PurposeWormian bones (WB) are accessory ossicles that develop within cranial sutures. While typically benign, their presence in large numbers has been associated with various genetic and developmental disorders. This study aims to characterize the prevalence, anatomical distribution, and clinical associations of WB in a pediatric population undergoing cranial CT imaging.MethodsA retrospective review was conducted at Monroe Carell Jr. Children’s Hospital at Vanderbilt. Pediatric patients aged 0 to 18 years who underwent cranial CT imaging for any clinical indication were included. WB were radiologically confirmed, and demographic data, cephalic index, and comorbidities were collected and analyzed.ResultsAmong the 13,519 patients who underwent cranial CT imaging, 77 (0.57%) had radiologically confirmed WB, totaling 476 ossicles. The prevalence increased to 2.1% when examining our clinic cohort over a 5-year period. The lambdoid suture was the most common site (343/476, 72.1%), followed by the posterior fontanelle (53/476, 11.1%). Multiple WB (≥ 2) represented the most common phenotype (52/77, 67.5% of cases), and 13 patients (16.8%) had at least one associated skeletal or craniofacial condition, most commonly craniosynostosis (10/13, 76.9%). Cephalic index analysis demonstrated a predominance of brachycephaly (54/77, 70.1%).ConclusionThis study presents a comprehensive evaluation of WB in a large pediatric cohort. WB may co-exist with craniosynostosis or other skeletal conditions such as osteogenesis imperfecta. Given the greater prevalence of WB compared to craniosynostosis (2.1%: clinic cohort & 0.57%: institutional cohort vs. 0.05%), clinicians should consider WB in the differential diagnosis of abnormal head shape in infancy, particularly in a subspecialist practice.

PurposeVasospasm is a potentially devastating condition that can develop in pediatric patients secondary to traumatic, inflammatory, infectious, and neoplastic etiologies and carries a high risk of morbidity and mortality (Isola et al. 2022, Krishna et al. 2005). Management is often adapted from adult to pediatric care given its relative rarity in children. There is level 3 evidence for the use of intrathecal (IT) calcium channel blocker (nicardipine) administration to treat refractory vasospasm in the adult subarachnoid hemorrhage (SAH) population. Its use has yet to be investigated in children (Krishna et al. 2005). We aim to review the safety profile and efficacy of IT nicardipine for non-traumatic SAH with refractory vasospasm at our institution.MethodsWe present a single institution retrospective series of pediatric patients treated with IT nicardipine for symptomatic vasospasm refractory to standard therapies including oral nimodipine, hyperdynamic augmentation therapies, and targeted intra-arterial therapy.ResultsPediatric patients (n = 3), ages 16–17 years old, admitted with non-traumatic SAH who developed severe, symptomatic, and refractory vasospasm were included. All patients presented with SAH from ruptured aneurysms. A 5-day course of IT nicardipine was initiated using a protocol adapted from our adult cerebrovascular/neurocritical care service. Decreases in peak middle cerebral artery velocity (range 61–290 cm/s) and Lindegaard ratios (range 0.3–4.2) were observed from initiation to completion of treatment.ConclusionsIT nicardipine for pediatric non-traumatic SAH with vasospasm refractory to standard treatments was used in our small series with no complications and no new ischemic events. Clinical and radiographic effects were encouraging. Further study is warranted.

IntroductionNeural tube defects (NTDs) have a high incidence of morbidity and mortality in resource-limited countries. Here, we determine the birth prevalence and describe the presentation, diagnosis, and management of NTDs at Komfo Anokye Teaching Hospital (KATH) in Ghana.MethodsAn ambispective study was conducted for all patients presenting to KATH with NTD from 2019 to 2023. Descriptive statistics and geospatial analysis were completed for relevant clinical data including patient demographics, clinical presentation, surgical details, treatment outcomes, and travel distance.ResultsOne hundred seventy-one patients presented with NTDs at a median age of 24 days [IQR 6, 113]. Birth prevalence was 4.7 per 1000 births. The majority of patients presented with myelomeningocele (MM) (79%) followed by encephalocele (16%). Surgical repair was performed for 131 (97%) MM cases at a median age of 33 days [IQR: 18–94 days] and for 25 (93%) encephalocele cases at a median age of 183 days [IQR: 41–384 days]. The median post-operative length of stay was 12 days [IQR 6, 22]. The surgical mortality rate was 7% (11/163). 76 (55%) MM patients developed hydrocephalus, of which 24 (32%) underwent CSF diversion at a median interval of 36 days [IQR 21, 143] following MM repair. Follow-up data were available for 158 (92%) patients, with a median follow-up duration of 16 months (IQR: 4–29 months). At the last follow-up, 70% of patients were alive.ConclusionTimely and affordable neurosurgical care remains a challenge for NTD patients at KATH and may contribute to elevated morbidity and mortality.

Hemispherotomy in infants under the age of 3 months is considered a high-risk procedure and is not routinely performed. A 2.5-month-old female infant weighing 5.1 kg successfully underwent a right vertical hemispherotomy after developing status epilepticus due to a right hemispheric Sturge-Weber meningo-angiomatosis. The surgical technique involved skull fixation at the skull base using kid pins to facilitate image-guided surgery with neuronavigation and augmented reality within the eyepiece of the microscope employing the vertical hemispherotomy technique. The surgical course was uneventful, with moderate blood loss (100 mL of red blood cells, 90 mL of fresh frozen plasma, and 150 mg of tranexamic acid transfused). The surgery duration was within 2 h. Postoperatively, seizures ceased immediately, and the infant experienced rapid developmental and neurological progress, remaining seizure-free for 8 months after surgery (Engel 1a) now.

PurposeWe faced and herein report a detailed description of pre-operative assessment, management, and post-operative follow-up of a 2-year and 10-month-old girl with neurofibromatosis 1 (NF1) who presented with severe, dystrophic, cervical kyphosis (170 degrees) associated with extensive pre- and para-vertebral plexiform neurofibromas, who also went under MEK inhibitors therapy. Cervical kyphosis in NF1 is particularly rare, and there is no extensive literature available on the subject in terms of clinico-radiological features, surgical approach, and outcomes. We therefore also performed a comprehensive review of the available literature on the topic.MethodsThe clinical report was made through the retrospective review of all medical documents and imaging of the patient. The systematic review was performed based on the inclusion and exclusion criteria set by the authors on surgical management of cervical kyphosis in NF1 patients according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).ResultsOur patient underwent a first-stage halo-gravity traction followed by a single-stage occipito-cervical posterior fusion. The six-week traction resulted in a reduction of the deformity from 170 to 90°. A further amelioration was obtained by surgery with a final 60% correction of the curvature (69° at last post-operative X-ray). No complications were observed at 1-and-a-half-year follow-up. The plexiform neurofibromas were treated with MEK inhibitors: trametinib for 1 year and 11 months until performing halo traction, and with selumetinib after surgery. We just found 19 papers suitable according to our selection criteria.ConclusionCombined anterior and posterior fusion (CAP) is generally the best treatment option, although it is not always feasible. When plexiform, symptomatic, inoperable neurofibromas coexist, surgery can be preceded or followed by MEK inhibitor treatment for better control or a volumetric reduction of the tumors. The best therapeutic choice should always be the result of a multidisciplinary, expert approach and patient-tailored design.

PurposeResearch into treatment options that complement surgery for open spinal dysraphism (OSD) is necessary to further improve outcomes following prenatal or postnatal surgery. We investigated erythropoietin (Epo) and its receptor (EpoR) as a potential neuroprotective agent and target in OSD.MethodsEpo- and EpoR-expression was examined on mRNA and immunohistochemical level in neuroplacodes obtained from a rat retinoic acid-induced OSD model at E16, E18, E22. Neural progenitor cells (NPCs) derived from spinal cords of adult Long Evans rats were exposed to varying concentrations of human artificial amniotic fluid (aAF). Cytotoxicity assays were conducted to assess the impact of aAF exposure, with and without Epo. The influence of Epo on NPC’s cellular marker expression was analyzed using qRT-PCR.ResultsEpoR mRNA expression was elevated significantly on E18 and E22 in OSD- compared to control tissue. EpoR immunoreactivity exhibited consistent expression in ventral and dorsal horns, central canal, and ganglia. It co-stained with Hif-2α, β-III-tubulin, NeuN, and Musashi1. Exposure of NPCs to aAF reduced proliferation and increased death rates dose-dependently. Adding Epo significantly counteracted antiproliferative and cytotoxic aAF effects on NPCs, resulting in similar or lower death rates than those observed in untreated controls. Exposure of NPCs to aAF reduced their expression of neuronal markers. Adding Epo restored their differentiation capacities.ConclusionInduction of EpoR in neuroplacodes, its co-staining with neuronal and NPC markers, and the neuroprotective effects of Epo on aAF-treated NPCs in vitro establish Epo as a promising candidate for neuroprotective therapies that can supplement surgical measures for OSD.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00381-025-07032-8.

IntroductionSpinal lipomas are common forms of closed spinal dysraphism, typically diagnosed in childhood but sometimes remaining unrecognized until adulthood. Adult presentation often includes pain, sensory disturbances, and urogenital dysfunction. While pediatric surgical management is well studied, adult outcomes, especially psychosocial ones, remain poorly understood. This study evaluates surgical, neurological, and psychosocial outcomes in adults undergoing their first surgery, comparing them with those of patients treated in childhood and followed into adulthood. It also emphasizes the importance of transitional care across the lifespan.MethodsThis retrospective cohort study included 24 patients treated at a single institution: 12 adults (SDA) undergoing first-time surgery from 2022 to 2023, and 12 historical controls (PSD) treated in childhood (2000 to 2005). Pre- and postoperative data were collected using a 14-item questionnaire assessing neurological, functional, and psychosocial outcomes. Statistical comparisons were performed within and between groups.ResultsThe median age at surgery was 30 years in SDA vs. 2 years in PSD. 75% of SDA patients had never been previously diagnosed with spinal dysraphism. Only urinary continence showed significant improvement neurologically in SDA patients (p = 0.025). However, psychosocial outcomes including pain, quality of life, social participation, and self-esteem, significantly improved postoperatively in the adult group. No surgical complications occurred in SDA; two CSF leaks were observed in PSD. Comparison of long-term outcomes revealed similar results across groups, except for a higher level of self-esteem in PSD patients (p = 0.039).ConclusionSurgical treatment of spinal lipomas in adulthood can provide meaningful symptomatic and psychosocial benefits, even without gross-total resection. This study underscores the limitations of viewing spinal dysraphism through a strict pediatric-adult binary and highlights the need for individualized, stage-specific care. The findings advocate for structured, lifelong transitional care pathways that integrate psychological and social support, addressing the full spectrum of patient well-being.

PurposeNeuroendoscopic lavage (NEL) has been described for post-hemorrhagic hydrocephalus management in intraventricular hemorrhage (IVH) of prematurity in European cohorts. We describe an initial single-center series from the USA.MethodsA retrospective review was performed for premature infants with IVH that underwent NEL at our institution between 2020 and 2023. Patient characteristics, clinical variables, and radiological assessments were collected.ResultsEleven patients (five female) with IVH grade III/IV underwent 13 procedures. Mean gestational age (GA) was 25 weeks and 2 days. Mean birth weight (BW) was 0.83 kg (kg). Average age at NEL was 40.4 ± 21.5 days, and mean weight was 1.51 ± 0.3 kg. Mean frontal horn index decreased from 0.68 to 0.56 after NEL (p < 0.001). Cerebrospinal fluid infection was diagnosed in 18.2%, secondary hemorrhage in 18.2%, and seizures in 27.3% of patients. One patient died postoperatively with refractory coagulopathy. Conversion to ventriculoperitoneal shunt at six-month follow-up was 8/11 (72.7%), with 50% 1-year revision-free shunt survival. No patients required a multi-catheter system. Rates of comorbidities and shunt dependency showed very strong positive correlations: hyaline membrane disease rate (R2 = 0.950), necrotizing enterocolitis (R2 = 0.999), and persistent ductus arteriosus (R2 = 0.975). Prematurity and shunt dependency showed a moderate to strong negative correlation: GA (R2 = 0.527) and BW (R2 = 0.344).ConclusionExtreme prematurity and comorbidities are associated with increased shunt dependency. However, NEL may decrease the development of complex multiloculated hydrocephalus and the need for multiple catheter systems. Larger, long-term studies are needed to define optimal timing and criteria for NEL and its benefits and impact on neurodevelopment in this fragile population.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00381-025-06994-z.

PurposeThis study presents a MATrix LABoratory (MATLAB)-based methodology for calculating intracranial volumes from head computed tomography (CT) data and compares it with established methods.MethodsRegions of interest (ROI) were manually segmented on CT images using a stylus pen, facilitated by mirroring a computer desktop onto a tablet. The volumetric process involved three main steps: (1) calculating the volume of a single voxel, (2) counting the total number of voxels within the segmented ROI, and (3) multiplying this voxel count by the single-voxel volume. This method was applied to 83 pediatric head CT scans from patients with minor head trauma, and the volumetric results were compared with those obtained from OsiriX.ResultsA paired t-test revealed a statistically significant difference (p < 0.001) between volumes obtained with our MATLAB-based method and those from OsiriX, with our method measuring 0.32% higher. However, an unpaired t-test found no statistically significant differences between the volumetric population groups (p = 0.84).ConclusionThe significant difference identified by the paired t-test likely reflects statistical distinctions arising from differences in the calculation methods of the two approaches. Conversely, the unpaired t-test suggests no statistically detectable differences between the volumetric populations. Although this does not imply that the two methods produce identical results, the volumetric populations derived from our method may originate from the same underlying population as those obtained using OsiriX. By taking these points into account, our method has the potential to serve as a valuable tool for volumetric measurements.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00381-024-06723-y.

IntroductionIn an effort to maximize benefit and minimize morbidity when performing fronto-orbital distraction osteogenesis (FODO) for unilateral coronal synostosis (UCS), we have transitioned to an endoscopic-assisted approach (“endo-FODO”). This study compares photogrammetric outcomes of patients who underwent FODO via an endoscopic-assisted versus open approach.MethodsWe retrospectively reviewed patients treated for UCS from 2013 to 2023. Photogrammetric outcomes at one to three years postoperatively were compared between patients who underwent endo-FODO and age- and sex-matched controls who underwent open FODO. Differences between pre- and postoperative periorbital symmetry ratios, canthal tilt symmetry, and orbital dystopia angle (ODA) were calculated.ResultsTwenty patients (ten per group) underwent surgery at a mean age of 6.1 ± 1.8 and 5.4 ± 1.1 months (p = 0.426) and were photographed at 1.6 ± 0.9 and 1.8 ± 0.9 years (p = 0.597) postoperatively in the endo-FODO and open FODO groups, respectively. Patients who underwent endo-FODO demonstrated significant improvements in margin-reflex distance 1 (MRD1) symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.004), canthal tilt symmetry (p = 0.020), and ODA (p = 0.009). Patients who underwent open FODO likewise demonstrated significant improvements in MRD1 symmetry ratio (p = 0.004), palpebral height symmetry ratio (p = 0.033), and ODA (p = 0.004). All postoperative measurements as well as degrees of improvement were similar between groups (p > 0.05).ConclusionsEndo- and open FODO were associated with significant and comparable improvements in soft tissue periorbital symmetry and orbital dystopia at nearly two years postoperatively. While continued follow-up until cranial maturity is needed to assess the durability of aesthetic results, these data support a minimally invasive, endoscopic alternative to fronto-orbital distraction.