Syndrome de Scott et grossesse: à propos d’un cas

Scott´s syndrome is a rare congenital thrombopathy with an autosomal recessive inheritance pattern. The purpose of our study is to report the case of a parturient woman with Scott´s syndrome secondary to a mutation of the ANO6 gene never described in the literature. After 3 bleeding episodes, in particular after spontaneous abortion, the diagnosis was confirmed by flow cytometry. This patient was monitored during pregnancy in our centre and then gave birth via vaginal delivery at full term. Special preventive measures were taken. Outcome was good for the mother and the newborn. The treatment of Scott´s syndrome is purely symptomatic and mainly involves platelet transfusions. Since it is a rare thrombopathy with a high bleeding risk and difficult diagnosis, pregnancy and childbirth in patients with this syndrome require rigorous monitoring, as the prognosis is life threatening.