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Objective: To identify predictors for intact motor function (MF) at birth and at 12 months of life in babies with prenatally versus postnatally repaired open spina bifida (OSB). Design: Retrospective cohort study. Setting: Texas Children's Hospital, 2011–2018. Population: Patients who underwent either prenatal or postnatal OSB repair. Methods: Prenatal MF of the lower extremities was evaluated by ultrasound following a metameric distribution at the time of diagnosis (US1), 6 weeks postoperatively (or 6 weeks after initial evaluation in postnatally repaired cases) (US2) and at the last ultrasound before delivery (US3). At birth and at 12 months, MF was assessed clinically. Intact MF (S1) was defined as the observation of plantar flexion of the ankle. Results from logistic regression analysis are expressed as odds ratios (95% confidence intervals, P values). Results: A total of 127 patients were included: 93 with prenatal repair (51 fetoscopic; 42 open hysterotomy repair) and 34 with postnatal repair. In the prenatal repair group, predictors for intact MF at birth and at 12 months included: absence of clubfeet (OR 11.3, 95% CI 3.2–39.1, P < 0.01; OR 10.8 95% CI 2.4–47.6, P < 0.01); intact MF at US1 (OR 19.7, 95% CI 5.0–76.9, P < 0.01; OR 8.7, 95% CI 2.0–38.7, P < 0.01); intact MF at US2 (OR 22, 95% CI 6.5–74.2, P < 0.01; OR 13.5, 95% 3.0–61.4, P < 0.01); intact MF at US3 (OR 13.7, 95% CI 3.4–55.9, P < 0.01; OR 12.6, 95% CI 2.5–64.3, P < 0.01); and having a flat lesion (OR 11.2, 95% CI 2.4–51.1, P < 0.01; OR 4.1, 95% CI 1.1–16.5, P = 0.04). In the postnatal repair group, the only predictor of intact MF at 12 months was having intact MF at birth (OR 15.2, 95% CI 2.0–113.3, P = 0.03). Conclusions: The detection of intact MF in utero from mid‐gestation to delivery predicts intact MF at birth and at 12 months in babies who undergo prenatal OSB repair. Detection of intact motor function in utero predicts intact motor function at birth and at 1 year in fetuses who undergo prenatal OSB repair. Detection of intact motor function in utero predicts intact motor function at birth and at 1 year in fetuses who undergo prenatal OSB repair. [ABSTRACT FROM AUTHOR]

Objectives: (1) To compare brain findings between large and non-large neural tube defect (NTD); (2) to evaluate the impact of large lesion on the surgical parameters; (3) to study any associations between the size of the lesions and brain findings 6 weeks postoperatively and neurological short-term outcomes.Design: Retrospective cohort study.Setting: Texas Children's Hospital, between 2011 and 2018.Population: Patients who underwent prenatal NTD repair.Methods: Large lesion was defined when the lesion's surface was >75th centile of our cohorts' lesions.Main Outcome Measures: Time of referral: ventriculomegaly and anatomical level of the lesion; surgery: duration and need for relaxing incisions. 6 weeks postoperative: hindbrain herniation (HBH) and ventriculomegaly. After delivery: dehiscence, need for hydrocephalus treatment and motor function.Results: A total of 99 patients were included, 25 of whom presented with large lesions. Type of lesion and ventriculomegaly were comparable between individuals with large and non-large lesions. Individuals with large lesions were associated with increased need for relaxing incisions by 5.4 times (95% CI 1.3-23.2, P = 0.02). Six weeks postoperatively, having a large lesion decreased by ten times the likelihood of having a postoperative reversal of HBH (odds ratio = 0.1, 95% CI 0.1-0.4, P < 0.01). At birth, larger lesions increased the risk for repair dehiscence by 6.1 times (95% CI 1.6-22.5, P < 0.01) and the risk of dehiscence or leakage of cerebrospinal fluid at birth by 5.5 times (95% CI 1.6-18.9, P < 0.01).Conclusion: Prenatal repair of patients with large NTD presents a lower proportion of HBH reversal 6 weeks after the surgery, a higher risk of dehiscence and a higher need for postnatal repair.Tweetable Abstract: Evaluation of the size of fetal NTD can predict adverse neurological outcomes after prenatal NTD repair. [ABSTRACT FROM AUTHOR]

Background: Controversy exists regarding the optimal mode of delivery for fetuses with open neural tube defects.Objective: To compare neurological outcomes among infants with open neural tube defects who underwent vaginal compared with caesarean delivery.Search Strategy: Electronic databases MEDLINE, EMBASE, Scopus, and Clinicaltrials.gov were searched from inception to November 2017.Selection Criteria: Eligible studies included observational or randomised studies comparing vaginal and caesarean delivery in pregnancies with fetal open neural tube defects who did not undergo prenatal repair.Data Collection and Analysis: Two reviewers independently reviewed abstracts and full-text articles. Outcomes were compared between vaginal and caesarean delivery and prelabour caesarean versus exposure to labour. The primary outcome was motor-anatomic level difference. Secondary outcomes included shunt requirement, sac disruption, meningitis, and ambulation at 2 years. Meta-analysis was performed and mean difference or odds ratios with 95% CI were calculated.Main Results: Of 201 abstracts identified in the primary search, nine studies (672 women) met the eligibility criteria. Comparing vaginal and caesarean delivery, there was no significant difference in motor-anatomic level difference (mean difference -0.10, 95% CI -0.58 to 0.38; I2  = 57%). The vaginal delivery group was less likely to require a shunt or have sac disruption [odds ratio (OR) 0.37, 95% CI 0.14-0.95 and OR 0.46, 95% CI 0.23-0.90, respectively]. Comparisons by prelabour caesarean versus exposure to labour showed no significant difference in motor-anatomic level difference (OR 1.29, 95% CI 0.63-3.21) or ambulation at 2 years (OR 2.13, 95% CI 0.35-13.12).Conclusion: Caesarean delivery was not associated with improved neurological outcomes among fetuses with open neural tube defects.Tweetable Abstract: Available evidence does not support routine caesarean delivery for fetuses with open neural tube defects. [ABSTRACT FROM AUTHOR]

Despite recent disappointments in the few medications that could treat IBS, diagnosing and managing this frustrating condition and improving your patients' quality of life are within reach. [ABSTRACT FROM AUTHOR]

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.

Background: Functional defecation disorders (FDD) are a common etiology of refractory chronic constipation (CC). FDD diagnosis (dyssynergic defecation [DD] and inadequate defecatory propulsion [IDP]), requires diagnostic tests including anorectal manometry (ARM) and balloon expulsion test (BET). Biofeedback (BF) is the treatment of choice for DD. The aims of our study were to evaluate: the outcome of BF in a group of constipated patients with defecatory disorders of any etiology; the efficacy of two simple diagnostic tools in predicting BF outcome in the short-term., Methods: One hundred and thirty-one refractory CC patients failing the BET underwent BF therapy. Before BF, all patients underwent the following: ARM. Straining questionnaire. The answers were: "belly muscles"; "anal muscles"; "both"; "Don't know/No answer." Digital rectal examination augmented by abdominal palpation on straining (augmented-DRE). The BF therapist was blinded to ARM, straining questionnaire, and augmented-DRE results., Key Results: Eighty-one patients responded to BF. Gender, age, and IBS-C showed no significant impact on BF response. Both DD and IDP responded equally to BF, while the rate of response in patients with isolated structural pelvic floor abnormalities was lower (p < 0.001). The answer "anal muscles" to straining questionnaire showed a strong association with BF response (p < 0.001). A lack in abdominal contraction and in anal relaxation on augmented-DRE were strongly associated with BF response (p < 0.01). Absence of manual maneuvers to facilitate defecation was associated with BF response (p < 0.001)., Conclusions & Inferences: BF is the therapy of choice for refractory constipation due to FDD of any etiology, inducing both clinical and anorectal physiology improvement in the short term. Comorbid IBS-C did not affect outcome while symptomatic isolated pelvic floor abnormalities appeared refractory to behavior treatment. The straining questionnaire and augmented-DRE outcomes showed a strong correlation with BF response and can be implemented in clinical practice to improve the management of constipated patients by prompting early referral to BF., (© 2024 The Author(s). Neurogastroenterology & Motility published by John Wiley & Sons Ltd.)

Objective: The purpose of this study was to conduct a literature review on transition programs from pediatric to adult care and the role of neurosurgery as individuals with spina bifida (SB) transition, and to provide a framework for neurosurgical providers to assist in the transition to adult-centered care., Methods: A comprehensive literature review was conducted according to the PRISMA statement, with a search in Medline and Embase to identify US clinical programs reporting on their experiences establishing a transition program for adolescents and young adults with SB. Data were collected for authors, year, transition clinic location, model of care for transition clinic, ages served, and specialty clinical team., Results: The literature search yielded 698 articles, 5 of which met the inclusion criteria. These 5 studies included 4 transition programs for which models of care and approach to transition, clinical services involved, establishment of goals, and age of initiation and transition were identified. All programs described setting transition goals, ranging from community services, to self-management, to health care navigation, to patient-driven goals, with 1 program reporting a quality-of-life measurement component to their model., Conclusions: Robust SB transition programs can be established by applying the expanded chronic care model, reviewing lessons learned by other programs, advocating at the institutional level, and seeking support via professional organizations. While the comprehensive role of neurosurgical providers in these programs is still being defined, a shared vision of enhancing the health and quality of life for individuals with SB and their families is needed by all subspecialists involved.

Objectives: To compare the ambulatory status of a cohort of children who had undergone prenatal repair of an open neural tube defect (ONTD) using one of two different methods (fetoscopic or open hysterotomy) with that of a cohort who had undergone postnatal repair, and to identify the best predictors of ambulation at 30 months of age., Methods: This was a retrospective review of a cohort of children who underwent ONTD repair either prenatally (n = 110), by fetoscopic surgery (n = 73) or open hysterotomy surgery (n = 37), or postnatally (n = 51), in a single tertiary hospital between November 2011 and May 2023. The cohort comprised a consecutive sample of cases who had undergone ONTD repair in-utero following Management of Myelomeningocele Study (MOMS) trial criteria and cases who had undergone postnatal repair, meeting the same criteria, which were also followed up after birth at the same institution. Motor function assessment by ultrasound was recorded at referral, 6 weeks after prenatal repair, or after referral in postnatally repaired cases, and at the last ultrasound scan before delivery. Clinical examinations to assess motor function at birth and at 12 months were retrieved from records. Intact motor function was defined as first sacral myotome (S1) motor function. Ambulatory status data at each follow-up visit were collected. The proportion of children who were able to walk independently after 30 months of age was compared between those who had undergone fetoscopic vs open prenatal surgery and between prenatal (by either fetoscopic or open surgery) and postnatal ONTD repair. Logistic regression analyses were performed to identify predictors for independent ambulation., Results: After 30 months, the proportion of infants who were able to walk independently was higher in prenatally vs postnatally repaired cases (51.8% vs 15.7%, P < 0.01), and there was no difference between those with fetoscopic (52.1%) vs open (51.4%) prenatal repair (P = 0.66). In the prenatally repaired group, having intact motor function at 12 months (adjusted odds ratio (aOR), 9.14 (95% CI, 2.64-31.63), P < 0.01) and at birth (aOR, 4.50 (95% CI, 1.21-16.80), P = 0.02) were significant predictors of independent walking at 30 months; an anatomical level of lesion below L2 at referral (aOR, 1.83 (95% CI, 1.30-2.58), P = 0.01) and female gender (aOR, 3.51 (95% CI, 1.43-8.61), P < 0.01) were also predictive for this outcome., Conclusions: Prenatally repaired cases of ONTD have a better chance of being able to walk independently at 30 months than do those who undergo postnatal repair. In patients with prenatally repaired ONTD, ambulatory status at 30 months can be predicted by observing a low lesion level at referral (below L2) and intact motor function postnatally. These results have implications for parental counseling and planning for supportive therapy in pregnancies affected by ONTD. © 2024 International Society of Ultrasound in Obstetrics and Gynecology., (© 2024 International Society of Ultrasound in Obstetrics and Gynecology.)

Introduction: Outcomes for pineal region and superior cerebellar tumors in young children often hinge on extent of microsurgical resection, and thus choosing an approach that provides adequate visualization of pathology is essential. The occipital interhemispheric transtentorial (OITT) approach provides excellent exposure while minimizing cerebellar retraction. However, this approach has not been widely accepted as a viable option for very young children due to concerns for potential blood loss when incising the tentorium. The aim of this paper is to characterize our recent institutional experience with the occipital interhemispheric transtentorial approach (OITT) for tumor resection in infants and toddlers., Methods: A retrospective study was performed between 2016 and 2023 of pediatric patients less than 36 months of age who underwent OITT for tumor resection at a high-volume referral center. Patients with at least 3 months of postoperative follow-up and postoperative MRI were included. Primary outcomes included extent of resection, intraoperative and postoperative complications, and neurologic outcome. Secondary outcomes included length of stay and estimated blood loss., Results: Eight patients, five male, were included. The median age at the time of surgery was 10 months (range 5-36 months). Presenting symptoms included macrocephaly, nausea/vomiting, strabismus, gait instability, or milestone regression. Hydrocephalus was present preoperatively in all patients. Average tumor volume was 38.6 cm 3 , ranging from 1.3 to 71.9 cm 3 . All patients underwent an OITT approach for tumor resection with stereotactic guidance. No intraoperative complications occurred, and no permanent neurologic deficits developed postoperatively. Gross total resection was achieved in all cases per postoperative MRI report, and no instances of new cerebellar, brainstem, or occipital lobe ischemia were noted., Conclusions: OITT approach for tumor resection in very young children (≤ 36 months) is an effective strategy with an acceptable safety profile. In our series, no significant intraoperative or postoperative complications occurred. To our knowledge, this is the first report describing this technique specifically in patients less than 36 months of age., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Spina bifida (SB) occurs in 3.5/10,000 live births and is associated with significant long-term neurologic and urologic morbidity. We explored the characteristics and outcomes of pediatric patients with SB and the facilities that treat them in Texas., Methods: We retrospectively reviewed a statewide hospital inpatient discharge database (2013-2021) to identify patients aged <18 y with SB using International Classification of Diseases 9/10 codes. Patients transferred to outside hospitals were excluded to avoid double-counting. Descriptive statistics and chi-square test were performed., Results: Seven thousand five hundred thirty one inpatient hospitalizations with SB were analyzed. Most SB care is provided by a few facilities. Two facilities (1%) averaged >100 SB admissions per year (33% of patients), while 15 facilities (8%) treat 10-100 patients per year (51% of patients). Most facilities (145/193, 75%) average less than one patient per year. Infants tended to be sicker (17% extreme illness severity, P < 0.001). Overall mortality is low (1%), primarily occurring in the neonatal period (8%, P < 0.001). Most admissions are associated with surgical intervention, with 63% of encounters having operating room charges with an average cost of $25,786 ± 24,884. Admissions for spinal procedures were more common among infants, whereas admissions for genitourinary procedures were more common among older patients (P < 0.001). The average length of stay was 8 ± 16 d with infants having the longest length of stay (19 ± 33, P < 0.001)., Conclusions: Patients have significant long-term health needs with evolving pediatric surgical indications as they grow. Pediatric SB care is primarily provided by a small number of facilities in Texas. Longitudinal care coordination of their multidisciplinary surgical care is needed to optimize patient care., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort., Methods: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months., Results: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013)., Conclusions: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Purpose: Emerging evidence suggests proton radiation therapy may offer cognitive sparing advantages over photon radiation therapy, yet dosimetry has not been compared previously. The purpose of this study was to examine dosimetric correlates of cognitive outcomes in children with medulloblastoma treated with proton versus photon radiation therapy., Methods and Materials: In this retrospective, bi-institutional study, dosimetric and cognitive data from 75 patients (39 photon and 36 proton) were analyzed. Doses to brain structures were compared between treatment modalities. Linear mixed-effects models were used to create models of global IQ and cognitive domain scores., Results: The mean dose and dose to 40% of the brain (D40) were 2.7 and 4.1 Gy less among proton-treated patients compared with photon-treated patients (P = .03 and .007, respectively). Mean doses to the left and right hippocampi were 11.2 Gy lower among proton-treated patients (P < .001 for both). Mean doses to the left and right temporal lobes were 6.9 and 7.1 Gy lower with proton treatment, respectively (P < .001 for both). Models of cognition found statistically significant associations between higher mean brain dose and reduced verbal comprehension, increased right temporal lobe D40 with reduced perceptual reasoning, and greater left temporal mean dose with reduced working memory. Higher brain D40 was associated with reduced processing speed and global IQ scores., Conclusions: Proton therapy reduces doses to normal brain structures compared with photon treatment. This leads to reduced cognitive decline after radiation therapy across multiple intellectual endpoints. Proton therapy should be offered to children receiving radiation for medulloblastoma., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 births in the United States. The most common treatment, ventricular shunting, has a failure rate of up to 85% within 10 years of placement. The authors aimed to analyze the association between ventricular catheter (VC) tissue obstructions and shunt malfunction for each hydrocephalus etiology., Methods: Patient information was collected from 5 hospitals and entered into a REDCap (Research Electronic Data Capture) database by hydrocephalus etiology. The hardware samples were fixed, and each VC tip drainage hole was classified by tissue obstruction after macroscopic analysis. Shunt malfunction data, including shunt revision rate, time to failure, and age at surgery, were correlated with the degree of tissue obstruction in VCs for each etiology., Results: Posthemorrhagic hydrocephalus was the most common etiology (48.9% of total cases). Proximal catheter obstruction was the most frequent cause of hardware removal (90.4%). Myelomeningocele (44% ± 29%), other congenital etiologies (48% ± 40%), hydrocephalus with brain tumors (45% ± 35%), and posthemorrhagic hydrocephalus (41% ± 35%) showed tissue aggregates in more than 40% of the VC holes. A total of 76.8% of samples removed because of symptoms of obstruction showed cellular or tissue aggregates. No conclusive etiological associations were detected when correlating the percentage of holes with tissue for each VC and age at surgery, shunt revision rates, or time between shunt implantation and removal., Conclusions: The proximal VC obstruction was accompanied by tissue aggregates in 76.8% of cases. However, the presence of tissue in the VC did not seem to be associated with hydrocephalus etiology.

Background and Purpose: Outward convexity of the basiocciput and posterior atlanto-occipital membrane are common in patients with Chiari II malformation associated with an open neural tube defect. We aimed to determine if the severity of these findings correlated with the need for future hydrocephalus treatment., Materials and Methods: A retrospective chart and imaging review identified patients who underwent open neural tube defect repair at a quaternary care pediatric hospital from July 2014 through September 2022. Patients were classified by the need for hydrocephalus treatment and whether they received prenatal or postnatal neural tube defect repair. Measurements of imaging parameters related to posterior fossa maldevelopment and skull base remodeling were performed., Results: Compared with 65 patients who did not require hydrocephalus treatment, 74 patients who required treatment demonstrated statistically significantly greater mean basiocciput convexity ( P < .001). While the mean basiocciput length in the hydrocephalus treatment group was smaller ( P < .001), the ratio of basiocciput convexity to length was larger ( P < .001). Notably, 100% of patients with a basiocciput convexity of ≥4 mm required hydrocephalus treatment. The mean posterior atlanto-occipital membrane convexity was significantly greater for patients who required hydrocephalus treatment in the postnatal group ( P = .02), but not the prenatal group ( P = .09)., Conclusions: Pediatric patients with Chiari II malformation who ultimately required surgical hydrocephalus treatment had greater outward convexity of the basiocciput but had greater posterior atlanto-occipital membrane outward convexity only if the repair was performed postnatally. Together these measurements may be useful in predicting the need for hydrocephalus treatment., (© 2024 by American Journal of Neuroradiology.)

Objective: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF)., Methods: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio., Results: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95)., Conclusions: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Objective: To evaluate the prenatal diagnosis of closed dysraphism (CD) and its correlation with postnatal findings and neonatal adverse outcomes., Methods: A retrospective cohort study including pregnancies diagsnosed with fetal CD by prenatal ultrasound (US) and magnetic resonance imaging (MRI) at a single tertiary center between September 2011 and July 2021., Results: CD was diagnosed prenatally and confirmed postnatally in 12 fetuses. The mean gestational age of prenatal imaging was 24.2 weeks, in 17% the head circumference was ≤fifth percentile and in 25% the cerebellar diameter was ≤fifth percentile. US findings included banana sign in 17%, and lemon sign in 33%. On MRI, posterior fossa anomalies were seen in 33% of cases, with hindbrain herniation below the foramen magnum in two cases. Mean clivus-supraocciput angle (CSA) was 74°. Additional anomalies outside the CNS were observed in 50%. Abnormal foot position was demonstrated prenatally in 17%. Neurogenic bladder was present in 90% of patients after birth., Conclusion: Arnold Chiari II malformation and impaired motor function can be present on prenatal imaging of fetuses with CD and may be associated with a specific type of CD. Prenatal distinction of CD can be challenging. Associated extra CNS anomalies are frequent and the rate of neurogenic urinary tract dysfunction is high., (© 2023 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Background and Aims: Defecation Disorders (DD) are a frequent cause of refractory chronic constipation. DD diagnosis requires anorectal physiology testing. Our aim was to evaluate the accuracy and Odds Ratio (OR) of a straining question (SQ) and a digital rectal examination (DRE) augmented by abdomen palpation on predicting a DD diagnosis in refractory CC patients., Methods: Two hundred and thirty-eight constipated patients were enrolled. Patients underwent SQ, augmented DRE and balloon evacuation test before entering the study and after a 30-day fiber/laxative trial. All patients underwent anorectal manometry. OR and accuracy were calculated for SQ and augmented DRE for both dyssynergic defecation and inadequate propulsion., Results: "Anal Muscles" response was associated to both dyssynergic defecation and inadequate propulsion, with an OR of 13.6 and 5.85 and an accuracy of 78.5% and 66.4%, respectively. "Failed anal relaxation" on augmented DRE was associated with dyssynergic defecation, with an OR of 21.4 and an accuracy of 73.1%. "Failed abdominal contraction" on augmented DRE was associated with inadequate propulsion with an OR >100 and an accuracy of 97.1%., Conclusions: Our data support screening constipated patients for DD by SQ and augmented DRE to improve management and appropriateness of referral to biofeedback., Competing Interests: Conflict of interest All authors have no Conflict of interest to be declared., (Copyright © 2023 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Objective: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities., Methods: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics., Results: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance., Conclusions: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.