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William T. Couldwell and Clough Shelton

This video demonstrates a very useful trajectory to a pontine lesion. A 68-year-old man presented with tongue numbness and weakness. The approach used was a transtemporal presigmoid retrolabyrinthine approach to enable an orthogonal trajectory to the lateral pons. Following the transtemporal opening, the root entry zone of the trigeminal nerve and the root exit zone of the facial nerve are identified. The lateral pons is incised to access the tumor, which upon histological analysis was found to be a metastasis. Excellent visualization of the lateral pons is achieved. The opening, relevant anatomy, and closure are illustrated.

The video can be found here: http://youtu.be/vS5fCOY6vp8.

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Roukoz Chamoun, Joel MacDonald, Clough Shelton and William T. Couldwell

Surgical removal remains one of the key treatment modalities for vestibular schwannomas. A team approach between a neurotologist and a neurosurgeon offers the patient the expertise of both specialties and maximizes the chances for an optimal outcome. Vestibular schwannomas can typically be resected through 1 of 3 main surgical approaches: the translabyrinthine, the retrosigmoid, or the middle fossa approaches. In this report and videos, the authors describe and illustrate the indications and surgical techniques for the removal of these tumors.

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Michael L. Mumert, Tamer Altay, Clough Shelton, H. Ric Harnsberger and William T. Couldwell

Ganglion cysts arising from the temporomandibular joint are rare entities that often present with swelling and minimal to no pain in the preauricular region. To the authors' knowledge, a temporomandibular joint ganglion cyst occurring with acute facial nerve palsy and intracranial extension has never been reported. The patient in the current case initially underwent treatment for Bell palsy and then draining of the cyst at an outside hospital with no relief of symptoms. Repeat MR imaging showed an increase in the size of the cystic, enhancing, middle fossa lesion measuring 4 cm. Resection of the lesion was undertaken using a middle fossa approach. After a satisfactory surgical decompression, the patient demonstrated a significant recovery in her facial palsy over a 3-month period of time. This case presents new clinical and radiographic findings associated with these lesions.

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James K. Liu, Christina M. Sayama, Clough Shelton and Joel D. MacDonald

✓Some evidence in the literature supports the topical application of papaverine to the cochlear nerve to prevent internal auditory artery vasospasm and cochlear ischemia as a method of enhancing the ability to preserve hearing during acoustic neuroma surgery. The authors report a case of transient facial nerve palsy that occurred after papaverine was topically applied during a hearing preservation acoustic neuroma removal. A 58-year-old woman presented with tinnitus and serviceable sensorineural hearing loss in her right ear (speech reception threshold 15 dB, speech discrimination score 100%). Magnetic resonance imaging demonstrated a 1.5-cm acoustic neuroma in the right cerebellopontine angle (CPA). A retrosigmoid approach was performed to achieve gross-total resection of the tumor. During tumor removal, a solution of 3% papaverine soaked in a Gelfoam pledget was placed over the cochlear nerve. Shortly thereafter, the quality of the facial nerve stimulation deteriorated markedly. Electrical stimulation of the facial nerve did not elicit a response at the level of the brainstem but was observed to elicit a robust response more peripherally. There were no changes in auditory brainstem responses. Immediately after surgery, the patient had a House–Brackmann Grade V facial palsy on the right side. After several hours, this improved to a Grade I. At the 1-month follow-up examination, the patient exhibited normal facial nerve function and stable hearing.

Intracisternal papaverine may cause a transient facial nerve palsy by producing a temporary conduction block of the facial nerve. This adverse effect should be recognized when topical papaverine is used during CPA surgery.

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Randy L. Jensen, David Gillespie, Paul House, Lester Layfield and Clough Shelton

Object. Endolymphatic sac (ELS) tumors are low-grade malignancies of the temporal bone that are associated with von Hippel—Lindau (VHL) disease but can also occur sporadically. The VHL gene product VHL protein is important in the regulation of hypoxia inducible factor (HIF)-1α, which controls expression of molecules that are important in angiogenesis and cell metabolism. In this study the authors examine the role of VHL and HIF-1 in ELS tumors.

Methods. The ELS tumors from three patients were examined using the following method: DNA from tumor tissue was isolated, amplified by polymerase chain reaction and the VHL gene sequence was compared with the known wild-type sequence. Loss of heterozygosity (LOH) studies were performed to confirm the sequencing data. Immunohistochemical evaluation for VHL, HIF-1α, vascular endothelial growth factor (VEGF), and carbonic anhydrase IX (CA IX) was performed. Snap-frozen tumor tissue was examined using Western blot and HIF-1 immunoassays for HIF-1α and VHL expression.

Two patients had sporadic ELS tumors and the other one suffered from VHL disease. Results of VHL gene sequencing were normal in the tissue derived from the sporadic ELS tumors. The ELS tumor, pheochromocytoma, and spinal hemangioblastoma were heterozygous for the same C-to-A transversion found in the germline carried by the patient with VHL disease. No LOH was detected in the tumor tissue obtained in the patient with VHL disease. Expression of HIF-1α, VEGF, and CA IX evaluated using immunohistochemical studies was elevated in the VHL-associated tumors. Nevertheless, Western blots and immunoassays for HIF-1α did not show elevated expression in these tumors.

Conclusions. The sporadic and VHL disease—associated ELS tumors in this study had normal VHL-mediated HIF-1 regulation. This is a result of normal VHL gene expression in the case of the sporadic ELS tumor. In the VHL-associated ELS tumor, this is due to one normal copy of the VHL gene and adequate VHL gene expression.

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Hussam Abou-Al-Shaar, Yair M. Gozal, Gmaan Alzhrani, Michael Karsy, Clough Shelton and William T. Couldwell

OBJECTIVE

Postoperative cerebral venous sinus thrombosis (CVST) is an uncommon complication of posterior fossa surgery. The true incidence of and optimal management strategy for this entity are largely unknown. Herein, the authors report their institutional incidence and management experience of postoperative CVST after vestibular schwannoma surgery.

METHODS

The authors undertook a retrospective review of all vestibular schwannoma cases that had been treated with microsurgical resection at a single institution from December 2011 to September 2017. Patient and tumor characteristics, risk factors, length of stay, surgical approaches, sinus characteristics, CVST management, complications, and follow-up were analyzed.

RESULTS

A total of 116 patients underwent resection of vestibular schwannoma. The incidence of postoperative CVST was 6.0% (7 patients). All 7 patients developed lateral CVST ipsilateral to the lesion. Four cases occurred after translabyrinthine approaches, 3 occurred after retrosigmoid approaches, and none occurred following middle cranial fossa approaches. Patients were managed with anticoagulation or antiplatelet therapy. Although patients were generally asymptomatic, one patient experienced intraparenchymal hemorrhage, epidural hemorrhage, and obstructive hydrocephalus, likely as a result of the anticoagulation therapy. However, all 7 patients had a modified Rankin scale score of 1 at the last follow-up.

CONCLUSIONS

Postoperative CVST is an infrequent complication, with an incidence of 6.0% among 116 patients who had undergone vestibular schwannoma surgery at one institution. Moreover, the management of postoperative CVST with anticoagulation therapy poses a serious dilemma to neurosurgeons. Given the paucity of reports in the literature and the low incidence of CVST, additional studies are needed to better understand the cause of thrombus formation and help to establish evidence-based guidelines for CVST management and prevention.

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Hussam Abou-Al-Shaar, Yair M. Gozal, Jason P. Hunt, Clough Shelton, Lyska L. Emerson, Evan Joyce and William T. Couldwell

Jugular foramen cavernous hemangiomas are extremely rare vascular malformations, and, to the best of the authors’ knowledge, their occurrence as multifocal lesions involving both intra- and extracranial compartments has never been reported before. Here, the authors describe the case of a 60-year-old woman with a complex multifocal jugular foramen cavernous hemangioma. The patient presented with signs and symptoms concerning for jugular foramen syndrome, as well as a right neck mass. Surgical extirpation of the lesion was achieved by a multidisciplinary team via a right infratemporal fossa approach (Fisch type A) with concurrent high neck dissection and a closure buttressed with an autologous fat graft and a temporoparietal fascial flap. Although rare, cavernous hemangiomas should be included in the differential diagnosis of jugular foramen masses.