Introduction: Klippel-Trenaunay Syndrome (KTS) is a rare congenital disease which affects 1 out of 100,000 people worldwide. It usually presents with a typical triad of asymmetric limb hypertrophy, cutaneous capillary malformation and lower extremity venous varicosities. To describe the salient imaging features of the disease, we reviewed a case of a 4 year old patient who was diagnosed with KTS and was referred to our institution for pre-operative imaging work-up. The patient presented with asymmetric limb hypertrophy, involving the left upper extremity and right lower extremity with several cutaneous lesions predominantly involving the affected limbs.
Methods: A contrast-enhanced computed tomography (CT) scan of upper and lower extremities was done at our institution.
Results: The scan revealed asymmetric limb soft tissue hypertrophy and several varicosities in the affected limb. Dilated venous structures in the right foot, leg and thigh are noted, predominantly draining into a persistent sciatic vein, a vascular feature highly associated with KTS.
Conclusion: The patient was subsequently referred back for surgical management, however, the complex venous varicosities of the patient's affected limbs would not permit surgery, hence medical management was advised. In complex congenital disease such as KTS, contrast-enhanced CT scan is an exceptional imaging modality of choice especially for pre-operative planning. It is fast, easy to perform and has high spatial resolution for better evaluation of the salient features of the disease, especially the venous anomalies.