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Subtotal gland resection for MR negative Cushing disease with no detectable tumor on gland exploration: operative video

Jamie J. Van Gompel, Irina Bancos, and Garret Choby

MRI–Negative Cushing disease is a very difficult disease to treat medically and surgically. In the past, after negative gland exploration, hemihypophysectomy was commonly performed on the localizing side of inferior petrosal sampling. However, this generally resulted in 50% remission/cure rates. Therefore, other techniques have arisen based on the percent chance of microadenoma tumor being present in the gland. Subtotal gland resection is a technique aimed at removing 75% of the gland that results in a similar chance of remission and a 10% chance of pituitary dysfunction. In this video, the authors demonstrate this important technique for MRI–Negative Cushing disease.

The video can be found here: https://thejns.org/doi/abs/10.3171/2023.4.FOCVID2320

In Neurosurgical Focus: Video Volume 9: Issue 1 (Jul 2023): Techniques in MRI–Negative Cushing Disease

Free access

Introduction. Best practices in telemedicine for optimizing patient care

Franco Servadei, Katharine J. Drummond, Ann Stroink, and Jamie J. Van Gompel

In Neurosurgical Focus Volume 52 (2022): Issue 6 (Jun 2022): Best Practice With Telemedicine for Optimizing Patient Care

Free access

Letter to the Editor. Stereotactic laser ablation for hypothalamic hamartomas after SRS failure

Nicholas J. Brandmeir and Michael D. Sather

In Neurosurgical Focus Volume 42: Issue 5 (May 2017): Robotics in Neurosurgery

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Editorial

Depth electrodes and outcome

Kim J. Burchiel

In Journal of Neurosurgery Volume 113 (2010): Issue 1 (Jul 2010)

Full access

Microvascular decompression for tinnitus: systematic review

Brenton Nash, Matthew L. Carlson, and Jamie J. Van Gompel

OBJECTIVE

The objective of this study was to examine operative outcomes in cases of microvascular decompression (MVD) of cranial nerve (CN) VIII for tinnitus through a critical review of the literature.

METHODS

Forty-three English-language articles were gathered from PubMed and analyzed. In this review, two different case types were distinguished: 1) tinnitus-only symptomatology, which was defined as a patient with tinnitus with or without sensorineural hearing loss; and 2) mixed symptomatology, which was defined as tinnitus with symptoms of other CN dysfunction. This review reports outcomes of those with tinnitus-only symptoms.

RESULTS

Forty-three tinnitus-only cases were found in the literature with a 60% positive outcome rate following MVD. Analysis revealed a 5-year cutoff of preoperative symptom duration before which a good outcome can be predicted with 78.6% sensitivity, and after which a poor outcome can be predicted with 80% specificity.

CONCLUSIONS

As the 60% success rate is more promising than several other therapeutic options open to the chronic tinnitus sufferer, future research into this field is warranted.

In Journal of Neurosurgery Volume 126 (2017): Issue 4 (Apr 2017): Pages 1029-A1444

Free access

Outcomes of cingulate epilepsy surgery: insights from an institutional and patient-level systematic review and meta-analysis

Panagiotis Kerezoudis, Rohin Singh, Gregory A. Worrell, and Jamie J. Van Gompel

OBJECTIVE

Due to their deep and medial location, range of seizure semiologies, and poor localization on ictal electroencephalography (EEG), cingulate gyrus seizures can be difficult to diagnose and treat. The aim of this study was to review the available evidence on postoperative outcomes after cingulate epilepsy surgery.

METHODS

A comprehensive literature search of the PubMed/MEDLINE, Ovid Embase, Ovid Scopus, and Cochrane Library databases was conducted to identify studies that investigated postoperative outcomes of patients with cingulate epilepsy. Seizure freedom at the last follow-up (at least 12 months) was the primary endpoint. The literature search was supplemented by the authors’ institutional series (4 patients).

RESULTS

Twenty-one studies were identified, yielding a total of 105 patients (68 with lesional epilepsy [65%]). Median age at surgery was 23 years, and 56% of patients were male. Median epilepsy duration was 7.5 years. Invasive EEG recording was performed on 69% of patients (53% of patients with lesional epilepsy and 97% of those with nonlesional epilepsy, p < 0.001). The most commonly resected region was the anterior cingulate (55%), followed by the posterior (17%) and middle (14%) cingulate. Lesionectomy alone was performed in 9% of patients. Additional extracingulate treatment was performed in 54% of patients (53% of patients with lesional epilepsy vs 57% of those with nonlesional epilepsy, p = 0.87). The most common pathology was cortical dysplasia (54%), followed by low-grade neoplasm (29%) and gliosis (8%). Seizure freedom was noted in 72% of patients (median follow-up 24 months). A neurological deficit was noted in 27% of patients (24% had temporary deficit), with the most common deficit being motor weakness (13%) followed by supplementary motor area syndrome (9.5%). Univariate survival analysis revealed significantly greater probability of seizure freedom in patients with lesional epilepsy (p = 0.015, log-rank test).

CONCLUSIONS

Surgical treatment of drug-resistant focal epilepsy originating from the cingulate gyrus is safe, leads to low rates of permanent adverse effects, and leads to high rates of long-term seizure freedom in carefully selected patients. These data may serve as a benchmark for surgical counseling of patients with cingulate epilepsy.

In Journal of Neurosurgery Volume 137: Issue 1 (Jul 2022)

Free access

Craniotomy for anterior cranial fossa meningiomas: historical overview

Saul F. Morales-Valero, Jamie J. Van Gompel, Ioannis Loumiotis, and Giuseppe Lanzino

The surgical treatment of meningiomas located at the base of the anterior cranial fossa is often challenging, and the evolution of the surgical strategy to resect these tumors parallels the development of craniotomy, and neurosurgery in general, over the past century. Early successful operations to treat these tumors were pioneered by prominent figures such as Sir William Macewen and Francesco Durante. Following these early reports, Harvey Cushing made significant contributions, allowing a better understanding and treatment of meningiomas in general, but particularly those involving the anterior cranial base. Initially, large-sized unilateral or bilateral craniotomies were necessary to approach these deep-seated lesions. Technical advances such as the introduction of electrosurgery, the operating microscope, and refined microsurgical instruments allowed neurosurgeons to perform less invasive surgical procedures with better results. Today, a wide variety of surgical strategies, including endoscopic surgery and radiosurgery, are used to treat these tumors. In this review, the authors trace the evolution of craniotomy for anterior cranial fossa meningiomas.

In Neurosurgical Focus Volume 36 (2014): Issue 4 (Apr 2014): History of Craniotomy, Cranioplasty, and Perioperative Care

Free access

Does pituitary compression/empty sella syndrome contribute to MRI-negative Cushing’s disease? A single-institution experience

Benjamin T. Himes, Adip G. Bhargav, Desmond A. Brown, Timothy J. Kaufmann, Irina Bancos, and Jamie J. Van Gompel

OBJECTIVE

Cushing’s disease arises from functioning adrenocorticotropic hormone (ACTH)–secreting pituitary adenomas. These tumors can be very small and evade detection by MRI. Empty sella syndrome is a phenomenon by which an arachnoid outpouching of CSF into the sella leads to compression of the pituitary, likely due to intracranial hypertension (a common issue in Cushing’s disease), further leading to difficulty in visualizing the pituitary gland that may contribute to difficulty in finding a tumor on MRI, so-called MRI-negative Cushing’s disease. The authors sought to examine the association between empty sella syndrome and MRI-negative Cushing’s disease.

METHODS

A single-institution database of Cushing’s disease cases from 2000 to 2017 was reviewed, and 197 cases were included in the analysis. One hundred eighty patients had a tissue diagnosis of Cushing’s disease and 17 had remission with surgery, but no definitive tissue diagnosis was obtained. Macroadenomas (tumors > 1 cm) were excluded. The degree of empty sella syndrome was graded on the degree of CSF visualized in the sella on midline sagittal T1-weighted MRI.

RESULTS

Of the 197 cases identified, 40 (20%) presented with MRI-negative disease, and empty sella syndrome was present in 49 cases (25%). MRI-negative disease was found in 18 (37%) of 49 empty sella cases versus 22 (15%) of 148 cases without empty sella syndrome present. Empty sella syndrome was significantly associated with MRI-negative disease (OR 3.32, 95% CI 1.61–6.74, p = 0.0018). Decreased thickness of the pituitary gland was also associated with MRI-negative disease (mean thickness 5.6 vs 6.8 mm, p = 0.0002).

CONCLUSIONS

Empty sella syndrome is associated with an increased rate of MRI-negative Cushing’s disease. Pituitary compression causing a relative reduction in the volume of the pituitary for imaging is a plausible cause for not detecting the tumor mass with MRI.

In Neurosurgical Focus Volume 48 (2020): Issue 6 (Jun 2020): Pituitary tumors

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Cystic deterioration of the C1–2 articulation: clinical implications and treatment outcomes

Clinical article

Jamie J. Van Gompel, Jonathan M. Morris, Jan L. Kasperbauer, Darlene E. Graner, and William E. Krauss

Object

Synovial cysts of the cervical spine are rare. Further, synovial cysts of the C1–2 articulation, or retroodontoid cysts, are very rare, with fewer than 20 cases reported overall. The authors report on a single-institution series of 10 patients with C1–2 retroodontoid synovial cysts.

Methods

The charts of 10 patients with pathologically confirmed synovial cysts following resection between January 1998 and June 2009 were reviewed. The clinical presentation, radiographic findings, surgical management, and pre- and postoperative functional levels (reported as a modified Rankin outcome) are reported.

Results

All 10 patients underwent attempts at complete resection of the synovial cyst; none of the lesions were simply aspirated. All 4 patients with an initial recommendation of observation alone ultimately underwent surgery because of their swift neurological deterioration. The mean age at surgery was 75.4 ± 4.6 years (range 54–81 years). The mean presenting cyst volume was 4.6 ± 1.7 cm3. Nine of 10 patients underwent transoral resection of the cyst with posterior fusion. Six of the 10 patients underwent additional posterior laminectomy for decompression. The hospital length of stay varied from 2 to 45 days, with a mean of 19 ± 7 days. All patients undergoing transoral decompression had varying degrees of difficulty with postoperative dysphagia, diagnosed using videofluoroscopy. All patients improved in their modified Rankin Scale score after surgical intervention with a mean follow-up of 42 months (95% CI 12–72 months).

Conclusions

Synovial cysts of the atlantoaxial joint are rare. They occur in older patients in whom clinical deterioration is likely to occur. In most cases, these cysts can be diagnosed preoperatively. Transoral decompression with posterior fusion is an effective treatment for C1–2 degenerative cysts and can be accomplished with few complications. However, the ideal treatment for these lesions remains unknown.

In Journal of Neurosurgery: Spine Volume 14 (2011): Issue 4 (Apr 2011)

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Surgically treated movement disorders associated with heterotopia

Report of 2 cases

Jeffrey P. Mullin, Jamie J. Van Gompel, Kendall H. Lee, Fredric B. Meyer, and Matt Stead

Heterotopic gray matter has been implicated in epilepsy; however, not much is known regarding heterotopia beyond epilepsy. Here, the authors describe 2 pediatric patients with deep heterotopias contiguous with basal ganglia structures. These heterotopias appear to have manifested as movement disorders. One patient presented with a left-sided myoclonus and choreiform movements associated with a right caudate heterotopia; she experienced vast improvement after resection of periventricular heterotopia. The other patient presented with progressive dystonia and a ballistic movement disorder. Initial bilateral globus pallidus internus stimulation resulted in successful treatment of the dystonia; however, her movement disorder worsened. After an extensive workup, including STATISCOM (statistical ictal SPECT coregistered to MR imaging), the patient underwent cortical stimulation with improvement in her movement disorder. To the best of our knowledge, these cases are the first reported instances of heterotopic gray matter associated with movement disorders. Both patients experienced significant improvements following resection of their heterotopias.

In Journal of Neurosurgery: Pediatrics Volume 6 (2010): Issue 3 (Sep 2010)