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先天性直肠会阴瘘肛门成形术后患者的临床特征及其功能结局:日本全国性调查结果。

Clinical features of patients who underwent anoplasty for cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

机构信息

Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, 8-35-1, Sakuragaoka, Kagoshima, 890-8520, Japan.

Research Team for the Establishment of Treatment Guidelines for Persistent Cloaca, Cloacal Exstrophy and Mayer-Rokitansky-Küster-Häuser Syndrome for the Proper Transitional Care of the Patients, The Research Project for Rare and Intractable Diseases of the Ministry of Health, Labour and Welfare (MHLW), Tokyo, Japan.

出版信息

Pediatr Surg Int. 2023 Feb 10;39(1):112. doi: 10.1007/s00383-023-05403-3.

DOI:10.1007/s00383-023-05403-3
PMID:36764963
Abstract

PURPOSE

This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan.

METHODS

A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated.

RESULTS

The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling.

CONCLUSION

In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered.

摘要

目的

本研究旨在通过日本全国性调查,阐明接受肛门成形术的会阴直肠外翻(CE)患者的临床特征及其功能结局。

方法

进行了问卷调查,并从 229 名 CE 患者中获得了数据。排除未存活患者和未知数据后,共纳入 195 名患者。我们比较了未行肛门成形术组(NAP 组)和行肛门成形术组(AP 组)的患者特征。评估了行肛门成形术患者的排便功能。

结果

AP 组的脐膨出(p=0.045)和脊柱缺陷(p=0.003)发生率明显低于 NAP 组。在本研究纳入的 195 名患者中,仅 17 名(8.7%)接受了肛门成形术。在接受肛门成形术的 17 名患者中,7 名(41.1%)最终在肛门成形术后永久性造口[回肠造口术:n=3(60.0%),结肠造口术:n=4(44.4%)]。关于污染,4 名患者(66.7%)无污染,2 名患者(33.3%)偶然污染,无患者频繁污染。

结论

在我们的研究中,脊柱缺陷可能影响肛门成形术的决策。肛门成形术可能改善生活质量,因为可以保持排便清洁,但包括结肠功能在内的适应证可能需要仔细考虑。

相似文献

1
Clinical features of patients who underwent anoplasty for cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.先天性直肠会阴瘘肛门成形术后患者的临床特征及其功能结局:日本全国性调查结果。
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本文引用的文献

1
Urinary and Fecal Continence in Adolescent and Adult Patients With Cloacal Exstrophy.先天性泄殖腔外翻患者青少年及成年期的尿便控情况。
Urology. 2022 Jun;164:293-299. doi: 10.1016/j.urology.2022.01.009. Epub 2022 Jan 14.
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Risk factor analysis of irreversible renal dysfunction based on fetal ultrasonographic findings in patients with persistent cloaca: Results from a nationwide survey in Japan.基于永存性肛门闭锁患者胎儿超声检查结果的不可逆性肾功能障碍风险因素分析:来自日本全国性调查的结果。
J Pediatr Surg. 2022 Feb;57(2):229-234. doi: 10.1016/j.jpedsurg.2021.10.038. Epub 2021 Oct 30.
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The current profile of persistent cloaca and cloacal exstrophy in Japan: the results of a nationwide survey in 2014 and a review of the literature.
日本持续性泄殖腔和泄殖腔外翻的现状:2014年全国性调查结果及文献综述
Pediatr Surg Int. 2017 Apr;33(4):505-512. doi: 10.1007/s00383-016-4053-4. Epub 2017 Jan 5.
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Single-stage surgical correction of anorectal malformation associated with rectourinary fistula in male neonates.男性新生儿肛门直肠畸形合并直肠尿道瘘的一期手术矫正
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The "rescue operation" for patients with cloacal exstrophy and its variants.泄殖腔外翻及其变异患者的“抢救手术”
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Prenatal diagnosis of cloacal exstrophy: a case report and review of the literature.先天性直肠阴道瘘的产前诊断:病例报告及文献复习。
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Bone Marrow Transplant. 2013 Mar;48(3):452-8. doi: 10.1038/bmt.2012.244. Epub 2012 Dec 3.
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Restoring hindgut continuity in cloacal exstrophy: a valuable method of optimising bowel length.恢复泄殖腔外翻患者的后肠连续性:优化肠管长度的一种有效方法。
Eur J Pediatr Surg. 2009 Jun;19(3):141-4. doi: 10.1055/s-0029-1192048. Epub 2009 Jun 4.
10
Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients.泄殖腔外翻——拖出术还是永久性造口?53例患者的回顾分析
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