Prevalence and clinical features of lymphedema in patients with lymphangioleiomyomatosis.

[lymphangioleiomyomatosis]

Lymphangioleiomyomatosis (LAM ) is a rare cystic lung disease predominantly affecting young women . Some of these patients develop lymphedema of the lower extremities and buttocks ; however , neither the exact frequency of LAM -associated lymphedema nor the clinical features of such patients is well delineated .To document the frequency , features , and treatment of LAM -associated lymphedema .W e reviewed all medical records of patients listed in the Juntendo University LAM registry for the 30 years preceding August 2010 .Of 228 patients registered with a diagnosis of LAM , eight (3 .5 %) had LAM -associated lymphedema of the lower extremities . All were females with sporadic LAM , and their mean age when diagnosed was 32 .5 years (range 23 -44 ). Lymphedema of the lower extremities was the chief or a prominent presenting feature in five of these LAM patients . CT scans showed that all eight patients had enlarged lymph nodes (lymphangioleiomyomas ) in the retroperitoneum and /or pelvic cavity . Yet , cystic destruction of the lungs was mild in four patients , moderate in two and severe only in two . Seven of these patients were treated by administering a fat-restricted diet and complex decongestive physiotherapy , and four received a gonadotropin-releasing hormone analog . With this combined protocol , all eight patients benefitted from complete relief or good control of the lymphedema .Lymphedema is a rare complication of LAM and may be associated with axial lymphatic involvement or dysfunction rather than severe cystic lung destruction . The combined multimodal treatments used here effectively resolved or controlled LAM -associated lymphedema .