Posterior Cranial Distraction in Craniosynostosis: A Systematic Review of the Literature.

OBJECTIVE: Posterior cranial distraction (PCD) is a surgical technique to address craniosynostosis, especially in syndromic patients. The technique has the ability to significantly expand the cranium, while requiring minimal dural dissection, compared to cranial remodeling. Our goals were to determine the patient characteristics and surgical outcomes of PCD. The two questions that we sought to answer were: 1) What is the average published complication rate and the most common complications of PCD? and 2) How much intracranial volume expansion can one expect with PCD? DESIGN: A PubMed database search of articles on PCD was performed. Case reports and articles with overlapping patients were excluded. A systematic review was performed using the remaining articles. MAIN OUTCOME MEASURES: Patient data were extracted in order to determine the total number of patients, patients with a syndrome, types of syndromes, mean age at surgery, mean distraction distance, mean increase in intracranial volume, and complications. RESULTS: 18 articles representing 325 patients were analyzed. A syndrome was present in 68.6% of patients. The mean age at time of surgery was 22.1 months. Mean distraction amount was 24.7 mm. Mean increase in intracranial volume was 253.2 cm3. The overall complication rate was 32.2%, with the most common complications being surgical-site infection, hardware-related complications and delayed wound healing. CONCLUSIONS: PCD is a powerful technique in the management of syndromic craniosynostosis, although complication rates are significantly higher than traditional remodeling techniques. Future studies should compare the effects of supratorcular and infratorcular osteotomies on intracranial volume, cosmesis and complications.

Current state and future direction of quality improvement in pediatric neurosurgery: a survey of pediatric neurosurgeons.

OBJECTIVE: Quality improvement (QI) is a methodology used to implement sustainable, meaningful change to improve patient outcomes. Given the complex pathologies observed in pediatric neurosurgery, QI projects could potentially improve patient care. Overall, there is a need to characterize the degree of QI opportunities, training, and initiatives within the field of pediatric neurosurgery. Herein the authors aimed to define the current QI landscape in pediatric neurosurgery. METHODS: A cross-sectional survey was sent to all members of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery via email. The responses were anonymized. Questions addressed several relatable QI topics including 1) training and participation in QI; 2) QI infrastructure; 3) QI program incentives; and 4) general opinions on the National Surgical Quality Improvement Program (NSQIP) database, various QI topics, and QI productivity. RESULTS: Responses were received from 129 participants (20% response rate). Most respondents practiced in an academic setting (59.8%) and at a free-standing pediatric hospital (65.4%). Participation in QI projects was high (81.7%), but only 23.8% of respondents had formal QI training. Only 36.5% of respondents had institutional requirements for QI work; the majority of those were only required to participate as a project team member. Nearly half of the respondents did not receive incentives or institutional support for QI. The majority agreed ("strongly" and "somewhat") that a QI course would be beneficial (75.5%), that QI projects should be considered for publication in neurosurgery journals (88.1%), and that there is a need for national quality metrics (81.4%). Over 88% have an interest in seeing QI project presentations at the annual Pediatric Joint Section meeting. Only 26.3% believed that the NSQIP was a useful QI guide. Respondents suggested further study of the following QI topics: overall rates of infection and their prevention, hydrocephalus, standardized treatment algorithms for common disorders, team communication, pediatric neurosurgery-specific database, access to care, and interprofessional education. CONCLUSIONS: Areas of opportunity include specialty-specific QI education, tactics for obtaining support to build the QI infrastructure, increased visibility of QI work within pediatric neurosurgery, and a review of available registries to provide readily available data relevant to this specialty.

Hospital Case-Volume and Patient Outcomes Following Pediatric Brain Tumor Surgery in the Pediatric Health Information System.

BACKGROUND: Markers of quality of care in various surgical specialties have been shown to correlate with hospital volumes. This study investigates the effect of hospital volume and patient-related factors on the outcomes of children undergoing brain tumor resection. METHODS: We examined the data within the Pediatric Health Information System (PHIS) for children aged zero to 17 years undergoing brain tumor resection between 2016 and 2020. Length of hospital stay (LOS), costs, and reoperation rates were analyzed for associations with hospital case-volume, patient factors, and other hospital-related factors. RESULTS: A total of 2568 patients were included in this PHIS analysis. After adjusting for covariates, care provided by high-case-volume hospitals led to shorter LOS (P = 0.01). The effect of hospital case-volume on median cost was present on univariate analysis (US $63,845 at low-volume hospital versus US $54,909 at high-volume hospital, P = 0.002); this finding was attenuated by LOS. A trend was observed between reoperation rates and hospital case-volume, with lowest quartile volume hospitals having higher odds of reoperation than hospitals with volumes in the highest quartile (P = 0.06). Racial and ethnic minorities, medical comorbidities, and other sociodemographic factors were associated with poorer outcomes following surgery. CONCLUSIONS: Centering care around high-case-volume hospitals can potentially lead to shorter hospital stays and decreased costs for children with brain tumors. This PHIS article highlights the association of the studied outcomes with certain sociodemographic factors and illustrates that inequalities in pediatric health care still exist. Further efforts are required to understand and eliminate these potentially harmful differences.

Comparison of Perioperative Outcomes and Parental Satisfaction Outcomes of Strip Craniectomy with Postoperative Helmet versus Spring-Mediated Remodeling in Sagittal Craniosynostosis.

INTRODUCTION: We sought to compare outcomes and parental satisfaction between 2 approaches for sagittal craniosynostosis: strip craniectomy with spring-mediated skull remodeling (SMSR) and strip craniectomy with postoperative helmet (SCH). METHODS: Perioperative and outcome data for SMSR or SCH patients between September 2010 and July 2019 were retrospectively reviewed. A telephone survey was administered to parents of children who underwent both procedures. RESULTS: A total of 62 children were treated for sagittal craniosynostosis by either SMSR (n = 45) or SCH (n = 17). The SCH group had a lower estimated blood loss (27 vs. 47.06 mL, p = 0.021) and age at surgery (13.0 vs. 19.8 weeks) than the SMSR group. Three patients underwent early springs removal due to trauma or dislodgement, all of whom converted to helmeting. Of the 62 children initially identified, 59 were determined to have an adequate follow-up time to assess long-term outcomes. The mean follow-up time was 30.1 months (n = 16) in the SCH group and 32.0 months in the SMSR group (n = 43, p = 0.39). Two patients in the SCH group and one in the SMSR group converted to open cranial vault reconstruction. Thirty parents agreed to respond to the satisfaction survey (8 SCH, 22 SMSR) based on a Likert scale of responses (0 being most dissatisfied possible, 4 most satisfied possible). Average satisfaction was 3.86/4.0 in the SCH group and 3.45/4.0 in the SMSR group. No parents in the SCH group would change to SMSR, while 3 of the 22 SMSR survey responders would have changed to SCH. CONCLUSIONS: Perioperative outcomes and average parental satisfaction were similar in both groups. Importance of helmet wear compliance and risk of spring dislodgement should be discussed with parents.

Case Series of Rapid Surgical Interventions in Fulminant Intracranial Hypertension.

AIMS: Examine ophthalmologic outcomes and complications of lumbar drain and cerebrospinal fluid shunts in pediatric fulminant intracranial hypertension. METHODS: Patients under 21 years of age with a diagnosis of fulminant intracranial hypertension with temporary lumbar drain only, shunt after lumbar drain, and shunt only were included. Parameters investigated include lumbar drain data, medication freedom, time to resolution of papilledema, improvement in cranial nerve palsy, afferent pupillary defects, visual fields, visual acuity, and complications of each intervention. RESULTS: Four patients had temporary lumbar drain, 2 temporary lumbar drain and cerebrospinal fluid shunt, and 3 shunt only. All achieved medication freedom and resolution of papilledema and cranial nerve palsies (if present). Most had resolution of preprocedure afferent pupillary defects. Minor residual visual field deficits occurred in 67%, and all had visual acuity improvement. One patient's lumbar drain dislodged, and one patient had 2 cerebrospinal fluid shunt revisions. CONCLUSION: Temporary lumbar drain with medical therapy may be a viable first approach to fulminant intracranial hypertension.

Molecular classification of a complex structural rearrangement of the RB1 locus in an infant with sporadic, isolated, intracranial, sellar region retinoblastoma.

Retinoblastoma is a childhood cancer of the retina involving germline or somatic alterations of the RB Transcriptional Corepressor 1 gene, RB1. Rare cases of sellar-suprasellar region retinoblastoma without evidence of ocular or pineal tumors have been described. A nine-month-old male presented with a sellar-suprasellar region mass. Histopathology showed an embryonal tumor with focal Flexner-Wintersteiner-like rosettes and loss of retinoblastoma protein (RB1) expression by immunohistochemistry. DNA array-based methylation profiling confidently classified the tumor as pineoblastoma group A/intracranial retinoblastoma. The patient was subsequently enrolled on an institutional translational cancer research protocol and underwent comprehensive molecular profiling, including paired tumor/normal exome and genome sequencing and RNA-sequencing of the tumor. Additionally, Pacific Biosciences (PacBio) Single Molecule Real Time (SMRT) sequencing was performed from comparator normal and disease-involved tissue to resolve complex structural variations. RNA-sequencing revealed multiple fusions clustered within 13q14.1-q21.3, including a novel in-frame fusion of RB1-SIAH3 predicted to prematurely truncate the RB1 protein. SMRT sequencing revealed a complex structural rearrangement spanning 13q14.11-q31.3, including two somatic structural variants within intron 17 of RB1. These events corresponded to the RB1-SIAH3 fusion and a novel RB1 rearrangement expected to correlate with the complete absence of RB1 protein expression. Comprehensive molecular analysis, including DNA array-based methylation profiling and sequencing-based methodologies, were critical for classification and understanding the complex mechanism of RB1 inactivation in this diagnostically challenging tumor.

A Case Series of the Use of Piezosurgery Instrument for Minimally Invasive Metopic Suturectomy.

BACKGROUND: Minimal invasive suturectomy is one of the many surgical approaches to treat isolated single suture craniosynostosis. This approach can be technically challenging in metopic craniosynostosis given the narrow corridor and steep angle of the forehead. New instruments such as the Piezosurgery device (Mectron) have the potential to improve the ability to safely perform minimal invasive surgery in metopic craniosynostosis. OBJECTIVE: To demonstrate the safety and efficacy of Piezosurgery technology in minimal invasive suturectomy for nonsyndromic metopic suture craniosynostosis and to describe our technique. METHODS: A retrospective chart review was performed of all the single metopic suturectomies performed at our single institution from March 2018 to November 2019. Pre-, intra-, and postoperative data were collected to assess the safety of Piezosurgery. RESULTS: The cohort consisted of 12 patients with an average of 95.25 d old and an average weight of 6.2 kg. A total of 91.7% were male, and 91.7% were Caucasian. There were no intraoperative or postoperative Piezosurgery device-related complications in the entire cohort. CONCLUSION: The use of the Piezosurgery instrument was safe in this cohort of minimal invasive metopic suturectomy. This device has greatly increased the ease of this procedure in our hands.

Management of hydrocephalus in infants with severe hemophilia A: report of 2 cases.

The authors report on the clinical course of two infants with severe hemophilia A (HA) and concomitant progressive hydrocephalus that required management with a ventriculoperitoneal shunt. The first child, with known HA, presented with a spontaneous intracranial hemorrhage and acquired hydrocephalus. He underwent cerebrospinal fluid diversion with a temporary external ventricular drain, followed by placement of a ventriculoperitoneal shunt. The second child had hydrocephalus secondary to a Dandy-Walker malformation and was diagnosed with severe HA during preoperative evaluation. He underwent placement of a ventriculoperitoneal shunt after progression of the hydrocephalus. The authors also review the treatment of hydrocephalus in patients with HA and describe the perioperative protocols used in their two cases. Treatment of hydrocephalus in infants with HA requires unique perioperative management to avoid complications.