OBJECTIVE: Chiari II malformation (CM-II) is a congenital malformation of the posterior fossa associated with myelomeningocele. Of the symptomatic patients, 10-33% require surgical treatment. To this date, there is not a consensus about the best surgical technique, and whether to do duroplasty.
METHODS: A literature search of the PubMed database and crossed references was performed, per PRISMA guidelines. Data regarding demographic features, extent of cervicomedullary deformity, clinical presentation, surgical techniques, and clinical outcomes were extracted. Pearson\'s chi-squared test was applied. The p-values under 0.05 were considered statistically significant.
RESULTS: Twenty studies (N = 330) were analyzed. C3 and C4 levels represented 56.4% of the lowest tonsil displacement. The most reported symptom was dysphagia/swallowing dysfunction (53.8%). Suboccipital craniectomy (SOC) and cervical spine expansion (CSE) with duroplasty were the most reported technique. Dural augmentation was performed in 57.4% of the patients. After surgery, 59.6% observed an improvement in symptoms and quality of life, 12.5% were unchanged, and 27.8% had a worsened clinical status. The mortality rate was 2.5% during the first month after surgery, and 17.4% at the last follow-up evaluation. Patients who underwent CSE presented a better clinical outcome (p = 0.002). The SOC procedure could not be correlated with symptom improvement (p = 0.06).
CONCLUSIONS: CM-II is associated with high morbidity and mortality. An early onset symptomatic CM-II demands intervention, which provided an improvement of outcome in most patients included in this review. The best surgical technique and the exact effect of the surgical management of CM-II on mortality are not yet clear.

OBJECTIVE: To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic.
METHODS: Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients\' information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies.
RESULTS: Three cases underwent posterior vertebral column resection (pVCR) of 2-4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other.
CONCLUSIONS: Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.

BACKGROUND: Myelomeningocele is a lifelong condition that features several comorbidities, such as hydrocephalus, scoliosis, club foot, and lower limb sensory and motor disabilities. Its management has progressed over time, ranging from supportive care to early postnatal closure to prenatal closure of the defect. Recent research discovered that fetal myelomeningocele closure (fMMC) provided superior neurological outcomes to those of postnatal closure. When performed at 12 months of age, fMMC can avert or delay the need for a ventriculoperitoneal shunt and reversed the hindbrain herniation. Moreover, fMMC reportedly enhanced motor function and mental development at 30 months of age. However, its long-term outcomes remain dubious.
OBJECTIVE: This systematic review aimed to determine the long-term neurological cognitive, behavioral, functional, and quality of life (QoL) outcomes after fMMC.
METHODS: The PubMed, Directory of Open Access Journals, EBSCO, and Cochrane databases were extensively searched for articles published in 2007-2022. Meta-analyses, clinical trials, and randomized controlled trials with at least 5 years of follow-up were given priority.
RESULTS: A total of 11 studies were included. Most studies revealed enhanced long-term cognitive, behavioral, functional, and QoL outcomes after fMMC.
CONCLUSIONS: Our results suggest that fMMC substantially enhanced patients\' long-term neurological cognitive, behavioral, functional, and QoL outcomes.

UNASSIGNED: Advancements in fetal interventions have enabled in utero management of several fetal congenital anomalies and conditions; these are accomplished via ultrasound-guided, fetoscopic or open techniques. Understanding the risk of preterm labor associated with each technique is critical for patient counseling and choice of intervention; however, data on obstetrical outcomes associated with each type of intervention remains limited.
UNASSIGNED: To provide descriptive information on the risk of preterm birth following fetal intervention, based on underlying disease and procedure performed.
UNASSIGNED: This is a retrospective cohort study of patients who underwent fetal intervention at our institution between 1 October 2016 and 31 December 2019. Interventions were stratified into three groups- ultrasound-guided, fetoscopic and open procedures. Procedures included fetoscopic laser ablation (FLA) for twin-to-twin transfusion syndrome (TTTS), fetoscopic endotracheal occlusion (FETO) for congenital diaphragmatic hernia (CDH), vesicoamniotic shunt (VAS) for lower urinary tract obstruction (LUTO), fetal cystoscopy for LUTO, and open and fetoscopic myelomeningocele repair. The primary outcomes were gestational age at delivery and frequency of premature rupture of the membranes.
UNASSIGNED: Sixty-eight patients were included; 20 (29.4%) underwent ultrasound- guided procedures, 37 (54.4%) underwent fetoscopy, and 11 (16.2%) open in utero intervention. The diagnoses and type of intervention within each group were different. The mean gestational age (GA) ± standard deviation (SD) at intervention for ultrasound- guided, fetoscopic, open procedures were 24.1 ± 4.4 weeks, 22.8 ± 3.7 weeks, and 25.0 ± 0.9 weeks, respectively. The mean GA ± SD at delivery were 31.9 ± 4.9 weeks, 31.6 ± 4.6 weeks, and 32.6 ± 5.5 weeks, respectively. The mean interval from time of intervention to delivery were 54 ± 39, 62 ± 37 and 55 ± 36 days, respectively (p = 0.82); and the risk of PPROM was 26.3%, 21.6% and 27.3%, respectively. The mean GA at delivery and the frequency of PPROM were different for each specific disease that was treated.
UNASSIGNED: The risk of preterm birth and PPROM following fetal intervention with different procedures, categorized under ultrasound- guided, fetoscopic and open fetal interventions at our institution were similar amongst the three groups, but they were different depending on the diagnosis of the treated anomaly.

UNASSIGNED: The purpose of this review is to describe the current scientific literature on the prevalence of metabolic syndrome in children with myelomeningocele and to gain insight into the baseline levels of aerobic fitness, endurance, and strength in this population in order to identify gaps in knowledge, suggest potential primary prevention strategies, and provide recommendations for future studies.
UNASSIGNED: A literature review of articles published in English and French between 1990 and April 2020 was conducted.
UNASSIGNED: Obese adolescents with myelomeningocele have an increased prevalence of components of the metabolic syndrome. Children and adolescents with myelomeningocele have decreased aerobic fitness and muscular strength, decreased lean mass, and increased fat mass, all of which, when combined with higher levels of physical inactivity, put them at higher risk of developing metabolic syndrome and cardiovascular diseases.
UNASSIGNED: Until more research is conducted, addressing weight-related challenges and promoting healthy habits (such as optimal activity levels) could be easily integrated into yearly myelomeningocele clinics. An actionable suggestion might be to systematically weigh and measure children in these clinics and utilize the results and trends as a talking point with the parents and children. The follow-up appointments could also be used to develop physical activity goals and monitor progress. We recommend that the health care practitioner tasked with this intervention (physician, nurse, etc.) should be aware of locally available accessible sports platforms and have knowledge of motivational interviewing to facilitate removal of perceived barriers to physical activity.

BACKGROUND: It remains unclear if fetal repair of myelomeningocele (MM) is associated with a greater risk of developing symptomatic dermal inclusion cysts (ICs) at the neural placode. We report two infants treated with fetal surgery who developed symptomatic IC at less than 1 year of age, and we discuss the current literature on symptomatic IC in children with MM.
METHODS: Two infants underwent fetal MM repair at 24 weeks of gestational age. Case 1 was born at 30 weeks and had two revisions of the MM wound early in life. At 8 months of age, the patient presented with meningismus and imaging findings of an IC, which was resected at the time of presentation. At 3 years of age, this patient was found to have recurrence of the IC after presenting with worsening bladder function and underwent repeat debulking with no recurrence at 6 years of age. Case 2 was born at 32 weeks of gestational age with uncomplicated recovery. At 8 months of age, the patient presented with irritability and fullness at the lumbar repair site. Imaging showed a large IC with restricted diffusion and extension into the subcutaneous tissue; this was resected completely at the time of presentation, see intraoperative photographs. There has been no sign of recurrence at age of 15 months.
CONCLUSIONS: Careful monitoring for IC in infancy in MM patients who have had fetal surgery is recommended.

The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to evaluate the association between SDB, morphological abnormalities, and neurological symptoms and to review the literature on patients with SDB and CM-II.
The study has a cross-sectional, case-control design. Patients with CM-II (patients) were compared to control patients referred for clinical polysomnography in the Sleep Medicine Unit, matched for age and sex. All patients underwent brain and spinal cord magnetic resonance imaging, and polysomnography was conducted for all participants. A review of the literature about SDB in patients with CM-II was performed.
Forty patients were included (20 patients vs 20 control patients). SDB was identified in 45% of patients, a significantly higher prevalence compared to control patients. Three patients presented with purely obstructive SDB, 3 patients with purely central SDB, and 3 patients with both obstructive and central SDB. Compared with control patients, patients with CM-II showed a higher oxygen desaturation index (median: CM-II, 3.7; interquartile range, 1.6-19.5; control patients: 1.1; interquartile range, 0.3-3.2) and obstructive apnea-hypopnea index (median: CM-II, 1.5; interquartile range, 0.5-5.1; control patients, 0.1; interquartile range, 0.0-0.7). A logistic regression showed that the risk of developing SDB in patients affected by CM-II was 14.7 times higher than in the control population.
Our study and literature review showed a high prevalence of SDB in patients with CM-II. These patients are often asymptomatic at diagnosis, suggesting that PSG should be routinely provided in this population.
Lazzareschi I, Curatola A, Massimi L, et al. Sleep-disordered breathing in patients with Chiari malformation type II: a case-control study and review of the literature. J Clin Sleep Med. 2022;18(9):2143-2154.

OBJECTIVE: Tethered cord release (TCR) is the gold standard treatment for tethered cord syndrome (TCS); however, there are significant shortcomings including high rates of retethering, especially in complex and recurrent cases. Spinal column shortening (SCS) is an alternative treatment for TCS intended to avoid these shortcomings. Early studies were limited to case reports and smaller case series; however, in recent years, larger case series and small cohort studies have been conducted. Given the increase in available data, a repeat systematic review and meta-analysis is warranted to assess the safety and efficacy of SCS for TCS.
METHODS: The authors conducted a systematic review using MEDLINE (OVID), Embase (Elsevier), and Web of Science records dating from 1944 to July 2021 to identify all articles investigating SCS for TCS. They performed standard and individual patient data (IPD) meta-analyses, with 2 independent reviewers using PRISMA-IPD guidelines. Primary outcomes were improvement of preoperative clinical symptoms of pain, motor weakness, and bladder and bowel dysfunction, and also surgical complication rate. Secondary outcomes included urodynamic improvement and health-related quality-of-life outcomes determined using patient-reported outcome tools. Individual study quality assessment was performed using a standardized assessment tool for case reports/series, and publication bias was assessed using funnel plot analyses.
RESULTS: The review yielded 15 studies with 191 cases of TCS treated with SCS. IPD were available in 11 studies with 89 cases. The average age at time of surgery was 28.0 years (range 5-76 years). The average follow-up time was 33.2 months (range 7-132 months). Improvement was observed at last follow-up in 60 of 70 (85.7%) patients with preoperative pain, in 38 of 60 (60.3%) patients with preoperative weakness, and in 36 of 76 (47.4%) patients with preoperative bladder or bowel dysfunction. Complications of CSF leak, new neurological deficit, wound infection, or reoperation occurred in 4 of 89 (4.5%) patients.
CONCLUSIONS: SCS may be considered a safe and efficacious treatment option for TCS in children and adults (level C evidence; class IIb recommendation), especially for recurrent and complex cases. Current evidence is likely to be affected by selection and publication bias. Prospective comparative studies of SCS and TCR for TCS are recommended to determine long-term duration of outcomes, long-term safety in skeletally immature children, and exact indications of SCS versus traditional TCR.

While ventriculoperitoneal shunt (VPS) insertion is the standard treatment for myelomeningocele-associated hydrocephalus (MAH), it can be complicated by infection and shunt malfunction. As such, endoscopic third ventriculostomy (ETV), with or without choroid plexus coagulation (CPC), has been proposed as an alternative. The aim of this review was to determine the success, technical failure, and complication rates of ETV with or without CPC in patients with MAH.
PubMed, Scopus, and Cochrane Central Register of Controlled Trials databases were searched from inception to June 2020 for case series, cohort studies, or randomized controlled trials reporting success, technical failure, or complication rates. Random-effects analysis was performed to determine the estimates for these outcome measures. Studies were evaluated using the Newcastle-Ottawa Scale for quality and risk of bias.
Thirteen studies with a total of 325 patients who underwent either ETV or ETV+CPC were included in the review. Using random-effects modeling, the pooled estimate of the success rate was 56% (95% CI 44%-68%, I2 = 78%), while the technical failure rate was 2% (95% CI 0%-6%, I2 = 32%). The estimate for the success rate had high heterogeneity, due to the type of surgical intervention (ETV vs ETV+CPC, p < 0.001). Random-effects analysis of 9 studies with 117 patients who underwent ETV alone yielded an estimated success rate of 48% (95% CI 0.39-0.57, I2 = 0%), while analysis of 4 studies with 166 patients who underwent ETV+CPC revealed a success rate of 75% (95% CI 67%-82%, I2 = 21%). The estimates for the mild/moderate, severe, and fatal complication rates were 0 (95% CI 0%-4%, I2 = 0%), 2% (95% CI 0%-10%, I2 = 52%), and 0 (95% CI 0%-1%, I2 = 0%), respectively.
ETV+CPC was associated with a higher success rate than ETV alone for MAH in a meta-analysis of published studies. ETV, with or without CPC, was technically feasible and safe for this patient population.

OBJECTIVE: Fetal surgery for myelomeningocele has become an established treatment that offers less risk of requiring a ventricular shunt and improved functional outcomes for patients. An increasing body of literature has suggested that social determinants of health have a profound influence on health outcomes. The authors sought to determine the socioeconomic and racial and ethnic backgrounds of patients who were treated with fetal surgery versus those who underwent postnatal repair.
METHODS: Demographic data, the method of myelomeningocele repair, insurance status, and zip code data for patients entered into the National Spina Bifida Patient Registry (NSBPR) from Children\'s Wisconsin were collected. The zip code was used to determine the Distressed Communities Index (DCI) score, a composite socioeconomic ranking with scores ranging from 0 (no distress) to 100 (severe distress). The zip code was also used to determine the median household income for each patient based on the US Census Bureau 2013-2017 American Community Survey 5-year estimates.
RESULTS: A total of 205 patients were identified with zip code and insurance data. There were 23 patients in the fetal surgery group and 182 patients in the postnatal surgery group. All patients were born between 2000 and 2019. Patients in the fetal surgery group were more likely to have commercial insurance (100% vs 52.2%, p < 0.001). Fetal surgery patients were also more likely to be non-Hispanic White (95.7% vs 68.7%, p = 0.058), just missing the level of statistical significance. Patients who underwent fetal surgery tended to reside in zip codes with a higher median household income (mean $66,507 vs $59,133, p = 0.122) and less-distressed communities (mean DCI score 31.3 vs 38.5, p = 0.289); however, these differences did not reach statistical significance.
CONCLUSIONS: Patients treated with fetal surgery were more likely to have commercial insurance and have a non-Hispanic White racial and ethnic background. The preliminary data suggest that socioeconomic and racial and ethnic disparities may exist regarding access to fetal surgery, and investigation of a larger population of spina bifida patients is warranted.