Solan K, Davies P
A 31-year-old woman with Ehlers-Danlos type IV syndrome developed multiple intensive care related complications following laparotomy for perforated bowel. Complications are more likely to occur with the Ehlers-Danlos syndrome.
Ehlers–Danlos syndromes are inherited disorders ofcollagen metabolism. The common features are skin hyperextensibility, articular hypermobility and tissuefragility. Eleven separate forms have been de scribed of which Type IV, the vascular or arterialecchymotic type, is the most severe. This autosomal dominant disorder results in the production of reduced or abnormal Type III collagen due to a mutat ion at the COL3A1 gene. Affected patients are at risk of arterial, bowel and uterinerupture, resulting in a median lifespan of 48 years. We describe the complicated postoperative course of a 31-year-old female with Type IV Ehlers–Danlos syn-drome, following a gastrointestinal perforation. The surgical complications of Type IV Ehlers–Danlos syn-drome are well described in the literature, but the anaesthetic and intensive care implications are not.
full text source