and timolol at week 12 were 7.2 and 5.7 mmHg, respectively, with a difference of 1.5 mmHg (95% CI, -0.8 to 3.7; P=0.21). Responder rates were 60% for latanoprost and 52% for timolol (P=0.33). Between-treatment differences in mean IOP reduction for PCG and non-PCG subgroups were 0.6 mmHg (95% CI, -2.3 to 3.4) and 2.6 mmHg (95% CI, -0.8 to 6.1), respectively. Responder rates for latanoprost versus timolol were 50% versus 46% for the PCG group and 72% versus 57% for the non-PCG group. Both therapies were well tolerated. CONCLUSIONS: Latanoprost 0.005% is not inferior (i.e., is either more or similarly effective) to timolol and produces clinically relevant IOP reductions across pediatric patients with and without PCG. Both latanoprost and timolol had favorable safety profiles over the duration of this 3-month trial. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

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.

Buphthalmos following systemic steroid treatment.

Corticosteroid-induced glaucoma is a well-recognized phenomenon in adults, but not children. We describe an infant who developed juvenile glaucoma with buphthalmos while receiving systemic steroid treatment. The intraocular pressure normalized several months following discontinuation of treatment.

[Results of treatment with timolol in problematic cases (author's transl)]. / Ergebnisse der Timololbehandlung bei Problemfällen.

The authors report on the effect of timolol in cases of secondary glaucoma and buphthalmus in 143 eyes. Constant regulation of intraocular pressure was achieved by treatment with timolol in 67 eyes. A remarkable effect was observed in cases of complicated buphthalmus. The results were least favorable in cases of hemorrhagic secondary glaucoma.

Simple glaucoma before the age of 30 years.

'Juvenile glaucoma' is a meaningless term which should not be used. Glaucoma in young subjects can be hydrophthalmus, secondary or simple glaucoma. In this paper, early simple glaucoma is described in 15 patients (8 males, 7 females) below the age of 30 years, starting in some subjects already at 8 years of age. Inheritance was dominant in 6 patients. Anticipation could be shown in 2 pedigrees through 4 and 5 generations, respectively. The course of the disease was severe in 8 patients, with intraocular pressures over 40 mm Hg and/or heavy functional loss. There were usually no subjective symptoms. The chamber angles were gonioscopically normal in all patients. Miotics were effective but did not normalize the intraocular pressure. Surgical treatment is discussed.