• Dusan D j Popović, Milan Spuran, Tamara Alempijević, Miodrag Krstić, Srdjan Djuranović, Nada Kovacević, Svetozar Damnjanović, and Marjan Micev.
• Klinicki centar Srbije, Sluzba za patohistologiju, Beograd, Srbija. dr.popovic@sezampro.rs
• Vojnosanit Pregl. 2020 Nov 3; 68 (3): 270-3.

BackgroundCongenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, 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 ReportA 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 supportive therapy.ConclusionCongenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

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