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Michael P. Steinmetz, Roseanna M. Lechner and James S. Anderson

Atlantooccipital dislocation (AOD) injuries are highly unstable, and usually result in significant neurological injury and death. Recently the postinjury survival period has increased. In a review of the literature the authors found 41 cases in which survival was greater than 48 hours. This is likely due to improved on-scene resuscitation, spinal immobilization, transportation, new diagnostic techniques, and a higher index of suspicion.

Diagnosis is usually made with plain cervical radiographs, but there are shortcomings associated with this modality in the pediatric population. Diagnosis is aided by high-resolution computerized tomography and magnetic resonance imaging. Infants and toddlers may undergo orthotic immobilization alone, whereas older children usually undergo early occipital cervical fusion. Those with incomplete AOD may be managed successfully with orthotic immobilization.

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Michael P. Steinmetz, Meg Verrees, James S. Anderson and Roseanna M. Lechner

✓ Two children who were 13 months and 3 years old and who had suffered traumatic atlantooccipital dislocation were each treated by being placed in a halo orthosis for 10 weeks. Because of a continued loss of reduction due to the poor fit of the halo vest, a dual-strap augmentation was developed. This strap augmentation allowed consistent reduction to be maintained. Both children were therefore successfully treated nonsurgically with a halo vest. One child remained neurologically intact and the other had improvement in motor strength. There were no complications from the use of strap augmentation for halo vest fixation.

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Mario Zuccarello, Jeffery T. Marsch, Gerald Schmitt, James Woodward and Douglas K. Anderson

✓ The purpose of this study was to use a new 21-aminosteroid (U-74006F) with in vitro antioxidant and antilipolytic properties as a pharmacological probe to assess the role of lipid hydrolysis and peroxidation in a rabbit model of subarachnoid hemorrhage (SAH)-induced vasospasm. Cerebral angiograms were performed on 15 rabbits. Eighteen hours later, 1 cc/kg of autologous blood was infused into the cisterna magna of all 15 animals. Six rabbits received no treatment, six received U-74006F starting 30 minutes after SAH, and three rabbits received the vehicle for U-74006F starting 30 minutes after SAH. At 72 hours post-SAH, a second angiogram was obtained. Digital subtraction angiographic techniques were used to measure the diameter of and contrast material flow through the basilar artery. At 72 hours post-SAH, vasospasm was evident in all untreated and vehicle-treated rabbits. The diameter of and the flow through the basilar artery were significantly reduced 42.3% ± 6.6% and 46.8% ± 5.8%, respectively, below pre-SAH levels (means ± standard error of the means). Treatment with U-74006F eliminated the SAH-induced vasospasm; in treated animals, both the flow through and the diameter of the basilar arteries were at pre-SAH levels. These findings indicate that: 1) membrane lipid changes (that is, hydrolysis with eicosanoid production and/or peroxidation) contribute to the chronic vasospasm resulting from SAH, and 2) U-74006F prevents the SAH-induced chronic vasospasm in this model by limiting these pathological membrane events.

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Richard F. Schmidt, Frederick Yick, Zain Boghani, Jean Anderson Eloy and James K. Liu

Object

Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease.

Methods

A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included.

Results

From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone.

Conclusions

Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.

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Smruti K. Patel, Qasim Husain, Jean Anderson Eloy, William T. Couldwell and James K. Liu

Developed over a century ago, the transsphenoidal approach to access lesions of the pituitary gland and sella turcica has transformed the field of neurosurgery, largely due to the work of Oskar Hirsch and Harvey Cushing. Furthermore, its use and modification in the early 1900s was perhaps one of Cushing's greatest legacies to skull base surgery. However, Cushing, who had worked relentlessly to improve the transsphenoidal route to the pituitary region, abandoned the approach by 1929 in his pursuit to master transcranial approaches to the suprasellar region. Hirsch and a few other surgeons continued to perform transsphenoidal operations, but they were unable to maintain the popularity of the approach among their peers.

During a time when transsphenoidal surgery was on the brink of extinction, a critical lineage of 3 key surgeons—Norman Dott, Gerard Guiot, and Jules Hardy—would resurrect the art, each working to further improve the procedure. Dott, Cushing's apprentice from 1923 to 1924, brought his experiences with transsphenoidal surgery to Edinburgh, Scotland, and along the way, developed the lighted nasal speculum to provide better illumination in the narrow working area. Guiot, inspired by Dott, adopted his technique and used intraoperative radiofluoroscopic technique for image guidance. Hardy, a fellow of Guiot, from Montreal, Canada, revolutionized transsphenoidal microsurgery with the introduction of the binocular microscope and selective adenomectomy.

The teachings of these pioneers have endured over time and are now widely used by neurosurgeons worldwide. In this paper, we review the lineage and contributions of Dott, Guiot, and Hardy who served as crucial players in the preservation of transsphenoidal surgery.

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Anuj V. Peddada, D. James Sceats, Gerald A. White, Gyongyver Bulz, Greg L. Gibbs, Barry Switzer, Susan Anderson and Alan T. Monroe

This case report of 74-year-old man with trigeminal neuralgia is presented to underscore the importance of evaluating the entire treatment plan, especially when delivering large doses where even a low percentage of the prescription dose can contribute a substantial dose to an unintended target. The patient was treated using the CyberKnife stereotactic radiosurgery system utilizing a nonisocentric beam treatment plan with a 5-mm fixed collimator generating 111 beams to deliver 6000 cGy to the 79% isodose line with a maximum dose of 7594 cGy to the target. Two weeks after treatment the patient's trigeminal neuralgia symptoms resolved; however, the patient developed oral mucositis due to the treatment. This case report reviews the cause of mucositis and makes recommendations on how to prevent unintended targets from receiving treatment.

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Richard F. Schmidt, Zain Boghani, Osamah J. Choudhry, Jean Anderson Eloy, Robert W. Jyung and James K. Liu

With the relatively recent increase in the use of MRI techniques, there has been a concurrent rise in the number of vestibular schwannomas (VSs) detected as incidental findings. These incidental VSs may be prevalent in up to 0.02%–0.07% of individuals undergoing MRI and represent a significant portion of all diagnosed VSs. The management of these lesions poses a significant challenge for practitioners. Most incidental VSs tend to be small and associated with minimal symptoms, permitting them to be managed conservatively at the time of diagnosis. However, relatively few indicators consistently predict tumor growth and patient outcomes. Furthermore, growth rates have been shown to vary significantly over time with a large variety of long-term growth patterns. Thus, early MRI screening for continued tumor growth followed by repeated MRI studies and clinical assessments throughout the patient's life is an essential component in a conservative management strategy. Note that tumor growth is typically associated with a worsening of symptoms in patients who undergo conservative management, and many of these symptoms have been shown to significantly impact the patient's quality of life. Specific indications for the termination of conservative management vary across studies, but secondary intervention has been shown to be a relatively safe option in most patients with progressive disease. Patients with incidental VSs will probably qualify for a course of conservative management at diagnosis, and regular imaging combined with the expectation that the tumor and symptoms may change at any interval is crucial to ensuring positive long-term outcomes in these patients. In this report, the authors discuss the current literature pertaining to the prevalence of incidental VSs and various considerations in the management of these lesions. It is hoped that by incorporating an understanding of tumor growth, patient outcomes, and management strategies, practitioners will be able to effectively address this challenging disease entity.

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James K. Liu, Qasim Husain, Vivek Kanumuri, Mohemmed N. Khan, Zachary S. Mendelson and Jean Anderson Eloy

OBJECT

Juvenile nasopharyngeal angiofibromas (JNAs) are formidable tumors because of their hypervascularity and difficult location in the skull base. Traditional transfacial procedures do not always afford optimal visualization and illumination, resulting in significant morbidity and poor cosmesis. The advent of endoscopic procedures has allowed for resection of JNAs with greater surgical freedom and decreased incidence of facial deformity and scarring.

METHODS

This report describes a graduated multiangle, multicorridor, endoscopic approach to JNAs that is illustrated in 4 patients, each with a different tumor location and extent. Four different surgical corridors in varying combinations were used to resect JNAs, based on tumor size and location, including an ipsilateral endonasal approach (uninostril); a contralateral, transseptal approach (binostril); a sublabial, transmaxillary Caldwell-Luc approach; and an orbitozygomatic, extradural, transcavernous, infratemporal fossa approach (transcranial). One patient underwent resection via an ipsilateral endonasal uninostril approach (Corridor 1) only. One patient underwent a binostril approach that included an additional contralateral transseptal approach (Corridors 1 and 2). One patient underwent a binostril approach with an additional sublabial Caldwell-Luc approach for lateral extension in the infratemporal fossa (Corridors 1–3). One patient underwent a combined transcranial and endoscopic endonasal/sublabial Caldwell-Luc approach (Corridors 1–4) for an extensive JNA involving both the lateral infratemporal fossa and cavernous sinus.

RESULTS

A graduated multiangle, multicorridor approach was used in a stepwise fashion to allow for maximal surgical exposure and maneuverability for resection of JNAs. Gross-total resection was achieved in all 4 patients. One patient had a postoperative CSF leak that was successfully repaired endoscopically. One patient had a delayed local recurrence that was successfully resected endoscopically. There were no vascular complications.

CONCLUSIONS

An individualized, multiangle, multicorridor approach allows for safe and effective surgical customization of access for resection of JNAs depending on the size and exact location of the tumor. Combining the endoscopic endonasal approach with a transcranial approach via an orbitozygomatic, extradural, transcavernous approach may be considered in giant extensive JNAs that have intracranial extension and intimate involvement of the cavernous sinus.

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James K. Liu, Lana D. Christiano, Smruti K. Patel, R. Shane Tubbs and Jean Anderson Eloy

Tuberculum sellae meningiomas frequently extend into the optic canals. Radical tumor resection including the involved dural attachment, underlying hyperostotic bone, and intracanalicular tumor in the optic canal offers the best chance of a Simpson Grade I resection to minimize recurrence. Decompression of the optic canal with removal of the intracanalicular tumor also improves visual outcome since this portion of the tumor is usually the cause of asymmetrical visual loss.

The purely endoscopic endonasal extended transsphenoidal approach offers a direct midline trajectory and immediate access to tuberculum sellae meningiomas without brain retraction and manipulation of neurovascular structures. Although the endoscopic approach has been previously criticized for its inability to remove tumor within the optic canals, complete Simpson Grade I tumor removal including intracanalicular tumor, dural attachment, and involved hyperostotic bone can be achieved in properly selected patients. Excellent visualization of the suprasellar region and the inferomedial aspects of both optic canals allows for extracapsular, extraarachnoid dissection of the tumor from the critical structures using bimanual microsurgical dissection.

In this report, the authors describe the operative nuances for removal of tuberculum sellae meningiomas with optic canal involvement using a purely endoscopic endonasal extended transsphenoidal (transplanum transtuberculum) approach. They specifically highlight the technique for endonasal bilateral optic nerve decompression and removal of intracanalicular tumor to improve postoperative visual function, as demonstrated in 2 illustrative cases. Special attention is also given to cranial base reconstruction to prevent CSF leakage using the vascularized pedicled nasoseptal flap.

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James K. Liu, Lana D. Christiano, Smruti K. Patel, R. Shane Tubbs and Jean Anderson Eloy

Olfactory groove meningiomas represent 10% of intracranial meningiomas and arise in the midline of the anterior cranial fossa along the dura of the cribriform plate and planum sphenoidale. Hyperostosis of the adjacent underlying bone is common, and further extension into ethmoid sinuses and nasal cavity can occur in 15%–25% of cases. Radical tumor resection including the involved dural attachment and underlying hyperostotic bone offers the best chance of a Simpson Grade I resection to minimize recurrence. Incomplete removal of involved hyperostotic bone can result in tumor recurrence at the cribriform plate with extension into the paranasal sinuses. Resection has traditionally been performed using a bifrontal or pterional approach, both of which require some degree of brain retraction or manipulation to expose the tumor.

The endoscopic endonasal transcribriform approach offers the most direct and immediate exposure to the tumor without brain retraction and manipulation of neurovascular structures. An endonasal “keyhole craniectomy” is performed in the ventral skull base directly over the basal dural attachment, extending from the posterior wall of the frontal sinus to the planum sphenoidale and tuberculum sellae in the anteroposterior plane, and from one medial orbit to the other in the coronal plane. Excellent panoramic visualization of the keyhole skull base defect can be obtained with a 30° endoscope after performing a modified Lothrop procedure. Because the dural attachment is adjacent to the paranasal sinuses, early devascularization and total Simpson Grade I removal of the tumor including the dural attachment and underlying hyperostotic bone can be achieved in properly selected patients. This approach is also very suitable for meningiomas that have recurred or extended into the paranasal sinuses. Extracapsular, extraarachnoid dissection of the tumor from the frontal lobes and neurovascular structures can be performed using conventional bimanual microsurgical techniques.

In this report, we review the surgical technique and describe our operative nuances for removal of olfactory groove meningiomas, including recurrent tumors with extension into the nasal cavity, using a purely endoscopic endonasal transcribriform approach. In addition, we discuss the advantages, limitations, patient selection, and complications of this approach. We specifically highlight our technique for multilayer reconstruction of large anterior skull base dural defects using fascia lata and acellular dermal allograft supplemented by bilateral vascularized pedicled nasoseptal flaps. Three new cases of endoscopically resected olfactory groove meningiomas are also presented.