Cystic lymphangioma is a rare tumour (6% of benign tumours in children), frequently situated in the cervical or axillary region and exceptionally intraabdominal. It arises due to a congenital defect in the connection of the primary lymphatic channels with the central collecting system. It presents clinically either in the form of a pseudo-appendix or pseudo-ascites syndrome or in the form of an abdominal tumour with or without compression of adjacent structures. It can now be easily diagnosed by ultrasonography. Treatment is well defined and consists of surgical resection of the isolated lymphangioma or associated with the adjacent intestine, depending on the site of the tumour. It has a good prognosis.
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