Associates' Presentations
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Klippel-Trenaunay (KT) syndrome with multiple pulmonary emboli: A challenging presentation.

C. Namasivayam, M.D. (Associate), L. Remeika, M.D., Marshfield Clinic, Marshfield, WI.

KT syndrome is a rare congenital angiodysplastic syndrome classically consisting of the triad of vascular nevus, varicose veins and bony and soft tissue hypertrophy. Other associated manifestations may include lymphangiomatous and deep vein anomalies, visceral and facial hemangiomas.

A 24 year old gentleman with KT syndrome presented with the acute onset of dyspnea, hypoxemia and hypotension. Initial resuscitation was started with oxygen and boluses of IV fluids in the ER. He remained symptomatic while being evaluated in the intensive care unit and a VQ scan revealed multiple large pulmonary emboli bilaterally. IV heparin, which had been started empirically earlier due to the high index of suspicion for PE, along with IV fluids and oxygen supplementation, stabilized the patient.

The patient had previous history of recurrent GI bleeding secondary to multiple visceral hemangiomas. Primary caval filter placement was contemplated to avoid long term oral anticoagulation. Radionuclide angiography and a magnetic resonance angiogram of the abdomen revealed the infrarenal IVC to be very large measuring 40-45 mm. Currently available IVC filters do not fit this "megacava". In addition large collaterals from the common femoral veins were found to be tracking cephalad along the abdominal wall that would allow lower extremity thrombi to reach the lungs. These collaterals would make both surgical ligation and filter placement futile. Hence, long term management options were limited to compression stockings and oral anticoagulation.

In conclusion, this was a very unusual and therapeutically challenging presentation of KT syndrome. There have been only 5 prior English language case reports of pulmonary emboli in patients with this rare syndrome. Lack of any large experience with thromboembolic phenomena in these patients makes any firm treatment recommendations impossible. However, warfarin anticoagulation with compression stocking therapy seems to be a logical approach. Caval filters should be considered if indicated and anatomically feasible.

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