Adams Oliver Syndrome: Our One-Year Experience

OpenAlex İngilizce

Adams-Oliver syndrome, although rare, is a congenital disease commonly characterized by aplasia cutis congenita and terminal limb defects. While small lesions often heal spontaneously, larger lesions may be associated with common fatal anomalies in the gastrointestinal, cardiopulmonary, genitourinary, and central nervous systems In this paper, we aimed to remind this syndrome, which affects many systems, and to emphasize the importance of the multidisciplinary approach in its treatment. The criteria for Adams-Oliver syndrome have been evaluated in three cases according to the textbooks and the current literature. In case one, aplasia cutis, nail hypoplasia in bilateral toes, polydactyly, and kutis marmaratus in the right upper extremity, in case two, aplasia cutis, kutis marmaratus, and intrauterine intestinal perforation, and in case three, aplasia cutis, kutis marmaratus, and cystic encephalomalasic changes on cranial MRI have been detected. Adams-Oliver syndrome is a rare syndrome with various clinical presentations. Hence, in cases presenting with dysmorphic findings and Adams-Oliver syndrome, all systems should be examined. After diagnosis, follow-up and treatment should be performed by the relevant branches with a multidisciplinary approach.