Özet:

Intestinal lymphangiectasia is an important cause of protein losing enteropathy in children, resulting in severe oedema and ascite. It occurs primarily due to congenital obstruction defect of lymphatics or secondary to diseases like congestive heart failure, retroperitoneal fibrosis, pancreatitis, tumoral infiltration, or Behcet's disease. Because of the loss of chylous fluid into the gut lumen, oedema and diarrhea are the leading clinical symptoms. Immun deficiency and lymphopenia, are the major causes of morbidity. Treatment modalities depend on underlying disease in secondary intestinal lymphangiectasia while replacement therapies and octreotid are choosen in primary cases. Dietary modifications are the first treatment of choices in all forms of the disease. We present a 5 year-old boy who was attended with swelling in his right leg, diagnosed as primary lymphangiectasia on further investigation and treated with partial jejunectomy as the condition was refractory to all standard medical therapies. Macroscopically, the jejenum segment was yellowish in color and the mucosa was covered by chili. Both mucosa and submucosa showed cystic lesions measuring 0.1-0.2 cm in diameter. In microscopic examination, the ectatic lymph vessels were also observed in serosa and lymph nodes. Postoperatively, his leg swelling was resolved, but symptoms due to protein loss were recurred in a years time. The case was presented to share our surgical experience in a case of primary intestinal lymphangiectasia along with the demonstrative macroscopic and microscopic findings in resected specimen.

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