Pediatric Glaucoma: a literature's review and analysis of surgical results

The purpose of this paper is to review the surgical options available for the management of pediatric glaucoma, to evaluate their advantages and disadvantages together with their long-term efficacy, all with the intent to give guidelines to physicians on which elements are to be considered when taking a surgical decision. Currently there is a range of surgical procedures that are being used for the management of pediatric glaucoma. Within these, some are completely new approaches, while others are improvements of the more traditional procedures. Throughout this vast range of surgical options, angle surgery remains the first choice in mild cases and both goniotomy and trabeculotomy have good success rates. Trabeculectomy with or without mitomycin C (MMC) is preferred in refractory cases, in aphakic eyes, and in older children. GDIs have a good success rate in aphakic eyes. Nonpenetrating deep sclerectomy is still rarely used; nevertheless the results of ongoing studies are encouraging. The different clinical situations should always be weighed against the risks associated with the procedures for the individual patients. Glaucomatous progression can occur many years after its stabilization and at any time during the follow-up period; for this reason life-long assessment is necessary

Comparison of Circumferential and Traditional Trabeculotomy in Pediatric Glaucoma

Purpose To compare intraocular pressure (IOP) control of pediatric glaucoma patients undergoing traditional trabeculotomy (<360 degrees or partial) with those receiving 360-degree circumferential trabeculotomy. Methods The medical records of pediatric glaucoma patients receiving trabeculotomy at a single institution from 2000 to 2012were retrospectively reviewed. Patients were divided into two groups: a traditional trabeculotomy group and 360-degree trabeculotomy group. IOP at baseline and at 1, 3, 6, and 12 months’ follow-up were compared within and each groups. Results A total of 77 eyes of 56 patients (age at surgery, 1.52 ± 2.68 years) in the traditional group and 14 eyes of 10 patients in the 360-degree group (age at surgery, 0.61 ± 0.42 years) were included. Mean baseline IOP was similar in both groups (traditional, 28.75 ± 8.80 mm Hg; 360-degree, 30.35 ± 6.04 mm Hg; t test; P = 0.43). Mean 1-year IOP was 17.05 ± 5.92 mm Hg in the traditional group and 11.0 ± 2.31 mm Hg in the 360-degree group. At 1-year, the surgical success rate was 58.44% in the traditional group and 85.71% in the 360-degree group; 32 eyes in the former and 2 eyes in the latter required another glaucoma procedure within 1 year for IOP control. For both groups, compared to baseline values, IOP decreased significantly with all postoperative measurements (paired t test, all P < 0.01). The 360-degree group had significantly lower IOP compared to the traditional group at 1-year (t test, P < 0.01). Conclusions Both 360-degree and traditional trabeculotomy significantly reduced IOP in children through 1 year’s follow-up, although the former procedure shows better 1-year postoperative IOP control, with higher rate of surgical success

Rare diseases leading to childhood Glaucoma. epidemiology, pathophysiogenesis, and management

Noteworthy heterogeneity exists in the rare diseases associated with childhood glaucoma. Primary congenital glaucoma is mostly sporadic; however, 10% to 40% of cases are familial. CYP1B1 gene mutations seem to account for 87% of familial cases and 27% of sporadic cases. Childhood glaucoma is classified in primary and secondary congenital glaucoma, further divided as glaucoma arising in dysgenesis associated with neural crest anomalies, phakomatoses, metabolic disorders, mitotic diseases, congenital disorders, and acquired conditions. Neural crest alterations lead to the wide spectrum of iridocorneal trabeculodysgenesis. Systemic diseases associated with childhood glaucoma include the heterogenous group of phakomatoses where glaucoma is frequently encountered in the Sturge-Weber syndrome and its variants, in phakomatosis pigmentovascularis associated with oculodermal melanocytosis, and more rarely in neurofibromatosis type 1. Childhood glaucoma is also described in systemic disorders of mitotic and metabolic activity. Acquired secondary glaucoma has been associated with uveitis, trauma, drugs, and neoplastic diseases. A database research revealed reports of childhood glaucoma in rare diseases, which do not include glaucoma in their manifestation. These are otopalatodigital syndrome, complete androgen insensitivity, pseudotrisomy 13, Brachmann-de Lange syndrome, acrofrontofacionasal dysostosis, caudal regression syndrome, and Wolf-Hirschhorn syndrome

Latanoprost and Dorzolamide for the Treatment of Pediatric Glaucoma: The Glaucoma Italian Pediatric Study (Gipsy), Design and Baseline Characteristics

INTRODUCTION: To investigate the efficacy of a treatment strategy with latanoprost and dorzolamide in primary pediatric glaucoma patients partially responsive to surgery. METHODS: Single arm, prospective, interventional multicenter study. Primary pediatric glaucoma patients younger than 13 years after a single surgical procedure with IOP between 22 and 26 mmHg were considered eligible. At baseline, patients were allocated to latanoprost monotherapy once daily. Depending on intraocular pressure (IOP) reduction at first visit, the patients were allocated to one of three groups: continuation of latanoprost monotherapy, addition of dorzolamide twice daily, or switch to dorzolamide three times daily. The same approach for allocation in medication groups was used in all subsequent visits. Patients in the dorzolamide monotherapy group with IOP reduction <20% from baseline were considered non-responders and withdrawn. Study treatment and patient follow-up will continue for 3 years or until treatment failure. The primary endpoint is the percentage of responders. Secondary endpoints are time to treatment failure and frequency of adverse events. RESULTS: A total of 37 patients (69 eyes) were enrolled. The mean age was 4.0 ± 3.8 years, the female/male ratio was 1/1.7, and the majority of patients were Caucasian. Eighty percent of patients had bilateral glaucoma. Goniotomy was the most frequently performed surgery (38.6%), followed by trabeculotomy (22.8%), trabeculectomy (21.1%), and trabeculectomy plus trabeculotomy (17.5%). The baseline IOP was 23.6 ± 1.5 mmHg. CONCLUSION: The study population is representative of patients frequently encountered after the first surgery for primary pediatric glaucoma. The study will produce evidence on the medium-term efficacy of a defined pharmacological approach

Long-Term Outcomes of Trabeculectomy Augmented with Mitomycin C Undertaken within the First 2 Years of Life

To evaluate the long-term effectiveness and safety of mitomycin C (MMC)–augmented trabeculectomy undertaken within the first 2 years of life for the surgical management of glaucoma. DESIGN: Retrospective, consecutive, noncomparative case series. PARTICIPANTS: All children who underwent MMC-augmented trabeculectomy within 2 years of birth between May 2002 and November 2012. METHODS: The medical records of 40 consecutive eyes of 26 children who underwent surgery by a single surgeon were reviewed. Data collected during routine clinical care were analyzed. MAIN MEASURE OUTCOMES: Assessment of clinical outcomes included intraocular pressure (IOP), final visual acuity, bleb morphology, surgical complications (early and late), postoperative interventions, and further glaucoma surgery performed. Surgical success was defined as final IOP of 5 mmHg or more and of 21 mmHg or less, with anti-glaucoma medications (qualified success) and without (complete success), stable ocular dimensions and optic disc cupping, and no further glaucoma surgery (including needling) or loss of light perception. Surgical outcomes were evaluated using Kaplan-Meier life table analysis. RESULTS: Forty eyes of 26 children were studied over a mean follow-up period of 62.8 months. Most cases (80%) were of primary congenital glaucoma after failed goniotomy surgery. Cumulative probabilities of survival at 1, 5, and 7 years were 78%, 67%, and 60%, respectively. Of eyes regarded as successful, 96% (25/26 eyes) had controlled IOP without topical medication and 44% achieved visual acuity of 20/40 or better. In only 1 of the 40 eyes did a cystic avascular bleb develop, with all the other eyes being non-cystic in nature (diffuse and elevated or flat) at final follow-up. Sixty-four percent (9/14 eyes) of cases regarded as failures ultimately underwent glaucoma drainage device implantation. CONCLUSIONS: A contemporary pediatric trabeculectomy technique augmented with MMC is an effective procedure in the management of glaucoma within the first 2 years of life, as shown by the successful long-term outcomes and low incidence of sight-threatening complications. Trabeculectomy after failed goniotomy surgery or as a primary surgical intervention may offer a phakic infant with glaucoma an excellent opportunity to achieve long-term control of IOP without medications and may be associated with optimal visual outcomes

Deep sclerectomy combined with trabeculectomy in pediatric glaucoma

Résumé : Ce travail porte sur l'étude rétrospective d'une série de jeunes patients opérés de glaucomes pédiatriques. Le but est d'évaluer le résultat au long cours d'une intervention chirurgicale combinant une sclérectomie profonde et une trabéculectomie (sclérectomie profonde pénétrante). Durant la période de mars 1997 à octobre 2006, 28 patients on été suivis pour évaluer le résultat de cette chirurgie effectuées sur 35 yeux. Un examen ophtalmologique complet a été pratiqué avant la chirurgie, 1 et 7 jours, puis 1, 2, 3, 4, 6, 9, 12 mois, enfin tous les 6 mois après l'opération. Les critères d'évaluation du résultat postopératoire sont : les changements de pression intraoculaire, le traitement antiglaucomateux adjuvant, le taux de complication, le nombre de reprises chirurgicales,- l'erreur de réfraction, la meilleure acuité visuelle corrigée, l'état et le diamètre de la cornée. L'âge moyen est de 3.6 ± 4.5 ans et le suivi moyen de 3.6 ± 2.9 ans. La pression intraoculaire préopératoire de 31.9 ± 11.5 mmHg baisse de 58.3% (p&lt;0.005) à la fin du suivi. Sur les 14 patients dont l'acuité visuelle a pu être mesurée, 8 (57.1 %) ont une acuité égale ou supérieure à 5/10e, 3 (21.4%) une acuité de 2/10e après intervention. Le taux de succès cumulatif complet à 9 ans est de 52.3%, le succès relatif 70.6%. Les complications menaçant la vision (8.6%) ont été plus fréquentes dans les cas de glaucome réfractaire. Pour conclure la sclérectomie profonde combinée à une trabéculectomie est une technique chirurgicale développée afin de contrôler la pression intraoculaire dans les cas de glaucomes congénitaux, juvéniles et secondaires. Les résultats intermédiaires sont encourageants et prometteurs. Les cas préalablement opérés avant cette nouvelle technique ont cependant un pronostic moins favorable. Le nombre de complications menaçant la vision est essentiellement lié à la sévérité du glaucome et au nombre d'interventions préalables. Abstract : Purpose : To evaluate the outcomes of combined deep sclerectomy and trabeculectomy (penetrating deep sclerectomy) in pediatric glaucoma. Design : Retrospective, non-consecutive, non-comparative, interventional case series. Participants : Children suffering from pediatric glaucoma who underwent surgery between March 1997 and October 2006 were included in this study. Methods : A primary combined deep sclerectomy and trabeculectomy was performed in 35 eyes of 28 patients. Complete examinations were performed before surgery, postoperatively at 1 and 7 days, at 1, 2, 3, 4, 6, 9, 12 months and then every 6 months after surgery. Main Outcome Measures : Surgical outcome was assessed in terms of intraocular pressure (IOP) change, additional glaucoma medication, complication rate, need for surgical revision, as well as refractive errors, best corrected visual acuity (BCVA), and corneal clarity and diameters. Results : The mean age before surgery was 3.6 ± 4.5 years, and the mean follow-up was 3.5 ± 2.9 years. The mean preoperative IOP was 31.9 ± 11.5 mmHg. At the end of follow-up, the mean IOP decreased by 58.3% (p&lt;0.005), and from 14 patients with available BCVA 8 patients (57.1 %) achieved. 0.5 (20/40) or better, 3 (21.4%) 0.2 (20/100), and 2 (14.3%) 0.1 (20/200) in their better eye. The mean refractive error (spherical equivalent) at final follow-up visits was +0.83 ± 5.4. Six patients (43%) were affected by myopia. The complete and qualified success rates, based on a cumulative survival curve, after- 9 years were 52.3% and 70.6%, respectively (p&lt;0.05). Sight threatening complications were more common (8.6%) in refractory glaucomas. Conclusions : Combined deep sclerectomy and trabeculectomy is a surgical technique developed to control IOP in congenital, secondary and juvenile glaucomas. The intermediate results are satisfactory and promising. Previous classic glaucoma surgeries performed before this new technique had less favourable results. The number of sight threatening complications is related to the severity of glaucoma and number of previous surgeries

Causes of congenital corneal opacities and their management in a tertiary care center.

PURPOSE: To evaluate causes and management of congenital corneal opacities (CCO) diagnosed in a tertiary care eye center and to compare the data with a previous study at the same institution. METHODS: Computerized medical records in all patients with congenital corneal opacities diagnosed in the Cornea Service at Wills Eye Hospital (Philadelphia, PA) between January 1, 2007, and December 31, 2015, were retrospectively reviewed. Children aged 12 years and younger at the first visit were included in the study. Patients\u27 demographics, ocular diagnosis, laterality, associated ocular abnormalities, other ocular surgery performed prior or subsequent to the first visit, and their treatment were extracted from the medical records. RESULTS: A total of 77 eyes in 56 patients were examined. The mean age at presentation was 32.8 ± 44.2 months, with the mean follow-up period of 26.7 ± 30.1 months. The most frequent diagnosis was Peters anomaly (53.2%), followed by limbal dermoid (13.0%), aniridia with glaucoma and microphthalmos (6.5%), sclerocornea and congenital glaucoma (5.2%), idiopathic (3.9%), Axenfeld-Rieger anomaly and Hurler syndrome (2.6%), and microcornea (1.3%). Primary keratoplasty was performed in 26 eyes, with the outcome rate in the clear cornea of 76.0% during the follow-up. CONCLUSION: Peters anomaly is the most common cause of congenital corneal opacities encountered at our institution. Penetrating keratoplasty is the most frequent choice of corneal surgery to treat congenital corneal opacities. Additional interventions during penetrating keratoplasty were moderately positively correlated with graft failure. This study also shows the rates of some etiologies of that changed over the recent decades in our tertiary care Cornea Service. Although Peters anomaly remains the most common presenting reason for congenital corneal opacities, its rate appears to be increasing over the recent decade. Congenital corneal opacities due to birth trauma, which is one of the preventable causes, were observed in a previous study in our clinic; however, no new cases were noted in this study

Bilateral diffuse choroidal hemangioma in Sturge Weber syndrome: a case report highlighting the role of multimodal imaging and a brief review of the literature

Purpose: The purpose of this paper is to present a patient with bilateral choroidal hemangioma in Sturge-Weber syndrome (SWS) and highlight multimodal imaging techniques for early detection and management of ocular alterations. Methods: A 37-year-old woman with diagnosis of SWS presented to our unit. The patient had been treated with pulsed dye laser for bilateral nevus flammeus and had right leptomeningeal angiomatosis. She had glaucoma, but ultrasound biomicroscopy did not show anterior chamber or ciliary body alterations. Results: Enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) showed bilateral diffuse choroidal hemangiomas in both eyes with choroidal thickness above 1000 μm. B-scan ultrasound examination showed diffuse choroidal hemangioma in both eyes, with a choroidal thickness of 1.53 mm and 1.94 mm in the right and left eye (RE, LE), respectively. Peripapillary retinal nerve fiber evaluation showed thinning of the retinal nerve fiber layer in both eyes. Conclusions: This report highlights multimodal imaging techniques for the critical assessment of patients with SWS, especially in rare cases with bilateral choroidal hemangioma of the choroid. Novel imaging modalities enable optimal management and follow-up of rare conditions, and our case adds further evidence to the existing literature