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Three cases of pancreatic pseudocysts associated with dorsal pancreatic agenesis.

ABSTRACT: Agenesis of the dorsal pancreas (ADP) is an extremely rare congenital anomaly. Human pancreas is formed by ventral and dorsal endodermal buds of the foregut endoderm. The dorsal bud forms the upper part of the head, neck, body, and tail of the pancreas and the ventral bud generates most of the head and uncinate process. ADP is derived from the embryologic failure of the dorsal pancreatic bud to form the pancreatic body and tail. ADP can be related to some diseases and conditions such as pancreatitis, hypoglycemia, and rarely pancreatic tumors. The association between cystic lesions with ADP has previously been reported. Three cases of cystic lesions of the pancreas with ADP were diagnosed clinically based on the imaging features and without any past history of pancreatitis. However, the pathologic diagnosis of resected lesions confirmed pseudocysts without pathologic evidence of tumor. We report 3 cases of pancreatic pseudocysts associated with ADP.

SUBMITTER: Suh PS

PROVIDER: S-EPMC6202790 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature

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Three cases of pancreatic pseudocysts associated with dorsal pancreatic agenesis.

Suh Pae Sun PS Lee Jei Hee JH Yu Jeong-Sik JS Hee Kim Joo J Kim Bohyun B Kim Hye Jin HJ Huh Jimi J Kim Jai Keun JK Lee Dakeun D

Radiology case reports 20181023 1

Agenesis of the dorsal pancreas (ADP) is an extremely rare congenital anomaly. Human pancreas is formed by ventral and dorsal endodermal buds of the foregut endoderm. The dorsal bud forms the upper part of the head, neck, body, and tail of the pancreas and the ventral bud generates most of the head and uncinate process. ADP is derived from the embryologic failure of the dorsal pancreatic bud to form the pancreatic body and tail. ADP can be related to some diseases and conditions such as pancreat ...[more]

PMID: 30377453

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Project description:This article aims to elucidate the classification of and optimal treatment for pancreatic pseudocysts. Various approaches, including endoscopic drainage, percutaneous drainage, and open surgery, have been employed for the management of pancreatic pseudocysts. However, no scientific classification of pancreatic pseudocysts has been devised, which could assist in the selection of optimal therapy. We evaluated the treatment modalities used in 893 patients diagnosed with pancreatic pseudocysts according to the revision of the Atlanta classification in our department between 2001 and 2010. All the pancreatic pseudocysts have course of disease >4 weeks and have mature cysts wall detected by computed tomography or transabdominal ultrasonography. Endoscopic drainage, percutaneous drainage, or open surgery was selected on the basis of the pseudocyst characteristics. Clinical data and patient outcomes were reviewed. Among the 893 patients, 13 (1.5%) had percutaneous drainage. Eighty-three (9%) had type I pancreatic pseudocysts and were treated with observation. Ten patients (1%) had type II pseudocysts and underwent the Whipple procedure or resection of the pancreatic body and tail. Forty-six patients (5.2%) had type III pseudocysts: 44 (4.9%) underwent surgical internal drainage and 2 (0.2%) underwent endoscopic drainage. Five hundred six patients (56.7%) had type IV pseudocysts: 297 (33.3%) underwent surgical internal drainage and 209 (23.4%) underwent endoscopic drainage. Finally, 235 patients (26.3%) had type V pseudocysts: 36 (4%) underwent distal pancreatectomy or splenectomy and 199 (22.3%) underwent endoscopic drainage. A new classification system was devised, based on the size, anatomical location, and clinical manifestations of the pancreatic pseudocyst along with the relationship between the pseudocyst and the pancreatic duct. Different therapeutic strategies could be considered based on this classification. When clinically feasible, endoscopic drainage should be considered the optimal management strategy for pancreatic pseudocysts.

Dataset Information

Three cases of pancreatic pseudocysts associated with dorsal pancreatic agenesis.

Publications

Three cases of pancreatic pseudocysts associated with dorsal pancreatic agenesis.

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