1/3. Klippel-Trenaunay syndrome.The association of three physical findings including capillary malformation, varicosities, and hypertrophy of bony and soft tissues corresponds to Klippel-Trenaunay syndrome. This triad of findings, described by the two French physicians Klippel and Trenaunay in 1900, differs from Parkes-Weber syndrome, in that Klippel-Trenaunay syndrome does not incorporate significant hemodynamic arteriovenous fistulas. Generally, management of this disease process should be individualized. Surgery should be considered in cases where skin ulcerations lead to persisting and recurrent bleeding, or where digital deformities lead to functional disabilities or where significant limb overgrowth leads to both functional and psychological impairment. Persistent hematochezia, hematuria, and vaginal and esophageal bleeding are considered indications for surgical intervention. Recurrent attacks of thrombophlebitis and cellulitis are treated medically with antiinflammatory agents and antibiotics. Otherwise, management of this syndrome is generally conservative, consisting of psychological encouragement, reassurance, and the continued use of graduated compressive stockings for varicosities and intermittent pneumatic compression pumps for lymphatic edema.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/3. klippel-trenaunay-weber syndrome: appearance in utero.We were able to detect hemihypertrophy of a fetus in a routine prenatal ultrasound examination. The presence of hemangiomas and varicose veins in the hypertrophied left lower extremity at birth enabled us to diagnose the klippel-trenaunay-weber syndrome. The possibility of a congenital arteriovenous fistula causing the constellation of findings was ruled out by a nuclear flow study and a thorough physical examination. A computerized tomographic scan revealed that the hemangiomas were superficial in the hypertrophied left lower extremity and did not interrupt the deep muscle bundles.- - - - - - - - - - ranking = 14.394604100143keywords = physical examination, physical (Clic here for more details about this article) |
3/3. Klippel-Trenaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trenaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trenaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |