Hemifacial spasm associated with Marfan's syndrome: A case report

The authors describe a case of hemifacial spasm associated with Marfan's syndrome. A 24-year-old-woman suffered from left hemifacial spasm for 6 years. She had undergone surgical treatments for bilateral ectopia lentis and kyphoscoliosis in the second decade of life. She also had unusually long and slim limbs with arachnodactyly. Three-dimensional CT angiography revealed bilateral tortuous and elongated vertebral arteries. Microvascular decompression was performed following a left lateral suboccipital craniotomy. The root exit zone of the left seventh nerve was directly compressed by the proximal segment of the anterior inferior cerebellar artery (AICA) and the left vertebral artery. These arteries were decompressed with pieces of Teflon cotton. The patient's symptoms completely resolved following surgery. We review cerebrovascular disorders in Marfan's syndrome and discuss the pathogenesis and possible mechanisms of vascular compression in these patients.