The authors report a case of Rathke cleft cyst presenting with severe hyponatremia. A 33-year-old man suffered sudden severe headaches, visual changes, dizziness, nausea, vomiting, and a metallic taste in his mouth. Initial laboratory values demonstrated severe hyponatremia. Magnetic resonance imaging revealed a cystic lesion with questionable intracystic hemorrhage, concerning for pituitary apoplexy. Transsphenoidal decompression and drainage of the cyst confirmed the diagnosis of Rathke cleft cyst and resolved the symptoms. Postoperative follow-up studies at 6 months demonstrated normal endocrine function and no evidence of a cyst.
Walavan Sivakumar, Chad D. Cole, and William T. Couldwell
Sarah T. Garber, Walavan Sivakumar, and Richard H. Schmidt
Dabigatran etexilate is an oral anticoagulant that acts as a direct, competitive thrombin inhibitor. Large randomized clinical trials have shown higher doses of dabigatran (150 mg taken twice daily) to be superior to warfarin in terms of stroke and systemic embolism rates in patients with nonvalvular atrial fibrillation. As a result, in 2010 the US FDA approved the use of dabigatran for the prevention of stroke and systemic embolism in patients with atrial fibrillation. Dabigatran is especially attractive in the outpatient setting because patients do not require routine monitoring with prothrombin times or international normalized ratios. To date, no effective reversal agent for dabigatran in the event of catastrophic hemorrhage has been identified. The authors report a case of an elderly patient, being treated with dabigatran for atrial fibrillation, who presented with a rapidly expanding intracranial hemorrhage after a ground-level fall. This case highlights an impending neurosurgical quandary of complications secondary to this new anticoagulation agent and suggests potential options for management.
Walavan Sivakumar, J. Bradley Elder, and Mark H. Bilsky
Anterior cervical discectomy and fusion (ACDF) is a common neurosurgical procedure, and the benefits, long-term outcomes, and complications are well described in the literature. The development of a juxtafacet joint cyst resulting in radiculopathy is a rare outcome after ACDF and merits further description. The authors describe a patient in whom a juxtafacet joint cyst developed after ACDF procedures, resulting in surgical intervention. When a juxtafacet joint cyst develops after ACDF, symptoms can include radiculopathy, neck pain, and neurological symptoms such as paresthesias and motor weakness. The presence of a juxtafacet joint cyst implies instability in that region of the spine. Patients with this pathological entity may require decompression of neural elements and fusion across the segment involved with the cyst.
Walavan Sivakumar, Roukoz Chamoun, Vinh Nguyen, William T. Couldwell, and Ph.D.
Pituitary incidentalomas are a common finding with a poorly understood natural history. Over the last few decades, numerous studies have sought to decipher the optimal evaluation and treatment of these lesions. This paper aims to elucidate the current evidence regarding their prevalence, natural history, evaluation, and management.
A search of articles on PubMed (National Library of Medicine) and reference lists of all relevant articles was conducted to identify all studies pertaining to the incidence, natural history, workup, treatment, and follow-up of incidental pituitary and sellar lesions, nonfunctioning pituitary adenomas, and incidentalomas.
The reported prevalence of pituitary incidentalomas has increased significantly in recent years. A complete history, physical, and endocrinological workup with formal visual field testing in the event of optic apparatus involvement constitutes the basics of the initial evaluation. Although data regarding the natural history of pituitary incidentalomas remain sparse, they seem to suggest that progression to pituitary apoplexy (0.6/100 patient-years), visual field deficits (0.6/100 patient-years), and endocrine dysfunction (0.8/100 patient-years) remains low. In larger lesions, apoplexy risk may be higher.
While the majority of pituitary incidentalomas can be managed conservatively, involvement of the optic apparatus, endocrine dysfunction, ophthalmological symptoms, and progressive increase in size represent the main indications for surgery.
Michael Karsy, Jian Guan, Walavan Sivakumar, Jayson A. Neil, Meic H. Schmidt, and Mark A. Mahan
Genetic alterations in the cells of intradural spinal tumors can have a significant impact on the treatment options, counseling, and prognosis for patients. Although surgery is the primary therapy for most intradural tumors, radiochemothera-peutic modalities and targeted interventions play an ever-evolving role in treating aggressive cancers and in addressing cancer recurrence in long-term survivors. Recent studies have helped delineate specific genetic and molecular differences between intradural spinal tumors and their intracranial counterparts and have also identified significant variation in therapeutic effects on these tumors. This review discusses the genetic and molecular alterations in the most common intradural spinal tumors in both adult and pediatrie patients, including nerve sheath tumors (that is, neurofibroma and schwannoma), meningioma, ependymoma, astrocytoma (that is, low-grade glioma, anaplastic astrocytoma, and glioblastoma), hemangioblastoma, and medulloblastoma. It also examines the genetics of metastatic tumors to the spinal cord, arising either from the CNS or from systemic sources. Importantly, the impact of this knowledge on therapeutic options and its application to clinical practice are discussed.
Gmaan Alzhrani, Yair M. Gozal, Ilyas Eli, Walavan Sivakumar, Amol Raheja, Douglas L. Brockmeyer, and William T. Couldwell
Surgical treatment of pathological processes involving the ventral craniocervical junction (CCJ) traditionally involves anterior and posterolateral skull base approaches. In cases of bilateral extension, when lesions extend beyond the midline to the contralateral side, a unilateral corridor may result in suboptimal resection. In these cases, the lateral extent of the tumor will prevent extirpation of the lesion via anterior surgical approaches. The authors describe a unilateral operative corridor developed along an extreme lateral trajectory to the anterior aspect of the clival and upper cervical dura, allowing exposure and resection of tumor on the contralateral side. This approach is used when the disease involves the bone structures inherent to stability at the anterior CCJ.
To achieve exposure of the ventral CCJ, an extreme lateral transcondylar transodontoid (ELTO) approach was performed with transposition of the ipsilateral vertebral artery, followed by drilling of the C1 anterior arch. Resection of the odontoid process allowed access to the contralateral component of lesions across the midline to the region of the extracranial contralateral vertebral artery, maximizing resection.
Exposure and details of the surgical procedure were derived from anatomical cadavers. At the completion of cadaveric dissection, morphometric measurements of the relevant anatomical landmarks were obtained. Illustrative case examples for approaching ventral CCJ chordomas via the ELTO approach are presented.
The ELTO approach provides a safe and direct surgical corridor to treat complex lesions at the ventral CCJ with bilateral extension through a single operative corridor. This approach can be combined with other lateral approaches or posterior infratemporal approaches to remove more extensive lesions involving the rostral clivus, jugular foramen, and temporal bone.
Vijay M. Ravindra, Jay Riva-Cambrin, Walavan Sivakumar, Ryan R. Metzger, and Robert J. Bollo
Computed tomography angiography (CTA) is frequently used to examine patients for blunt cerebrovascular injury (BCVI) after cranial trauma, but the pediatric population at risk for BCVI is poorly defined. Although CTA is effective for BCVI screening in adults, the increased lifetime risk for malignant tumors associated with this screening modality warrants efforts to reduce its use in children. The authors' objective was to evaluate the incidence of BCVI diagnosed by CTA in a pediatric patient cohort and to create a prediction model to identify children at high risk for BCVI.
Demographic, clinical, and radiographic data were collected retrospectively for pediatric patients who underwent CTA during examination for traumatic cranial injury from 2003 through 2013. The primary outcome was injury to the carotid or vertebral artery diagnosed by CTA.
The authors identified 234 patients (mean age 8.3 years, range 0.04–17 years, 150 [64%] boys) who underwent CTA screening for BCVI. Of these, 24 (10.3%) had a focal neurological deficit, and 153 (65.4%) had intracranial hemorrhage on a head CTA. Thirty-seven BCVIs were observed in 36 patients (15.4%), and 16 patients (6.8%) died. Multivariate regression analysis identified fracture through the carotid canal, petrous temporal bone fracture, Glasgow Coma Scale (GCS) score of < 8, focal neurological deficit, and stroke on initial CT scan as independent risk factors for BCVI. A prediction model for identifying children at high risk for BCVI was created. A score of ≤ 2 yielded a 7.9% probability of BCVI and a score of ≥ 3 a risk of 39.3% for BCVI.
For cranial trauma in children, fracture of the petrous temporal bone or through the carotid canal, focal neurological deficit, stroke, and a GCS score of < 8 are independent risk factors for BCVI.
Walavan Sivakumar, Gabriel Zada, Parham Yashar, Steven L. Giannotta, George Teitelbaum, and Donald W. Larsen
Spinal dural arteriovenous fistulas (DAVFs) are the most common spinal vascular malformations and can be a significant cause of myelopathy, yet remain inefficiently diagnosed lesions. Over the last several decades, the treatment of spinal DAVFs has improved tremendously due to improvements in neuroimaging, microsurgical, and endovascular techniques. The aim of this paper was to review the existing literature regarding the clinical characteristics, classification, and endovascular management of spinal DAVFs.
A search of the PubMed database from the National Library of Medicine and reference lists of all relevant articles was conducted to identify all studies pertaining to spinal DAVFs, spinal dural fistulas, and spinal vascular malformations, with particular attention to endovascular management and outcomes.
The ability to definitively treat spinal DAVFs using endovascular embolization has significantly improved over the last several decades. Overall rates of definitive embolization of spinal DAVFs have ranged between 25 and 100%, depending in part on the embolic agent used and the use of variable stiffness microcatheters. The majority of recent studies in which N-butyl cyanoacrylate or other liquid embolic agents were used have reported success rates of 70–90%. Surgical treatment remains the definitive option in cases of failed embolization, repeated recanalization, or lesions not amenable to embolization. Clinical outcomes have been comparable to surgical treatment when the fistula and draining vein remain persistently occluded. Improvements in gait and motor function are more likely following successful treatment, whereas micturition symptoms are less likely to improve.
Endovascular embolization is an increasingly effective therapy in the treatment of spinal DAVFs, and can be used as a definitive intervention in the majority of patients that undergo modern endovascular intervention. A multidisciplinary approach to the treatment of these lesions is required, as surgery is required for refractory cases or those not amenable to embolization. Newer embolic agents, such as Onyx, hold significant promise for future therapy, yet long-term follow-up studies are required.
Gabriel Zada, Walavan Sivakumar, Dawn Fishback, Peter A. Singer, and Martin H. Weiss
Following successful transsphenoidal surgery in patients with growth hormone (GH)–secreting pituitary adenomas, a characteristic fluid diuresis has been described. In this paper the authors aimed to further analyze the degree of fluid diuresis as it relates to postoperative GH levels.
Between 2000 and 2008, 85 patients underwent transsphenoidal surgery for a GH-secreting adenoma at the USC University Hospital. A retrospective chart review was conducted. Postoperative fluid intake, output, and balance within 48 hours following surgery, as well as endocrinological data were recorded and analyzed. Patients with postoperative diabetes insipidus and those with insufficient data were excluded from analysis.
Seventy-one patients were included in the analysis. The mean age was 46 years (range 16–74 years). There were 36 males (51%) and 35 females (49%). Patients with negative fluid balances at 48 hours after surgery were more than twice as likely to have a GH level of < 1.5 ng/ml (55 vs 25%, p = 0.023). At 48 hours after surgery, patients with a negative overall fluid balance had a significantly lower median GH level than those with a positive overall fluid balance (1.3 vs 2.4 ng/ml, p = 0.039). This difference was even more pronounced in patients with microadenomas and a negative overall fluid balance. By 48 hours following surgery, patients with postoperative Day 1 GH levels < 1.5 ng/ml had, on average, experienced diuresis of fluid > 1.1 L (median 1.5 L) more than patients with GH levels > 1.5 ng/ml.
Successful resection of GH-secreting adenomas is associated with a more pronounced fluid diuresis and negative overall fluid balance within 48 hours following transsphenoidal surgery. Patients with a negative fluid balance by postoperative Day 2 have a higher likelihood of having significantly reduced postoperative GH levels that may correlate with long-term surgical remission.
Marcus D. Mazur, Walavan Sivakumar, Jay Riva-Cambrin, Jaes Jones, and Douglas L. Brockmeyer
Surgical arthrodesis for pediatric occipitocervical (OC) instability has a high rate of success in a wide variety of challenging circumstances; however, identifying potential risk factors can help to target variables that should be the focus of improvement. The aim of this paper was to examine risk factors predictive of failure in a population of patients who underwent instrumented OC arthrodesis using a uniform surgical philosophy.
The authors conducted a retrospective cohort study of pediatric patients who underwent OC fusion from 2001 to 2013 at a single institution to determine risk factors for surgical failure, defined as reoperation for revision of the arthrodesis or instrumentation. The primary study outcome was either radiographic confirmation of successful OC fusion or surgical failure requiring revision of the arthrodesis or instrumentation. The secondary outcome was the underlying cause of failure (hardware failure, graft failure, or infection). Univariate analysis was performed to assess the association between outcome and patient demographics, cause of OC instability, type of OC instrumentation, bone graft material, biological adjuncts, and complications.
Of the 127 procedures included, 20 (15.7%) involved some form of surgical failure and required revision surgery. Univariate analysis revealed that patients with deep wound infections requiring debridement were more likely to require surgical revision of the hardware or graft (p = 0.002). Subgroup analysis revealed that patients with skeletal dysplasia or congenital spinal anomalies were more likely to develop hardware failure than patients with other causes of OC instability (p = 0.020). Surgical failure was not associated with the method of C-2 fixation, type of rigid OC instrumentation, bone graft material, use of bone morphogenetic protein or biological adjuncts, cause of instability, sex, age, or having previous OC fusion operations.
Pediatric patients in the present cohort with postoperative wound infections requiring surgical debridement had higher surgical failure rates after OC fusion. Those with skeletal dysplasia and congenital spinal anomalies were more likely to require reoperation for hardware failure. Better understanding of the mode of surgical failure may enable surgeons to develop strategies to decrease the need for reoperation in pediatric patients with OC instability.