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Genetic and Rare Diseases Information Center (GARD)

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Agenesis of the dorsal pancreas


Other Names for this Disease

  • Complete agenesis of the dorsal pancreas
  • Congenital short pancreas
  • Pancreas agenesis, dorsal
  • Pancreas, dorsal, agenesis of
  • Partial agenesis of the dorsal pancreas
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I was diagnosed in 2009 prior to having gallbladder surgery with agenesis of the dorsal pancreas. I was wondering if there is new or updated information. I have developed chronic pancreatitis after having several acute episodes in the past 2 years. I was wondering as well if there is any new treatment.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is agenesis of the dorsal pancreas?

Agenesis of the dorsal pancreas describes a congenital malformation of the pancreas in which either the entire dorsal pancreas or part of the dorsal pancreas fails to develop (complete agenesis or partial agenesis, respectively). Some individuals experience no symptoms, while others may develop hyperglycemia, diabetes mellitus, bile duct obstruction, abdominal pain, pancreatitis, or other conditions.[1][2][3] Hyperglycemia has been shown to be present in approximately 50% of affected individuals.[3] The cause of agenesis of the dorsal pancreas is currently not well understood. It may occur in individuals with no history of the condition in the family (sporadically) and in some cases, autosomal dominant or X-linked dominant inheritance has been suggested.[4][5] It has also been reported to occur with very rare conditions including polysplenia and polysplenia/heterotaxy syndrome.[6]
Last updated: 7/22/2011

What causes agenesis of the dorsal pancreas?

Partial or complete agenesis of the dorsal pancreas results from the failure of the dorsal pancreatic bud to form the body and tail of the pancreas in the developing fetus.[5] It may occur from the absence, or regression of, the dorsal bud during fetal development. Heredity may play a role in the development of this condition, but further research is needed to clarify this.[5]

There have been reports in the literature of the condition being associated (rarely) with other congenital diseases, specifically a very rare disorder called polysplenia/heterotaxy syndrome. In this case, it may occur due to errors in development of the asymmetric organs and may be associated with benign to severe congenital cardiac (heart) malformations.[6]
Last updated: 7/22/2011

How might agenesis of the dorsal pancreas be treated?

Because agenesis of the dorsal pancreas is considered rare and few cases have been reported in the literature, there is limited information about how the condition as a whole might be treated or managed. However, there is current information about how some of the signs and symptoms associated with agenesis of the dorsal pancreas (such as pancreatitis) may be managed.

For pancreatitis, individuals may be able to make themselves more comfortable during an attack, but they will most likely continue to have attacks until treatment is received for the underlying cause of the symptoms (when possible). If symptoms are mild, people might try the following preventive measures: stopping all alcohol consumption; adopting a liquid diet consisting of foods such as broth, gelatin, and soups (these simple foods may allow the inflammation process to get better); over-the-counter pain medications; and avoiding pain medications that can affect the liver (such as acetaminophen). Medical treatment is usually focused on relieving symptoms and preventing further aggravation to the pancreas. Certain complications of either acute pancreatitis or chronic pancreatitis may require surgery or a blood transfusion. In acute pancreatitis, the choice of treatment is based on the severity of the attack. Most people who are having an attack of acute pancreatitis are admitted to the hospital for oxygen (if having trouble breathing) and an intravenous (IV) line for medications and fluids. If needed, medications for pain and nausea may be prescribed. It may be recommended that no food or liquid is taken by mouth for a few days (this is called bowel rest). Some people may need a nasogastric (NG) tube to remove stomach juices which rests the intestine further, helping the pancreas recover. If the attack lasts longer than a few days, nutritional supplements may be administered through an IV line. In chronic pancreatitis, treatment focuses on relieving pain and avoiding further aggravation to the pancreas.[7]

Hyperglycemia (high blood sugar) management may depend on the exact cause if the condition in the affected individual. Management may include checking blood sugar levels with a blood glucose meter; checking urine for ketones; and adopting strategies to lower blood sugar level. Strategies might include exercise (only if urine ketones are not present); diet as discussed with a diabetes health educator or registered dietitian; and/or medication (especially if diet and exercise are not keeping blood sugar levels in the normal range) which may include insulin and/or other medications.[8]

Individuals seeking treatment options for themselves or others should speak with their health care provider about an individualized treatment plan; the information here is provided for general educational purposes only.
Last updated: 7/25/2011

Where can I find the most up-to-date information about agenesis of the dorsal pancreas, including treatment information?

The National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. No studies involving agenesis of the dorsal pancreas are listed at this time, but check this site often for updates. To search for a study, use "agenesis dorsal pancreas" as your search term.

There are studies involving pancreatitis listed at this time; to see a list of studies involving pancreatitis, click here.

You can also find relevant articles on agenesis of the dorsal pancreas through PubMed, a searchable database of biomedical journal articles. Although not all of the articles are available for free online, most articles listed in PubMed have a summary available. To obtain the full article, contact a medical/university library or your local library for interlibrary loan. You can also order articles online through the publisher’s Web site. Using "agenesis dorsal pancreas" as your search term should help you locate articles. Click here to view a search.

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.
Last updated: 7/25/2011

References
  • Whitcomb DC, Lowe ME. Hereditary, Familial, and Genetic Disorders of the Pancreas and Pancreatic Disorders in Childhood. In: Feldman. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010;
  • Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009 Mar;
  • Wolfgang J Schnedl, Claudia Piswanger-Soelkner, Sandra J Wallner, Robert Krause, Rainer W Lipp. Agenesis of the dorsal pancreas. World Journal of Gastroenterology. January 2009; 15(3):376-377. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2653338/pdf/WJG-15-376.pdf. Accessed 7/22/2011.
  • Joanna S. Amberger . Pancreas, Dorsal, Agenesis of. OMIM. May 10, 2011; http://omim.org/entry/167755. Accessed 7/22/2011.
  • Joo YE, Kang HC, Kim HS, Choi SK, Rew JS, Chung MY, Kim SJ. Agenesis of the dorsal pancreas: a case report and review of the literature. Korean J Intern Med. December 2006; 21(4):236-239. http://www.kjim.or.kr/upload/abstract/files/062104236.pdf. Accessed 7/22/2011.
  • Suraj Kapa, Ferga C Gleeson, Santhi Swaroop Vege. Dorsal Pancreas Agenesis and Polysplenia/Heterotaxy Syndrome: A Novel Association with Aortic Coarctation and a Review of the Literature. JOP. J Pancreas (Online). 2007; 8(4):433-437. http://www.joplink.net/prev/200707/200707_02.pdf. Accessed 7/22/2011.
  • Pancreatitis. eMedicineHealth. 2011; http://www.emedicinehealth.com/pancreatitis/page6_em.htm. Accessed 7/22/2011.
  • Robert Ferry, Jr. High Blood Sugar (Hyperglycemia). eMedicineHealth. 2011; http://www.emedicinehealth.com/high_blood_sugar_hyperglycemia/article_em.htm. Accessed 7/25/2011.
Other Names for this Disease
  • Complete agenesis of the dorsal pancreas
  • Congenital short pancreas
  • Pancreas agenesis, dorsal
  • Pancreas, dorsal, agenesis of
  • Partial agenesis of the dorsal pancreas
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.