Dr.SAMRUDDHI DANI, S21583
Parents of 1 year male noticed redness & whitish discoloration in OU since 10 days.He had lack of sensations over body,didn’t cry on giving injections,self mutilation,lip ulceration,anhydrosis & Hb 5.
OU circumcorneal congestion,exposure keratitis staining with fluorescein & diffuse stromal haze.OU corneal reflex & sensations absent,reduced blinking (4/min),partial blink & no nocturnal lagophthalmos.Bell’s phenomenon couldn’t be elicited.EMG/NCV studies- peripheral sensory & autonomic neuropathy.Neuro examination-hypotonia in all limbs & absent DTRs,suggesting HSAN type II.Genetics awaited.
He was started on lubricating eye drops,epithelial defect healed with nebular scar.He was lost to f/up & came 1 month later with OU large epithelial defect & diffuse stromal haze.OU lateral tarsorrhaphy was done.
HSAN is a rare genetic disorder,few reports on corneal manifestations.This case highlights importance of early tarsorrhaphy in HSAN neurotrophic keratopathy to avoid complications.
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