Complete Agenesis of Dorsal Pancreas with Pancreatic Cyst: A Case Report

Ustabaşıoğlu, Fethi Emre; Kurt, Nazmi; Tunçbilek, Nermin

doi:10.14744/nci.2020.24444

Cited by 4 publications

(2 citation statements)

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“…Dorsal pancreatic agenesis is a rare congenital disorder characterized by abnormal embryogenesis and is compatible with life whereas complete agenesis of the pancreas or ventral agenesis is incompatible with life [ 1 ]. Dorsal pancreatic agenesis can be asymptomatic or cause epigastric discomfort, hyperglycemia, and acute or chronic pancreatitis [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

Jha¹,

Prasad²,

Kumar³

et al. 2023

Cureus

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Background Agenesis of the dorsal pancreas is a rare entity and is caused due to the defective embryological development of the pancreas. The clinical manifestations may range from diabetes mellitus, pancreatitis, and abdominal pain to no symptoms at all. We here present a case series of 10 cases with complete or partial agenesis of the dorsal pancreas. Objectives To correlate the clinical symptoms in the patients with the dorsal agenesis of the pancreas, to study any biochemical abnormality present with the dorsal agenesis of the pancreas, and to look for other coexistent finding in the patients. Results We observed that out of 10 patients, six were males and four were females. Four had symptoms related to the pancreas and six were discovered incidentally. Diabetes mellitus was present in five patients, seven patients had pain in the abdomen, and jaundice was seen in three patients. Out of 10 patients, four had complete agenesis and six had partial agenesis of the dorsal pancreas. Conclusions We conclude that the diagnosis of this rare entity and establishing its association with clinical conditions like diabetes mellitus and non-specific abdomen pain with the aid of cross-sectional imaging helps in the better evaluation and management of the patients.

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Section: Introductionmentioning

confidence: 99%

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

Jha¹,

Prasad²,

Kumar³

et al. 2023

Cureus

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“…Agenesis of the dorsal pancreas (ADP) is an extremely rare congenital malformation, which is featured by the absence of corpus and cauda of the pancreas. The first case of ADP was described in 1911 and 134 cases have been reported up till 2021 (1)(2)(3)(4)(5)(6)(7)(8). Genes, whose mutation may cause pancreatic agenesis, include GATA binding protein 6 (GATA6), insulin promoter factor-1 (IPF1), pancreas transcription factor 1alpha (PTF1A), hepatocyte nuclear factor 1A (HNF1A), and hepatocyte nuclear factor 1B (HNF1B).…”

Section: Introductionmentioning

confidence: 99%

Association of Agenesis of the Dorsal Pancreas With HNF1B Heterozygote Mutation: A Case Report

Guo

et al. 2021

Front. Endocrinol.

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BackgroundAgenesis of the dorsal pancreas (ADP) is a rare disease, the pathogenic mechanism of which is partially related to variants of hepatocyte nuclear factor 1B (HNF1B) gene.Case PresentationWe report a case of ADP, which presented with acute ketoacidosis, hyperuricemia, and liver dysfunction. In this case, the HNF1B score was estimated as 16 and a heterozygous variant of HNF1B in exon 2 (c.513G>A-p.W171X) was identified through gene sequencing.ConclusionsA good understanding of the clinical comorbidities of ADP is essential for avoiding missed diagnosis to a great extent. Moreover, estimation of HNF1B score is recommended before genetic testing.

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Dorsal pancreatic agenesis: a case report

Pant

Dhakal

Choudhary³

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and importance: The pancreas develops from a small ventral bud and a larger dorsal bud. During the rotation of the foregut, the ventral pancreas rotates toward the dorsal pancreas and joins together to form a complete pancreas with ducts. Among the various developmental congenital anomalies, dorsal pancreatic agenesis is one of the rare entities, with less than a hundred cases reported so far. It involves the absence of the dorsal bud derivatives (head, body, and tail). Case presentation: Herein, we present a case of a 50-year-old woman who presented to general outpatient department with a complaint of abdominal pain. The patient was diagnosed with cholelithiasis with a contrast-enhanced computed tomography finding of dorsal pancreatic agenesis on a detailed workup. However, the patient did not have any other associated anomalies or symptoms associated with dorsal pancreatic agenesis. The patient was managed for cholelithiasis with laparoscopic cholecystectomy. Clinical discussion: Failure in development due to aberrant embryogenesis may cause partial or complete agenesis of the dorsal pancreas. The minor papilla, the accessory pancreatic duct, the body, and the tail of the pancreas are not present in the case of complete dorsal agenesis. Most cases of dorsal pancreatic agenesis are asymptomatic and diagnosed incidentally, whereas some of the cases may present with other associated abnormalities. It is almost always diagnosed via imaging modalities. Conclusion: Dorsal pancreatic agenesis is a very rare congenital anomaly of the pancreas. It can be diagnosed via various imaging modalities and almost always remains a radiological diagnosis with incidental findings.

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Complete Agenesis of Dorsal Pancreas with Pancreatic Cyst: A Case Report

Cited by 4 publications

References 0 publications

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

Association of Agenesis of the Dorsal Pancreas With HNF1B Heterozygote Mutation: A Case Report

Dorsal pancreatic agenesis: a case report

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