J Korean Pediatr Soc.  1999 Oct;42(10):1452-1458.

Follow up of a Case of Primary Intestinal Lymphangiectasia

• Ha SJ
• Lee SJ
• Koh DK

Abstract

Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or the peritoneal cavity. The mechanism of this lymphatic loss is believed to be due to a rupture of lymphatics across the mucosa with subsequent leakage of the lymph into the bowel lumen. Presentation of primary intestinal lymphangiectasia may occur any time throughout infancy and childhood. Prominent clinical features include protein-losing enteropathy, lymphocytopenia, hypogammaglobulinemia, edema, growth failure, and chronic diarrhea. The mainstay of treatment for intestinal lymphangiectasia is the use of a low-fat, high- protein, medium-chain triglycerides diet. The natural history of this rare disorder is not well known as only a few cases have been reported in the literature. We present herein the follow up of a case with primary intestinal lymphangiectasia who responded to octreotide therapy but did not respond to dietary or antiplasmin therapy.

Keyword

Primary intestinal lymphangiectasia; Protein-losing enteropathy; Octreotide; Antiplasmin

MeSH Terms

Agammaglobulinemia
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Diarrhea
Diet
Dilatation, Pathologic
Edema
Follow-Up Studies*
Lymphatic Abnormalities
Lymphocytes
Lymphopenia
Mucous Membrane
Natural History
Octreotide
Peritoneal Cavity
Protein-Losing Enteropathies
Rupture
Triglycerides
Octreotide
Triglycerides