Adams-Oliver Syndrome (OSA)

Abstract

Introduction: Adams-Oliver's syndrome (SAO) is a rare genetic condition characterized by the association of lumb anomalies and Aplasia cutis congenita in vertex, often accompanied by underlying ossification defect and vascular lesions. The objective through this new observation is to describe the clinical, radiological, therapeutic and evolutionary elements according to what was described up to there in the literature. Observation: Female newborn child, stemming from a pregnancy followed with normal obstetric ultrasounds. He presents a scalp agenesis, underlying cranial bone aplasia and toes hypoplasia with syndactyly. The association of these two major criteria allowed making the diagnosis of SAO. Moreover, she presents disjointed and large sagittal suture with left parietal bone borders hypoplasia. The notion of periventricular bleeding. The rest of the balance sheet deformation, namely the abdominal ultrasound and echocardiography was without anomalies. The newborn was sent in neurosurgery for restorative treatment, which will be planned at the age of 3 months. Discussion and Conclusion: This observation illustrates sporadic and not complicated SAO. The absence of complete gene mapping in SAO and therefore of any genetic counseling, prenatal morphological ultrasound, is making important in evolutionary terms of subsequent pregnancies.