关键词: X-ray abdomen antenatal sonography coloplasty colostomy congenital malformation congenital pouch colon

来  源:   DOI:10.1093/bjrcr/uaad005   PDF(Pubmed)

Abstract:
Congenital pouch colon (CPC) is highly uncommon congenital anorectal malformation where a distended pouch-like structure replaces either some part of the colon or the entire colon and communicates to the genitourinary tract through a fistula. Diagnosis of CPC is usually made after birth when neonate/infant presents with abdominal distension and absence of anal opening. Making antenatal diagnosis of CPC is difficult because of the lack of specific and verifiable signs on sonography. Hence, only a few cases of antenatal diagnosis of CPC have been reported.1,2 In our case, CPC was suspected on a routine antenatal growth scan ultrasound in the late third trimester, showing a hypoechoic tubular-shaped lesion in the pre-sacral region. With this suspicion, we suggested an institutional delivery at a tertiary level centre, and diagnosis of type III CPC was confirmed on post-delivery imaging and emergency primary surgery, done on the day 3 of life (pouch resection, division of fistula, and protective colostomy). The child also underwent further corrective surgeries in a staged manner in second year of life and recovered completely. Beforehand diagnosis prevented any unnecessary delay in operative care, reduced postoperative complications, and improved the overall outcome of this otherwise complex condition.
摘要:
先天性袋状结肠(CPC)是非常罕见的先天性肛门直肠畸形,其中扩张的袋状结构取代了结肠的某些部分或整个结肠,并通过瘘管与泌尿生殖道连通。当新生儿/婴儿出现腹胀和没有肛门开口时,通常在出生后进行CPC的诊断。由于超声检查缺乏特定且可验证的体征,因此很难对CPC进行产前诊断。因此,仅报道了少数病例的产前诊断。1,2在我们的病例中,在妊娠晚期的常规产前生长扫描超声检查中怀疑CPC,显示骶骨前区低回声管状病变。带着这种怀疑,我们建议在三级中心进行机构交付,诊断为III型CPC在分娩后影像学和急诊初次手术中得到证实,在生命的第3天完成(囊袋切除术,瘘管分裂,和保护性结肠造口术)。孩子在生命的第二年还分阶段进行了进一步的矫正手术,并完全康复。事先诊断可以防止手术护理的任何不必要的延迟,减少术后并发症,并改善了这种复杂状况的整体结果。
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