Endoscopy-Assisted Craniosynostosis Surgery without Postoperative Helmet Molding Therapy.

OBJECTIVE: Endoscopy-assisted craniosynostosis surgery (EACS) yields excellent surgical outcomes by minimizing blood loss, operative time, and hospital stays. Postoperative helmet therapy (PHT), commonly employed for head shape correction, involves frequent adjustments, potential complications, and high costs. Given the rising cost of helmet therapy, reduced insurance coverage, and limited availability in low- and middle-income countries, understanding success rates without helmet use is crucial. The present study analyses the anthropometric results of the first EACS series without PHT. METHODS: A retrospective analysis of a single-center series involving 90 consecutive patients who underwent EACS without PHT from 2012 to 2022 was conducted, with a follow-up exceeding 3 years. The study exclusively included patients with nonsyndromic isolated sagittal synostosis, with 33 meeting the criteria. Craniometric measurements were obtained from preoperative, 1-year postoperative, and the latest computed tomography scans. For isolated sagittal synostosis cases, the cephalic index (CI) was calculated (CI >75 for excellent results, CI 70-75 for good results, and <70 for poor results). Collected data encompassed patient sex, age, and follow-up time. RESULTS: The mean age was 84.8 ± 45.3 days (2.79 ± 1.49 months) within a range of 3-172 days. The preoperative mean CI was 68 ± 42, increasing to 76 ± 6 1 year postoperatively (mean difference +8 ± 6.3; P = 0.0001). Seventy-one percent of patients achieved excellent results, 23% good (CI = 70-75), and 6% poor. Reintervention was unnecessary. CONCLUSIONS: EACS without PHT demonstrates favorable anthropometric results, cost reduction, and simplified postoperative management.

Intracranial leptomeningeal CNS ganglioneuroblastoma. First report and review of the literature.

BACKGROUND: CNS ganglioneuroblastoma in an extremely rare embryonal tumour, specifically in the pediatric population. Bad prognosis is documented due to aggressiveness and absence of protocolized treatment at the moment. CLINICAL DESCRIPTION: We present the case of a 5-year-old boy who presented with sudden loss of consciousness. CT scan was performed showing a large posterior fossa lesion with several intraventricular focal lesions, suggesting metastases, the largest one located inside the III ventricle. The patient underwent a posterior fossa resection of the lesion and a subtotal resection of the III ventricle lesion, with adjuvant chemotherapy. The evolution was poor and the patient finally died 3 months after diagnosis. CONCLUSION: Ganglioneuroblastoma is extremely likely to recur quickly and extensively. There is little knowledge about treatment options but is documented that gross total resection followed by adjuvant radiotherapy and chemotherapy is the best management in these patients.

Presigmoid approach preserving the superior petrosal sinus in a pontine cavernous malformation associated to abnormal venous drainage of the brainstem: how I do it.

BACKGROUND: The presigmoid approach classically includes the ligature and section of the superior petrosal sinus to get a wider visibility window to the antero-lateral brainstem surface. In some cases, the separation of this venous structure should not be performed. METHOD: We present our experience getting safely to a pontine cavernous malformation through a conventional mastoidectomy presigmoid approach preserving an ingurgitated superior petrosal sinus because the association with an abnormal venous drainage of the brainstem. CONCLUSIONS: When sectioning the superior petrosal sinus in classical presigmoid approaches is contraindicated, its preservation could also offer good surgical corridors to get to small-medium anterior and lateral brainstem cavernous malformations.

Posterolateral trans dentate approach to anterior extensive extramedullary tumor of the cervical spine in a pediatric case: how I do it.

BACKGROUND: Extramedullary anterior cervical canal tumors can be challenging lesions to reach. The posterolateral trans dentate approach offers an alternative route. METHOD: Classic posterior laminoplasty is done to expose the medulla; the dentate ligament is identified as a fibrous structure running from the lateral pial surface of the medulla to the lateral dura between nerve roots spaces. Once the ligament is cut, the medulla can be gently rotated to access the anterior cervical canal. Intraoperative neurophysiological stimulation is mandatory. CONCLUSION: This approach allows a safe route, without the need for corpectomies. It should be considered especially in children where multilevel corpectomies could be challenging.

Bilateral chiasm and optic nerve micro-decompression in a child with extensive cranial fibrous dysplasia: how I do it.

BACKGROUND: Surgical decompression to the optic-chiasmatic region in craneofacial fibrous dysplasia (CFD) must be performed safely to improve or stabilize visual loss. METHOD: We describe a technical nuance when facing on a huge, deformed skull with potentially imbricated dura mater. Craniectomy was performed in concentric arches allowing to expose surgical field and elevated step by step. Bilateral micro-decompression was performed after without difficulties. CONCLUSIONS: Decompressing both optic nerves using this technique is safe and relatively simple to perform.

Technical Nuances in Neuroendoscopic Lavage for Germinal Matrix Hemorrhage in Preterm Infants: Twenty Tips and Pearls after More than One Hundred Procedures.

INTRODUCTION: Posthemorrhagic hydrocephalus in preterm infants is a serious entity related to high mortality and morbidity. Neuroendoscopic lavage (NEL) is a suitable alternative for the management of this pathology. However, as with every endoscopic technique, it requires some experience and several cases to master. METHODS: We present a descriptive study of some technical nuances, tips, and tricks that have been learned in the last 8 years with over a hundred NELs performed in preterm infants. These variations are classified into 3 categories according to their temporal relationship with the surgical procedure: preoperative stage, intraoperative stage, and postoperative stage. We include a brief description of each one and the reasons why they are included in our current clinical practice. RESULTS: Twenty tips and pearls were described in detail and are reported here. Preoperative, intraoperative, and postoperative variations were exposed and related to the most frequent complications of this procedure: infection, cerebrospinal fluid leak, and rebleeding. CONCLUSIONS: NEL is a useful technique for the management of germinal matrix hemorrhage in preterm infants. These technical nuances have improved the results of our technique and helped us to prevent complications related to the procedure.

Two-Stage Approach for Unstable Pediatric Craniocervical Junction Anomalies with a Halo Vest and Delayed Occipitocervical Fusion: Technical Note, Case Series, and Literature Review.

OBJECTIVE: Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes. METHODS: A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis. RESULTS: Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from -3.26 mm to -6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41). CONCLUSIONS: The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

Chest Implantation of Adjustable Gravitational Valves: An Easy, Safe, and Stable Alternative to Control Symptomatic Overdrainage in Shunted Children.

OBJECTIVE: Shunt overdrainage is a potential complication in pediatric hydrocephalus. The addition of adjustable gravitational units to previous shunt systems has been proposed as effective management for this problem. These devices have been traditionally implanted over the occipital bone. We propose chest implantation as an easier, safer, and more stable alternative in the pediatric population, especially in those cases with parieto-occipital shunts. METHODS: This study comprises a retrospective analysis from a unicentric case series of pediatric patients affected by overdrainage and managed with adjustable gravitational valves implanted in the chest. The device implantation technique is described in detail and takes no more than 15 minutes. RESULTS: Thirty-seven patients met the criteria. The mean age of implantation was 9.62 years. The mean follow-up in the series was 38 months. The mean number of pressure adjustments was 2.48. The mean "deviation angle" of the device to the longitudinal body axis was 5.8°. The complications per year of shunt were <0.02 with no disconnection of the catheters in any case during follow-up. CONCLUSIONS: In our experience, chest implantation for adjustable gravitational devices was a suitable shunt modification in pediatric patients suffering from overdrainage.

Cranial expansion and aqueductoplasty for combined isolated fourth ventricle and slit-ventricle syndrome: a surgical alternative.

INTRODUCTION: An isolated fourth ventricle (IFV) is a rare entity observed in shunted patients and its treatment is still uncertain. Endoscopic aqueductoplasty has shown good results for restoring CSF flux between the third and fourth ventricles. However, it needs some grade of ventricular dilation to be performed. Some patients affected by IFV show slit-ventricle morphology in CT/MRI. Usually, the rise of opening pressure or the shunt externalization gets enough ventricular dilation. However, the lack of intracranial compliance in some patients makes these options unsuitable and high-ICP symptoms are developed without ventricular dilation. METHODS: We present a two cases series affected by IFV with no ventricular dilation in radiological exams. ICP sensors were implanted, observing high-ICP and establishing the diagnosis of craniocerebral disproportion. A two-stage surgical plan based on a dynamic cranial expansion followed by a supratentorial endoscopic aqueductoplasty was performed. A physical and mathematical model explaining our approach was also provided. RESULTS: Chess-table cranial expansion technique was performed in both patients. Six/seven days after the first surgery, respectively, ventricular dilation was observed in CT. Endoscopic precoronal aqueductoplasty was then performed. No postoperative complications were described. IFV symptoms improved in both patients. Eighteen and 12 months after the two-stage surgical plan, the patients remain symptom-free and void of flow is still observed between the third and the fourth ventricles in MRI. CONCLUSION: The two-stage approach was a suitable option for the treatment of these complex patients affected by both craniocerebral disproportion and isolated fourth ventricle.

Diastematobulbia type II without associated dermoid tumor: case report.

Split cord malformation (SCM) is a term used for all double spinal cords. It represents 3.8%-5% of spinal dysraphisms. Pang et al.'s embryological theory proposes the formation of an "accessory neurenteric canal" that communicates with the yolk sac and amnion. To the authors' knowledge, only three cases of diastematobulbia (basicranial SCM) associated with a spur or dermoid have been reported in the literature.The case patient is a newborn girl with an occipitocervical meningocele and dermal sinus associated with an anomaly of notochordal development in the transition between the medulla oblongata and the spinal cord (diastematobulbia) without a bony septum or dermoid cyst. The patient also has agenesis of the atlas and an absence of corticospinal tract decussation. This patient underwent reconstruction of the occipital meningocele and dermal sinus excision.To the authors' knowledge, this is the first described case of type II diastematobulbia (basicranial SCM), without a dermoid cyst. The authors analyzed the embryological errors present in the case patient and considered the option of further surgical treatment depending on the evolution of the patient's condition. At the time of this report, the patient had shown normal psychomotor development. However, this fact may only be due to the patient's young age. Considering that after initial untethering the patient remained clinically asymptomatic, conservative and close surveillance has been and continues to be the proposed treatment.

Manifestaciones endocrinas de los tumores germinales del sistema nervioso central en niños / Endocrine manifestations of central nervous system germ cell tumors in children

INTRODUCCIÓN: Los tumores germinales del sistema nervioso central deben ser correctamente diagnosticados, pues su tratamiento suele ser eficaz y no siempre requieren cirugía. Los objetivos del estudio son describir las manifestaciones endocrinas de estas neoplasias y comparar su momento de aparición con el de las alteraciones neurológicas y visuales. PACIENTES Y MÉTODOS: Revisión de historias de pacientes menores de 14 años atendidos en una unidad de endocrinología pediátrica desde 2000 hasta 2018. Pruebas estadísticas: Wilcoxon y Fisher. RESULTADOS: Se estudió a 12 pacientes (10 mujeres) con una edad al diagnóstico de 9,4±1,7 años y un tiempo de seguimiento de 5,5±3,0 años; 10 presentaban tumores de la región selar, uno pineal y uno bifocal. Las alteraciones clínicas que llevaron al diagnóstico eran neurológicas o visuales en 9casos y hormonales en 3. De los que consultaron por síntomas neurológicos o visuales, 7 refirieron previamente alteraciones hormonales, luego, estas estaban presentes en 10 de los niños al diagnóstico; la más frecuente fue la diabetes insípida central (8 casos). El periodo medio de presencia de síntomas endocrinológicos previos al diagnóstico fue de 25,0±26,2 meses, mucho más largo que el de los neurooftalmológicos, de 2,0±2,1 meses (p = 0,012). CONCLUSIONES: Casi todos los tumores germinales intracraneales presentaron al diagnóstico manifestaciones endocrinas, la más frecuente de las cuales fue la diabetes insípida central. Los síntomas hormonales suelen presentarse bastante antes que los neurooftalmológicos. La correcta valoración clínica y endocrinológica puede adelantar el diagnóstico de estos tumores

INTRODUCTION: Central nervous system germ cell tumors need to be adequately diagnosed because their treatment is usually effective and they do not always require surgery. The study objectives are to describe the endocrine manifestations of these tumors and to compare the time of their onset to that of the occurrence of neurological and visual changes. PATIENTS AND METHODS: The medical histories of patients under 14 years of age seen at a pediatric endocrinology unit between 2000 and 2018 were reviewed. Wilcoxon and Fisher statistical tests were performed. RESULTS: We found 12patients (10 females) with an age at diagnosis of 9.4±1.7 years and a follow-up time of 5.5±3.0 years, 10with tumors in the sellar region, and each one with a pineal gland and a bifocal tumor. Clinical changes leading to diagnosis were neurological and/or visual in 9patients and hormonal in three. Seven patients diagnosed on the basis of neurological or visual symptoms had previously reported hormonal changes, giving us a total of 10 children at diagnosis (the most common diagnosis was central diabetes insipidus, found in 8). Endocrine symptoms had been present before diagnosis for 25.0±26.2 months, considerably longer than neuro-ophthalmological complaints (2.0±2.1 months, p = 0.012). CONCLUSIONS: Almost all intracranial germ cell tumors have associated endocrine manifestations at diagnosis, with central diabetes insipidus the most common. Hormonal symptoms usually appear long before neuro-ophthalmological manifestations. Adequate clinical and endocrinological assessment may allow for an earlier diagnosis of these tumors

Correction to: Combined microsurgical fluorescence for optimizing resection in refractory empyema and cerebritis.

The original version of this article unfortunately contained an error. The corresponding author did not notice that one of the co-authors "Javier Márquez Márquez Rivas" was incorrectly presented. The correct name is "Javier Márquez Rivas". Given in this article is the corrected author name.

Combined microsurgical fluorescence for optimizing resection in refractory empyema and cerebritis.

PURPOSE: Due to the generalization of new microsurgical equipment, intraoperative fluorescence techniques have extended in neurosurgical practice, mainly in neurovascular and neuro-oncology patients. The aim of identifying pathological tissue and also differentiating from the normal brain helps neurosurgeons to approach other kinds of intracranial entities such as infections. METHODS: It is described in the case of an 11-year-old patient who underwent a subdural empyema by performing a craniotomy and evacuation of the purulent collection. After a non-optimal evolution, a frontobasal meningoencephalitis was assessed with cerebral involvement and associated intracranial hypertension. Indocyanine green (ICG) was used in reintervention for demonstrating a great damage of cortical vascularization around the infected area as well as fluorescein (FL), which identified a large area of avascularized tissue. RESULTS: Both techniques allowed a selective excision of the affected brain parenchyma while preserving viable parenchymal areas. Radiological evolution and clinical outcome were good. CONCLUSIONS: The identification of vascular patterns in brain lesions and the recognition of viable or necrotized tissues are suitable for a selective resection of the parenchyma, minimizing morbidity. Clinical outcome is related to a safe and effective management of inflammatory and infectious processes.

Neuroendoscopic lavage for the management of posthemorrhagic hydrocephalus in preterm infants: safety, effectivity, and lessons learned.

OBJECTIVE: Treatment of posthemorrhagic hydrocephalus in preterm infants is a matter of debate among pediatric neurosurgeons. Neuroendoscopic lavage (NEL) has been proposed as a suitable technique for the management of this pathology. The authors present their experience with 46 patients treated with NEL after germinal matrix hemorrhage, describe their technique, and analyze the outcomes. METHODS: A retrospective analysis of patients affected by grade III or IV intraventricular hemorrhage (IVH) treated with NEL was performed. Nonmodifiable risk factors such as gestational age, weight at birth, modified Papile grade, and intercurrent diseases were reviewed. Safety analysis was performed, evaluating the incidence of postoperative complications. Effectivity was measured using radiological (frontal horn index and white matter injury), CSF biochemical (proteins, blood, and cellularity), and clinical variables. Permanent shunt rate and shunt survival were analyzed. The motor outcome was measured using the Gross Motor Function Classification System (GMFCS) at 18-24 months, and the neurocognitive outcome was evaluated according to the grade of adaptation to schooling. RESULTS: Forty-six patients met the inclusion criteria. Patients were treated with a single NEL in 21 cases, 2 lavages in 13 cases, and 3 lavages in 12 cases. The mean gestational age at birth was 30.04 weeks, and the mean weight at birth was 1671.86 g. Hyaline membrane disease was described in 4.8% of the cases, hematological sepsis in 43.47%, persistent ductus arteriosus in 23.9%, and necrotizing enterocolitis in 10.9% of the cases. Modified Papile grade III and IV IVHs were observed in 60.9% and 39.1% of the cases, respectively. Postoperative infection was diagnosed in 10 of the 46 cases, CSF leak in 6, and rebleeding in 3. The mean frontal horn index decreased from 49.54 mm to 45.50 mm after NEL. No white matter injury was observed in 18 of 46 patients, focal injury was described in 13 patients, and diffuse bilateral white matter injury was observed in 15 patients. All CSF biochemical levels improved after lavage. The shunt rate was 58.7%, and shunt survival at 1 year was 50%. GMFCS grades I, II, III, IV, and V were observed in 44.74%, 21.05%, 2.63%, 15.79%, and 15.79% of patients, respectively. Good neurocognitive results were observed in 53.3% of the cases. CONCLUSIONS: Neuroendoscopic lavage was a suitable alternative for the management of IVH in preterm infants in our series. Good motor and neurocognitive results were achieved by this technique, and the permanent shunt rate was reduced compared with historical controls.

Endocrine manifestations of central nervous system germ cell tumors in children. / Manifestaciones endocrinas de los tumores germinales del sistema nervioso central en niños.

INTRODUCTION: Central nervous system germ cell tumors need to be adequately diagnosed because their treatment is usually effective and they do not always require surgery. The study objectives are to describe the endocrine manifestations of these tumors and to compare the time of their onset to that of the occurrence of neurological and visual changes. PATIENTS AND METHODS: The medical histories of patients under 14 years of age seen at a pediatric endocrinology unit between 2000 and 2018 were reviewed. Wilcoxon and Fisher statistical tests were performed. RESULTS: We found 12patients (10 females) with an age at diagnosis of 9.4±1.7 years and a follow-up time of 5.5±3.0 years, 10with tumors in the sellar region, and each one with a pineal gland and a bifocal tumor. Clinical changes leading to diagnosis were neurological and/or visual in 9patients and hormonal in three. Seven patients diagnosed on the basis of neurological or visual symptoms had previously reported hormonal changes, giving us a total of 10 children at diagnosis (the most common diagnosis was central diabetes insipidus, found in 8). Endocrine symptoms had been present before diagnosis for 25.0±26.2 months, considerably longer than neuro-ophthalmological complaints (2.0±2.1 months, p=0.012). CONCLUSIONS: Almost all intracranial germ cell tumors have associated endocrine manifestations at diagnosis, with central diabetes insipidus the most common. Hormonal symptoms usually appear long before neuro-ophthalmological manifestations. Adequate clinical and endocrinological assessment may allow for an earlier diagnosis of these tumors.

Fully Endoscopic Transforaminal-Transchoroidal Approach for Tectal Area Tumor Removal.

BACKGROUND: The surgical approaches to lesions located in the tectal area have remained controversial. The essential functions in the surrounding areas and the difficulties in obtaining a good surgical view during tumor removal have made these procedures risky and challenging. Endoscopic transforaminal approaches have been previously described for biopsy and intraventricular tumor removal. However, the endoscopic transforaminal-transchoroidal gross resection technique for such cases has barely been described. METHODS: The endoscopic entry points and trajectories were planned using preoperative magnetic resonance imaging. Once the endoscope was inside the ventricular system, the angles of work and tumor exposure of the upper posterior part of the third ventricle were carefully evaluated. If the angle of work was insufficient for tumor removal, the choroidal fissure was opened using endoscopic bipolar electrode and dissectors. Tumor removal was performed using an endoscopic ultrasonic aspirator. We have presented a 3-case series of patients affected by tectal tumors that were treated using a fully endoscopic transforaminal-transchoroidal approach. RESULTS: Total gross resection of the tumors was achieved in 2 patients. Subtotal resection was achieved in the third patient. No major complications had developed in relationship to the procedure. No new cognitive impairment was reported secondary to this technique. CONCLUSIONS: In our experience, a fully endoscopic transforaminal-transchoroidal approach was a suitable treatment for this complex pathological entity. Opening of the choroidal fissure added an extra angle of work and improved the exposure of the upper posterior part of the third ventricle.

Microvascular Fluorescence in Spinal Cord Repair.

The release of the placode and reconstruction of the myelomeningocele, preserving the maximum neural tissue is a challenge for the neurosurgeon. Vascular fluorescence with indocyanine green and/or fluorescein allows observation of the microvascularization of the spinal cord and adequately identifies viable tissue.

Dynamic Chess-Table Cranial Expansion for Treatment of Craniocerebral Disproportion: Technical Note and Volumetric Results.

OBJECTIVE: Craniocerebral disproportion (CCD) is a challenging disease, and several expansile procedures have been used for its treatment. This report describes the dynamic chess-table cranial expansion technique and reports on 9 patients with primary and secondary CCD treated with this procedure. METHODS: Nine patients affected by CCD were treated with chess-table cranial expansion and reviewed. Symptoms of increased intracranial pressure (ICP) and radiologic findings were analyzed. ICP was monitored using epidural or telemetric sensors. Intracranial volume was measured using computed tomography image processing tools before the surgery, 24 hours after the surgery, and 1 month later. A mathematical model was developed to explain the clinical and surgical results. RESULTS: Five patients had secondary CCD and 4 had primary CCD. The mean age for cranial expansion was 16.78 years. The most frequent symptoms were headaches, nausea/vomiting, and decreased consciousness. Slit ventricles and sutural sclerosis were observed in 33.3% and 55.6% of patients. The mean ICP before the procedure was 48.67 mm Hg. Progressive cranial expansion was seen in all patients after surgery. The mean ICP decreased to 11 mm Hg and mean intracranial volume expansion was 85.8 cm3 at 1 month after surgery. There were no serious complications after surgery. All patients improved their symptoms, and no patient needed additional cranial expansion procedures. CONCLUSIONS: Chess-table cranial expansion is a safe and effective procedure and may be used as an alternative treatment for CCD. Progressive expansion of the intracranial volume is related to a decrease in ICP recordings and an improvement of symptoms.

Long-term reliability of the telemetric Neurovent-P-tel sensor: in vivo case report.

Intracranial pressure (ICP) measurements are imperative for the proper diagnosis and treatment of several neurological disorders. Telemetric sensors have shown their utility for ICP estimation in short-term monitoring in humans. However, their long-term reliability is uncertain. The authors present the case of a 37-year-old woman diagnosed with benign intracranial hypertension and obesity. The patient underwent gastric bypass surgery for ICP control. In order to monitor ICP before and after bariatric surgery, a Neurovent-P-tel sensor was implanted in the left frontal lobe. After gastric bypass, normal ICP values were recorded, and the patient's visual fields improved. However, the patient experienced incapacitating daily headaches. The authors decided to implant a Codman Microsensor ICP transducer in the right frontal lobe to assess the long-term reliability of the Neurovent-P-tel measurements. A comparison of the recordings at 24 and 48 hours showed good correlation and reliability during long-term monitoring with the Neurovent-P-tel, with minimal zero drift after 11 months of implantation.