Microvascular decompression for a unique case of glossopharyngeal neuralgia with provokable symptomatic bradycardia: 2-Dimensional operative video.

BACKGROUND: Glossopharyngeal neuralgia is a rare neurovascular compression syndrome that can lead to paroxysmal craniofacial pain and sometimes cardiovascular symptoms.[1,2] The characteristic pathology involves a vessel (commonly a branch/loop of PICA) compressing the nerve at the root entry/exit zone at the brainstem.[1] Microvascular decompression is a commonly used treatment approach for patients that have failed conservative measures.[2]. CASE DESCRIPTION: A 72-year-old male presented to the ED following four episodes of syncope. The patient had a multi-year history of right-sided burning/stabbing pain involving the submandibular area and posterior throat. His syncope was related to symptomatic bradycardia that would occur during episodes of pain. His pain was exacerbated by speaking and swallowing and could be triggered by placing his finger in the right external auditory meatus. Interestingly, this maneuver would also trigger his bradycardia. The patient had failed previous pharmacotherapy, and a pacemaker had been placed to protect him from periods of hypotension. MRI/MRA of the brain and cervical spine were unremarkable. Due to his profoundly symptomatic status, the patient was offered a right retrosigmoid craniotomy for microvascular decompression of the right glossopharyngeal nerve. The patient had complete resolution of his pain and bradycardia immediately post-operatively. He was discharged on the second postoperative day and his pacemaker was ultimately removed. The patient continues to be pain free and off medication. CONCLUSION: Here we present a video case report of microvascular decompression with favorable outcome for an interesting presentation of glossopharyngeal neuralgia. The patient gave informed consent for surgery and video recording.

Cavernous Malformations in and Around the Third Ventricle: Indications, Approaches, and Outcomes.

BACKGROUND: Cavernous malformations in structures in and around the third ventricle are a challenging conceptual and surgical problem. No consensus exists on the ideal approach to such lesions. OBJECTIVE: To perform a retrospective review of our institutional database to identify and evaluate approaches used to treat cavernous malformations located in and around the third ventricle. METHODS: Information was extracted regarding lesion size and location, extent of resection, time to last follow-up, surgical approach, presenting symptoms, preoperative and postoperative neurological status, and specific approach-related morbidity. RESULTS: All 39 neurosurgical operations (in 36 patients) were either an anterior interhemispheric (AIH) (44%, 17/39) or a supracerebellar infratentorial (SCIT) (56%, 22/39) approach. Gross-total resection was achieved in 23 of 39 procedures (59%), a near-total resection in 1 (3%), and subtotal resection in 15 (38%). For the 31 patients with at least 3 mo of follow-up, the mean modified Rankin Scale (mRS) score was 1.5. Of the 31 patients, 25 (81%) had an mRS score of 0 to 2, 4 had a mRS score of 3 (13%), and 1 each had a mRS score of 4 (3%) or 5 (3%). CONCLUSION: Most approaches to cavernous malformations in and around the third ventricle treated at our institution have been either an AIH or a SCIT approach. The AIH approach was used for lesions involving the lateral wall of the third ventricle or the midline third ventricular floor, whereas the SCIT approach was used for lesions extending from the third ventricle into the dorsolateral midbrain, with acceptable clinical results.

Benefits of Treating Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Retrospective Analysis of 14 Patients.

BACKGROUND: Arteriovenous malformations (AVMs) are a cardinal feature of hereditary hemorrhagic telangiectasia (HHT). However, whether to treat brain AVMs in patients with HHT remains questionable because of the possible risks. METHODS: We performed a retrospective study of patients with HHT who had been treated for brain AVMs at our institution from January 1, 2003, to December 31, 2016. An institutional database was queried for the phrases "hereditary hemorrhagic telangiectasia" and "HHT," and those patients who had been treated during the study period were identified. Data were extracted regarding presentation, AVM characteristics, treatment modality, and treatment outcomes. RESULTS: We identified 14 patients (10 males, 4 females) with HHT who had had AVMs (n = 27) from the institutional database. The mean age of the patients was 43 years (range, 2-64). Of the 27 brain AVMs, 13 were Spetzler-Martin grade I, 12 were grade II, and 2 were grade III; none were grade IV or V. Treatment was by microsurgery only (11 AVMs in 10 patients), embolization followed by microsurgery (2 AVMs in 2 patients), and radiosurgery only (12 AVMs in 2 patients). AVM obliteration was achieved in 100% of the patients. No new fixed neurologic deficits developed after treatment of unruptured HHT AVMs. CONCLUSIONS: The risk of treatment of brain AVMs in patients with HHT is quite low for appropriately selected patients with treatment individualized to radiosurgery, microsurgery, or a combination of embolization and microsurgery.

Microsurgical Treatment of Recurrent Conus Medullaris Arteriovenous Malformation: 2-Dimensional Operative Video.

Arteriovenous malformations (AVMs) involving the conus medullaris have a unique angioarchitecture due to their involvement of the arterial basket of the conus medullaris, which represents an arterial anastomotic network between the anterior spinal artery (ASA) and posterior spinal arteries (PSAs) at the level of the conus medullaris.1 These lesions consist of a combination of a true AVM nidus, which is usually extramedullary, and direct shunts between the ASA, PSAs, and the venous system. Patients may present with radiculopathy, myelopathy, or subarachnoid hemorrhage.2A 40-yr-old woman status post T11-L1 laminoplasty for resection of a ruptured conus AVM 6 yr prior presented with routine follow-up angiography suggestive of an arteriovenous fistula. She was counseled regarding treatment options including endovascular embolization and microsurgical ligation or resection, and she elected to proceed with surgical treatment. At the time of surgery, a recurrent AVM was noted. A 2-dimensional intraoperative video illustrates the microsurgical treatment of her recurrent conus AVM. The patient recovered well postoperatively. Spinal angiography demonstrated complete obliteration of the lesion. The patient experienced transient urinary retention that was self-limited but otherwise was without any new neurological deficit. Due to the retrospective nature of this report, informed consent was not required.Video used with permission from Barrow Neurological Institute, all rights reserved.

Carotid-Falciform Optic Neuropathy: Microsurgical Treatment.

BACKGROUND: Several recent reports have implicated vascular ectasia and vessel contact in dysfunction of the visual apparatus. A subset of patients with prechiasmatic visual deterioration have an ectatic internal carotid artery (ICA) that displaces and flattens the optic nerve (ON) rostrally as the ON exits the skull base. We describe a proposed pathophysiologic mechanism and a straightforward surgical technique for dealing with this problem. METHODS: Via an ipsilateral pterional craniotomy, the bony roof of the optic canal is removed. The falciform ligament is opened in parallel to the ON. Adhesions between the ICA and ON are then dissected, and a Teflon pledget is placed between the ICA and ON to complete the decompression. RESULTS: Patients both in the literature and in this series experienced an improvement in their vision postoperatively. CONCLUSIONS: We propose that 3 mechanisms contribute to this caroticofalciform optic neuropathy: 1) mass effect from ICA ectasia, 2) ON irritation from vessel pulsatility, and 3) indirect compression by the falciform ligament from above. This disease process can be treated safely using standard microsurgical techniques with excellent outcomes.

Cerebral vascular malformations in hereditary hemorrhagic telangiectasia.

OBJECT: Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder characterized by mucocutaneous telangiectasias, frequent nosebleeds, and visceral arteriovenous malformations (AVMs). Few reports have outlined the prevalence of the various cerebral vascular malformations found in patients with HHT. The authors set out to define the prevalence of cerebral vascular malformations in a population of HHT patients who underwent imaging with 3-T imaging (MRI/MR angiography [MRA]) of the brain. METHODS: A retrospective review of prospectively collected data was carried out using a database of 372 HHT patients who were seen and examined at the Georgia Regents University HHT Center and screened with 3-T MRI/MRA. Data were tabulated for numbers and types of vascular malformations in this population. RESULTS: Arteriovenous malformations were identified in 7.7%, developmental venous anomalies in 4.3%, and cerebral aneurysms in 2.4% of HHT patients. The HHT AVMs tended to be supratentorial, small, and cortical in this series, findings consistent with other recent studies in the literature. An arteriovenous fistula, cavernous malformation, and capillary telangiectasia were identified in 0.5%, 1%, and 1.9% of HHT patients, respectively. CONCLUSIONS: Few studies have investigated the prevalence of the various vascular malformations found in HHT patients screened with 3-T MRI/MRA of the brain. Hereditary hemorrhagic telangiectasia AVMs are more likely to be multiple and have a tendency toward small size and cortical location. As such, they are often treated using a single-modality therapy.

Teaching neuroimages: microvascular decompression of the optic nerve.

Vascular compression is a known cause of cranial nerve dysfunction in a variety of disease states including trigeminal neuralgia, hemifacial spasm, and hypoglossal neuralgia.(1,2.)

Bilateral transverse sinus stenosis causing intracranial hypertension.

Idiopathic intracranial hypertension (IIH) has been associated with transverse sinus stenosis in a number of cases. Some authors advocate for stent placement when transverse sinus stenosis is responsible for pseudotumor symptoms. A 19-year-old man with IIH, bilateral transverse sinus stenosis was apparent on a CT venography, with reconstitution of the lateral sinus. MRI was then performed, and transverse sinus stenosis was confirmed. An impressive network of dilated collateral veins became apparent on VENBOLD sequences. Treatment options in this case include cerebrospinal fluid (CSF) shunting, optic nerve fenestrations and transverse sinus stenting. A ventriculoperitoneal shunt was placed in this patient, with prompt resolution of pseudotumor symptoms.