Exploring Twisted Pouch Syndrome in Ileal Pouch-Anal Anastomosis: A Scoping Review on Diagnosis, Treatment, and Prevention

Background:
Twisted pouch syndrome (TPS) is a rare complication in patients undergoing ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC) and familial adenomatous polyposis (FAP). It is characterized by erratic bowel habits, changes in frequency and urgency, and obstructive symptoms due to the twisting of the pelvic pouch during construction. Due to its rarity, a delay in diagnosis can occur and lead to pouch complications or even pouch failure. We performed a scoping review of the literature on pouch twist and TPS, including diagnosis, treatment, and prevention.
Methods:
A systematic search of the published literature was performed on August 12, 2024, using the OVID Medline, OVID Embase, and OVID Cochrane Central Register of Controlled Trials databases. The inclusion criteria were as follows: pouch twist, or twisted pouch syndrome in the patients with IPAA. Papers discussing pouch volvulus, or the twisting of an appropriately constructed pouch at the organo-axial axis, were excluded. Two researchers identified and independently reviewed the literature. Pertinent information on the diagnosis, presentation, and treatment of the pouch twists was extracted.
Results:
Eighteen articles were identified that met the inclusion criteria. The articles included case series (10, 55.6%), case reports (6, 33.3%), 1 narrative review (5.6%), and 1 video case report (5.6%). These studies were conducted between 2011 and 2024. All studies were conducted in the United States. Presenting symptoms were consistent with TPS, including changes in bowel habits, urgency and partial obstructive symptoms. Most articles have focused on endoscopic and operative interventions for pouch twists. Pouch twist was diagnosed on endoscopy in a minority of cases, but was largely discovered intraoperatively. After initial pouchoscopy, pouch twist was treated with pouch revision or creation of neo-IPAA in most cases. One case discussed an inlet twist that was managed with resection of the twisted small bowel and new end-to-end anastomosis between distal small bowel and the afferent limb of the pouch. Two articles presented cases of pouch twist that only underwent endoscopic therapy without operative intervention, where needle-knife septectomy was used to release distal IPAA stenosis. These patients had no recurrence of TPS symptoms during follow-up. Three case series discussed TPS in radiology, and 2 reported new radiologic advances in 3D computed tomography segmentation and 3D printing of staple line morphology to better identify aberrant pouch anatomy. One study discussed successful management of postoperative ileus with rescue pyridostigmine in a patient with TPS who underwent pouch excision and neo-IPAA creation. Lastly, 1 case series showed that patients living with TPS had decreased quality of life and increased overall distress.
Conclusions:
TPS is a rare but important complication in patients with IPAA construction. Preoperative diagnosis can be challenging even with imaging or endoscopy, but should be suspected in any pouch patients presenting with TPS symptoms. A low threshold for operative intervention should be kept as most patients will need pouch revision or neo-IPAA.