Surgical management of giant retroperitoneal lymphangioma in a child

Retroperitoneal lymphatic malformations are extremely rare and comprise less than 1% of the abdominal lymphatic malformations.1 Three histological types of lymphangiomas are described: cystic, capillary and cavernous.2 Retroperitoneal lymphangioma is commonly of cystic type.3 Most cystic lymphangiomas in the body are present in the first 2 years of life, however lymphangiomas of the retroperitoneum are usually diagnosed in older children or adults.3 It can present as a soft, slowly growing and painless mass or it may be detected incidentally during the evaluation of an unrelated complaint. Ultrasound is a sensitive and often the initial tool for evaluation of cystic abdominal masses, particularly for identifying septations and floating debris.3 On CT, cystic lymphangiomas are well-defined, homogeneous, usually multicystic and may have mild enhancement of the septa or the wall after intravenous contrast administration.3 They may not be limited to a particular abdominal compartment and may displace intra-abdominal organs and vessels. Complete resection as the treatment of choice is recommended in all cases including asymptomatic cases due to risk of future complications like haemorrhage, infection, malignant transformation or compression of vital structures.5 Conservative methods, including aspiration, cyst enterostomy and peritoneal marsupialisation, are now rarely considered due to the high rate of recurrence.6 Surgically unresectable lesions can be treated with intracystic injection of a sclerosing agent, preferably OK-432.7 Patient’s perspective Patient’s father: I was told by my doctor that my son has a fluid-filled swelling in his belly which did not look like cancer but required removal by surgery.