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.


METHODS
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.
Akpek
E K
EK
Wilmer Eye Institute, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Haddad
R S
RS
Winkelstein
J A
JA
Gottsch
J D
JD
D000361:Agammaglobulinemia / Q000150:complications*; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications*; D003316:Corneal Diseases / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D003319:Corneal Stroma / Q000382:microbiology / Q000473:pathology*; D016039:Corneal Transplantation; D009877:Endophthalmitis / Q000382:microbiology; D015818:Eye Infections, Bacterial / Q000209:etiology; D005260:Female; D006192:Haemophilus Infections / Q000209:etiology; D006193:Haemophilus influenzae / Q000302:isolation & purification; D006801:Humans; D012422:Rupture, Spontaneous / Q000209:etiology / Q000473:pathology