What is the treatment for Klippel-Trenaunay-Weber Syndrome?
Q: I am 10 years old and diagnosed with Klippel-Trenaunay-Weber Syndrome. The doctor who was treating me is no more. I have heard that Mayo Clinic, Rochester, Minnesota, USA, is the best hospital for this case? Is this true? In India, where is treatment possible?
A:There are a number of rare congenital defects affecting the skin, blood vessels, and underlying soft tissue one of, which is the Klippel-Trenaunay syndrome. This is characterised by a triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophy involving a limb. When there is associated arteriovenous malformation of the affected extremity, the condition is referred to as Klippel-Trenaunay-Weber syndrome. The management of this condition is by both medical and surgical interventions. Conservative treatment involves symptomatic relief with use of compression stockings that decrease oedema, act as a barrier for minor trauma, and reduce venous insufficiency. Haemangiomas can be treated by laser surgery. Surgical treatment of varicosities and venous malformations remains controversial. If there is a leg length discrepancy of more than 2.0 cm, epiphysiodesis is done and hypertrophied digits with severe deformity and infection may require amputation. Some of the Genetic Centres in India are: . Centre for Genetic Disorders, Department of Human Genetics, Guru Nanak Dev University, Amritsar, Punjab. . Department of Human Genetics and Anatomy, St. Johns Medical College, Bangalore. . Department of Paediatrics, K.E.M. Hospital, Mumbai. . ICMR Immunohaematology Centre, KEM Hospital. Mumbai. . ICMR Genetic Research Centre, Indian Council of Medical Research, Wadia Hospital for Children, Mumbai. . Department of Genetics, Ramakrishna Mission Hospital, Calcutta. . Departments of Paediatrics and Haematology, Postgraduate Institute of Medical Education and Research, Chandigarh. . Genetics Unit, Department of Paediatrics, All India Institute of Medical Sciences, New Delhi. . Department of Genetic Medicine, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi. . Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow. . Department of Genetics, Institute of Child Health and Institute of Obstetrics and Gynaecology, Chennai. . Genetics Centre, Department of Paediatrics, B.J. Medical College, Pune. This facility also exists at Department of Paediatric metabolic disorder, MGM Hospital, Navi Mumbai, and Jaslok Hospital, Mumbai, You can also access the following site for more information: http://www.ornl.gov/sci/techresources/Human_Genome/medicine/assist.shtml