[Spontaneous colonic perforations revealing Ehlers-Danlos syndrome type IV].
BACKGROUND
The malignant form of Ehlers-Danlos syndrome type IV owes its bad reputation to a proneness to spontaneous rupture of bowel or large vessels, which may reveal the disease.


METHODS
A girl suffered acute rupture of the sigmoid at the age of 5 years and rupture of the left colon, twice, at the age of 11 and 13 years, respectively. These ruptures required colostomy and finally colectomy. A proneness to bruisability, history of dislocation of hips, hypermobile joints, ovarian cysts and some minor abnormalities of her face resembled that of the Ehlers-Danlos syndrome which was confirmed by optic and electronic microscopy of the skin biopsy.


CONCLUSIONS
This is the youngest case of rupture of bowel reported in Ehlers-Danlos syndrome. Long-term prognosis is influenced by repetition of intestinal ruptures and occurrence of vascular complications.
Service de chirurgie pédiatrique, centre de la Mère et de l'Enfant, Le Parc, Colmar, France.
C
C
Henry
S
S
Geiss
E
E
Wodey
A
A
Pennerath
M T
MT
Zabot
S
S
Peyrol
H
H
Plauchu
D002675:Child, Preschool; D003108:Colonic Diseases / Q000209:etiology*; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D005260:Female; D006801:Humans; D007416:Intestinal Perforation / Q000209:etiology*
Perforations coliques spontanées révélatrices d'un syndrome d'Ehlers-Danlos de type IV.