Pampal Arzu, Ozbayoglu Asli, Kaya Cem, Pehlivan Yildiz, Poyraz Aylar, Ozen I Onur, Percin Ferda E, Demirogullari Billur
Department of Pediatric Surgery, Faculty of Medicine, Ufuk University, Ankara, Turkey.
Pediatr Int. 2013 Aug;55(4):e86-9. doi: 10.1111/ped.12086.
Rectal duplication (RD) cysts are rare congenital anomalies that can be diagnosed with the presence of another opening in the perineum. They seldom accompany anorectal malformations (ARM). Two cases of RD accompanying ARM at opposite ends of the phenotypic spectrum, are described. A 3-month-old baby and a 2-year-old girl with ARM were scheduled for posterior sagittal anorectoplasty. The infant had an orifice at the anal dimple and the other had an orifice at the vestibulum posterior to the rectovestibular fistula. The infant presented with no other anomalies whereas the older one presented with an unusual coexistence of caudal duplication and caudal regression syndromes. Perioperatively both orifices were found to be related to retrorectal cysts, and were excised. Clinicians should always be alert when dealing with complex malformations. Because these malformations have variable anatomical and clinical presentations, they can represent a diagnostic and therapeutic challenge.
直肠重复(RD)囊肿是罕见的先天性异常,可通过会阴处存在另一个开口来诊断。它们很少伴有肛门直肠畸形(ARM)。本文描述了两例处于表型谱两端伴有ARM的RD病例。一名3个月大的婴儿和一名2岁女孩患有ARM,计划进行后矢状位肛门直肠成形术。婴儿在肛门凹处有一个开口,另一名婴儿在直肠前庭瘘后方的前庭处有一个开口。婴儿没有其他异常,而较大的患儿同时存在不寻常的尾部重复和尾部退化综合征。术中发现两个开口均与直肠后囊肿有关,并将其切除。临床医生在处理复杂畸形时应始终保持警惕。由于这些畸形具有可变的解剖和临床表现,它们可能构成诊断和治疗挑战。