Thalamic connectivity topography in newborns with spina bifida: association with neurological functional level but not developmental outcome at 2 years.

Spina bifida affects spinal cord and cerebral development, leading to motor and cognitive delay. We investigated whether there are associations between thalamocortical connectivity topography, neurological function, and developmental outcomes in open spina bifida. Diffusion tensor MRI was used to assess thalamocortical connectivity in 44 newborns with open spina bifida who underwent prenatal surgical repair. We quantified the volume of clusters formed based on the strongest probabilistic connectivity to the frontal, parietal, and temporal cortex. Developmental outcomes were assessed using the Bayley III Scales, while the functional level of the lesion was assessed by neurological examination at 2 years of age. Higher functional level was associated with smaller thalamo-parietal, while lower functional level was associated with smaller thalamo-temporal connectivity clusters (Bonferroni-corrected P < 0.05). Lower functional levels were associated with weaker thalamic temporal connectivity, particularly in the ventrolateral and ventral anterior nuclei. No associations were found between thalamocortical connectivity and developmental outcomes. Our findings suggest that altered thalamocortical circuitry development in open spina bifida may contribute to impaired lower extremity function, impacting motor function and independent ambulation. We hypothesize that the neurologic function might not merely be caused by the spinal cord lesion, but further impacted by the disruption of cerebral neuronal circuitry.

Education and employment as young adults living with spina bifida transition to adulthood in the USA: A study of the National Spina Bifida Patient Registry.

AIM: To describe the education and employment transition experience of young adults with spina bifida (YASB) and investigate factors associated with employment. METHOD: We queried education and employment data from the US National Spina Bifida Patient Registry from 2009 to 2019. We applied generalized estimating equations models to analyze sociodemographic and disease-related factors associated with employment. RESULTS: A total of 1909 participants (850 males, 1059 females) aged 18 to 26 years contributed 4379 annual visits. Nearly 84% had myelomeningocele and, at last visit, the median age was 21 years (mean 21 years 5 months, SD 2 years 10 months). A total of 41.8% had at least some post-high school education, and 23.9% were employed. In a multivariable regression model, employment was significantly associated with education level, lower extremity functional level, bowel continence, insurance, and history of non-shunt surgery. This large, national sample of YASB demonstrated low rates of post-secondary education attainment and employment and several potentially modifiable factors associated with employment. INTERPRETATION: Specific sociodemographic, medical, and functional factors associated with employment are important for clinicians to consider when facilitating transition for YASB into adulthood. Additional research is needed to understand the impact of cognitive functioning and social determinants of health on transition success in YASB. WHAT THIS PAPER ADDS: There were low education attainment and employment rates in a large sample of young adults with spina bifida. Specific sociodemographic, medical, and functional factors are associated with employment. Some employment-associated factors, such as continence and self-management skills, are modifiable.

Caring for the Child with Spina Bifida.

Care for a child with spina bifida can be complex, requiring multiple specialists. Neurosurgical care centers around the initial closure or repair of the spinal defect, followed by management of hydrocephalus, symptoms of the Chiari 2 malformation, and tethered cord. This article reviews definitions and types of spina bifida, considerations surrounding the initial treatment, including fetal surgery, and the ongoing neurosurgical management of common comorbid conditions. The role of interdisciplinary care is stressed, as well as the importance of coordinated transition to adult care at an appropriate age and developmental stage.

'We did everything we could'- a qualitative study exploring the acceptability of maternal-fetal surgery for spina bifida to parents.

OBJECTIVE: To explore the concepts and strategies parents employ when considering maternal-fetal surgery (MFS) as an option for the management of spina bifida (SB) in their fetus, and how this determines the acceptability of the intervention. METHODS: A two-centre interview study enrolling parents whose fetuses with SB were eligible for MFS. To assess differences in acceptability, parents opting for MFS (n = 24) were interviewed at three different moments in time: prior to the intervention, directly after the intervention and 3-6 months after birth. Parents opting for termination of pregnancy (n = 5) were interviewed only once. Themes were identified and organised in line with the framework of acceptability. RESULTS: To parents opting for MFS, the intervention was perceived as an opportunity that needed to be taken. Feelings of parental responsibility drove them to do anything in their power to improve their future child's situation. Expectations seemed to be realistic yet were driven by hope for the best outcome. None expressed decisional regret at any stage, despite substantial impact and, at times, disappointing outcomes. For the small group of participants, who decided to opt for termination of pregnancy (TOP), MFS was not perceived as an intervention that substantially could improve the quality of their future child's life. CONCLUSION: Prospective parents opting for MFS were driven by their feelings of parental responsibility. They recognise the fetus as their future child and value information and care focusing on optimising the child's future health. In the small group of parents opting for TOP, MFS was felt to offer insufficient certainty of substantial improvement in quality of life and the perceived severe impact of SB drove their decision to end the pregnancy.

Sexuality of Female Spina Bifida Patients: Predictors of a Satisfactory Sexual Function / Sexualidade feminina em pacientes com espinha bífida: preditores de uma função sexual satisfatória

Abstract Objective To assess the sexual function of women with spina bifida (SB), and to verify the factors that influence their sexual function. Methods A cross-sectional study in which a validated female-specific questionnaire was applied to 140 SB female patients from four different cities (Porto Alegre, Brazil; and Barcelona, Madrid, and Málaga, Spain) between 2019 and 2020. The questionnaires collected data on the clinical characteristics of SB, and female sexual function was assessed using the 6-item version of the Female Sexual Function Index (FSFI-6) validated to Portuguese and Spanish. Results Half of the patients had had sexual activity at least once in the life, but most (57.1%) did not use any contraception method. Sexual dysfunction was present in most (84.3%) patients, and all sexual function domains were impaired compared those of non-neurogenic women. The presence of urinary and fecal incontinence significantly affected the quality of their sexual activity based on the FSFI-6. Conclusion The specific clinical aspects of the SB patients, such as urinary and fecal incontinence, should be properly addressed by their doctors, since they are associated with reduced sexual activity and lower FSFI-6 scores in the overall or specific domains. There is also a need to improve gynecological care among sexually-active SB patients, since most do not use any contraceptive methods and are at risk of inadvertent pregnancy.

Resumo Objetivo Analisar a função sexual de pacientes do sexo feminino com espinha bífida (EB), e avaliar quais fatores influenciam na função sexual. Métodos Uma pesquisa transversal em que um questionário validado para mulheres foi aplicado em 140 pacientes com EB de quatro cidades diferentes (Porto Alegre, Brasil; e Barcelona, Madri e Málaga, Espanha) entre 2019 e 2020. Os questionários coletaram dados sobre características clínicas da espinha bífida, e a função sexual feminina foi avaliada com a versão de seis itens do Índice de Funcionamento Sexual Feminino (IFSF-6) nas versões validadas para português e espanhol. Resultados Metade das pacientes havia praticado atividade sexual pelo menos uma vez na vida, mas a maioria (57.1%) não utilizava nenhum método contraceptivo. A disfunção sexual estava presente na maioria das pacientes (84.3%), sendo todos os domínios de função sexual prejudicados em comparação com os de mulheres não neurogênicas. A presença de incontinência urinária e fecal afetou significativamente a qualidade da atividade sexual das pacientes. Conclusão Aspectos clínicos específicos da EB, como incontinência urinária e fecal, devem ser adequadamente abordados pelos médicos assistentes, visto que estão associados à redução na atividade sexual e piores resultados no IFSF-6. Também é necessário melhorar o atendimento ginecológico das pacientes sexualmente ativas, uma vez que a maioria não utiliza métodos contraceptivos e corre o risco de gravidez inadvertida.

Resilience and associated characteristics in adults with spina bifida.

AIM: To measure resilience and identify associated demographic and clinical factors in individuals with spina bifida. METHOD: An anonymous survey was distributed via Facebook advertising to individuals with congenital urological conditions. Respondents 18 years or older with spina bifida were included in this study. Resilience was measured with the 10-item Connor-Davidson Resilience Scale. Mean resilience levels in the study population and a US general population sample were compared with Student's t-test. Multiple linear regression assessed demographic and clinical factors associated with resilience. RESULTS: The mean resilience score for participants (n=195; 49 males, 146 females; mean age 40y 2mo [SD 12y 7mo] range 18-74y) was 27.2 (SD 7.5), which differed from a mean of 31.8 (SD 5.4) for a US general population sample (p<0.01). Multiple linear regression demonstrated significant positive associations between resilience and older age (p=0.04), prior urological surgeries (p=0.03), higher household education (p<0.01), and higher physical function (p<0.01). INTERPRETATION: Resilience in individuals with spina bifida is moderately poor, relative to the general population, and is associated with certain demographic and clinical factors. As a modifiable construct with positive effects on quality of life, psychological well-being, and health-related behaviors, resilience is a promising target for intervention in individuals with spina bifida. What this paper adds Resilience in individuals with spina bifida is moderately poor. Resilience is lower in individuals with spina bifida than the general population. Resilience is associated with age, household education, physical function, and urological surgery.

Translation and validation of the brazilian version of the quality of life assessment in spina bifida questionnaire for children and teenagers / Tradução e validação da versão brasileira do questionário quality of life assessment in spina bifida para crianças e adolescentes

ABSTRACT Objective: To assess the reliability and validity of the Quality of Life Assessment in Spina Bifida (QUALAS), children and teenager's versions (QUALAS C and T, respectively). This is the first self-applicable quality of life assessment tool for patients under 13 years of age, which also addresses the issue of urinary and fecal incontinence. Methods: Two urologists performed the translation of both QUALAS versions. A commission produced a consensus version (Version 2), which was applied as a pilot study to define Version 3. It was then backtranslated into English and compared with the original version for equivalence of concepts. Internal consistency with Cronbach's alpha and the intraclass correlation coefficient (ICC) reproducibility was analyzed after two assessments with an interval from two to four weeks. Convergent and divergent validities between the QUALAS and a generic health-related quality of life questionnaire, the KIDSCREEN-27, were studied through Pearson's correlation. Results: The reliability analysis showed good internal consistency for QUALAS-C (α=0.73) and QUALAS-T (α=0.79) and good reproducibility in both questionnaires (QUALAS-C - ICC=0.86; QUALAS-T - ICC=0.92). For QUALAS-C convergent validity, there was a low correlation between its items (r=0.35). In addition, a low correlation was also found in the divergent validity analysis, when compared to the KIDSCREEN-27 (r≤0.33). Convergent and divergent validities of the QUALAS-T questionnaire had similar results: r=0.46 and r≤0.49, respectively. Conclusions: After the adaptation and validation process, QUALAS-C and QUALAS-T questionnaires showed to be reliable and valid instruments for measuring the health-related quality of life of children and teenagers with spina bifida aged 8 years or older.

RESUMO Objetivo: Avaliar a confiabilidade e a validade do questionário QUALAS (Quality of Life Assessment in Spina Bifida) nas versões para crianças e adolescentes (QUALAS-C e QUALAS-T, respectivamente). Este é o primeiro instrumento autoaplicável de avaliação da qualidade de vida para pacientes menores de 13 anos e que também aborda a questão das incontinências urinária e fecal. Métodos: Dois urologistas realizaram a tradução das duas versões do QUALAS. Uma comissão produziu uma versão de consenso (Versão 2), a qual foi aplicada no estudo piloto para definir a Versão 3. Esta foi retrotraduzida para o inglês e comparada à versão original para equivalência de conceitos. Para verificar a confiabilidade, analisou-se a consistência interna com o alfa de Cronbach e a reprodutibilidade com o coeficiente de correlação intraclasse (CCI) após duas aplicações do questionário em intervalo de duas a quatro semanas. As validades convergente e divergente foram estudadas por meio da correlação de Pearson entre o QUALAS e um questionário genérico de qualidade de vida relacionada à saúde, o KIDSCREEN-27. Resultados: A análise de confiabilidade revelou que ambos os questionários apresentaram boa consistência interna (QUALAS-C - α=0,73; QUALAS-T - α=0,79) e boa reprodutibilidade (QUALAS-C - CCI=0,86; QUALAS-T - CCI=0,92). Na análise da validade convergente do QUALAS-C, observou-se baixa correlação entre os itens (r=0,35). Além disso, a análise da validade divergente também demonstrou baixa correlação quando comparada ao KIDSCREEN-27 (r≤0,33). As validades convergente e divergente do questionário QUALAS-T tiveram resultados semelhantes: r=0,46 e r≤0,49, respectivamente. Conclusões: Após o processo de adaptação e validação, pode-se afirmar que os questionários QUALAS-C e QUALAS-T são instrumentos confiáveis e válidos para a mensuração da qualidade de vida relacionada à saúde de crianças e adolescentes com espinha bífida a partir dos 8 anos de idade.

Measures of Health-Related Quality of Life Outcomes in Pediatric Neurosurgery: Literature Review.

BACKGROUND: Improving value in healthcare means optimizing outcomes and minimizing costs. The emerging pay-for-performance era requires understanding of the effect of healthcare services on health-related quality of life (HRQoL). Pediatric and surgical subspecialties have yet to fully integrate HRQoL measures into practice. The present study reviewed and characterized the HRQoL outcome measures across various pediatric neurosurgical diagnoses. METHODS: A literature review was performed by searching PubMed and Google Scholar with search terms such as "health-related quality of life" and "pediatric neurosurgery" and then including the specific pathologies for which a HRQoL instrument was found (e.g., "health-related quality of life" plus "epilepsy"). Each measurement was evaluated by content and purpose, relative strengths and weaknesses, and validity. RESULTS: We reviewed 68 reports. Epilepsy, brain tumor, cerebral palsy, spina bifida, hydrocephalus, and scoliosis were diagnoses found in reported studies that had used disease-specific HRQoL instruments. Information using general HRQoL instruments was also reported. Internal, test-retest, and/or interrater reliability varied across the instruments, as did face, content, concurrent, and/or construct validity. Few instruments were tested enough for robust reliability and validity. Significant variability was found in the usage of these instruments in clinical studies within pediatric neurosurgery. CONCLUSIONS: The HRQoL instruments used in pediatric neurosurgery are currently without standardized guidelines and thus exhibit high variability in use. Clinicians should support the development and application of these methods to optimize these instruments, promote standardization of research, improve performance measures to reflect clinically modifiable and meaningful outcomes, and, ultimately, lead the national discussion in healthcare quality and patient-centered care.

Sonhar-se existindo: a produção onírica em um atendimento infantil como reveladora para a compreensão clínica / Dreaming of existing: oniric production in child consulting as revealing to the clinical understanding / Soñarse existiendo: la producción onírica en un atendimiento infantil como reveladora para la comprensión clínica

A relação terapeuta-paciente em psicanálise dá-se para além do setting analítico, incluindo a produção onírica, entre outros conteúdos. Este artigo relata a experiência formativa de uma terapeuta que, durante as entrevistas iniciais com a família de uma paciente infantil, foi capaz de capturar a essência subjacente do que foi dito e converter esse conteúdo em material onírico. Essa elaboração serviu de esteio para a apropriação realizada pelos pais nas sessões seguintes. A partir dessa nova condição, o desenvolvimento saudável da criança tornou-se possível e desejado. Discute-se a importância do processo do sonhar como parte essencial da própria existência.

The therapist-patient relationship in psychoanalysis goes beyond the analytic setting, including the oneiric production, among other contents. This article reports the formative experience of a therapist who during the initial interviews with the family of a child patient was able to capture the underlying essence of what was said and convert that content into dream material. This elaboration served as a mainstay for the appropriation carried out by the parents in the following sessions. From this new condition, the child's healthy development has become possible and desired. The importance of the process of dreaming is discussed as an essential part of existence itself.

la relación terapeuta-paciente en el psicoanálisis está más allá del encuadre analítico, incluyendo la producción onírica, entre otros contenidos. Este artículo relata la experiencia formadora de una terapeuta que durante las entrevistas iniciales con la familia de una paciente infantil, fue capaz de capturar la esencia subyacente de lo que se dijo y convertir ese contenido en material de sueño. Esa elaboración sirvió como pilar para la apropiación hecha por los padres en las siguientes sesiones. A partir de esa nueva condición, el desarrollo saludable del niño se hizo posible y deseado. Se discute la importancia del proceso de soñar como parte esencial de la propia existencia.

Attention lapses in children with spina bifida and hydrocephalus and children with attention-deficit/hyperactivity disorder.

Attentional lapses are usually defined as temporary and often brief shifts of attention away from some primary task to unrelated internal information processing. This study addressed the incidence of attention lapses and differences in attentional functioning in 30 children with attention-deficit/hyperactivity disorder (ADHD), 26 healthy children, and 29 children with spina bifida myelomeningocele and hydrocephalus (SBH). Assessments were conducted using computerized tonic and phasic attention tests, the Symbol Digit Modalities Test (SDMT), and the Trail Making Test Form B (TMT-B). The group with SBH differed from normal controls on cognitive measures of attention and executive functions. The ADHD group obtained lower scores than the SBH group and healthy children. ANOVA results showed that there was an effect of shunt revisions and shunt-related infections on neuropsychological performance. Lapses of attention together with reaction time may thus represent important factors for the understanding of cognitive deficits in SBH.

Lateralidad, perfil cognitivo y rendimiento escolar en pacientes con mielomeningocele / Laterality, cognitive profile, and school performance in children with myelomeningocele

El mielomeningocele (MMC) es la forma más grave de los disrafismos de la columna vertebral. Afecta a 1/1200 recién nacidos vivos. Su etiología es multifactorial. El 80% cursa con hidrocefalia (HC) asociada a malformación de Arnold Chiari. El coeficiente intelectual (CI) oscila en un rango entre fronterizo y normal promedio. La tasa mundial de la población general de zurdos es del 10% pero se describe un aumento de su presentación en niños con MMC. Además se evidencian déficits visoespaciales, grafomotores, atencionales, memorísticos, dificultades en aritmética y en la comprensión de textos. Materiales y métodos: Este es un trabajo analítico, descriptivo, transversal. Se evaluaron 179 pacientes derivados del Consultorio Interdisciplinario de MMC; edad: entre 5 años, 0 meses y 15 años, 11 meses. No se emplearon controles sanos. Instrumentos administrados: Stanford Binet IV, Prolec ­ Prolec-Se, Wrat3, TMT A y B, d2, Stroop y pruebas de lateralidad de Zazzo. Objetivo: Describir la lateralidad, el perfil cognitivo y el rendimiento escolar en pacientes pediátricos con patología de mielomeningocele, analizando también las funciones ejecutivas, los procesos atencionales, memorísticos y la modalidad escolar en la cual se encuentran inmersos. Resultados: El 20% (35/179 pacientes) con edades que oscilan entre los 5 años, 0 meses y 15 años, 11 meses han presentado lateralidad zurda, y de ellos presentaban antecedentes familiares de zurdera 26%. Se observó lateralidad cruzada en 6% y eran ambidiestros 11%. El perfil cognitivo de la muestra completa (N: 179) fue homogéneo en todas las áreas (verbal, abstracto/visual y memoria de corto plazo), arrojando puntuaciones fronterizas. En pruebas atencionales el 100% obtuvo niveles descendidos. Todos reportaron dificultades en el rendimiento escolar. Sólo el 14% tuvo plan de integración y el 11% accedió a algún tipo de tratamiento. Conclusiones: Los niños con MMC evidencian déficits neuropsicológicos y alta tasa de zurdera, siendo probablemente los problemas de lateralidad una de las causas que ocasionarían el bajo rendimiento escolar y afectando el rendimiento académico (AU)

Myelomeningocele (MMC) is the most severe form of spinal dysraphism, affecting 1/1200 live newborns. Its etiology is multifactorial. Overall, 80% develops hydrocephalus (HC) associated with an Arnold Chiari malformation. The intelligence quotient (IQ) ranges from borderline to average. The worldwide incidence in the general population of left-handedness is 10%; however, an increase is found among children MMC. Additionally, visuospatial, graphomotor, attention, memory deficits as well as difficulties in arithmetic and text comprehension is found. Material and methods: A cross-sectional, analytical, descriptive study was conducted. Overall, 179 patients aged between 5 years 0 months and 15 years 11 months referred from the interdisciplinary MMC clinic were evaluated. No healthy control group was used. Instruments administrated: Stanford Binet IV, Prolec ­ Prolec-Se, Wrat3, TMT A and B, d2, Stroop and Zazzo's laterality test. Aim: To describe laterality, cognitive profile, and school performance in pediatric patients with MMC, analyzing executive functions, attention and memory, and the school system the children were attending. Results: 20% (35/179 patients) aged between 5 years, 0 months and 15 years, 11 months were left-handed, 26% of whom had a family history of left-handedness. Cross dominance was observed in 6% and ambidexterity in 11%. Overall, the cognitive profile of the sample (N: 179) was homogeneous in all areas (verbal, abstract/visual, and short-term memory), with borderline scores. In attention tasks, levels were low in 100%. All children had difficulties in school performance. Only 14% had an integration program and 11% received some type of treatment. Conclusions: Children with MMC show neuropsychological deficits and a high rate of left-handedness, the latter of which may be one of the causes of poor school performance and affecting academic achievements (AU)

Central Somatosensory Networks Respond to a De Novo Innervated Penis: A Proof of Concept in Three Spina Bifida Patients.

INTRODUCTION: Spina bifida (SB) causes low spinal lesions, and patients often have absent genital sensation and a highly impaired sex life. TOMAX (TO MAX-imize sensation, sexuality and quality of life) is a surgical procedure whereby the penis is newly innervated using a sensory nerve originally targeting the inguinal area. Most TOMAX-treated SB patients initially experience penile stimulation as inguinal sensation, but eventually, the perception shifts to penis sensation with erotic feelings. The brain mechanisms mediating this perceptual shift, which are completely unknown, could hold relevance for understanding the brain's role in sexual development. AIM: The aim of this study was to study how a newly perceived penis would be mapped onto the brain after a lifelong disconnection. METHODS: Three TOMAX-treated SB patients participated in a functional magnetic resonance imagery experiment while glans penis, inguinal area, and index finger were stimulated with a paint brush. MAIN OUTCOME MEASURE: Brush stimulation-induced activation of the primary somatosensory cortex (SI) and functional connectivity between SI and remote cerebral regions. RESULTS: Stimulation of the re-innervated side of the glans penis and the intact contralateral inguinal area activated a very similar location on SI. Yet, connectivity analysis identified distinct SI functional networks. In all three subjects, the middle cingulate cortex (MCC) and the parietal operculum-insular cortex (OIC) were functionally connected to SI activity during glans penis stimulation, but not to SI activity induced by inguinal stimulation. CONCLUSIONS: Investigating central somatosensory network activity to a de novo innervated penis in SB patients is feasible and informative. The consistent involvement of MCC and OIC above and beyond the brain network expected on the basis of inguinal stimulation suggests that these areas mediate the novel penis sensation in these patients. The potential role of MCC and OIC in this process is discussed, along with recommendations for further research.

Health related quality of life and mental health in children with SCI/D from Neiva, Colombia.

OBJECTIVE: To date, no research has been published on the health related quality of life (HRQOL) and mental health of children with spinal cord injury and disorders (SCI/D) in Latin America, although limited previous research in Western countries has demonstrated the debilitating and chronic nature of these conditions in children. The aim was to examine the connections between HRQOL and mental health in children with SCI/D from Neiva, Colombia. STUDY DESIGN: Thirty children (8- 17 years) were recruited from the Hospital Universatario Hernando Mocaleano Perdomo in Neiva, Colombia. Participants completed self-report measures administered verbally by trained research staff. RESULTS: A correlation matrix generally suggested that higher HRQOL was robustly associated with better mental health. A series of multiple regressions found that HRQOL explained 50.5% of the variance in children's depression, 31.5% of the variance in worry, and 41.9% of the variance in social anxiety. Within these regressions, emotional and social functioning were uniquely associated with depression, and emotional functioning was uniquely associated with social anxiety. CONCLUSION: This is the first published study to examine psychosocial outcomes in children with SCI/D in Latin America, and its findings suggest that future research and interventions for children with SCI/D in Colombia - and possibly in other regions of Latin America - would benefit from emphasizing emotional and social functioning.

The influence of social adjustment on normative and risky health behaviors in emerging adults with spina bifida.

OBJECTIVE: To understand the rates of normative and risky health behaviors and the influence of prior and current social adjustment on health risk behaviors in emerging adults with spina bifida (SB). METHOD: These data are part of a larger longitudinal study of youth with SB; at ages 18-19, 50 emerging adults with SB and 60 typically developing (TD) youth participated. Social adjustment was measured at ages 12/13, 14/15, 16/17, and 18/19. Substance use and sexual activity were self-reported by emerging adults. RESULTS: The SB group reported similar frequencies (i.e., number of days in the previous month) of cigarette and marijuana use. Fewer individuals with SB reported initiation of both alcohol use (i.e., ever used) and sexual activity (i.e., ever had sex) compared to TD peers. The SB group also reported less frequent alcohol use and fewer sexual partners. Better social adjustment during early adolescence (ages 12/13) predicted more frequent alcohol use and a greater number of sexual partners for all youth. Social adjustment also mediated the effect of group status on health risk behaviors. CONCLUSIONS: Emerging adults with SB lag behind TD peers in terms of normative initiation of alcohol use and sexual activity. However, this population participates in some risky health behaviors at similar rates compared to their TD peers (e.g., smoking). Youths' health risk behaviors may be influenced by their level of social adjustment. A challenge for future interventions for this population will be finding methods of improving social functioning without increasing the rate of health risk behavior.

Sexual function of young women with myelomeningocele.

OBJECTIVES: To assess the sexual function of young women with spina bifida and myelomeningocele and to determine the factors influencing their sexual function. METHODS: A postal cross-sectional study using a self-administered questionnaire was performed in 44 women, mean age 27.66 ± 5.89 years, with spina bifida and myelomeningocele. The questionnaire included the Brief Index of Sexual Functioning for Women and questions about voiding mode, urinary symptoms, socioeconomic status, education level, lifestyle, and partnership. In parallel, data were also collected from the paediatric surgery records of patients who returned the questionnaire. RESULTS: The response rate was 56.8% (25/44). All domains of female sexual function (thoughts/desires, arousal, frequency of sexual activity, receptivity/initiation, pleasure/orgasm, relationship satisfaction) were altered. Urinary incontinence was likely to be the main factor responsible for altered sexual function and was associated with lower thoughts/desires, arousal, and receptivity/initiation scores. Wearing pads also constituted a limitation to achieving intimacy. CONCLUSIONS: Young myelomeningocele women report poor sexual functioning. The presence of urinary incontinence is associated with lower thoughts/desire, arousal, and receptivity/initiation.

Reinnervating the penis in spina bifida patients in the United States: ilioinguinal-to-dorsal-penile neurorrhaphy in two cases.

INTRODUCTION: Penile sensation is absent in some patients with myelomeningocele owing to the dysfunction of the pudendal nerve. Here, we describe the introduction of penile sensation via ilioinguinal-to-dorsal-penile neurorrhaphy in two patients with penile anesthesia due to neural tube defects. AIM: To establish penile sensation via ilioinguinal-to-dorsal-penile-nerve neurorrhaphy. METHODS: A 20-year-old and a 35-year-old male with L5/S1 myelomeningocele were both highly functioning and ambulatory, with intact ilioinguinal nerve distribution sensation but anesthesia of the penis and glans. They were sexually active and able to ejaculate antegrade. Both had high International Index of Erectile Function scores for confidence to achieve erection sufficient for intercourse. An incision was made from anterior superior iliac crest to the glans penis to expose the inguinal canal and ilioinguinal nerve. The ilioinguinal and dorsal penile nerve were transected and anastomosed. The anastomotic site was then wrapped in a hemostatic agent and a drain was left in place. For penile rehabilitation, both patients were instructed to stimulate the penis while looking at the genitalia to encourage redistribution of perceived sensation. MAIN OUTCOME MEASURES: Presence of erogenous penile sensation was tested by neurologic examination and patient feedback, and patients completed sexual health questionnaires. RESULTS: Both patients reported paresthesias of the groin with penile stimulation 1 month after surgery. Both patients are now 24 months postoperative and have erogenous sensation on the ipsilateral glans and shaft during intercourse. Neither patient has difficulty achieving or maintaining erections. CONCLUSIONS: We present two patients with dorsal penile reinnervation via the ilioinguinal nerve. Although nerve reinnervation has been used in urological procedures, this is the first description of an attempt to resupply penile sensation via the dorsal penile nerve in the United States with a minimum of 18 months follow-up. Early follow-up suggests successful neuronal remapping and regained sensation of the penis.

Health risk behaviors among young adults with spina bifida.

AIM: Persons with spina bifida who adopt unhealthy lifestyles could be at increased risk of adverse health outcomes because the presence of spina bifida may magnify this risk. We estimated overall and age-specific prevalence of selected health risk behaviors (HRBs) in young people with spina bifida and examined the association between HRBs and depression. METHOD: We performed analyses on data obtained from individuals with spina bifida (n=130; mean age 23y SD 4y 5mo; 64 males, 66 females; 64% lumbosacral lesion; 77% with shunt) who participated in a population-based survey conducted by the Arkansas Spinal Cord Commission in 2005. RESULTS: Compared with national estimates, young people with spina bifida tend to eat less healthy diets, do less exercise, and engage in more sedentary activities. Respondents were less likely to use substances (alcohol, tobacco, illegal drugs), which peaked among 25 to 31 year olds. About 90% saw a doctor in the previous year. Nearly one half reported mild or major depressive symptoms. In the logistic regression analysis after controlling for potential confounders (age, sex, ethnic group, education, employment, marital status, living arrangement, level of lesion, presence of shunt, mobility, self-rated health and healthcare utilization), major depressive symptoms were associated with current alcohol drinking (adjusted odds ratio: 4.74; 95% CI 1.18-19.04). INTERPRETATION: Young adults with spina bifida exhibit unhealthy behaviors that continue into their late 20s. The findings highlight the need to increase awareness of their health risk profiles in the spina bifida community and show opportunities for mental health and health risk screening and counseling by healthcare providers.

Adult consequences of spina bifida: a cohort study.

BACKGROUND: Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated. QUESTIONS/PURPOSE: In adults with spina bifida we determined the cause of death for those who had died, IQ, level of education, work history, independent living status, physical disabilities, and urologic status; we then identified surgical procedures that led to better adult function. METHODS: We retrospectively reviewed 84 individuals with myelomeningocele, examining social, cognitive, and physical disabilities. The minimum age at followup was 20 years (mean, 31 years; range, 20-64 years). RESULTS: Forty-two percent had normal IQs. Seventy percent never needed a neurosurgical shunt and those without shunts had higher IQs. Forty-four percent had regular education and 8% achieved college degrees. Fifty-six percent were unemployed. Thirty percent lived independently. Twenty-three percent were either married or divorced with nine normal offspring. Eighty-five percent dressed themselves, 65% shopped independently, 54% drove. Thirty-one percent were at the thoracic neurologic level (all used wheelchairs), 12% were at L1-L3 (all used a wheelchair except one), 33% were at L4-L5 (78% used a wheelchair at least part-time), and 24% were at S1 and below (all walked). Fifty-four percent experienced decubiti and as a consequence, four required major extremity amputations. Spinal fusions protected sitting balance, but hip surgery did not produce congruent hips and occasionally resulted in debilitating stiffness. Pressure sores resulted in partial foot amputations despite plantigrade feet. CONCLUSIONS: Our observations confirm the consequences associated with the physical and cognitive disabilities of patients with spina bifida. Many of these consequences can likely be mitigated by modern multidisciplinary care and special education to increase independence.

Computer-mediated support for adolescents with cerebral palsy or spina bifida.

Social support plays a key role in improving health outcomes for children with chronic conditions. Internet connections are an important component of adolescents' social networks and may overcome geographic and environmental barriers for those with disabilities. This article focuses on the processes associated with a 6-month online support intervention for adolescents with cerebral palsy or spina bifida. Specifically, the purpose was to determine the extent to which adolescents used an online peer support intervention, the processes used, and the perceived benefits and satisfaction with the intervention. Five peer mentors with the same disabilities provided information, affirmation, and emotional support. The online environment created a safe space to foster reciprocal interpersonal connections and appropriate social comparison. Two-thirds of the participants viewed the computer-mediated support intervention as fun. Factors influencing the perceived utility of the intervention included typing speed, cognitive skills, and perceived need for additional support. Girls were significantly more likely to contribute messages than were boys. Peer mentors wished that this type of support program had been available when they were teens, appreciated the supportive elements, and reported learning from the teen participants. Health professionals wanting to implement online support need to consider the age and ability levels of participants and the optimal length and format of the support program.