Synonyms
Absence of the pancreas; Partial agenesis of the dorsal pancreas/ Pancreas Hypoplasia/ Short Pancreas
Definition
Pancreatic agenesis is a malformation resulting in the absence of the pancreas or parts of the pancreas.
Complete agenesis of the pancreas is a congenital condition with complete absence of pancreatic tissue due to missing pancreatic anlage.
Partial agenesis of the pancreas in form of agenesis of the dorsal or ventral pancreas exists, depending on which anlage – ventral or dorsal – fails to develop during embryogenesis.
Clinical Features
Incidence
Pancreatic agenesis is a very rare anomaly without clear numbers yet, e.g., for complete dorsal pancreatic pancreas, which is the most reported form of pancreatic agenesis, only around 100 cases of have been published since 1911 (Schnedl et al. 2009).
Age
Agenesis of the pancreas is a congenital condition.
Sex
Due to the low number of cases, the gender distribution of pancreatic agenesis is not clear yet.
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References and Further Reading
Cienfuegos, J. A., Rotellar, F., Salguero, J., Benito, A., Solórzano, J. L., & Sangro, B. (2016). Agenesis of the dorsal pancreas: Systematic review of a clinical challenge. Revista espanola de enfermedades digestivas: organo oficial de la Sociedad Espanola de Patologia Digestiva, 108(8), S. 479–S. 484. https://doi.org/10.17235/reed.2016.4474/2016.
Guimarães, A. B., Guimarães, C. A., & Manso, J. E. F. (2015). Agenesis or pseudoagenesis of the dorsal pancreas. Revista do Colégio Brasileiro de Cirurgiões, 42(5), S. 352–S. 355. https://doi.org/10.1590/0100-69912015005015.
Robert, A. P., Iqbal, S., & John, M. (2016). Complete agenesis of the dorsal pancreas: A rare clinical entity. International Journal of Applied & Basic Medical Research, 6(4), S. 290–S. 292. https://doi.org/10.4103/2229-516X.192599.
Schnedl, W. J., Piswanger-Soelkner, C., Wallner, S. J., Reittner, P., Krause, R., Lipp, R. W., & Hohmeier, H. E. (2009). Agenesis of the dorsal pancreas and associated diseases. Digestive Diseases and Sciences, 54(3), S. 481–S. 487. https://doi.org/10.1007/s10620-008-0370-3.
Voldsgaard, P., Kryger-Baggesen, N., & Lisse, I. (1994). Agenesis of pancreas. Acta Paediatrica (Oslo, Norway: 1992), 83(7), S. 791–S. 793. https://doi.org/10.1111/j.1651-2227.1994.tb13144.x.
Xuan, S., Borok, M. J., Decker, K. J., Battle, M. A., Duncan, S. A., Hale, M. A., et al. (2012). Pancreas-specific deletion of mouse Gata4 and Gata6 causes pancreatic agenesis. The Journal of Clinical Investigation, 122(10), S. 3516–S. 3528. https://doi.org/10.1172/JCI63352.
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Ingenhoff, L. (2021). Agenesis. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5501-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5501-1
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