Background. Congenital intestinal lymphangiectasia is a disease which leads
to protein losing enteropathy. Tortous, dilated lymphatic vessels in the
intestinal wall and mesenterium are typical features of the disease.
Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema
and effusions. Specific diet and medication are required for disease
control. Case report. A 19-year old male patient was hospitalized due to
diarrhea, abdominal swelling, weariness and fatigue. Physical examination
revealed growth impairment, ascites, and lymphedema of the right hand and
forearm. Laboratory assessment indicated iron deficiency anaemia,
lymphopenia, malabsorption, inflammatory syndrome, and urinary infection.
Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic
vessels in the small intestine. The diagnosis was confirmed by intestinal
biopsy. The patient was put on high-protein diet containing medium-chain
fatty acids, somatotropin and suportive therapy. Conclusion. Congenital
intestinal lymphangiectasia is a rare disease, usually diagnosed in
childhood. Early recognition of the disease and adequate treatment can
prevent development of various complications.
S3026 Extensive Intestinal Lymphangiectasia Results in Hemorrhagic Duodeno-Jejunitis
