UNASSIGNED: To evaluate the association between wildfire exposure in pregnancy and spina bifida risk.
UNASSIGNED: This retrospective cohort study used the California Office of Statewide Health Planning and Development Linked Birth File with hospital discharge data between 2007 and 2010. The Birth File data were merged with the California Department of Forestry and Fire Protection data of the same year. Spina bifida was identified by its corresponding ICD-9 code listed on the hospital discharge of the newborn. Wildfire exposure was determined based on the zip code of the woman\'s home address. Pregnancy was considered exposed to wildfire if the mother lived within 15 miles of a wildfire during the pregnancy or within 30 days prior to pregnancy.
UNASSIGNED: There were 2,093,185 births and 659 cases of spina bifida between 2007 and 2010. The births were analyzed using multivariable logistic regression models and adjusted for potential confounders. Exposure to wildfire in the first trimester was associated with higher odds of spina bifida (aOR= 1.43 [1.11-1.84], p-value = 0.01). Wildfire exposure 30 days before the last menstrual period and during the second and third trimesters were not associated with higher spina bifida risk.
UNASSIGNED: Wildfire exposure has shown an increased risk of spina bifida during the early stages of pregnancy.

OBJECTIVE: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair.
METHODS: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion.
RESULTS: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months.
CONCLUSIONS: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, p = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms.

METHODS: Retrospective and cross-sectional study.
OBJECTIVE: The study aimed to carry out telemonitoring to identify the impact of the pandemic on physical and functional disabilities in children and adolescents with SB, as reported by their caregivers, and to investigate adherence to a teleservice.
METHODS: Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP).
METHODS: Retrospective and cross-sectional study. Fifty-three patients with SB (mean age 12.0 (4.0) years; 23 males) participated in the telemonitoring. A questionnaire - \'Health management, health conditions, rehabilitation, interest in teleservice, and the well-being of the main caregiver\' - investigated the clinical impact of the coronavirus pandemic. Only three caregivers participated in the teleservice (video call).
RESULTS: According to telemonitoring, 62% of the patients discontinued physiotherapy sessions, and 69% reported needing adjustments in locomotion devices. The main complaints were muscle weakness and pain.
CONCLUSIONS: We monitored general health and identified demands related to physical rehabilitation using telemonitoring in 42.4% of children and adolescents with SB monitored at the HCFMRP-USP. Telemonitoring and teleservice may be methods used for monitoring health conditions in patients with SB.

The etiology of spina bifida, a neural tube birth defect, is largely unknown, but a majority of cases are thought to be genetic in origin. Although maternal blood type was found not to be associated with the occurrence of spina bifida, the analysis was never extended to other aspects of the disorder. The purpose of this descriptive study was to determine if maternal blood type was related to characteristics of children with spina bifida. The blood type of 221 mothers of children with spina bifida enrolled on the Arkansas Spinal Cord Disability Registry from 1995 to 2008 was obtained by mailed questionnaire. All children were community-dwelling and from singleton pregnancies. As expected, analysis of mother-child data showed that the distribution of mothers\' blood type was not statistically different from the general population (chi-squared, P = 0.9203). However, the blood type of these mothers was associated with their child\'s lesion level (chi-squared, P = 0.011). Mothers with blood type A more frequently had children with thoracic lesions; mothers with non-A blood types more frequently had children with lumbar and sacral lesions. In addition, mean birthweight differed by mothers\' blood type (analysis of variance, P = 0.025). Children of mothers with blood type A had the highest mean birthweight, while those of mothers with blood type AB had the lowest. Also, hydrocephalus was present more frequently in children with thoracic lesions compared to those with lumbar and sacral lesions (chi-squared, P = 0.001). Interestingly, these results were significant for female children but not for male children. In conclusion, maternal blood type was associated with lesion level and birthweight of children with spina bifida.

A primigravida in the extremist-affected region of a third-world nation gave birth to a newborn who was remotely consulted through video rounds from the capital of the state. Unfortunately, these abnormalities are often overlooked and left untreated. The baby had multiple limb defects, gastroschisis, exstrophy of the bladder and spina bifida. Tragically, the newborn did not survive due to the lack of clinical and surgical expertise in the area. It is crucial to emphasise the importance of establishing e-clinics for expectant mothers in underserved areas, providing them with access to high-quality anomaly scans.

Interdisciplinary care and pediatric to adult transitional programs have consistently shown medical and social value for individuals with complex medical conditions such as spina bifida (SB). Such interdisciplinary clinics are common in pediatrics but are rarely offered for adults. This survey-based study reports information related to transition, daily pain burden, and satisfaction with care delivery in an adult SB clinic.
A 23-question survey that was based on empirical observations from the adult SB clinic was formulated, IRB approved, and distributed to adult patients. Many respondents had previously received care at the institution\'s pediatric SB clinic and completed transition to the adult program. Responses were de-identified, categorized, stored in a secure database, and statistically analyzed using SPSS.
Of 245 patients approached, 116 (47%) surveys were completed and analyzed. Those who had a direct transition (defined as a less than 24-month gap in care) from the pediatric to the adult clinic comprised 44% (n = 51) of responders. The alternative group of 56% (n = 65) had a longer gap, disorganized or absent transition, or had pediatric care elsewhere. The study population had an average age of 36 years, had mostly received childhood care at the authors\' institution, regardless of whether they made a direct transition or had a gap in care (68%), and held the diagnosis of open myelomeningocele (78%). Overall satisfaction with the clinic experience was high (mean score 9.04 on a 10-point subjective scale). Differences regarding independence in activities of daily living based on transition status were not significant, but on multivariate analysis, those who reported independence in activities of daily living had an almost 4-fold higher odds of daily pain (p = 0.024; OR 3.86, 95% CI 1.19-12.5). The most frequently identified areas for improvement included improved access to care and pain control.
Pediatric transitional processes and interdisciplinary clinics may contribute to improved patient-perceived outcomes and satisfaction with their SB care in comprehensive settings. Further elucidation of barriers to pain control is warranted, in addition to ways in which comprehensive and longitudinal care can improve them.

The pediatric neurosurgical community has increasingly recognized the importance of healthcare transition, the process of moving a patient from a pediatric to an adult model of care. However, surveys of pediatric neurosurgeons have revealed that few institutions have formal transition programs. Here, the authors share their preliminary experience with the development of a formal transition pilot program for patients with spina bifida and/or hydrocephalus.
Patients 18 years of age or older with a diagnosis of spina bifida and/or hydrocephalus who were followed by a pediatric neurosurgeon at Connecticut Children\'s from January 2017 to December 2023 and were recommended to transition to an adult neurosurgeon were retrospectively reviewed. Patients in the informal transition program (ITP) cohort (i.e., the recommendation to transition was made before the formal transition program [FTP] was developed in early 2020) were compared with those in the FTP cohort.
Twenty-two patients met inclusion criteria with 7 (31.8%) in the ITP cohort and 15 (68.2%) in the FTP cohort. The median age at the time of the recommendation to transition was similar in both ITP and FTP cohorts (24 [IQR 20-35] years vs 25 [IQR 24-27] years, respectively). Four (57.1%) patients in the ITP cohort had a confirmed visit with an adult neurosurgeon, compared with 13 (86.7%) patients in the FTP cohort (p = 0.274). One patient in the ITP cohort with a failed transition returned to pediatric neurosurgical care, and 1 patient in the FTP cohort required a shunt revision by an adult neurosurgeon within 1 year of the recommendation to transition.
Healthcare transition is recognized as a priority within pediatric neurosurgery, but structured, formal transition programs remain underdeveloped. The authors\' preliminary experience with a pilot transition program demonstrated that patients who underwent a formal transition were more likely to successfully establish care with an adult neurosurgeon and trended toward less resource utilization.

暂无摘要。

In India, adult neurosurgeons are required to care for children regularly because the concept of dedicated pediatric specialty care is not yet entirely established in the subcontinent. Likewise, pediatric neurosurgeons do not exclusively offer their services to the young, but they also provide care to adult patients with neurosurgical disorders. This creates a medical system where the transition between specialties is not often a formal and recognized aspect of neurosurgical care because most neurosurgeons provide care for patients of all ages. Additionally, there are very few teams geared toward caring for conditions in children that merit lifelong medical support, with spina bifida (SB) being one of them. Since there are no focused or structured pediatric programs on a large scale, developing a multidisciplinary clinic for adults becomes challenging. A pragmatic approach using technology-based education, supported by an organized system or a coordinator, may be a new strategy. A new system utilizing telemedicine and smartphones for established patients maybe an alternative option for SB children in India. During virtual video conferences, an established patient may benefit from multispecialty care and education toward a smooth transition that avoids significant issues with time, transportation, or financial constraints. Achieving a seamless transition among allied specialists from the pediatric to adult systems is a utopia. The current system in the subcontinent may be improved, with an opportunity to develop smooth transition care between coordinated specialists (who simultaneously treat children and adults). Learning from various global SB management styles, the Indian transition situation may offer another model in the near future.