FAP is an autosomal dominant inherited neoplasm that causes multiple adenomatous polyps in the colon and is caused by a pathological variant of the adenomatous polyposis coli gene in the germline. The correlation between genotype and phenotype in FAP is useful for making decisions regarding its screening and surgical management . With the advent of IPAA  and the development of the J-shaped ileal pouch , postoperative outcomes improved; however, a few patients experienced complications. Pouch-related complications (PRC) are one of the most important factors affecting the long-term outcomes, and some cases of PRC lead to permanent ileostomy [9, 10]. Among them, ileal pouch perforation is a rare, long-term complication of restorative proctocolectomy.
Several reports of ileal pouch perforation have been reported in the literature, all of which are listed below [11,12,13,14,15,16,17,18,19,20] (Table 1). Hsu and Leonid et al. reported perforation of the ileal pouch due to external factors, such as blunt trauma [11, 12]. In pregnant women with an enlarged uterus, increased pouch pressure associated with perforation in relation to adhesions were reported [13, 14]. Other perforations are caused by Salmonella typhimurium infection , volvulus and subsequent obstruction of the terminal ileum , and idiopathic spontaneous perforation of the pouch . Shapiro et al. reported two cases associated with rapid ingestion of a high-fiber, high-calorie meal. In one patient, perforation occurred twice; however, the second perforation occurred 6 weeks postoperatively, which might have been an after-effect of the first pouch rupture . Takahashi et al. reported that the combination of an enlarged J-pouch blind end and pouchitis could result in perforation . Dogan et al. also deduced that perforation could be caused by pouchitis in the ileal J-pouch . In our observed two perforations, stricture at the anastomosis was confirmed at both instances. Upon initial perforation, there was no ulcer on the mucosal surface in the resected specimen. Since endoscopy was not performed early following surgery, the existence of pouchitis could not be verified, despite dilatation of the pouch. Immediately before perforation, the patient also had symptoms of constipation. Therefore, the perforation was believed to be caused by increased intraluminal pressure in the pouch arising from an anastomotic stricture; this increased pressure was also suspected in the second perforation. Multiple ulcers in the pouch were confirmed by postoperative endoscopy; hence, pouchitis was also suspected to have contributed to the ileal pouch rupture. In addition, the results of the ascites culture test revealed only E. coli, which was believed to be derived from intestinal perforation and was not the cause of perforation.
Holubar et al. stated that obstruction from pouch–anal anastomosis stricture is common and requires surgical dilation with Hegar dilators and endoscopic balloon dilation. Needle–knife stricturotomy, chronic self-dilation at home, and a hand-sewn reanastomosis are required in refractory cases . In our case, the anastomosis, which was dilated during the initial pouch perforation, was transiently maintained by self-bougie and constant observation. However, at some point, the self-bougie was not performed, and the patient’s follow-up was irregular. Accordingly, stenosis recurred, and pouchitis could not be primarily detected, which might have caused a second perforation. During that time, if the symptoms associated with the stricture, such as intestinal obstruction, were observed, early detection could have been possible. By doing so, his double perforation might have been prevented. Therefore, it is important to regularly observe such patients.
There are several reports on IPAA methods in patients with FAP. Konishi et al. reported that PRC in FAP patients were lower in stapled IPAA than in hand-sewn IPAA. In contrast, there were no differences in overall complication rates, fecal incontinence scores, ostomy rates, and overall survival between the two techniques. Therefore, they concluded that stapled IPAA might be a safer option for FAP patients to reduce PRC . Ganschow et al. suggested that rectal mucosa, especially the wide mucosal seams and rectal adenomas, are often observed after a stapled than a hand-sewn anastomosis, which might be related to long-term outcomes . Therefore, the preference between stapled IPAA or hand-sewn IPAA remains controversial. As Smith et al. stated , regardless of the anastomotic technique, careful regular surveillance and functional maintenance of pouches are critical.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.