Síndrome de Adams-Oliver – descrição clínica e acompanhamento da evolução de um caso

Resumo (EN)

Introduction – Adams-Oliver syndrome or Aplasia Cutis Congenita (ACC) is a rare condition characterized by a congenital absence of a localized or generalized area of skin at birth. It most often occurs as small lesions in the scalp, which at birth could have already been healed, or it may remain a shallow ulcer until it becomes a deep ulceration, occasionally impairing the meninges. Due to Adams-Oliver syndrome singularity, we report a case of a child who was born with a gestational age of 36 weeks with a congenital malformation, such as partial absence of the skullcap, absence of skin and subcutaneous tissue, and the upper and lower limbs can also present malformation. After the syndrome diagnosis, the patient had undergone both plastic surgery to reconstruct the encephalocele and further treatment of the skin lesion in parietal-occipital-temporal regions. Because of the scarcity of skin, the lesion was not totally covered, and we have chosen to wait for the clinical outcome to perform further approaches. At the age of three months, the child died due to respiratory failure. She had been intubated with mechanical ventilation for 7 days.