Hemifacial spasm and jugular foramen syndrome caused by diametrically opposite aneurysms on the vertebral artery

Abstract

Vertebral artery (VA) aneurysms commonly occur on a fusiform segment of the artery and have unusual appearances and characteristics [1]. They commonly present with symptoms of cranial nerve compression secondary to related dolichoectasia, mass effect or subarachnoid hemorrhage. In the setting of a VA aneurysm, hemifacial spasm occurs when the VA is ectatic or fusiform, while individual lower cranial nerve palsies have been reported in saccular and fusiform cases [1]. A simultaneous occurrence of these two clinical syndromes related to an aneurysmal VA has not been described. A 50-year-old lady presented with left-sided hemifacial spasm of 2-year duration and nasal regurgitation and altered phonation of 1-year duration. Both the symptoms were insidious in onset and were progressive. There was no history of facial pain or numbness, tinnitus or vertigo. Her neurological deficits included left sided IX, X nerve palsies. Examination of the other cranial nerves was normal. MRI of the brain showed a mildly ectatic vertebro-basilar artery (VBA) on the left side with compression on the VII–VIII nerve complex (Fig. 1a). Digital subtraction angiogram (DSA) revealed an ectatic left VA with a ventrolaterally pointing, distal 12 9 8 9 6 mm sized saccular aneurysm and a small blister aneurysm (3 9 3 mm) directed dorsomedially (Fig. 1b, c). At surgery via a far lateral craniectomy, the saccular aneurysm directed toward the jugular foramen was found arising proximal to origin of the posterior inferior cerebellar artery (PICA) which was draped on the aneurysm (Fig. 2a). It was clipped with a 10-mm angled ring clip, with the ring enclosing the VA (Fig. 2b). The clipped aneurysm was then opened and decompressed. The dorsally directed blister aneurysm compressing the facial nerve at its exit from the pons (Fig. 2b) was wrapped with muscle and dura. Teflon was interposed between the nerve and the ectatic VA segment. The risk of rupturing the nearby blister aneurysm and narrowing the ectatic VA precluded reconstruction of the VA with clips. Postoperatively, the patient was relieved of her hemifacial spasm, while her lower cranial nerve palsy remained status quo. An aneurysmal etiology is noted in 0.3–0.6 % of all cases of hemifacial spasm. The postulated pathogenesis is related to the compression of the root entry zone (REZ) of the facial nerve by the pulsations of the aneurysm. In 7.5–21 % of the cases an ectatic VBA is the causative factor [2]. A Pubmed search revealed 12 cases of VA aneurysms presenting with hemifacial spasm. One report describes Foville’s syndrome secondary to a dissecting VA aneurysm [3]. Our first-of-its-kind report demonstrates the simultaneous occurrence of hemifacial spasm and jugular foramen syndrome due to the mass effect of two aneurysms on an ectatic VA loop. Microsurgical clipping and debulking are performed for VA aneurysms causing brainstem compression and neurological deficits [4], while endovascular coiling is an optional in other cases [5]. In our case, the complex nature of the contiguous aneurysms on the VA segment and the need for decompression precluded endovascular intervention. S. V. Furtado (&) S. Thakar N. A. Saikiran A. S. Hegde Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences (SSSIHMS), EPIP Area, Whitefield, Bangalore 560066, Karnataka, India e-mail: sunilvf@gmail.com