Intracranial aneurysms in klippel-trenaunay/weber syndromes: case report.

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 x 2 x 2 cm), fusiform right proximal P1 (2 x 1.3 x 1.3 cm), fusiform right distal P1 (2.8 x 2.7 x 2 cm), and saccular left distal posterior inferior cerebellar artery (2.5 x 2.5 x 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.