Our Summary

This research paper discusses a rare disease called Primary Intestinal Lymphangiectasia (PIL), also known as Waldmann’s disease. The cause of this disease is unknown, but it is characterized by enlarged intestinal passageways which result in the leakage of lymph (a fluid containing white blood cells) into the small intestine. This causes a condition where proteins are lost from the gut, leading to a decrease in proteins, albumin and antibodies in the blood.

PIL is typically diagnosed before the age of 3, but can also be found in older patients. The main symptom is swelling in both lower legs, which can range from mild to severe. Other symptoms may include a build-up of fluid around the lungs, heart or in the abdomen.

Diagnosis of PIL is confirmed through certain stool tests and by observing the disease’s characteristics in the intestines through an endoscopy - a procedure that uses a small camera to view the inside of the body. A video capsule endoscopy, which involves swallowing a small capsule with a camera inside, may be used if the initial endoscopy doesn’t provide enough information.

Patients with PIL may also develop B-cell lymphomas, a type of cancer that starts in the white blood cells and can be found in the gastrointestinal tract or other areas of the body.

The main treatment for PIL involves a strict low-fat diet, supplemented with medium-chain triglycerides (a type of fat) and fat-soluble vitamins. Some patients may also be given Octreotide, a medication that mimics a hormone in the body, although its effectiveness varies. In some rare cases, surgery to remove a portion of the small intestine may be necessary.

The paper suggests that patients with PIL should be monitored for a long time due to the potential development of lymphomas and other complications.

FAQs

  1. What is Primary intestinal lymphangiectasia (PIL) or Waldmann’s disease?
  2. How is Primary intestinal lymphangiectasia (PIL) diagnosed and managed?
  3. What is the relevance of surgical small-bowel resection in the treatment of PIL?

Doctor’s Tip

A doctor may advise a patient who has undergone intestinal resection to follow a low-fat diet and take medium-chain triglyceride and liposoluble vitamin supplements to help manage their condition. It is also important for the patient to have regular follow-up appointments to monitor their progress and overall health.

Suitable For

Patients with primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, are typically recommended intestinal resection in rare cases with segmental and localized intestinal lymphangiectasia. This may be considered when conservative medical management, such as a long-term low-fat diet and supplementation, is not effective in managing protein-losing enteropathy and other symptoms associated with the condition. It is important for patients with PIL to have a prolonged clinical and biological follow-up to monitor their condition and response to treatment.

Timeline

Before intestinal resection:

  • Patient experiences symptoms such as bilateral lower limb edema, pleural effusion, pericarditis, or ascites
  • Protein-losing enteropathy is confirmed through elevated 24-hour stool α1-antitrypsin clearance
  • Diagnosis is made through endoscopic observation of intestinal lymphangiectasia with corresponding histology of biopsies
  • Videocapsule endoscopy may be used if endoscopic findings are inconclusive
  • Medical management includes a low-fat diet, medium-chain triglyceride, and liposoluble vitamin supplementation
  • Octreotide, a somatostatin analog, may be used with diet but has inconsistent efficacy

After intestinal resection:

  • Surgical small-bowel resection may be performed in rare cases with segmental and localized intestinal lymphangiectasia
  • Prolonged clinical and biological follow-up is recommended to monitor for any complications or recurrence of symptoms

What to Ask Your Doctor

  1. What is the reason for recommending an intestinal resection in my case?
  2. What are the potential risks and complications associated with this procedure?
  3. How long is the recovery process expected to be after the surgery?
  4. Will I need to make any changes to my diet or lifestyle after the intestinal resection?
  5. Are there any alternative treatment options available for my condition?
  6. Will I need to undergo any additional tests or procedures before the surgery?
  7. How often will I need to follow up with you after the surgery?
  8. What symptoms should I watch out for that may indicate a complication after the surgery?
  9. Are there any long-term effects or implications of having an intestinal resection?
  10. Will I need any additional medications or supplements after the surgery?

Reference

Authors: Vignes S, Bellanger J. Journal: Rev Med Interne. 2018 Jul;39(7):580-585. doi: 10.1016/j.revmed.2017.07.009. Epub 2017 Sep 1. PMID: 28867533