Muerte materna por shock hemorrágico en gestante con síndrome de Ehlers-Danlos vascular tipo IV

Ehlers-Danlos syndrome is a very rare inherited connective tissue disorder associated with a mutation in the collagen gene. The diagnosis is mainly clinical, with subsequent confirmation by genetic study. It is divided into several types according to their clinical presentation, with symptoms that can range from skin manifestations, to vascular, digestive, neurological or joint disorders. Treatment is only symptomatic, requiring prophylactic management if surgery is needed; and genetic counselling for the study of first-degree relatives. It is considered a high-obstetric-risk disease, so close monitoring of the patient during pregnancy is required, with special attention to the mode of delivery, which must be agreed by a multidisciplinary team given the morbidity and mortality of this disease.