Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature.

PURPOSE: Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings METHODS: Here we present a 9-year-old female patient with 4 months progressive headaches, personality changes, and vomiting. We also present a review of the current literature of intracranial ganglioneuroblastomas. RESULTS: Imaging demonstrated a partially calcified suprasellar mass measuring 4.6 × 6.3 × 5 cm composed of both solid and cystic components, diagnosed to be a ganglioneuroblastoma, with mass effect on the lateral and 3rd ventricles, with a midline shift of right to left of 6-7 mm. She was treated with subtotal surgical resection, an intensive chemotherapeutic regimen, and radiation and has no residual disease on imaging 1 year and 4 months status post-surgery. CONCLUSION: To our knowledge, this is the first case of a ganglioneuroblastoma to mimic a craniopharyngioma based upon imaging findings and suprasellar location. As these cases are extremely rare, an optimal therapeutic regimen has not been defined. However, a combination of surgical resection, chemotherapy, and radiation therapy can be effective, as shown here with successful treatment and no evidence of residual disease.

Intracranial malignant peripheral nerve sheath tumor variant: an unusual neurovascular phenotype sarcoma case invading through the petrous bone.

INTRODUCTION: Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent cranial nerve. METHODS: This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1). RESULTS: The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo. MRI was performed that demonstrated a large extra-axial tumor compressing the left infratemporal posterior temporal region. The tumor was associated with significant destruction of the superior portion of the petrous bone and extension through the petrous into the upper posterior fossa, immediately below the tentorium. The patient underwent surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide. Pathology demonstrated a variant malignant peripheral nerve sheath tumor with both nerve sheath and vascular phenotype by immunostains. The patient's symptoms improved following treatment. CONCLUSION: We present the first reported case of an intracranial MPNST variant that developed along the neurovascular bundle as a sarcoma with both nerve sheath and vascular phenotype through the petrous bone and not associated with a major cranial nerve or with stigmata of neurofibromatosis type 1 (NF1). Although this is an extremely unusual presentation due to location and lack of prominent cranial nerves in that location, it is not unusual for benign nerve sheath tumors to follow the neurovascular bundle through foramen of cortical long bone or pelvis. This case suggests that physicians should incorporate intracranial MPNST variant into their differential diagnosis in the cranium, even when tumor is not located near a prominent cranial nerve. Surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide has led to improvement in patient symptoms.

Choroid plexus: normal size criteria on neuroimaging.

PURPOSE: The lack of a well-recognized normal size criterion for the choroid plexus makes small or diffuse choroid plexus pathologies difficult to recognize. The purpose of this study was to determine the normal size of the choroid plexus in the pediatric population utilizing magnetic resonance imaging (MR) and ultrasound (US). As volume measurement across multiple slices is a laborious process, we wanted to propose a simple clinical tool that is easy to use, reproducible, and quick to obtain measurements. METHODS: This study retrospectively evaluated choroid plexus size in 90 children between the ages of 0 and 16. To determine the choroid plexus thickness, a total of 97 studies (71 MRIs and 26 Ultrasounds) were reviewed, from children without any signs of choroid plexus pathology; 6 measurements were taken from MR studies, and 3 measurements were taken from US studies. Averages and ranges of choroid plexus thickness were computed across age groups and gender. RESULTS: Across all ages, the mean choroid plexus thickness in the lateral ventricles was found to be 3.4, 3.3, and 3.1 mm in the axial, coronal, and sagittal views; 3.2 mm in the temporal horns; 2.5 mm in the fourth ventricle with the lateral limb of the choroid plexus in the fourth ventricle measuring 11.3 mm in length. No trends were observed with respect to age subgroups or gender. CONCLUSION: Choroid plexus thickness on average was 3.2 mm in the lateral ventricles and 2.5 mm in the fourth ventricle.

Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors' goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis. METHODS: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°). RESULTS: Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB-C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude. CONCLUSIONS: Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.

Improving Responsiveness to Patient Phone Calls: A Pilot Study.

Optimal patient-physician communication in the outpatient clinical setting is critical for safe and effective patient care. Keeping track of multiple patient telephone messages can be difficult and hazardous if a structured system is not in place. A multidisciplinary group at Hershey Medical Center developed a standardized approach for addressing patient telephone calls at their outpatient surgical clinics. This program was designed to improve the patient experience by providing a realistic time frame for phone calls to be returned and requests fulfilled. Additionally, this system permitted phone calls to be tracked and documented appropriately and allowed for prioritization of urgent and emergent messages. Our intent for this program was to close potential gaps within the communication chain at our outpatient surgical clinics, improve overall communication between clinicians and their patients, and improve both patient and employee satisfaction.

Results of endoscopic third ventriculostomy.

ETV is emerging as the treatment of choice for aqueductal stenosis caused by anatomic, inflammatory, and selected neoplastic etiologies. The technique has also proven useful in the pathologic diagnosis and treatment of these conditions. Long-term results of this procedure and comparison to standard shunting procedures are necessary to define indications for patients with pathologic findings in the intermediate response groups. Development of new studies for pre-operative assessment of CSF absorptive capacity and quantitative postoperative measures of ventriculostomy function would be invaluable additions to our ability to assess candidates for this procedure and their eventual outcome. Further study and technical refinements will, no doubt, lead to many more potential uses for these procedures in the treatment of hydrocephalus and its associated etiologies. The challenge for neuro-surgeons will be to define the operative indications and outcomes, while refining techniques for safely performing these useful procedures.

Perceived benefits and barriers to a career in pediatric neurosurgery: a survey of neurosurgical residents.

OBJECT: Research suggests that there may be a growing disparity between the supply of and demand for both pediatric specialists and neurosurgeons. Whether pediatric neurosurgeons are facing such a disparity is disputable, but interest in pediatric neurosurgery (PNS) has waxed and waned as evidenced by the number of applicants for PNS fellowships. The authors undertook a survey to analyze current neurosurgical residents' perceptions of both benefits and deterrents to a pediatric neurosurgical career. METHODS: All residents and PNS fellows in the United States and Canada during the academic year 2008-2009 were invited to complete a Web-based survey that assessed 1) demographic and educational information about residents and their residency training, particularly as it related to training in PNS; 2) residents' exposure to mentoring opportunities from pediatric neurosurgical faculty and their plans for the future; and 3) residents' perceptions about how likely 40 various factors were to influence their decision about whether to pursue a PNS career. RESULTS: Four hundred ninety-six responses were obtained: 89% of the respondents were male, 63% were married, 75% were in at least their 3rd year of postgraduate training, 61% trained in a children's hospital and 29% in a children's "hospital within a hospital," and 72% were in programs having one or more dedicated PNS faculty members. The residencies of 56% of respondents offered 6-11 months of PNS training and nearly three-quarters of respondents had completed 2 months of PNS training. During medical school, 92% had been exposed to neurosurgery and 45% to PNS during a clinical rotation, but only 7% identified a PNS mentor. Nearly half (43%) are considering a PNS career, and of these, 61% are definitely or probably considering post-residency fellowship. On the other hand, 68% would prefer an enfolded fellowship during residency. Perceived strengths of PNS included working with children, developing lasting relationships, wider variety of operations, fast healing and lack of comorbidities, and altruism. Perceived significant deterrents included shunts, lower reimbursement, cross-coverage issues, higher malpractice premiums and greater legal exposure, and working with parents and pediatric health professionals. The intrinsic nature of PNS was listed as the most significant deterrent (46%) followed by financial concerns (25%), additional training (12%), longer work hours (12%), and medicolegal issues (4%). The majority felt that fellowship training and PNS certification should be recommended for surgeons treating of all but traumatic brain injuries and Chiari I malformations and performing simple shunt-related procedures, although they felt that these credentials should be required only for treating complex craniosynostosis. CONCLUSIONS: The nature of PNS is the most significant barrier to attracting residents, although reimbursement, cross-coverage, and legal issues are also important to residents. The authors provide several recommendations that might enhance resident perceptions of PNS and attract trainees to the specialty.

Primary intramedullary spinal cord germinoma.

Primary intramedullary spinal cord germinoma (PISCG) is an exceedingly rare diagnosis, with fewer than 30 cases reported in the literature. It is even less common in the pediatric population. Usually, initial imaging at patient presentation reveals a mass. The authors describe the unique case of a child whose initial imaging showed only focal spinal cord atrophy, which was the earliest sign of a slowly growing intramedullary lesion that was eventually proven via biopsy to represent a PISCG. The authors outline this child's diagnostically challenging presentation, review the events leading up to a diagnosis, briefly discuss PISCG, and summarize their recommendations for other physicians who may encounter a similar case. They assert that PISCG should be considered as a rare entity in the differential diagnosis of progressive spinal cord dysfunction even in the absence of an MRI abnormality of an intrinsic spinal cord mass, especially if there is unexplained focal atrophy of the cord.

Variability among pediatric neurosurgeons in the threshold for ventricular shunting in asymptomatic children with hydrocephalus.

OBJECT: The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds. METHODS: The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario. RESULTS: Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7-6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent's age, training, board certification, or type or location of practice. CONCLUSIONS: This study demonstrates that pediatric neurosurgeons' thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.