Title : Managing a rare case of familial Radius Maumenee syndrome
Abstract:
Introduction: Idiopathic elevated episcleral venous pressure (IEEVP), also known as Radius-Maumenee syndrome, is a rare condition characterized by dilated episcleral veins and open angle glaucoma. It can pose a diagnostic and management challenge for clinicians, often necessitating filtration surgery due to medical treatment proving ineffective. We describe to our knowledge the first case of familial Radius-Maumenee syndrome in a father and son duo and outline their management using glaucoma drainage devices.
Case Report: A 77-year-old male was referred with a history of bilateral ocular hypertension secondary to raised episcleral venous pressure. Past ocular history was significant for right eye trabeculectomy complicated by an intra-operative bleed. Examination demonstrated prominent episcleral blood vessels bilaterally. Previous MRI/MRA of orbits and brain and chest imaging revealed no pathology. Despite maximal medical therapy and selective laser trabeculoplasty, his visual field continued to progress in the left side and he underwent a primary Baerveldt Tube (BVT) with Mitomycin C (MMC) and tutoplast. The post-operative period was complicated with uveal effusion, severe fibrinous uveitis and macular oedema. Following management of the post-operative complications, IOP in left side was controlled on dual treatment and OCT demonstrated a stable neuroretinal.
The son, a 53-year-old male presented with a 20-year history of glaucoma secondary to idiopathic raised episcleral venous pressure. Examination demonstrated dilated episcleral veins in the right eye and blood in the Schlemm’s canal. Right optic nerve examination revealed inferior neuroretinal rim loss. Previous MRA head excluded carotid venous fistula. Due to his suboptimal IOP and visual field progression, the patient underwent an uneventful PAUL tube insertion with MMC and tutoplast. His post operative course was uncomplicated and IOP remained settled with a single topical IOP-lowering medication.
Conclusion: This case report highlights the use of a glaucoma drainage device as a primary management option in these challenging cases which is not only more effective (compared to bleb forming MIGS surgery) but a safer option (compared to trabeculectomy). The post operative course can be unpredictable and needs close monitoring to prevent/ manage complications that may be self-limiting.
