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Long-Term Catheterization of the Intestinal Lymph Trunk and Collection of Lymph in Neonatal Pigs
Published on: March 5, 2016
[Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports]
A Munck1, G Sosa Valencia, C Faure
1Service de gastroentérologie et nutrition pédiatriques, hôpital Robert-Debré, 48, boulevard Sérurier, 75019 Paris, France. anne.munck@rdb.ap-hop-paris.fr
Insights
Primary intestinal lymphangiectasia requires a lifelong low-fat diet for symptom management. Even asymptomatic patients show persistent lymphatic abnormalities, highlighting the need for long-term dietary adherence.
Area of Science:
- Gastroenterology
- Pediatric Medicine
- Endocrinology
Context:
- Primary intestinal lymphangiectasia (PIL) is a rare disorder causing protein-losing gastroenteropathy.
- Long-term follow-up data for pediatric PIL patients are scarce.
- Understanding the long-term prognosis and management of PIL is crucial for patient care.
Purpose:
- To evaluate the long-term outcomes of children diagnosed with primary intestinal lymphangiectasia.
- To assess the efficacy and necessity of a strict low-fat diet in managing PIL.
- To investigate the persistence of underlying lymphatic defects and biological abnormalities.
Summary:
- Six children diagnosed with PIL (mean age 17 months) were followed for 11 years.
- A strict low-fat diet with medium-chain triglycerides and vitamin supplementation resolved symptoms in all patients.
- Despite symptom resolution, persistent chyle leakage was observed in most; dietary relaxation led to severe relapses in two patients, while three on long-term strict diets remained asymptomatic.
Impact:
- The study underscores that primary intestinal lymphangiectasia often involves persistent lymphatic dysfunction and biological abnormalities, even in asymptomatic individuals.
- Lifelong dietary management is indicated for most patients due to the risk of severe clinical relapses upon dietary liberalization.
- Findings emphasize the critical role of sustained dietary intervention in preventing complications and ensuring long-term well-being for pediatric PIL patients.
Unlabelled:
Primary intestinal lymphangiectasia induce symptoms of protein-losing gastroenteropathy. Only very few studies evaluate the long term follow up of such patients. We reviewed six children diagnosed at 17 +/- 12 months and followed for 11 +/- 4.9 years.
Case Reports:
As soon as the diagnosis was made the patients were submitted to a strict low fat diet with added medium chain triglycerides and intermittent liposoluble vitamins perfusions. The diet allowed the disappearance of symptoms for all the patients but laboratory findings indicated continuing chyle leak for most of the children. Only one child who had normal biological parameters tolerates a normal diet since four years. Relaxation of the diet by two patients who had moderate hypoalbuminemia and lymphopenia led to severe clinical relapses 14 and 17 years after the diagnosis period with therapeutic difficulties. Three patients with long term strict low fat diet remain asymptomatic.
Conclusion:
In most asymptomatic patients, the underlying lymphatic defect remains with permanent biological abnormalities. Clinical relapses may be severe and difficult to treat; thus the need for dietary treatment appears to be permanent.