Learn About Milroy Disease
Milroy disease is a condition that affects the normal function of the lymphatic system. The lymphatic system produces and transports fluids and immune cells throughout the body. Impaired transport with accumulation of lymph fluid can cause swelling (lymphedema). Individuals with Milroy disease typically have lymphedema in their lower legs and feet at birth or develop it in infancy. The lymphedema typically occurs on both sides of the body and may worsen over time.
Mutations in the FLT4 gene cause some cases of Milroy disease. The FLT4 gene provides instructions for producing a protein called vascular endothelial growth factor receptor 3 (VEGFR-3), which regulates the development and maintenance of the lymphatic system. Mutations in the FLT4 gene interfere with the growth, movement, and survival of cells that line the lymphatic vessels (lymphatic endothelial cells). These mutations lead to the development of small or absent lymphatic vessels. If lymph fluid is not properly transported, it builds up in the body's tissues and causes lymphedema. It is not known how mutations in the FLT4 gene lead to the other features of this disorder.
Milroy disease is a rare disorder; its incidence is unknown.
Milroy disease is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In many cases, an affected person inherits the mutation from one affected parent. Other cases may result from new mutations in the FLT4 gene. These cases occur in people with no history of the disorder in their family. About 10 percent to 15 percent of people with a mutation in the FLT4 gene do not develop the features of Milroy disease.
Texas Health Heart, Lung And Vascular Surgical Specialists
Raul Ortega, M.D., is a vascular surgeon who specializes in carotid disease, aortic disease, vein disease, adult cardiac surgery, minimally invasive thoracic surgery and lung cancer screening program. He is fellowship-trained in minimally-invasive techniques for the treatment of arterial and venous disease.Originally from McAllen, Texas, Ortega has dedicated his life to studying vascular disorders and learning how to both prevent and treat them. He attended Baylor University and obtained his M.D. degree at the University of Texas Medical Branch in Galveston. Upon finishing in Galveston, he spent the next eight years in the Northeast completing his surgical training.It was during this time that Ortega was able to finish his vascular fellowship under the auspices of the renowned Dr. H. Dardik at Mt. Sinai-Englewood program in Englewood, N.J. Dardik is widely accepted as a leader in the practice of limb salvage surgery, and in 2002 the Society for Clinical Vascular Surgery conferred a Lifetime Achievement Award to Dardik in recognition of his contributions to both the society and vascular surgery.It didn't take long for Ortega and his family to realize they missed the friendly people and sense of community in Texas, and in 2010 they relocated and he established his clinic in Denton. Ortega is happy to be involved in the ground-breaking medical field of vascular surgery because he sees the wide array of applications it provides to people in need: it can potentially help save lives by aortic surgery, save limbs through leg and arm revascularization, prevent strokes using carotid surgery, help treat vein disease with various varicose vein treatments, and help people on dialysis by creating arteriovenous fistulas.In his spare time, Ortega enjoys playing tennis and spending time with his wife and three children. Dr. Ortega is rated as a Distinguished provider by MediFind in the treatment of Milroy Disease. His top areas of expertise are Aagenaes Syndrome, Milroy Disease, Lymphedema-Distichiasis Syndrome, Carotid Artery Surgery, and Endoscopy.
Takumi Yamamoto practices in Tokyo, Japan. Mr. Yamamoto is rated as an Elite expert by MediFind in the treatment of Milroy Disease. His top areas of expertise are Milroy Disease, Lymphedema, Breast Cancer, Mastectomy, and Lymphadenectomy.
Hisako Hara practices in Tokyo, Japan. Ms. Hara is rated as an Elite expert by MediFind in the treatment of Milroy Disease. Her top areas of expertise are Lymphedema, Milroy Disease, Lymphangiectasis, Hysterectomy, and Pancreaticoduodenectomy.
Summary: Lymphedema is a debilitating disorder that severely impairs the quality of life of the patients and requires life-long attention. Treatment for lymphatic dysfunction remains largely symptomatic, without real cure. According to the International Society of Lymphology, lymphedema has to be treated with Decongestive Lymphatic Therapy. Research in the lab of Angiogenesis and Vascular Metabolism (PCA l...
Summary: This study is being done to see the long-term results for patients who choose to have surgery for lymphedema (with the vascularized lymph node transfer), compared to patients who choose not to have surgery.
Published Date: April 01, 2013
Published By: National Institutes of Health