Ehlers-Danlos syndrome (EDS) Type IV results in inadequate production of type III collagen and can lead to devastating vascular, gastrointestinal, and uterine complications. A case is presented of spontaneous colonic perforation in a patient with EDS Type IV complicated by multiple intestinal fistulas managed with porcine small intestinal submucosa. Early success with this technique would suggest additional applications in a variety of complicated gastrointestinal problems.
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References
1.
BerneyT, La ScalaG, VettorelD, . Surgical pitfalls in a patient with type IV Ehlers-Danlos syndrome and spontaneous colonic rupture: Report of a case. Dis Col Rectum1994;37: 1038–42.
2.
SykesEM. Colon perforation in Ehlers-Danlos syndrome: Report of two cases and review of the literature. Am J Surg1984; 147:410–3.
3.
PepinM, SchwarzeU, Superti-FurgaA, ByersPH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med2000;342:673–80.
4.
BadylakS, MeurlingS, ChenM, . Resorbable bioscaffold for esophageal repair in a dog model. J Pediatr Surg2000; 35:1097–103.
5.
KajitaniM, WadiaY, HindsMT, . Successful repair of esophageal injury using an elastin based biomaterial patch. ASAIO J2001;47:342–5.
6.
KajitaniM, WadiaY, XieH, . Use of a new elastin patch and glue for repair of a major duodenal injury. ASAIO J2000;46:409–14.
7.
SchultzDJ, BraselKJ, SpinelliKS, . Porcine small intestinal submucosa as a treatment for enterocutaneous fistulas. J Am Coll Surg2002;194:541–3.
8.
KiniS, GagnerM, de CsepelJ, . A biodegradeable membrane from porcine intestinal submucosa to reinforce the gastrojejunostomy in laparoscopic Roux-en-Y gastric bypass: Preliminary report. Obes Surg2001;11:469–73.
9.
BeightonPH, MurdochJL, VottelerT. Gastrointestinal complications of the Ehlers-Danlos syndrome. Gut1969;10:1004–8.
10.
SoucyP, EidusL, KeeleyF. Perforations of the colon in a 15-year old girl with Ehlers-Danlos syndrome type IV. J Pediatr Surg1990;25:1180–2.
11.
SentongoTA, LichtensteinG, NathansonK, . Intestinal perforations in Ehlers-Danlos syndrome after enema treatment for constipation. J Pediatr Gastroenterol Nutr1998;27:599–602.