[Effect of lymphedema treatment--the interim report of prospective study at multiple facilities].

We are in the process of analyzing the effect of complex physical therapy (CPT) for the patients of secondary lymphedema associated with cancer treatment. At this time, we present an interim report of the past 3 months started in March 2009. We classified the lymphedema stage into four stages based on the International Society of Lymphology criterion using the imaging diagnosis (US, CT, etc.) in addition to a conventional observation. We also classified the CPT treatment period into two in accordance with water retention in the subcutaneous adipose tissue of the limbs. CPT was performed by the specialist of lymphedema treatment. We used the change in volume of limbs and QOL evaluation by SF-36 and Skindex29 for analysis. On this report, the volume of limbs, which has lymphedema and an improvement on SF-36, did not change significantly. This may have been resulted from not having enough follow-up period and lack of few cases at each stage in the analysis. However, we confirmed that the general and emotion evaluation on Skindex29 showed a meaningful change. We are still planning to accumulate more cases and study them for further detailed analysis.

Prevalence and clinical features of lymphedema in patients with lymphangioleiomyomatosis.

BACKGROUND: 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. OBJECTIVES: To document the frequency, features, and treatment of LAM-associated lymphedema. METHODS: We reviewed all medical records of patients listed in the Juntendo University LAM registry for the 30 years preceding August 2010. RESULTS: 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. CONCLUSIONS: 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.