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Clinical features of pediatric patients with cerebral venous sinus thrombosis after isolated head trauma

Saki Kotani, Gaku Fujiwara, Keisuke Fuji, Toyonobu Maekawa, Shogo Ogita, Yudai Goto, Mamoru Murakami, Satoshi Kimura, Nobukuni Murakami, and Naoya Hashimoto

OBJECTIVE

An association between blunt head trauma and cerebral venous sinus thrombosis (CVST) has been recognized, but its symptoms are nonspecific and the duration of symptoms remains unclear. Anticoagulation therapy is not considered necessary in most cases of traumatic CVST; however, this is controversial. The aim of this study was to describe the clinical characteristics and outcomes of children with CVST after isolated head trauma.

METHODS

The records of pediatric patients with isolated head trauma admitted for observation at 3 medical centers between January 2018 and May 2023 were reviewed retrospectively. CVST was diagnosed on MR venography (MRV). Clinical presentation, therapeutic management, and outcomes were evaluated in patients who had follow-up MRV.

RESULTS

Of 260 pediatric patients with head trauma admitted to the 3 hospitals, 26 patients underwent MRV and 8 (30.8%) were diagnosed with CVST. One patient was treated with heparin, while the others received conservative treatment. All patients were discharged home asymptomatic. MRV performed during follow-up displayed complete recanalization in all cases, except for 1 case with partial recanalization. The median hospital stay was longer in patients with CVST than in those without CVST (9.5 vs 3.0 days, p = 0.001).

CONCLUSIONS

The length of stay of pediatric patients with traumatic CVST was prolonged compared with those without CVST, but most patients had good outcomes with spontaneous recanalization following conservative treatment.

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Hemispherectomy at The Hospital for Sick Children: expanded indications and lessons learned over 35 years

Karim Mithani, Jennifer L. Quon, Sara Breitbart, Patrick E. Steadman, Ladina Greuter, Oliver L. Richards, Ann K. Schmitz, Hrishikesh Suresh, Noor Malik, Abdullah AlRamadan, George M. Ibrahim, and James T. Rutka

OBJECTIVE

Functional hemispherectomy is an effective surgical intervention for select patients with drug-resistant epilepsy. The last several decades have seen dramatic evolutions in preoperative evaluation, surgical techniques, and postoperative care. Here, the authors present a retrospective review of the medical records of 146 children who underwent hemispherectomy between 1987 and 2022 at The Hospital for Sick Children, providing a unique overview of the evolution of the procedure and patient outcomes over 35 years.

METHODS

The medical records of all children who underwent hemispherectomy at The Hospital for Sick Children between 1987 and 2022 were reviewed. Demographic information, preoperative clinical features, short-term and long-term seizure outcomes, and details regarding postoperative complications were recorded.

RESULTS

The seizure outcomes of 146 children were analyzed. There were 68 females and 78 males with a mean age of 5.08 years, 123 of whom demonstrated seizure freedom (Engel class IA) in the short-term postoperative follow-up period and 89 in the long term. The effectiveness of hemispherectomy in achieving long-term seizure control has improved over time (β = 0.06, p < 0.001). Factors associated with overall seizure freedom included younger age at the time of hemispherectomy and stroke as the etiology of seizures, as well as complete disconnection during the first surgery. Additionally, the etiologies of epilepsy for which hemispherectomy is performed have expanded over time, while complication rates have remained unchanged.

CONCLUSIONS

Hemispherectomy is an increasingly effective treatment for certain cases of drug-resistant epilepsy. The etiologies of epilepsy for which hemispherectomy is performed are broadening, with no change in its safety profile. Seizure outcomes are better when the etiology of epilepsy is an ischemic injury, and the most common complication after the procedure is hydrocephalus. These findings reinforce the ongoing use of hemispherectomy as a safe and effective treatment option for certain individuals with drug-resistant epilepsy, support its application to a broader range of etiologies, and highlight areas of future investigation.

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AXIN1 mutations in nonsyndromic craniosynostosis

Andrew T. Timberlake, Kshipra Hemal, Jonas A. Gustafson, Le Thi Hao, Irene Valenzuela, Anne Slavotinek, Michael L. Cunningham, Kristopher T. Kahle, Richard P. Lifton, and John A. Persing

OBJECTIVE

Occurring once in every 2000 live births, craniosynostosis (CS) is the most frequent cranial birth defect. Although the genetic etiologies of syndromic CS cases are well defined, the genetic cause of most nonsyndromic cases remains unknown.

METHODS

The authors analyzed exome or RNA sequencing data from 876 children with nonsyndromic CS, including 291 case-parent trios and 585 additional probands. The authors also utilized the GeneMatcher platform and the Gabriella Miller Kids First genome sequencing project to identify additional CS patients with AXIN1 mutations.

RESULTS

The authors describe 11 patients with nonsyndromic CS harboring rare, damaging mutations in AXIN1, an inhibitor of Wnt signaling. AXIN1 regulates signaling upstream of key mediators of osteoblast differentiation. Three of the 6 mutations identified in trios occurred de novo in the proband, while 3 were transmitted from unaffected parents. Patients with nonsyndromic CS were highly enriched for mutations in AXIN1 compared to both expectation (p = 0.0008) and exome sequencing data from > 76,000 healthy controls (p = 2.3 × 10−6), surpassing the thresholds for genome-wide significance.

CONCLUSIONS

These findings describe the first phenotype associated with mutations in AXIN1, with mutations identified in approximately 1% of nonsyndromic CS cases. The results strengthen the existing link between Wnt signaling and maintenance of cranial suture patency and have implications for genetic testing in families with CS.

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Effect of vagus nerve stimulation on emergency department utilization in children with drug-resistant epilepsy: a retrospective cohort study

Nallammai Muthiah, Hope M. Reecher, and Taylor J. Abel

OBJECTIVE

Epilepsy affects approximately 470,000 children in the United States. The estimated median incidence is 50.4 cases per 100,000 persons per year. There are approximately 3.1 million seizure-related emergency department (ED) visits per year among children. Vagus nerve stimulation (VNS) is a treatment option for drug-resistant epilepsy (DRE). While its primary goal is to decrease seizure burden, VNS may decrease seizure intensity and improve quality of life. The authors assessed whether VNS decreased the number of seizure-related ED visits in a cohort of children with DRE.

METHODS

The authors performed a retrospective chart review of pediatric patients (aged 0–21 years) who underwent implantation of a vagus nerve stimulator between January 2009 and January 2020 at the University of Pittsburgh Medical Center Children’s Hospital of Pittsburgh. They used paired t-tests to assess differences in the number of ED visits 2 years before versus 2 years after VNS device implantation. Univariable linear regression analyses were used to test associations of preoperative characteristics with change in the number of ED visits following vagus nerve stimulator insertion.

RESULTS

This study included 240 patients. Compared with patients without seizure-related ED visits before VNS, patients with ≥ 1 ED visits were younger in age at first VNS surgery (9.5 vs 10.8 years), had a shorter epilepsy duration before VNS surgery (5.8 vs 7.4 years), had a later year of device implantation (2014 vs 2012), and on average took more antiseizure medications (ASMs; 2.4 vs 2.1). There was no significant difference between the total number of seizure-related ED visits pre– versus post–VNS surgery (1.72 vs 1.59, p = 0.50), and no difference in status epilepticus–related visits (0.59 vs 0.46, p = 0.17). Univariable linear regression analyses revealed a mean change in ED visits of +0.3 for each year prior to 2022 and −0.5 for each additional ASM that patients took before vagus nerve stimulator insertion.

CONCLUSIONS

This single-institution analysis demonstrated no significant change in the number of seizure-related ED visits within 2 years following VNS device implantation. Earlier VNS surgery was associated with more seizure-related ED visits after device insertion, suggesting that medical management and center experience may play a role in decreasing seizure-related ED visits. A greater number of ASMs was associated with fewer seizure-related ED visits after VNS device insertion, suggesting the role of medical management, patient baseline seizure threshold, and caregiver comfort with at-home seizure management.

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Reduction cranioplasty for hydrocephalic macrocephaly: a systematic review of surgical outcomes

Steven P. Moura, Alexandra D. Center, Manasa Kalluri, Jessica Blum, Ellen C. Shaffrey, Samuel Lee, Jinggang J. Ng, Bermans J. Iskandar, Catharine B. Garland, and Daniel Y. Cho

OBJECTIVE

Hydrocephalic macrocephaly can result in poor psychosocial development, positioning difficulties, skin breakdown, and poor cosmesis. Although reduction cranioplasty can address these sequelae, the postoperative outcomes, complications, and mortality risk of reduction cranioplasty are not well understood given the rarity of hydrocephalic macrocephaly. Therefore, the primary objective of this systematic review was to evaluate the surgical outcomes of reduction cranioplasty for the treatment of hydrocephalic macrocephaly.

METHODS

A systematic review was performed using the PubMed, Scopus, and Web of Science databases while following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two independent reviewers screened 350 studies; 27 studies reporting surgical outcomes on reduction cranioplasty for hydrocephalic macrocephaly met inclusion criteria. Data on study design, patient demographics, operative details, and surgical outcomes were collected.

RESULTS

There were 65 reduction cranioplasties among the 27 included studies. Eighteen (66.7%) studies presented level V evidence, 7 (25.9%) presented level IV evidence, and 2 (7.4%) presented level III evidence. Following reduction cranioplasty, there was improvement in postoperative head positioning in 23 (85.2%) studies, improvement in postoperative cosmesis in 22 (81.5%) studies, and improvement in global postoperative neurological functioning in 20 (74.1%) studies. The median estimated blood loss was 633 mL (range 20–2600 mL). Shunt revisions were the most common complication, reported in 9 (47.4%) of the 19 studies assessing complications. Of the 65 patients, there was a mortality rate of 6.2% (n = 4).

CONCLUSIONS

The majority of the included studies reported improvement in head size, head positioning, cranial cosmesis, and global neurological functioning following reduction cranioplasty for hydrocephalic macrocephaly. However, the prevalence of lower-level evidence, risk of blood loss, complications, and mortality indicates the need for a serious discussion of surgical indication, an experienced team, and thorough perioperative planning to perform these complex surgeries.

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Treatment of hydrocephalus following fetal repair of myelomeningocele: comparing endoscopic third ventriculostomy with choroid plexus cauterization to ventricular shunting

Justine Izah, Joseline Haizel-Cobbina, Shilin Zhao, E. Haley Vance, Michelle Dunlap, Stephen R. Gannon, Campbell Liles, Aaron M. Yengo-Kahn, Matthew E. Pontell, Robert P. Naftel, John C. Wellons III, and Michael C. Dewan

OBJECTIVE

The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC).

METHODS

This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings).

RESULTS

Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors’ institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (−0.06 vs −0.09, p = 0.37) and change in head circumference percentile (−1.33 vs −28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort.

CONCLUSIONS

In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.

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The association of COVID-19 with increased pediatric ventriculoatrial shunt failures: a national retrospective cohort

Uma V. Mahajan, Dana Defta, David C. Kaelber, Sanjay P. Ahuja, Brian D. Rothstein, and Krystal L. Tomei

OBJECTIVE

During the COVID-19 pandemic, the authors’ institution managed ventriculoatrial (VA) shunt complications in 2 teenage patients in close proximity to a symptomatic COVID-19 infection. Systemic thrombotic events are an established complication of COVID-19 infection due to a hypercoagulable state. Thrombotic complications, particularly elevated central venous pressure, can cause VA shunt failure. The true effect of COVID-19 on patients with intravascular devices is currently unknown. In this study, the authors aimed to determine if there was an association between COVID-19 infection and VA shunt failure.

METHODS

TriNetX, an aggregated electronic health record platform, was used to analyze data of more than 13 million US pediatric patients. Two matched cohorts of patients < 18 years of age with a VA shunt were defined. Group 1 (n = 311) had a positive laboratory test for COVID-19 from March 1, 2020, to March 31, 2022. Group 2 (n = 311), a control group, had any medical appointment from March 1, 2020, to March 31, 2022, and never had a positive laboratory test for COVID-19. The authors analyzed outcomes 1 year after testing positive for COVID-19 in group 1, and after the medical appointment in group 2. Outcomes included shunt complications, shunt revisions or replacements, and thromboembolic complications. To protect patient privacy, individual results of fewer than 10 patients are not specified in TriNetX.

RESULTS

Group 1 had a greater odds of mechanical shunt complication than group 2 (20% vs 4%, OR 5.71, 95% CI 3.07–10.62). Group 1 had a greater odds of shunt reoperation than group 2 (11% vs < 3%, OR > 3.7, 95% CI 1.72–7.62). There were 1–10 patients in group 1 (≤ 3% of group 1) who experienced a thromboembolism due to the shunt, compared with no patients in group 2 who had a thromboembolism due to the shunt.

CONCLUSIONS

This analysis demonstrates an association of shunt complications, reoperations, and thromboembolic events in patients with VA shunts following COVID-19 infection.

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Focal selective dorsal rhizotomy and concurrent deformity correction: a combined approach

Nisha Gadgil, Aloysia L. Schwabe, Edward Wright, Amy Barbuto, Eric L. Dugan, Sruthi P. Thomas, Jeffrey S. Shilt, Dorothy Beauvais, Yushane Shih, Brian G. Smith, David F. Bauer, and Daniel J. Curry

OBJECTIVE

Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria.

METHODS

The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures.

RESULTS

Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range −6 to 29, p < 0.001).

CONCLUSIONS

Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.

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The role of genetics on behavioral outcomes in nonsyndromic sagittal synostosis

David P. Alper, Mariana N. Almeida, Kevin G. Hu, Jenny F. Yang, Andrew T. Timberlake, Jinesh Shah, John A. Persing, and Michael Alperovich

OBJECTIVE

Previous work identified an association between genetics and neurodevelopmental delays in patients with nonsyndromic craniosynostosis. The authors investigated the role of genetic mutations on behavioral outcomes of patients with treated sagittal synostosis.

METHODS

Parents of children aged 6–18 years with surgically corrected sagittal synostosis were recruited to complete the Child Behavioral Checklist (overall behavioral problems), Conners 3rd Edition–Parent (attention-deficit/hyperactivity disorder), Social Responsiveness Scale 2nd Edition (autism spectrum disorder [ASD]), and Behavior Rating Inventory of Executive Function 2nd Edition (executive function). Genomic analysis was completed, and patients were identified if they had mutations in high probability of loss of function intolerant (pLI) genes (high pLI vs nonhigh pLI). Genetic burden was assessed relative to controls. Multivariate linear regression determined the association of mutations in high pLI genes with behavioral scores, while controlling for sociodemographic factors, age at surgery, surgery type, and IQ.

RESULTS

Sixteen of 45 patients were in the high pLI group. There were no differences between the groups in terms of sociodemographic factors. A greater proportion of children in the high pLI group scored at or above borderline clinical levels for aggression (18.8% vs 0.0%, p = 0.05) and externalizing problems (31.3% vs 3.7%, p = 0.02). Among children in the nonhigh pLI group, older age at surgery was associated with worse scores on the rule-breaking, aggression, and externalizing problems domains and four out of five ASD domains.

CONCLUSIONS

Children with treated nonsyndromic sagittal synostosis and mutations in high pLI genes had worse behavioral problems in externalizing behaviors and aggression, whereas older age at surgery was a significant predictor of worse behavioral outcomes in patients without mutations in high pLI genes.

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Letter to the Editor. Ventriculogallbladder shunts as treatment alternative for infants with necrotizing enterocolitis

Hector E. James