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IgG4-related hypophysitis is a rare clinical entity that forms part of an emerging group of multi-organ IgG4-related fibrosclerotic systemic diseases. The rare prevalence of the disease, presenting features that overlap with other sellar pathologies, and variable imaging features can make preoperative identification challenging. We report three cases of isolated IgG4-related hypophysitis with atypical clinical and imaging features that mimicked those of pituitary apoplexy and other sellar lesions. Additionally, we review the literature of IgG4-related hypophysitis to provide context for individual patient data described herein. All patients presented with symptoms that mimicked those of pituitary apoplexy and visual disturbance, and MRI findings suggestive of pituitary macroadenoma, Rathke’s cleft cyst and craniopharyngioma. The clinical presentation warranted surgical decompression, resulting in rapid symptomatic improvement. Preoperative high-dose followed by postoperative low-dose glucocorticoid replacement therapy was administered in all cases. Histopathology showed dense infiltrate of IgG4 cells. Post-operative follow-up monitoring for 12–26 months revealed normal serum IgG4 levels with no other organ involvement, while endocrinological testing revealed persistent pituitary hormone deficiencies. Our cases highlight the importance of considering IgG4-related hypophysitis in the differential diagnosis of solid and cystic sellar lesions presenting acutely with pituitary apoplexy symptoms. Existing diagnostic criteria may not be sufficiently precise to permit rapid and reliable identification, or avoidance of surgery in the acute setting. In contrast to other reports of the natural history of this condition, despite the severity of presenting features, the disease in our cases was pituitary-restricted with normal serum IgG4 levels.

OBJECT Middle fossa floor dehiscence (MFFD) can present as multiple syndromes depending on dehiscence location, tissue herniation, and dural integrity. The authors propose a classification system for MFFD with the potential to guide clinical decision making. METHODS A retrospective analysis of the electronic medical records (years 1995–2012) of patients who had undergone temporal craniotomy for the surgical repair of an MFFD syndrome at a single institution was undertaken. Reviewed data included demographic, operative, presentation, and outcome details. Middle fossa floor dehiscence was classified as follows: Class A, bony dehiscence without herniation of the brain and/or meninges; Class B, herniation of the brain and/or meninges through the middle fossa floor without CSF leakage; Class C, dehiscence with CSF leakage without meningitis; or Class D, dehiscence with meningitis. RESULTS Fifty-one patients, 22 males and 29 females, were included in the analysis. The mean age was 48.7 ± 15.5 years, mean body mass index was 32.65 ± 6.86 kg/m2, and mean symptom duration was 33 ± 42 months. Seven patients underwent repeat surgery for symptomatic recurrence; therefore, there were 58 surgical encounters. Repair included bony reconstruction with hydroxyapatite with or without resection of encephaloceles and/or repair of a dural defect. According to the MFFD classification system described, 15, 8, 27, and 8 cases were categorized as Class A, B, C, and D, respectively. The prevalence of hearing loss was 87%, 63%, and 70% in Classes A, B, and C, respectively. Vestibular symptoms were more prevalent in Class A. Seven patients reported persistent symptoms at the last follow-up. Transient complications were similar in each classification (13%–25%), and a single permanent complication related to anesthesia was observed. There were no mortalities or severe neurological morbidities in the series. CONCLUSIONS Middle fossa floor dehiscence has a spectrum of clinical presentations. A classification system may help to clarify the diagnosis and guide therapy. Surgery, the mainstay of treatment, is safe and well tolerated.

Objective To analyze whether cavernous sinus sampling (CSS) and dynamic magnetic resonance imaging (dMRI) are consistent with intraoperative findings in Cushing disease (CD) patients. Design Retrospective outcomes study. Setting Oregon Health & Science University; 2006 and 2013. Participants A total of 37 CD patients with preoperative dMRI and CSS to confirm central adrenocorticotropic hormone (ACTH) hypersecretion. Patients were 78% female; mean age was 41 years (at diagnosis), and all had a minimum of 6 months of follow-up. Main Outcome Measures Correlations among patient characteristics, dMRI measurements, CSS results, and intraoperative findings. Results All CSS indicated presence of CD. Eight of 37 patients had no identifiable tumor on dMRI. Three of 37 patients had no tumor at surgery. dMRI tumor size was inversely correlated with age (rs = − 0.4; p = 0.01) and directly correlated to intraoperative lateralization (rs = 0.3; p

Background Anterior and posterior clinoidectomies have been proposed to augment exposure of the basilar apex. A sequential quantitative benefit analysis offered by these maneuvers has not been reported. Methods Fourteen datasets from eight cadaveric specimens were analyzed. A modified orbitozygomatic frontotemporal craniotomy was performed. The extent of proximal control of the basilar artery was determined through the exposed opticocarotid and carotidoculomotor triangles before and after clinoidectomies and mobilization of the third nerve at the porous oculomotarius. Results Removal of the anterior and posterior clinoids significantly improved proximal basilar artery access (p Conclusion Anterior and posterior clinoidectomies significantly improved the area of exposure of the opticocarotid triangle, carotidoculomotor triangle, and the exposed length of the basilar artery available for proximal control. This improvement is extremely important for large or giant aneurysms of the upper basilar artery or aneurysms hidden by the posterior clinoid.

Objectives To analyze surgical outcomes and predictive factors of disease remission in acromegaly patients who underwent microscopic transsphenoidal surgery (TSS) for a growth hormone (GH)-secreting adenoma. Design A 6-year retrospective review of 86 consecutive acromegaly surgeries. Setting Procedures performed at a single institution by a single surgeon. Participants Seventy acromegaly patients. Main Outcome Measures Demographic information, preoperative laboratory values, tumor imaging data, and morphological and immunohistochemical data were collected. Predictive values using the latest and most stringent biochemical remission criteria were determined using univariate and multivariate statistical analyses. Results Remission rate for 59 (18 males) acromegaly patients meeting the study inclusion criteria was 52.5%. Remission rates for micro- and macroadenomas were 81.8% and 45.8%, respectively. Patients of older age, with a smaller tumor, lower Knosp grade, lower preoperative GH, and insulinlike growth factor 1 levels were more likely to achieve remission. Remission rate decreased significantly with repeat surgeries. Those patients with adenomas that stained positive for somatostatin receptor subtype 2A were less likely to experience tumor recurrence and more likely to respond to medical treatment with persistent or elevated GH hypersecretion. Conclusions Microscopic TSS continues to be a viable means for treating acromegaly patients. Patients should be followed long term.

Background: Endoscopic third ventriculostomy (ETV) has become one of the most common neuroendoscopic procedures.Methods: In this article, we will review the major milestones in the history of ETV development from its early use by Walter Dandy to the techniques currently employed with advanced technology.Conclusions: ETV has become an important technique in the armamentarium of the neurosurgeon. From a meager beginning with few applications, our knowledge of long-term outcomes has evolved. ETV has a rich history and more recently, has had a renewed interest in its use. Our current understanding of its indications is growing and is based on a century of development through trial and error.

A 69-year-old female with a history of breast cancer and hypertension presented with a rare case of herpes simplex encephalitis (HSE) isolated to her left parietal lobe. The patient's first biopsy was negative for herpes simplex virus (HSV) I/II antigens, but less than two weeks later, the patient tested positive on repeat biopsy. This initial failure to detect the virus and the similarities between HSE and symptoms of intracranial hemorrhage (ICH) suggests repeat testing for HSV in the presence of ICH. Due to the frequency of patients with extra temporal HSE, a diagnosis of HSE should be more readily considered, particularly when a patient may not be improving and a concrete diagnosis has not been solidified.

Subfrontal schwannomas, sometimes referred to as olfactory groove schwannomas, are rare tumors (34 cases reported to date). Despite the name and several theories proposed in the literature, there is no officially recognized description of the tumor's cell origin. Yasuda proposed the concept of an olfactory ensheathing cell (OEC) tumor in 2006. Olfactory ensheathing cells are glial cells that ensheath the axons of the first cranial nerve. Microscopically, both olfactory ensheathing cells and Schwann cells have similar morphological and immunohistochemical features. However, immunohistochemically olfactory ensheathing cells are negative for Leu7 and Schwann cells positive. A 30-year-old woman presented with a subfrontal, extraaxial, enhancing tumor, and underwent gross total resection. Immunohistochemical reactivity data suggested a schwannoma (positive for S-100 and negative for epithelial membrane antigen). However, the tumor was negative for Leu7. Accordingly, our final diagnosis was that of an OEC tumor. Subfrontal schwannoma immunohistochemical staining, if negative for Leu7, is indicative of an OEC tumor. It is possible that schwannoma-like extraaxial tumors at the anterior skull base are OEC tumors, which negative Leu7 staining can confirm.

Despite recent advances in the treatment of malignant gliomas (World Health Organization grade III and grade IV tumors- Glioblastoma Multiforme, Anaplastic Astrocytoma and Anaplastic Oligodendroglioma), the overall prognosis remains poor. Tumor recurrence in malignant glioma is inevitable, and associated with reduced overall survival (OS). Bevacizumab is approved for use in progressive GBM as a second line treatment, and is associated with improvements in progression free survival (PFS). However, all GBM patients eventually recur on bevacizumab therapy, with a very short OS after bevacizumab failure. No FDA-approved therapy is available for this clinical setting. Etoposide crosses the blood-brain barrier and has activity in recurrent malignant gliomas. The use of bevacizumab with etoposide in recurrent malignant gliomas in the setting of bevacizumab resistance is evaluated in this review. Bevacizumab and etoposide combined therapy is associated with radiographic response and effectiveness in selected patients.

Neurosurgeons perform operations every day, many of which involve the scalp. There is evidence supporting similar or decreased wound infection rates in the unshaven scalp. Patients with standard scalp incisions were assigned to either shave or shaveless preparations (n = 20). The timing of preparation and skin closure was recorded for both groups as were infectious complications. All of the patients have been followed for an average of 10 months. There were no cases of infection. The timing of scalp preparation and closure was not significantly different between the two groups (P < .05). We have previously suggested that shaving the scalp is not a critical step in the prevention of infection. We confirm that the timing of this technique is not prolonged over that of standard preparations.

Introduction: High-grade dural arteriovenous fistulas (DAVFs) with retrograde cortical leptomeningeal drainage are formidable lesions because of their risk for intracranial hemorrhage. Treatment is aimed at occluding venous outflow to achieve obliteration of the fistula. In DAVFs that involve a large dural venous sinus, occluding venous outflow can be accomplished endovascularly with transvenous embolization. In cases of DAVFs with reflux into cortical leptomeningeal veins, there is usually venous restrictive disease downstream that can prohibit endovascular access via the transfemoral or transjugular routes. Methods: In this study, we describe our technique of surgically assisted transvenous embolization in three patients with high-grade DAVFs involving a large dural sinus with retrograde cortical leptomeningeal drainage. Results: Surgically assisted transvenous embolization of the DAVF resulted in complete angiographic obliteration in all three patients on postembolization angiography. All had improvement of their preoperative symptoms, and there were no recurrences and no further clinical events after a mean follow-up of 37 months. There were no complications of venous hypertension or venous infarction. Conclusion: Our technique is unique in that the craniectomy and embolization procedures are performed as a single stage in the operating room with intraoperative angiography and stereotactic image guidance. In these instances, a transcranial approach can be performed to expose the large dural venous sinus distal to the site of occlusion for direct catheterization of the venous outflow for transvenous embolization. This combined surgical and endovascular strategy provides direct access to the venous outflow and bypasses the site of thrombotic obstruction.

Object Cavernous malformations (CMs) of the optic pathway and hypothalamus (OPH) are extremely rare. Patients with these lesions typically present with chiasmal apoplexy, characterized by sudden visual loss, acute headaches, retroorbital pain, and nausea. Surgical removal is the recommended treatment to restore or preserve vision and to eliminate the risk of future hemorrhage. However, the anatomical location and eloquence of nearby neural structures can make these lesions difficult to access and remove. In this study, the authors review the literature for reported cases of OPH CMs to analyze clinical and radiographic presentations as well as surgical approaches and neurological outcomes. Methods A MEDLINE/PubMed search was performed, revealing 64 cases of OPH CMs. The authors report an additional case in the study, making a total of 65 cases. Each case was analyzed for clinical presentation, lesion location, radiographic features, treatment method, and visual outcome. Results In 65 patients with OPH CMs, the optic chiasm was affected in 54 cases, the optic nerve(s) in 35, the optic tract in 13, and the hypothalamus in 5. Loss of visual field and acuity was the most common presenting symptom (98%), followed by headache (60%). The onset of symptoms was acute in 58% of patients, subacute in 15%, and chronic progressive in 26%. Computed tomography scans revealed hyperdense suprasellar lesions, with calcification visible in 56% of cases. Magnetic resonance imaging typically demonstrated a heterogeneous lesion with mixed signal intensities suggestive of blood of different ages. The lesion was often surrounded by a peripheral rim of hypointensity on T2-weighted images in 60% of cases. Minimal or no enhancement occurred after the administration of gadolinium. Hemorrhage was reported in 82% of cases. Most patients were surgically treated (97%) using gross-total resection (60%), subtotal resection (6%), biopsy procedure alone (6%), biopsy procedure with decompression (23%), and biopsy procedure followed by radiation (2%). Those patients who underwent gross-total resection had the highest rate of visual improvement (85%). Two patients were treated conservatively, resulting in complete blindness in 1 patient and spontaneous recovery of vision in the other patient. Conclusions Cavernous malformations of the OPH are rare and challenging lesions. Gross-total resection of these lesions is associated with favorable visual outcomes. Emergent surgery is warranted in patients presenting with chiasmal apoplexy to prevent permanent damage to the visual pathway.

Refractory anaplastic oligodendroglioma and oligoastrocytoma tumors are challenging to treat. This trial primarily evaluated toxicity and estimated the maximum tolerated dose of intra-arterial (IA) melphalan, IA carboplatin, and intravenous (IV) etoposide phosphate in conjunction with blood-brain barrier disruption in these tumors. The secondary measure was efficacy.Thirteen patients with temozolomide-refractory anaplastic oligodendroglioma (11 patients) or oligoastrocytoma (2 patients) underwent blood-brain barrier disruption with carboplatin (IA, 200 mg/m(2)/d), etoposide phosphate (IV, 200 mg/m(2)/d), and melphalan (IA, dose escalation) every 4 weeks, for up to 1 year. Patients underwent melphalan dose escalation (4, 8, 12, 16, and 20 mg/m(2)/d) until the maximum tolerated dose (1 level below that producing grade 4 toxicity) was determined. Toxicity and efficacy were assessed.Two of 4 patients receiving IA melphalan at 8 mg/m(2)/d developed grade 4 thrombocytopenia; thus, the melphalan maximum tolerated dose was 4 mg/m/d. Adverse events included asymptomatic subintimal tear (1 patient) and grade 4 thrombocytopenia (3 patients). Two patients demonstrated complete response, 3 had partial responses, 5 demonstrated stable disease, and 3 progressed. Median overall progression-free survival was 11 months. Patients with complete or partial response demonstrated deletion of chromosomes 1p and 19q. In the 5 patients with stable disease, 2 demonstrated 1p and 19q deletion, and 3 demonstrated 19q deletion only.In patients with anaplastic oligodendroglioma or oligoastrocytoma tumors in whom temozolomide treatment has failed, osmotic blood-brain barrier disruption with IA carboplatin, IV etoposide phosphate, and IA melphalan (4 mg/m(2)/d for 2 days) shows acceptable toxicity and encouraging efficacy, especially in patients demonstrating 1p and/or 19q deletion.

Cushing disease is considered an aggressive pituitary endocrinopathy because of the devastating effects from untreated hypercortisolemia. Although they are histologically benign, these adrenocorticotropic hormone (ACTH)-secreting pituitary tumors are associated with significant morbidity and premature death. Currently, transsphenoidal surgery is the primary treatment of Cushing disease associated with an ACTH-secreting pituitary tumor, resulting in remission rates ranging from about 50 to 90%. Some patients, however, will not achieve sustained remission after transsphenoidal surgery and can exhibit persistent or recurrent Cushing disease that requires multimodal treatment to achieve remission. In these patients, options for treatment include repeat transsphenoidal resection, radiation therapy (including conventional fractionated radiation therapy and stereotactic radiosurgery), and medical therapy. Despite undergoing multiple treatment modalities, some patients may ultimately require bilateral adrenalectomy for definitive treatment to eliminate hypercortisolemia associated with Cushing disease. In this article, the authors review the treatment options for patients who have persistent or recurrent Cushing disease after unsuccessful transsphenoidal surgery. The indications, current results reported in the literature, and complications of each treatment modality are discussed. (DOI: 10.3171/FOC-07/09/E8)

Introduction: Basilar apex aneurysms remain one of the most difficult aneurysms to control proximally. The subtemporal approach to basilar apex aneurysms is often advocated when the aneurysm is below the posterior clinoids. We used a stereotactic method to quantify increased proximal control of the basilar artery obtained by transecting the tentorium during a subtemporal approach. Methods: A subtemporal approach to the basilar apex was performed on three latex-injected cadaveric heads (three sides). Using a stereotactic method, the point of maximum proximal control of the basilar artery was measured from the basilar apex. Proximal control was compared before and after transecting the tentorium both anterior and posterior to the trochlear nerve. Results: Our preliminary results show that working inferior to the oculomotor nerve the proximal control measured before transecting the tentorium was 7.7 ± 2.8 mm. After transecting the tentorium just behind the trochlear nerve this was essentially unchanged at 8.6 ± 2.4 mm, but increased significantly to 12.7 ± 2.0 mm after cutting in front of the trochlear nerve (P = 0.022, repeated measures ANOVA, BonFeronni adjusted). Conclusion: We conclude that transecting the tentorium in a subtemporal approach to the basilar apex improves proximal control. In the future we intend to investigate the effect of cutting the tentorium on other measurements such as working area and surgical freedom.

The authors report on their unique experience with a patient harboring a cervical spinal myeloid sarcoma presenting with cervical radiculopathy and neck pain. The authors succinctly present a rare clinical presentation of cervical radiculopathy and neck pain in an otherwise healthy 43-year-old man. Their diagnostic workup revealed a posterior extradural extramedullary epidural and soft tissue cervical mass spanning the cervicothoracic junction. Multidisciplinary treatment was followed with surgical debulking and biopsy, followed by diagnosis of the primary myeloproliferative neoplasm by bone biopsy. The patient received adjuvant chemoradiation. Follow-up visual analog scale scores were all improved, and his preoperative symptoms resolved. In addition, he showed local control of the soft tissue and epidural cervical disease on follow-up imaging. In the operative intervention, the authors chose a midline approach with laminectomy and decompression of the epidural tumor without posterior stabilization. Their follow-up imaging showed no evidence of destabilization or kyphosis. This approach is reasonable and appropriate for posterior midline epidural disease. Care must be taken, as is known, to minimize the soft tissue dissection and facet violation during laminectomy to prevent deformity. An argument may be made to perform an instrumented arthrodesis given the fact that the lesion and laminectomy spanned the cervicothoracic junction and that there was no known primary at the time, making it difficult to prognosticate the overall expected length of survival, which others have shown can influence the aggressiveness of surgical management.1 Though this is not an unreported presentation, it is a rare pathology2 3; the previously published literature does support the same treatment paradigm that was undertaken for this patient. Surgical decompression with adjuvant chemoradiation has been the standard used by other practitioners.3 4 In summary, the authors effectively share their case report of cervical spinal myeloid sarcoma presenting with radiculopathy. The case outlines their treatment and highlights the insidious course this pathology took in a patient who otherwise appeared healthy. Tumors of any variety should always be kept in the differential diagnosis when evaluating patients with neurologic symptoms.

Objectives/Hypothesis Surgery has been the most common treatment for acoustic neuromas, but gamma knife radiosurgery has emerged as a safe and efficacious alternative to microsurgery. This meta-analysis compares the outcomes of the two modalities. Study Design A retrospective MEDLINE search was used to find all surgical and gamma knife studies published from 1990 to 1998 and strict inclusion criteria were applied. Results For tumors less than 4 cm in diameter, there is no difference in hearing preservation (P = .82) or facial nerve outcome (P = .2). Surgery on all sized tumors has a significantly lower complication rate than radiosurgery performed on tumors smaller than 4 cm (P = 3.2 × 10−14). Surgery also has a lower major morbidity rate than gamma knife radiosurgery (P = 2.4 × 10−14). Tumor control was defined as no tumor recurrence or no tumor re-growth. Surgery has superior tumor control when tumors are totally resected (P = 9.02 × 10−11). Assuming that all partially resected tumors will recur, surgery still retains a significant advantage over radiosurgery for tumor control (P = .028). Conclusion Data from these studies date back to the late 1960s and do not completely reflect outcomes using current imaging and procedures. A major difficulty encountered in this study is inconsistent data reporting. Future surgical and radiation reports should use standardized outcomes scales to allow valid statistical comparisons. In addition, long-term results from gamma knife radiosurgery using lower dosimetry have not been reported. Surgery should remain the therapy of choice for acoustic neuromas until tumor control rates can be established.

In the treatment of acoustic neuroma, operative results have improved greatly during recent years, with high rates of functional cranial nerve preservation. Because of this, it has become more important to consider issues of patient satisfaction and quality of life (QOL) following treatment for these lesions. The authors have developed a novel questionnaire designed to measure QOL in patients with acoustic neuromas, and they administered it to 50 consecutive patients at least 6 months after acoustic neuroma surgery. Overall QOL was judged to be good but with definite minor difficulties, including some problems with hearing, facial nerve function, headache, tinnitus, dizziness, activity level, enjoyment of life, and emotional well-being. No significant differences were found between age groups and different operative approaches, and only minor differences were found in relation to tumor size. Patients with intracanalicular tumors fared no better than those with cerebellopontine angle tumors. Analysis of the data suggests an overall good outcome from acoustic neuroma surgery; however, when discussing the possible effects on postoperative QOL, even the potential minor problems should not be minimized, especially in patients undergoing operation for small or intracanalicular tumors.