title: [Recurrent perforation of of the sigmoid colon associated with Ehlers-Danlos syndrome type IV].
abstract: Ehlers-Danlos syndrome type IV is a heritable disease of type III collagen metabolism. Most patients have a defect either in the synthesis or structure of type III procollagen, a finding consistent with the fact that these patients are prone to spontaneous rupture of the aorta and intestines, tissues rich in type III collagen. The diagnosis is suspected in a patient with a combination of clinical manifestations and family history, but it is confirmed only by culture of the patient's skin fibroblasts and the demonstration of a defect in type III collagen metabolism. We present a patient with Ehlers-Danlos type IV syndrome who developed a recurrent colon perforation, and discuss the surgical strategy to prevent recurrences.
authors.affiliation: Servicio de Cirugía General y Aparato Digestivo, Hospital de la Princesa, Madrid.
authors.forename: B
authors.initials: B
authors.lastname: Doblado Cardellach
authors.forename: J M
authors.initials: JM
authors.lastname: Figueroa Andolla
authors.forename: E
authors.initials: E
authors.lastname: Martín Pérez
authors.forename: S
authors.initials: S
authors.lastname: Delgado de Torres
authors.forename: A
authors.initials: A
authors.lastname: Pérez García
authors.forename: O
authors.initials: O
authors.lastname: Blasco Delgado
authors.forename: J
authors.initials: J
authors.lastname: López-Yarto
authors.forename: P
authors.initials: P
authors.lastname: Serrano Sánchez
mesh_terms: D000328:Adult; D012809:Colon, Sigmoid / Q000473:pathology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D007416:Intestinal Perforation / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D012008:Recurrence; D012810:Sigmoid Diseases / Q000209:etiology* / Q000473:pathology / Q000601:surgery