Challenges in Surgical Intervention for a Rare Case of Anterior Segment Dysgenesis: A Case Report

Abstract

Anterior Segment Dysgenesis (ASD) represents a spectrum of rare, congenital disorders that pose significant challenges to ophthalmological management due to their complex and heterogeneous nature. The management of ASD becomes particularly complex when associated with other serious ocular conditions. This report discusses the case of a 4-year-old girl diagnosed with ASD exhibiting a combination of sclerocornea, aphakia, aniridia, and secondary glaucoma. Owing to the complexity of such condition, a multi-disciplinary approach is required. Despite successful initial surgical interventions on the left eye, eye was lost due to subsequent endophthalmitis and retinal detachment, resulting in a decision to adopt a conservative, non-surgical approach for the right eye. Although a series of therapeutic interventions have been performed, the final visual outcome was poor, demonstrating the complexity and seriousness of such cases. This case serves as a reminder of the need for regular follow-up, prompt recognition, and management of potential complications. Further research is necessary to optimize the outcomes in patients with similar presentations.

Keywords:

• congenital aniridia
• aphakia
• secondary glaucoma
• sclerocornea
• case report

Ethical Considerations

The case procedures and publication were approved by the local ethical committee of the Dhahran Eye Specialist Hospital (IRB: DESH1101). A written informed consent was obtained from the patient’s parents, and they approved the publication of the patient’s data and any accompanying images.

Disclosure

The authors report no conflicts of interest in this work.

Additional information

Funding

There is no funding to report.