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一名年轻患者同时患有胰腺背侧发育不全、胆总管囊肿和先天性巨结肠的罕见病例。

A Rare Case of Dorsal Agenesis of Pancreas, Choledochal Cyst, and Hirschsprung Disease in a Young Patient.

作者信息

Chapa Uday Kumar, Dutta Souradeep, Remesh Athul Minija, Jain Ankit, Abhinaya Reddy, Nelamangala Ramakrishnaiah Vishnu Prasad

机构信息

Division of GI and HPB Surgery Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

出版信息

ACG Case Rep J. 2021 Apr 26;8(4):e00561. doi: 10.14309/crj.0000000000000561. eCollection 2021 Apr.

Abstract

Dorsal pancreatic agenesis is a rare congenital pancreatic malformation. There is just 1 reported case associating it with choledochal cyst. However, no cases have reported yet with both coexisting with Hirschsprung disease. We report a case of a 23-year-old man, presenting with on and off epigastric pain, sometimes radiating to the back. His medical records showed he had Hirschsprung disease as a neonate, for which he underwent Duhamel procedure. Ultrasound imaging revealed a choledochal cyst and a nonvisualized distal portion of the pancreas. Further cross-sectional imaging confirmed the findings-a type 1 choledochal cyst and a dorsal agenesis of the pancreas in a patient with Hirschsprung disease.

摘要

胰腺背侧发育不全是一种罕见的先天性胰腺畸形。仅有1例报告将其与胆总管囊肿相关联。然而,尚未有同时合并先天性巨结肠病的病例报告。我们报告1例23岁男性患者,表现为间歇性上腹部疼痛,有时放射至背部。他的病历显示其新生儿期患有先天性巨结肠病,并接受了杜哈梅尔手术。超声成像显示一个胆总管囊肿以及胰腺远端未显影。进一步的断层成像证实了这些发现——一名患有先天性巨结肠病的患者存在1型胆总管囊肿和胰腺背侧发育不全。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a94b/8078483/6fb36888cf99/ac9-8-e00561-g001.jpg

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