Short Pancreas in Children With Fanconi Anemia: A Report of Three Cases

A short pancreas (SP), characterized by the absence of the pancreatic tail, is a rare anomaly resulting from abnormal development of the dorsal pancreas and has been documented to cause endocrine pancreatic insufficiency. Fanconi anemia (FA) is associated with multiple congenital malformations and abnormalities in glucose-insulin metabolism; however, dorsal pancreatic anomalies have not been recognized as part of its phenotype. We report three children with FA in whom computed tomography (CT) demonstrated an SP anomaly. SP may be an underrecognized feature of FA, with potential implications for imaging assessment, endocrine surveillance, and future genotype-phenotype studies.

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Publication Details

Journal
Pediatric Blood & Cancer
Published
2026-08-25
DOI
https://doi.org/10.1002/1545-5017.70653
Primary Topic
Intestinal Malrotation and Obstruction Disorders
Type
article
Field-Weighted Citation Impact
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article

Short Pancreas in Children With Fanconi Anemia: A Report of Three Cases

Çiğdem Öztunalı, Zeynep Canan Özdemir, Ahmet Cem Demirşah
Pediatric Blood & Cancer
Intestinal Malrotation and Obstruction Disorders
article

Short Pancreas in Children With Fanconi Anemia: A Report of Three Cases

Çiğdem Öztunalı, Zeynep Canan Özdemir, Ahmet Cem Demirşah
article en

Abstract

A short pancreas (SP), characterized by the absence of the pancreatic tail, is a rare anomaly resulting from abnormal development of the dorsal pancreas and has been documented to cause endocrine pancreatic insufficiency. Fanconi anemia (FA) is associated with multiple congenital malformations and abnormalities in glucose-insulin metabolism; however, dorsal pancreatic anomalies have not been recognized as part of its phenotype. We report three children with FA in whom computed tomography (CT) demonstrated an SP anomaly. SP may be an underrecognized feature of FA, with potential implications for imaging assessment, endocrine surveillance, and future genotype-phenotype studies.

Pediatric Blood & Cancer
Eskişehir Osmangazi University (TR), Afyon Kocatepe University (TR)
Good health and well-being
Openalex Percentile: Top 8%
Intestinal Malrotation and Obstruction Disorders
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