Department of Pediatric Surgery, Buzzi Children's Hospital, 20154, Milan, Italy.
Department of Pediatric Radiology, Buzzi Children's Hospital, 20154, Milan, Italy.
Pediatr Surg Int. 2024 Nov 1;40(1):283. doi: 10.1007/s00383-024-05881-z.
Alimentary tract duplications (ATDs) are rare congenital lesions often associated with anomalies such as spinal, urinary and GI tract malformations. The purpose of this study was to report the experience of a single center with ATDs in children, focusing on the natural history, associated malformations, and their impact on patient management.
We performed a retrospective analysis over 14 years, collecting prenatal, clinical, surgical, and follow-up data. We focus on associated anomalies prenatal and postnatal management, and outcomes.
Sixty-three patients with ATD (thirty-six females, twenty-seven males, aged 1 day to 14 years) were enrolled in this study. Prenatal diagnosis was made in 22 patients (35%), of whom 8 showed compression signs. Elective surgery was performed at a mean age of 1.5 years in prenatally diagnosed cases. The others presented symptoms at a mean age of 5.2 years (55.5%) or were detected incidentally (9.5%) at a mean age of 10.7 years. In four patients (6.3%), we identified multiple duplications, and ten cases (15.8%) were found with associated anomalies.
The wide spectrum of clinical appearance of ATDs and a comprehensive knowledge of human embryology might define surgical management, which should always be patient-tailored and respectful of the child's development.
消化道重复畸形(ATD)是一种罕见的先天性病变,常与脊柱、泌尿和胃肠道畸形等异常相关。本研究的目的是报告一个单中心的儿童 ATD 经验,重点关注其自然史、相关畸形及其对患者管理的影响。
我们进行了为期 14 年的回顾性分析,收集了产前、临床、手术和随访数据。我们重点关注相关畸形的产前和产后管理以及结局。
本研究共纳入 63 例 ATD 患儿(女性 36 例,男性 27 例,年龄 1 天至 14 岁)。22 例(35%)患儿产前诊断为 ATD,其中 8 例存在压迫征象。产前诊断的患儿在平均 1.5 岁时进行了择期手术,其他患儿在平均 5.2 岁时因症状出现(55.5%)或平均 10.7 岁时偶然发现(9.5%)而就诊。4 例(6.3%)患儿存在多发重复畸形,10 例(15.8%)患儿存在相关畸形。
ATD 的临床表现广泛,对人类胚胎学有全面的了解,可能有助于确定手术管理方案,该方案应始终根据患者的具体情况制定,并尊重儿童的发育。