[Abdominal emergencies in type IV ehlers-Danlos syndrome].
Ehlers-Danlos syndrome denotes a group of inherited connective tissue diseases comprising nine types. Type IV Ehlers-Danlos syndrome is the most life-threatening form. It is characterized by a type III collagen deficiency resulting in arterial fragility and death from vascular rupture or bowel perforation. This disease involves a col 3A1 gene mutation. We report the case of a 44 year-old woman with type IV Ehlers-Danlos syndrome. The medical history of our patient included bowel necrosis and two vascular ruptures. We indicate data required to establish Ehlers-Danlos syndrome diagnosis and guidelines for patient management.
Service de Chirurgie Générale et Digestive, Hôpital Trousseau, CHU, Tours.
Z A
ZA
Benchellal
N
N
Huten
E
E
Danquechin Dorval
L
L
Podeur
A
A
Rahili
S
S
Lemeret
A
A
De Muret
D000328:Adult; D003094:Collagen / Q000172:deficiency; D004064:Digestive System / Q000473:pathology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000150:complications; D006801:Humans; D007813:Laparotomy
D003094:Collagen
Urgences abdominales du syndrome d'Ehlers-Danlos de type IV.