Background: We often identify our vocation with a calling. Pediatric neurosurgeons are vulnerable to burnout and moral injury given the high emotional stakes, relentless pursuit of precision, and the enduring demands of patient care. Physician wellness is a prerequisite for safe, high-quality care, and professional longevity. Summary: This article highlights physician wellness as a critical issue in pediatric neurosurgery, explores the public health crisis of physician suicide and burnout, and outlines strategies to sustain performance and resilience through relationships, purpose, and self-compassion. Key Messages: In this narrative reflective article, we make recommendations based on our personal experience and on the belief that wellness is not optional – it is essential to fulfill our calling as healers.

Plain Language SummarySagittal craniosynostosis is a deformity of the skull that occurs in infants due to premature fusion of the sagittal suture – a fibrous joint between the two parietal bones in the midline that normally closes in adulthood. The sagittal suture along with certain other sutures of the skull, allows for symmetric expansion of the skull as the developing brain enlarges. Early closure of the sagittal suture results in babies having heads that are abnormally elongated from front to back and is often a cause of significant cosmetic concern. There are two surgical treatment options for this condition – a more extensive open operation that involves exposing almost the entire skull and making multiple cuts in the bone to reshape the skull (performed in older infants), and a less invasive, less morbid endoscope-assisted operation that involves only cutting out a strip of bone containing the fused sagittal suture (performed in younger infants). Traditional belief has been that the best results with the endoscopic surgery are achieved in infants 3–4 months of age. However, in the last decade, neurosurgeons have increasingly started performing the operation in infants up to 6 months of age and reported good outcomes, although robust data is lacking. In this study, we compared the surgical outcomes of endoscopic strip craniectomy between infants <5 months and ≥5 months of age, in a large series of infants with sagittal craniosynostosis. We found that in both groups of patients, the operation was safe and the shape of the skull on follow-up was comparable.

Introduction: Endoscopic third ventriculostomy (ETV) is a well-established neurosurgical procedure. Concurrently, the Endoscopic Third Ventriculostomy Success Score (ETVSS) is a recognized validation tool commonly used to prognosticate the efficacy of this intervention. The main aims of this study are to review our institutional experience with ETV for pediatric hydrocephalus and evaluate its correlation with the ETVSS. Secondary aims include identification of other factors that are not part of the existing ETVSS and to corroborate our findings with contemporary literature. Methods: This is a single-institution, retrospective study. Patients under 19 years old who underwent ETV were included. Variables of interest such as patient characteristics, hydrocephalus etiology, procedural details, perioperative complications, neuroimaging features, and outcomes were collected. Radiological parameters curated from the literature such as third ventricular floor bowing, lamina terminalis bowing, third ventricular morphology index, and presence of prepontine adhesions (PPAs) are also included. For this study, the primary outcome measure is “ETV success,” defined as no need for shunt insertion to divert CSF at any point in time after ETV. Subsequent outcome of each ETV is correlated with the ETVSS. Additional factors are also independently assessed for their impact on the ETVSS in our study cohort. Results: Sixty-nine ETV cases were recruited for this study whereby ETV was successful in 63.8% (n = 44) cases. At 12 months’ follow-up, their ETV stomas remained patent. Of note, 24.6% (n = 17) ETV failures occurred within 30 days of the procedure. In our series, ETV success correlated well with ETVSS. The ETV success rate was 0% for post-infectious and post-hemorrhagic etiologies. For the cases of ETV failure, definitive CSF diversion procedures were necessary within 3 months from their initial ETV. Logistic regression analysis showed ETVSS (odds ratio 1.068, p = 0.037) and the presence of PPA (p = 0.02) significantly correlated with ETV success. Conclusion: Our institutional experience in the use of ETV for pediatric hydrocephalus corroborates with findings from contemporary literature. In the context of our study, the ETVSS is applicable and the absence of PPA on neuroimaging demonstrates good correlation with ETV success.

Introduction: Intrathecal baclofen therapy and baclofen pumps can lead to complications, including recurrent cycles of alternating hypertonia and hypotonia. The causes and optimal treatments for this issue remain unclear. This study reviews presentations, radiologic and surgical findings, treatments, and outcomes in cases of cyclic hypertonia/hypotonia. Methods: We reviewed patients with baclofen pumps treated at our hospital from 1998 to 2024 who developed cyclic hypertonia/hypotonia. Data collected included patient sex, etiology and type of tone abnormality, age at pump placement, age and weight at symptom onset, infusion rate and type at onset, side port access and catheter dye study results, treatments, surgical findings, and outcomes. Results: We identified 15 cases in 14 patients (10 females, 71%; 4 males, 29%). Among 248 patients with baclofen pumps, 38% were female. Females were significantly more likely to develop cyclic hypertonia/hypotonia (p < 0.01). The mean age at pump implantation was 10.2 years, and the mean time from last pump surgery to symptom onset was 645 days. All patients received enteric baclofen initially; 14 also had pump rate adjustments. Three patients improved without surgery, and one died during a hypotonia episode. Side port access showed patent catheters in 7 of 8 cases, with 6 of these showing good intrathecal dye dispersion. Surgery was performed in 11 cases, including two surgeries in 6 cases. Nine patients had pump replacements; 8 also had catheter revisions. Surgical findings included catheter kinks in 4 cases (36% of surgical cases) and leaks in 5 cases (45%), with 4 leaks at the pump-connector catheter connection. Six of 11 partial revisions (55%) and all 5 complete catheter replacements (100%) resulted in symptom resolution without recurrence. Replacing the pump-connector segment resolved symptoms without recurrence in 4 of 5 patients with leaks at this site. Overall, all patients undergoing surgery experienced symptom improvement without recurrence after one or two revisions. Conclusion: Adjusting pump rates and adding enteric baclofen may help some patients with cyclic hypertonia/hypotonia. If a leak at the pump-connector catheter segment is identified, segment replacement should be considered. When partial revisions fail, complete catheter replacement appears more effective, especially as a second surgery. Cyclic hypertonia/hypotonia is a treatable complication of baclofen pumps.

The editors and Karger Publishers would like to thank the following reviewers for the ongoing support in reviewing manuscripts for Pediatric Neurosurgery:Gregory W. Albert, Little Rock, AR, USARichard C.E. Anderson, Ridgewood, NY, USASuchanda Bhattacharjee, Hyderabad, IndiaDavid Bonda, Seattle, WA, USAFrederick Boop, Memphis, TN, USABruno Braga, Dallas, TX, USAUttara Chatterjee, Kolkata, IndiaIan Coulter, Newcastle upon Tyne, UKAnnie I. Drapeau, Winnipeg, MB, CanadaHassan I. El Shafei, Cairo, EgyptMaurizio Elia, Troina, ItalyRamin Eskandari, Charleston, SC, USAJustin Fraser, Lexington, KY, USAPasquale Gallo, Birmingham, UKCatherine Garcia, Los Angeles, CA, USAFlavio Giordano, Florence, ItalyLance S. Governale, Gainesville, FL, USABenjamin Hall, Liverpool, UKAndrew M. Hersh, Baltimore, MD, USAJames Johnston, Birmingham, AL, USAGokmen Kahilogullari, Ankara, TurkeyJohn Kestle, Salt Lake City, UT, USAJeffrey Leonard, Columbus, OH, USAFrancesco Mangano, Cincinnati, OH, USAJosé Hinojosa Mena-Bernal, Barcelona, SpainPeter Morgenstern, New York, NY, USAOliver Mueller, Dortmund, GermanyDattatraya Muzumdar Parel, Mumbai, IndiaFarideh Nejat, Tehran, IranMark Proctor, Boston, MA, USAJarod L. Roland, St. Louis, MO, USAKazuaki Shimoji, Chiba, JapanMarc Sindou, Lyon, FranceJehuda Soleman, Basel, SwitzerlandPeter Spazzapan, Ljubljana, SloveniaDominic Thompson, London, UKTadanori Tomita, Chicago, IL, USAAlbert Tu, Ottawa, ON, CanadaMichael Vassilyadi, Ottawa, ON, Canada

Introduction: At our institution, revascularization after indirect moyamoya surgery is routinely evaluated using magnetic resonance angiography (MRA) rather than catheter angiography. In this paper, we review how revascularization can be visualized on axial MRA and compare its visualization on MRA to that on catheter angiography. We also compare clinical outcomes of patients followed with routine postoperative MRA with outcomes of patients followed with routine catheter angiography. Methods: We retrospectively reviewed the records of all patients treated at our institution who underwent unilateral encephaloduroarteriosynangiosis (EDAS) and/or pial synangiosis between the ages of 1 and 21 years and between December 31, 2003, and May 1, 2021. We included patients who underwent EDAS/pial synangiosis at other hospitals as long as they met all inclusion criteria. Inclusion criteria included having a preoperative MRA within 18 months of surgery and a postoperative MRA 3−30 months after surgery. Clinical outcomes included development of postoperative stroke and transient ischemic attacks (TIAs) and changes in symptoms (improved, unchanged, or worsened), including seizures, balance issues, and headaches. Clinical outcomes were compared between patients who had routine postoperative MRA only versus those who had routine postoperative angiograms, with or without routine MRA. For each surgery, we determined the ratios of the diameters and areas of the donor vessel and the contralateral corresponding vessel, as well as the relative signal intensities of these two vessels, on preoperative and 3- to 30-month postoperative MRA. We did the same for the middle meningeal artery (MMA) ipsilateral to the donor artery and the contralateral MMA. We assessed changes from pre- to post-operation in diameter ratios, area ratios, relative signal intensity, ivy sign, and in brain perfusion on arterial spin labeled (ASL) imaging. MRI and MRA measures of revascularization and flow were compared to Matsushima grades in patients who had postoperative catheter angiograms. Results: Fifty-one operations for 42 unique patients were included. There were no significant differences in the rates of postoperative strokes, postoperative TIAs, changes in symptoms, or new symptoms after surgeries evaluated by routine postoperative MRA versus catheter angiogram (p = 0.282, 1, 0.664, and 0.727, respectively). There were significant associations between greater collateralization on postoperative MRA and greater median increases in preoperative-to-postoperative ratios of donor-vessel-over-contralateral-vessel diameter (p = 0.0461), ipsilateral-MMA-over-contralateral-MMA diameter (p = 0.0135), and the summed donor and ipsilateral MMA diameters over the summed contralateral vessel diameters (p < 0.001). The median increase in the ratio of the donor vessel and contralateral corresponding vessel diameters was significantly higher for Matsushima grade A versus B (p = 0.036). The median increase in the ratio of the sum of donor and ipsilateral MMA diameters over the sum of the contralateral vessel diameters was significantly higher for improved-versus-unchanged perfusion on ASL imaging (p = 0.0074). There was a nonsignificant association between greater postoperative collateralization on MRA and Matsushima grade (p = 0.1160). Conclusion: Cerebral revascularization after EDAS and pial synangiosis can be evaluated on axial MRA by comparing the diameter and/or signal intensity of the donor vessel and corresponding contralateral vessel, as well as the ipsilateral and contralateral MMA, on postoperative-versus-preoperative MRA. The use of routine postoperative MRA rather than catheter angiography does not appear to negatively affect outcomes.

Introduction: The association between trigonocephaly and Sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature. However, the real incidence of this association and its clinical relevance remain unknown. Methods: The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario “Agostino Gemelli” IRCCS from January 2014 to June 2023. Results: During the study period, 136 patients with trigonocephaly underwent surgery. Analysis of the clinical charts revealed that in 39.7% of the cases (54/136), preoperative CT scan depicted the presence of a Sylvian fissure AC. Of these, AC was bilateral in 23 cases and unilateral in the remaining 31. All unilateral ACs were on the left side. The ACs were classified as Galassi grade I in 52 cases (96.3%) and Galassi grade II in 2 cases (3.7%). Interestingly, in 1 case we reported a Galassi grade I AC enlargement during follow-up, thereby necessitating surgical fenestration. Conclusion: ACs and trigonocephaly are well-known conditions for pediatric neurosurgeons; however, their association is poorly defined. Despite the lack of reports on the incidence and clinical significance of this association, it is worth knowing that radiological follow-up is essential in monitoring AC evolution.

Background: Paediatric low-grade gliomas (pLGGs) are the most common primary brain tumour in children. Though considered benign, slow-growing lesions with excellent overall survival, their long-term morbidity can be significant, both from the tumour and secondary to treatment. Vast progress has been made in recent years to better understand the molecular biology underlying pLGGs, with promising implications for new targeted therapeutic strategies. Summary: A multi-layered classification system of biologic subgroups, integrating distinct molecular and histological features has evolved to further our clinical understanding of these heterogeneous tumours. Though surgery and chemotherapy are the mainstays of treatment for pLGGs, many tumours are not amenable to surgery and/or progress after conventional chemotherapy. Therapies targeting common genetic aberrations in the RAS-mitogen-activated protein kinase (RAS/MAPK) pathway have been the focus of many recent studies and offer new therapeutic possibilities. Here, we summarise the updated molecular classification of pLGGs and provide a review of current treatment strategies, novel agents, and open trials. Key Messages: (1) There is a need for treatment strategies in pLGG that provide lasting tumour control and better quality of survival through minimising toxicity and protecting against neurological, cognitive, and endocrine deficits. (2) The latest World Health Organisation classification of pLGG incorporates a growing wealth of molecular genetic information by grouping tumours into more biologically and molecularly defined entities that may enable better risk stratification of patients, and consideration for targeted therapies in the future. (3) Novel agents and molecular-targeted therapies offer new therapeutic possibilities in pLGG and have been the subject of many recent and currently open clinical studies. (4) Adequate molecular characterisation of pLGG is therefore imperative in today’s clinical trials, and treatment responses should not only be evaluated radiologically but also using neurological, visual, and quality of life outcomes to truly understand treatment benefits.