Pediatric pilocytic astrocytoma (PPA) requires prolonged follow-up after initial resection. The landscape of transitional care for PPA patients is not well characterized. The authors sought to examine the clinical course and transition to adult care for these patients to better characterize opportunities for improvement in long-term care.
Hospital readmission after discharge is a commonly used quality measure. In a previous study, the authors had documented the rate of readmission and reoperation after pediatric CSF shunt surgery. This study documents the rate of readmission and reoperation after pediatric neurosurgical procedures excluding those related to CSF shunts.Between May 1, 2009, and April 30, 2013, 3098 non-shunt surgeries during 2924 index admissions were performed at a single institution. Demographic, socioeconomic, and clinical characteristics were prospectively collected in the administrative, business, and clinical databases. Clinical events within the 30 days following discharge were reviewed and analyzed. The following events of interest were analyzed for risk factor associations using multivariate logistic regression: return to the emergency department (ED), all-cause readmission, readmission to the neurosurgical service, and reoperation.The number of all-cause readmissions within 30 days of discharge was 304 (10.4%, 304/2924). Admission sources consisted of the ED (n = 173), hospital transfers (n = 47), and others (n = 84). One hundred eighty of the 304 readmissions were associated with an operation, but only 153 were performed by the neurosurgical service (reoperation rate = 5.2%). These procedures included wound revisions (n = 30) and first-time shunt insertions (n = 35). The remaining 124 readmissions were nonsurgical, and only 54 were admitted to the neurosurgical service for issues related to the index non-shunt surgery. Thus, the rate of related readmission was 7.1% ([153 + 54]/2924). A longer length of stay and admission to the neonatal intensive care unit during the index admission were associated with an increased likelihood of return to the ED and readmission. Certain procedures, such as baclofen pump insertion and intracranial pressure monitor placement, were also found to be associated with adverse clinical events in the 30-day period. Lastly, patients were more likely to a undergo reoperation if the index procedure had started after 3 p.m.The all-cause readmission rate within 30 days of discharge after a pediatric neurosurgical procedure was 10.4%, and the rate of related readmission was 7.1%. Whether these readmissions are preventable and to what extent they are preventable requires further study.
The purpose of this study was to correlate lumbar ultrasound (LUS) and MRI findings in patients suspected of having occult spinal dysraphism (OSD).Over a 5-year period, 1273 consecutive infants underwent an LUS study at a major pediatric tertiary referral center. Of these, 106 patients had abnormal LUS findings suggestive of an OSD, and 103 underwent subsequent MRI studies. The anatomical descriptions of the 2 studies were compared for agreement.The average age of the infants was 34 days at the time of the LUS study; OSD was suspected in these patients because of the presence of cutaneous stigmata and congenital defects. The most common anatomical descriptions from the LUS study included a thickened or fatty filum (32 cases), filum cyst (11 cases), and presence of a terminal ventricle or syrinx (9 cases). Using MRI findings as the standard reference, the sensitivity of LUS in detecting a thickened or fatty filum was 20%. The sensitivity of detecting an abnormal conus level at or below L-3 was 76.9%.In the patient population chosen to undergo LUS studies, abnormal findings had poor sensitivity at detecting anatomical findings consistent with OSD.
Cysts of the choroidal fissure are often incidentally identified. Symptoms from such cysts appear to be exceedingly rare. Herein, the authors report a case series of symptomatic enlargement of choroidal fissure cysts that were surgically treated. Although cysts of the choroidal fissure do not normally become symptomatic, the neurosurgeon should be aware of such a complication. Based on the authors' experience, surgical fenestration of such cysts has good long-term results.
Object Most children with spina bifida aperta have implanted CSF shunts. However, the efficacy of adding surveillance imaging to clinical evaluation during routine follow-up as a means to minimize the hazard of shunt failure has not been thoroughly studied. Methods A total of 396 clinic visits were made by patients with spina bifida aperta and shunt-treated hydrocephalus in a spina bifida specialty clinic during the calendar years 2008 and 2009 (initial clinic visit). All visits were preceded by a 6-month period during which no shunt evaluation of any kind was performed and were followed by a subsequent visit in the same clinic. At the initial clinic visit, 230 patients were evaluated by a neurosurgeon (clinical evaluation group), and 166 patients underwent previously scheduled surveillance CT scans in addition to clinical evaluation (surveillance imaging group). Subsequent unexpected events, defined as emergency department (ED) visits and caregiver-requested clinic visits, were reviewed. The time to an unexpected event and the likelihood of event occurrence in each of the 2 groups were compared using Cox proportional hazards survival analysis. The outcome and complications of shunt surgeries were also reviewed. Results The clinical characteristics of the 2 groups were similar. In the clinical evaluation group, 2 patients underwent shunt revision based on clinical findings in the initial visit. In the subsequent follow-up period, there were 27 visits to the ED and 25 requested clinic visits that resulted in 12 shunt revisions. In the surveillance imaging group, 11 patients underwent shunt revision based on clinical and imaging findings in the initial visit. In the subsequent follow-up period, there were 15 visits to the ED and 9 requested clinic visits that resulted in 8 shunt revisions. Patients who underwent surveillance imaging on the day of initial clinic visit were less likely to have an unexpected event in the subsequent follow-up period (relative risk 0.579, p = 0.026). The likelihood of needing shunt revision and the morbidity of shunt malfunction was not significantly different between the 2 groups. Conclusions Surveillance imaging in children with spina bifida aperta and shunted hydrocephalus decreases the likelihood of ED visits and caregiver-requested clinic visits in the follow-up period, but based on this study, its effect on mortality and morbidity related to shunt malfunction was less clear.
A 2 month old child with a large hemispheric tumor; after subtotal resection, pathology showed pilomyxoid astrocytoma with BRAFV600E mutation by immunohistochemistry and pyrosequencing. He had multiple episodes of radiographic and clinical progression. Over the course of 5 years, therapy included carboplatin, TPCV, velban, and immunotherapy. Vemurafenib and dabrafenib were administered with no response. MRI showed rapid tumor progression in the brain with spinal dissemination and significant clinical deterioration. Patient underwent tumor debulking and pathology confirmed a high grade astrocytoma, without BRAF V600E mutation, but with a novel BEND4-NTRK2 fusion by molecular genetic testing. He underwent whole brain radiation of 33 Gy in 16 fractions and boost to primary tumor bed to a total dose of 43 Gy. No radiation was given to the spine. One month later, patient started compassionate treatment with entrectinib, a novel, CNS-active, TRK inhibitor, at 400 mg/m2/day. MRI obtained after 28 days on treatment demonstrated significant radiographic regression, with rapid improvement in clinical symptoms. His dose was then increased to 550 mg/m2/day. After 2 additional cycles, the tumor continues to decrease in size in the brain and a spine MRI demonstrated complete response despite no radiation to the spine. Toxicities included Grade 1 ALT, cholesterol and peripheral edema. In summary, we report the case of a refractory BRAF V600E mutated low grade glioma transformed into a high grade with a novel BEND4-NTRK2 fusion, and profound radiographic and clinical response to entrectinib.
Abstract BACKGROUND: Challenges around the design and investigation of cell culture models of adamantinomatous craniopharyngioma (ACP) have arisen from the cellular heterogeneity of these tumors, with populations that harbor disparate requirements in culture. Novel approaches to in vitro modeling of ACP are needed. METHODS: Intraoperatively collected tumor specimens were mechanically digested and plated under conditions tailored to the cell population of interest. ACP tumor-derived fibroblasts and epithelial cells were isolated using serum-containing and keratinocyte-specific media respectively. ACP-derived epithelial cells were immortalized via SV40 virus transfection and puromycin treatment for stable cell-line generation. Cell line validation included immunofluorescence with markers appropriate for the cell population of interest. RNA sequencing of cell lines was compared to ACP transcriptome reference data. Cell typing was conducted using short tandem repeat sequencing. RESULTS: ACP fibroblasts and ACP epithelial cells maintained spindle-like and cobblestone morphologies respectively, even after 4 passages. Immunofluorescence staining confirmed high levels of Vimentin expression in ACP-derived fibroblasts, and panCK and B-catenin in ACP-derived epithelial cells. Point mutation in exon 3 of the CTNNB1 gene was identified in ACP-derived epithelial cells. CONCLUSION: Initial limits related to cell line development in ACP may be addressed through the isolation and culture-specific ACP cell populations. This experience demonstrates the maintenance of validated markers of the cell populations of interest ex vivo. While preliminary, such cell lines offer promise as tools for the identification and study of potential therapeutic vulnerabilities in ACP.
Chance fracture (CFx) with concomitant intra-abdominal injury has variable occurrence rates ranging from 33 to 89%. No single study has compared the incidence of simultaneous abdominal injury between pediatric and adult populations. This study compares the rate of simultaneous intra-abdominal injury and chance fracture in these populations.A retrospective review of pediatric and adult patients with chance fracture in comparable pediatric and adult trauma centers was performed. Patient demographics, mechanism of injury (MOI), and injury patterns were collected from 2002 to 2019 for pediatric patients and 2015 to 2018 for adults. Student t-test analyses were performed to determine statistical significance between the cohorts.The pediatric group had a similar incidence of abdominal solid organ injuries compared to adults (16 [20.5%] vs. 40 [19.7%], p<0.879), but the pediatric group had a greater number of total intra-abdominal (49 [62.8%] vs. 47 [23.1%], p < 0.001) and hollow organ injuries (40 [51.3%] vs. 17 [8.4%], p < 0.001). Motor vehicle collision was the most common mechanism of injury for both groups (72 pediatric [92.3%] vs. 85 adult [41.7%]) but adults suffered from more falls (3 pediatric vs. 81 adult, p < 0.001). Pediatric patients with CFx caused by MVCs had more intra-abdominal injuries (48 [66.7%] vs. 25[29.8%], p < 0.001) and hollow organ injuries compared to adults (39 [54.2%] vs. 8[9.5%], p < 0.001).In the setting of Chance fracture after trauma, pediatric patients are more likely to have a concomitant intra-abdominal organ injury (63% vs. 23%), especially hollow viscus injury (51.3% vs. 8.4%) compared with adults regardless of mechanism.
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