Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous syndrome that was
characterized by unilateral, smooth, hairless fatty tissue nevi of the scalp, termed nevus psiloliparus, facial
lesions, multiple anomalies involving the eye, and ipsilateral porencephalic cysts with cortical atrophy,
cranial asymmetry, marked developmental delays, and mental retardation. A 12-month-old boy was referred
to our clinic for evaluation of non-scarring alopecia on the left side with an underlying fatty mass in the
left parietal scalp and left-sided multiple periocular masses. It showed a large lipomatous mass on the
scalp with overlying alopecia. Multiple skin tags and defects in the left periocular area were also observed.
Additional ocular anomalies included epibulbar lipodermoid, iris coloboma, and localized peripapillary
hypopigmentation lesions. After complete excision, the wound was covered with a local flap. The histologic
examination revealed a mass surrounded by a well-developed capsule within the dermal layer, adipose
tissue and connective tissue septa extending into the reticular dermis. No recurrence was observed at
follow-up. It is essential to differentiate suspected ECCL from other syndromes which present with epibulbar
chorisotomas. Neuroimaging, and pathological studies may be helpful for correct diagnosis. We will correct
multiple periocular lesions in preschool age and follow up developmental problems like developmental delay
and mental retardation constantly.
(Archives of Aesthetic Plastic Surgery 18: 147, 2012)