Intracranial Aneurysms in Klippel-Trenaunay/Weber Syndromes

Abstract

Abstract OBJECTIVE We present a comprehensive review of intracranial aneurysms in Klippel-Trenaunay and Klippel-Trenaunay-Weber syndromes (KTS/KTWS), and examine factors influencing the risks of surgery vs conservative management. CLINICAL PRESENTATION A 58-year-old physician with KTS affecting the right extremities presented with left hemispheric cerebellar stroke and was discovered to harbor four intracranial aneurysms of the posterior circulation: fusiform mid and distal BA (2.6 × 2 × 2 cm), fusiform right proximal P1 (2 × 1.3 × 1.3 cm), fusiform right distal P1 (2.8 × 2.7 × 2 cm), and saccular left distal posterior inferior cerebellar artery (2.5 × 2.5 × 2.5 cm). Ten years later he had an infarct in the paramedian distribution of the basilar artery and a right internal capsule stroke. Two months later, he developed hydrocephalus, ultimately presenting in status epilepticus 4 months later secondary to ongoing aneurysm expansion and mass effect. INTERVENTION Systemic anticoagulation for acute thrombosis with possible distal arterioarterial embolization from giant P1 aneurysms. Ventriculoperitoneal shunting for hydrocephalus. The patient died within 9 days after admission and 10 years after the initial discovery of aneurysms. CONCLUSION Strict control of modifiable risk factors compromising vascular integrity and periodic neuroimaging are warranted in KTS/KTWS patients. KTS/KTWS patients are hypercoagulable, and may be predisposed to aneurysm thrombosis with increased risk for distal arterial microembolization. Stroke-related morbidity secondary to distal arterioarterial aneurysm thrombus embolization and acute aneurysm thrombosis may be decreased with systemic anticoagulation in this patient population. KTS/KTWS patients have significantly higher rates of DVT and PE than the general population, and should be classified in the high-risk category for venous thromboembolism prophylaxis. Both endovascular and open cerebrovascular techniques have been used successfully in KTS/KTWS patients with intracranial aneurysms.