ABSTRACT
Children comprise a unique population of patients in regard to the diagnostic and therapeutic approach of uveitic glaucoma. The management of glaucoma secondary to uveitis in children is extremely challenging and presents various difficulties, which are associated both with the underlying uveitis and the young age of the patients. The treatment of uveitic glaucoma calls for a thorough and individualized approach, involving both pharmacotherapeutic and surgical modalities. It appears that the efficient control of inflammatory activity plays a significant role in the final visual outcome of these patients. This study aims to review the current literature about the management of uveitic glaucoma in pediatric patients.
Introduction
The evaluation and management of uveitis in children is extremely challenging for the ophthalmologists that have to confront this clinical entity, whereas glaucoma in children is a potentially blinding condition. Uveitis can lead to several complications, such as secondary glaucoma, cataracts, synechiae, band keratopathy, and macular edema.1 There is some evidence that the rates of complications differ between adults and children, and some of the complications may be unique to children.1 Uveitic glaucoma represents a special category of secondary glaucoma in both adult and pediatric populations. The clinical outcomes of uveitic glaucoma in children depend on several factors (e.g., type, severity, and duration of the disease) and are often guarded, especially in complicated cases. The successful management of uveitic glaucoma in children calls for an early and accurate diagnosis and control of inflammation and intraocular pressure (IOP) to reduce the risk of progressive damage to the optic nerve and the risk of amblyopia.2 Treatment with ocular and systemic steroids, as well as with corticosteroid-sparing therapy has significantly contributed to the control of inflammation and improved the visual prognosis.3 In many cases, the successes of medical treatments are limited because of poor compliance or intolerable local or systemic side effects.2 Moreover, many uveitic patients with glaucoma may need surgical intervention to control IOP and preserve vision. There is high risk of significant visual loss from complications of uveitis and/or glaucoma over the lifespan of these patients, and this has significant impacts in terms of financial burdens, quality of life, and loss of productivity for the patients.2 This study focuses on the clinical features and management of uveitic glaucoma in childhood.
Discussion
In the past, patients with uveitic glaucoma had poor visual outcome due to delayed diagnosis and the limited anti-inflammatory and antiglaucoma therapeutic options.1,2 Over the last two decades, advances in diagnostic tools and new systemic anti-inflammatory medications have provided clinicians with more sophisticated approaches that can prevent late consequences of uveitis.1 However, uveitis remains a potentially devastating condition that can have severe impacts on vision through various complications such as glaucoma, cataract formation, macular edema, and formation of synechiae.3 More specifically, cataracts are very often associated with uveitis, either directly due to the inflammation or indirectly due to the use of topical and oral steroids. In eyes with chronic inflammation activity, cataract extraction can cause an exuberant postoperative inflammatory reaction, which can lead to complications including glaucoma, hypotony, macular edema, and optic disc swelling.50
In young children, regardless of whether reduced visual acuity derives from glaucoma, uncontrolled inflammation, or other complications, it can lead to amblyopia and consequently to life-long visual disability. This is also expected to affect the child’s education and performance at school. Early, prompt, and efficient management of uveitic glaucoma is significant, especially in patients of amblyogenic age (i.e., younger than 7-8 years old).21 Amblyopia should be treated with occlusion therapy, and when the issue is resolved and the eye is not inflamed, the child can have a refraction test for optimizing visual function. Furthermore, in children that have gone through postoperative aphakic rehabilitation, the presence of a specialist pediatric contact lens optometrist would be more than helpful.21
The treatment of glaucoma secondary to uveitis has several challenges, especially when it comes to surgical intervention. One of the major issues is the fact that in many cases there is an intense inflammatory reaction, which complicates both the control of uveitis and eye pressure.41 The administration of topical and periocular steroids has been correlated with high risk of several ocular complications in children. IOP elevation and steroid-induced glaucoma in particular can develop rapidly in children, become refractory to treatment, and persist even after stopping topical corticosteroids. Likewise in the adult population, systemic corticosteroids should be used mainly for limited periods due to the wide spectrum of adverse systemic effects. Moreover, systemic steroids can cause adverse ocular effects including glaucoma, cataract, and retinal and choroidal emboli.1,2 Additionally, when it comes to deciding the most suitable surgical intervention in those patients, it is important to take into account the status of the angle (i.e., whether the angle is open and the extent of synechiae formation). Ophthalmic surgeons should have a strategy that will offer the maximal chances of preserving vision and IOP over the long term with minimal ocular damage.41
Holistic management is one of the cornerstones of a successful approach to pediatric glaucoma. The management of this vulnerable group of patients calls for the expertise and collaboration of a multidisciplinary team. It is vital for the ophthalmologist to be in direct and continuous communication with the pediatricians and rheumatologists in order to ensure a thorough investigation for underlying systemic diseases and prompt initiation of disease-modifying agents if required. Before the administration of systemic medications, clinicians and pharmacists need to check that any kind of immunomodulatory was prescribed only if laboratory investigations were within normal limits.21 A pediatric glaucoma or uveitis nurse specialist could play a critical role in the training of patients and family in the administration of medications, especially when it comes to subcutaneous drugs.
Adequate monitoring of the uveitic glaucoma and response to treatment is crucial in children. Special attention should be paid to visual acuity and any changes in vision in children at risk for amblyopia. Regular and periodic follow-up examinations should be carried out to assess levels of inflammation (i.e., anterior chamber cells and flare, vitreous humor cells and vitreous haze), signs of uncontrolled inflammation (i.e., keratic precipitates and iris nodules), possible complications, and evidence of drug toxicity.2 Children should be followed up more closely than adults for evidence of uveitic glaucoma, as glaucomatous optic disc changes can progress very quickly in pediatric patients. Therefore, frequent visual field testing and dilated pupil examination of the optic discs along with optical coherence tomography when needed are strongly recommended. Chronic anterior uveitis patients with no previous systemic disorders (at presentation) should be questioned about the development of joint symptoms due to the fact that arthritis may present after the onset of ocular inflammation in some patients.21
Assessment of compliance with the treatment regimen is also critical, because children may need to receive their medications while at school or even apply the topical medications on their own. Compliance issues are common among teenagers that may need to receive a long-term drug therapy. Thus, parents and/or guardians must support and assist with the administration of medications, making sure that doses are not skipped. Considering that pediatric glaucoma can be a chronic, sight-threatening, and stressful condition, support from a team of child psychologists would be beneficial to help the patients and their parents cope with the disease and to improve compliance to treatment and regular follow-up.21
Conclusion
Childhood uveitic glaucoma is one of the most challenging entities in the field of glaucoma, not only because of the unpredictable nature of uveitis but also the difficulty of surgical management due to the risk of failure and complications. Over the last 70 years, a number of operations have been incorporated in the management of childhood glaucoma. Interestingly, most of them have stood the test of time, whereas others have still to prove their efficacy. The fact that there is a wide spectrum of approaches in regard with the management of uveitic glaucoma in children reflects the diversity of its causes and the complexity of its pathogenesis. The challenge of controlling both the inflammatory process and the glaucoma progression together with the absence of controlled trials to facilitate decision-making adds to the perplexity of the situation. The prognosis for childhood uveitic glaucoma has improved substantially over the last decades. However, increasing surgical success rates and reducing complications remains a Gordian knot in modern ophthalmology for specialists who want to ensure a favorable and long-lasting visual outcome for their young patients.
