Cranial and spinal oligodendrogliomatosis: a case report and review of the literature.

INTRODUCTION: Oligodendrogliomatosis is a rarely reported entity in literature associated with poor prognosis in terms of length and quality of life. In this paper, we describe oligodendrogliomatosis in a 15-year-old male who initially presented with altered mental status due to diabetic ketoacidosis. CLINICAL PRESENTATION: He was refractory to temozolomide initially but demonstrated disease regression with radiotherapy (XRT). More recently, he has had disease recurrence, which was stabilized with temozolomide therapy for a period of time. CONCLUSION: Contrary to most reports in literature, our patient has had excellent quality of life since his initial diagnosis and continues to carry good prognosis. In addition to oligodendrogliomatosis, our patient also developed multiple intracranial cavernomas secondary to radiation therapy, which have remained stable and asymptomatic.

Postnatal myelomeningocele closure in smallest reported neonate: illustrative case.

BACKGROUND: Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors' knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair. OBSERVATIONS: A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age. LESSONS: To the authors' knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

The Re-Evaluation of Frailty in Predicting Complications after Long-Segment Spinal Fusion for Adult Spinal Deformity.

OBJECTIVE: To evaluate on a national scale how frailty status (stratified using the 5-item Modified Frailty Index (mFI-5)) affects the operative characteristics of and complications after long-segment spinal fusion (LSF) for adult spinal deformity (ASD). METHODS: Adults undergoing LSF of ≥3 vertebrae in the National Surgical Quality Improvement Program database years 2015-2020 were split into 2 cohorts: nonfrail with mFI = 0 or 1; frail with mFI ≥2. Demographics, operative characteristics, and 30-day complications were contrasted between the cohorts using the Student's t-test, the Fisher's exact test, or a multivariate analysis when appropriate. RESULTS: In the 340 LSF cases collected, 268 fell into the nonfrail cohort and 72 into the frail cohort. The frail cohort constituted a high rate of geriatric age (65.3% vs. 38.1%; P < 0.001), higher body mass index (32.9 ± 0.86 vs. 30.2 ± 0.39; P = 0.005), and more comorbidities in 9 of 14 measures. After surgery, the frail cohort experienced more urinary tract infections (odds ratio [OR], 3.33; confidence interval [CI], 1.01-10.94; P = 0.04). However, the frail cohort shared similarities with the nonfrail cohort in terms of length of stay (5.11 ± 0.51 vs. 6.01 ± 1.62 days; P = 0.60), home discharge (OR, 0.76; CI, 0.42-1.39; P = 0.38), readmission (OR, 2.45; CI, 0.87-6.89; P = 0.09), and overall rate of complications (OR, 0.89; CI, 0.50-1.59; P = 0.70). CONCLUSIONS: Despite trends found in past studies of ASD, this analysis showed that the frailty status of mFI ≥2 is a poor predictor of surgical and hospitalization course and overall complications in LSF when examined up to 30 days postoperatively.

Intraoperative neural monitoring during head and neck surgery in patients with concern for cervical spine instability.

Cervical and craniocervical instability are associated with catastrophic procedural outcomes. We discuss three individuals who required otolaryngologic surgical intervention: two with symptomatic spinal instability and one in whom spinal stability was unable to be assessed. Two cases were managed with procedural positioning precautions and evoked potential monitoring, and the other with procedural positioning precautions alone. Methods of monitoring and triggers for repositioning are discussed. This series is intended to discuss the approach and potential added value of evoked potential monitoring for risk mitigation in pediatric patients with concern for cervical spine instability.

Neurosurgical Evaluation and Management of Patients with Chromosomal Abnormalities.

Patients with chromosomal abnormalities are at risk for numerous neurosurgical pathologies, given the broad impact and multisystem involvement of these disorders. Down syndrome (trisomy 21), Edwards syndrome (trisomy 18), Patau syndrome (trisomy 13), Klinefelter syndrome (47,XXY), and velocardiofacial or DiGeorge syndrome (22q11.2 deletion) are particularly associated with neurosurgical concerns. Given the heterogeneity of concerns and presentations, these patients benefit from multidisciplinary care provided by teams familiar with their specific syndrome.

Safety and Quality in Syndromic Neurosurgery.

The developing field of syndromic neurosurgery has significant challenges and opportunities in quality and safety. Quality care must be safe, effective, patient-centered, timely, efficient, and equitable; the Donabedian model focused on system structures, processes, and outcomes is a helpful framework to guide improvement in these areas. Ultimately, a successful syndromic neurosurgery program will bring together an interested multidisciplinary team of experts who will grow care through open communication and steady improvement efforts.

Neurosurgical Evaluation and Management of Children with Achondroplasia.

Achondroplasia is the most common of skeletal dysplasias and is caused by a defect in endochondral bone formation. In addition to skeletal deformities, patients with achondroplasia possess significant abnormalities of the axial skeleton, including small skull base with a narrowed foramen magnum and small vertebral bodies with shortened pedicles. Consequently, patients with achondroplasia are at risk of several severe neurologic conditions, such as cervicomedullary compression, spinal stenosis, and hydrocephalus, which frequently require the attention of a neurosurgeon. This article provides an updated review on the neurosurgical evaluation and care of children with Achondroplasia.

Neurosurgical Evaluation and Management of Adults with Achondroplasia.

Much of the current medical discussion for within centers for skeletal dysplasia and specifically patients with achondroplasia focuses on infancy and early childhood. Most neurosurgical concerns arise due to a defect in the endochondral ossification, resulting on early fusion of the synchondrosis. As patients age, the neurosurgical focus shifts from primarily cranial to spinal concerns. Often pediatric neurosurgeons may continue to follow their patients with skeletal dysplasia. However, general adult neurosurgeons and orthopedic surgeons may see these graduated adults in their practice. This article provides a review of the common neurosurgical concerns for patients with achondroplasia.

Management of Craniocervical Instability in Spondyloepiphyseal Dysplasia Congenita: Assessment of Literature and Presentation of Two Cases.

Spondyloepiphyseal dysplasia congenita (SEDC) is a rare autosomal dominant skeletal dysplasia resulting in impairment of type II collagen function. Phenotypically, this results in various skeletal, ligamentous, ocular, and otologic abnormalities. Platyspondyly, scoliosis, ligamental laxity, and odontoid hypoplasia are common, resulting in myelopathy in a high number of patients due to atlantoaxial instability. Despite patients undergoing surgical fixation, complication rates such as nonunion have been reported to be high. Here within, we present two patients treated with occipitocervical fusion for atlantoaxial instability and early symptoms of progressive myelopathy. We additionally provide a detailed review of the literature to inform practitioners of the spinal manifestations and clinical considerations in SEDC.

Management of failed Chiari decompression and intrasyringeal hemorrhage in Noonan syndrome: illustrative cases.

BACKGROUND: Noonan syndrome (NS) is a rare genetic RASopathy with multisystem implications. The disorder is typically characterized by short stature, distinctive facial features, intellectual disability, developmental delay, chest deformity, and congenital heart disease. NS may be inherited or arise secondary to spontaneous mutations of genes in the Ras/mitogen activated protein kinase signaling pathways. OBSERVATIONS: Numerous case reports exist detailing the association between NS and Chiari I malformation (CM-I), although this relationship has not been fully established. Patients with NS who present with CM-I requiring operation have shown high rates reoperation for failed decompression. The authors reported two patients with NS, CM-I, and syringomyelia who had prior posterior fossa decompressions without syrinx improvement. Both patients received reoperation with successful outcomes. LESSONS: The authors highlighted the association between NS and CM-I and raised awareness that patients with these disorders may be at higher risk for failed posterior fossa decompression, necessitating reoperation.