doiSerbia

Congenital intestinal lymphangiectasia

Popović Dušan Đ. (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Špuran Milan (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Alempijević Tamara (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Krstić Miodrag (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Đuranović Srđan (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Kovačević Nada (Klinički centar Srbije, Klinika za gastroenterologiju, Beograd)
Damjanović Svetozar (Klinički centar Srbije, Institut za endokrinologiju, dijabetes i bolesti metabolizma, Beograd)
Micev Marjan (Klinički centar Srbije, Klinika za abdominalnu hirurgiju, Služba za patohistologiju, Beograd)

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.

Keywords: ileal diseases, lymphangiectasis, intestinal, congenital abnormalities, diagnosis, differential, drug therapy