Adams–Oliver Syndrome With an Encephalocele

Sahana Devadas, Rashmi Patil, Sarala Sabapathy, Shivaprakash Sosale, Shilpa Krishnapura Lakshminarayana

Abstract

Aplasia cutis congenita (ACC) is a heterogenous group of disorders characterized by the absence of a portion of the skin in a localized or widespread area at birth, most commonly on the scalp. The incidence of ACC is 1 in 1,00,000 births. Freiden classification has categorized solitary midline scalp defects with limb reduction anomalies as group 2, which is called Adams–Oliver syndrome (AOS).

Here we present case of AOS associated with an encephalocele. The neonate developed an encephalocele at the end of the second week of life, which makes this a distinctive case. Early intervention by a plastic surgeon and case-specific (individualized) treatment based on the size, location, and nature of the defect is necessary.