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.

Pediatric mild head trauma: is outpatient follow-up imaging necessary or beneficial?

OBJECTIVE: Pediatric traumatic brain injury (TBI) is the leading cause of death among children and is a significant cause of morbidity. However, the majority of injuries are mild (Glasgow Coma Scale score 13-15) without any need for neurosurgical intervention, and clinically significant neurological decline rarely occurs. Although the question of repeat imaging within the first 24 hours has been discussed in the past, the yield of short-term follow-up imaging has never been thoroughly described. In this paper, the authors focus on the yield of routine repeat imaging for pediatric mild TBI (mTBI) at the first clinic visit following hospital discharge. METHODS: The authors conducted a retrospective review of patients with pediatric brain trauma who had been admitted to Johns Hopkins All Children's Hospital (JHACH). Patients with mTBI were identified, and their presentation, hospital course, and imaging results were reviewed. Those pediatric patients with mTBI who had undergone no procedure during their initial admission (only conservative treatment) were eligible for inclusion in the study. Two distinct groups were identified: patients who underwent repeated imaging at their follow-up clinic visit and those who underwent only clinical evaluation. Each case was assessed on whether the follow-up imaging had changed the follow-up course. RESULTS: Between 2010 and 2015, 725 patients with TBI were admitted to JHACH. Of those, 548 patients qualified for analysis (i.e., those with mTBI who received conservative treatment without any procedure and were seen in the clinic for follow-up evaluation within 8 weeks after the trauma). A total of 392 patients had only clinic follow-up, without any diagnostic imaging study conducted as part of their clinic visit, whereas the other 156 patients underwent repeat MRI. Only 1 patient had a symptomatic change and was admitted after undergoing imaging. For 30 patients (19.2%), it was decided after imaging to continue the neurosurgical follow-up, which is a change from the institutional paradigm after mTBI. None of these patients had a change in neurological status, and all had a good functional status. All of these patients had one more follow-up in the clinic with new MRI, and none of them required further follow-up. CONCLUSIONS: Children with mTBI are commonly followed up in the ambulatory clinic setting. The authors believe that for children with mTBI, normal clinical examination, and no new symptoms, there is no need for routine ambulatory imaging since the clinical yield of such is relatively low.

Adolescent Disc Disease: Risk Factors and Treatment Success-Related Factors.

OBJECTIVE: A paucity of literature is available discussing the associated risk factors, treatment options (including the use of minimally invasive surgery), and outcomes related to lumbar disc herniation (LDH) in children. We have discussed the risk factors for disc disease among pediatric patients and evaluated the efficacy of the minimally invasive approach. METHODS: A retrospective review of pediatric patients with lumbar disc disease who had undergone microdiscectomy at our institution from 2005 to 2016 was conducted. The preoperative presentation, hospital course, postoperative course, and follow-up data (≥3 years) were reviewed. We evaluated the risk factors for LDH and the surgical outcomes for both groups. RESULTS: A total of 52 pediatric patients had undergone 61 lumbar disc surgeries for LDH in our department from 2005 to 2016. Their average age at surgery was 16.65 years. Of the 61 procedures, 48 (78.7%) had been performed via the minimally invasive spine microdiscectomy approach and 13 (21.3%) via the open microdiscectomy approach. The average body mass index for all cases was 29.3 kg/m2. The average interval to diagnosis was 7.9 months. Of the 61 cases, 21 (34.4%) had been required for patients who were competitive athletes. In addition, 15 had been for LDH related to trauma (24.6%). In 46 of the 61 cases, complete resolution of the symptoms had occurred at the 1-year follow-up visit (79.2% of minimally invasive spine microdiscectomy vs 61.5% of open microdiscectomy). CONCLUSION: Risk factors similar to those for adult LDH, such as an elevated body mass index, can be seen in the pediatric population. However, some unique risk factors such as post-traumatic LDH were found in the pediatric age group. Minimally invasive techniques are demonstrably safe and useful in this patient population.

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: In patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression. METHODS: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging. RESULTS: Of 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis. CONCLUSIONS: In this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.

OBJECTIVE: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction. METHODS: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx. RESULTS: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications. CONCLUSIONS: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors' goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis. METHODS: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°). RESULTS: Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB-C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude. CONCLUSIONS: Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.

Increased complications without neurological benefit are associated with prophylactic spinal cord untethering prior to scoliosis surgery in children with myelomeningocele.

PURPOSE: Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. METHODS: A multidisciplinary, retrospective cohort study from seven children's hospitals was performed including asymptomatic children with MMC < 21 years old, managed with or without prophylactic untethering prior to scoliosis surgery. Patients were divided into three groups for analysis: (1) untethering at the time of scoliosis surgery (concomitant untethering), (2) untethering within 3 months of scoliosis surgery (prior untethering), and (3) no prophylactic untethering. Baseline data, intra-operative reports, and 90-day post-operative outcomes were analyzed to assess for differences in neurologic outcomes, surgical complications, and overall length of stay. RESULTS: A total of 208 patients were included for analysis (mean age 9.4 years, 52% girls). No patient in any of the groups exhibited worsened motor or sensory function at 90 days post-operatively. However, comparing the prophylactic untethering groups with the group that was not untethered, there was an increased risk of surgical site infection (SSI) (31.3% concomitant, 28.6% prior untethering vs. 12.3% no untethering; p = 0.0104), return to the OR (43.8% concomitant, 23.8% prior untethering vs. 17.4% no untethering; p = 0.0047), need for blood transfusion (51.6% concomitant, 57.1% prior untethering vs. 33.8% no untethering; p = 0.04), and increased mean length of stay (LOS) (13.4 days concomitant, 10.6 days prior untethering vs. 6.8 days no untethering; p < 0.0001). In multivariable logistic regression analysis, prophylactic untethering was independently associated with increased adjusted relative risks of surgical site infection (aRR = 2.65, 95% CI 1.17-5.02), unplanned re-operation (aRR = 2.17, 95% CI 1.02-4.65), and any complication (aRR = 2.25, 95% CI 1.07-4.74). CONCLUSION: In this study, asymptomatic children with myelomeningocele who underwent scoliosis surgery developed no neurologic injuries regardless of prophylactic untethering. However, those who underwent prophylactic untethering were more likely to experience SSIs, return to the OR, need a blood transfusion, and have increased LOS than children not undergoing untethering. Based on these data, prophylactic untethering in asymptomatic MMC patients prior to scoliosis surgery does not provide any neurological benefit and is associated with increased surgical risks.

Postoperative Drain Use in Cranial Vault Remodeling: A Survey of Craniofacial Surgeon Practices and a Review of the Literature.

BACKGROUND: The use of subgaleal drains following primary cranioplasty for craniosynostosis has undergone limited investigation. Proposed benefits include prevention of seroma, detection of postoperative bleeding, and cerebrospinal fluid leak. We conducted a systematic review of the literature and surveyed craniofacial surgeons to ascertain the current evidence pertaining to drain use following primary cranioplasty for craniosynostosis and to determine surgical practice patterns. METHODS: PubMed and Embase databases were searched to identify relevant articles. Abstracts were reviewed by 2 investigators, and a Cohen κ statistic was calculated. Patient demographic and outcome data were extracted and compared. A 9-question survey was e-mailed to active and associate members of the American Society of Craniofacial Surgeons. RESULTS: A total of 7395 unique citations were identified. Only 2 retrospective chart reviews met inclusion criteria. All objective parameters demonstrated no difference between patients with and without drains. A subjective benefit of limiting facial swelling was proposed without objective analysis. Fifty (32.5%) of the 154 craniofacial surgeons responded to the survey. Forty-two percent used postoperative drains. A significant association (P = .01) was found between the belief that drains limited facial swelling and their use. CONCLUSIONS: The literature examining postoperative drain use in primary cranioplasty for craniosynostosis is restricted. The current studies show no definite benefit to drain use but are limited in their assessment of key outcomes. There is wide variability among surgeons regarding drain use, and this seems to be motivated by belief and tradition.

Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis.

Despite a known association of mucopolysaccharidoses (MPS) and craniosynostosis, treatment of elevated intracranial pressure (ICP) in these patients is primarily cerebrospinal fluid (CSF) shunting. We present a unique case of Hurler-Scheie syndrome with multisuture craniosynostosis and elevated ICP, without ventriculomegaly, where elevated ICP was successfully treated with extensive cranial vault expansion and shunt placement was avoided. Patients with MPS should be evaluated for craniosynostosis, and calvarial vault expansion may be considered as a viable treatment alternative to CSF shunting for elevated ICP in select patients.

Incidentally found brain tumors in the pediatric population: a case series and proposed treatment algorithm.

PURPOSE: With the increasing use of imaging, there has been an increase in the number of incidentally found brain lesions in pediatric patients resulting in a treatment dilemma for physicians and emotional strain for patients and families. Adult studies support initial surveillance of incidentally found low grade appearing lesions as the most appropriate approach. The aim of this study was to evaluate incidental lesions in the pediatric population and propose an initial treatment algorithm for such lesions. METHODS: Pediatric records were retrospectively reviewed at Johns Hopkins All Children's Hospital for incidentally found brain tumors between 2000 and 2017. Demographic data, presenting symptoms, treatment approach, and outcomes were reviewed for 55 patients, age 0-18. RESULTS: Of the 55 patients included in the study, 14 underwent surgical resection, 3 underwent biopsy, and 38 with benign imaging characteristics at presentation were monitored with radiology alone. Only one patient, out of the 17 that underwent resection or biopsy, had pathology consistent with a high grade glioma. Of the patients monitored radiographically 10 total patients showed an increase in the overall size of the lesion; however after a median follow up of 34.2 months only 2 increased to a degree that required surgical intervention. CONCLUSIONS: The majority of incidentally found brain lesions with benign imaging characteristics at presentation may be managed conservatively. Surveillance is an important part of the initial management of incidental lesions in the pediatric population, but careful scrutiny must be paid to the potential for higher grade lesions or malignant transformation.

Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature.

OBJECTIVE: Hydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively. METHODS: A retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review. RESULTS: Over the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population. CONCLUSIONS: De novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.

Growth and alignment of the pediatric subaxial cervical spine following rigid instrumentation and fusion: a multicenter study of the Pediatric Craniocervical Society.

OBJECTIVE The long-term effects of surgical fusion on the growing subaxial cervical spine are largely unknown. Recent cross-sectional studies have demonstrated that there is continued growth of the cervical spine through the teenage years. The purpose of this multicenter study was to determine the effects of rigid instrumentation and fusion on the growing subaxial cervical spine by investigating vertical growth, cervical alignment, cervical curvature, and adjacent-segment instability over time. METHODS A total of 15 centers participated in this multi-institutional retrospective study. Cases involving children less than 16 years of age who underwent rigid instrumentation and fusion of the subaxial cervical spine (C-2 and T-1 inclusive) with at least 1 year of clinical and radiographic follow-up were investigated. Charts were reviewed for clinical data. Postoperative and most recent radiographs, CT, and MR images were used to measure vertical growth and assess alignment and stability. RESULTS Eighty-one patients were included in the study, with a mean follow-up of 33 months. Ninety-five percent of patients had complete clinical resolution or significant improvement in symptoms. Postoperative cervical kyphosis was seen in only 4 patients (5%), and none developed a swan-neck deformity, unintended adjacent-level fusion, or instability. Of patients with at least 2 years of follow-up, 62% demonstrated growth across the fusion construct. On average, vertical growth was 79% (4-level constructs), 83% (3-level constructs), or 100% (2-level constructs) of expected growth. When comparing the group with continued vertical growth to the one without growth, there were no statistically significant differences in terms of age, sex, underlying etiology, surgical approach, or number of levels fused. CONCLUSIONS Continued vertical growth of the subaxial spine occurs in nearly two-thirds of children after rigid instrumentation and fusion of the subaxial spine. Failure of continued vertical growth is not associated with the patient's age, sex, underlying etiology, number of levels fused, or surgical approach. Further studies are needed to understand this dichotomy and determine the long-term biomechanical effects of surgery on the growing pediatric cervical spine.

Early postnatal cranial vault reduction and fixation surgery for severe hydrocephalic macrocephaly.

OBJECTIVE Infants with severe hydrocephalus and extreme macrocephaly typically undergo CSF diversion early in life, which can result in significant cranial deformity due to CSF overdrainage. In this scenario, overlap of the cranial plates can precede the development of secondary synostosis and/or severe, permanent cranial deformity. As a result, extensive cranial vault remodeling is sometimes undertaken later in life, which is often challenging and has been associated with mortality and a high morbidity rate. The authors have previously described a technique for early postnatal cranial vault reduction and fixation (CVRF), in which the calvarial bones are stabilized using absorbable fixation plates in the neonatal period, in an attempt to facilitate patient positioning, simplify hydrocephalus management, and improve cosmesis. Here, the authors describe their institutional experience managing patients with extreme neonatal hydrocephalus with CSF diversion, with and without CVRF, over the past 12 years. METHODS The authors retrospectively reviewed the charts of infants with extreme hydrocephalus (head circumference > 49 cm) treated at their children's hospital with ventriculoperitoneal shunting, with or without CVRF, between 2005 and 2017. Data collected included age, sex, etiology of hydrocephalus, type of CVRF performed (anterior, posterior, or combined), follow-up duration, orbitofrontal circumference, craniometric measurements, intraoperative blood loss, operative duration, and postoperative complications. Developmental data were collected using the third edition of the Ages and Stages Questionnaire. Photographic imaging was used to demonstrate esthetic outcomes, and family questionnaires were used to evaluate satisfaction with the esthetic outcome. RESULTS Eleven patients with extreme neonatal hydrocephalus underwent CSF shunting; 5 underwent shunting alone and 6 patients underwent shunting and CVRF. For patients who underwent shunting and CVRF, the median age at CVRF was 6 days and the median interval between shunt placement and CVRF was 2.5 days. The mean extent of calvarial vault volume reduction was 44.5% (± 3.9%). The mean duration of the CVRF procedure was 108 minutes, and 5 of 6 patients required intraoperative transfusion. Of the 5 patients who underwent shunting alone, 3 developed severe cranial deformities. Of 6 patients who underwent shunting and CVRF, 1 had a poor cosmetic outcome. In the shunting-alone group, 2 patients died and 1 required extensive cranial vault correction at 10 years of age. One patient in the shunting and CVRF group also died. CONCLUSIONS CVRF in combination with CSF shunting in the neonatal period can simplify the treatment of the rare case of severe hydrocephalic macrocephaly and leads to cosmetic outcomes that are considered good by their families.

Lumbar to sacral root rerouting to restore bladder function in a feline spinal cord injury model: Urodynamic and retrograde nerve tracing results from a pilot study.

AIMS: Lumbar to sacral rerouting surgery can potentially allow voiding via a skin-central nervous system-bladder reflex pathway. Here, we assessed if this surgery was effective in treating neurogenic bladder dysfunction/sphincter in felines. METHODS: Eight cats underwent spinal cord transection (SCT) at thoracic level 10/11. Unilateral L7 to S1 ventral root anastomosis was performed 1 month later in six cats. Two cats served as transection-only controls. Electrical and manual stimulation of L6-S1 dermatomes, and urodynamics were performed at 3, 5, 7, and 9/10 months post transection. At 9/10 months, cats were also evaluated by direct electrophysiological testing of anastomosed roots with urodynamics, then tissue collection and examination of the root anastomosis site and lumbosacral cord ventral horns for cells retrogradely labeled from tracer dye injected 2 weeks earlier into the bladder wall. RESULTS: At 9/10 months, four of six rerouted cats exhibited increased detrusor pressure provoked by cutaneous stimulation, one cat bilaterally. Two cats presented with a voiding stream after ipsilateral cutaneous stimulation at 7 and 9 months. All six rerouted animals showed regrowth of axons from the L7 ventral horn to the bladder, although some aberrant axonal regrowth was also observed. CONCLUSION: L7 to S1 ventral root rerouting below the level of SCT showed successful axonal regrowth to the bladder from the L7 spinal cord segment in all rerouted animals, and induced increased detrusor pressure response to cutaneous stimulation in a subset. This feasibility study paves the way for future animal studies for bladder reinnervation.

Evaluation and management of tethered cord syndrome in occult spinal dysraphism: Recommendations from the international children's continence society.

AIMS: As awareness and frequency of tethered spinal cord (TSC) related to occult spinal dysraphism (OSD) has increased with magnetic resonance imaging (MRI), variability exists in its evaluation and management. Due to no published level I data, we summarize the current International Children's Continence Society (ICCS) recommendations for diagnosis and treatment of OSD. METHODS: Guidelines were formulated based on analysis of pertinent literature and consensus among authors. This document was vetted by the multidisciplinary members of the ICCS via its website before submission for peer review publication. RESULTS: The more frequent diagnosis of OSD is associated with increased operative intervention. Spinal cord untethering (SCU) has a highly variable risk profile, largely dependent on the specific form of OSD. Progressive neurological deterioration attributed to "tethered cord" may occur, with or without surgery, in selected forms of OSD whereas other cohorts do well. CONCLUSION: Infants with classic cutaneous markers of OSD, with progressive neurologic, skeletal, and/or urologic findings, present no diagnostic or therapeutic dilemma: they routinely undergo MRI and SCU. Conversely, in asymptomatic patients or those with fixed, minor abnormalities, the risk profile of these OSD cohorts should be carefully considered before SCU is performed. Irrespective of whether or not SCU is performed, patients at risk for progression should be followed carefully throughout childhood and adolescence by a multidisciplinary team.

Antibiotic Powder Reduces Surgical Site Infections in Children After Single-Incision Laparoscopic Appendectomy for Acute Appendicitis.

BACKGROUND: Single-incision laparoscopic appendectomy (SILA) has a higher rate of wound infection than the multiport technique. The purpose of this project was to determine whether the use of topical antibiotic powder reduces surgical site infections (SSIs) in pediatric patients who undergo SILA. METHODS: Patients aged 0-21 years who underwent SILA for acute appendicitis from April 2015 to November 2016 were included in this quality improvement initiative. Cefoxitin powder was placed in the umbilical wound before skin closure. Data were prospectively collected and outcome measures were compared with a historical cohort who underwent SILA before the implementation of antibiotic powder. RESULTS: There were 108 patients in the historical group (HIST) and 126 in the powder group (POWD). The groups were similar in age (HIST: 11.5 ± 3.6 versus POWD: 12.2 ± 3.7 years, P = .15) and body mass index percentile (HIST: 57.6 ± 30.7 versus POWD: 58.8 ± 27.8, P = .84). Operative time was longer in the powder group (HIST: 26.5 ± 7.5 versus POWD: 29.7 ± 8.9 minutes, P = .004). Length of stay (HIST: 0.2 ± 0.4 versus POWD: 0.1 ± 0.4 days, P = .06), 30-day return to emergency department (HIST: 7% versus POWD: 8%, P = 1.0), and hospital readmissions (HIST: 5% versus POWD: 2%, P = .8) were similar. There was a significantly lower rate of superficial SSIs in the powder group (HIST: 4.6% versus POWD: 0%, P = .02). CONCLUSIONS: In pediatric patients undergoing SILA for acute appendicitis, the use of cefoxitin powder in the umbilical wound is a simple intervention to reduce the incidence of superficial SSIs.

Pediatric cerebral venous sinus thrombosis or compression in the setting of skull fractures from blunt head trauma.

OBJECTIVE Pediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented. METHODS The medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included. RESULTS Of 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up. CONCLUSIONS In this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

A Modified Technique for Occipitocervical Fusion Using Compressed Iliac Crest Allograft Results in a High Rate of Fusion in the Pediatric Population.

BACKGROUND: In children, high rates of occipitocervical (OC) fusion have been demonstrated with the use of rigid instrumentation in combination with harvested autograft, with or without bone morphogenetic protein (BMP). Historically, the use of allograft materials demonstrated inferior OC fusion outcomes compared with autograft. However, autograft harvest harbors an increased risk of patient morbidity, and the use of BMP is costly and controversial in children. Thus, there remains a need for safer, less costly, yet efficacious techniques for OC fusion in the pediatric population. METHODS: We retrospectively reviewed the charts of patients younger than 21 years of age who underwent OC fusion with structural allograft placement at our institution from 2010 to 2015. Data collected included age, sex, follow-up duration, fusion outcomes, and postoperative complications. RESULTS: A total of 19 patients (8 female and 11 male) underwent OC fusion with our surgical technique. Mean age was 8.5 ± 4.3 years. Radiographic follow up data were available for 18 of 19 patients. One patient was lost to clinical follow up but had radiographic confirmation of fusion. Thus, 18 of 18 (100%) of patients with radiographic follow-up achieved successful arthrodesis as determined by computed tomography. Median duration to documented fusion was 4.5 months. Clinical follow-up was available for 17 of 19 patients and was on average 18.8 ± 13.5 months. One patient required reoperation for graft fracture 8 months after radiographic confirmation of successful fusion. There were no vertebral artery injuries or other postoperative complications. CONCLUSIONS: We demonstrate a modified technique for OC fusion in children with unique structural allograft shaping and affixation, leading to excellent fusion outcomes at follow up. This technique obviates the need for autograft harvest or BMP, and may decrease postoperative morbidity.

Acute Shunt Failure Due to Perforation of Ventriculoperitoneal Shunt Tubing during Percutaneous Gastrostomy.

The placement of a percutaneous endoscopic gastrostomy (PEG) in a patient with a pre-existing ventriculoperitoneal shunt is generally regarded as safe. A critical but often overlooked technical consideration is confirmation of the course of the distal shunt tube prior to PEG insertion. We present the case of a 4-month-old male infant with shunted hydrocephalus who experienced shunt malfunction due to perforation of the distal shunt tubing after PEG placement.

Analysis and interrater reliability of pB-C2 using MRI and CT: data from the Park-Reeves Syringomyelia Research Consortium on behalf of the Pediatric Craniocervical Society.

OBJECTIVE The distance to the ventral dura, perpendicular to the basion to C2 line (pB-C2), is commonly employed as a measure describing the anatomy of the craniovertebral junction. However, both the reliability among observers and the clinical utility of this measurement in the context of Chiari malformation Type I (CM-I) have been incompletely determined. METHODS Data were reviewed from the first 600 patients enrolled in the Park-Reeves Syringomyelia Research Consortium with CM-I and syringomyelia. Thirty-one cases were identified in which both CT and MRI studies were available for review. Three pediatric neurosurgeons independently determined pB-C2 values using common imaging sequences: MRI (T1-weighted and T2-weighted with and without the inclusion of retro-odontoid soft tissue) and CT. Values were compared and intraclass correlations were calculated among imaging modalities and observers. RESULTS Intraclass correlation of pB-C2 demonstrated strong agreement between observers (intraclass correlation coefficient [ICC] range 0.72-0.76). Measurement using T2-weighted MRI with the inclusion of retro-odontoid soft tissue showed no significant difference with measurement using T1-weighted MRI. Measurements using CT or T2-weighted MRI without retro-odontoid soft tissue differed by 1.6 mm (4.69 and 3.09 mm, respectively, p < 0.05) and were significantly shorter than those using the other 2 sequences. Conclusions pB-C2 can be measured reliably by multiple observers in the context of pediatric CM-I with syringomeyelia. Measurement using T2-weighted MRI excluding retro-odontoid soft tissue closely approximates the value obtained using CT, which may allow for the less frequent use of CT in this patient population. Measurement using T2-weighted MRI including retro-odontoid soft tissue or using T1-weighted MRI yields a more complete assessment of the extent of ventral brainstem compression, but its association with clinical outcomes requires further study.