Lymphatic malformation causing intractable chylorrhagia

Lymphatic malformation is a developmental error usually noticed at birth or in early childhood. Lesions of the upper leg and lower trunk are the most difficult to remove, because they are often the largest ones encountered and also because they tend to extend proximally into the retroperitoneal tissues. Chyle reflux, usually associated with lymphedema of the extremity, has not been reported to be caused by lymphatic malformation. We report a case of intrapelvic retroperitoneal lymphatic malformation with an extension of gluteal-thigh soft-tissue involvement causing intractable chylorrhagia. The tumor was subtotally excised, and the defect was closed by a distally based, peninsular latissimus dorsi myocutaneous flap. The flap served both as a filling material and as a "bridge" between the residual tumor, including abnormal lymphatics, and normal lymph flow.