Treatment of depressed skull fractures with vacuum devices in the neonatal period: A case series.

PURPOSE: The management of depressed skull fractures in infants can be either conservative or surgical. This study aimed to examine the outcomes of management with a negative-pressure vacuum device on depressed skull fractures in newborns. METHODS: Twenty-eight patients (aged 1-6 days) with simple depressed skull fractures underwent skull elevation using negative-pressure vacuum devices. A protocol for nonsurgical management was adopted for infants with such fractures between 2010 and 2023. All patients were initially evaluated with neurological examination and complementary assessments-hematological and coagulation studies, transfontanel transcranial ultrasound, skull radiography, and computed tomography scanning with three-dimensional reconstruction-according to availability and clinical needs. Gentle (negative) extraction pressure was applied with one of several devices (according to institutional availability) for a maximum duration of 60 s; this was performed as soon as possible after diagnosis, preferably within 72 h. Follow-up data, available in the clinical records, were reported. RESULTS: All patients exhibited satisfactory elevation of the depressed bone without associated injuries, except one patient who presented with an associated cephalohematoma which prevented optimal device coupling to generate sufficient vacuum pressure for correction. Neither neurological deficits nor development of epilepsy was noted; normal neurological assessment and oral alimentation tolerance were confirmed within 24 h post procedure. CONCLUSIONS: According to our data, ping-pong skull fracture elevation using the vacuum method is a safe and satisfactory treatment in the neonatal period. Early treatment allows for quick resolution, and in our opinion is the strategy of choice for depressed skull fractures in newborns.

SIOP PODC-adapted treatment guidelines for craniopharyngioma in low- and middle-income settings.

Pediatric craniopharyngioma is a rare tumor with excellent survival but significant long-term morbidities due to the loco-regional tumor growth or secondary to its treatment. Visual impairment, panhypopituitarism, hypothalamic damage, and behavioral changes are among the main challenges. This tumor should be managed under the care of a multidisciplinary team to determine the optimum treatment within the available resources. This is particularly important for low middle-income countries where resources are variable. This report provides risk-stratified management guidelines for children diagnosed with craniopharyngioma in a resource-limited setting.

Posterior C1-C2 fusion for atlantoaxial rotatory fixation after posterior fossa craniotomy in a 4-year old: a case report.

INTRODUCTION: This study aimed to highlight that atlantoaxial rotatory fixation (AARF) can be related to neurosurgery procedures in children, with an afterward demonstration of good results after halo-gravity traction and C1-C2 stabilization using the Harms technique. CASE DESCRIPTION: To the best of our knowledge, this is the first case to report a 4-year-old boy who presented with AARF after a posterior fossa craniotomy to treat a cerebellar astrocytoma. At our medical facility, AARF was diagnosed using plain radiography and computed tomography imaging. The patient was treated with continuous cranial traction for 14 days. Initially, we detected that the patient had no C1 posterior arch or C2 spinous process; therefore, the best option was to perform the Harms technique. Postoperatively, the patient was placed in a cervical collar for 4 weeks. At the 4-year postoperative follow-up, the patient was doing well and had not developed any complications. CONCLUSION: Herein, we report a case in which AARF can be developed after neurosurgical procedure. Surgical techniques used for atlantoaxial subluxation should be carefully selected. In our case, the Harms technique after cranial traction was an excellent option for correcting and stabilizing the abnormal neck position. However, further studies are required to determine the best technique to use in the pediatric population.

Diencephalic syndrome-like presentation of brainstem tumor: a series case-based review.

PURPOSE: Russel described a rare clinical entity known as diencephalic syndrome (DS) in 1951, which was traditionally caused by a neoplasm in the hypothalamic-optic chiasmatic region. DS is characterized by severe emaciation despite adequate or slightly reduced caloric intake, locomotor hyperactivity, euphoria and other minor features. Current evidence suggests that a rare population of children with a similar phenotype may have their tumor located in the posterior fossa instead, defining the DS-like presentation, a rare entity with few cases reported in the literature. METHODS: A thorough search of three databases (PubMed, Ovid Medline, and Ovid Embase) was conducted to identify relevant papers reporting children with DS associated with brainstem tumors. To our knowledge, only seven cases have been documented in the literature. Moreover, we present four of our own cases, focusing on the unusual clinical presentation, the diagnosis process, and the lag time between the initial symptoms and the definitive diagnosis. RESULTS: In this review, the mean lag time between the onset of symptoms and diagnosis was 20.9 months (median: 16 months; range: 1.5-72 months), whereas in our series of cases, the time was 32.5 months (median: 33 months; range: 7-57 months). CONCLUSION: Despite recent significant advances in neuro-oncology diagnostic tools, this mean lag time did not improve when compared with the previous literature review from 1976. Throughout these data, we aim to raise awareness in the hopes of detecting intracranial neoplasms earlier in cases of children with profound emaciation of unknown cause.

Mansonic neuroschistosomiasis in the childhood: an undiagnosed pathology?

Schistosomiasis is an endemic parasitic disease in several tropical countries. In Brazil, the only prevalent species of parasite responsible for schistosomiasis is Schistosoma mansoni. Neuroschistosomiasis is the second most frequent form of infection and the primary ectopic manifestation, with predominant involvement of the lower thoracic spinal cord and lumbar and lumbosacral regions. The frequent contact of children with contaminated ponds and the immaturity of their immune systems make this age group especially susceptible to infection by this parasite. Therefore, neuroschistosomiasis mansoni should always be considered in cases of transverse myelitis in children from endemic regions. The treatment for this condition is quite simple and effective, resulting in total recovery of neurological deficits if the diagnosis is made early.

Prognostic factors of pediatric pineal region tumors at a single institution.

PURPOSE: This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period. MATERIAL AND METHODS: Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types. RESULTS: Germinoma was found in 33.1%, with an overall 60-month survival rate of 88%; the female sex was the only factor of a worse prognosis. Non-germinomatous germ cell tumors were found in 27.1%, with an overall 60-month survival rate of 67.2%; metastasis upon diagnosis, residual tumor, and the absence of radiotherapy were associated with a worse diagnosis. Pineoblastoma was found in 22.5%, with an overall 60-month survival rate of 40.7%; the male sex was the only factor of a worse prognosis; a tendency toward a worse outcome was found in patients < 3 years of age and those with metastasis upon diagnosis. Glioma was identified in 12.5%, with an overall 60-month survival rate of 72.6%; high-grade gliomas were associated with a worse prognosis. Atypical teratoid rhabdoid tumors was found in 3.3%, and all patients died within a 19-month period. CONCLUSION: Pineal region tumors are characterized by the heterogeneity of histological types, which exert an influence on the outcome. Knowledge of the prognostic factors for each histological types is of extreme importance to the determination of guided multidisciplinary treatment.

Intrinsic epidermoid cyst of the brainstem in children-review and case report.

PURPOSE: The study aimed to summarize all published cases of intrinsic brainstem epidermoid cysts in a timeline to highlight the specific characteristics and individualize the disease, in addition to discussing the best treatment used. METHODS: The scientific literature on pediatric cases of intrinsic epidermoid cysts of the brainstem was analyzed. We present the case of a 1.5-year-old male with incidental presentation, who was treated with gross total resection. We summarize all previously published cases to individualize the disease. RESULTS: We identified 21 patients, including 10 boys and 11 girls, with a mean age of 4.85 (1-15) years at the time of surgery. The most frequent symptoms were cranial nerve palsy (71.4%), pyramidal tract deficit (57.14%), and headache (52.38%). Among the affected cranial nerves, VII was the most frequently reported in 10 patients. CONCLUSION: Brainstem epidermoid cysts are extremely rare pathologies with relevant age involvement in young children. The treatment objective should be the maximum resection of the lesion through a careful approach and with the appropriate tools for the functional preservation of the patient.

Fluorescein-guided resection for pediatric low-grade gliomas: institutional experience on two cases and a narrative literature review.

PURPOSE: Low-grade gliomas compose 30% of pediatric central nervous system tumors and outcomes of disease-free progression, and survival is directly correlated to the extent of resection. The use of sodium fluorescein (Na-Fl) is an intraoperative method in the localization of tumor cells in adult patients to optimize resection. Our purpose is to describe the use of Na-Fl in pediatric low-grade gliomas and its outcomes. METHODS: Patients under 18 years of age with low-grade gliomas at the author's institution underwent resection with the use of Na-Fl, with review of preoperative imaging findings, intraoperative results, and follow-up. Then, a comprehensive, narrative literature review of the use of Na-Fl in pediatric low-grade glioma was performed. RESULTS: Our single-institution use of Na-Fl in pediatric patients with suspected low-grade glioma demonstrated excellent results of intraoperative enhancement of tumor cells as well as gross total resection. The literature demonstrated 84% Na-Fl staining and 59.2% of gross total resection in pediatric low-grade gliomas with few small case studies, a range of reported findings, and few side effects. CONCLUSION: Na-Fl has a promising use in low-grade glioma resection in the pediatric patient population. Further research is warranted, such as randomized controlled studies, to assess Na-Fl as a potential tool in improving resection and long-term favorable outcomes.

Ultrasound evaluation of uterine scar thickness after open fetal surgery for myelomeningocele.

PURPOSE: This study aimed to analyse the evolution of uterine scar thickness after open fetal surgery for myelomeningocele (MMC) by ultrasonography, and to establish a cut-off point for uterine scar thickness associated with high-risk of uterine rupture. METHODS: A prospective longitudinal study was conducted with 77 pregnant women who underwent open fetal surgery for MMC between 24 and 27 weeks of gestation. After fetal surgery, ultrasound follow-up was performed once a week, and the scar on the uterine wall was evaluated and its thickness was measured by transabdominal ultrasound. At least five measurements of the uterine scar thickness were performed during pregnancy. A receiver operating characteristics (ROC) curve was constructed to obtain a cut-off point for the thickness of the scar capable of detecting the absence of thinning. Kaplan-Meier curves were constructed to evaluate the probability of thinning during pregnancy follow-up. RESULTS: The mean ± standard deviation of maternal age (years), gestational age at surgery (weeks), gestational age at delivery (weeks), and birth weight (g) were 30.6 ± 4.5, 26.1 ± 0.8, 34.3 ± 1.2 and 2287.4 ± 334.4, respectively. Thinning was observed in 23 patients (29.9%). Pregnant women with no thinning had an average of 17.1 ± 5.2 min longer surgery time than pregnant women with thinning. A decrease of 1.0 mm in the thickness of the uterine scar was associated with an increased likelihood of thinning by 1.81-fold (95% confidence interval [CI]: 1.32-2.47; p < 0.001). The area below the ROC curve was 0.899 (95% CI: 0.806-0.954; p < 0.001), and the cut-off point was ≤ 3.0 mm, which simultaneously presented greater sensitivity and specificity. After 63 days of surgery, the probability of uterine scarring was 50% (95% CI: 58-69). CONCLUSION: A cut-off point of ≤ 3.0 mm in the thickness of the uterine scar after open fetal surgery for MMC may be used during ultrasonography monitoring for decision-making regarding the risk of uterine rupture and indication of caesarean section.

Risk for hydrocephalus, hygroma, and tumor dissemination after ventricular opening during resection of supratentorial neoplasms in children.

PURPOSE: The possibility that ventricular opening generates postoperative complications after surgical tumor treatment often restricts the degree of tumor resection. This study aims to determine whether the ventricular opening is associated with more complications in surgeries for resectioning supratentorial intra-axial brain tumors in the pediatric population. METHODS: A retrospective review analysis was performed of patients treated at IOP/GRAACC between 2002 and 2020 under 19 years of age and underwent surgery for supratentorial intra-axial primary brain tumor resection. Data were collected from 43 patients. RESULTS: Glial tumor was more common than non-glial (65% vs. 35%, p = 0.09). The ventricular opening was not related to neoplastic spreads to the neuroaxis (6% vs. 0, p > 0.9) or leptomeningeal (3% vs. 0, p > 0.9). Of the patients whose ventricle was opened, 10% developed hydrocephalus requiring treatment, while none of the patients in the group without ventricular opening developed hydrocephalus (p = 0.5). There was also no statistical difference regarding ventriculitis. Postoperative subdural hygroma formation correlated with the ventricular opening (43% vs. 0, p = 0.003). The survival at 1, 5, and 10 years of cases with the ventricular opening was 93.2%, 89.7%, and 75.7%, respectively, while in cases without ventricular opening, it was 100%, 83%, and 83%, respectively, respectively, with no statistical difference between the mortality curves. CONCLUSION: Our study demonstrated that ventricular violation was not associated with the occurrence of significant complications. It was related to the formation of subdural hygroma, which did not require additional treatment.

Training model for the fetal myelomeningocele correction with multiportal endoscopic technique.

PURPOSE: The recent history of myelomeningocele has shown that treatment during the fetal life may reduce the risk of developing hydrocephalus in individuals by approximately 50%. Thus, a significant advancement involves fetal surgery performed through an endoscopic technique in which portals are placed to introduce the forceps and laparoscopic instruments. However, the development of this technique requires training; therefore, this study aimed to develop a training model for fetal myelomeningocele repair technique with multi-portal endoscopy. METHODS: Two stages of endoscopic technique development were performed. The first stage consisted of exercises in order to familiarize the surgeon with 2D-vision endoscopic surgery, associated with the application of exercises focused on surgical skills, such as the development of laparoscopic knots in a synthetic model. The second stage involved the creation and application of the stages of myelomeningocele closure with a non-living animal model consisting of a chicken breast to simulate the myelomeningocele and a basketball to simulate the gravid uterus, in which perforations were made to introduce vascular introducers (portals) that, as in vivo, are used as portals (trocars) for the introduction of laparoscopic instruments. Overall, two different scenarios with three portals and two portals were tested. RESULTS: In three-portal simulator, the triangular apex trocar was used for the introduction of 4-mm 0° or 30° optics or even Minop type neurodoscope (Aesculap®, Germany) that was operated by the assistant surgeon; the other two portals are used for the introduction of laparoscopic instruments. Thus, the surgeon is able to perform maneuvers bimanually since dissection to laparoscopic sutures. In two-portal simulator, the surgeon and assistant stay side by side and one of the portals is used for the optic and the other for the laparoscopic instruments. There is no possibility of bimanual dissection in this method. CONCLUSION: Realistic simulation models for endoscopic fetal surgery for myelomeningocele correction are easily performed and help develop the necessary skills for fetal surgery teams.

Fetal neurosurgery.

Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal period. To perform fetal neurosurgical procedures, there is a need for specialized centers that have experts in the diagnosis of fetal pathologies and a highly complex obstetrics service with specialized maternal-fetal teams associated with a pediatric neurosurgery center with expertise in the diverse pathologies of the fetus and the central nervous system that offers multidisciplinary follow-up during postnatal life. Services that do not have these characteristics should refer their patients to these centers to obtain better treatment results. It is essential that the fetal neurosurgical procedure be performed by a pediatric neurosurgeon with extensive experience, as he will be responsible for monitoring these patients in the postnatal period and for several years. The objective of this manuscript is to demonstrate the diagnostic and treatment possibilities, in the fetal period, of some neurosurgical diseases such as hydrocephalus, tumors, occipital encephalocele, and myelomeningocele.

Assessment of lower limb trophism in fetuses with open spina bifida using fractional thigh volume of three-dimensional ultrasound.

OBJECTIVE: To assess the trophism of the lower limbs of fetuses with open spina bifida using fractional thigh volume (TVOL) of three-dimensional (3D) ultrasound. METHODS: A prospective cross-sectional study was carried out with normal fetuses and with open spina bifida (myelomeningocele and rachischisis) at 26 weeks' gestation. The TVOL (delimitation of five cross-sectional areas of the middle portion of the limb) was evaluated, as well as the subjective assessment of hypotrophy and lower limb movement. RESULTS: Thirty-one fetuses with open spina bifida, 21 with myelomeningocele and 10 with rachischisis, and 51 normal fetuses were included. There were no significant differences in the TVOL between normal and spina bifida fetuses (p = 0.623), as well as between normal fetuses, with myelomeningocele and with rachischisis (p = 0.148). There was no significant difference in the TVOL of fetuses with spina bifida with or without lower limb hypotrophy (p = 0.148). Fetuses with spina bifida and with lower limb movement had higher TVOL values than fetuses without lower limb movement (p = 0.002). CONCLUSION: There were no significant differences in the TVOL measurement of normal and spina bifida fetuses (rachischisis and myelomeningocele). Fetuses with spina bifida without spontaneous movement of the lower limbs had lower TVOL values.

Reduction in the cerebrospinal fluid protein level after bevacizumab treatment in patients with optic pathway low-grade gliomas.

Optic pathway gliomas (OPG) can cause elevated cerebrospinal fluid (CSF) protein concentrations. We report on two patients with suprasellar low-grade gliomas and high CSF protein levels (590 and 551 mg/dl) that precluded shunt implantation. After two and three doses of bevacizumab, respectively, the levels dropped dramatically to 191 and 178 mg/dl, respectively. Bevacizumab treatment was associated with a decrease in CSF protein level, allowing successful shunt placement. Our results are consistent with the pharmacological mechanism of bevacizumab, which decreases protein leakage from blood vessels to the ventricles.

Measurement of the Area and Circumference of the Leg: Preliminary Results of a New Method for Estimating Leg Muscle Trophism in Fetuses With Open Lumbosacral Spina Bifida.

OBJECTIVE: To standardize a new technique to evaluate the fetal leg muscle trophism by measuring the area and circumference, and comparing this technique between normal and fetuses with open lumbosacral spina bifida (SB). METHODS: Observational cross-sectional study was carried out on pregnant women with 26 weeks who were divided into two groups: Group I-fetuses with diagnosis of open lumbosacral SB; Group II-normal fetuses (control). In fetuses with SB, subjective evaluation of the lower limbs was performed (muscle echogenicity and leg movements). To estimate the leg muscle trophism, the measurement of the area and circumference of the leg were standardized, and the reproducibility of this method was performed. RESULTS: Thirty-one fetuses with open lumbosacral SB and 51 normal fetuses were evaluated. The measurements of the area and circumference of the leg proved to be highly reproducible (intraclass correlation coefficient-ICC > 0.95). The leg area and circumference measurements were statistically lower in the SB group than in the control group (p < .001). When subjective ultrasound assessment demonstrated hypotrophy of the lower limbs, the measurements of the area and circumference of the leg were statistically lower when compared to normal fetuses (p < .001). Fetuses with open SB with abnormal lower limb movements had lower measurements of the area and circumference of the leg than fetuses with normal movements (p < .001). CONCLUSION: A new technique for estimating fetal leg muscle trophism was standardized, which proved to be highly reproducible and was able to show the difference between normal and fetuses with SB.

In utero myelomeningocele repair and high-risk bladder pattern. a prospective study.

OBJECTIVES: High-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup. PATIENTS AND METHODS: From our prospective cohort of in utero MMC we have identified patients in the high-risk group. Treatment consisted of anticholinergics (Oxybutynin 0.2 mg/Kg) 2 or 3 times daily in association with CIC. At every UE, patients were reclassified in high-risk or low-risk patterns. Patients not responding were proposed bladder reconstruction or diversion according to age. RESULTS: Between 2011 to 2020, we have been following 121 patients and 60 (49.6%) of them were initially categorized as high-risk. The initial UE was performed at a mean age of 7.9 months and detrusor overactivity was found in 83.3% (mean maximum pressure of 76.5cmH20). When evaluating patients with 2 or more UE, we identified 44 patients (follow-up: 36.8months).  It was observed in the group of patients who underwent 2 to 5 UE, that response to treatment was validated by the finding of 40% of low-risk bladder patterns in the second UE and between 62% to 64% in the third to the fifth UE. The incidence of surgery was 13.3%. CONCLUSIONS: Early urological treatment of high-risk bladder pattern was effective in approximately 60%. We reinforce the need to correctly treat every patient with myelomeningocele, in accordance with UE, whether undergoing in utero or postnatal treatment.

Molecular profiling of pediatric and adolescent ependymomas: identification of genetic variants using a next-generation sequencing panel.

PURPOSE: Ependymoma (EPN) accounts for approximately 10% of all primary central nervous system (CNS) tumors in children and in most cases, chemotherapy is ineffective and treatment remains challenging. We investigated molecular alterations, with a potential prognostic marker and therapeutic target in EPNs of childhood and adolescence, using a next-generation sequencing (NGS) panel specific for pediatric neoplasms. METHODS: We selected 61 samples with initial diagnosis of EPN from patients treated at Pediatric Oncology Institute-GRAACC/UNIFESP. All samples were divided according to the anatomical compartment of the CNS - 42 posterior fossa (PF), 14 supratentorial (ST), and five spinal (SP). NGS was performed to identify somatic genetic variants in tumor samples using the Oncomine Childhood Cancer Research Assay® (OCCRA®) panel, from Thermo Fisher Scientific®. RESULTS: Genetic variants were identified in 24 of 61 (39.3%) tumors and over 90% of all variants were pathogenic or likely pathogenic. The most commonly variants detected were in CIC, ASXL1, and JAK2 genes and have not been reported in EPN yet. MN1-BEND2 fusion, alteration recently described in a new CNS tumor type, was identified in one ST sample that was reclassified as astroblastoma. Additionally, YAP1-MAMLD1 fusion, a rare event associated with good outcome in ST-EPN, was observed in two patients diagnosed under 2 years old. CONCLUSIONS: Molecular profiling by the OCCRA® panel showed novel alterations in pediatric and adolescent EPNs, which highlights the clinical importance in identifying genetic variants for patients' prognosis and therapeutic orientation.

Validated quantitative needs assessment differences in the management of children with central nervous system cancer between Brazil, an upper middle-income country, and the United States of America, a high income country.

BACKGROUND: Pediatric cancer cure rates differ among high-income countries (HIC) and upper middle-income countries (UMIC). We have compared individual capacities of two major referral pediatric centers from a HIC and an UMIC caring for children with central nervous system (CNS) cancer. METHODS: A quantitative needs assessment questionnaire and key informant interviews, distributed in March of 2017, were used to evaluate the treatment of children with CNS cancer at Grupo de Apoio ao Adolescente e à Criança com Câncer (GRAACC) children's cancer center in São Paulo, Brazil and Nationwide Children's Hospital (NCH) in Columbus, Ohio, United States of America (USA). RESULTS: Both hospitals had 24-hour pediatric oncology, nursing and intensivist coverage. Supportive care available at both institutions included social workers, psychologists, child life specialists, and physical/occupational/speech therapists. Differences included two part-time neuroradiologists and one pathologist specializing in neuropathology at IOP/GRAACC/UNIFESP, whereas eight full-time neuroradiologists and two neuropathologists at NCH/OSU. There were four pediatric neurosurgeons on staff at each hospital; however, there were only 2 operative days per week at IOP/GRAACC/UNIFESP, compared with 7 days at NCH/OSU. Additionally, time to initiation of radiation therapy at IOP/GRAACC/UNIFESP extended 2-4 weeks compared with less than 1 week at NCH/OSU. CONCLUSIONS: Center-specific differences in resources exist in highly specialized hospitals caring for children with CNS cancer in HIC and UMIC. This quantitative needs assessment may facilitate the development of targeted strategies for effective interventions to improve on the management of children with CNS cancers.

Everolimus as a possible prenatal treatment of in utero diagnosed subependymal lesions in tuberous sclerosis complex: a case report.

INTRODUCTION: The association between cardiac rhabdomyoma and intraventricular tumors and/or subcortical nodules is characteristic of tuberous sclerosis complex (TSC). Patients with TSC may have refractory seizures, autistic behavior, and cognitive decline. CASE REPORT: The patient received the fetal diagnosis of TSC at the age of 19 weeks of gestations, where presented at prenatal ultrasound cardiac and brain tumors. Fetal MRI showed a lesion in the right and left lateral ventricles near the foramen of Monro associated with subependymal lesions along the entire ependyma of the lateral ventricles and several subcortical tubercles, and the fetal Doppler echocardiogram revealed three cardiac lesions. The fetus underwent intrauterine treatment with everolimus and presented regression and subsequent stabilization of the cardiac and brain lesions; additionally, the patient did not develop seizures or autism and presented good neuropsychomotor development. CONCLUSION: It is the first evidence that mTOR inhibitors may help to prevent neurological complications associated with TSC.

Hydrocephalus in myelomeningocele.

PURPOSE: To investigate certain aspects of hydrocephalus in patients with myelomeningocele. METHODS: We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6 h of life, group 2 consisted of patients who underwent surgery within the first 6 h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7 weeks of gestation. RESULTS: There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4 days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus. CONCLUSION: The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.