Indolent corneal ulcers in a patient with congenital insensitivity to pain with anhidrosis: A case report and literature review

PURPOSE. To report a case of bilateral corneal neurotrophic ulcer in patient with congenital insensitivity to pain with anhidrosis (CIPA) and review the literature. CASE REPORT. A 6 year-old boy presented with bilateral central corneal sterile ulcer, decreased corneal sensitivity, moderately altered corneal reflex and normal tearing response. History taken, systemic evaluation and medical chart review were undertaken. DISCUSSION. Fifty-two cases of CIPA have been reported worldwide. Fourteen cases had corneal involvement. The clinical picture of our patient is characteristic of CIPA. CONCLUSIONS. Congenital insensitivity to pain with anhidrosis may present as neurotrophic corneal ulcer. We report herewith, this vision threatening corneal congenital abnormality. Early diagnosis and prompt treatment are mandatory to prevent corneal complications such as scarring and perforation.