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INTRODUCTION: The transition from paediatric to adult medical care is a critical period for adolescents with spina bifida, often marked by deteriorating health and functional status. OBJECTIVES: To describe the health status and functioning of individuals with spina bifida at the time of transition from paediatric to adult care and to identify risk profiles for potentially modifiable secondary health conditions (urinary tract infections, pressure injuries, respiratory problems and obesity). METHODS: Descriptive study of data (derived from medical records) from all adolescents and young adults with spina bifida aged 15-25 years who were referred to a single specialised spinal cord injury centre as part of the transition programme during the period from 1 September 2015 to 31 May 2022. Descriptive statistics were used to describe the study population and to analyse the frequency and co-occurrence of congenital and acquired secondary health conditions. The equality of proportions test was used to test the prevalence of secondary health conditions based on important personal and clinical characteristics. RESULTS: We included 43 adolescents with spina bifida with a mean age of 18.4 years (SD 2.5); in 63% of them the neurological level was lumbar, and around 50% of them were wheelchair-dependent. The median Spinal Cord Injury Measure (SCIM) III score was 72 (IQR 61-89). The mean number of secondary health conditions at time of transition was 8.8. The most prevalent secondary health conditions were lower urinary tract dysfunction, bowel dysfunction, sexual dysfunction and contractures. Respiratory problems were more prevalent in females and in individuals with lower SCIM III scores, whereas no differences were observed in the prevalence of other modifiable secondary health conditions. Clustering of secondary health conditions was mainly seen for urinary tract infection + pressure injury and for urinary tract infection + pressure injury + obesity. CONCLUSION: The prevalence of secondary health conditions among individuals with spina bifida at time of transition is alarmingly high and functional profiles underscore the need for supporting adolescents and young adults with daily medical issues. The study highlights the critical role of transition programmes and interdisciplinary follow-up care in preventing health problems and improving functioning and independence in everyday life.
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Estado de Salud , Disrafia Espinal , Transición a la Atención de Adultos , Infecciones Urinarias , Humanos , Disrafia Espinal/epidemiología , Disrafia Espinal/complicaciones , Adolescente , Femenino , Masculino , Estudios Transversales , Transición a la Atención de Adultos/estadística & datos numéricos , Adulto Joven , Infecciones Urinarias/epidemiología , Úlcera por Presión/epidemiología , Adulto , Factores de Riesgo , Obesidad/epidemiología , Obesidad/complicaciones , PrevalenciaRESUMEN
OBJECTIVE: Maldevelopment of the posterior corpus callosum is common in spina bifida myelomeningocele (SBM) due to hydrocephalus-related hypoplasia and congenital partial hypogenesis. This study examined the relations of macro- and microstructural integrity of the interhemispheric temporal tract in SBM and auditory interhemispheric transfer using consonant-vowel dichotic listening. METHOD: We collected T1-weighted and diffusion tensor imaging data from 46 individuals with SBM and 15 typically developing individuals. Probabilistic tractography was used to isolate the interhemispheric white matter connecting auditory processing regions in both hemispheres. Interhemispheric transfer was assessed with a dichotic listening task. RESULTS: Although the typically developing group and the group with SBM showed the normative right-ear advantage, fewer participants showed a right-ear advantage in the group with SBM. The absence of the right-ear advantage was largely in the subgroup with hypogenesis of the splenium or severe posterior hypoplasia. Sex, anterior commissure cross-sectional area, and number of shunt pathways visible on magnetic resonance imaging predicted right-ear superiority. CONCLUSIONS: Interhemispheric transfer is disrupted in individuals with SBM and hypogenesis or severe hypoplasia of the posterior corpus callosum. Preservation of interhemispheric transfer is related to expected connections through the posterior corpus callosum and possibly compensatory pathways in the anterior commissure. (PsycInfo Database Record (c) 2024 APA, all rights reserved).
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Cuerpo Calloso , Imagen de Difusión Tensora , Lateralidad Funcional , Meningomielocele , Disrafia Espinal , Humanos , Masculino , Femenino , Meningomielocele/complicaciones , Cuerpo Calloso/diagnóstico por imagen , Cuerpo Calloso/patología , Adolescente , Adulto , Disrafia Espinal/complicaciones , Disrafia Espinal/fisiopatología , Adulto Joven , Lateralidad Funcional/fisiología , Niño , Pruebas de Audición Dicótica , Percepción Auditiva/fisiología , Imagen por Resonancia MagnéticaRESUMEN
INTRODUCTION: Clean intermittent catheterisation (CIC) is the standard of care for treating neurogenic lower urinary tract dysfunction (NLUTD), the most common bladder dysfunction in children diagnosed with spinal dysraphism (SD) and spinal cord injury (SCI). Failure to follow the prescribed CIC regimen results in urinary tract infections, incontinence and renal insufficiency. Adherence to CIC is suboptimal, with reported non-adherence rates of 18%-66%. Despite the efficacy of CIC, the research on CIC adherence is not well defined in the literature and even less for caregivers of children on CIC protocols. METHODS: This proposed study aims to identify caregiver CIC adherence and determinants while exploring the personal experiences of performing CIC from the perspective of caregivers of children with NLUTD due to SD and SCI. This cross-sectional, correlational, convergent mixed methods study design in which qualitative and quantitative data will be collected simultaneously will be used to study the level of adherence and the relationship of caregiver determinants to CIC in children with SD and SCI and adherence to the CIC protocol. Convenience sampling will be used to identify 60 adult caregivers who can read and write English or Spanish and have a child diagnosed with SD and SCI who is currently prescribed CIC by a urology provider. ANALYSIS: The adherence data will be reported as frequency and percentages. A correlation analysis will be computed to assess the association between determinants measured by the Clean Intermittent Catheterization-Caregiver Questionnaire and adherence levels measured with the Intermittent Catheterization Adherence Scale. Thematic analysis will be used to analyse and interpret the interview data. A comparison joint display will be developed to compare quantitative and qualitative data results. ETHICAL AND DISSEMINATION: Institutional review board approval was obtained from the Children's Mercy Kansas City (Study00003003) and the University of Missouri-Kansas City (#2100185). The study's main results will be disseminated to caregiver participants, published in peer-reviewed journals and presented at conferences.
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Cuidadores , Cateterismo Uretral Intermitente , Traumatismos de la Médula Espinal , Disrafia Espinal , Vejiga Urinaria Neurogénica , Humanos , Traumatismos de la Médula Espinal/complicaciones , Disrafia Espinal/complicaciones , Estudios Transversales , Vejiga Urinaria Neurogénica/terapia , Vejiga Urinaria Neurogénica/etiología , Niño , Femenino , Masculino , Infecciones Urinarias/etiología , Proyectos de Investigación , Cooperación del Paciente/estadística & datos numéricosRESUMEN
Spina bifida is a congenital malformation of foetal neural structures which may present as a skin fold or sac containing cerebrospinal fluid and neural structures with a variety of neurological deficits. Surgical repairs of spina bifida may not ensure complete functions, neural improvement or recovery. We present this palliative report of an adult male in his early 40s with a medical history of meningocele repair in his infancy, with long-standing Marjolin ulcers, fractures, contractures, diverting ileostomy and urostomy and a fungating mass externally measuring 33×25 cm. The mass involved the buttocks, perineum and scrotum with a tumour overlying the meningocele and extending into the thigh through an internally draining tract. Factors such as immobility, pressure injuries and poor social support in the setting of chronic disability led to a conservative approach in the management of this unresectable carcinoma.
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Carcinoma de Células Escamosas , Disrafia Espinal , Humanos , Masculino , Disrafia Espinal/complicaciones , Disrafia Espinal/cirugía , Carcinoma de Células Escamosas/cirugía , Carcinoma de Células Escamosas/complicaciones , Adulto , Escroto/cirugía , Nalgas , Perineo/cirugía , Cuidados Paliativos/métodos , Neoplasias Cutáneas/cirugía , Neoplasias Cutáneas/complicacionesRESUMEN
Background: Ultrasonography is not chosen as the common imaging modality to diagnose spinal cord diseases. The present report indicates good diagnostic efficacy of ultrasonography for identifying spinal cord compressed by subcutaneous mass when scanning through the defected vertebral laminae and spinous process. Case Description: A five-month-old female Holstein calf presented with progressive hindlimb paralysis following a surgical resection of a back mass conducted at 21 days of age. The mass was subsequently histopathologically diagnosed as a pulmonary choristoma. Alongside hindlimb paralysis, the calf developed a swollen back at the lumbar region where the mass was removed. This suggested regrowth of the resected mass, causing injury to the underlying spinal cord. Ultrasonography identified the subcutaneous involvement of the capsular mass, which had three anechoic cavities separated by the echogenic septal structures. The spinal cord could be ultrasonographically demonstrated as adjacent to the mass through the defected vertebral laminae and spinous process in the second and third lumbar vertebras. Ultrasound-guided centesis allowed the collection of purulent exudates in which Escherichia coli was isolated. Myelography and subsequent computed tomography (CT) revealed a partial blockage of the intradural flow of contrast media at the levels of the second and third lumbar vertebras, diagnosed as spina bifida on the CT images. When applying ultrasonography to the spinal cord within a saline pool soon after the subcutaneous abscess was successfully resected, the spinal cord was characterized by the interrupted and partly extended hyperechogenic line of the central canal within the echogenic parenchyma. The echotexture of the spinal cord showed damage due to compression from the subcutaneous abscess. The animal had a sub-optimal postoperative outcome, including limited improvement of the neurological signs. Conclusion: In the present case, combining ultrasonography, radiography (myelography), and CT was very effective for diagnosing spina bifida, with the subcutaneous abscess inducing spinal cord compression. Additionally, using intraoperative ultrasonographic scanning to evaluate the degree of spinal cord damage can contribute to predicting the postoperative outcome.
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Absceso , Enfermedades de los Bovinos , Ultrasonografía , Animales , Femenino , Bovinos , Enfermedades de los Bovinos/cirugía , Enfermedades de los Bovinos/diagnóstico , Enfermedades de los Bovinos/diagnóstico por imagen , Ultrasonografía/veterinaria , Absceso/veterinaria , Absceso/cirugía , Absceso/diagnóstico , Absceso/diagnóstico por imagen , Miembro Posterior , Parálisis/veterinaria , Parálisis/etiología , Parálisis/cirugía , Tomografía Computarizada por Rayos X/veterinaria , Coristoma/veterinaria , Coristoma/cirugía , Coristoma/complicaciones , Disrafia Espinal/veterinaria , Disrafia Espinal/complicaciones , Disrafia Espinal/cirugía , Disrafia Espinal/diagnóstico por imagenRESUMEN
BACKGROUND: Children with spina bifida (SB) face an elevated risk of obesity, which necessitates precise methods for assessing body composition to ensure effective weight management. Conventional measures like BMI are inadequate for this population because of variations in growth patterns and skeletal structure. Bioelectrical impedance analysis (BIA) is a method that offers a clearer picture of body composition, yet its use in children with SB remains underexplored. METHODS: Conducted on 57 children with SB and 28 healthy controls, with a median age of 11 years, this study evaluated anthropometrics, including BMI and BIA-derived metrics. The Hoffer's scale to assess physical activity was applied in SB children. RESULTS: Results showed that while 32% of SB patients were classified as overweight or obese based on BMI, 62% exhibited high body fat percentage via BIA. Fat-free mass, muscle and fat mass, and fat-to-muscle ratio (FMR) differed significantly compared to the reference group. Non-ambulators showed a higher median body fat mass percentage (25.9% vs. 17.8%, p = 0.01) and FMR (0.92 vs. 0.44, p = 0.003) in comparison to the community walkers. CONCLUSIONS: In SB children, BIA-measured fat mass is a better obesity indicator than BMI. Non-ambulatory, SB patients with obesity had the highest FMR values, indicating a higher risk for metabolic syndrome.
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Composición Corporal , Índice de Masa Corporal , Impedancia Eléctrica , Disrafia Espinal , Humanos , Disrafia Espinal/complicaciones , Disrafia Espinal/fisiopatología , Niño , Femenino , Masculino , Adolescente , Obesidad Infantil/terapia , Obesidad Infantil/fisiopatología , Estudios de Casos y ControlesRESUMEN
BACKGROUND: This commentary discusses the social impact of bowel management programs (BMPs) on children with colorectal diseases, including anorectal malformations (ARM), Hirschsprung disease (HD), functional constipation (FC), and spina bifida. Previous studies focused on functional outcomes, but this study bridges the gap to daily life experiences. METHODS: The study examined children's experiences in BMPs, focusing on school participation, vacation ability, and overall patient experience. Cleanliness, defined as fewer than one stool soiling episode per week, was achieved by 70% of participants. RESULTS: Positive patient experiences were linked to achieving stool cleanliness, regardless of the management method. Invasive methods like enemas did not negatively affect experiences if cleanliness was maintained. Validated patient-reported experience measures (PREMs) and patient-reported outcomes measures (PROMs) were used, though the median age of 8.9 years posed limitations. CONCLUSION: The commentary highlights the significance of stool cleanliness in improving patient experiences and supports the effectiveness of various BMP methods. Future research should include longitudinal follow-ups to assess BMP durability and gather data from older children.
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Malformaciones Anorrectales , Estreñimiento , Disrafia Espinal , Humanos , Niño , Estreñimiento/terapia , Malformaciones Anorrectales/cirugía , Disrafia Espinal/psicología , Disrafia Espinal/complicaciones , Enfermedad de Hirschsprung/terapia , Medición de Resultados Informados por el Paciente , Incontinencia Fecal/terapia , Incontinencia Fecal/psicología , Femenino , Masculino , Satisfacción del Paciente/estadística & datos numéricosRESUMEN
STUDY DESIGN: Retrospective and cross-sectional study. OBJECTIVES: 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. SETTING: 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. CONCLUSION: 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.
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COVID-19 , Disrafia Espinal , Humanos , Adolescente , Masculino , Femenino , Disrafia Espinal/rehabilitación , Disrafia Espinal/complicaciones , Niño , Estudios Transversales , COVID-19/epidemiología , Estudios Retrospectivos , Telemedicina , Pandemias , Cuidadores , Modalidades de FisioterapiaRESUMEN
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.
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Extrofia de la Vejiga , Gastrosquisis , Disrafia Espinal , Humanos , Gastrosquisis/cirugía , Gastrosquisis/diagnóstico , Extrofia de la Vejiga/cirugía , Extrofia de la Vejiga/complicaciones , Disrafia Espinal/cirugía , Disrafia Espinal/complicaciones , Disrafia Espinal/diagnóstico por imagen , Recién Nacido , Femenino , Anomalías Múltiples/cirugía , Embarazo , Deformidades Congénitas de las Extremidades/cirugía , AdultoRESUMEN
OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
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Hidrocefalia , Disrafia Espinal , Humanos , Disrafia Espinal/cirugía , Disrafia Espinal/complicaciones , Hidrocefalia/cirugía , Proyectos Piloto , Masculino , Femenino , Estudios Retrospectivos , Adulto Joven , Adolescente , Adulto , Transición a la Atención de Adultos/tendencias , Neurocirugia/métodos , Procedimientos Neuroquirúrgicos/métodos , Niño , NeurocirujanosRESUMEN
PURPOSE: We aim to estimate the odds of UTI-related hospital care in spina bifida (SB) patients aged 18 to 25 years as compared with patients with SB in adolescence (11-17 years) or adulthood (26-35 years). We hypothesize that patients with SB in the typical transitional age, 18 to 25 years, will have higher odds of UTI-related hospital care as compared to adolescent SB patients or adult SB patients. MATERIALS AND METHODS: Using Cerner Real-World Data, we performed a retrospective cohort analysis comparing SB patients to age- and gender-matched controls. SB cases between 2015 and 2021 were identified and compared in 3 cohorts: 11 to 17 years (adolescents), 18 to 25 years (young adults [YA]), and 26 to 35 years (adults). Logistic regression analysis was used to characterize the odds of health care utilization. RESULTS: Of the 5497 patients with SB and 77,466 controls identified, 1839 SB patients (34%) and 3275 controls (4.2%) had at least 1 UTI encounter. UTI-related encounters as a proportion of all encounters significantly increased with age in SB patients (adolescents 8%, YA 12%, adult 15%; P < .0001). Adjusting for race, sex, insurance, and comorbidities, the odds of a UTI-related encounter in YA with SB were significantly higher than for adolescents with SB (adolescent odds ratio = 0.65, 95% CI: 0.57-0.75, P < .001). YA had lower odds of a UTI-related encounter as compared with adults with SB (adult odds ratio = 1.31, 95% CI: 1.16-1.49, P < .001). CONCLUSIONS: YA with SB have higher odds of UTI-related hospital care than adolescents, but lower odds of UTI-related hospital care when compared with adults.
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Disrafia Espinal , Infecciones Urinarias , Humanos , Disrafia Espinal/complicaciones , Disrafia Espinal/epidemiología , Masculino , Adolescente , Femenino , Adulto , Adulto Joven , Estudios Retrospectivos , Infecciones Urinarias/epidemiología , Infecciones Urinarias/etiología , Niño , Hospitalización/estadística & datos numéricos , Factores de Edad , Estudios de Casos y ControlesRESUMEN
INTRODUCTION: The Set Brave Goals app is the first digital health app (DHA) aimed at helping children with spina bifida (SB) aged 8-17 years old to select and track their urinary and fecal continence goals. Developed by children, parents, providers and researchers, its usability, or appropriateness to a purpose ("user-friendliness"), remains unknown. In an alpha testing stage, our aim was to determine the usability of the app prior to clinical use. METHODS: We recruited children with SB and their parents in clinic and via social media. A recruitment goal of 10 children exceeded industry standard of 5 participants (10 participants are expected to identify approximately 96% of usability problems). Participants downloaded and used the app for a week. They completed a questionnaire, including the System Usability Scale (SUS) and closed and open-ended questions. The SUS is a 10-item validated usability questionnaire most widely used in DHA usability testing (scores range from 0 to 100, 100 representing greatest usability). Median/mean SUS scores for DHAs are 68. Mann-Whitney-U and t-tests were used. RESULTS: Ten children with SB participated (median age: 14 years old, 6 female, 8 shunted, 8 using bladder catheterizations). Twenty parents participated (17 mothers, median age: 42 years old). Median SUS score was 77.5 for children and 73.8 for parents, corresponding to "good" to "excellent" usability (Summary Figure). There were no significant differences between child/parent scores (p = 0.69) or those for other DHAs (p = 0.11). It took a median 5-10 min to go through the app (2 parents felt it took too much time). All participants felt the app was easy to understand and use (100%). Most participants would recommend it to children and parents (children: 89%, parents: 80%), and believed it would be valuable to children (90%, 75%) and urologists (80%, 80%). Free text responses related to more varied color schemes, keeping free-text notes within the app and more flexible goal/alarm setting. These changes were incorporated into the app. COMMENT: Usability testing prior to clinical launch identified areas for app improvement. Although this study sample met industry standards, findings are limited by a small group of participants. The app will undergo further refinement during prospective beta testing. CONCLUSIONS: The Set Brave Goals app for children with SB has acceptable usability parameters. This justifies proceeding with wider use to formalize children's continence goal setting/tracking and testing its value to children with SB and their healthcare providers.
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Incontinencia Fecal , Aplicaciones Móviles , Disrafia Espinal , Incontinencia Urinaria , Humanos , Disrafia Espinal/complicaciones , Niño , Adolescente , Femenino , Masculino , Incontinencia Urinaria/etiología , Incontinencia Urinaria/diagnóstico , Encuestas y Cuestionarios , Incontinencia Fecal/etiología , ObjetivosRESUMEN
INTRODUCTION: Studies have shown a high prevalence of sleep-disordered breathing (SDB) in children with spina bifida. International standards for regular testing for SDB in this population are lacking. While there are studies investigating the prevalence of SDB in children with spina bifida, there are close to no studies in neonates. AIM AND OBJECTIVE: To evaluate if routine respiratory polygraphy (RPG) testing is indicated for neonates with spina bifida and if yes, with what therapeutic consequence. METHODS: We conducted a retrospective cohort study of all neonates with spina bifida at the University (Children's) Hospital Zurich after fetal spina bifida repair born between 2017 and 2022, who had undergone at least 1 RPG evaluation during hospitalization on the neonatal ward. RPG were evaluated by a blinded group of experienced pediatric pulmonologists. Based on the neonatal RPG results and pediatric pulmonologist's recommendation for caffeine therapy the spina bifida cohort was divided into two groups. Neonatal baseline RPG and follow-up RPG at the age of the 3 months were evaluated. RESULTS: 48 neonates with RPG were included. Compared to the standard values in healthy neonates, the RPG results of this spina bifida cohort showed findings of SDB with central apnea and hypopnea. 22 (45.8%) neonatal RPG evaluations detected central SDB, prompting caffeine therapy. Follow-up RPG conducted after 3 months showed significant improvement of SDB with (almost) no need for continuation of caffeine. CONCLUSION: We recommend the implementation of routine RPG testing in neonates with spina bifida to detect SDB and facilitate early targeted treatment.
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Polisomnografía , Síndromes de la Apnea del Sueño , Disrafia Espinal , Humanos , Estudios Retrospectivos , Recién Nacido , Masculino , Disrafia Espinal/complicaciones , Síndromes de la Apnea del Sueño/diagnóstico , Síndromes de la Apnea del Sueño/terapia , Femenino , Lactante , Suiza/epidemiologíaRESUMEN
OBJECTIVES: Urinary tract infections (UTIs) are common, but overdiagnosed, in children with spina bifida. We sought to evaluate the diagnostic test characteristics of urinalysis (UA) findings for symptomatic UTI in children with spina bifida. METHODS: Retrospective cross-sectional study using data from 2 centers from January 1, 2016, to December 31, 2021. Children with myelomeningocele aged <19 years who had paired UA (and microscopy, when available) and urine culture were included. The primary outcome was symptomatic UTI. We used generalized estimating equations to control for multiple encounters per child and calculated area under the receiver operating characteristics curve, sensitivity, and specificity for positive nitrites, pyuria (≥10 white blood cells/high-powered field), and leukocyte esterase (more than trace) for a symptomatic UTI. RESULTS: We included 974 encounters from 319 unique children, of which 120 (12.3%) met our criteria for UTI. Pyuria had the highest sensitivity while nitrites were the most specific. Comparatively, nitrites were the least sensitive and pyuria was the least specific. When the cohort was limited to children with symptoms of a UTI, pyuria remained the most sensitive parameter, whereas nitrites remained the least sensitive. Nitrites continued to be the most specific, whereas pyuria was the least specific. Among all encounters, the overall area under the receiver operating characteristics curve for all components of the UA was lower in children who use clean intermittent catheterizations compared with all others. CONCLUSIONS: Individual UA findings have moderate sensitivity (leukocyte esterase or pyuria) or specificity (nitrites) but overall poor diagnostic accuracy for symptomatic UTIs in children with spina bifida.
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Hidrolasas de Éster Carboxílico , Disrafia Espinal , Urinálisis , Infecciones Urinarias , Humanos , Estudios Retrospectivos , Estudios Transversales , Infecciones Urinarias/diagnóstico , Infecciones Urinarias/orina , Infecciones Urinarias/complicaciones , Urinálisis/métodos , Femenino , Masculino , Niño , Disrafia Espinal/complicaciones , Disrafia Espinal/orina , Preescolar , Adolescente , Lactante , Hidrolasas de Éster Carboxílico/orina , Sensibilidad y Especificidad , Piuria/diagnóstico , Piuria/orina , Nitritos/orina , Meningomielocele/complicaciones , Meningomielocele/orina , Curva ROCRESUMEN
INTRODUCTION: While the Ponseti method is the primary treatment for idiopathic clubfoot, its application in treating myelodysplastic clubfeet is less certain. Myelodysplastic clubfoot tends to be more severe and difficult to treat. Although the Ponseti method can initially correct these cases, there is conflicting evidence about recurrence rates and the need for additional treatment. This study aims to assess the effectiveness of the Ponseti method in treating myelodysplastic clubfeet compared with idiopathic clubfeet over a 20-year period. METHODS: The study conducted a retrospective review of medical records from patients treated for clubfoot at a single institution (2002 to 2021), comparing children with myelodysplastic and idiopathic clubfoot. Included patients were under 18, initially treated with Ponseti-casting, and had a minimum 2-year follow-up. Data on demographics, treatment details, recurrence, and Patient-reported Outcomes Measurement Information System (PROMIS) scores were analyzed. RESULTS: Forty-nine myelodysplastic and 512 idiopathic clubfeet in 366 patients met the inclusion criteria. Myelodysplastic cases had a median age of 5 months at presentation versus 2 months for idiopathic cases ( P =0.002). Initial correction was achieved in 95% of idiopathic and 87.8% of myelodysplastic feet ( P =0.185). Recurrence rates were higher in the myelodysplastic cohort, 65.3% versus 44.1% ( P =0.005). Surgery was necessary to treat recurrence in 59.2% of myelodysplastic and 37.7% of idiopathic cases, P =0.003. Follow-up was 3.9±1.8 years for myelodysplastic and 3.3±1.5 years for idiopathic feet, P =0.030. Myelodysplastic feet had lower PROMIS mobility scores; 31.94±7.56 versus 49.21±8.64, P <0.001. CONCLUSIONS: To the best of our knowledge, we report the largest series of myelodysplastic clubfeet treated by Ponseti casting and the first to assess PROMIS data. Overall, the Ponseti method is as effective in obtaining initial correction in myelodysplastic clubfoot as it is in idiopathic clubfoot. However, myelodysplastic clubfeet has a higher risk of relapse and increased need for surgical interventions. Children with spina bifida may need closer follow-ups and more stringent adherence to bracing. LEVEL OF EVIDENCE: Level III-therapeutic studies-investigating the results of treatment.
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Moldes Quirúrgicos , Pie Equinovaro , Recurrencia , Disrafia Espinal , Humanos , Pie Equinovaro/terapia , Pie Equinovaro/cirugía , Estudios Retrospectivos , Masculino , Femenino , Disrafia Espinal/complicaciones , Lactante , Resultado del Tratamiento , Preescolar , Estudios de Seguimiento , Niño , Medición de Resultados Informados por el PacienteRESUMEN
BACKGROUND: Ankle Foot Orthoses (AFOs) are frequently prescribed to manage gait impairments in children with physical disability, and it is important that AFOs are prescribed and fitted appropriately to maximize potential benefits. AFO tuning, manipulation of the AFO footwear combination (AFO-FC) by means of video vector analysis, is routinely used to optimize AFO use. However, the incidence or types of changes that are implemented after this type of orthotic review are unknown. RESEARCH QUESTION: To investigate the impact of a multi-disciplinary video vector clinic on AFO provision in children with physical disability. METHODS: All children who attended a video vector clinic over a period of 10-years from the establishment of the clinic were included in the study. Outcomes of the clinic were grouped into 5 categories: (1) No change to AFO-FC; (2) Altered/tuned AFO-FC; (3) Discontinued AFO-FC; (4) Recast AFO; (5) Change in prescription. Data were summarised narratively. RESULTS: 141 independently ambulant children were included. The diagnoses were bilateral cerebral palsy (39â¯%, n=55), unilateral cerebral palsy (38â¯%, n=54), spina bifida (9â¯%, n=13), hereditary spastic paraparesis (2â¯%, n=3) and other (11â¯%, n=16). No changes were made in 52â¯% of cases (n=74), tuning in 22â¯% of cases (n=31), the AFO was recast in 13â¯% of cases (n=19) and discontinued in 10â¯% of cases (n=14). A prescription change was recommended in 3â¯% of cases (n=4). SIGNIFICANCE: Our findings suggest that the video vector clinic is a time efficient and effective means of assessing gait function in children with AFOs. Without assessment at the clinic, most of the children assessed would likely have been referred for a full and more time consuming 3-dimensional gait analysis. Video vector analysis at the initial AFO fitting may improve alignment and possibly reduce non-compliance at an earlier stage.
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Parálisis Cerebral , Ortesis del Pié , Humanos , Niño , Masculino , Femenino , Parálisis Cerebral/rehabilitación , Parálisis Cerebral/fisiopatología , Grabación en Video , Niños con Discapacidad/rehabilitación , Adolescente , Preescolar , Trastornos Neurológicos de la Marcha/etiología , Trastornos Neurológicos de la Marcha/rehabilitación , Trastornos Neurológicos de la Marcha/fisiopatología , Disrafia Espinal/complicaciones , Estudios RetrospectivosRESUMEN
OBJECTIVE: To compare the occurrence of fetal bradycardia in open versus fetoscopic fetal spina bifida surgery. METHODS: This is a single-institution retrospective cohort study of patients undergoing open (n = 25) or fetoscopic (n = 26) spina bifida repair between 2017 and 2022. From October 2017 to June 2020, spina bifida repairs were performed via an open classical hysterotomy, and from November 2020 to June 2022 fetoscopic repairs were performed following transition to this technique. Fetal heart rate (FHR) in beats per minute (bpm) was recorded via echocardiography every 15 min during the procedure. Cohort characteristics, fetal bradycardia and maternal physiologic parameters were compared between the groups. RESULTS: Fetuses undergoing an open repair more frequently developed bradycardia defined as <110 bpm (32% vs. 3.8%, p = 0.008), and a trend was observed for FHR decreases more than 25 bpm from baseline (20% vs. 3.8%, p = 0.073). Profound bradycardia less than 80 bpm was rare, occurring in only three operations (two in open, one in fetoscopic repair) with two fetuses (one in each group) requiring emergency cesarean delivery. CONCLUSION: When compared to open fetal surgery, fetal bradycardia occurred less frequently in fetoscopic surgery despite a significantly greater anesthetic exposure and the use of the intraamniotic carbon dioxide insufflation.