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儿童结直肠血管畸形的临床特征与处理:23 例回顾性研究

Clinical Characteristics and Management of Colorectal Vascular Malformation in Children: A Retrospective Study of 23 Cases.

机构信息

Department of Surgery, Children's Hospital of Fudan University, Shanghai, China.

Department of Anesthesiology, Children's Hospital of Fudan University, Shanghai, China.

出版信息

Eur J Pediatr Surg. 2023 Aug;33(4):279-286. doi: 10.1055/s-0042-1751222. Epub 2022 Aug 13.

Abstract

INTRODUCTION

The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children.

METHODS

We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019.

RESULTS

A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Hematochezia and anemia were cardinal symptoms. Fourteen patients (60.9%) were misdiagnosed as anal fissures ( = 4), internal hemorrhoids ( = 3), rectal polyps ( = 2), inflammatory bowel disease ( = 2), portal hypertension ( = 2), and Meckel's diverticulum ( = 1), respectively. The average time from symptom onset to diagnosis was 4.5 ± 4.4 years. Other vascular malformations were detected in eight patients (34.8%). All patients showed a positive anomalous vascular image on contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). The sensitivity of colonoscopy in the diagnosis of CRVM was 82.6% (19/23). A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the colorectum and showed transmural diffuse distribution, with an average length of 20 ± 5.4 cm. Two patients (9.5%) experienced surgical complications. Bloody stools reappeared in two patients (9.5%), and colonoscopy showed abnormal angiogenesis at the anastomotic site, which were cured by sclerotherapy and/or electrocautery. The median follow-up time was 78 months. Bloody stools were absent at the last time of follow-up, and hemoglobin was in the normal range for all patients.

CONCLUSION

The identification of CRVM in children often is delayed. Colonoscopy, CT, and MRI are essential in making the correct diagnosis. The modified Soave procedure is safe and feasible to treat CRVM in children. Endoscopic sclerotherapy and/or electrocautery can be used for residual lesions.

摘要

简介

本研究旨在总结儿童结直肠血管畸形(CRVM)的临床特征和治疗方法。

方法

我们回顾性分析了 2004 年至 2019 年复旦大学附属儿科医院收治的 CRVM 患儿的临床资料。

结果

共纳入 23 例患儿(男 16 例,女 7 例),中位起病年龄为 1.4 岁。便血和贫血为主要症状。14 例(60.9%)患儿误诊为肛裂( = 4)、内痔( = 3)、直肠息肉( = 2)、炎症性肠病( = 2)、门静脉高压症( = 2)和 Meckel 憩室( = 1),平均确诊时间为起病后 4.5 ± 4.4 年。8 例患儿还存在其他部位血管畸形。所有患儿均行增强 CT 或 MRI 检查,提示存在异常血管影像。结肠镜诊断 CRVM 的敏感性为 82.6%(19/23)。21 例行改良 Soave 手术,病变主要局限于结直肠,呈全层弥漫分布,平均长度为 20 ± 5.4 cm。2 例患儿发生手术并发症。2 例(9.5%)患儿术后出现便血复发,结肠镜检查提示吻合口异常血管生成,经硬化剂治疗和/或电凝治疗后治愈。中位随访时间为 78 个月,末次随访时患儿均无便血,血红蛋白均恢复正常。

结论

儿童 CRVM 常延误诊断,结肠镜、CT 和 MRI 对明确诊断至关重要。改良 Soave 手术治疗儿童 CRVM 安全可行,对于残留病变可采用内镜下硬化剂治疗和/或电凝治疗。

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