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一名4岁儿童不完全性肠旋转不良的延迟诊断:非典型胃肠道旋转不良的病例报告,资源有限的医疗机构中的诊断和管理挑战

A delayed diagnosis of incomplete intestinal rotation in a 4-year-old: A case report of atypical gastrointestinal malrotation, diagnostic and management challenges in a resource-constrained health facility.

作者信息

Shadrack Mathayo, Bayyo Neema Lala, Ngotta Victor, Ngiloi Petronilla, Bokhary Zaituni, Salim Mohammed Sultan

机构信息

Muhimbili University of Health and Allied Science (MUHAS), P.O. Box 65001, Dar es salaam, Tanzania; Department of Pediatric Surgery, Muhimbili National Hospital (MNH), P.O. Box 65000, Dar es salaam, Tanzania; Department of Pediatric Surgery, Kilimanjaro Christian Medical Center, P.O. Box 3010, Moshi, Kilimanjaro, Tanzania.

Muhimbili University of Health and Allied Science (MUHAS), P.O. Box 65001, Dar es salaam, Tanzania; Department of Pediatric Surgery, Muhimbili National Hospital (MNH), P.O. Box 65000, Dar es salaam, Tanzania.

出版信息

Int J Surg Case Rep. 2025 May;130:111309. doi: 10.1016/j.ijscr.2025.111309. Epub 2025 Apr 15.

DOI:10.1016/j.ijscr.2025.111309
PMID:40245682
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12032304/
Abstract

BACKGROUND

Gastrointestinal malrotation is a rare congenital anomaly caused by incomplete midgut rotation during embryogenesis. It affects approximately 1 in 500 live births, with symptomatic cases seen in about 1 in 6000 children. Although over 75 % are diagnosed in the neonatal period, older children may present with nonspecific symptoms.

CASE PRESENTATION

We report a 4-year-old male with a two-year history of recurrent bilious vomiting and intermittent abdominal distension. Initially misdiagnosed as gastritis and treated with proton pump inhibitors without improvement, the patient underwent an abdominal ultrasound that revealed a normal mesenteric vessel but an abnormal duodenal position. The third part of the duodenum (D3) was located to the right of the superior mesenteric vessels instead of posteriorly, and localized dilation of the proximal duodenum and stomach suggested intestinal malrotation.

DISCUSSION

Surgical exploration via a supra-umbilical transverse incision confirmed incomplete midgut rotation. The duodenojejunal junction was abnormally positioned in a mid-right lateral orientation, and the cecum was found in the right upper anterior quadrant. Significant Ladd's bands were compressing the anterior duodenum, causing dilation, while the mesenteric base was markedly narrowed and shortened, increasing volvulus risk. A standard Ladd's procedure was performed, dividing the bands and broadening the mesenteric base. Prophylactic appendectomy was omitted because the appendix appeared normal, the corrected anatomy minimized future diagnostic confusion, and the risk of appendicitis was low.

CONCLUSION

This case highlights the diagnostic value of ultrasound in older children with gastrointestinal symptoms and underscores early recognition and prompt surgical intervention. These findings greatly impact clinical decision-making and management.

摘要

背景

胃肠道旋转不良是一种罕见的先天性异常,由胚胎发育过程中中肠旋转不完全引起。其在活产儿中的发生率约为1/500,在儿童中的症状性病例约为1/6000。虽然超过75%的病例在新生儿期被诊断出来,但大龄儿童可能表现出非特异性症状。

病例介绍

我们报告一名4岁男性,有两年反复胆汁性呕吐和间歇性腹胀病史。最初被误诊为胃炎,用质子泵抑制剂治疗后无改善,患者接受腹部超声检查,显示肠系膜血管正常,但十二指肠位置异常。十二指肠第三部(D3)位于肠系膜上血管右侧而非后方,十二指肠近端和胃的局限性扩张提示肠道旋转不良。

讨论

经脐上横向切口进行手术探查证实中肠旋转不完全。十二指肠空肠交界处异常位于右中外侧位,盲肠位于右上腹前象限。明显的Ladd束压迫十二指肠前部,导致扩张,而肠系膜根部明显变窄和缩短,增加了肠扭转风险。进行了标准的Ladd手术,切断束带并加宽肠系膜根部。由于阑尾外观正常,纠正后的解剖结构可减少未来诊断的混淆,且阑尾炎风险较低,因此未进行预防性阑尾切除术。

结论

本病例突出了超声在有胃肠道症状的大龄儿童中的诊断价值,并强调了早期识别和及时手术干预的重要性。这些发现对临床决策和管理有很大影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/9d8dd7e66873/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/6bd2580584ec/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/5c90c1c79483/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/09fd6e5d4e89/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/8a6a14f516cf/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/345dc89c33f3/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/9d8dd7e66873/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/6bd2580584ec/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/5c90c1c79483/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/09fd6e5d4e89/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/8a6a14f516cf/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/345dc89c33f3/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c872/12032304/9d8dd7e66873/gr6.jpg

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本文引用的文献

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Surg Case Rep. 2024 Sep 26;10(1):226. doi: 10.1186/s40792-024-02029-y.
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