Colon perforation in Ehlers-Danlos syndrome

Occult small bowel perforation in a patient with Ehlers Danlos syndrome: a case report and review of the literature

“…The recommendation is either an end colostomy or Hartmann's procedure. The reported experience although small, indicates that a high incidence of recurrent perforation can be expected if bowel continuity is re-established [7]. A decision was made not to leave a stoma as the ends for anastomosis were free of diverticulae and suitable for anastomosis.…”

Anaesthetic and intensive care management of a patient with Ehlers–Danlos Type IV syndrome after laparotomy

“…Previous reports on Type IV Ehlers–Danlos syndrome have concentrated largely on the surgical aspects of the disease, namely, arterial or visceral rupture in relatively young patients [3–7]. Surgery is reported as ‘laborious and frustrating’, complicated by extreme tissue fragility, poor wound healing and dehiscence [3, 4].…”

“…Following long, difficult surgical procedures, a prolonged, complicated postoperative recovery may be expected. Previous reports mention patients suffering sepsis, multiple organ failure and death, but the emphasis is surgical with minimal discussion of postoperative management [3–5, 8].…”

Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

“…A significantly higher risk of re-perforation was seen in those patients left with their colon in situ (log rank P = 0.031, v 2 = 4.63, 95% median CI 67-100). Segmental resection Emergency Gastroaortic fistula Sykes [30] Total colectomy and ileorectal anastomosis Elective Multiple organ failure Blaker et al [17] Closure colostomy Elective Multiple organ failure Sentongo et al [14] Closure colostomy Elective Anastomotic leak Collins et al [39] Closure colostomy Elective Anastomotic leak Soonawala et al [26] Hartmann's procedure Emergency Retroperitoneal haemorrhage McAleese [21] Hartmann's procedure Emergency Myocardial infarction Demirogullari et al [18] Total colectomy and ileorectal anastomosis Emergency Multiple organ failure Schippers and Ditters [24] Closure colostomy Elective Upper gastrointestinal haemorrhage Silva et al [48] Hartmann's procedure Emergency Spontaneous rupture of left renal and iliac arteries Fogel [31] Hartmann's procedure Emergency Multiple organ failure Type IV Ehlers-Danlos, Colonic complications in this group of patients, a decision to undertake ileorectal anastomosis should be made only after detailed discussion with the patient with respect to the increased risk of potentially serious complications. The present study also shows that, unlike in cancer or diverticular disease, restoration of colonic continuity in EDS is associated with a risk of further colonic perforation which is likely to be regarded as unacceptably high by both surgeons and patients.…”

“…A number of strategies to manage patients with suspected or confirmed EDS and colonic perforation have been described in the literature. These include primary repair [9][10][11], primary repair and defunctioning stoma [5,6,9,[11][12][13][14][15], Hartmann's procedure [9,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] and total abdominal colectomy (TAC) with either an endileostomy or ileorectal anastomosis (IRA) [8,[32][33][34][35][36]. While total colectomy and IRA may prevent further colonic perforation, the leak rate of IRA in such cases has been reported to be as high as 50% [35].…”