Correction to: Rathke's cleft cysts: a 6-year experience of surgery vs. observation with comparative volumetric analysis.

The original version of this article unfortunately contained errors in legend numbers of Figure 2 caption.

Rathke's cleft cysts: a 6-year experience of surgery vs. observation with comparative volumetric analysis.

BACKGROUND: Rathke's cleft cysts (RCCs) are common sellar lesions. Their management remains controversial, particularly when small or asymptomatic. Herein we review a consecutive series of RCC patients managed with surgery or observation. METHODS: All patients with a new diagnosis of presumed RCC, based on MRI, from February 2012-March 2018 were retrospectively divided into observational and surgical cohorts based on an intent-to-treat model. The cohorts were compared for clinical presentation, and cyst volume. The observational cohort was followed for change in cyst size. The surgical cohort was followed for changes in endocrinopathy, visual symptoms, headache and recurrence. RESULTS: Of 90 patients (mean age 36.7 ± 19.4 years; 68% female), 60% (n = 54) were in the observational cohort and 40% (n = 36) in the surgical cohort. Average follow-up was 13 ± 23 months in the observational cohort and 24 ± 19 months in the surgical group. In comparing the cohorts, mean ages were similar with more women in the surgical group (81% vs. 56%, p = 0.04). Most patients in the observational cohort had incidentally-discovered RCCs (n = 50, 88%) as opposed to the surgical cohort (n = 6, 17%). The surgical cohort had higher rates of headache (89% vs 26%, p < 0.001), endocrinopathy (36% vs 0%, p < 0.001), and visual dysfunction (19% vs 0%, p = 0.001). Mean cyst volume and maximal cyst dimensions were greater in the surgical cohort (0.94 ± 0.77 cm3 and 14.2 ± 4.1 mm), compared to the observational cohort (0.1 ± 0.14 cm3 and 6.4 ± 3 mm), (p < 0.001). Among the 53% (n = 30/54) of patients in the observational group with follow-up, 3 (10%) had spontaneous RCC shrinkage, 1 (3%) had modest asymptomatic growth (at 10 months from initial MRI), and 87% had stable cyst size. Of the 36 patients recommended to have surgery, 89% (n = 32) did so. Post-operatively, complete or partial resolution of headache, endocrinopathy and visual dysfunction were documented in 90% (n = 28/30), 75% (n = 10/12), and 100% (n = 7/7), respectively. On follow-up MRI, 8 (22%) patients had some cyst reaccumulation, of whom 3 (8%) were symptomatic and underwent uneventful reoperation. No major complications such as hematoma, CSF leak, new endocrinopathy or visual deficits occurred. CONCLUSION: From this consecutive series, a majority (60%) of RCCs do not appear to warrant surgical intervention and have a low risk of cyst progression. However, surgical cyst removal appears to be indicated and safe for patients with larger, symptomatic RCCs. Simple cyst drainage has a high rate of improvement in pituitary gland function, visual function and headache resolution with low complication rates and symptomatic recurrence risk. These findings stress the importance of careful case selection and potential utility of volumetric assessment for patients with RCCs.

Pregnancy-associated Cushing's disease? An exploratory retrospective study.

PURPOSE: In most clinical series of Cushing's disease (CD), over 80% of patients are women, many of whom are of reproductive age. The year following pregnancy may be a common time to develop CD. We sought to establish the incidence of CD onset associated with pregnancy. METHODS: A retrospective review was conducted for patients with biochemically-proven CD. Demographics, clinical history, biochemistry, imaging, pathology, and outcomes were reviewed. Pregnancy-associated CD was defined as symptom onset within 1 year of childbirth. RESULTS: Over 10 years, 77 patients including 64 women (84%), with CD underwent endonasal surgery. Of the 64 women, 64% were of reproductive age (15-45 years) at the time of diagnosis, and 11 (27%) met criteria for pregnancy-associated CD. Of these 11 women, median number of pregnancies prior to onset of CD was 2 (range 1-4) compared to zero (range 0-7) for 30 other women with CD onset during reproductive age (p = 0.0024). With an average follow-up of 47 ± 34 months, sustained surgical remission rates for woman with pregnancy-associated CD, other women of reproductive age, and women not of reproductive age were 91%, 80% and 83%, respectively. The average lag-time from symptom onset to diagnosis for women with pregnancy-associated CD was 4 ± 2 years. CONCLUSIONS: In this exploratory study, over one quarter of women of reproductive age with CD appeared to have symptomatic disease onset within 1 year of childbirth. This relatively high rate of pregnancy-associated CD suggests a possible causal relationship related to the stress of pregnancy and pituitary corticotroph hyperactivity in the peripartum period. This possible association suggests a heightened degree of clinical suspicion and biochemical testing for CD may be warranted after childbirth. Further study of this possible link between pregnancy and CD is warranted.

Imaging-Ambiguous Lesions of Meckel's Cave-Utility of Endoscopic Endonasal Transpterygoid Biopsy.

INTRODUCTION: Meckel's cave is a dural-lined cavity in the middle fossa skull base in which lies the Gasserian ganglion, a potential site for tumors and inflammatory lesions. A variety of lesions can be predominantly isolated to Meckel's cave, including extension from head and neck cancers, other malignant tumors, as well as benign lesions. Clinical presentation and imaging findings are often insufficient to establish a diagnosis. Hence, histologic confirmation is required to determine the appropriate treatment strategy. Several surgical approaches have been used to reach this deep-seated area, often with significant morbidity and prolonged recovery. Given advancements in endoscopy and greater facility with the technique, the endoscopic endonasal approach has been used increasingly to reach lesions in the region. METHODS: A single-institution, retrospective chart review over a 10-year period was performed to identify and describe patients with pathologically differing but imaging-similar lesions with their epicenter in Meckel's cave. RESULTS: Of a total of 21 cases of lesions in Meckel's cave approached by an endoscopic endonasal transpterygoid approach, we present 6 patients with imaging-ambiguous lesions involving Meckel's cave that were biopsied via the extended endoscopic endonasal approach. Among this diverse group, pathology included B-cell lymphoma, squamous cell carcinoma, adenocarcinoma, malignant schwannoma, benign schwannoma, and neurosarcoidosis. CONCLUSIONS: We explore not only the relevance of this approach in the armamentarium of the modern skull-base surgeon but also its limitations and conclude that the endoscopic endonasal approach provides a safe and relatively direct, minimally invasive corridor to many lesions of Meckel's cave.

Early Recognition and Initiation of Temozolomide Chemotherapy for Refractory, Invasive Pituitary Macroprolactinoma with Long-Term Sustained Remission.

OBJECTIVE: Invasive, medically refractory, and multiply recurrent pituitary adenomas pose a rare, but nevertheless significant, challenge for conventional management modalities. Temozolomide (TMZ) has been reported to be useful as an adjunctive treatment for some patients. We describe the efficacy of TMZ when used early in the management of invasive prolactinoma. CASE DESCRIPTION: A 56-year-old man presented with an invasive, refractory macroprolactinoma for which long-term dopamine agonists, stereotactic radiosurgery, and multiple transsphenoidal surgical resections had failed. He had experienced persistent hyperprolactinemia and tumor progression. Thus, TMZ was started. During the 11 cycles of TMZ therapy, the patient's prolactin level decreased from 696 ng/mL to 15.2 ng/mL, with a >90% decrease in tumor size. Nearly 6 years after discontinuing chemotherapy, the patient remained in sustained remission (prolactin level, 3.1 ng/mL) requiring only 1.5 mg of cabergoline weekly, without radiographic or clinical evidence of tumor recurrence. CONCLUSIONS: We conclude that TMZ can be efficacious in the management of medically and surgically refractory, invasive atypical prolactinomas, resulting in normalization of the prolactin levels and control of the tumor size. We encourage the inclusion of TMZ in the management of refractory, recurrent, and invasive prolactinomas, as a fourth-line treatment strategy, after dopamine agonist treatment, transsphenoidal resection, and radiation therapy. We especially advocate the early use of TMZ for aggressive and otherwise refractory prolactinomas.

Complications of Advanced Kadish Stage Esthesioneuroblastoma: Single Institution Experience and Literature Review.

INTRODUCTION: In esthesioneuroblastoma, greater disease extent and Kadish staging correlate with greater recurrence, complications, and mortality. These advanced stage malignancies require extensive resections and aggressive adjuvant therapy. This increases the risk of complications such as cerebrospinal fluid leak, neurologic deficits, and osteomyelitis. We present our case series and then analyze the literature to ascertain whether advanced stage tumors corresponds to greater rates of complications. METHODS: A retrospective review of consecutive patients with histologically-proven esthesioneuroblastoma who were aggressively managed at our institution was performed. This was followed by an extensive literature search of published original data, in large series from 2006-2016, where both surgery and adjuvant therapy were used for the treatment of esthesioneuroblastoma. RESULTS: Single institution review revealed eight patients with esthesioneuroblastoma, half with advanced Kadish staging. All Kadish A patients ( Kadish A: confined to nasal cavity) underwent endoscopic approaches alone, while Kadish C patients (Kadish C: extends beyond nasal cavity and paranasal sinuses) and D patients (Kadish D: lymph node or distant metastases) underwent craniofacial approaches, while all patients received post-operative adjuvant therapies. Complications such as cerebrospinal fluid (CSF) leak, seizures, meningitis, and abscess only occurred in high Kadish stage patients. Literature review demonstrated a higher proportion of advanced Kadish stage cases correlated with increasing rates of pneumocephalus, infection, and recurrence. A higher proportion of Kadish C and D tumors was inversely correlated with CSF leak rate and overall survival. DISCUSSION: Advanced stage tumors are often associated with a higher incidence of adverse events up to 33%, both due to disease burden and treatment effect. There is increasing use of endoscopy and neoadjuvant therapy, which have the potential to decrease complication rates. CONCLUSION: Advanced Kadish stage esthesioneuroblastoma necessitates meticulous surgical resection and aggressive adjuvant therapies, together, these increase the likelihood of adverse events, including CSF leak, neurologic deficits, and infections, and may represent the real morbidity cost of radically treating these tumors to achieve an improvement in overall survival. In selected patients, less-invasive approaches or neo-adjuvant therapies can be used without compromising on a curative resection.

Socioeconomic and regional differences in the treatment of cervical spondylotic myelopathy.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is the leading cause of spinal cord dysfunction in the world. Surgical treatment is both medically and economically advantageous, and can be achieved through multiple approaches, with or without fusion. We used the Nationwide Inpatient Sample (NIS) database to better elucidate regional and socioeconomic variances in the treatment of CSM. METHODS: The NIS database was queried for elective admissions with a primary diagnosis of CSM (ICD-9 721.1). This was evaluated for patients who also carried a diagnosis of anterior (ICD-9 81.02) or posterior cervical fusion (ICD-9 81.03), posterior cervical laminectomy (ICD 03.09), or a combination. We then investigated variances including regional trends and disparities according to hospital and insurance types. RESULTS: During 2002-2012, 50605 patients were electively admitted with a diagnosis of CSM. Anterior fusions were more common in Midwestern states and in nonteaching hospitals. Fusion procedures were used more frequently than other treatments in private hospitals and with private insurance. Median hospital charges were also expectedly higher for fusion procedures and combined surgical approaches. Combined approaches were found to be significantly greater in patients with concurrent diagnoses of ossification of the posterior longitudinal ligament (OPLL) and CSM. Ultimately, there has been an increased utilization of fusion procedures versus nonfusion treatments, over the past decade, for patients with cervical myelopathy. CONCLUSIONS: Fusion surgery is being increasingly used for the treatment of CSM. Expensive procedures are being performed more frequently in both private hospitals and for those with private insurance, whereas the most economical procedure, posterior cervical laminectomy, was underutilized.

Complex Skull Base Reconstructions in Kadish D Esthesioneuroblastoma: Case Report.

Introduction Advanced Kadish stage esthesioneuroblastoma requires more extensive resections and aggressive adjuvant therapy to obtain adequate disease-free control, which can lead to higher complication rates. We describe the case of a patient with Kadish D esthesioneuroblastoma who underwent multiple surgeries for infectious, neurologic, and wound complications, highlighting potential preventative and salvage techniques. Case Presentation A 61-year-old man who presented with a large left-sided esthesioneuroblastoma, extending into the orbit, frontal lobe, and parapharyngeal nodes. He underwent margin-free endoscopic-assisted craniofacial resection with adjuvant craniofacial and cervical radiotherapy and concomitant chemotherapy. He then returned with breakdown of his skull base reconstruction and subsequent frontal infections and ultimately received 10 surgical procedures with surgeries for infection-related issues including craniectomy and abscess evacuation. He also had surgeries for skull base reconstruction and CSF leak, repaired with vascularized and free autologous grafts and flaps, synthetic tissues, and CSF diversion. Discussion Extensive, high Kadish stage tumors necessitate radical surgical resection, radiation, and chemotherapy, which can lead to complications. Ultimately, there are several options available to surgeons, and although precautions should be taken whenever possible, risk of wound breakdown, leak, or infection should not preclude radical surgical resection and aggressive adjuvant therapies in the treatment of esthesioneuroblastoma.

Increased Utilization of Cervical Disk Arthroplasty in University Hospitals with Regional Variation and Socioeconomic Discrepancies.

OBJECTIVE: Treatment of cervical radiculopathy with disk arthroplasty has been approved by the U.S. Food and Drug Administration since 2007. Recently, a significant increase in clinical data including mid- and long-term follow-up has become available, demonstrating the superiority of disk arthroplasty compared with anterior discectomy and fusion. The aim of this project is to assess the nationwide use of cervical disk arthroplasty. METHODS: The University Healthcare Consortium database was accessed for all elective cases of patients treated for cervical radiculopathy caused by disk herniation (International Classification of Diseases [ICD] 722.0) from the fourth quarter of 2012 to the third quarter of 2015. Within this 3-year window, temporal and socioeconomic trends in the use of cervical disk replacement for this diagnosis were assessed. RESULTS: Three thousand four hundred forty-six cases were identified. A minority of cases (10.7%) were treated with disk arthroplasty. Median hospital charges were comparable for cervical disk replacement ($15,606) and anterior cervical fusion ($15,080). However, utilization was seen to increase by nearly 70% during the timeframe assessed. Disk arthroplasty was performed in 8% of patients in 2012 to 2013, compared with 13% of cases in 2015. Disk replacement use was more common for self-paying patients, patients with private insurance, and patients with military-based insurance. There was widespread variation in the use of cervical disk replacement between regions, with a nadir in northeastern states (8%) and a peak in western states (20%). CONCLUSION: Over a short, 3 -year period there has been an increase in the treatment of symptomatic cervical radiculopathy with disk arthroplasty. The authors predict a further increase in cervical disk arthroplasty in upcoming years.

Combined approaches to the skull base for intracranial extension of tumors via perineural spread can improve patient outcomes.

Many neoplasms of the head and neck extend centripetally, gaining access to the central nervous system via nerves through the skull base foramina. Often patients with perineural spread have been excluded from aggressive interventions given the overall poor prognosis and technical difficulty when addressing the perineural components. However, in carefully selected patients combined surgical approaches can provide the greatest potential for disease control as well as neural decompression for symptom relief. We performed a retrospective chart review of 20 consecutive patients who underwent skull base approaches for resection of tumors with intracranial extension via perineural spread from 2011 to 2014. Patients were evaluated for symptom change, surgical approaches, histopathology, adjuvant therapy, outcome, and prognosis. The most common presenting symptoms were pain or cranial nerve palsies. 55% of patients underwent endoscopic endonasal approaches, 50% transcranial approaches, and 15% underwent transfacial approaches. Overall 85% of patients reported symptom improvement in the post-operative period while 40% were completely asymptomatic following surgical resection. Ultimately, we observed a 45% mortality rate with an average survival of 8 months after diagnosis. In carefully selected patients, an aggressive multidisciplinary approach using a combination of surgical avenues to the skull base for the treatment of intracranial tumor via perineural extension can improve patient quality of life.

An aggressive multidisciplinary approach reduces mortality in rhinocerebral mucormycosis.

BACKGROUND: Rhinocerebral mucormycosis occurs in immunocompromised hosts with uncontrolled diabetes, solid organ transplants, and hematologic malignancies. Primary disease is in the paranasal sinuses but often progresses intracranially, via direct extension or angioinvasion. Rhinocerebral mucormycosis is rapidly fatal with a mortality rate of 85%, even when maximally treated with surgical debridement, antifungal therapy, and correction of underlying processes. METHODS: We performed a retrospective chart review of patients with rhinocerebral mucormycosis from 2011 to 2014. These patients were analyzed for symptoms, surgical and medical management, and outcome. We found four patients who were diagnosed with rhinocerebral mucormycosis. All patients underwent rapid aggressive surgical debridement and were started on antifungal therapy on the day of diagnosis. Overall, we observed a mortality rate of 50%. RESULTS: An early aggressive multidisciplinary approach with surgical debridement, antifungal therapy, and correction of underlying disease have been shown to improve survivability in rhinocerebral mucormycosis. CONCLUSION: A multidisciplinary approach to rhinocerebral mucormycosis with otolaryngology, neurosurgery, and ophthalmology, infectious disease and medical intensivists can help reduce mortality in an otherwise largely fatal disease. Even despite these measures, outcomes remain poor, and a high index of suspicion must be maintained in at-risk populations, in order to rapidly execute a multifaceted approach.

Lumbar corpectomy for correction of degenerative scoliosis from osteoradionecrosis reveals a delayed complication of lumbar myxopapillary ependymoma.

Osteoradionecrosis is a known complication following radiation therapy, presenting most commonly in the cervical spine as a delayed consequence of radiation that is often necessary in the management of head and neck cancers. In contrast, osteoradionecrosis has rarely been described in the lumbar spine. Here we describe, to our knowledge, the first reported case of lumbar spine osteoradionecrosis, after adjuvant radiation for a primary spinal cord tumor, leading to progressive degenerative scoliosis which required subsequent operative management. Established guidelines recommend that mature bone can tolerate a dose of up to 6000 cGy without injury. However, once bone has been exposed to radiation over this level progressive soft tissue changes may lead to devascularization, leaving the bone vulnerable to osteonecrosis, specifically when manipulated. Radiation necrosis can be progressive and lead to eventual mechanical instability requiring debridement and surgical fixation. In the setting of the lumbar spine, osseous necrosis can lead to biomechanical instability, deformity, pain, and neurologic deficit.

Morbidity and mortality of patients with endovascularly treated intracerebral aneurysms: does physician specialty matter?

OBJECT Endovascular treatment of cerebrovascular pathology, particularly aneurysms, is becoming more prevalent. There is a wide variety in clinical background and training of physicians who treat cerebrovascular pathology through endovascular means. The impact of clinical training background on patient outcomes is not well documented. METHODS The authors conducted a retrospective analysis of a large national database, the University HealthSystem Consortium, that was queried in the years 2009-2013. Cases of both unruptured cerebral aneurysms and subarachnoid hemorrhage treated by endovascular obliteration were studied. Outcome measures of morbidity and mortality were evaluated according to the specialty of the treating physician. RESULTS Elective embolization of an unruptured aneurysm was the procedure code and primary diagnosis, respectively, for 12,400 cases. Patients with at least 1 complication were reported in 799 cases (6.4%). Deaths were reported in 193 cases (1.6%). Complications and deaths were varied by specialty; the highest incidence of complications (11.1%) and deaths (3.0%) were reported by neurologists. The fewest complications were reported by neurosurgeons (5.4%; 1.4% deaths), with a higher incidence of complications reported in cases performed by neurologists (p < 0.0001 for both complications and deaths) and to a lesser degree interventional radiologists (p = 0.0093 for complications). Subarachnoid hemorrhage was the primary diagnosis and procedure for 8197 cases. At least 1 complication was reported in 2385 cases (29%) and deaths in 983 cases (12%). The number of complications and deaths varied among specialties. The highest incidence of complications (34%) and deaths (13.5%) in subarachnoid hemorrhage was in cases performed by neurologists. The fewest complications were in cases by neurosurgeons (27%), with a higher incidence of complications in cases performed by neurologists (34%, p < 0.0001), and a trend of increased complications with interventional radiologists (30%, p < 0.0676). The lowest incidence of mortality was in cases performed by neurosurgeons (11.5%), with a significantly higher incidence of mortality in cases performed by neurologists (13.5%, p = 0.0372). Mortality rates did not reach statistical significance with respect to interventional radiologists (12.1%, p = 0.4884). CONCLUSIONS Physicians of varied training types and backgrounds use endovascular treatment of ruptured and unruptured intracerebral aneurysms. In this study there was a statistically significant finding that neurosurgically trained physicians may demonstrate improved outcomes with respect to endovascular treatment of unruptured aneurysms in this cohort. This finding warrants further investigation.

Combined posterior hemiosteotomies and stabilization with lateral thoracotomy for en bloc resection of thoracic paraspinal primary bone tumors: technical note.

Primary bone tumors of the spine are rare entities with a poor prognosis if left untreated. En bloc excision is the preferred surgical approach to minimize the rate of recurrence. Paraspinal primary bone tumors are even less common. In this technical note the authors present an approach to the en bloc resection of primary bone tumors of the paraspinal thoracic region with posterior vertebral body hemiosteotomies and lateral thoracotomy. They also describe 2 illustrative cases.

Use of a stop-flow programmable shunt valve to maximize CNS chemotherapy delivery in a pediatric patient with acute lymphoblastic leukemia.

BACKGROUND: The requirement for frequent intraventricular drug delivery in the setting of shunt dependence is particularly challenging in the treatment of central nervous system infection, neoplastic disease, and hemorrhage. This is especially relevant in the pediatric population where both hematogenous malignancy requiring intrathecal drug delivery and shunt-dependent hydrocephalus are more prevalent. Intrathecal and intraventricular chemotherapy agents can be prematurely diverted in these shunt-dependent patients. CASE DESCRIPTION: We report the use of a stop-flow programmable shunt valve to maximize delivery of intraventricular chemotherapy in a child with acute lymphoblastic leukemia and disseminated intravascular coagulation who presented with spontaneous intracerebral and intraventricular hemorrhages. The patient then developed posthemorrhagic hydrocephalus and eventually progressed to shunt dependence but still required frequent intraventricular chemotherapy administration. A ventriculoperitoneal shunt, equipped with a valve that allows for near cessation of cerebrospinal fluid flow (Certas(®), Codman, Raynham, MA), and a contralateral Ommaya reservoir were inserted to maximize intraventricular dissemination of chemotherapy. CONCLUSIONS: To the best of our knowledge, this is the first reported case of the use of a high-resistance programmable valve being used to virtually cease cerebrospinal fluid flow through the distal catheter temporarily in order to maximize intraventricular drug dissemination in a pediatric patient with acute lymphoblastic leukemia.

Minimally invasive lateral transpsoas interbody fusion using a stand-alone construct for the treatment of adjacent segment disease of the lumbar spine: review of the literature and report of three cases.

We describe 3 patients who presented with radiographic signs and clinical symptoms of adjacent segment disease several years after undergoing L4-S1 posterior pedicle screw fusion. All patients underwent successful lateral lumbar interbody fusion (LLIF) at 1-2 levels above their previous constructs, using stand-alone cages, with complete resolution of radiculopathy and a significant improvement in low-back pain. In addition to a thorough analysis of these cases, we review the pertinent literature regarding treatment options for adjacent segment disease and the applications of the lateral lumbar interbody technique.

Short segment internal maxillary artery to middle cerebral artery bypass: a novel technique for extracranial-to-intracranial bypass.

BACKGROUND: Traditional high-flow extracranial-to-intracranial (EC-IC) bypass procedures require a cervical incision and a long (20-25 cm) radial artery or saphenous vein graft. This technical note describes a less invasive, EC-IC bypass technique using a short-segment (8-10 cm) of the radial artery to anastomose the internal maxillary artery (IMAX) to the middle cerebral artery. CLINICAL PRESENTATION: Anatomic dissections were performed on 6 cadaveric specimens to assess the location of the IMAX artery using an extradural middle fossa approach. Subsequently, the procedure was implemented in a patient with a giant fusiform internal carotid artery aneurysm. TECHNIQUE: A straight line was drawn anteriorly from the V2/V3 apex along the inferior edge of V2. The IMAX was found 8.6 mm on average anteriorly from the lateral edge of the foramen rotundum. We drilled to a depth of 4.2 mm on average to find the medial extent of the artery and then lateral and deep drilling exposed an average of 7.8 mm of graft. The IMAX was consistently found running just anterior and parallel to a line between the foramens rotundum and ovale. In the clinical case presented, both intraoperative indocyanine green and postoperative conventional angiography revealed a patent graft. The patient did well clinically without any new deficits. CONCLUSION: The advantages of this new technique include the avoidance of a long cervical incision and potentially higher patency rates secondary to shorter graft length than currently practiced.