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Background: Myelomeningocele (MMC) causes significant morbidity and mortality. Efforts have been directed to correct this defect in utero. The neuropathology literature on antenatally repaired MMC and associated complications in humans is limited. Case report: A 12-day-old female, who underwent prenatal MMC repair via a two-layer closure (dural replacement patch, primary skin closure), was born at 34 weeks' gestation. Her group B streptococcus positive mother received appropriate antepartum prophylactic antibiotics. She remained stable until day 11 of life when she underwent rapid clinical deterioration. Despite aggressive intervention, she expired on day 12. Review of placental pathology showed maternal and fetal inflammatory response. Autopsy revealed Gram-positive cocci and inflammation within the basilar leptomeninges and lumbosacral region. Neural and dermal elements were present within the MMC repair. Conclusion: This case documents integration of the dermal matrix patch to neural elements, adhering the spinal cord to scar tissue, the clinical implications of which remain unclear. [ABSTRACT FROM AUTHOR]

Study Objectives: Advances in prenatal repair of myelomeningocele have improved outcomes involving different organ systems. There are limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between patients with spina bifida who have undergone prenatal vs postnatal repair., Methods: We performed a retrospective study of 46 infants < 1 year with spina bifida seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation, ventriculoperitoneal shunt, polysomnography results, and need for supplemental oxygen were collected. Unpaired t test and χ 2 test were used to analyze results., Results: A total of 31/46 had prenatal repair of myelomeningocele; average age at repair was 27 weeks postconception. Average age at postnatal repair was 37 weeks postconception. There was no difference in age at polysomnography. There was no difference in Chiari II malformation presence ( P = .61). Sixty pecent of patients with postnatal repair and 23% in the prenatal group underwent ventriculoperitoneal shunt placement ( P = .01). There was no difference in polysomnography findings between the 2 groups: central apnea index ( P = .11), obstructive apnea-hypopnea index ( P = .64), average oxygen saturation baseline ( P = .91), average oxygen saturation nadir ( P = .17), average end-tidal carbon dioxide baseline ( P = .87), and average end-tidal carbon dioxide maximum ( P = .54). There were no significant differences in the proportion of patients on supplemental oxygen ( P = .25), central sleep apnea or obstructive sleep apnea between groups., Conclusions: Patients with spina bifida who have undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair vs postnatal repair., Citation: Stark KG, Wang RY, Smith KA. Sleep-related breathing disorders in infants with spina bifida repaired prenatally and postnatally. J Clin Sleep Med . 2024;20(10):1579-1583., (© 2024 American Academy of Sleep Medicine.)

Background: Obesity prevalence in youth with spina bifida is higher than in their typically developing peers. Obesity is associated with lifelong medical, psychological, and economic burdens. Successful prevention or treatment of obesity in individuals with spina bifida is compromised by (1) the lack of valid and reliable methods to identify body fat in a clinical setting and (2) limited data on energy expenditure that are necessary to provide daily caloric recommendations., Objective: The objectives of this study will be to develop 2 algorithms for use in youth with spina bifida in a clinical setting, one to model body fat and one to predict total daily energy expenditure. In addition, physical activity and dietary intake will be described for the sample., Methods: This multisite, prospective, national clinical study will enroll 232 youth with myelomeningocele aged 5 to 18 years (stratified by age and mobility). Participants will be enrolled for 1 week. Data obtained include 4 measures of body composition, up to 5 height measures, a ramped activity protocol, and a nutrition and physical activity screener. Participants will wear an accelerometer for the week. On the final study day, 2 samples of urine or saliva, which complete the doubly labeled water protocol, will be obtained. The analysis will include descriptive statistics, Bland-Altman plots, concordance correlation, and regression analysis., Results: The study received extramural federal funding in July 2019. Data collection was initiated in March 2020. As of April 2024, a total of 143 (female participants: n=76, 53.1%; male participants: n=67, 46.9%) out of 232 participants have been enrolled. Data collection is expected to continue throughout 2024. A no-cost extension until November 2025 will be requested for data analysis and dissemination of findings., Conclusions: This study furthers previous pilot work that confirmed the acceptability and feasibility of obtaining alternate height, body composition, and energy expenditure measures. The findings from this study will enhance screening, prevention, and treatment of abnormal weight status by facilitating the accurate identification of youths' weight status category and recommendations of daily caloric needs for this population that is at higher risk of obesity. Furthermore, the findings have the potential to impact outcomes for youth diagnosed with disabilities other than spina bifida who experience similar challenges related to alterations in body composition or fat distribution or measurement challenges secondary to mobility issues or musculoskeletal problems., International Registered Report Identifier (irrid): DERR1-10.2196/52779., (©Michele Polfuss, Kathryn Smith, Betsy Hopson, Andrea Moosreiner, Chiang-Ching Huang, Michele N Ravelli, Dan Ding, Zijian Huang, Brandon G Rocque, Rosemary White-Traut, Alexander Van Speybroeck, Kathleen J Sawin. Originally published in JMIR Research Protocols (https://www.researchprotocols.org), 02.07.2024.)