Dupont-Sellier-Chochillon Syndrome

Dupont-Sellier-Chochillon Syndrome

Article
Digestive Health
Kids' Zone
+4
Contributed byMaulik P. Purohit MD MPHApr 13, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Pancreatic Lipomatosis Duodenal Atresia Syndrome 
  • Pancreatic Lipomatosis Duodenal Stenosis or Atresia Syndrome 

What is Dupont-Sellier-Chochillon Syndrome? (Definition/Background Information)

  • Dupont-Sellier-Chochillon Syndrome is a rare congenital disorder, characterized by fat replacing cells in the pancreas, pancreatic hormone disturbances and under-development or narrowing of duodenum (a portion of the small intestine)
  • The replacement of pancreatic cells by mature fat cells can either be localized or diffuse

Who gets Dupont-Sellier-Chochillon Syndrome? (Age and Sex Distribution)

  • Dupont-Sellier-Chochillon Syndrome is a rare congenital disorder. The presentation of symptoms may occur at birth
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Dupont-Sellier-Chochillon Syndrome? (Predisposing Factors)

  • Currently, no risk factors have been clearly identified for Dupont-Sellier-Chochillon Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dupont-Sellier-Chochillon Syndrome? (Etiology)

The exact underlying cause of Dupont-Sellier-Chochillon Syndrome is currently unknown.

What are the Signs and Symptoms of Dupont-Sellier-Chochillon Syndrome?

The signs and symptoms of Dupont-Sellier-Chochillon Syndrome may vary among affected individuals, depending on the extent of fat in the pancreas and the narrowing of duodenum. While some of those affected may be asymptomatic, others may exhibit the following signs and symptoms:

  • Failure to thrive in the neonatal and infancy phases
  • Nausea and vomiting
  • Indigestion
  • Diarrhea
  • Foul-smelling, fatty stools (particularly after ingestion of fatty foods)
  • Malabsorption
  • Poor weight gain
  • Fatigue (getting tired easily)

How is Dupont-Sellier-Chochillon Syndrome Diagnosed?

Dupont-Sellier-Chochillon Syndrome is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dupont-Sellier-Chochillon Syndrome?

The complications of Dupont-Sellier-Chochillon Syndrome may develop due to an increase in the size of pancreas due to fat mass or severe narrowing of duodenum, causing:

  • Dehydration
  • Delayed growth and development
  • Gastrointestinal obstruction
  • Biliary tract obstruction, causing jaundice
  • Compression of adjoining organs, which may cause the organs to dysfunction or function abnormally
  • Diabetes, if enough insulin producing cells are replaced by fat
  • Severe malabsorption

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Dupont-Sellier-Chochillon Syndrome Treated?

The treatment for Dupont-Sellier-Chochillon Syndrome may be undertaken, if an affected individual has severe symptoms, and may include:

  • Avoiding fatty food
  • Surgical correction of narrowed duodenum
  • Surgery to remove fat tissue from pancreas

How can Dupont-Sellier-Chochillon Syndrome be Prevented?

  • Dupont-Sellier-Chochillon Syndrome may not be preventable, since the cause of the disorder is unknown
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Dupont-Sellier-Chochillon Syndrome? (Outcomes/Resolutions)

  • The prognosis of Dupont-Sellier-Chochillon Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications
  • Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Dupont-Sellier-Chochillon Syndrome:

The following DoveMed website links are useful resources for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

http://www.dovemed.com/diseases-conditions/lipoma-pancreas/

Was this article helpful

On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

0 Comments

Please log in to post a comment.

Related Articles

Test Your Knowledge

Asked by users

Related Centers

Loading

Related Specialties

Loading card

Related Physicians

Related Procedures

Related Resources

Join DoveHubs

and connect with fellow professionals

Related Directories

Who we are

At DoveMed, our utmost priority is your well-being. We are an online medical resource dedicated to providing you with accurate and up-to-date information on a wide range of medical topics. But we're more than just an information hub - we genuinely care about your health journey. That's why we offer a variety of products tailored for both healthcare consumers and professionals, because we believe in empowering everyone involved in the care process.
Our mission is to create a user-friendly healthcare technology portal that helps you make better decisions about your overall health and well-being. We understand that navigating the complexities of healthcare can be overwhelming, so we strive to be a reliable and compassionate companion on your path to wellness.
As an impartial and trusted online resource, we connect healthcare seekers, physicians, and hospitals in a marketplace that promotes a higher quality, easy-to-use healthcare experience. You can trust that our content is unbiased and impartial, as it is trusted by physicians, researchers, and university professors around the globe. Importantly, we are not influenced or owned by any pharmaceutical, medical, or media companies. At DoveMed, we are a group of passionate individuals who deeply care about improving health and wellness for people everywhere. Your well-being is at the heart of everything we do.

© 2023 DoveMed. All rights reserved. It is not the intention of DoveMed to provide specific medical advice. DoveMed urges its users to consult a qualified healthcare professional for diagnosis and answers to their personal medical questions. Always call 911 (or your local emergency number) if you have a medical emergency!