Short neck as a cause of stroke? Bilateral vertebral artery dissection in a patient with Klippel-Feil Syndrome.
Case Report
Vertebral dissections are a recognized cause of stroke in young people. Its association with Klippel-Feil Syndrome (KFS) is unusual. We describe the case of a male with simultaneous bilateral vertebral artery dissection with SKF and review the related literature. A 30-year-old male, with no relevant medical history, was transferred to the hospital with code stroke due to dizziness, dysarthria and clumsiness of the right limbs of sudden onset. Eight days prior, he had experienced intense right-sided neck pain after making a sudden neck turn while playing football, which persisted over the days. Phenotypically, he had a noticeable short neck. A cranial CT scan showed an acute right cerebellar infarction and an AngioCT scan showed bilateral vertebral artery dissection. An MRI revealed vertebral artery wall signal hyperintensity, compatible with intramural hematoma. The findings were confirmed by arteriography. Fusion of the cervical vertebrae C2-C3 compatible with SKF was observed at the same level where both dissections were observed. The patient was discharged asymptomatic with antiplatelet treatment. He received single antiplatelet therapy with 100 mg aspirin, remaining stable without recurrences. A follow-up MR angiogram showed resolution of the lesions after 3 months. SKF is a rare malformation consisting of a triad of fusion of cervical vertebrae, short neck and low hairline implantation. Its association with stroke is scarce and there are few descriptions of cases, none with bilateral dissection.
