Case 1
A 61-year-old male patient presented with non-healing ulcer in the right foot with varicosities in the right lower limb, which was present since childhood. He was being managed conservatively for the varices. Physical examination revealed varicose veins in the right thigh and leg and there was overall atrophy of the involved limb (Fig. 1). The patient had undergone a radiograph of the right lower limb which showed shortening of the right femur with osteonecrosis of the femoral head, shallow dysplastic acetabula, and coxa vara deformity, which concurred with the previous history of hip tuberculosis elicited by the patient (Fig. 2a). Surgical treatment of the varicose vein was planned and the patient was referred for a preoperative color Doppler evaluation study of varicose veins. Doppler study revealed extensive varicosities involving the right great saphenous vein and dilated lesser saphenous veins showing venous incompetance. No intersaphenous anastomoses were identified. Scanning of the deep venous system showed a complete absence of right external iliac, common and superficial femoral, profunda femoris and popliteal veins. Suspecting a congenital agenesis of deep venous system, a CT venography was performed using a multidetector row 64-slice CT scanner (light speed, GE medical systems, Milwaukee, WI, USA) which confirmed the Doppler findings of complete absence of deep venous system of the right lower limb in addition to atrophic right lower limb, extensive varicosities, (Fig. 2). The patient was managed conservatively as the absence of the deep venous system precluded any active surgical or radiological intervention to ablate the superficial venous varicosities.
Case 2
A 23-year-old woman presented with hypertrophied left lower limb with multiple varicosities in the affected limb present since childhood. On physical examination, there was left lower limb hypertrophy with prominent tortuous vascular channels along the medial and lateral aspect of the lower limb. No evidence of any skin discoloration or ulceration was noted. Plain radiography showed osseous and soft tissue hypertrophy of the left lower limb. Diffuse cortical thickening of the femur, tibia, and fibula was seen. No bony deformity was observed. The patient was referred for color Doppler evaluation which revealed multiple superficial and intramuscular varicosities involving the left thigh and leg. The varicosities showed both usual (medial aspect) and unusual (lateral) distribution. Both the saphenofemoral and saphenopopliteal junctions were incompetent and showed reflux times of 2–3 s and 4–6 s, respectively. The deep venous system did not show any evidence of thrombosis or reflux. Multiple dilated perforator vessels were identified in the thigh and calf. No intersaphenous anastmoses were seen. A suspicion of KTS was raised on color Doppler based on the unusual distribution of the varicosities and intramuscular extension, and CT venography was performed.
CT angiography revealed multiple varicosities along the superficial venous system and abnormal vascular channels in intramuscular and superficial planes showing contrast filling on delayed scans suggestive of venous malformations (Fig. 3). In addition, the malformed vessels were seen showing an intraosseous extension into distal femur, patella, and tibia. Also, there was evidence of soft tissue, muscular and osseous hypertrophy of the left lower limb. The patient was managed by US-guided sclerotherapy of abnormal vascular channels and is on follow-up.
Case 3
A 14-year-old male presented with hypertrophy of the right lower limb and multiple venous prominences in the lateral aspect of the limb and overlying cutaneous lesions. Physical examination revealed an increased girth and length of the right lower limb and multiple varicosities in the anterolateral aspect of the right thigh and leg. Multiple macular lesions were noted along the anterior and lateral aspects of the right leg (Fig. 4). Color Doppler revealed multiple superficial and intramuscular varicosities in the right thigh and leg in the anterior, posterior, and lateral compartments. An abnormal lateral marginal vein and multiple dilated perforator channels were identified in the calf and thigh with evidence of venous reflux. The saphenofemoral junction was competent while saphenopopliteal junctions showed Doppler evidence of venous insufficiency with reflux time of 3 s. The deep venous system did not show any evidence of thrombosis or venous insufficiency. No intersaphenous anastomotic channels were identified. Underlying the cutaneous macular lesions, ill-defined subcutaneous lesions having a tuft of small vascular channels and minimal soft tissue components were seen. The vessels showed slow flow on spectral Doppler study, and the lesions were identified as capillary malformations. On CT angiography, there was evidence of multiple dilated vascular channels in subcutaneous and intramuscular plane (involving hamstrings and peroneal muscles) which showed early filling in the arterial phase with slow washout in delayed phase images (Fig. 5). Multiple subcutaneous soft tissue density lesions with surrounding dilated vessels were seen in areas corresponding to the cutaneous malformations in the patient. There was osseous and soft tissue hypertrophy of the right lower limb. MRI was performed to look for any other vascular malformation and assess soft tissue extension of the varicosities (Fig. 5). MRI confirmed the CT findings and also demonstrated the lateral marginal vein of Servelle (Fig. 5d) running along the lateral aspect of the leg.
The patient has been planned for endovenous laser ablation of the superficial varicosities. For compensating the limb length discrepancy, the patient was advised to use customized shoes. He has been counseled regarding the use of stockings and avoiding any risk factor for recurrence of varices.