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超短段型成人先天性巨结肠病:一例腹胀、便秘20余年的病例报告

Ultrashort segment adult Hirschsprung disease: A case report of periodic abdominal distension and constipation spanning for more than 20 years.

作者信息

Jha Amit, Sapkota Hari, Ghimire Prasanna, Paudel Nabin, Ranabhat Roshani, Jha Sushmita

机构信息

Department of Radiology, Nepalgunj Medical College and Teaching Hospital, Banke, Nepal.

出版信息

Radiol Case Rep. 2024 Aug 28;19(11):5328-5331. doi: 10.1016/j.radcr.2024.08.011. eCollection 2024 Nov.

DOI:10.1016/j.radcr.2024.08.011
PMID:39280731
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11399626/
Abstract

The diagnosis of adult Hirschsprung disease (HD) is rare, furthermore there are only 3 or 4 cases of adult ultrashort segment HD (USHD) reported previously in English literature to our knowledge. Herein, we present a case of a 22-year-old female presented with long standing history of constipation and abdominal distension secondary to USHD. Imaging modalities included plain abdominal X-ray, ultrasound (USG) and computed tomography (CT) scan which collectively aided in establishing the diagnosis. Through this case report, we aim to emphasize that HD should be suspected in every adult population with chronic constipation. Also, it highlights the role of CT scan as how it should be the preferred diagnostic tool for suspected adult HD, as, not only it helps correctly identify the transition zone but rule out other possible causes of chronic obstruction.

摘要

成人先天性巨结肠病(HD)的诊断较为罕见,据我们所知,此前英文文献中仅报道过3或4例成人超短段HD(USHD)病例。在此,我们报告一例22岁女性,因USHD出现长期便秘和腹胀病史。影像学检查包括腹部平片、超声(USG)和计算机断层扫描(CT),这些检查共同辅助确诊。通过本病例报告,我们旨在强调,对于每一位患有慢性便秘的成人患者都应怀疑HD。此外,它还突出了CT扫描的作用,即它应是疑似成人HD的首选诊断工具,因为它不仅有助于正确识别移行区,还能排除慢性梗阻的其他可能原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/8ba11febf30e/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/fac691632be6/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/58c0ec46f119/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/cb772af79579/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/8ba11febf30e/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/fac691632be6/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/58c0ec46f119/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/cb772af79579/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bed8/11399626/8ba11febf30e/gr4.jpg

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1
Ultrashort segment adult Hirschsprung disease: A case report of periodic abdominal distension and constipation spanning for more than 20 years.超短段型成人先天性巨结肠病:一例腹胀、便秘20余年的病例报告
Radiol Case Rep. 2024 Aug 28;19(11):5328-5331. doi: 10.1016/j.radcr.2024.08.011. eCollection 2024 Nov.
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本文引用的文献

1
Classification and diagnostic criteria of variants of Hirschsprung's disease.先天性巨结肠症变异型的分类与诊断标准。
Pediatr Surg Int. 2013 Sep;29(9):855-72. doi: 10.1007/s00383-013-3351-3.
2
The developmental etiology and pathogenesis of Hirschsprung disease.先天性巨结肠病的发育病因和发病机制。
Transl Res. 2013 Jul;162(1):1-15. doi: 10.1016/j.trsl.2013.03.001. Epub 2013 Mar 22.
3
Adult large bowel obstruction: a review of clinical experience.成人大肠梗阻:临床经验综述
Ann Afr Med. 2011 Jan-Mar;10(1):45-50. doi: 10.4103/1596-3519.76586.