Journal of neurosurgery. Pediatrics最新文献

Objective: Studies have shown a high prevalence of sleep-disordered breathing (SDB) among children with myelomeningocele (MMC), but there are few published data on the longitudinal care of these patients. The objective of this study was to determine the effectiveness of standard treatments for SDB in children with MMC.

Methods: The authors analyzed records from three multidisciplinary spina bifida clinics to identify all patients with both MMC and SDB diagnosed by polysomnography (PSG). The primary outcome of this study was a change in apnea-hypopnea index (AHI; the number of apneic or hypopneic events per hour of sleep) before and after clinically recommended SDB treatments. Clinical and demographic variables were recorded and evaluated for possible association with posttreatment improvement of AHI. Analysis included change in AHI (a continuous variable) and whether SDB improved (defined as an AHI < 2.5 or decrease of AHI by ≥ 50% from baseline).

Results: Seventy-one eligible patients (aged 2 days-21 years, 52% male) had an initial AHI > 2.5 and had follow-up PSG after treatment for SDB. The mean AHI decreased from 20.5 (SD 21.6) at baseline to 11.6 (SD 15.7) after treatment (p = 0.0006). Children treated with supplemental oxygen and with continuous positive airway pressure had improvement on PSG (18 of 25 and 12 of 18, respectively). Children treated with adenotonsillectomy were less likely to improve (7 of 19). Forty-one patients (58%) improved from a baseline AHI > 2.5 to an AHI < 2.5 after treatment.

Conclusions: Children with MMC and SDB who undergo standard SDB treatments guided by pediatric sleep medicine physicians show improvement in PSG parameters after treatment.

Objective: The exact association between the frequently present anomalous intracranial venous vasculature, emissary collaterals, ventriculomegaly, and increased intracranial pressure (ICP) in children with Apert and Crouzon syndromes remains an enigma. This study aimed to evaluate the association between the aberrant venous system and ventricle size and increased ICP, and to assess the development of the venous structures over time.

Methods: This retrospective cohort study included all patients with Apert or Crouzon syndrome with available CT venography (CTV) scans of the brain. Anomalous venous vasculature was assessed by the total collateral score (TCS), which scored 9 intra- and extracranial venous structures (TCS range 0-16). Ventricle size (fronto-occipital horn ratio [FOHR]) was measured on the same scan. The presence of increased ICP, a shunt, tonsillar herniation, and head circumference were extracted from electronic patient records and were used as secondary covariates. Subsequent CTV scans were scored when available.

Results: Ninety patients were included in this study. The mean TCS was 7.5 ± 2.5, and was comparable for patients with Apert and Crouzon syndromes (mean 8.0 and 7.3, respectively). The presence of an extra abnormal venous structure was associated with an increase of the FOHR of 3.2% (p < 0.01). After dividing the venous structures into intra- and extracranial, a similar association between both and the FOHR was found (4.1% and 2.3%, respectively; p < 0.01). The TCS was similar for patients with normal and increased ICP at the time of the scan. Sixteen patients had dual CTV scans. The median time between both scans was 3.2 years. The presence of collaterals remained stable over time (median ΔTCS = 0.3). Seven patients with functioning shunts also maintained high TCSs (median TCS = 9).

Conclusions: In patients with Apert and Crouzon syndromes, a close relationship between venous collaterals and ventricle size was observed, in which a more extensive aberrant venous drainage pattern, both intra- and extracranial, was associated with larger ventricles. Preliminary longitudinal data suggested that the presence of venous collaterals remained constant over time despite effective treatment of increased ICP, indicating reliance on these collaterals even in cases of normal ICP. The authors hypothesize that this dependence on collateral drainage is the result of their aberrant internal venous anatomy and predisposes individuals to increased ICP.

Objective: This study reports the infant to preschool outcomes of a laparotomy-assisted, two-port fetoscopic myelomeningocele (MMC) repair and compares the results with those of a contemporary, same-center cohort that underwent either fetal MMC surgery via hysterotomy or postnatal MMC repair.

Methods: All MMC closures between December 2011 and July 2021 were screened. Singleton pregnancies with hindbrain herniation and MMC between T1 and S1 were included. Fetuses were excluded for genetic abnormalities, severe kyphosis, and other congenital anomalies. The pregnant woman determined the method of MMC repair (fetoscopic, hysterotomy, or postnatal repair).

Results: Two hundred MMC closures met the study criteria (100 fetoscopic, 41 hysterotomy, and 59 postnatal). The median length of follow-up was beyond 46 months for all groups. The median gestational age at delivery was 38.1 weeks (IQR 35.1, 39.1 weeks) for the fetoscopic group, 35.7 weeks (IQR 33.6, 37.0 weeks) for the hysterotomy group, and 38.6 weeks (IQR 37.7, 39.0 weeks) for the postnatal group. Vaginal delivery occurred in 51% of the fetoscopic cases, and there were no instances of uterine dehiscence or rupture. Treatment for hydrocephalus in the 1st year occurred in 35% (95% CI 27%-50%) of fetoscopic, 33% (95% CI 20%-50%) of hysterotomy, and 81% (95% CI 70%-90%) of postnatal repair cases. At 30 months, patients who underwent fetal intervention were twice as likely to be community ambulators (with or without devices) as those who underwent postnatal repair (52% [95% CI 42%-62%] of fetoscopic, 54% [95% CI 39%-68%] of hysterotomy, and 24% [95% CI 14%-36%] of postnatal cases). Surgery for symptomatic tethered cord occurred in 12% (95% CI 7%-19%) of fetoscopic, 17% (95% CI 8%-31%) of hysterotomy, and 2% (95% CI 1%-8%) of postnatal repair cases. Surgery for symptomatic spinal inclusion cysts was required in 4% (95% CI 1%-9%) of fetoscopic, 7% (95% CI 2%-18%) of hysterotomy, and none (95% CI 0%-8%) of the postnatal cases.

Conclusions: Laparotomy-assisted, two-port fetoscopic repair provides significant benefits for maternal health. It negates the risk of uterine rupture for the index pregnancy and subsequent pregnancies and allows for vaginal delivery. The benefits to the fetus are the same as those of hysterotomy repairs, with a lower risk of prematurity. There was no difference in the rate of surgery for tethered cord or spinal inclusion cysts between fetoscopic and hysterotomy procedures. Overall, laparotomy-assisted, two-port fetoscopic repair is safer for the fetus and the mother than fetal MMC surgery via hysterotomy.

Objective: Patient- and surrogate-reported outcomes are increasingly recognized as important and historically limited dimensions of satisfaction with medical care. Evaluating caregiver satisfaction for cerebral palsy (CP) patients with pediatric movement disorders (PMDs) remains undefined, limited by a lack of appropriate tools and the heterogeneity of the patient population. The authors identified caregiver satisfaction with the neurosurgical management of PMDs as a key quality metric and report their results across an institutional experience.

Methods: A retrospective single-institution survey study was performed on caregivers of consecutive children who underwent PMD surgery from March 2022 to December 2023. The authors designed a brief 4-question satisfaction survey with dichotomous yes/no answers. The telephone survey solicited answers from primary caregivers, and contact attempts were made 3 times before labeling a nonresponder. Non-English speakers were included. The survey answers were correlated with demographic characteristics, clinical data, and complications. Descriptive statistics were performed using Excel.

Results: Seventy patients were identified in the study period with 50 associated caregivers voluntarily responding to the questionnaire (50/70 [71.4%]). Forty-six male and 24 female patients with a mean (range) age of 13.1 (2-34) years and a follow-up range of 3-20 months were included. All 50 caregivers reported satisfaction with the surgical care their child received: 100% confirmed they would refer others to the program and 94% confirmed that they would have the surgery again in retrospect. Ten caregivers (10/50 [20%]) recalled complications, but only 5 (5/50 [10%]) surgical complications resulted in hospital readmission.

Conclusions: Caregivers were overwhelmingly satisfied with the neurosurgical care for PMDs and would recommend the functional pediatric neurosurgery program to others. A large percentage would again submit to the surgery. There is a perception disparity between caregiver- and hospital-identified complications; it may be beneficial to emphasize expected adverse effects with caregivers prior to surgery. Caregiver satisfaction remains an important quality dimension and future research may benefit from more objective metrics.

Objective: Detecting neurological deterioration and diagnosing tethered cord syndrome (TCS) in patients with myelomeningocele (MMC) can be challenging due to the presence of symptoms at birth and the lack of objective indicators. This retrospective analysis focused on urological manifestations and evaluated whether tethered cord release (TCR) for TCS at an early stage could improve or stabilize video urodynamic study (VUDS) findings and lower urinary tract function.

Methods: This study analyzed 55 of 64 children who underwent MMC repair at Miyagi Children's Hospital, Sendai, Japan, between 2003 and 2016. The follow-up duration for these children exceeded 6 years. Clinical records were reviewed, and surgical indicators and outcomes of TCR and preoperative and postoperative results of VUDS were evaluated.

Results: The duration of follow-up was mean ± SD (range) 12.6 ± 3.5 (6.0-19.0) years. TCR was performed on 27 (49.1%) of 55 patients with MMC, totaling 33 procedures. Five patients underwent 2 TCR procedures, whereas 1 received 3 TCR procedures. The mean ± SD (range) age was 7.5 ± 2.9 (2.2-12.7) years at the first TCR procedure (n = 27) and the mean (range) was 10.9 (10.1-11.7) years at the second TCR (n = 5); in addition, 1 patient underwent a third TCR procedure at age 15.9 years. There were no identified risk factors associated with the TCS. The first TCR procedure was performed due to worsened lower-extremity (LE) motor symptoms in 1 patient, worsened LE sensory symptoms in 3 patients, and worsened VUDS findings in 26 patients. After the procedure, LE motor symptoms improved in 1 patient (100%), LE sensory symptoms improved in 2 patients (66.7%), and VUDS findings improved in 18 patients (66.7%). Preoperative VUDS revealed urological deterioration characterized by a high-pressure bladder, reduced bladder capacity, increased detrusor overactivity (DO), and vesicoureteral reflux. Postoperative VUDS showed improvements in bladder function, including decreased bladder pressure and DO, increased bladder capacity, and compliance. None of the patients underwent augmentation cystoplasty or had renal dysfunction.

Conclusions: Routine VUDS can detect urological deterioration, which can be a significant indicator for early diagnosis of TCS. Performing TCR at an early stage is beneficial, not only to protect renal function but also to improve VUDS findings and lower urinary tract function in patients with MMC. It is important to explore a standardized approach for the diagnosis and treatment of TCS.

Objective: Abusive head trauma (AHT) is the leading cause of death from physical child abuse in children younger than 5 years of age in the United States. The mortality rate among patients with AHT is 25%, and the recurrence rate of child abuse rises to 35% when there is a lack of intervention. Thus, identifying child abuse is crucial yet especially challenging for infants and toddlers as they are preverbal. Current guidelines for child abuse do not sufficiently address the specific needs of a younger population. This study aimed to evaluate clinical factors associated with abuse among such populations.

Methods: The National Trauma Data Bank was queried from 2017 to 2019 for patients younger than 3 years with acute head trauma. Patients who were suspected of having experienced child abuse (suspected child abuse [SCA] group) were propensity score matched with patients who were not suspected of having experienced child abuse (non-SCA group) based on demographics, comorbidities, and Glasgow Coma Scale (GCS) scores. Paired Student t-test and chi-square tests were used to compare differences in hospital outcomes between the two groups. Multivariable regression analysis was used to determine factors associated with SCA (p < 0.05).

Results: The authors identified 10,844 patients in the SCA group and 27,912 in the non-SCA group. Regression analysis results showed that patients in the SCA group had higher rates of prematurity (OR 2.30, p < 0.001), GCS scores < 13 (OR 1.79, p < 0.001), congenital disorders (OR 1.56, p < 0.001), and public insurance use (68.38% vs 52.88% p < 0.001). Black and Hispanic patients were more likely to be in the SCA group (OR 1.56, p < 0.001 and OR 1.35, p < 0.001, respectively). Following propensity score matching, SCA patients had a longer length of hospital stay (3.17 vs 1.34 days, p < 0.001) and higher mortality rate (4.89% vs 3.58%, p < 0.001).

Conclusions: Acute head injuries in the SCA group were associated with prematurity, congenital disorder, low GCS score, and public insurance use. As such, the current guidelines should implement clinical history and insurance type to better reflect the at-risk patient population when evaluating infants and toddlers for potential abuse. There could be overidentification of child abuse among Black and Hispanic patients, and further research is warranted.

Objective: The goal of this study was to assess the complications associated with vertical parasagittal hemispherotomy (VPH), the impact of incomplete disconnection on long-term seizure freedom, and how VPH impacts cognitive development.

Methods: A retrospective evaluation was performed in all patients who had undergone VPH during 1991-2022 at the authors' institution. Two-year follow-up data were available for 45 patients, and there were 6-month data for 3 more. All available postoperative MRI studies (31/48, 64.6%) were reviewed. Before 2010, postoperative MRI was only performed if seizures recurred.

Results: Primary VPH led to Engel class I in 73% of patients. Acquired etiologies had a higher rate of Engel I compared to developmental and progressive etiologies (96% vs 46% and 44%, p < 0.001). Nearly half of patients (45%) showed improved cognitive trajectories as opposed to their preoperative ones, whereas in 45% trajectories remained unchanged. Additionally, 5 patients (10%) exhibited new major deficits or accelerated cognitive deterioration after VPH. Surgical complications occurred in 14 patients (29%) after the first VPH; 4 cases were classified as transient, resolving during follow-up without surgical intervention. Nontransient complications included 8 cases of hydrocephalus requiring surgical treatment, 1 shunted subdural hygroma, and 1 case of CSF leakage from the wound. Diabetes insipidus occurred in 6 patients, with all resolving spontaneously. Residual connections were present in 16 patients, primarily in the temporomesial region. Seven patients remained seizure free despite visible residual connections.

Conclusions: VPH is a highly effective treatment for drug-resistant hemispheric epilepsy, resulting in durable seizure freedom and often favorable cognitive outcomes. Diabetes insipidus in addition to hydrocephalus is a common complication after VPH. Incomplete disconnection does not necessarily preclude seizure freedom.

Objective: The aim of this study was, through comprehensive, multilevel models of social determinants of health (SDoH) factors, including the Yost Index socioeconomic status (SES) score, to determine whether community- or individual-level SDoH factors quantifiably influence pediatric CNS tumor disparities more in care and prognosis across the US.

Methods: The authors performed a retrospective cohort study assessing specialized Surveillance, Epidemiology, and End Results data of pediatric patients (≤ 19 years old) with nonmalignant and malignant tumors of the CNS from 2010 to 2018. A census-level Yost Index SES score and rurality/urbanicity measures were incorporated with individual characteristics of age, sex, and race/ethnicity. Chi-square analyses for clinical and demographic descriptions, multivariate Cox proportional hazards logistic regressions for survival, and multivariate logistic regressions for resection, radiation treatment, treatment delay, and advanced staging on preliminary presentation were performed.

Results: Across 18,236 patients, age-adjusted analyses showed substantially increased mortality risk among 6 of 11 subtypes (highest hazard ratio [HR] 1.91, 95% CI 1.59-2.28, p < 0.001 for glioma NOS), decreased odds of first-line therapy among 7 of 18 subtypes (lowest OR 0.36, 95% CI 0.11-0.97, p = 0.043 for resection of choroid plexus papilloma), increased odds of treatment delay among 6 of 11 subtypes (highest OR 2.47, 95% CI 1.01-6.49, p = 0.047 for germinoma), increased odds of advanced staging on preliminary presentation among 3 of 10 malignant subtypes (highest OR 2.56, 95% CI 1.27-5.52, p = 0.008 for malignant ependymomas), and increased odds of receipt of radiation therapy among 3 of 10 malignant subtypes (highest OR 2.30, 95% CI 1.87-2.84, p < 0.001) observed across many disease subtypes contributed by certain individual- and community-level SDoH factors.

Conclusions: Through comprehensive analyses combining individual- and community-level SDoH factors, this study identified detrimental interrelated SDoH associations with poorer care and prognosis of pediatric patients with CNS tumors, delineating how both levels differentially contribute to observed disparities across different subtypes.