The role of radiology in the planning management of Klippel Trenaunay Syndrome (KTS)

Purpose: KTS represents a challenging problem in both diagnosis and management. This study presents an example of a radiological protocol which may prove extremely beneficial in the diagnostic and surgical planning strategy. Study design: This is a prospective study involving 20 patients that had been clinically diagnosed as having KT syndrome. The proposed radiology imaging protocol involved scanning by Skeletal survey, CT, MRI, and MRA/MRV as basic routine studies for all patients as well as abdominal US and CT for associated visceral involvements. Data analysis classified patients into groups undergoing orthopedic procedures for limb shortening, surgical management for varicosities and others who will be under conservative measures. Results: Twenty patients had met the three criteria of KT syndrome. CT scanogram revealed leg length discrepancy (LLD) in all patients. MRI and MRV results revealed different varieties of cavernous haemangioma and varicose veins, a form of surgical intervention was resorted to in 12 patients. Conclusion: Coronal T2, STIR, postcontrast fat suppression sequences and MRA can guide the plan of management in our series by providing exact delineation of extent and accurate characterization of vascular malformations in the extremities, orthopedic and surgical interventions aim to correct deformities, decrease morbidity and rehabilitate these patients. © 2011 Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier B.V. All rights reserved.