A Rare Neonatal Intramedullary Immature Teratoma Undiagnosed in utero: A Case Report.

BACKGROUND AND IMPORTANCE: Immature teratoma is a known pediatric tumor. However, spinal variants are rare and can present both a diagnostic and therapeutic challenge, particularly regarding aggression as it pertains to extent of resection, likelihood of recurrence and concordant prognosis, and the need and efficacy of adjuvant therapies. CLINICAL PRESENTATION: The patient is a 27-day-old female who presented with 10 days of poor feeding, irritability, and progressive hypotonia. Although upon immediate presentation emergency providers' differential diagnoses included meningitis, inborn error of metabolism, and genetic neurodegenerative disease, a subsequent magnetic resonance (MR) imaging of the total spine revealed a large intradural intramedullary mass extending from the medulla to the thoracic cord at T12. The patient underwent multilevel cervical and thoracic laminectomies/laminoplasty for maximal safe resection. Histopathology revealed mostly mature tissue elements originating from all 3 germ layers, interspersed with foci of immature neuroepithelium, consistent with grade 1 immature teratoma. Following surgical intervention, the patient regained strength and spontaneous movement and underwent physical therapy. Follow-up MR imaging of the total spine was obtained every 3 months, and at 9 months, recurrence was demonstrated, which was successfully treated with chemotherapy. Further surveillance MR imaging of the total spine has demonstrated cystic myelomalacia changes without definite tumor recurrence, at 5-year follow-up. Clinically, the patient has developed scoliosis without weakness, pain, or urinary symptoms. CONCLUSION: This case demonstrates an exceptionally rare and unusual variant neoplasm in a neonate and highlights the difficulty of diagnosis and the important role of MR imaging. It also illustrates the importance of gross total resection, the risk of recurrence, and the need for close radiographic follow-up of these lesions. It also provides a useful example of the efficacy of adjuvant chemotherapy in treating recurrence.

Medical student education in neurosurgery: optional or essential?

ISSUE: Current medical school curricula emphasize general practice principles, and this has led predictably to increasingly limited exposure to subspecialties, including neurosurgery. However, a significant amount of neurosurgical disease and/or emergencies present in primary care settings or emergency rooms. In light of an already acknowledged shortage of neurosurgery providers, this means that general practitioners should be well educated and prepared to diagnose and manage neurosurgical disease. Considering the devastating consequences of a missed or delayed neurosurgical diagnosis, limiting future physicians' exposure to the field of neurosurgery is not in the best interests of the patient. EVIDENCE: In this article, the authors review and discuss the results of several studies investigating the prevalence, presentation, diagnosis, and management of neurosurgical disease in emergency and general practice settings. They then discuss the current status of neurosurgical education in medical schools, both from the educators' and students' perspectives, and how this status might impact patient care. Finally, they offer suggestions for the improvement of neurosurgical education during medical school. IMPLICATIONS: Despite being considered highly subspecialized, neurosurgical diagnosis and care is a field in which all physicians should receive proper education and training. To properly serve patients and produce competent physicians, steps should be taken to re-emphasize the importance of neurosurgical education for medical students.

Temporary Posterior C1-C2 Instrumentation without Fusion for Treatment of Displaced Atlantoaxial Fractures.

OBJECTIVE: To describe open reduction internal fixation (ORIF) with posterior C1-C2 instrumentation without fusion to treat displaced atlantoaxial fractures with later instrumentation removal. METHODS: A retrospective review identified 14 patients (mean age 44 years) with displaced atlantoaxial fractures treated with ORIF without fusion. Patient demographics, fracture morphology, trauma etiology, instrumentation levels, timing of hardware removal, and complications were collected. Patients were maintained in a cervical collar, and healing was confirmed via imaging before instrumentation removal. RESULTS: Fractures included type III odontoid, C2 pars, C1 ring, and complex C1 or C2 fractures. All cases used C1 lateral mass screws and C2 pars or pedicle screws with a C1 cross-link. Two patients had C3 lateral mass screws. All patients showed fracture healing on imaging within 4 months after ORIF. Instrumentation removal was performed in 13 patients. No complications were noted. CONCLUSIONS: Displaced atlantoaxial fractures have been traditionally managed with halo-vest immobilization closed reduction or ORIF with fusion. ORIF without fusion and subsequent hardware removal is an alternative approach. This strategy preserves mobility at C1-C2, avoids halo-vest immobilization, and appears to be a safe option for treatment of atlantoaxial fractures.

Extracranial-intracranial bypass in a neonate with multiple ruptured middle cerebral artery aneurysms: illustrative case.

BACKGROUND: Reports of ruptured neonatal aneurysms are rare in neurosurgical literature. Pediatric aneurysms differ from adult aneurysms, notably in morphology, size, number, and risk of rerupture. Many authors report experience with clipping, citing durability and decreased use of radiation as benefits over endovascular intervention. Few authors report extracranial-to-intracranial bypass because small pediatric vessels make this option challenging. The authors discussed a case of a newborn with multiple ruptured aneurysms, one of the youngest reported cases involving extracranial-intracranial bypass. OBSERVATIONS: A 3-week-old baby presented with hemorrhage from multiple complex middle cerebral artery (MCA) aneurysms. Because of young age, endovascular intervention was not possible; therefore, the patient received craniotomy. Upon exploration, clip reconstruction was impossible; the vessel was trapped, and superficial temporal artery (STA)-MCA bypass was performed. The recipient vessel diameter was 0.3 mm. The postoperative course was complicated by seizures as well as symptomatic vasospasm, which was treated with intraarterial verapamil and ventriculostomy. At last follow-up, the patient was developing normally and was ambulatory with minimal deficit. LESSONS: This case, one of the youngest patients reported, highlighted details of pediatric aneurysm management, such as propensity for multiple/fusiform aneurysms and high risk of re-hemorrhage, with significant mortality. The authors recommended aggressive, early intervention in pediatric aneurysms at centers with surgeons familiar with both endovascular intervention and cerebral bypass.

Sectioning of C2 nerve roots during C1-2 fusion: report of aberrant vertebral artery during C2 nerve root sectioning. Illustrative case.

BACKGROUND: Sectioning the C2 nerve root is increasingly utilized during posterior C1-2 fusion, as the nerve overlies the entry point for C1 lateral mass screws and the C1-2 joint. Nerve sectioning improves visualization for screw placement and enables joint decortication for arthrodesis. While rare, vascular injury is a devastating complication of atlantoaxial fusion. Anomalous vascular anatomy at C1-2 greatly increases risk of iatrogenic injury. OBSERVATIONS: A 78-year-old female with rheumatoid arthritis and prior C2-7 fusion presented with myelopathy from a compressive pannus at C1-2. She underwent C1 laminectomy and C1-2 posterior instrumented fusion. Intraoperatively, arterial bleeding occurred as the right C2 nerve root was sectioned. Vertebral artery injury was suspected, and tamponade was performed while vascular control was established. The artery passed aberrantly beneath the nerve root in the C1-2 foramen. It was repaired microsurgically, and patency was confirmed using indocyanine green. The remainder of the fusion was aborted. The patient wore a cervical collar and was treated with aspirin for 6 weeks before undergoing instrumented fusion. The patient suffered no deficits. LESSONS: Although rare, anomalous vertebral artery anatomy increases risk of injury at time of C2 nerve root sectioning. Preoperative assessment of the vasculature is vital.

Interleaved programming of subthalamic deep brain stimulation to avoid adverse effects and preserve motor benefit in Parkinson's disease.

Subthalamic nucleus (STN) deep brain stimulation (DBS) is the most common surgical treatment for managing motor complications in Parkinson's disease (PD). Ultimately, outcomes depend on a variety of factors including lead location, access and expertize in programming and PD medical management. Nevertheless, achieving ideal programming settings can be difficult in certain patients, leading to suboptimal control of symptoms and stimulation-induced side effects, notably dysarthria and dyskinesia. Interleaved stimulation (ILS) is a newer programming technique that attempts to optimize the stimulation field, improving control of symptoms while minimizing stimulation-induced adverse effects. A retrospective chart review was performed on PD patients receiving STN DBS over the past 12 months. Clinical and demographic data were collected from patients identified as having received ILS. The rationale and clinical efficacy of ILS was analyzed. Nine patients received ILS due to incomplete PD symptom control or stimulation-induced side effects after attempting multiple programming options. Appropriate lead location was confirmed with postoperative MRI except in one case. Following ILS, patients reported improvement in symptoms and resolution of side effects, while preserving adequate control in Parkinsonism with a mean improvement in UPDRS-MOTOR scores of 51.2 %. ILS continues to emerge as a safe and effective programming strategy for maximizing symptom control in PD while diminishing stimulation-induced side effects.