Neurotrophic keratitis and congenital insensitivity to pain with anhidrosis--a case report with 10-year follow-up.

PURPOSE: To report a rare case of congenital insensitivity to pain with anhidrosis.

METHODS

A 3-year-old girl presented with watering in the right eye for 3 days. Slit-lamp examination showed an epithelial defect and hypopyon in the right eye and a corneal scar with thinning and vascularization in the left eye. There was bilateral reduced corneal sensation and evidence of self-mutilated lips and fingers.

RESULTS

Neurological manifestations along with ocular features confirmed the diagnosis of congenital insensitivity to pain with anhidrosis.

CONCLUSIONS

Patients with congenital insensitivity to pain with anhidrosis are asymptomatic even when they develop corneal ulcer. Parents should be advised regular follow-up and prompt treatment because this is a vision-threatening corneal abnormality.