Management Of Congenital Glaucoma Research Articles

Penetrating Keratoplasty in Congenital Glaucoma.

Childhood glaucoma is one of the most common causes of corneal opacity in childhood and is associated with various pathological corneal changes, including corneal enlargement, corneal clouding, and edema. Congenital glaucoma (CG) may cause a decrease in vision outcomes due to corneal opacity or clouding, which is often associated with stimulus deprivation amblyopia. Therefore, to create a balance between preventing amblyopia and sustaining corneal clearance, patients with CG can be managed with early penetrating corneal transplantation surgery along with advanced glaucoma management. To investigate the graft survival rate and factors affecting graft survival in patients with congenital glaucoma who underwent penetrating keratoplasty (PKP). Cross-sectional. Patients with congenital glaucoma who underwent PKP were retrospectively evaluated. The associations between age, corneal diameter, presence of ocular comorbidities, concurrent ocular surgeries with corneal graft, and visual outcomes were assessed. Among the 30 eyes enrolled in the study, 6 (20%) had aniridia, 6 (20%) had Axenfeld-Rieger syndrome, and 18 (60%) were diagnosed with primary congenital glaucoma. Graft survival rates were 66.6% and 63.33% at 12 and 24 months, respectively. At the end of the follow-up, the overall graft survival rate was 60%. Statistical significance was observed between patient age at the time of surgery and graft failure (p = 0.02). Graft failure was associated with a younger patient age. Functional vision was achieved in 53.3% of patients. The management of congenital glaucoma and its corneal complications is a delicate issue that requires great effort. PKP in congenital glaucoma was moderately successful in the present study. To provide functional vision, PKP could be the treatment of choice.

Bilateral Congenital Glaucoma – A Rare Case Report

Background: Glaucoma in childhood is a potentially blinding condition, yet, there is a lack of epidemiologic and clinical data regarding this pathology. Unlike glaucoma in adults, which is notoriously difficult to detect since there are no symptoms, glaucoma in children does present with signs and symptoms which can be detected by paediatricians and even parents, who are usually the first contacts of these children. Methodology: A detailed evaluation under general or local anaesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy–trabeculectomy offers the best hope of success in advanced cases. Result: The childhood glaucoma research network (CGRN) has provided a new classification system of paediatric glaucoma’s. Careful diagnosis and timely intervention leads to the most promising outcome and gives these children a long morbidity free life. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Conclusion: Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must.

Evolving Perspectives on Congenital Glaucoma

Congenital glaucoma differs from adult glaucoma in that it represents a panocular disorder defined by structural, functional, and physiologic parameters. In order to achieve excellent visual outcomes, the management of congenital glaucoma involves concurrently controlling intraocular pressure and treating amblyopia, while minimizing any influence of a compromised visual axis. Thus far, difficulty in assessing visual function in pre-verbal children has resulted in a paucity of long-term visual outcome data. However, based on commonly recognized obstacles to structural/physiologic stability and functional rehabilitation status, we propose a congenital glaucoma severity scale in order to provide a cross-sectional stratification of disease severity. As the severity of pathology evolves over time, the severity score should be adjusted accordingly. The initial score and rate of changes in severity (“severity slope”) will help in prognosticating outcome. Future studies will be required to validate, calibrate, and discriminate this severity scale before clinical and research applications.

Rare Diseases of the Anterior Segment of the Eye: Update on Diagnosis and Management

This special issue is focused on the current approaches used to identify and manage rare diseases of the anterior segment of the eye, which range from congenital to acquired disorders that are caused by ocular or systemic conditions and often have consequences that extend beyond the anterior segment of the eye. Specifically, the groups of Dr. R. Cascella et al. and Dr. G. L. Scuderi et al. provided a complete overview on primary congenital glaucoma and pediatric glaucoma, respectively, from diagnosis to treatment outcomes; the groups of Dr. D. Dobrowolski et al. and Dr. B. E. Ramirez et al. described the use of oral mucosa and stem cell therapy, respectively, for ocular surface reconstruction in rare diseases such as aniridia; Dr. F. Mantelli and colleagues reviewed the diagnosis and management of congenital corneal anesthesia and neurotrophic keratitis; Dr. A. K. Nowinska and colleagues compared different tomographic approaches to evaluate rare corneal dystrophies; the groups of Dr. M. Oldak and Dr. S. P. Patel explained the pathophysiology of Fuchs endothelial corneal dystrophy and congenital hereditary endothelial corneal dystrophy, respectively; Dr. M. Sacchetti and colleagues provide a guide to diagnose and manage the iridocorneal endothelial (ICE) syndrome; Dr. S. Abdolrahimzadeh and colleagues described a series of rare diseases and ophthalmic alterations that may lead to glaucoma; Dr. M. F. Suarez et al. described how environmental agents can influence a rare corneal disease, climatic droplet keratopathy. As can be seen from the variety of topics presented in this special issue, the disorders grouped under the umbrella term of rare diseases of the anterior segment of the eye can be considered quite heterogenous and numerous and, in turn, represent a substantial clinical burden on a worldwide basis [1]. In fact, although single disorders are rarely encountered in clinical practice, when taken together, they are not seldom seen. In addition, these diseases often represent a clinical challenge due to the paucity of specific diagnostic criteria and the lack of specific treatments. In fact, rare disorders tend to remain orphan drug indications due to the difficulty of running clinical trials, and surgical approaches are often inadequate due to concomitant anatomical and congenital anomalies. To make the clinical picture of the rare diseases of the anterior segment of the eye even more difficult to manage, they often have consequences that extend beyond the anterior segment and can severely impair vision. Specifically, while corneal opacities and iris anomalies may directly affect visual function, several diseases such as iridocorneal angle anomalies may induce glaucoma with consequent damage to the optic nerve. In addition, several congenital diseases of the anterior segment of the eye are also associated with facial anomalies and other malformations of intraocular structures, making this group of disorders particularly complex for the ophthalmologist, who encounters obstacles in patients' management from diagnosis to treatment [2]. The recent advances in translational research, which are reshaping modern ophthalmology, allowed identifying the link between different clinical phenotypes and specific mutations in genes regulating the normal formation and maturation of the anterior segments of the eye [3]. In turn, our ability to understand the pathogenesis of rare and complex ophthalmic diseases has improved and, recently, novel treatments are being proposed for selected disorders of the anterior segment. For instance, biotechnology approaches for the pharmaceutical development of recombinant human molecules as well as novel procedures for ex vivo expansion of limbal stem cells may soon open novel therapeutic perspectives for patients with previously untreatable corneal disorders [4, 5]. Similarly, pre- and posttest genetic counseling plays an essential role in the achievement of an appropriate management of congenital glaucoma, in terms of medical, social, and psychological impact of the disease [6]. Nevertheless, due to the complexity of most of the rare anterior segment disorders of the eye, ranging from corneal disorders such as corneal dystrophies, neurotrophic keratitis, iridocorneal anomalies, and congenital glaucoma, further progress is mandatory to exert a significant impact on the natural history of the diseases [7–10]. Hopefully, the constant growth of novel diagnostic tools, accompanied by a better understanding of the disease pathophysiology, will also foster more preclinical and clinical research in this area of high medical need. Alessandro Lambiase Flavio Mantelli Marta Sacchetti Siavash Rahimi Giacomina Massaro-Giordano

Trabeculectomy for congenital glaucoma in University College Hospital, Ibadan: A 7 year review of cases

Introduction: Congenital glaucoma is an uncommon ocular condition affecting about 1/15,000 live births. About two third of cases are bilateral with a higher prevalence in males. The definitive treatment requires surgery which could be trabeculectomy, trabeculotomy, goniotomy, drainage tubes or a combination of trabeculotomy and trabeculectomy. Aim: To determine the outcome of trabeculectomy in patients with congenital glaucoma seen at the University College Hospital Ibadan between 2007 and 2013. Methods: The medical records of all patients with congenital glaucoma seen between the years 2007 and 2013 were retrieved. Inclusion criteria were patients who had trabeculectomy with or without anti-metabolites within this period. The main outcome measures assessed were postoperative intraocular pressure (IOP) and corneal clarity. Data was analyzed with the statistical Package for Social Sciences version 21. Results: Thirty-four eyes of 18 patients were reviewed out of which 16 patients had bilateral trabeculectomy while two had unilateral trabeculectomy. The mean preoperative IOP was 26.31 mmHg while the mean IOP in the immediate postoperative period, at 3 months after surgery and at last follow-up visit were 13.31 mmHg, 12.78 mmHg and 15.89 mmHg respectively. IOP was <21 mmHg in 81.82% of the eyes as at last clinic visit. All eyes had cloudy corneas preoperatively while 38.24% had minimal residual corneal opacity (Haab's striae) as at last follow-up visit. Conclusion: In the absence of equipment for other surgical options, trabeculectomy is an effective surgical approach in the management of congenital glaucoma in the short term.

Update on congenital glaucoma

Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge to the ophthalmologist. A detailed evaluation under general anesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy–trabeculectomy offers the best hope of success in advanced cases. Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the management of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must.

Management of congenital glaucoma in neurofibromatosis type 1: a report of two cases

The most frequent ophthalmic lesions associated to neurofibromatosis type 1 include iris Lisch nodules, optic nerve gliomas, and neurofibromas located on the eyelid, conjunctiva or orbit. Glaucoma is much less frequent, and it may be difficult to diagnose and treat. We present two patients with neurofibromatosis type 1 and associated congenital glaucoma. In case 1, the glaucoma was present at birth, and was the first symptom of the disease. It was surgically treated by means of an Ahmed glaucoma valve implantation, with good intraocular pressure (IOP) control after 13 months of follow-up. In case 2, the glaucoma appeared at 5 months old, and a trabeculectomy was initially performed; it failed 2 years later and an Ahmed glaucoma valve was then implanted, with adequate IOP control after 7 years of follow-up. Both children were males, and in both cases there were orbital neurofibromas and a dysplasia of the greater wing of the sphenoid of the same side. In case 2, orbital enlargement surgery was also performed at 4 years old. Congenital glaucoma management in the context of neurofibromatosis is very complex due to the frequent association of orbital and eyelid tumors and bone dysplasia, and its prognosis is usually poor. We present two cases in which good control of IOP has been achieved with Ahmed glaucoma valve implantation after 13 months and 7 years of follow-up.

Long Term Outcome of Surgical Management of Congenital Glaucoma “Arabic abstracts”

Long Term Outcome of Surgical Management of Congenital Glaucoma “Arabic abstracts”

Long Term Outcome of Surgical Management of Congenital Glaucoma

Abstract Objective To look into the long-term outcome of surgical treatment of congenital glaucoma. Methods Children with congenital glaucoma that underwent goniotomy, trabeculotomy, combined trabeculotomy–trabeculectomy, trabeculectomy, cyclocryotherapy, Nd-Yag-cyclophotocoagulation, and Ahmed valve implant from January 1988 to December 1997 were reviewed in December 2007. Results 44 eyes of 25 patients, 16 male and 9 female, reviewed. The mean age at diagnosis was 10.5 (range 0.03–180) months. Twenty three (92%) patients were diagnosed to have congenital glaucoma at or shortly after birth. Thirty seven eyes (84.1%) had primary infantile glaucoma, 3 eyes (6.8%) had developmental glaucoma and 4 eyes (9.1%) had secondary glaucoma. The mean corneal diameter 12.7 (range 9–18) mm, and mean cupping 0.7 (range 0.2–1.0). 78 glaucoma procedures were performed with the mean patient's age at the time of surgery 34.4 (range 0.07 - 181) months. The mean intraocular pressure reduced from 26.7 to 14.0 mmHg in 15 eyes (34%) after a mean number of operation/eye of 1.8, and a mean follow up of 54.3 months. Kaplan-Meier life-table analysis showed decreasing success rates with time of all procedures. However, trabeculectomy was clearly the most successful in our hands. No serious surgical complications. Vision (≥ 6/12) achieved in 27.3% of eyes, and 63.6% of the eyes were legally blind (≤ 6/60). Conclusion Primary trabeculectomy is safe and effective in children with CG operated on by general ophthalmologist. The success rate of all surgical procedures decreased on long term follow up. The visual outcome is satisfactory in about 1/3 of the investigated eyes.

Congenital glaucoma surgery: a neglected field in ophthalmology?

Primary congenital glaucoma is a rare disease that is not usually seen by the general ophthalmologist. Only one out of 10 000–18 000 newly born children in European populations presents...

Problems of management of primary congenital glaucoma in Benin City, Nigeria

To determine the problems encountered in the management of primary congenital glaucoma in developing countries. A prospective study of new patients with a diagnosis of primary congenital glaucoma attending the eye clinic of the University of Benin Teaching hospital, Benin City, Nigeria over a 5 year period, from July 2001 to June 2006. The age, sex, presenting complaints and duration were recorded. They were examined by the aid of a pen torch, hand-held slit lamp and direct ophthalmoscope. Intraocular pressure and corneal diameter were measured under anaesthesia. The patients were followed up till June 2006 and the problems encountered in management were recorded. Eight children comprising 5 males (62.5%) and 3 females (37.5%) with primary congenital glaucoma were seen. All the patients (100%) presented with buphthalmos and 7 (87.5%) had corneal opacity. Four (50%) accepted surgery but only 2 (25%) eventually had surgery. Maximum follow-up was 7 months. Presentation with features of advanced disease, poor acceptability of surgery, poor follow-up and low surgical rate are problems associated with the management of congenital glaucoma in Benin City, Nigeria.

Management of Intracorneal Bleb After Trabeculectomy for Congenital Glaucoma

Congenital glaucoma is a well-recognized entity that can occur in the presence of anterior segment dysgenesis. Trabeculectomy is an accepted intervention in the management of congenital glaucoma. The surgical technique as well as complications is well described. This is a case report of a 3-month-old girl with anterior segment dysgenesis and glaucoma. She was referred post-trabeculectomy with persistent corneal opacity to be considered for penetrating keratoplasty and was found to have intrastromal corneal bleb. Ultrasound biomicroscopy confirmed communication of the corneal bleb with the anterior chamber, and the bleb was treated by autologous blood injection at the trabeculectomy site, under acetazolamide cover. We present evidence suggesting that abnormal structure was the etiologic basis for corneal bleb formation and describe our management of this previously unreported complication of trabeculectomy.

Serial axial length measurements in congenital glaucoma

PURPOSE: To illustrate the utility of axial length measurements in the management of congenital glaucoma. METHODS: Interventional case series. Twelve eyes of six consecutive patients with congenital glaucoma were followed with serial axial length measurements. The axial growth patterns were compared with the normal growth curve. RESULTS: Eight (66.7%) of 12 eyes had initial axial lengths longer than the normal range, and 4 eyes (33.3%) were within the normal range. After surgical intervention with good intraocular pressure control in 10 eyes, 3 eyes showed a decrease of axial length and 7 eyes showed no decrease of axial length, but the axial growth pattern paralleled the normal growth curve. After surgical intervention with poor intraocular pressure control, 2 eyes had axial growth pattern greater than the normal pattern. CONCLUSION: With intraocular pressure control in congenital glaucoma, axial length may decrease or the axial growth pattern may parallel the normal growth curve. Axial length measurements may be useful to monitor the control of congenital glaucoma.