Lymphedema in Prader-Willi syndrome.
A 20-year-old woman with Prader-Willi syndrome presented with heaviness and swelling in the lower legs and feet, which had developed after a fall. Lymphoscintigraphy showed a disturbed lymphatic drainage pattern in both lower extremities. Based on the clinical findings and the results of lymphoscintigraphic examination we made the diagnosis of lymphedema. The patient was successfully treated with manual lymphatic drainage in combination with multilayer compressive bandaging. After edema reduction, elastic compressive stockings were fit. To the best of our knowledge, this is the first report on primary lymphedema in a patient with Prader-Willi syndrome, an association that is probably often missed.
Heitink
Martijn V
MV
Department of Dermatology, Maastricht University Center for Molecular Dermatology, GROW-School for Oncology and Developmental Biology, and Governor Kremers Center, University Medical Center Maastricht, The Netherlands. mv.heitink@mumc.nl
Sinnema
Margje
M
van Steensel
Maurice A M
MA
Schrander-Stumpel
Connie T R M
CT
Frank
Jorge
J
Curfs
Leopold M G
LM
D000328:Adult; D004322:Drainage; D005260:Female; D006801:Humans; D007866:Leg; D008209:Lymphedema / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D011218:Prader-Willi Syndrome / Q000150:complications*; D053828:Stockings, Compression