Bilateral consecutive central corneal perforations associated with hypogammaglobulinemia

Objective: To describe the presentation and the clinical course of a patient with consecutive central sterile corneal perforations associated with common variable immunodeficiency. Design: Case report. Methods: Multiple corneal cultures and scrapings were performed in an effort to identify an infectious cause and all were negative. Corneal biopsy did not demonstrate any evidence of micro-organisms, An extended investigation failed to uncover a collagen vascular cause or atopy, Results: Progressive sterile stromal thinning with intact epithelium in the left eye proceeded to perforation despite topical treatment, and cyanoacrylate gluing was performed, However, a secondary Haemophilus influenza endophthalmitis developed, and the eye was eventually lost. The fellow eye proceeded along the same clinical course with sterile stromal thinning. A lamellar patch graft was performed when the central ulceration progressed to a descemetocele, The eye remained quiet with 20/25 vision for 2 years, until the patient died from complications of a liver transplant. Conclusions: Devastating central sterile corneal thinning leading to perforation may occur in patients with hypogammaglobulinemia. Clinical features and course suggest an autoimmune cause. Ophthalmology 2000; 107:123-126 (C) 2000 by the American Academy of Ophthalmology.