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Objective: This study reports the infant to preschool outcomes of a laparotomy-assisted, two-port fetoscopic myelomeningocele (MMC) repair and compares the results with those of a contemporary, same-center cohort that underwent either fetal MMC surgery via hysterotomy or postnatal MMC repair., Methods: All MMC closures between December 2011 and July 2021 were screened. Singleton pregnancies with hindbrain herniation and MMC between T1 and S1 were included. Fetuses were excluded for genetic abnormalities, severe kyphosis, and other congenital anomalies. The pregnant woman determined the method of MMC repair (fetoscopic, hysterotomy, or postnatal repair)., Results: Two hundred MMC closures met the study criteria (100 fetoscopic, 41 hysterotomy, and 59 postnatal). The median length of follow-up was beyond 46 months for all groups. The median gestational age at delivery was 38.1 weeks (IQR 35.1, 39.1 weeks) for the fetoscopic group, 35.7 weeks (IQR 33.6, 37.0 weeks) for the hysterotomy group, and 38.6 weeks (IQR 37.7, 39.0 weeks) for the postnatal group. Vaginal delivery occurred in 51% of the fetoscopic cases, and there were no instances of uterine dehiscence or rupture. Treatment for hydrocephalus in the 1st year occurred in 35% (95% CI 27%-50%) of fetoscopic, 33% (95% CI 20%-50%) of hysterotomy, and 81% (95% CI 70%-90%) of postnatal repair cases. At 30 months, patients who underwent fetal intervention were twice as likely to be community ambulators (with or without devices) as those who underwent postnatal repair (52% [95% CI 42%-62%] of fetoscopic, 54% [95% CI 39%-68%] of hysterotomy, and 24% [95% CI 14%-36%] of postnatal cases). Surgery for symptomatic tethered cord occurred in 12% (95% CI 7%-19%) of fetoscopic, 17% (95% CI 8%-31%) of hysterotomy, and 2% (95% CI 1%-8%) of postnatal repair cases. Surgery for symptomatic spinal inclusion cysts was required in 4% (95% CI 1%-9%) of fetoscopic, 7% (95% CI 2%-18%) of hysterotomy, and none (95% CI 0%-8%) of the postnatal cases., Conclusions: Laparotomy-assisted, two-port fetoscopic repair provides significant benefits for maternal health. It negates the risk of uterine rupture for the index pregnancy and subsequent pregnancies and allows for vaginal delivery. The benefits to the fetus are the same as those of hysterotomy repairs, with a lower risk of prematurity. There was no difference in the rate of surgery for tethered cord or spinal inclusion cysts between fetoscopic and hysterotomy procedures. Overall, laparotomy-assisted, two-port fetoscopic repair is safer for the fetus and the mother than fetal MMC surgery via hysterotomy.

Objective: Patients who experience postoperative pediatric cerebellar mutism syndrome (CMS) during treatment for medulloblastoma have long-term deficits in neurocognitive functioning; however, the consequences on functional or adaptive outcomes are unknown. The purpose of the present study was to compare adaptive, behavioral, and emotional functioning between survivors with and those without a history of CMS., Methods: The authors examined outcomes in 45 survivors (15 with CMS and 30 without CMS). Comprehensive neuropsychological evaluations, which included parent-report measures of adaptive, behavioral, and emotional functioning, were completed at a median of 2.90 years following craniospinal irradiation., Results: Adaptive functioning was significantly worse in the CMS group for practical and general adaptive skills compared with the group without CMS. Rates of impairment in practical, conceptual, and general adaptive skills in the CMS group exceeded expected rates in the general population. Despite having lower overall intellectual functioning, working memory, and processing speed, IQ and related cognitive processes were uncorrelated with adaptive outcomes in the CMS group. No significant group differences or increased rates of impairment were observed for behavioral and emotional outcomes., Conclusions: Survivors with CMS, compared with those without CMS, are rated as having significant deficits in overall or general adaptive functioning, with specific weakness in practical skills several years posttreatment. Findings from this study demonstrate the high risk for ongoing functional deficits despite acute recovery from symptoms of CMS, highlighting the need for intervention to mitigate such risk.

Objective: The role of surgery in recurrent ependymomas and its contribution to the outcome are not well defined. While gross-total resection (GTR) has shown benefit in newly diagnosed patients with improvement in progression-free survival (PFS), its impact after recurrence is not known. Its role in distant relapses or multiple local recurrences is similarly less well understood. The objective of this study was to investigate whether GTR could prolong survival after recurrence., Methods: In this paper, the authors identified patients with ependymomas who underwent surgery at Texas Children's Hospital for recurrent ependymomas between December 2000 and December 2021. Surgical treatment was stratified as GTR, subtotal resection (STR), or a biopsy. Kaplan-Meier analysis was performed for PFS and overall survival (OS), and the log-rank test was used to assess statistical significance. The Cox regression model was used for multivariable analysis., Results: Forty children were identified with a first ependymoma recurrence and follow-up data were collected. The median age was 5.46 years (95% CI 4.52-6.39 years) with a mean follow-up of 3.92 years (95% CI 2.42-5.42 years). In 26 patients (65%), the original tumor was located in the infratentorial space. Twenty-nine patients (72.5%) presented with local recurrence. Within this group, the 5-year PFS rates for the GTR and STR groups were 40.1% and 26.8%, respectively. The 5- and 10-year OS rates were 58.3% and 50% in the GTR group and 51% and 16.7% in the STR group, respectively. Fifteen patients presented with a second recurrence. The 5-year PFS and OS rates in patients who had GTR after a second recurrence were 33% and 50%, respectively., Conclusions: GTR of local recurrent ependymomas can result in long-term survival in first and second recurrences. Further and larger studies are necessary to elucidate the role of surgery in distal recurrences.

Objective: The optimal management of pediatric craniopharyngioma patients remains controversial, shifting from radical resection (gross-total resection [GTR]) to a more conservative approach with partial resection/biopsy followed by radiotherapy (PR+RT). To the authors' knowledge, no previous studies have compared neurocognitive and quality-of-life (QOL) outcomes between the two main treatments. In this study, the authors compared changes in intellectual, adaptive, and QOL scores in children treated for craniopharyngioma with GTR and those treated with PR+RT., Methods: Patients underwent annual neurocognitive and QOL evaluations for up to 10 years posttreatment, including the Full-Scale IQ, Verbal Comprehension Index (VCI), Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI). Child- and parent-reported QOL scores and adaptive behavior in different domains were assessed. General linear mixed models were used to examine change in scores over time by treatment group with adjustment for significant covariates., Results: Scores from 43 patients treated between 2009 and 2019 (21 GTR, 22 PR+RT) were examined. Within the PR+RT group, 9 patients had intensity-modulated RT and 13 had proton beam therapy. The treatment groups were similar in sex (44% male) and age (median 7.3 years). There were no significant differences in the trajectory of intellectual functioning or QOL scale scores between the two groups. However, patients who underwent GTR exhibited significant improvement over time in overall adaptive behavior (p = 0.04) and conceptual skills (p = 0.01), which was not observed in patients treated with PR+RT., Conclusions: Long-term pediatric craniopharyngioma survivors treated with GTR and PR+RT have similar intellectual function and QOL. Larger studies are needed to explore small but clinically significant differences between the two groups.

Objective: In utero repair of fetal posterior cephaloceles (meningocele and encephalocele) is being performed based on the premise that fetal surgery prevents progressive herniation of neural tissue and brain damage during pregnancy. However, the extent to which progressive herniation occurs during pregnancy, specifically from prenatal diagnosis to after delivery, is not well known. The objective of this study was to describe the natural history of patients with fetal cephaloceles focusing on the incidence of progressive herniation., Methods: The authors conducted a retrospective cohort study of all patients referred to their center for posterior fetal cephalocele between 2006 and 2021. All patients underwent prenatal and postnatal MRI. Progressive herniation (primary outcome) was defined as an increase in the absolute volume of neural tissue within the cephalocele of > 5% or new herniation of a critical structure into the cephalocele. Total brain and cephalocele volumes were calculated to determine herniation progression from prenatal to postnatal MRI. Information on the presence of hydrocephalus, epilepsy, and developmental delay (secondary outcomes) was collected at 1 year of age., Results: Twenty patients met all study criteria. Ten patients (50%; 95% CI 0.27-0.73) demonstrated progressive herniation from prenatal to postnatal MRI. Three patients with progressive herniation were diagnosed with a meningocele prenatally and had an encephalocele postnatally. Two patients without progression had meningocele identified prenatally that regressed and became atretic by birth. Both prenatal hindbrain herniation (p = 0.03) and prenatal microcephaly (p = 0.05) were predictive of progressive herniation. The rates of hydrocephalus (44%), epilepsy (44%), and developmental delay (63%) were not associated with the occurrence of progressive herniation in this study., Conclusions: In this study, progressive herniation was not a rare event (50%). Fetal hindbrain herniation and fetal microcephaly were associated with the occurrence of progressive herniation. These results support further investigations into why progressive herniation occurs in utero and if progressive cerebral herniation in utero plays a significant role in determining clinical outcome.

Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing surgical morbidity. In this video, the authors show an interhemispheric transcallosal approach to a pineal region tumor in a 15-year-old boy. The advantage of this corridor over posterior approaches is that it provides a nice view of the tumor plane with the venous complex, especially while dissecting tumor from the anterior aspect of the internal cerebral veins on their vertical path. Thus, this approach represents a safe and effective alternative for selected pineal tumors. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2120., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2021, The Authors.)

Objective: The authors compared survival and multiple comorbidities in children diagnosed with craniopharyngioma who underwent gross-total resection (GTR) versus subtotal resection (STR) with radiation therapy (RT), either intensity-modulated radiation therapy (IMRT) or proton beam therapy (PBT). The authors hypothesized that there are differences between multimodal treatment methods with respect to morbidity and progression-free survival (PFS)., Methods: The medical records of children diagnosed with craniopharyngioma and treated surgically between February 1997 and December 2018 at Texas Children's Hospital were reviewed. Surgical treatment was stratified as GTR or STR + RT. RT was further stratified as PBT or IMRT; PBT was stratified as STR + PBT versus cyst decompression (CD) + PBT. The authors used Kaplan-Meier analysis to compare PFS and overall survival, and chi-square analysis to compare rates for hypopituitarism, vision loss, and hypothalamic obesity (HyOb)., Results: Sixty-three children were included in the analysis; 49% were female. The mean age was 8.16 years (95% CI 7.08-9.27). Twelve of 14 children in the IMRT cohort underwent CD. The 5-year PFS rates were as follows: 73% for GTR (n = 31), 54% for IMRT (n = 14), 100% for STR + PBT (n = 7), and 77% for CD + PBT (n = 11; p = 0.202). The overall survival rates were similar in all groups. Rates of hypopituitarism (96% GTR vs 75% IMRT vs 100% STR + PBT, 50% CD + PBT; p = 0.023) and diabetes insipidus (DI) (90% GTR vs 61% IMRT vs 85% STR + PBT, 20% CD + PBT; p = 0.004) were significantly higher in the GTR group. There was no significant difference in the HyOb or vision loss at the end of study follow-up among the different groups. Within the PBT group, 2 patients presented a progressive vasculopathy with subsequent strokes. One patient experienced a PBT-induced tumor., Conclusions: GTR and CD + PBT presented similar rates of 5-year PFS. Hypopituitarism and DI rates were higher with GTR, but the rate of HyOb was similar among different treatment modalities. PBT may reduce the burden of hypopituitarism and DI, although radiation carries a risk of potential serious complications, including progressive vasculopathy and secondary malignancy. Further prospective study comparing neurocognitive outcomes is necessary.

Texas Children's Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children's Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.

Craniopharyngioma represents one of the most challenging brain tumors to treat. Surgery may be the definitive treatment, and multiple surgical approaches have been described, each based on different anatomical considerations. Some approaches require working through narrow corridors that do not often provide a sufficient view of the critical anatomical structures around the tumor. The choice of the right approach is key for optimizing resection and minimizing risk. In this paper, the author presents the case of a pediatric patient with a large suprasellar craniopharyngioma who underwent complete resection of the tumor through a novel approach: a transcallosal translamina terminalis corridor. This particular transcallosal corridor, behind the anterior communicating artery, allows the optimal opening of the lamina terminalis extending up to the anterior commissure. This novel variation of the traditional operation provides a wider exposure of the tumor compared with the classic approaches through the lamina terminalis. This technique has not been sufficiently described in the pediatric literature. The author describes it here as an alternative method for treating patients with sellar and suprasellar tumors.

Objective: Resection of brain tumors may lead to new-onset seizures but may also reduce seizure rates in patients presenting with seizures. Seizures are seen at presentation in about 24% of patients with brain tumors. For lesional epilepsy in general, early resection is associated with improved seizure control. However, the literature is limited regarding the occurrence of new-onset postoperative seizures, or rates of seizure control in those presenting with seizures, following resections of extratemporal low-grade gliomas (LGGs) in children., Methods: Data were collected retrospectively from 4 large tertiary centers for children (< 18 years of age) who underwent resection of a supratentorial extratemporal (STET) LGG. The patients were divided into 4 groups based on preoperative seizure history: no seizures, up to 2 seizures, more than 2 seizures, and uncontrolled or refractory epilepsy. The authors analyzed the postoperative occurrence of seizures and the need for antiepileptic drugs (AEDs) over time for the various subgroups., Results: The study included 98 children. Thirty patients had no preoperative seizures, 18 had up to 2, 16 had more than 2, and 34 had refractory or uncontrolled epilepsy. The risk for future seizures was higher if the patient had seizures within 1 month of surgery. The risk for new-onset seizures among patients with no seizures prior to surgery was low. The rate of seizures decreased over time for children with uncontrolled or refractory seizures. The need for AEDs was higher in the more active preoperative seizure groups; however, it decreased with time., Conclusions: The resection of STET LGGs in children is associated with a low rate of postoperative new-onset epilepsy. For children with preoperative seizures, even with uncontrolled epilepsy, most have a significant improvement in the seizure activity, and many may be weaned off their AEDs.

Objective: The Management of Myelomeningocele Study demonstrated that fetal surgery, as compared to postnatal repair, decreases the rate of hydrocephalus and improves expected motor function. However, fetal surgery is associated with significant maternal and neonatal risks including uterine wall dehiscence, prematurity, and fetal or neonatal death. The goal of this study was to provide information about counseling expectant mothers regarding myelomeningocele in the era of fetal surgery., Methods: The authors conducted an extensive review of topics pertinent to counseling in the setting of myelomeningocele and introduce a new model for shared decision-making to aid practitioners during counseling., Results: Expectant mothers must decide in a timely manner among several potential options, namely termination of pregnancy, postnatal surgery, or fetal surgery. Multiple factors influence the decision, including maternal health, fetal heath, financial resources, social support, risk aversion, access to care, family planning, and values. In many cases, it is a difficult decision that benefits from the guidance of a pediatric neurosurgeon., Conclusions: The authors review critical issues of prenatal counseling for myelomeningocele and discuss the process of shared decision-making as a framework to aid expectant mothers in choosing the treatment option best for them.

The safe entry zones into the brainstem provide access to challenging lesions in this region that do not reach the surface. However, in large brainstem tumors there are two issues to bear in mind before deciding the approach. First, the anatomy can be distorted, and it can be difficult to recognize the entry zone. Second, for large brainstem lesions it may be challenging to address the whole tumor from only one zone and combined approaches may be required. Thus, we show a combined approach, infratentorial supracerebellar and telovelar transventricular, to remove a large brainstem tumor. Appropriate consent was obtained. The video can be found here: https://youtu.be/nCcG9zPq7ug., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2019, Guillermo Aldave.)

Objective: Resection of posterior fossa tumors in children may be associated with persistent neurological deficits. It is unclear if these neurological deficits are associated with persistent structural damage to the cerebellar pathways. The purpose of this research was to define longitudinal changes in diffusion tensor imaging (DTI) metrics in white matter cerebellar tracts and the clinical correlates of these metrics in children undergoing resection of posterior fossa tumors., Methods: Longitudinal brain DTI was performed in a cohort of pediatric patients who underwent resection of posterior fossa tumors. Fractional anisotropy (FA) of the superior cerebellar peduncles (SCPs) and middle cerebellar peduncles (MCPs) was measured on preoperative, postoperative, and follow-up DTI. Early postoperative (< 48 hours) and longer-term follow-up neurological deficits (mutism, ataxia, and extraocular movement dysfunction) were documented. Statistical analysis was performed to determine differences in FA values based on presence or absence of neurological deficits. Statistical significance was set at p < 0.05., Results: Twenty children (mean age 6.1 ± 4.1 years [SD], 12 males and 8 females) were included in this study. Follow-up DTI was performed at a median duration of 14.3 months after surgery, and the median duration of follow-up was 19.7 months. FA of the left SCP was significantly reduced on postoperative DTI in comparison with preoperative DTI (0.44 ± 0.07 vs 0.53 ± 0.1, p = 0.003). Presence of ataxia at follow-up was associated with a persistent reduction in the left SCP FA on follow-up DTI (0.43 ± 0.1 vs 0.55 ± 0.1, p = 0.016). Patients with early postoperative mutism who did not recover at follow-up had significantly decreased FA of the left SCP on early postoperative DTI in comparison with those who recovered (0.38 ± 0.05 vs 0.48 ± 0.06, p = 0.04)., Conclusions: DTI after resection of posterior fossa tumors in children shows that persistent reduction of SCP FA is associated with ataxia at follow-up.

OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and -13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and -12.8%, respectively. CONCLUSIONS Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.