Operative Technique: Angiomatoid Fibrous Histiocytoma-Unique Case and Management.

Objective We describe the first jugular foramen angiomatoid fibrous histiocytoma (AFH) case and the first treatment with preoperative endovascular embolization. AFH is a rare intracranial neoplasm, primarily found in pediatric patient extremities. With an increase in AFH awareness and a well-described genetic profile, intracranial prevalence has also subsequently increased. Study Design We compare this case to previously reported cases using PubMed/Medline literature search, which was performed using the algorithm ["intracranial" AND "angiomatoid fibrous histiocytoma"] through December 2020 (23 manuscripts with 46 unique cases). Patient An 8-year-old female presented with failure to thrive and right-sided hearing loss. Work-up revealed an absence of right-sided serviceable hearing and a large jugular foramen mass. Angiogram revealed primary arterial supply from the posterior branch of the ascending pharyngeal artery, which was preoperatively embolized. Intervention Gross total resection was performed via a translabyrinthine approach. Conclusion The case presented is unique; the first reported AFH at the jugular foramen and the first reported case utilizing preoperative embolization. Preoperative embolization is a relatively safe technique that can improve the surgeon's ability to perform a maximally safe resection, which may decrease the need for adjuvant radiation in rare skull base tumors in young patients.

Rare Case of Pediatric Disseminated Choroid Plexus Papilloma: Literature Review and Call for Reclassification.

INTRODUCTION: Choroid plexus tumors are rare neuroectodermal tumors that arise from the choroid plexus. Choroid plexus papillomas (CPPs) represent the lowest grade of these types of tumors and have a WHO grade I designation. Despite their typical low grade, some CPPs can exhibit aggressive behaviors including parenchymal invasion and dissemination throughout the neuro-axis. Due to their association with the choroid plexus, patients with CPP commonly present with signs and symptoms of hydrocephalus and increased intracranial pressure. CASE PRESENTATION: A 2-year-old male presented in extremis with acute hydrocephalus and seizure. He was found to have a large left intraventricular mass with innumerable intraparenchymal and extra-axial cysts throughout his neuro-axis. A literature review revealed five similar disseminated CPP cases with innumerable lesions. This is the youngest reported patient with disseminated CPP and the first with multiple compressive lesions. Following cranial resection and thoracic decompression, the patient's lesions have remained stable (2 years of follow-up). A literature search of the PubMed/Medline databases was performed using the search terms ["disseminated choroid plexus papilloma" OR "choroid plexus papilloma" OR "metastatic choroid plexus papilloma"] up to March 2021. Articles were then screened for similar patient radiographic presentation and histological diagnosis. To mitigate publication bias, referenced articles were utilized to identify other case reports and case series. DISCUSSION/CONCLUSION: We describe a rare case of a lateral ventricle CPP with widespread leptomeningeal dissemination causing acute obstructive hydrocephalus and compressive myelopathy requiring cerebrospinal fluid diversion and intracranial resection followed by thoracic spine decompression. This case report serves to broaden knowledge of disseminated CPP and to encourage complete neuro-axis imaging for choroid plexus tumors. Additionally, we propose a naming paradigm refinement that includes radiographic characteristics.

Standardizing postoperative care for pediatric intradural Chiari decompressions to decrease length of stay.

OBJECTIVE: Amid national and local budget crises, cutting costs while maintaining quality care is a top priority. Chiari malformation is a relatively common pediatric neurosurgical pathology, and postoperative care varies widely. The postoperative course can be complicated by pain and nausea, which can extend the hospital stay. In this study, the authors aimed to examine whether instituting a standardized postoperative care protocol would decrease overall patient hospital length of stay (LOS) as well as cost to families and the hospital system. METHODS: A retrospective study of pediatric patients who underwent an intradural Chiari decompression with expansile duraplasty at a single institution from January 2016 to September 2019 was performed. A standardized postoperative care protocol was instituted on May 17, 2018. Pre- and postprotocol groups were primarily analyzed for demographics, LOS, and the estimated financial expense of the hospital stay. Secondary analysis included readmissions, opioid consumption, and follow-up. RESULTS: The analysis included 132 pediatric patients who underwent an intradural Chiari decompression with expansile duraplasty. The preprotocol group included 97 patients and the postprotocol group included 35 patients. Patient age ranged from 0.5 to 26 years (mean 9.5 years). The mean LOS preprotocol was 55.48 hours (range 25.90-127.77 hours), and the mean postprotocol LOS was 46.39 hours (range 27.58-77.38 hours). The comparison between means showed a statistically significant decrease following protocol initiation (95% CI 1.87-16.31 hours, p = 0.014). In the preprotocol group, 21 of 97 patients (22%) were discharged the first day after surgery compared with 14 of 35 patients (40%) in the postprotocol group (p = 0.045). The estimated cost of one night on the pediatric neurosurgical intermediate ward was approximately $4500, which gives overall cost estimates for 100 theoretical cases of $927,800 for the preprotocol group and $732,900 for the postprotocol group. CONCLUSIONS: By instituting a Chiari protocol, postoperative LOS was significantly decreased, which resulted in decreased healthcare costs while maintaining high-quality and safe care.

Incidence of symptomatic tethered spinal cord in pediatric patients presenting with neurofibromatosis types 1 and 2.

OBJECTIVE The relationship between a tethered cord (TC) and neurofibromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to define the incidence of TC in pediatric neurosurgical patients who present with NF. METHODS The authors performed a single-institution (tertiary care pediatric hospital) 10-year retrospective analysis of patients who were diagnosed with or who underwent surgery for a TC and/or NF. Clinical and radiological characteristics were analyzed, as was histopathology. RESULTS A total of 424 patients underwent surgery for a TC during the study period, and 67 patients with NF were seen in the pediatric neurosurgery clinic. Of these 67 patients, 9 (13%) were diagnosed with a TC, and filum lysis surgery was recommended. Among the 9 patients with NF recommended for TC-release surgery, 4 (44%) were female, the mean age was 8 years (range 4-14 years), the conus position ranged from L1-2 to L-3, and 3 (33%) had a filum lipoma, defined as high signal intensity on T1-weighted MR images. All 9 of these patients presented with neuromotor, skeletal, voiding, and/or pain-related symptoms. Histopathological examination consistently revealed dense fibroconnective tissue and blood vessels. CONCLUSIONS Despite the lack of any known pathophysiological relationship between NF and TC, the incidence of a symptomatic TC in patients with NF1 and NF2 who presented for any reason to this tertiary care pediatric neurosurgery clinic was 13%. Counseling patients and families regarding TC symptomatology might be indicated in this patient population.

Transfrontal and Transsphenoidal Approaches to Pediatric Craniopharyngioma: A National Perspective.

INTRODUCTION: This study compared transsphenoidal (TS) and transfrontal (TF) approaches to craniopharyngioma utilizing a national database. METHODS: The Kids' Inpatient Database (2003, 2006, and 2009) was surveyed for patients with a diagnosis of craniopharyngioma who underwent a subset of surgical interventions to compare TS and TF surgery. Demographics, hospital variables, and complications/comorbidities were analyzed with multivariate regression. RESULTS: 314 admissions (TS = 104, TF = 210) were identified. The mean age was 14.8 (TS) versus 9.8 (TF) years (p < 0.001). The mean number of diagnoses was 4.6 (TS) versus 6.2 (TF) (p < 0.001). Diabetes insipidus was associated with 38% (TS) and 69% (TF). Cerebrospinal fluid (CSF) leak affected 19% TS and 4% TF resections. Other complications and comorbidities included postoperative stroke (2% TS vs. 5% TF), panhypopituitarism (5 vs. 8%), death (0 vs. 1%), cranial nerve deficits (1 vs. 6%), thrombotic events (7 vs. 17%), and seizures (0 vs. 12%). 98% of patients were discharged home after a mean 6-day length of stay (LOS) after TS, whereas 90% of TF patients had a LOS of 12 days. TS cases were more likely to be privately insured (68%) and from higher income brackets (61%) than TF ones (56 and 2%, respectively) (p < 0.05). In multivariate regression models adjusting for age, sex, race, number of diagnoses, surgical approach, hospital volume, and insurance type, the TS approach was associated with an increased incidence of CSF leak (OR 10, p < 0.001). More documented diagnoses (OR 16-60, p < 0.01) and TF approach (OR 2.6, p < 0.01) were associated with an increased incidence of other complications and comorbidities. Age younger than 10 (ß-coefficient 2.3, p = 0.01), more diagnoses (ß-coefficient 1.2, p < 0.001), and TF approach (ß- coefficient 3.0, p < 0.01) were associated with increased LOS. A higher number of diagnoses were associated with nonhome discharge destinations (ß-coefficient 1.29, p < 0.001). CONCLUSION: TS surgery was associated with an increased incidence of CSF leak but shorter LOS; TF surgery was associated with an increased incidence of other complications. Patients undergoing TS surgery were more likely to have private insurance and a higher family income bracket.

Multicenter retrospective evaluation of the validity of the Thoracolumbar Injury Classification and Severity Score system in children.

OBJECTIVE The Thoracolumbar Injury Classification and Severity Score (TLICS) system was developed to streamline injury assessment and guide surgical decision making. To the best of the authors' knowledge, external validation in the pediatric age group has not been undertaken prior to this report. METHODS This study evaluated the use of the TLICS in a large retrospective series of children and adolescents treated at 4 pediatric medical centers (Texas Children's Hospital, Children's Healthcare of Atlanta, Riley Children's Hospital, and Doernbecher Children's Hospital). A total of 147 patients treated for traumatic thoracic or lumbar spine trauma between February 1, 2002, and September 1, 2015, were included in this study. Clinical and radiographic data were evaluated. Injuries were classified using American Spinal Injury Association (ASIA) status, Denis classification, and TLICS. RESULTS A total of 102 patients (69%) were treated conservatively, and 45 patients (31%) were treated surgically. All patients but one in the conservative group were classified as ASIA E. In this group, 86/102 patients (84%) had Denis type compression injuries. The TLICS in the conservative group ranged from 1 to 10 (mean 1.6). Overall, 93% of patients matched TLICS conservative treatment recommendations (score ≤ 3). No patients crossed over to the surgical group in delayed fashion. In the surgical group, 26/45 (58%) were ASIA E, whereas 19/45 (42%) had neurological deficits (ASIA A, B, C, or D). One of 45 (2%) patients was classified with Denis type compression injuries; 25/45 (56%) were classified with Denis type burst injuries; 14/45 (31%) were classified with Denis type seat belt injuries; and 5/45 (11%) were classified with Denis type fracture-dislocation injuries. The TLICS ranged from 2 to 10 (mean 6.4). Eighty-two percent of patients matched TLICS surgical treatment recommendations (score ≥ 5). No patients crossed over to the conservative management group. Eight patients (8/147, 5%) had a calculated TLICS of 4, which meant they were candidates for surgery or conservative therapy by TLICS criteria. Excluding these patients, the degree of agreement between TLICS and surgeon decision was deemed to be very good (κ = 0.878). CONCLUSIONS The TLICS results and recommendations matched treatment in 96% of conservative group cases. In the surgical group, TLICS recommendations matched treatment in 93% of cases. The TLICS recommendations and surgeon decision making displayed very good concordance. The TLICS appears to be effective in the classification of thoracic and lumbar spine injuries and in guiding treatment in the pediatric age group.

Patient-, procedure-, and hospital-related risk factors of allogeneic and autologous blood transfusion in pediatric spinal fusion surgery in the United States.

STUDY DESIGN: Cross-sectional study using data from the Health Care Cost and Utilization Project Kids' Inpatient Database. OBJECTIVE: Blood loss during spinal fusion surgery may lead to the need for transfusion. Preoperative identification of patient-related, procedure-related, or hospital-related risk factors for blood transfusion would allow for implementation of interventions designed to control excessive bleeding. SUMMARY OF BACKGROUND DATA: Several studies have analyzed predictors associated with transfusion in spinal fusion. Identified predictors include age, female sex, anemia, comorbidities, number of fusion levels, osteotomy, and greater hospital volume. There have been few studies examining these predictors in children undergoing spinal fusion. METHODS: Using Kids' Inpatient Database data, univariate and multivariate logistic regression was used to calculate unadjusted and adjusted odds ratios (aOR). P values of less than 0.05 were considered statistically significant. RESULTS: We identified 9538 pediatric hospitalizations (patients <21 yr) with spinal fusion in 2009. Overall, 25.1% were associated with blood transfusion. The following factors were associated with transfusions: female sex (aOR 1.14, P = 0.023), black race (aOR 1.35, P = 0.005), length of hospital stay (aOR 1.03, P < 0.001), anterior approach/lumbar segment (aOR 2.11, P = 0.011) and posterior approach/lumbar segment (aOR 2.75, P < 0.001) compared with anterior approach/cervical segment, midlength fusion (aOR 1.71, P < 0.001), and long length fusion (aOR 2.85, P < 0.001) compared with short length. Higher transfusion rates were observed in patients with complications of fever and hematoma but not wound infection. CONCLUSION: This study showed significant patient-, procedure-, and hospital-related predictors of allogeneic and autologous blood transfusion in spinal fusion in the pediatric age group. Higher health care resource utilization of length of stay and additional procedures are directed toward care of this transfused subgroup. Therapies to reduce blood loss and transfusion requirement are necessary for this pediatric population. LEVEL OF EVIDENCE: 4.

Increased spasticity from a fracture in the baclofen catheter caused by Charcot spine: case report.

In patients with Charcot spine, a loss of normal feedback response from the insensate spine results in spinal neuropathy. Increasing deformity, which can manifest as sitting imbalance, crepitus, or increased back pain, can result. We present the case of a patient with a high-thoracic spinal cord injury (SCI) who subsequently developed a Charcot joint at the T10-11 level that resulted in a dramatic increase in previously controlled spasticity after fracture of an existing baclofen catheter. The 68-year-old man with T4 paraplegia presented with increasing baclofen requirements and radiographic evidence of fracture of the intrathecal baclofen catheter with an associated Charcot joint with extensive bony destruction. The neuropathic spinal arthropathy caused mechanical baclofen catheter malfunction and resulting increased spasticity. The patient was found to have transected both his spinal cord and the baclofen catheter. Treatment consisted of removal of the catheter and stabilization with long-segment instrumentation and fusion from T6 to L2. Follow-up radiographs obtained a year and a half after surgery showed no evidence of hardware failure or significant malalignment. The patient has experienced resolution of symptoms and does not require oral or intrathecal baclofen. This is the only reported case of a Charcot spine causing intrathecal catheter fracture, leading to increased spasticity. This noteworthy case suggests that late spinal instability should be considered in the setting of SCI and increased spasticity.

Multilevel thoracic hemangioma with spinal cord compression in a pediatric patient: case report and review of the literature.

PURPOSE: Vertebral hemangiomas are common benign vascular tumors of the spine. It is very rare for these lesions to symptomatically compress neural elements. If spinal cord compression does occur, it usually involves only a single level. Multilevel vertebral hemangiomas causing symptomatic spinal cord compression have never been reported in the pediatric population to the best of our knowledge. METHODS: We report the case of a 15-year-old boy presenting with progressive paraparesis due to thoracic spinal cord compression from a multilevel thoracic hemangioma (T5-T10) with epidural extension. RESULTS: Because of his progressive neurological deficit, he was initially treated with urgent multilevel decompressive laminectomies from T4 to T11. This was to be followed by radiotherapy for residual tumor, but the patient was unfortunately lost to follow-up. He re-presented 3 years later with recurrent paraparesis and progressive disease. This was treated with urgent radiotherapy with good response. As of 6 months follow-up, he has made an excellent neurological recovery. CONCLUSIONS: In this report, we present the first case of a child with multilevel vertebral hemangiomas causing symptomatic spinal cord compression and review the literature to detail the pathophysiology, management, and treatment of other cases of spinal cord compression by vertebral hemangiomas.

Cervical spine metastases: techniques for anterior reconstruction and stabilization.

The surgical management of cervical spine metastases continues to evolve and improve. The authors provide an overview of the various techniques for anterior reconstruction and stabilization of the subaxial cervical spine after corpectomy for spinal metastases. Vertebral body reconstruction can be accomplished using a variety of materials such as bone autograft/allograft, polymethylmethacrylate, interbody spacers, and/or cages with or without supplemental anterior cervical plating. In some instances, posterior instrumentation is needed for additional stabilization.

Capillary telangiectasias: clinical, radiographic, and histopathological features. Clinical article.

OBJECT: Brain capillary telangiectasias (BCTs) are small, clinically benign, angiographically occult lesions that are usually incidental findings. Large capillary telangiectasias have not been reported previously as most BCTs are very small. Symptomatic BCTs are also rare, with few reports in the literature. The authors review the clinical manifestations, imaging, and histopathological characteristics of BCTs to further elucidate the diagnostic and clinical features of these vascular malformations. METHODS: The authors completed a retrospective radiological review of all cases of BCTs in the neuroradiology database at the University of Utah involving patients treated between January 1993 and December 2007. The MR imaging scans were reviewed, and the BCT was measured in 2 dimensions. They arbitrarily chose > 1 cm to define a large BCT as a majority of these lesions were smaller than that. The medical chart and the electronic database were used to gather each patient's clinical information. RESULTS: One hundred thirty patients were identified in the archived neuroradiology database of capillary telangiectasias. Cases involving 105 patients with definite capillary telangiectasias were reviewed, and from these, 7 patients were identified to have a large capillary telangiectasia measuring > 1 cm. Upon further review, 2 of these patients were identified as having symptoms likely related to their capillary telangiectasia. These 2 cases are reported in the article. No patients with smaller BCTs were found to have symptoms related to their lesion. CONCLUSIONS: Brain capillary telangiectasias are small vascular malformations that rarely cause symptoms. They are often overlooked on imaging because of their clinically benign nature; however, they have been misdiagnosed as glial tumors in the past. Specific MR imaging sequences (T1-weighted postcontrast and gradient refocused echo) are valuable in aiding diagnosis, as histopathological diagnosis is often not possible. These cases highlight that BCTs can cause symptoms, a finding that may actually be related to the size of the lesion (28.6% of large BCTs in this series were symptomatic, whereas none of the small ones were).

Aneurysmal bone cyst of the petrous bone: case presentation and review of the literature.

Aneurysmal bone cysts (ABC) were originally described by Jaffe and Lichtenstein [Arch Surg 1942;44:1004-1025] in 1942 as nonneoplastic benign lesions with obscure pathogenesis. ABC occurring in the temporal bone are uncommon. Those arising from the petrous portion of the temporal bone are exceedingly rare. We report a right petrous ABC in a 16-year-old girl who presented clinically with hearing loss, facial weakness, and facial numbness and tingling. Her symptoms were preceded by a coincidental traumatic concussion 4 months earlier. Preoperative magnetic resonance imaging and computed tomography findings were both consistent with an ABC, although the lesion was noted to be in a very unusual location. Surgical resection was performed with adjuvant preoperative embolization. The patient recovered complete facial sensation and movement, and follow-up imaging documented complete resection.

The utility of MRI in the evaluation of odontoid fractures.

STUDY DESIGN: Retrospective radiographic review. OBJECTIVE: The purpose of this study was to reassess the utility of magnetic resonance imaging (MRI) in the assessment of type II and shallow type III odontoid fractures. SUMMARY OF BACKGROUND DATA: The authors of previous studies have reported a 10% incidence of transverse atlantal ligament (TAL) injury with odontoid fractures and suggested that all odontoid fractures be evaluated preoperatively with MRI. METHODS: A retrospective radiographic review was performed on all odontoid fractures treated with anterior screw fixation from 1987 to 2006. Patients were not screened for TAL injury with MRI or dynamic radiographs before surgery. Each patient had dynamic studies using intraoperative fluoroscopy after screw placement. Evidence of TAL injury was also evaluated on follow-up radiographs by measuring the atlantodental interval (ADI) on neutral, flexion, and extension films. For the purpose of this study, an ADI>3 mm indicated possible TAL injury. Neutral follow-up radiographs were available for 77 patients (mean follow-up, 17.5 mo), and flexion/extension films were available for 34 patients (mean follow-up, 16.4 mo). The mean ADI of the patients with neutral films was 1.1 mm (range=0.5 to 2.1 mm). The mean ADI of the patients with flexion/extension films was 1.2 mm (range=0.6 to 1.8 mm) for flexion and 1.2 mm for extension (range=0.5 to 2.8 mm). There was no evidence of atlantoaxial instability to suggest TAL disruption. CONCLUSIONS: The results of our study demonstrate that the patients with type II and shallow type III odontoid fractures do not require MRI screening for TAL injury. We found no cases of patients with late instability to suggest that a TAL injury was missed.

Transient facial nerve palsy after topical papaverine application during vestibular schwannoma surgery. Case report.

Some evidence in the literature supports the topical application of papaverine to the cochlear nerve to prevent internal auditory artery vasospasm and cochlear ischemia as a method of enhancing the ability to preserve hearing during acoustic neuroma surgery. The authors report a case of transient facial nerve palsy that occurred after papaverine was topically applied during a hearing preservation acoustic neuroma removal. A 58-year-old woman presented with tinnitus and serviceable sensorineural hearing loss in her right ear (speech reception threshold 15 dB, speech discrimination score 100%). Magnetic resonance imaging demonstrated a 1.5-cm acoustic neuroma in the right cerebellopontine angle (CPA). A retrosigmoid approach was performed to achieve gross-total resection of the tumor. During tumor removal, a solution of 3% papaverine soaked in a Gelfoam pledget was placed over the cochlear nerve. Shortly thereafter, the quality of the facial nerve stimulation deteriorated markedly. Electrical stimulation of the facial nerve did not elicit a response at the level of the brainstem but was observed to elicit a robust response more peripherally. There were no changes in auditory brainstem responses. Immediately after surgery, the patient had a House-Brackmann Grade V facial palsy on the right side. After several hours, this improved to a Grade I. At the 1-month follow-up examination, the patient exhibited normal facial nerve function and stable hearing. Intracisternal papaverine may cause a transient facial nerve palsy by producing a temporary conduction block of the facial nerve. This adverse effect should be recognized when topical papaverine is used during CPA surgery.