Adams oliver syndrome

Abstract

Adam’ s Oliver syndrome (AOS)[lehmen A] is an atypical hereditary congenital disorder, but most cases reported so far are sporadic. Patients primarily show aplasia cutis congenita (ACC) of the scalp where lesions are in midline or occipital regions and sometimes also on the abdomen or limbs, and terminal transverse limb defects (TTC)[Garzon MC]. The patients have underdeveloped skin and hair growth is absent in the affected regions. Sometimes, patients also have cutis marmorata telangiectatica congenita. ACC is seen in almost 80% of the patients and TCC in 85%. Terminal transverse defects many of the time refer to abnormal nails, fingers or/ and toes. The fingers and toes can be fused (syndactyly), short (brachydactyly) or be missing altogether (oligodactyly). In some patients, bones of arms and lower limbs are malformed or missing. Patients can develop pulmonary hypertension and other blood-heart defects. They can also suffer abnormalities in the brain and less frequently renal, liver, and eye anomalies. Although rare, severe morbidity and mortality results from haemorrhage or infection involving large and deep calvarial lesions, or from cardiovascular anomalies including severe heart malformations.