Appendiceal Abnormalities Associated with Congenital Pouch Colon.

Abstract

Purpose: This retrospective cohort study aims to identify the incidence and type of appendiceal abnormalities in the various subtypes of congenital pouch colon (CPC) associated with anorectal malformation. The possible embryopathogenesis of appendiceal abnormalities in CPC is also discussed.

Methods: The case records of 74 patients of CPC, managed from 1998 to 2022, were studied, and the subtype of CPC, details regarding the vermiform appendix (presence/absence, number, morphology and location) and other relevant findings were noted.

Results: The CPC subtypes were Type I (n = 12), Type II (n = 41), Type III (n = 11) and Type IV (n = 10). Overall, 26 patients had a single, normal appendix, 12 patients a short, stubby appendix and 17 appendiceal duplication (Type A2 [n = 9], Type B1 [n = 3] and Type C [n = 5]). Type I CPC cases had appendicular agenesis. A short stubby appendix (26.8%) or Type A2/B1/C appendicular duplication (31.3%) was frequent in Type II CPC and Type C duplication in Type III CPC. Type IV CPC patients had a single, normal appendix. In 10 (24.4%) patients with Type II CPC, the caecum and normal proximal colon appeared dilated.

Conclusions: Appendiceal abnormalities are frequent in Type I-III CPC and may result from the abnormal development and dilatation, along with shortening, of a varying length of colon. As in other reports of appendiceal duplication associated with major intestinal, genitourinary or vertebral malformations, appendiceal duplication in patients with CPC was of the Type A2/B1/C subtypes.