Outcomes of surgical management of total colonic aganglionosis.

IF 4.6 Q2 MATERIALS SCIENCE, BIOMATERIALS
E K Choe, S B Moon, H Y Kim, S C Lee, K W Park, S E Jung
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引用次数: 10

Abstract

Purpose: The purpose of this study was to assess the long-term clinical outcomes and bowel function of patients with total colonic aganglionosis (TCA) after surgery.

Methods: The hospital records of 17 TCA patients treated surgically during 1985 to 2004 were reviewed. Long-term follow-up was done by telephone interviews with the parents.

Results: Primary enterostomy was performed in 13 (76%) patients. In three (17%) patients, TCA was not suspected initially. They underwent conservative surgery primarily, which required a second operation soon after. One had transverse colectomy with ileostomy. By pathologic review, nine (53%) patients had small bowel involvement of aganglionosis. Six (35%) patients died before corrective surgery. They all had extensive small bowel involvement. Among 11 patients who had a corrective operation, 10 were treated with Martin's procedure. Long-term (mean 74 months) follow-up was available in seven patients, and the mean weight-for-age percentiles was 27.1% (range 5-50%), the frequency of defecation was three to five times a day in four patients (57%), one or two times a day in two patients (28%), and more than five times a day in one patient (15%).

Conclusions: TCA is difficult to diagnose; but once it is diagnosed correctly and treated by corrective surgery, outcomes seem promising. Martin's operation brought about a good outcome and enabled patients to have acceptable bowel habits. The prognosis is highly dependent on the extent of aganglionosis.

全结肠神经节病手术治疗的结果。
目的:本研究的目的是评估全结肠神经节病(TCA)患者术后的长期临床结果和肠功能。方法:回顾我院1985 ~ 2004年收治的17例TCA患者的临床资料。通过与父母的电话访谈进行长期随访。结果:13例(76%)患者行原发性肠造口术。在3例(17%)患者中,TCA最初未被怀疑。他们首先接受了保守手术,随后需要进行第二次手术。1例行横结肠切除术并回肠造口术。病理检查显示,9例(53%)患者有神经节病累及小肠。6例(35%)患者在矫正手术前死亡。他们都有广泛的小肠病变。在接受矫正手术的11名患者中,有10人接受了马丁的手术。对7例患者进行了长期(平均74个月)随访,平均体重年龄百分位数为27.1%(范围5-50%),4例(57%)患者每天排便3 -5次,2例(28%)患者每天排便1 - 2次,1例(15%)患者每天排便5次以上。结论:TCA诊断困难;但一旦得到正确诊断并通过矫正手术治疗,结果似乎很有希望。马丁的手术带来了良好的结果,使病人有了可接受的排便习惯。预后高度依赖于神经节病的程度。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
ACS Applied Bio Materials
ACS Applied Bio Materials Chemistry-Chemistry (all)
CiteScore
9.40
自引率
2.10%
发文量
464
期刊介绍: ACS Applied Bio Materials is an interdisciplinary journal publishing original research covering all aspects of biomaterials and biointerfaces including and beyond the traditional biosensing, biomedical and therapeutic applications. The journal is devoted to reports of new and original experimental and theoretical research of an applied nature that integrates knowledge in the areas of materials, engineering, physics, bioscience, and chemistry into important bio applications. The journal is specifically interested in work that addresses the relationship between structure and function and assesses the stability and degradation of materials under relevant environmental and biological conditions.
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