Grey Callie A, Desai Ajinkya, Nowicki Michael J, Bhesania Natalie
From the Division of Interventional Radiology, Department of Pediatrics, University of Mississippi Medical Center, Jackson, MS.
Division of Pediatric Gastroenterology, Division of Interventional Radiology, University of Mississippi Medical Center, Jackson, MS.
JPGN Rep. 2023 Jul 17;4(3):e337. doi: 10.1097/PG9.0000000000000337. eCollection 2023 Aug.
Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly that occurs when the body and tail of the pancreas fail to develop from the dorsal bud in utero. ADP may be discovered when evaluating conditions arising from the anomaly, such as diabetes mellitus, pancreatitis, and pancreatic insufficiency, but is more commonly found as an incidental finding. To date, fewer than 120 cases have been reported in the literature. We report a 6-year-old male who was found to have ADP on computed tomography during the investigation of abdominal pain and vomiting. We review the variable presentation, genetic mutations, and age-related differences between children and adults with this rare condition.
背侧胰腺发育不全(ADP)是一种罕见的先天性异常,发生于胰腺体部和尾部在子宫内未能从背侧芽发育而来时。ADP可能在评估由该异常引发的疾病(如糖尿病、胰腺炎和胰腺功能不全)时被发现,但更常见的是作为偶然发现。迄今为止,文献报道的病例少于120例。我们报告一名6岁男性,在腹痛和呕吐检查期间通过计算机断层扫描发现患有ADP。我们回顾了这种罕见疾病在儿童和成人中的不同表现、基因突变以及年龄相关差异。