Bristol, Ruth E.

Malignant transformation of intracranial nongerminomatous germ cell tumors (NGGCTs) is a rare but clinically relevant phenomenon. We present the case of a 13-year-old boy with a localized, pineal NGGCT. After an initial favorable response, tumor growth was noted while still on chemotherapy. Histopathologic characterization revealed that 40% of the tumor was embryonal rhabdomyosarcoma (RMS), consistent with potential malignant transformation. Due to the rare nature of NGGCT malignant transformation, the best clinical approach to these cases remains unclear. We explore existing cases, treatments, and outcomes of malignant transformation in intracranial NGGCTs to help inform future clinical decision-making and the establishment of treatment guidelines.

OBJECTIVE Neurosurgeon scientists play a unique role in advancing neuroscience research. While previous publications have explored trends in federal and foundation funding among neurosurgeons, funding is often dominated by neurosurgical oncologists and functional neurosurgeons. Less is known about the research efforts of pediatric neurosurgeons. The aim of this study was to survey the members of the American Society of Pediatric Neurosurgeons (ASPN) to provide an overview of past research experience, current involvement, funding, and research priorities among pediatric neurosurgeons, and to gather insights that could shape future efforts to advance pediatric neurosurgical research. METHODS A survey was developed using the REDCap platform and distributed to all ASPN members via email. Survey questions used branching logic and were organized into 5 sections: 1) demographics, 2) research experience during training, 3) research experience as an attending physician, 4) research priorities, and 5) multicenter consortiums. RESULTS One hundred thirty-nine respondents completed more than half of the survey, for an overall response rate of 52.1%. Most respondents (96.4%) participated in research during their training, but only 38.1% had received a grant during training. In contrast, 83.9% of respondents were actively engaged in research as an attending physician, and 48.7% reported active funding (60.7% federal, 41.8% from foundations, and 42.9% internal). Furthermore, 74.8% of respondents reported being a member of a multicenter research consortium, and 82.4% agreed that multicenter research is important. Seventy percent of respondents agreed that the ASPN should facilitate multicenter consortium-based pediatric neurosurgical research, offering free-text responses with the following suggestions: 1) set aside time at the annual meeting to discuss multicenter research (22.9%); 2) encourage collaboration and facilitate networking (42.9%); 3) provide centralized core services such as a data coordinator and biostatistician (12.9%); and 4) provide training, education, and mentoring (7.1%). CONCLUSIONS The survey provided a cross-sectional analysis of the pediatric neurosurgical research landscape, highlighting the current state of research experience, funding, and the perspectives of pediatric neurosurgeons regarding research priorities. Despite the challenges, there is clear recognition of the importance of multicenter research collaboration. These findings reinforce the ongoing necessity of organized initiatives to support pediatric neurosurgical research and offer actionable insights into how organized pediatric neurosurgery can contribute to this critical endeavor.

Craniofacial surgery requires a multidisciplinary care approach in the perioperative period. In an effort to guide clinical decision making and standardize care, many institutions have implemented craniofacial surgical pathways, describing numerous variables to monitor and specific actions that must be completed by each involved discipline in the perioperative period. Adherence to these pathways can be challenging given the complexity of the protocol and the multiple disciplines involved. In an effort to improve adherence to our institution’s perioperative pathway, the authors implemented a quarterly, multidisciplinary craniofacial operative team meeting to review the pathway and improve the quality of care. The authors report results from a retrospective review of craniosynostosis suturectomy operations at a single site in the 2 years before and 2 years after the start of our team meeting to review perioperative care. A total of 141 operations were included (70 before the meeting and 71 after). Perioperative outcomes and adherence to our surgical pathway were compared. Following the start of our team meetings, there was a significant decrease in the time under anesthesia, patients requiring blood transfusion, and total hospital length of stay ( P <0.05). There was also improved adherence to the craniofacial pathway, with significantly more operations applying peripheral nerve blocks to all locations specified in our pathway with the proper drug contents ( P <0.05). Our findings suggest that craniofacial operative team meetings with a goal of reviewing the surgical pathway and discussing improvements in care plans with all involved providers can significantly improve adherence to operative pathways and surgical outcomes.

Abstract BACKGROUND Non-germinomatous germ cell tumors (NGGCT) constitute 35%- 45% of intracranial germ cell tumors. Growing teratoma syndrome has been observed in patients with NGGCTs after starting chemotherapy. While growing teratomas are typically benign, this process can rarely involve malignant transformation and result in poor prognosis. We present the case of a patient with pineal NGGCT and growing teratoma syndrome with malignant transformation to embryonal rhabdomyosarcoma. CASE REPORT A 13-year-old male presented with headaches and vision changes. An MRI brain demonstrated a 26 x 25 x 19 mm (AP X TR X CC) mixed solid and cystic pineal mass with no evidence of metastatic disease. Due to elevated alpha fetoprotein levels in the serum and cerebrospinal fluid, he was diagnosed with intracranial NGGCT. Treatment was initiated with alternating cycles of Carboplatin/Etoposide and Ifosfamide/Etoposide. After 2 cycles of chemotherapy, a complete biochemical response was noted but MRI showed increased tumor size (presumed growing teratoma syndrome). The patient underwent complete tumor resection. Histology showed embryonal rhabdomyosarcoma as a somatic malignancy arising in a mature teratoma. Further molecular characterization revealed somatic NF1 and KRAS pathogenic mutations that are consistent with the primary diagnosis. Methylation profiling, however, did not exhibit a match with any known malignancies. Continued complete response was noted following two additional chemotherapy cycles. The patient is planned for six total chemotherapy cycles followed by high-dose craniospinal radiation with boost to the primary tumor. CONCLUSIONS Malignant transformation is a rare phenomenon in NGGCT. There is no standard of care for these patients. Further studies are needed to define ideal therapy for this patient population.

Purpose: The purpose of this article was to appraise the various methods of reconstruction for meningomyelocele (MMC) defects. Methods: A systematic review of the literature was performed to evaluate all reconstructions for MMC. The method of reconstruction was categorized by: primary closure with and without fascial flaps, random pattern flaps, VY advancement flaps (VY), perforator flaps, and myocutaneous flaps. Perforator flaps were subsequently subcategorized based on the type of flap. Results: Upon systematic review, 567 articles were screened with 104 articles assessed for eligibility. Twenty-nine articles were further reviewed and included for qualitative synthesis. Two hundred seventy patients underwent MMC repair. The lowest rates of major wound complications (MWC) were associated with myocutaneous and random pattern flaps. A majority of MWC was in the lumbrosacral/sacral region (87.5% of MWC). In this region, random patterns and perforator flaps demonstrated the lowest rate of MWC (4.5, 8.1%). Conclusions: Plastic surgery consultation should be strongly considered for MMC with defects in the lumbosacral/sacral region. Perforator flaps are excellent options for the reconstruction of these defects.

Abstract Consensus does not exist regarding the best dosage regimen for using tranexamic acid (TXA) for patients undergoing open calvarial vault remodeling in craniosynostosis surgery. The purpose of this study was to evaluate 2 dosing protocols, as well as the cost of using TXA. Previously, the institutional protocol was to give patients undergoing open calvarial vault remodeling a loading infusion of TXA (10 mg/kg) at the start of their procedure, after which intravenous TXA (5 mg/kg/h) was given throughout surgery and for 24 hours postoperatively. In July 2015, the protocol changed to a reduced postoperative infusion time of 4 hours. A retrospective review was conducted of records of 30 patients who had surgery before the protocol change (24-hour group) and 23 patients whose surgery occurred after the protocol change (4-hour group). The following data were collected: blood volume transfused, hemoglobin levels, estimated blood loss, and intensive care days; and costs of TXA and blood transfusion. Results showed a 4-hour infusion was as effective as a 24-hour infusion for reducing blood loss in patients undergoing craniosynostosis. Transfusion requirements, hemoglobin and hematocrit levels, and estimated blood loss were not significantly different for the groups. The cost of TXA and transfusion in the 4-hour group was significantly less (P < 0.001) than in the 24-hour group. No significant difference in cost existed for patients who received blood transfusion alone versus patients who received the 4-hour TXA infusion.

Purpose: Cranial defects in children have been repaired with various materials ranging from autologous bone to synthetic materials. There is little published literature on the outcomes of titanium mesh cranioplasty (TMC) in calvarial reconstruction in the pediatric population. This study evaluates a pediatric cohort who underwent calvarial defect reconstruction with titanium mesh and assesses the efficacy and outcomes of TMC. Methods: An Institutional Review Board approved retrospective review of patients ≤18 years of age who underwent cranioplasty from 1999 to 2014 at 2 centers was performed. The cohort undergoing TMC was studied. Results: A total of 159 cranioplasties were performed. Autologous reconstruction included 84 bone flap replacements and 36 split calvarial bone graft reconstructions. Six patients underwent PEEK implant reconstruction. Titanium mesh cranioplasty was performed on 33 patients. Two patients underwent 2 separate cranioplasties. The median age of patients was 6 years (19 months to 18 years). The most common underlying etiologies were congenital syndromes/craniosynostosis (13 patients), and trauma (11). The majority of patients had prior cranial surgeries (85%). Various types of titanium mesh were used with sizes ranging from 2×3 cm to 19×20 cm, with some patients requiring distinct areas of defect reconstruction. Perioperative complications were noted in 2 patients that subsequently improved. Two patients had late soft tissue problems with complications of wound infections requiring resection of a portion of the mesh. Patients were followed an average of 4 years (range 13 days to 6.8 years), with 2 patients lost to follow-up. Overall, all patients with follow-up achieved a cranial contour with good symmetry to the unaffected side, as well as effective protection to the brain. Conclusions: Titanium mesh cranioplasty is an effective option for correcting pediatric cranial defects when autologous bone availability is limited and soft tissue coverage allows placement of an implant. The interim outcome for these patients is favorable with few complications and no evidence of growth restriction in the authors’ series. Follow-up will be ongoing for these patients.