The short bowel syndrome (SBS) can result from a variety of conditions, including postoperative complications and malignancy. Continence preserving operations are generally performed for either ulcerative colitis (UC) or familial polyposis (FAP). These procedures can be associated with high morbidity and the potential for future malignancy. Our aim was to determine the causes and consequences of SBS in patients undergoing these procedures.
Methods: 22 patients (11 men and 11 women) 18 to 64 years of age were identified with SBS after continence preserving procedures. Follow up ranged from 6 to 192 months.
Results: 16 patients had pelvic procedures and 6 had continent ileostomies. Overall 14 patients had UC, seven had FAP, and one had functional disease. Eight patients with an initial diagnosis of UC had subsequent Crohns disease necessitating further resection and pouch excision. Eight patients (5 with UC, two FAP, and one with functional disease) had postoperative complications, including obstruction or mesenteric occlusion requiring resections. One UC patient developed adenocarcinoma in a continent ileostomy. Five of the seven patients undergoing pelvic procedures for FAP required resection for desmoid tumors. Two died at 11 and 13 months after SBS from liver failure and sepsis while awaiting transplant, one died from an unrelated cardiac operation at 23 months, another has recurrent desmoid at 30 months, and the other was alive and well at six months. All SBS patients had a proximal ostomy. Remnant length measured <60cm in 4 patients, 60-120 cm. in 8 patients, and >120cm in 10 patients. Overall 11 patients required long term PN. One patient with UC underwent intestinal transplant. Four patients died.
Conclusions: SBS can develop after continence preserving procedures. This occurs with inflammatory bowel disease when unsuspected Crohns disease is present or complications occur. SBS related to desmoid tumors has a poor prognosis in patients undergoing resection alone. A more aggressive approach to intestinal transplantation in these patients may be warranted.