Primary Diffuse Leptomeningeal Melanomatosis in a Child with Extracranial Metastasis: Case Report.

Primary meningeal melanomatosis is an extremely rare tumor with very few documented responses to treatment. A 3-year-old male with a complex past medical history, including prematurity and shunted hydrocephalus, was diagnosed with primary meningeal melanomatosis with peritoneal implants. Molecular testing revealed an NRAS Q61R mutation. The patient received proton craniospinal radiation followed by immunotherapy with nivolumab (1 mg/kg) and ipilimumab (3 mg/kg) IV every 3 weeks and, upon progression, he was switched to a higher dose of nivolumab (3 mg/kg IV every 2 weeks) and binimetinib (24 mg/m2/dose, twice a day). The patient had significant improvement of CNS disease with radiation therapy and initial immunotherapy but progression of extracranial metastatic peritoneal and abdominal disease. Radiation was not administered to the whole abdomen. After two cycles of nivolumab and treatment with the MEK inhibitor binimetinib, he had radiographic and clinical improvement in abdominal metastasis and ascitis. He ultimately died from RSV infection, Klebsiella sepsis, and subdural hemorrhage without evidence of tumor progression. This is the first report of a child with primary meningeal melanomatosis with extracranial metastatic disease with response to a combination of radiation, immunotherapy and MEK inhibitor therapy.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Unexpected Decrease in Shunt Surgeries Performed during the Shelter-in-Place Period of the COVID-19 Pandemic.

BACKGROUND: It is expected that the incidence of cerebrospinal fluid (CSF) shunt malfunctions would remain unchanged during the shelter-in-place period related to the COVID-19 pandemic. OBJECTIVE: To examine the number of shunt surgeries performed in a single institution during this time interval in comparison to equivalent periods in past years. METHODS: The numbers of elective and emergent/urgent shunt surgeries performed at a single institution were queried for a 28-d period starting on the third Monday of March, between years 2015 and 2020. These were further stratified by how they presented as well as the type of surgery performed. RESULTS: During the 28-d period of interest, in the years between 2015 and 2020, there was a steady increase in the number of shunt surgeries performed, with a maximum of 64 shunt surgeries performed in 2019. Of these, approximately 50% presented in urgent fashion in any given year. In the 4-wk period starting March 16, 2020, a total of 32 shunt surgeries were performed, with 15 of those cases presenting from the outpatient setting in emergent/urgent fashion. For the surgeries performed, there was a statistically significant decrease in the number of revision shunt surgeries performed. CONCLUSION: During the 2020 COVID-19 pandemic, there was an unexpected decrease in the number of shunt surgeries performed, and particularly in the number of revision surgeries performed. This suggests that an environmental factor related to the pandemic is altering the presentation rate of shunt malfunctions.

Ventricular access device infection rate: a retrospective study and review of the literature.

PURPOSE: Ventricular access devices (VAD) are often used for treatment of posthemorrhagic hydrocephalus (PHH) in preterm infants. The reported rates of infection have varied and range from 0 to 22 %. The objective of our study is to present our VAD associated infection at our institution. METHODS: The charts for patients that had VADs inserted between May 1, 2009 and October 31, 2013 at a single institution (Children's Healthcare of Atlanta) were retrospectively reviewed. The number of VAD infections, defined as either cerebrospinal fluid (CSF)-positive cultures or wound complication, was recorded. Of patients that survived, the number of VAD to shunt conversions was also examined. The data from 15 previously published studies were pooled to determine overall VAD infection and VAD to shunt conversion rates. RESULTS: A total of 142 VADs were placed. There were 13 infections (9.2 %), 11 of which had CSF-positive cultures (7.7 %). There were two wound complications with negative CSF cultures. Six patients died after VAD placement for reasons unrelated to their VAD surgeries (4.2 %). In the remaining patients, there were 113 VAD to shunt conversions (83.1 %). Fifteen studies that reported VAD infections were analyzed; an overall infection rate of 7.0 % and VAD to shunt conversion rate of 79 % were calculated. CONCLUSIONS: While VAD is a valuable tool to treat PHH, it remains a procedure with an infection rate between 7.0 and 8.0 %. Close follow-up is needed to capture these adverse events as early as possible. Approximately 80 % of patients with PHH will require permanent CSF diversion.

Utility of surveillance imaging after minor blunt head trauma.

OBJECT: Nonoperative blunt head trauma is a common reason for admission in a pediatric hospital. Adverse events, such as growing skull fracture, are rare, and the incidence of such morbidity is not known. As a result, optimal follow-up care is not clear. METHODS: Patients admitted after minor blunt head trauma between May 1, 2009, and April 30, 2013, were identified at a single institution. Demographic, socioeconomic, and clinical characteristics were retrieved from administrative and outpatient databases. Clinical events within the 180-day period following discharge were reviewed and analyzed. These events included emergency department (ED) visits, need for surgical procedures, clinic visits, and surveillance imaging utilization. Associations among these clinical events and potential contributing factors were analyzed using appropriate statistical methods. RESULTS: There were 937 admissions for minor blunt head trauma in the 4-year period. Patients who required surgical interventions during the index admission were excluded. The average age of the admitted patients was 5.53 years, and the average length of stay was 1.7 days; 15.7% of patients were admitted for concussion symptoms with negative imaging findings, and 26.4% of patients suffered a skull fracture without intracranial injury. Patients presented with subdural, subarachnoid, or intraventricular hemorrhage in 11.6%, 9.19%, and 0.53% of cases, respectively. After discharge, 672 patients returned for at least 1 follow-up clinic visit (71.7%), and surveillance imaging was obtained at the time of the visit in 343 instances. The number of adverse events was small and consisted of 34 ED visits and 3 surgeries. Some of the ED visits could have been prevented with better discharge instructions, but none of the surgery was preventable. Furthermore, the pattern of postinjury surveillance imaging utilization correlated with physician identity but not with injury severity. Because the number of adverse events was small, surveillance imaging could not be shown to positively influence outcomes. CONCLUSIONS: Adverse events after nonoperative mild traumatic injury are rare. The routine use of postinjury surveillance imaging remains controversial, but these data suggest that such imaging does not effectively identify those who require operative intervention.

Return to system within 30 days of pediatric neurosurgery.

OBJECT: Quality assessment measures have not been well developed for pediatric neurosurgical patients. This report documents the authors' experience in extracting information from an administrative database to establish the rate of return to system within 30 days of pediatric neurosurgical procedures. METHODS: Demographic, socioeconomic, and clinical characteristics were prospectively collected in administrative, business, and operating room databases. The primary end point was an unexpected return to the hospital system within 30 days from the date of a pediatric neurosurgical procedure. Statistical methods were used to identify clinical and demographic factors associated with the primary end point. RESULTS: There were 1358 pediatric neurosurgical procedures performed in the Children's Healthcare of Atlanta operating rooms in 2012, with 37.4% of these surgeries being preceded by admissions through the emergency department. Medicare or Medicaid was the payor for 54.9% of surgeries, and 37.6% of surgeries were shunt related. There were 148 unexpected returns to the system within 30 days after surgery, and in 109 of these cases, the patient had a presenting complaint that was attributable to the index surgery (related returns). The most common complaints were headache, nausea, vomiting, or seizure after shunt revision or cranial procedures (n = 62). The next most common reason for re-presentation was for wound concerns (n = 30). Thirty-seven of the 109 related returns resulted in a reoperation. The monthly rate of related returns was 8.1% ± 2.5% over the 12-month study period. When using related returns as the dependent variable, the authors found that patients who underwent a shunt-related surgery were both more likely to unexpectedly return to the system (OR 1.86, p = 0.008) and to require surgery upon readmission (OR 3.28, p = 0.004). Because an extended hospitalization shortened the window of time for readmission after surgery, extended length of stay was protective against return to system within 30 days of surgery. Importantly, if related and unrelated returns were analyzed together as the dependent variable (n = 148), no independent clinical and demographic risk factor could be identified. CONCLUSIONS: Quality assessment measures need to be clearly and carefully defined, as the definition itself will impact the analytical results. Clinicians must play a leading role in the development of these measures to ensure their clinical meaningfulness.

Response of subependymal giant cell astrocytoma with spinal cord metastasis to everolimus.

BACKGROUND: Brain subependymal giant cell astrocytomas (SEGAs) in patients with tuberous sclerosis have been reported to respond to everolimus. METHODS: A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. He received daily everolimus at a dose of 3 mg/m for 6 weeks, which was then increased to 6 mg/m. RESULTS: Magnetic resonance image of the brain and spine showed significant reduction in the size of SEGAs after 6 weeks of treatment. The patient has remained free of progression for 24 months. Additional benefits included: excellent seizure control, decrease in the size of cardiac rhabdomyomas, and improved quality of life. CONCLUSIONS: We describe a rare case of metastatic SEGA, which was successfully treated with everolimus.

Neurofibromatosis-2 and spinal cord ependymomas: Report of two cases and review of the literature.

OBJECT: The incidence of ependymoma in patients with neurofibromatosis-2 (NF-2) is low and information regarding treatment and prognosis is lacking. We present two cases of cervicomedullary tumors in patients with NF-2 from our institution, and we provide a review of the literature in order to summarize the known clinical information about this rare occurrence. PATIENTS AND METHODS: Patient #1 had histological confirmation of ependymoma and was treated with subtotal resection followed by observation and has had no evidence of progression for 11 months. Patient #2 has been observed for 4 1/2 years without treatment for a cervicomedullary tumor, which appears to be an ependymoma by imaging. Although it has increased in size very slowly, there have been no clinical symptoms. Among the additional 21 cases of NF-2 and ependymoma from the literature, the most common location is the cervical spine (70%), and the median age at diagnosis is 15 years. Surgical resection was performed in 85% of the cases and subtotal resection in 64% of cases. Fifteen patients (75%) were reported alive at the time of the published reports, with survival ranging from 0.1 to 10 years, and the 8-year survival estimated as 51%. Survival was related to the location of the tumor. CONCLUSIONS: We conclude from our two cases and review of the existing literature that NF-2 associated spinal ependymomas have an indolent course and typically can be observed or treated by surgical excision alone.

Application of the spanning plate concept to frontal orbital advancement: techniques and clinical experience in 60 patients.

Frontal orbital advancement (FOA), is the procedure of choice in treatment of coronal and metopic synostosis. Resorbable plates and screws have been widely accepted for use in pediatric craniofacial surgery, including FOA. We have applied the concept of extended resorbable spanning plates to FOA for metopic, unilateral, and bilateral coronal synostosis in infants and children during a 5-year period. We report on 60 patients, ages 4 to 15 months (mean, 7 months); 28 girls, 32 boys. Follow-up ranged from 12 to 36 months (mean, 24 months). There were no structural failures, no infections, and no complications related to the use of extended spanning plates. Extended spanning plates decrease mobility between bone segments, confer greater stability to the construct, and reduce both the number of plates and of screws that are necessary and reduce the operative time. Application of these plates simplifies FOA surgery and represents a step in the evolution of plating technology.

Performance and complications associated with the synchromed 10-ml infusion pump for intrathecal baclofen administration in children.

OBJECT: The objectives of this multicenter study were to monitor the performance of a 10-ml pump infusing intrathecal baclofen to treat 100 children with cerebral spasticity, to monitor complications associated with the pump, and to correlate pump-related complications with body habitus. METHODS: Age at implantation of the pump ranged from 1.4 to 16.8 years (mean 8.1 years). The effects of ITB on spasticity in the upper and lower extremities were evaluated using the Ashworth Scale. Data were collected regarding implant site, infection, complication, and body mass index (BMI). Ashworth Scale scores decreased significantly in the upper and lower extremities at 6 and 12 months after pump implantation (p < 0.001). There were four serious system-related complications, all specific to catheters. There were 32 serious procedure-related complications in 21 patients: 11 complications were infections that occurred in nine patients. Four of nine pump-induced infections were treated with pump removal and antibiotic therapy; five infections were treated successfully with antibiotic therapy alone, without pump removal. In children younger than 8 years of age there was a significantly higher incidence of serious procedure-related adverse events than in older children. There was no significant correlation between BMI and the incidence of pump pocket-related complications or infections. CONCLUSIONS: The 10-ml pump can be used therapeutically in small children, particularly those weighing less than 40 lbs, with greater ease and less wound tension, than the conventional 18-ml pump. The incidence of complications associated with the 10-ml pump in younger children appears to be similar to that previously reported with the 18-ml pump in larger-sized children.

Predicting the risk of reoperation in metopic synostosis: a quantitative CT scan analysis.

Children with metopic synostosis have a well-described clinical picture of trigonocephaly, often with hypotelorbitism. The craniofacial disorder itself is well recognized; however, objective prognostic factors for predicting the risk of reoperation are not well known. In 39 children with metopic synostosis, measurements of the cranial length, cranial width, anterior intercoronal distance, anterior interorbital distance (intercanthal distance), lateral orbital distance, and interzygomatic buttress distance were taken from preoperative computed tomographic (CT) scans and were normalized relative to each child's age. To separate overall facial hypoplasia from regional hypoplasia, a ratio of intercanthal distance to interzygomatic buttress distance was determined. These prognostic factors were analyzed with respect to reoperation rate. A stepwise logistic regression analysis was used to determine the interrelationships between the prognostic factors. Twenty-eight percent of the children underwent reoperation (N=11, 1 total reoperation and 10 minor recontouring). All of the reoperations occurred in children with a decreased intercanthal distance (p=0.30). The ratio of intercanthal distance to midfacial width was related to reoperation rate, with those children who had a ratio < or =0.80 having a reoperation rate of 44% (8 of 18 total children with an intercanthal-to-zygomatic ratio < or =0.8, p=0.07). This relationship was significant in children younger than the age of 12 months (6 of 13 total children with an intercanthal-to-zygomatic ratio < or =0.8, 46% reoperation rate, p=0.006). This study suggests that preoperative CT measurements can be used as a means of risk stratification in outcome analyses of the surgical treatment of craniosynostosis. In children treated for metopic suture synostosis, a foreshortened intercanthal distance compared with the interzygomatic buttress distance was related to reoperation rate, especially in children younger than 12 months of age.