Surgical interventions and short-term outcomes for preterm infants with post-haemorrhagic hydrocephalus: a multicentre cohort study.

OBJECTIVE: To (1) describe differences in types and timing of interventions, (2) report short-term outcomes and (3) describe differences among centres from a large national cohort of preterm infants with post-haemorrhagic hydrocephalus (PHH). DESIGN: Cohort study of the Children's Hospitals Neonatal Database from 2010 to 2022. SETTING: 41 referral neonatal intensive care units (NICUs) in North America. PATIENTS: Infants born before 32 weeks' gestation with PHH defined as acquired hydrocephalus with intraventricular haemorrhage. INTERVENTIONS: (1) No intervention, (2) temporising device (TD) only, (3) initial permanent shunt (PS) and (4) TD followed by PS (TD-PS). MAIN OUTCOME MEASURES: Mortality and meningitis. RESULTS: Of 3883 infants with PHH from 41 centres, 36% had no surgical intervention, 16% had a TD only, 19% had a PS only and 30% had a TD-PS. Of the 46% of infants with TDs, 76% were reservoirs; 66% of infants with TDs required PS placement. The percent of infants with PHH receiving ventricular access device placement differed by centre, ranging from 4% to 79% (p<0.001). Median chronological and postmenstrual age at time of TD placement were similar between infants with only TD and those with TD-PS. Infants with TD-PS were older and larger than those with only PS at time of PS placement. Death before NICU discharge occurred in 12% of infants, usually due to redirection of care. Meningitis occurred in 11% of the cohort. CONCLUSIONS: There was significant intercentre variation in rate of intervention, which may reflect variability in care or referral patterns. Rate of PS placement in infants with TDs was 66%.

Periventricular nodular heterotopia in patients with a prenatal diagnosis of myelomeningocele/myeloschisis: associations with seizures and neurodevelopmental outcomes during early childhood.

PURPOSE: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH). METHODS: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy. RESULTS: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains. CONCLUSION: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Use of a machine learning algorithm with a focus on spinopelvic parameters to predict development of symptomatic tethered cord after initial untethering surgery.

OBJECTIVE: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters. METHODS: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters. RESULTS: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%). CONCLUSIONS: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.

Putting our heads together: The future of craniopagus twin separation.

Computer-assisted design and computer-assisted modeling (CAD/CAM), virtual surgical planning (VSP) and augmented/virtual reality (AR/VR) aid our ability to plan and perform complex craniofacial procedures. This study seeks to define the role of the aforementioned techniques in the separation of craniopagus conjoined twins. Six teams were identified who had successfully performed craniopagus twin separation with the use of CAD/CAM, VSP and/or AR/VR. Surgeons involved in separating craniopagus twins have increasingly utilized tools such as CAD/CAM models, VSP and AR/VR to plan and execute successful separation, and these tools are associated with higher success rates than historical controls.

Near-infrared spectroscopy monitoring in severe pediatric abusive head trauma.

OBJECTIVE: Abusive head trauma (AHT) is one of the most devastating forms of pediatric traumatic brain injury (TBI). It commonly presents with seizures, which may contribute to poor neurological outcome following trauma. Noninvasive near-infrared spectroscopy (NIRS) neuromonitoring may provide information on cerebral oxygenation and perfusion. In this study, the authors evaluated whether NIRS regional cerebral oxygen saturation (rSO2) values were associated with seizure activity confirmed by electroencephalography (EEG) and whether NIRS neuromonitoring could aid in seizure detection in patients with severe AHT. METHODS: The authors retrospectively analyzed pediatric patients aged ≤ 18 years who were admitted to a quaternary urban pediatric hospital from 2016 to 2022 with severe AHT, who received NIRS and EEG monitoring during their hospital course. They evaluated clinical presentation and hospital course, including imaging findings, EEG findings, and NIRS rSO2 values. RESULTS: Nineteen patients with severe AHT were monitored with both EEG and NIRS. The median age was 3.4 months, and 14 patients experienced seizures confirmed by EEG. On average, rSO2 values before, during, and after seizure did not differ significantly. However, within individual patients, bilateral regional NIRS rSO2 (bilateral forehead region) was seen to rise in the hour preceding seizure activity and during periods of frequent seizure activity, confirmed by EEG in the bilateral frontal-midline brain regions. CONCLUSIONS: To the best of the authors' knowledge, this is the largest study to analyze NIRS and seizures confirmed by EEG in the severe AHT population. The relationship between NIRS values and seizures in this series of pediatric patients with severe AHT suggests that, overall, regional NIRS cannot predict early seizures. However, increased cerebral oxygenation preceding seizure activity and during seizure activity may be detected by regional NIRS in certain patients with local seizure activity. Future studies with larger sample sizes may help elucidate the relationship between seizures and cerebral oxygenation in different regions in severe pediatric AHT.

Intracranial Pressure and Brain Tissue Oxygen Multimodality Neuromonitoring in Gunshot Wounds to the Head in Children.

OBJECTIVE: Gunshot wounds to the head (GSWH) are a cause of severe penetrating traumatic brain injury (TBI). Although multimodal neuromonitoring has been increasingly used in blunt pediatric TBI, its role in the pediatric population with GSWH is not known. We report on 3 patients who received multimodal neuromonitoring as part of clinical management at our institution and review the existing literature on pediatric GSWH. METHODS: We identified 3 patients ≤18 years of age who were admitted to a quaternary children's hospital from 2005 to 2021 with GSWH and received invasive intracranial pressure (ICP) and Pbto2 (brain tissue oxygenation) monitoring with or without noninvasive near-infrared spectroscopy (NIRS). We analyzed clinical and demographic characteristics, imaging findings, and ICP, Pbto2, cerebral perfusion pressure, and rSo2 (regional cerebral oxygen saturation) NIRS trends. RESULTS: All patients were male with an average admission Glasgow Coma Scale score of 4. One patient received additional NIRS monitoring. Episodes of intracranial hypertension (ICP ≥20 mm Hg) and brain tissue hypoxia (Pbto2 <15 mm Hg) or hyperemia (Pbto2 >35 mm Hg) frequently occurred independently of each other, requiring unique targeted treatments. rSo2 did not consistently mirror Pbto2. All children survived, with favorable Glasgow Outcome Scale-Extended score at 6 months after injury. CONCLUSIONS: Use of ICP and Pbto2 multimodality neuromonitoring enabled specific management for intracranial hypertension or brain tissue hypoxia episodes that occurred independently of one another. Multimodality neuromonitoring has not been studied extensively in pediatric GSWH; however, its use may provide a more complete picture of patient injury and prognosis without significant added procedural risk.

Craniosynostosis in primary metabolic bone disorders: a single-institution experience.

PURPOSE: The incidence of metabolic bone diseases in pediatric neurosurgical patients is rare. We examined our institutional experience of metabolic bone diseases along with a review of the literature in an effort to understand management for this rare entity. METHODS: Retrospective review of the electronic medical record database was performed to identify patients with primary metabolic bone disorders who underwent craniosynostosis surgery between 2011 and 2022 at a quaternary referral pediatric hospital. Literature review was conducted for primary metabolic bone disorders associated with craniosynostosis. RESULTS: Ten patients were identified, 6 of whom were male. The most common bone disorders were hypophosphatemic rickets (n = 2) and pseudohypoparathyroidism (n = 2). The median age at diagnosis of metabolic bone disorder was 2.02 years (IQR: 0.11-4.26), 2.52 years (IQR: 1.24-3.14) at craniosynostosis diagnosis, and 2.65 years (IQR: 0.91-3.58) at the time of surgery. Sagittal suture was most commonly fused (n = 4), followed by multi-suture craniosynostosis (n = 3). Other imaging findings included Chiari (n = 1), hydrocephalus (n = 1), and concurrent Chiari and hydrocephalus (n = 1). All patients underwent surgery for craniosynostosis, with the most common operation being bifronto-orbital advancement (n = 4). A total of 5 patients underwent reoperation, 3 of which were planned second-stage surgeries and 2 of whom had craniosynostosis recurrence. CONCLUSIONS: We advocate screening for suture abnormalities in children with primary metabolic bone disorders. While cranial vault remodeling is not associated with a high rate of postoperative complications in this patient cohort, craniosynostosis recurrences may occur, and parental counseling is recommended.

Tumors of the brachial plexus region: A 15-year experience with emphasis on motor and pain outcomes and literature review.

Background: Brachial plexus region tumors are rare. In this study, we reviewed our experience with resection of tumors involving or adjacent to the brachial plexus to identify patterns in presentation and outcome. Methods: We report a retrospective case series of brachial plexus tumors operated on by a single surgeon at a single institution over 15 years. Outcome data were recorded from the most recent follow-up office visit. Findings were compared to a prior internal series and comparable series in the literature. Results: From 2001 to 2016, 103 consecutive brachial plexus tumors in 98 patients met inclusion criteria. Ninety percent of patients presented with a palpable mass, and 81% had deficits in sensation, motor function, or both. Mean follow-up time was 10 months. Serious complications were infrequent. For patients with a preoperative motor deficit, the rate of postoperative motor decline was 10%. For patients without a preoperative motor deficit, the rate of postoperative motor decline was 35%, which decreased to 27% at 6 months. There were no differences in motor outcome based on extent of resection, tumor pathology, or age. Conclusion: We present one of the largest recent series of tumors of the brachial plexus region. Although the rate of worsened postoperative motor function was higher in those without preoperative weakness, the motor deficit improves over time and is no worse than antigravity strength in the majority of cases. Our findings help guide patient counseling in regard to postoperative motor function.

Intracranial Pressure Patterns in Children with Sagittal Craniosynostosis.

BACKGROUND: Elevated intracranial pressure (ICP) in sagittal craniosynostosis has a wide spectrum of reported incidence, and patterns are not well understood across infancy and childhood. Characterizing the natural history of ICP in this population may clarify risks for neurocognitive delay and inform treatment decisions. METHODS: Infants and children with sagittal craniosynostosis and unaffected control subjects were prospectively evaluated with spectral-domain optical coherence tomography (OCT) from 2014-2021. Elevated ICP was determined based on previously validated algorithms utilizing retinal OCT parameters. RESULTS: Seventy-two patients with isolated sagittal craniosynostosis, and 25 control subjects were evaluated. Overall, 31.9% (n=23) of patients with sagittal craniosynostosis had evidence of ICP ≥15 mmHg, and 27.8% (n=20) of patients had ICP ≥20 mmHg.Children with sagittal craniosynostosis younger than 6 months of age were more likely to have normal intracranial pressure (88.6% <15 mmHg, 91.4% <20 mmHg) than those between 6-12 months of age (54.5%, p=.013; 54.5%, p=.005) and than those older than 12 months of age (46.2%, p<.001; 53.8%, p=.001). Intracranial pressure was directly correlated with severity of scaphocephaly (p=.009). No unaffected control subjects at any age exhibited retinal thickening suggestive of elevated ICP. CONCLUSIONS: Elevated ICP is rare in isolated sagittal craniosynostosis below 6 months of age, but becomes significantly more common after 6 months of age, and may correlate with severity of scaphocephaly.

Spinal pseudomeningocele closure: capsulofascial interposition technique.

INTRODUCTION: The development of a spinal pseudomeningocele is a complication of dural repair or reconstruction that carries significant morbidity for pediatric patients. In addition to cerebrospinal fluid (CSF) hypotension and positional headaches, CSF leaking into the extradural space increases the risk for incisional breakdown, meningitis, and cosmetic deformity. Spinal pseudomeningocele management is challenging, and reported techniques range from local wound revisions/exploration to shunt placement for permanent CSF diversion. METHODS: At our institution, we have recently implemented a multidisciplinary approach of capsulofascial interposition for the surgical repair of the symptomatic spinal pseudomeningocele with a combined neurosurgery-plastic surgery team. To our knowledge, this technique has not previously been characterized, and we describe the technical aspects of this surgery here. RESULTS: Among 10 patients treated with this technique, none required reoperation or developed infections following pseudomeningocele closure; 2 patients received postoperative transfusions; and 2 patients underwent CSF diversion procedures. CONCLUSION: We propose that this capsulofascial interposition technique should be employed in the surgical treatment of symptomatic spinal pseudomeningoceles.

Long-term aesthetic and photogrammetric outcomes in non-syndromic unicoronal synostosis: comparison of fronto-orbital distraction osteogenesis and fronto-orbital advancement and remodeling.

BACKGROUND: Fronto-orbital advancement and remodeling (FOAR) is among the most common surgical approaches for unicoronal craniosynostosis (UCS), although some data demonstrate failure to achieve long-term aesthetic normalcy, leading some to seek alternative treatment paradigms such as fronto-orbital distraction osteogenesis (FODO). This study compares long-term aesthetic outcomes of patients with UCS treated with FOAR and FODO. METHODS: Twenty patients (four males) with non-syndromic UCS presenting to our institution and undergoing distraction were compared to a matched cohort of 20 patients (six males) undergoing FOAR. Clinical photographs and ImageJ were used to quantify periorbital anatomy including palpebral fissures, pupil-to-brow distance (PTB), and margin-reflex distance (MRD1) in pixels. Whitaker classification was blindly assigned by craniofacial surgeons. RESULTS: Photogrammetric analysis and Mann-Whitney U tests demonstrated significantly improved postoperative symmetry in distraction patients for palpebral width (p = 0.020), MRD1 (p = 0.045), and canthal tilt (p = 0.010). Average Whitaker classification scores between FOAR (1.94) and distraction (1.79) cohorts were similar (p = 0.374). CONCLUSIONS: UCS patients demonstrated significant postoperative improvements in periorbital symmetry, with distraction patients demonstrating superior results in palpebral width and canthal tilt. FOAR and FODO patients achieved similar Whitaker classification scores. These cohorts will be followed until craniofacial maturity prior to making any definitive conclusions.

Tethered cord release in patients after open fetal myelomeningocele closure: intraoperative neuromonitoring data and patient outcomes.

PURPOSE: The purpose of the study was to better understand the clinical course and impact of tethered cord release surgery on patients who have previously undergone open spinal dysraphism closure in utero. METHODS: This is a single-center retrospective observational study on patients undergoing tethered cord release after having previously had open fetal myelomeningocele (MMC) closure. All patients underwent tethered cord release surgery with a single neurosurgeon. A detailed analysis of the patients' preoperative presentation, intraoperative neuromonitoring (IONM) data, and postoperative course was performed. RESULTS: From 2009 to 2021, 51 patients who had previously undergone fetal MMC closure had tethered cord release surgery performed. On both preoperative and postoperative manual motor testing, patients were found to have on average 2 levels better than would be expected from the determined anatomic level from fetal imaging. The electrophysiologic functional level was found on average to be 2.5 levels better than the anatomical fetal level. Postoperative motor levels when tested on average at 4 months were largely unchanged when compared to preoperative levels. Unlike the motor signals, 46 (90%) of patients had unreliable or undetectable lower extremity somatosensory evoked potentials (SSEPs) prior to the tethered cord release. CONCLUSION: Tethered cord surgery can be safely performed in patients after open fetal MMC closure without clinical decline in manual motor testing. Patients often have functional nerve roots below the anatomic level. Sensory function appears to be more severely affected in patients leading to a consistent motor-sensory imbalance.

Survey of Quaternary Neonatal Management of Posthemorrhagic Hydrocephalus.

OBJECTIVE: This study aimed to determine clinical care practices for infants at risk for posthemorrhagic hydrocephalus (PHH) across level IV neonatal intensive care units (NICUs). STUDY DESIGN: Cross-sectional survey that addressed center-specific surveillance, neurosurgical intervention, and follow-up practices within the Children's Hospitals Neonatal Consortium. RESULTS: We had a 59% (20/34 sites) response rate, with 10 sites having at least two participants. Respondents included neonatologists (53%) and neurosurgeons (35%). Most participants stated having a standard guideline for PHH (79%). Despite this, 42% of respondents perceive inconsistencies in management. Eight same-center pairs of neonatologists and neurosurgeons were used to determine response agreement. Half of these pairs disagreed on nearly all aspects of care. The greatest agreement pertained to a willingness to adopt a consensus-based protocol. CONCLUSION: Practice variation in the management of infants at risk of PHH in level IV NICUs exists despite the perception that a common practice is available and used. KEY POINTS: · Practice variation exists despite the perception that common practices are available/used for PHH.. · Our survey had same-center pairs of neonatologist and neurosurgeons to determine response agreement.. · The greatest agreement pertained to a willingness to adopt a consensus-based protocol..

Preprocedural Electrophysiological Monitoring in Craniofacial Surgery for a Patient with Chiari Malformation.

Head and neck positioning is a key element of craniofacial reconstructive surgery and can become challenging when intervention necessitates broad exposure of the calvarium. We present a case of craniosynostosis secondary to Apert's syndrome requiring anterior and posterior cranial vault access during surgical correction. A modified sphinx position was used that required significant neck extension. The patient had concurrent Chiari I malformation with brain stem compression so intraoperative neuromonitoring (IONM) was used to ensure that there were no negative effects on the neural elements with positioning. This highlights benefits of IONM in a setting not typically associated with its use.

How Low Should We Go? Safety and Craniometric Impact of the Low Occipital Osteotomy in Posterior Vault Remodeling.

BACKGROUND: A larger volume cranial vault expansion is likely facilitated by a low posterior cranial osteotomy beneath the torcula; however, this may impart an increased risk of venous bleeding. The authors compared the safety of infratorcular versus supratorcular osteotomy in patients undergoing posterior vault reconstruction or posterior vault distraction osteogenesis and analyzed volumetric changes. METHODS: Patients undergoing initial posterior vault reconstruction or distraction osteogenesis between 2009 and 2021 at the authors' institution were grouped by occipital osteotomy location and analyzed retrospectively. Craniometric analysis was performed if patients had high-resolution computed tomography scans available within 180 days preoperatively and postoperatively. RESULTS: A total of 187 patients were included: 106 (57 percent) who underwent posterior vault distraction osteogenesis and 81 (43 percent) who underwent posterior vault reconstruction. Infratorcular osteotomy was more common in reconstruction [ n = 65 (80 percent)] than in distraction osteogenesis [ n = 61 (58 percent); p < 0.002]. Blood transfused was similar between low and high osteotomy cohorts in the distraction osteogenesis ( p = 0.285) and reconstruction ( p = 0.342) groups. However, median transfused blood volume per kilogram of patient weight was greater in the low versus high osteotomy distraction osteogenesis ( p = 0.010) and reconstruction ( p = 0.041) cohorts. Intraoperative venous sinus injury was rare. In the distraction osteogenesis cohort, there was increased median intracranial volumetric gain in the low (263 ml) compared with the high osteotomy cohort (127 ml; p = 0.043); however, when controlled for distraction distance, only a trend was observed ( p = 0.221). Patients undergoing distraction osteogenesis showed a larger median intracranial volume increase (168 ml) compared with those undergoing reconstruction (73 ml; p < 0.001). CONCLUSION: Infratorcular osteotomy can be performed safely in most patients undergoing cranial vault remodeling and does not appear to be associated with greater hemodynamic instability or sinus injury. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Time to shunt failure in children with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

OBJECTIVE: Hydrocephalus is common among children with myelomeningocele and is most frequently treated with a ventriculoperitoneal shunt (VPS). Although much is known about factors related to first shunt failure, relatively less data are available about shunt failures after the first one. The purpose of this study was to use a large data set to explore time from initial VPS placement to first shunt failure in children with myelomeningocele and to explore factors related to multiple shunt failures. METHODS: Data were obtained from the National Spina Bifida Patient Registry. Children with myelomeningocele who were enrolled within the first 5 years of life and had all lifetime shunt operations recorded in the registry were included. Kaplan-Meier survival curves were constructed to evaluate time from initial shunt placement to first shunt failure. The total number of children who experienced at least 2 shunt failures was calculated. A proportional means model was performed to calculate adjusted hazard ratios (HRs) for shunt failure on the basis of sex, race/ethnicity, lesion level, and insurance status. RESULTS: In total, 1691 children met the inclusion criteria. The median length of follow-up was 5.0 years. Fifty-five percent of patients (938 of 1691) experienced at least 1 shunt failure. The estimated median time from initial shunt placement to first failure was 2.34 years (95% confidence interval [CI] 1.91-3.08 years). Twenty-six percent of patients had at least 2 shunt failures, and 14% of patients had at least 3. Male children had higher likelihood of shunt revision (HR 1.25, 95% CI 1.09-1.44). Children of minority race/ethnicity had a lower likelihood of all shunt revisions (non-Hispanic Black children HR 0.74, 95% CI 0.55-0.98; Hispanic children HR 0.74, 95% CI 0.62-0.88; children of other ethnicities HR 0.80, 95% CI 0.62-1.03). CONCLUSIONS: Among the children with myelomeningocele, the estimated median time to shunt failure was 2.34 years. Forty-five percent of children never had shunt failure. The observed higher likelihood of shunt revisions among males and lower likelihood among children of minority race/ethnicity illustrate a possible disparity in hydrocephalus care that warrants additional study. Overall, these results provide important information that can be used to counsel parents of children with myelomeningocele about the expected course of shunted hydrocephalus.

High prevalence of gram-negative and multiorganism surgical site infections after pediatric complex tethered spinal cord surgery: a multicenter study.

OBJECTIVE: Complex tethered spinal cord (cTSC) release in children is often complicated by surgical site infection (SSI). Children undergoing this surgery share many similarities with patients undergoing correction for neuromuscular scoliosis, where high rates of gram-negative and polymicrobial infections have been reported. Similar organisms isolated from SSIs after cTSC release were recently demonstrated in a single-center pilot study. The purpose of this investigation was to determine if these findings are reproducible across a larger, multicenter study. METHODS: A multicenter, retrospective chart review including 7 centers was conducted to identify all cases of SSI following cTSC release during a 10-year study period from 2007 to 2017. Demographic information along with specific microbial culture data and antibiotic sensitivities for each cultured organism were collected. RESULTS: A total of 44 SSIs were identified from a total of 655 cases, with 78 individual organisms isolated. There was an overall SSI rate of 6.7%, with 43% polymicrobial and 66% containing at least one gram-negative organism. Half of SSIs included an organism that was resistant to cefazolin, whereas only 32% of SSIs were completely susceptible to cefazolin. CONCLUSIONS: In this study, gram-negative and polymicrobial infections were responsible for the majority of SSIs following cTSC surgery, with approximately half resistant to cefazolin. Broader gram-negative antibiotic prophylaxis should be considered for this patient population.

Risk of ventriculoperitoneal shunt malfunction in operatively treated early onset spinal deformity.

PURPOSE: Ventriculoperitoneal (VP) shunt placement is a common neurosurgical procedure performed in patients with early onset scoliosis (EOS). To provide insight into the risks of spine lengthening operations, we investigate the rate of VP shunt complications in patients with EOS undergoing spinal deformity correction interventions. METHODS: A retrospective review was performed of all patients with EOS at a single institution undergoing spinal deformity correction procedures from 2007 to 2018. Patients having undergone VP shunt implantation prior to deformity correction were included. A minimum of 2-year follow-up was required for inclusion. Clinical records and imaging studies were reviewed. RESULTS: Nineteen patients with VP shunts underwent Vertical Expandable Prosthetic Titanium Rib (VEPTR) implantation for treatment of early onset spinal deformity. The mean age at shunt placement and spine instrumentation surgery was 13.7 months (1 day to 13 years) and 6.1 years (0.5-15.1) respectively. The diagnoses associated with shunt implantation were: 12 spina bifida, 3 structural defects or obstructions, 2 intraventricular hemorrhage, 1 cerebral palsy, and 1 campomelic dwarfism. During the first 2 years following rib-based insertion, there was a mean of 2.5 expansion/revision procedures (0-5) with no shunt-related complications. The mean length of follow-up in this series was 7.0 years (2.6-13.2). A total of three (16%) patients required shunt revision following their rib-based device insertion, two patients with proximal shunt malfunctions and one with a mid-catheter breakage, at 2.4, 2.6, and 5.6 years, respectively, after rod implantation (Fig. 2). Each of these shunt revisions occurred more than 50 days following an expansion procedure (1.9, 2.9, and 5.7 months, respectively). CONCLUSION: Growing instrumentation procedures in EOS are associated with low risk for post-operative shunt complications in patients with ventriculoperitoneal shunts. There were no shunt revision procedures performed in the first 2 years following rib-based device insertion. Sixteen percent of patients went on to require a shunt revision at some point during their follow-up, which is comparable to the baseline rate of shunt revision in non-EOS patients. LEVEL OF EVIDENCE: IV, Case series.

Invasive brain tissue oxygen and intracranial pressure (ICP) monitoring versus ICP-only monitoring in pediatric severe traumatic brain injury.

OBJECTIVE: Severe traumatic brain injury (TBI) is a leading cause of disability and death in the pediatric population. While intracranial pressure (ICP) monitoring is the gold standard in acute neurocritical care following pediatric severe TBI, brain tissue oxygen tension (PbtO2) monitoring may also help limit secondary brain injury and improve outcomes. The authors hypothesized that pediatric patients with severe TBI and ICP + PbtO2 monitoring and treatment would have better outcomes than those who underwent ICP-only monitoring and treatment. METHODS: Patients ≤ 18 years of age with severe TBI who received ICP ± PbtO2 monitoring at a quaternary children's hospital between 1998 and 2021 were retrospectively reviewed. The relationships between conventional measurements of TBI were evaluated, i.e., ICP, cerebral perfusion pressure (CPP), and PbtO2. Differences were analyzed between patients with ICP + PbtO2 versus ICP-only monitoring on hospital and pediatric intensive care unit (PICU) length of stay (LOS), length of intubation, Pediatric Intensity Level of Therapy scale score, and functional outcome using the Glasgow Outcome Score-Extended (GOS-E) scale at 6 months postinjury. RESULTS: Forty-nine patients, including 19 with ICP + PbtO2 and 30 with ICP only, were analyzed. There was a weak negative association between ICP and PbtO2 (ß = -0.04). Conversely, there was a strong positive correlation between CPP ≥ 40 mm Hg and PbtO2 ≥ 15 and ≥ 20 mm Hg (ß = 0.30 and ß = 0.29, p < 0.001, respectively). An increased number of events of cerebral PbtO2 < 15 mm Hg or < 20 mm Hg were associated with longer hospital (p = 0.01 and p = 0.022, respectively) and PICU (p = 0.015 and p = 0.007, respectively) LOS, increased duration of mechanical ventilation (p = 0.015 when PbtO2 < 15 mm Hg), and an unfavorable 6-month GOS-E score (p = 0.045 and p = 0.022, respectively). An increased number of intracranial hypertension episodes (ICP ≥ 20 mm Hg) were associated with longer hospital (p = 0.007) and PICU (p < 0.001) LOS and longer duration of mechanical ventilation (p < 0.001). Lower minimum hourly and average daily ICP values predicted favorable GOS-E scores (p < 0.001 for both). Patients with ICP + PbtO2 monitoring experienced longer PICU LOS (p = 0.018) compared to patients with ICP-only monitoring, with no significant GOS-E score difference between groups (p = 0.733). CONCLUSIONS: An increased number of cerebral hypoxic episodes and an increased number of intracranial hypertension episodes resulted in longer hospital LOS and longer duration of mechanical ventilator support. An increased number of cerebral hypoxic episodes also correlated with less favorable functional outcomes. In contrast, lower minimum hourly and average daily ICP values, but not the number of intracranial hypertension episodes, were associated with more favorable functional outcomes. There was a weak correlation between ICP and PbtO2, supporting the importance of multimodal invasive neuromonitoring in pediatric severe TBI.