ABSTRACT
Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by combined vascular malformations of capillary, venous, and lymphatic types usually observed during infancy or childhood. In this report, we describe two KTWS patients treated with radiotherapy after long-term conservative treatment or multiple surgical interventions. The first patient, a 15-year-old female, presented with vascular masses located on the right upper extremity. She was treated conservatively for 14 years, and amputation was offered at the age of 14 due to cardiac failure. A course of radiotherapy to a total dose of 40 Gy with conventional daily fractionation was administered as an alternative to the mutilating surgery, and prominent improvement both subjectively and objectively was detected 1 year after radiotherapy. The second patient, a 40-year-old male, suffered from huge vascular masses of both the trunk and lower extremities. Despite multiple surgical interventions and conservative treatment, disease progressed and neurological symptoms occurred due to compression of vertebral structures. He was administered a course of 30 Gy radiotherapy with 1.5 Gy daily fractions to the lower thoracic region and whole abdominal cavity. Six months after radiotherapy, subjective relief described by the patient but without any objective response was detected. Though considered as a benign disease, KTWS can cause life-threatening complications. The response of one of our patients is a hopeful finding for considering radiotherapy as an alternative treatment modality for these patients.
Subject(s)
Klippel-Trenaunay-Weber Syndrome/radiotherapy , Adolescent , Adult , Disease Progression , Dose Fractionation, Radiation , Female , Humans , Klippel-Trenaunay-Weber Syndrome/diagnosis , Magnetic Resonance Imaging , MaleABSTRACT
A 28-year old woman with extensive right-leg capillary malformation associated with limb hypertrophy and limb-length discrepancy consistent with Klippel-Trenaunay syndrome is presented. Treatment with the new long-pulsed-dye lasers may lead to improvement of the superficial hemangioma component of the affected areas.
Subject(s)
Klippel-Trenaunay-Weber Syndrome , Adult , Female , Humans , Klippel-Trenaunay-Weber Syndrome/pathology , Klippel-Trenaunay-Weber Syndrome/radiotherapy , Low-Level Light TherapySubject(s)
Anemia/etiology , Klippel-Trenaunay-Weber Syndrome/radiotherapy , Multiple Myeloma/etiology , Neoplasms, Radiation-Induced/etiology , Spinal Neoplasms/etiology , Adult , Anemia/drug therapy , Anemia/pathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Bone Marrow/pathology , Female , Humans , Immunoglobulin Light Chains/analysis , Immunoglobulin lambda-Chains/analysis , Klippel-Trenaunay-Weber Syndrome/pathology , Multiple Myeloma/drug therapy , Multiple Myeloma/pathology , Neoplasm Staging , Neoplasms, Radiation-Induced/drug therapy , Neoplasms, Radiation-Induced/pathology , Spinal Neoplasms/drug therapy , Spinal Neoplasms/pathologyABSTRACT
Under examination there were 12 patients aged from 5 till 14 years. An investigation of hemostatic potential of blood in the diseased extremity and peripheral blood was performed. A mosaic character of blood coagulative alterations in this category of patients was detected. Autotransfusion of UV-irradiated blood is a pathogenetically grounded and accessible method of correction of hemocoagulation and may be included in the complex treatment of patients with dysplasia of the profound veins of lower extremities.
Subject(s)
Arm/blood supply , Blood Transfusion, Autologous , Blood/radiation effects , Klippel-Trenaunay-Weber Syndrome/radiotherapy , Leg/blood supply , Ultraviolet Therapy , Adolescent , Blood Coagulation/physiology , Child , Child, Preschool , Disseminated Intravascular Coagulation/prevention & control , Humans , Klippel-Trenaunay-Weber Syndrome/blood , Klippel-Trenaunay-Weber Syndrome/surgery , Preoperative CareABSTRACT
A patient with intravascular coagulation caused by the Klippel-Trenaunay syndrome is presented; this is a rare phenomenon. The patient was treated with fast electrons. Sonography is a suitable method for determining the extension of the haemangiomas occurring in Klippel-Trenaunay's syndrome. This is important for therapy planning. With colour-coded Doppler sonography, arteriovenous fistulas can be ruled out, this allowing to distinguish the Klippel-Trenaunay syndrome from the Parkes-Weber syndrome.
Subject(s)
Angiomatosis/complications , Klippel-Trenaunay-Weber Syndrome/complications , Thrombocytopenia/complications , Ultrasonography/methods , Adult , Blood Flow Velocity , Humans , Klippel-Trenaunay-Weber Syndrome/diagnosis , Klippel-Trenaunay-Weber Syndrome/radiotherapy , Leg/blood supply , Male , Radiotherapy DosageABSTRACT
The triad of varicose veins, hypertrophy of soft tissue and bone, and cutaneous hemangiomas (The Klippel-Trenaunay syndrome) often results in major malformations. A review of eleven children treated for this condition showed that the overgrowth of the limb was a major problem in management. In three patients the altered hemodynamics resulted in cardiac complications. Venous stripping, excision of the arteriovenous fistulae, and radiation therapy should be avoided.
