Background Stimulation of the left vagus nerve is a well-established therapy for patients with drug-resistant epilepsy who are not candidates for resective or disconnection surgery. Right-sided vagus nerve stimulation is generally avoided due to concerns about serious cardiac side effects, as the right vagus nerve is anatomically associated with the innervation of the sinoatrial node. Only a few cases of right-sided vagus nerve stimulation in drug-resistant epilepsy have been reported to date. Materials and Methods An 8-year-old child with drug-resistant epilepsy and a left-sided Port-a-Cath positioned in the left internal jugular vein underwent uneventful insertion of a right-sided vagus nerve stimulator device. Results The vagus nerve stimulator was deactivated due to postoperative cardiac side effects, which subsequently resolved. Conclusion We report the first case of a child with drug resistant epilepsy in whom a right-sided vagus nerve stimulator was deactivated due to postoperative cardiac effects. Nonetheless, right-sided implantation may be a viable therapeutic option when left-sided implantation is contraindicated, provided careful cardiac monitoring and individualized risk-benefit analysis are undertaken.

Scaphocephaly is the most common form of craniosynostosis, characterized by premature closure of the sagittal suture. Surgical intervention is the standard treatment, aimed at correcting cranial shape and improving the cephalic index (CI), the key metric to quantify postsurgical outcomes. Controversies exist in the literature regarding the optimal timing of surgery and the choice of surgical technique. At our center, children aged 6 to 24 months presenting with sagittal synostosis and frontal bossing without significant occipital bullet undergo subtotal (anterior 2/3) vault remodeling, whereas those with both occipital bullet and frontal bossing are treated with total vault remodeling. This retrospective monocenter study tracks the progression of CI in pediatric patients undergoing these 2 techniques at 3 timepoints. From 2007 to 2015 the authors operated on 32 patients, excluding syndromic cases and reoperations. Twenty-three patients underwent subtotal and 9 total vault remodeling. The mean age at surgery was 12.66 months (range 6-24 months). The CI improved significantly in all patients after surgery. Overall, the CI increased from 66.67% preoperatively to 76.67% postoperatively and to 76.83% at 4 years. In the subtotal vault remodeling group, CI increased from 67.27% to 76.81% postoperatively, reaching 77.27% at 4 years. In the total vault remodeling group, CI rose from 65.11% to 76.29% postoperatively but decreased to 75.07% at 4 years. The authors' data show a sustained improvement in CI postoperatively, with a slight reduction in the total vault remodeling group at long-term follow-up.

Endoscopic endonasal transsphenoidal surgery (EETS) results in disturbance of nasal mucosa and airflow. Previous studies have demonstrated associated sinonasal morbidity but have not prospectively assessed olfactory function.To obtain prospective objective measure of sinonasal morbidity associated with EETS.We report our prospective patient-reported outcome study of 20 unselected, consecutive patients undergoing EETS. Baseline assessment of olfactory function was performed using the validated University of Pennsylvania Smell Identification Test alongside quality of life (QoL) assessments using the 22-item Sino-Nasal Outcomes Test (SNOT-22) and Anterior Skull Base Questionnaire (ASBQ) prior to surgery. Repeat olfactory function testing and QoL questionnaires were performed at 3 months and 12 months postoperatively. All patients underwent pituitary surgery and there was one extended anterior approach.Mean olfactory function score was worse at 3 months (25.9) compared with baseline (30.5), p = 0.02. This improved back toward baseline at 1 year (29.4). Three patients (15.8%) had significant residual olfactory impairment at 1 year. There were no significant differences in SNOT-22 score or ASBQ at 3-month or 1-year follow-up. EETS is associated with minor disturbances in olfactory and sinonasal QoL.Formal assessment of olfactory function has proven more sensitive in identifying deficiencies in olfaction following surgery; however, there is little or no correlation with QoL scores.

Unicoronal synostosis is a rare condition leading to anterior plagiocephaly with facial scoliosis and deformation of the anterior cranial fossa. Fronto-orbital advancement and remodelling (FOAR) is the standard of care for management, aiming to normalise the brow shape and position while ameliorating the risk of raised intracranial pressure (ICP) throughout childhood. Published long-term surgical outcome data for unicoronal synostosis is lacking. The authors present our series of 151 cases of FOAR for unicoronal synostosis between January 2000 and January 2023. The average age at surgery was 22.7 months, with an average follow-up of 87 months. Ninety-six patients (66.2%) had no comorbidity. Nineteen (13.1%) had named genetic or chromosomal disorders. There was a 33.1% dural tear rate with no related postoperative CSF leak. Ninety-three patients (67.8%) had a blood transfusion with average donor exposure <1. The total early complication rate was 8.6% most commonly infection and wound dehiscence at 3.4% and 2.8%, respectively. The most common late complication was temporal recession in 30 (20.1%) patients and 3 of these patients required revision surgery. None of our patients required investigation for, or treatment of, raised ICP after the primary surgery. There were no life-threatening complications or mortalities. The authors compare our results to a previous publication on trigonocephaly patients and other available published data. The authors present our recommendations, which include support for a supraregionalized service that encompasses high-volume workload and multidisciplinary care.

BACKGROUND:Intraoperative magnetic resonance imaging (ioMRI) is increasingly used in neurosurgery to enhance surgical precision and decision-making. However, its implementation remains limited due to high costs, infrastructure demands, and workflow considerations. While well studied in adult neurosurgery, the role of ioMRI in pediatric epilepsy surgery is less clear. Reported challenges include wound complications, infections, and positioning difficulties. This study evaluates the application, utility, and safety of intraoperative 3T MRI in pediatric epilepsy surgery over a ten-year period at a tertiary specialized referral center. METHODS:This retrospective study analyzed data from all pediatric epilepsy surgeries performed between 2013 and 2022 in our unit. We reviewed patient demographics, the treated pathologies, the application of intraoperative 3T MRI (ioMRI), the extent of continued ioMRI-guided surgery, seizure outcomes at one year postoperatively, and the requirement for reoperation. Intraoperative MRI-related complications documented within 90 days of surgery were also assessed. RESULTS:Altogether, 125 pediatric epilepsy patients underwent surgery. Resections were performed in 107 (85.6 %) and disconnection procedures in 18 (14.4 %) patients. Their overall median age was 10 years (range 0.5-19 years). In total, 108 (86.4 %) patients underwent surgery with ioMRI. Of these, 91 (84.3 %) had resection and 17 (15.7 %) had disconnection surgery. Of the 108 patients with ioMRI 56 (51.9 %) returned to theater, 45 (49.5 %) out of 91 for further resection, and 11 (64.7 %) out of 17 to complete disconnection. Thirteen (12 %) of the 108 patients were reoperated upon later in another session. In total, 17 (13.6 %) patients had surgery without ioMRI, 16 (94.1 %) of these for resection and 1 (5.9 %) for disconnection surgery; of these 3 (17.6 %) were reoperated upon later in another session. The complication rate for surgeries performed with ioMRI was 3.7 %. There were no wound healing disorders, position-related damages, or anesthesia-related complications. CONCLUSIONS:While the technology may present certain logistical and financial challenges, we observed a lower rate of reoperations and a low complication rate in ioMRI-guided resective epilepsy surgeries in children compared to surgeries without ioMRI. These findings require confirmation through larger, multi-center studies to better define the role of ioMRI in pediatric epilepsy surgery.

Gangliogliomas are rare, slow-growing brain tumors frequently associated with seizures in pediatric patients. This study evaluated the utility of high-field intraoperative magnetic resonance imaging (ioMRI) in the surgical management of ganglioglioma-related drug-resistant epilepsy in children. Specifically, it sought to determine whether ioMRI improves outcomes by optimizing the extent of resection, enhancing seizure control, reducing reoperations, and minimizing complications. This retrospective single-center study included 14 pediatric patients with cerebral ganglioglioma who underwent epilepsy surgery with ioMRI from 2014 to 2022. The median age was 11.5 years (range 3–16 years). Patient demographics, the rate of continued ioMRI-guided surgery, the extent of resection, histology, complications, reoperations, and seizure outcomes at 1 year postoperatively were assessed. Residual tumor was detected using ioMRI in 9 of 14 patients (64.3

Trigeminal neuralgia (TN) without neurovascular conflict poses a therapeutic challenge. Microsurgical internal neurolysis (IN), also known as nerve combing, has emerged as a viable surgical option when microvascular decompression (MVD) is not indicated. This systematic review and meta-analysis, conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, included eight case series with a total of 193 patients. Pain relief outcomes were assessed using the University of California at San Francisco (UCSF) criteria and the Barrow Neurological Institute (BNI) pain intensity score. Excellent pain relief (UCSF criteria) was achieved in 76.3

OBJECTIVE The Neurolocate module is a 3D frameless patient registration module that is designed for use with the Neuromate stereotactic robot. Long-term electrical stimulation of the globus pallidus internus (GPi) and subthalamic nucleus (STN) via deep brain electrode implantation is particularly successful in a select group of movement disorders in pediatric patients. This study aimed to review the targeting accuracy of deep brain stimulation (DBS) electrode implantation in a single center, comparing standard frame-based techniques to the frameless Neurolocate module. METHODS Twenty-four pediatric patients underwent implantation of bilateral DBS electrodes under general anesthesia during the period of August 2018-August 2022. All patients underwent robot-assisted stereotactic implantation of DBS electrodes using an intraoperative O-arm 3D scanner to confirm the final electrode position. These coordinates were compared with the planned entry and target, with attention to depth, radial, directional, and absolute errors, in addition to Euclidean distance (ED). The primary outcome evaluated the accuracy and safety of the Neurolocate frameless technology compared with standard frame-based techniques. RESULTS Of the 24 bilateral DBS electrode implantations performed, 62.5% used Neurolocate technology: 87.5% were delivered to the GPi and the remaining 12.5% to the STN. The mean patient age was 11.0 (range 4-18) years and 70.8% were male. The median absolute errors in x-, y-, and z-axes were 0.35, 0.75, and 0.9 mm, respectively, using the Neurolocate module compared with 0.30, 0.95, and 1.1 mm using the standard frame-based technique. The median ED from the planned target to the actual electrode position with the Neurolocate module was 1.28 mm versus 1.69 mm using standard frame-based techniques. No major perioperative complications occurred. CONCLUSIONS Stereotactic robot-assisted DBS implantation with the frameless Neurolocate module is safe for use in the pediatric population, showing good surgical accuracy and no inferiority to standard frame-based techniques. The Neurolocate module for robotic DBS surgery has the potential to improve surgical targeting accuracy, surgical time, patient comfort, and safety.

Trigonocephaly is a craniofacial malformation caused by premature fusion of the metopic suture. Surgical correction frequently results in the need for blood transfusion. Transfusion complications include transfusion-transmitted infections (TTIs), immune-mediated reactions, and volume overload. Donor exposure (DE) describes the number of blood products from unique donors with increasing DE equating to an increased risk of TTI. We evaluate data on 204 trigonocephaly patients covering 20 years of practice with respect to blood transfusions and DE. This represents the largest series from a single unit to date. A protocol based on our experiences has been devised that summarizes the key interventions we recommend to minimize blood transfusions and DE in craniofacial surgery. Patients operated on between 2000 and 2020 were included. DE and a range of values were calculated including estimated red cell loss (ERCL) and estimated red cell volume transfused (ERCVT). Groups were established by relevant interventions and compared using the Mann–Whitney U test. Mean DE fell from 1.46 at baseline to 0.85 ( P <0.05). Median allogenic transfusion volume fell from 350 mL at baseline to 250 mL ( P <0.05). Median ERCL fell from 15.05 mL/kg at baseline to 12.39 mL/kg and median ERCVT fell from 20.85 to 15.98 mL/kg. Changes in ERCL and ERCVT did not reach statistical significance. DE can be minimized with the introduction of key interventions such as a restrictive transfusion policy, preoperative iron, cell saver, tranexamic acid, and use of a matchstick burr for osteotomies.

Introduction: Posterior vault distraction osteogenesis (PVDO) allows significant increase in intracranial volume but is associated with complications including cerebrospinal fluid (CSF) leaks, infection and device failure. The authors outline their outcomes over 12 years and the impact of PVDO on pre-existing Chiari malformation type 1 (CM). Method: Retrospective review of all PVDOs in our unit over a period of 12 years from 2011 to 2023. Results: 51 patients. The mean age of surgery 9 months. The mean length of follow-up 72 months. 82% no complications, 12% dural tear with 2% CSF leak; 4% infection, 2% device failure, 10% blood transfusion. CM in 20% of patients—all are multisuture with 90% lambdoid synostosis involvement. CM is unchanged in the majority of patients post-PVDO. Conclusion: PVDO safe and effective with no re-do posterior vault expansions over 12 years. CM does not tend to resolve after PVDO and high-risk groups include multisuture and Crouzons.

Background Complete resection is essential for achieving seizure freedom in children with drug-resistant epilepsy due to focal cortical dysplasia (FCD). However, identifying altered structures intraoperatively can be challenging, potentially leading to reoperations. This study assessed the impact of intraoperative MRI (iMRI). Methods We conducted a single-center retrospective study at Alder Children’s Hospital from 2013 to 2022. The study included all children diagnosed with FCD, analyzing demographics, iMRI use, the rate of continued iMRI-guided surgery and reoperations, histology, and seizure outcomes at 1-year follow-up. Results Thirty-two pediatric patients (median age: 10 years, 21 males and 11 females) underwent surgery for FCD. Of them 8 (25 %) had FCD Type I, 19 (59.4 %) had FCD Type II, and 1 patient (3.1 %) had FCD Type III. iMRI was used in 27 patients (84.4 %), and 17 (63 %) of these patients required further iMRI-guided surgery. Four patients underwent reoperation in a separate session. At 1-year follow-up, seizure outcomes in the iMRI group were Engel I in 15 patients (71.4 %), Engel II in 4 (19 %), and Engel III and IV in 1 patient (4.8 %) each. Five patients (15.6 %) had superficially localized lesions and underwent surgery without iMRI. None of them required reoperation, and four (80 %) were seizure-free at 1 year. Seven patients were lost to follow-up. Conclusion iMRI plays a significant role in identifying residual epileptogenic tissue in surgery for FCD, influencing surgical decisions and leading to additional iMRI-guided resections. Most patients with iMRI-guided surgery achieved favorable seizure outcomes (Engel I) at 1 year of follow-up. Long-term follow-up is needed to validate these findings and to confirm sustained seizure control.

BackgroundWe assessed the accuracy and performed a directional analysis of robot-assisted implantation of stereoelectroencephalography (SEEG) depth electrodes in children using the frameless neurolocate 3D registration module.MethodsThirteen children with epilepsy undergoing stereotactic robot-assisted insertion of SEEG electrodes were included. Six children were operated on with standard frame-based registration while 7 with the use of the frameless neurolocate registration module. Accuracy and directional analysis of orthogonal and oblique electrodes were assessed by calculating the absolute error, the radial error, the angle error, and the Euclidean distance.ResultsOf 172 electrodes 89 were implanted in the 6 standard frame-based mode patients and 83 in the 7 neurolocate patients. The overall mean age was 12.2 +/- 4.4 years (range 2-17). The mean number of electrodes in each patient was 13.2 +/- 2.04 (range 9-17). The median radial error of electrode placement in the neurolocate patients (1.08 mm, [IQR: 1.26]) was significantly less when compared with standard frame-based mode patients (1.49 mm, [IQR 1.25)]; p = 0.04). The same applies to the median angle error which was in the neurolocate group 1.61 degrees [IQR: 1.46] and in the standard frame-based group 2.16 degrees [IQR: 2.09]; p = 0.019. Directional analysis of electrode trajectories in the neurolocate group showed that in the x-axis the median absolute error of orthogonal electrodes (0.4 mm, [IQR: 0.475]) was less when compared with oblique electrodes (0.7 mm, [IQR: 1.2]; p = 0.007). In the standard frame-based mode group in the y-axis, the median absolute error of orthogonal electrodes (0.7 mm, [IQR: 1.3]) was less compared with oblique electrodes (1.25 mm, [IQR: 1.6]; p = 0.03).ConclusionIn paediatric patients, robot-assisted SEEG depth electrode implantation with the non-invasive and easy-to-use frameless neurolocate registration module is a consistent and accurate procedure.

PURPOSE:Chorea is a clinical sign characterized by involuntary, rapid, unpredictable, and irregular muscle movements that can affect various parts of the body. It can be seen in various medical conditions, both neurological and systemic, of genetic and acquired etiology. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been used to treat various types of chorea. The aim of this study was to evaluate the efficacy of GPi DBS for chorea in pediatric patients. METHODS:The authors undertook a single-center retrospective study of all pediatric patients who underwent DBS in the period from July 2017 to April 2024 to identify those presenting with chorea. RESULTS:Three patients with chorea underwent bilateral posteroventral GPi DBS without surgical complications. The mean age at operation was 14.2 years (range: 1.5 years), and the mean follow-up was 49 months (range: 15 months). Two of the 3 patients experienced a positive effect on chorea with an improvement in functional status. In one patient, the pre- and postop Gross Motor Function Classification System (GMFCS) score was 4, while his Burke-Fahn-Marsden Dystonia Scale (BFMDS) score improved from 102/20 pre- to 53.5/20 postop. In the other patient the GMFCS score improved from 4 preop to 3 postop. His preop BFMDS score was not available, postop it was 83/120. In the patient who did not experience a positive effect on chorea the pre- and postop GMFCS score was 4, her BFMDS score was 84.5/120 pre- and 100/120 postop. CONCLUSION:Bilateral GPi DBS can be safely administered to pediatric patients with choreiform movement disorders, and it could be an effective treatment option for managing chorea in certain patients.

Craniosynostosis is the premature fusion of the skull sutures, resulting in abnormal skull shape and volume. Timely management is a priority in avoiding raised intracranial pressure which can result in blindness and neurodevelopmental delay. Due to the COVID-19 pandemic, theater access was reduced. A risk stratification scoring system was thus devised to score patients attending surgery and aid in prioritization according to surgical need. The authors present the Paediatric Vault Score (PVS), which can also be customized to each unit’s individual protocols. Ten patients on the waiting list were randomly selected and their clinical information was summarized in uniform anonymized reports. Six craniofacial consultants were selected as assessors and given 1 week to independently rank the patients from 1 to 10. Each scorer’s ranking was verified against the PVS template and concordance was analyzed using the Kendall tau correlation coefficient (KT). Three cycles of the scoring process were carried out. Improvements were made to the scoring tool following cycle 1. Cycle 1 revealed 2 clinicians to be concordant with the PVS system and 4 to be discordant. Cycle 2 revealed all 6 clinicians to be concordant, with a mean KT score of 0.61. The final cycle revealed all 6 clinicians to be concordant, with a mean KT score of 0.70. Four scorers increased their concordance once the scoring sheet was introduced. Kendall’s correlation of concordance calculated the interrater reliability to be 0.81. The PVS is the first known vault scoring system to aid in risk stratification and waiting list prioritization.

The aim of the study is to enhance understanding, raise awareness and inform prevention programmes regarding potential factors that lead to severe paediatric injuries caused by unintentional falls from windows. This is a retrospective review from a major Trauma Centre, covering the majority of North West England and North Wales and included children under the age of 16 that had sustained falls from windows and were hospitalised between April 2015 and June 2020. Overall, 825 patients’ records have been reviewed, 39

Introduction: We present the largest series of paediatric intracranial empyemas occurring after COVID-19 infection to date, and discuss the potential implications of the pandemic on this neurosurgical pathology. Methods: Patients admitted to our centre between January 2016 and December 2021 with a confirmed radiological diagnosis of intracranial empyema were retrospectively reviewed, excluding non-otorhinological source cases. Patients were grouped according to onset before or after onset of the COVID-19 pandemic and COVID-19 status. A literature review of all post-COVID-19 intracranial empyemas was performed. SPSS v27 was used for statistical analysis. Results: Sixteen patients were diagnosed with intracranial empyema: n = 5 prior to 2020 and n = 11 after, resulting in an average annual incidence of 0.3% prior to onset of the pandemic and 1.2% thereafter. Of those diagnosed since the pandemic, 4 (25%) were confirmed to have COVID-19 on recent PCR test. Time from COVID-19 infection until empyema diagnosis ranged from 15 days to 8 weeks. Mean age for post-COVID-19 cases was 8.5 years (range: 7–10 years) compared to 11 years in non-COVID cases (range: 3–14 years). Streptococcus intermedius was grown in all cases of post-COVID-19 empyema, and 3 of 4 (75%) post-COVID-19 cases developed cerebral sinus thromboses, compared to 3 of 12 (25%) non-COVID-19 cases. All cases were discharged home with no residual deficit. Conclusion: Our post-COVID-19 intracranial empyema series demonstrates a greater proportion of cerebral sinus thromboses than non-COVID-19 cases, potentially reflecting the thrombogenic effects of COVID-19. Incidence of intracranial empyema at our centre has increased since the start of the pandemic, causes of which require further investigation and multicentre collaboration.

Dermal sinus tract is a congenital abnormality, which falls within the spinal dysraphism spectrum. Occurring as a result of focal incomplete disjunction during primary neurulation, it presents as a small and innocuous-appearing punctum in the dorsal midline (most frequently over the lumbar spine). The clinical course and complications are all but innocuous, however, as they are frequently associated with recurrent meningitis, spinal abscesses and epidermoid and dermoid inclusion tumours. Progressive and disabling neurological sequelae may occur as a result of delayed identification and management. Tethered cord syndrome is a condition associated with all spinal dysraphic conditions, wherein abnormal tension develops within the spinal cord. This is often as a result of tethering of the conus medullaris at a lower-than-normal position. Traction, resulting in ischaemia and hypoxia, leads to neural injury and a chronic and slowly progressive neurological syndrome, characterised by weakness, gait disturbances, sensory impairment and bladder/bowel dysfunction.

Saethre-Chotzen syndrome (SCS) is a syndromic craniosynostosis with pathogenic variants in the TWIST1 gene showing a broad phenotypic spectrum. Controversies exist in the literature regarding surgical management with single one-stage versus patient-tailored surgery and the related reoperation rate for intracranial hypertension of up to 42%. At our center, SCS patients are offered patient-tailored surgery with single-stage fronto-orbital advancement and remodeling or fronto-orbital advancement and remodeling and posterior distraction in an individually determined order. The authors' database identified 35 confirmed SCS patients between 1999 and 2022. Involved sutures in craniosynostosis were left unicoronal (22.9%), bicoronal (22.9%), sagittal (8.6%), bicoronal and sagittal (5.7%), right unicoronal (2.9%), bicoronal and metopic (2.9%), bicoronal, sagittal and metopic (2.9%), and bilateral lambdoid (2.9%). There was pansynostosis in 8.6% and no craniosynostosis in 14.3% of the patients. Twenty-six patients, 10 females, and 16 males were operated on. Mean age at the first surgery was 1.70 years, and 3.86 years at the second surgery. Eleven of 26 patients had invasive intracranial pressure monitoring. Three patients presented with papilledema before the first surgery and 4 afterward. Four of the 26 operated patients were operated initially elsewhere. The other 22 patients were initially referred to our unit and underwent patient-tailored surgery. Nine of these patients (41%) had a second surgery, and 3 (14%) of them were because of raised intracranial pressure. Seven (27%) of all operated patients had a complication. Median follow-up was 13.98 years (range, 1.85-18.08). Patient-tailored surgery in a specialized center and long-term follow-up allow for a low reoperation rate for intracranial hypertension.

Subcortical band heterotopia is a rare X-linked neuronal migration disorder primarily in females often associated with drug-resistant epilepsy. The aim of this study is to review the literature for non-pharmacological treatment options of drug-resistant epilepsy in subcortical band heterotopia. In accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, we performed a systematic review. Entering the keywords “double cortex,” “subcortical band heterotopia,” and “subcortical laminar heterotopia,” we searched Scopus and PubMed databases. We paid particular attention to type of invasive and non-invasive treatment, radiological presentation, and outcome. We also describe a related case report, managed at Alder Hey Children’s Hospital, Liverpool. The systematic literature review yielded 25 patients with subcortical band heterotopia and drug-resistant epilepsy who underwent non-pharmacological treatment. Including our patient, 26 patients were reported. The patients’ mean age at seizure onset was 6.5 years (range 0.2–23) with a female sex predilection (5.25:1). The patients’ mean age at invasive or non-invasive treatment was 21.5 years (range 6.5–51). The 26 patients underwent 29 non-pharmacological treatments. Ten patients underwent corpus callosotomy; 8 patients had a formal temporal lobectomy. Three patients had focal cortical resection. Two patients respectively had multiple subpial transections, insertion of a vagal nerve stimulator, or deep brain stimulation of the bilateral anterior nuclei of the thalamus. One patient underwent responsive focal neurostimulation. Another patient had transcutaneous stimulation of the vagal nerve. Sixteen patients reported a reduction or the disappearance of the seizures; 1 patient had no improvement. The outcome of 2 patients was classified class I, of 1 patient class II, of 1 patient class III, and of 5 patients class IV according to the Engel Epilepsy Surgery Outcome Scale. Mainly corpus callosotomy and formal temporal lobectomy have been performed as non-pharmacological treatment with few cases published overall. Several other invasive procedures and one non-invasive technique are based on case reports. The small number of reported cases prevents drawing a firm conclusion as to which non-pharmacological treatment is the best treatment option for refractive epilepsy in patients with subcortical band heterotopia.

The thyroid transcription factor 1 (TITF-1) gene plays an important role in the development of the ventral forebrain, thyroid and lungs. Mutations of this gene are known to cause benign hereditary chorea (BHC) and can cause the full spectrum of abnormalities seen in the brain-thyroid-lung syndrome. Abnormalities of the ventral forebrain on imaging have been variably documented in the literature. Multiple previous reports describe a cystic pituitary mass, as well as duplication of the pituitary stalk and communication between an intrasellar cyst and the third ventricle. The initial MRI performed in our case was interpreted as an intrasellar cyst, but the high-resolution MRI performed later was able to resolve this as a persisting embryonal infundibular recess (PEIR), rather than the cystic pituitary mass which has previously been described. This case illustrates the role of the TITF-1 gene in the development of the pituitary and hypothalamus.