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Year : 2019  |  Volume : 57  |  Issue : 2  |  Page : 163-166

Encephalocraniocutaneous lipomatosis: A rare neurocutaneous disorder

Department of Pediatric Ophthalmology, Joseph Eye Hospital, Institute of Ophthalmology, Trichy, Tamil Nadu, India

Correspondence Address:
Dr. Antony Arokiadass Baskaran
Department of Pediatric Ophthalmology, Joseph Eye Hospital, Trichy, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_38_19

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A 5-year-old boy presented with the left eye epibulbar choristomas, upper lid coloboma, small periocular papules, and lipomatous swelling in the frontoparietal area with patchy alopecia on the left parietal side of the scalp. Neuroimaging showed left-sided cerebral hemiatrophy and ex vacuo dilation of the ventricle. The case was diagnosed as a case of encephalocraniocutaneolipomatosis based on the Moog's criteria. On follow-up after 4 years, the clinical features remained the same with no other systemic issues.

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