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1.
Curr Urol Rep ; 22(3): 15, 2021 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-33534013

RESUMO

PURPOSE OF REVIEW: Patients with congenital urologic conditions present unique challenges as adults. Herein, we review the literature relevant to the adult reconstructive urologist confronted with complex surgical concerns affecting their patients with a history of hypospadias, spina bifida, and other syndromes affecting the genitourinary tract. RECENT FINDINGS: Urethral stricture disease related to hypospadias is complex, but successful urethroplasty and penile curvature correction can be achieved with an anatomically minded approach. Multiple urinary diversion techniques can be considered in a patient-centered approach to bladder management in the adult spina bifida patient, but complications are common and revision surgeries are frequently required. Strong evidence is lacking for most surgical techniques in this population, but experiences reported by pediatric and adult urologists with genitourinary reconstruction training can help foster consensus in decision-making. Urologists trained in genitourinary reconstruction may be uniquely positioned to care for the transitional urology patient as they enter adolescence and adulthood.


Assuntos
Papel do Médico , Procedimentos Cirúrgicos Reconstrutivos , Disrafismo Espinal/complicações , Transição para Assistência do Adulto , Anormalidades Urogenitais/cirurgia , Procedimentos Cirúrgicos Urológicos , Adolescente , Adulto , Criança , Feminino , Humanos , Hipospadia/cirurgia , Masculino , Reoperação , Estreitamento Uretral/etiologia , Estreitamento Uretral/cirurgia , Sistema Urinário/cirurgia , Anormalidades Urogenitais/terapia , Doenças Urológicas/etiologia , Doenças Urológicas/cirurgia , Urologistas
2.
J Urol ; 205(1): 250-256, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32716680

RESUMO

PURPOSE: Given the increasing prevalence of chronic kidney disease in people with spina bifida, we sought to determine if this is associated with an increase in end stage kidney disease. We examined population based data to measure the frequency of procedures to establish renal replacement therapy-a marker for end stage kidney disease-among patients with spina bifida. MATERIALS AND METHODS: We used the Healthcare Cost and Utilization Project State Inpatient Database and State Ambulatory Surgery and Services Database from Florida, Kentucky, Maryland and New York (2000 to 2014), which include encounter level data. With a diagnosis code based algorithm we identified all procedural encounters made by patients with spina bifida. We determined the percentage of these encounters that were for facilitating renal replacement therapy (ie arteriovenous anastomosis, renal transplantation). We assessed for changes over time in this percentage with the Cochran-Armitage trend test. Bivariate analysis was performed using chi-square test. RESULTS: Of all procedures performed on patients with spina bifida over this time the proportion of procedures performed to establish renal replacement therapy significantly decreased in both the inpatient and outpatient settings (p=0.042 and p <0.001, respectively). People with spina bifida undergoing procedures to establish renal replacement therapy were, on average, young adults (mean age 34.5 and 36.0 years) with a high prevalence hypertension (75.8% of inpatients, 68.6% of outpatients). CONCLUSIONS: The frequency of surgeries to initiate renal replacement therapy among people with spina bifida undergoing procedures is low and is not increasing. This highlights the importance of consistent care throughout adolescence and young adulthood, and hypertension screening.


Assuntos
Hipertensão/epidemiologia , Falência Renal Crônica/terapia , Terapia de Substituição Renal/tendências , Disrafismo Espinal/complicações , Adolescente , Adulto , Fatores Etários , Criança , Estudos de Coortes , Feminino , Humanos , Hipertensão/diagnóstico , Hipertensão/etiologia , Hipertensão/prevenção & controle , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Masculino , Programas de Rastreamento/normas , Pessoa de Meia-Idade , Guias de Prática Clínica como Assunto , Terapia de Substituição Renal/estatística & dados numéricos , Fatores de Risco , Disrafismo Espinal/terapia , Estados Unidos/epidemiologia , Adulto Jovem
3.
Pediatr Surg Int ; 36(7): 773-777, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32405766

RESUMO

AIM: To compare the quality of life (QoL) in children with spina bifida with a control group of their peers using a validated questionnaire, the Neurogenic Bowel Dysfunction Score (NBDS). METHODS: The NBDS questionnaire was prospectively distributed to children attending a multi-disciplinary Spina Bifida clinic and healthy controls attending pediatric urology clinics. A score (out of 41) was assigned to each child based on their responses to the validated questionnaire. A lower score indicates better bowel function-related quality of life. SPSS software (v.25) was used for all statistical analysis. RESULTS: There were 98 respondents to the questionnaire, 48 children with spina bifida and 50 controls. The average age of respondents was 7.88 years (3-16 years). Of those with Spina Bifida, 33 (69%) were on retrograde rectal irrigations, [19 (58%) Peristeen® system, 11 (33%) tube rectal irrigations, and 3 (9%) Willis system], 6 (12%) were on laxatives, and 9 (19%) were on no treatment. The median NBDS for Spina Bifida patients was significantly higher 13.5 (2-32) compared to the control group 2 (0-26, p < 0.001). Amongst Spina Bifida patients, there was no difference in quality of life between the modalities of bowel management (p = 0.203). CONCLUSIONS: Despite active bowel management, children with spina bifida report a worse quality of life compared to the control group. In those with spina bifida, the lack of a difference between various bowel management strategies, including no treatment, indicates the need for a longitudinal study to evaluate the basis for this unexpected finding.


Assuntos
Intestino Neurogênico/complicações , Intestino Neurogênico/diagnóstico , Qualidade de Vida , Disrafismo Espinal/complicações , Inquéritos e Questionários , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Intestino Neurogênico/fisiopatologia
4.
World Neurosurg ; 140: 37-45, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32407913

RESUMO

BACKGROUND: Spinal arteriovenous fistula (AVF) may rarely associate with spinal dysraphism, that is, tethered spinal cord and spinal intradural lipoma. Spinal extradural angiolipoma coexisting with spinal AVF has not been reported in the literature. We reported an extremely rare case of sacral angiolipoma associated with tight filum terminale and sacral spina bifida coexisting with spinal AVF within this tumor. CASE DESCRIPTION: A 55-year-old women presented with progressive myelopathy for 10 months. She had a painless, slow-growing mass at her left buttock since birth. Magnetic resonance imaging of the lumbosacral spine showed an extradural mass at the level of S3-S4, extending from the spinal canal through the spina bifida to the subcutaneous fat of the left buttock. There was a low conus medullaris at S2. Magnetic resonance imaging of the thoracic spine disclosed venous congestion with tortuous intradural flow voids along both ventral and dorsal surfaces of the spinal cord. Magnetic resonance angiography and spinal angiography revealed a hypervascular mass at the sacral level and associated arteriovenous shunt with cranial drainage into an enlarged medullary vein. Due to an infected pressure sore on the mass, endovascular treatment was initially performed with minimal recovery. Six months after complete healing of her infected pressure ulcer, the patient underwent surgical removal of extradural mass containing the AVF, and subsequent release of the tight filum. Histologic findings were consistent with angiolipoma. CONCLUSIONS: Sacral extradural angiolipoma in the present case may be congenital in origin with development of an acquired spinal AVF within the tumor.


Assuntos
Angiolipoma/complicações , Malformações Vasculares do Sistema Nervoso Central/complicações , Doenças do Sistema Nervoso Periférico/complicações , Neoplasias da Medula Espinal/complicações , Disrafismo Espinal/complicações , Angiolipoma/patologia , Cauda Equina/patologia , Malformações Vasculares do Sistema Nervoso Central/patologia , Feminino , Humanos , Região Lombossacral , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/patologia , Neoplasias da Medula Espinal/patologia , Disrafismo Espinal/patologia
5.
Ann Epidemiol ; 43: 71-74.e1, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32014336

RESUMO

PURPOSE: Adults with spina bifida (SB) may be susceptible to accelerated progression of chronic kidney disease (CKD) to advanced stages. However, little is known regarding risk of advanced CKD for this underserved population. The objective was to estimate the risk of advanced CKD among adults with vs. without SB. METHODS: Data were from Optum Clinformatics Data Mart. Adults (18+ years) without advanced CKD (CKD stages 4+) in 2013 were followed from 01/01/2014 to advanced CKD, death, loss to follow-up, or 12/31/2017. Diagnostic, procedure, and diagnosis-related group codes were used to identify SB, advanced CKD, and baseline cardiovascular diseases, hypertension, and diabetes. Incidence rate (IR) and IR ratio and their 95% confidence intervals (CIs) of advanced CKD were estimated. Cox regression estimated adjusted hazard ratio (HR) for incidence of advanced CKD. RESULTS: The crude IR of advanced CKD was 7.40 for adults with SB (n = 4295) and 6.25 for adults without SB (n = 6.6 M). After adjusting for demographics, adults with SB had greater risk of advanced CKD compared to adults without SB (HR = 2.12; 95% CI = 1.72-2.60), which remained elevated with further adjustment for cardiovascular diseases, hypertension, and diabetes (HR = 1.91; 95% CI = 1.55-2.35). CONCLUSIONS: Adults with SB may have greater risk of advanced CKD incidence compared to adults without SB.


Assuntos
Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Disrafismo Espinal/complicações , Adulto , Idoso , Estudos de Casos e Controles , Progressão da Doença , Feminino , Taxa de Filtração Glomerular , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Insuficiência Renal Crônica/diagnóstico , Disrafismo Espinal/patologia
6.
J Urol ; 203(6): 1207-1213, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31951496

RESUMO

PURPOSE: We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup. MATERIALS AND METHODS: We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000). RESULTS: A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%. CONCLUSIONS: Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.


Assuntos
Disrafismo Espinal/complicações , Bexiga Urinaria Neurogênica/cirurgia , Bexiga Urinária/cirurgia , Adenoma/epidemiologia , Adenoma/cirurgia , Adolescente , Criança , Feminino , Seguimentos , Humanos , Obstrução Intestinal/epidemiologia , Obstrução Intestinal/cirurgia , Masculino , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Bexiga Urinária/lesões , Cálculos da Bexiga Urinária/epidemiologia , Cálculos da Bexiga Urinária/cirurgia , Neoplasias da Bexiga Urinária/epidemiologia , Neoplasias da Bexiga Urinária/cirurgia , Bexiga Urinaria Neurogênica/etiologia , Derivação Urinária/estatística & dados numéricos , Adulto Jovem
7.
Pediatr Neurosurg ; 55(1): 46-50, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31962322

RESUMO

This report describes a case of an ependymoma found in the setting of tethered cord syndrome. We present a 3-month-old girl with prenatal diagnosis of lumbar meningocele who later underwent tethered cord release. After birth, she was neurologically intact and only found to have a skin-covered meningocele. An MRI was obtained and significant for low-lying conus terminating at L5, a focal syrinx, and Chiari II malformation. She underwent an elective meningocele repair and resection of thickened filum for tethered cord release at 3 months of age. Unexpectedly, microscopic evaluation of the filum was consistent with a small focus of ependymoma in addition to the filum tissue. Previous case reports have suggested a link between thickened filum in the setting of spinal dysraphism and myxopapillary ependymoma, but to our knowledge, this is the first report of ependymoma in the setting of tethered cord syndrome.


Assuntos
Cauda Equina/patologia , Ependimoma/diagnóstico , Meningocele/cirurgia , Neoplasias do Sistema Nervoso Periférico/diagnóstico , Feminino , Humanos , Lactente , Laminectomia , Imagem por Ressonância Magnética , Defeitos do Tubo Neural/cirurgia , Disrafismo Espinal/complicações
8.
Urology ; 137: 200-204, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31734348

RESUMO

OBJECTIVE: To assess and analyze the contemporary causes of in-hospital deaths of spina bifida patients. METHODS: It was a cross-sectional observational study of the longitudinal national cohort of all patients hospitalized in French public and private hospitals. We analyzed the data from the French hospital discharge database (Programme de Médicalisation des Systemes d'Information, PMSI) from 2009 to 2014. The number of in-hospital deaths was extracted using the combination of the ICD-10 codes "Q05" or "Q760" and a discharge code = 9. RESULTS: There were 138 in-hospital deaths of spina bifida patients over the 6-year study period. The median age at death was 41 years (IQR: 25-52). The median age at death was significantly lower in patients with vs without hydrocephalus (26.6 vs 45.5 years; P <.0001). The leading cause of in-hospital death was urologic disorders (n = 24; 17.3%). Other main causes of death were pulmonary disorders (n = 23; 16.7%), neurologic disorders (n = 19; 13.8%), and bowel disorders (n = 15; 10.9%). Upper urinary tract damage accounted for most of the urologic causes of death: 8 patients died from urinary tract infections (33.3%), 7 patients died from renal failure (29.2%), 4 died from bladder cancer (16.7%), and 5 from other urologic causes. The only variable significantly associated with a death from urologic causes was the absence of hydrocephalus (OR = 0.26; P = .009). CONCLUSION: Urologic disorders remain the leading cause of in-hospital death in spina bifida patients in France. The present study highlights that efforts to improve the urologic management of the spina bifida population are still greatly needed.


Assuntos
Causas de Morte , Hidrocefalia , Disrafismo Espinal , Doenças Urológicas , Adulto , Estudos Transversais , Feminino , França/epidemiologia , Mortalidade Hospitalar , Hospitalização/estatística & dados numéricos , Humanos , Hidrocefalia/complicações , Hidrocefalia/mortalidade , Estudos Longitudinais , Masculino , Mortalidade , Determinação de Necessidades de Cuidados de Saúde , Disrafismo Espinal/complicações , Disrafismo Espinal/terapia , Doenças Urológicas/classificação , Doenças Urológicas/diagnóstico , Doenças Urológicas/etiologia , Doenças Urológicas/mortalidade
9.
World Neurosurg ; 134: 532-535, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31760189

RESUMO

BACKGROUND: An expanding cohort of patients with spina bifida live well into adulthood and pose complex management challenges due to unique combinations of adult health issues overlying congenital problems. CASE DESCRIPTION: We present a case of a 45-year-old woman with an expanding, disfiguring, painful lumbar meningocele more than 40 years after her only surgery as a 3-year-old child. A team of pediatric and adult neurosurgeons as well as plastic/reconstructive surgeons successfully performed surgery to obliterate the meningocele, with preservation of her baseline functional status, and no evidence of recurrence after more than 1 year of follow-up. CONCLUSIONS: Symptomatic meningocele may present in a long-delayed fashion in adult patients with a history of spina bifida. Surgical treatment may provide symptomatic benefit.


Assuntos
Meningocele/etiologia , Meningomielocele/complicações , Feminino , Humanos , Meningocele/cirurgia , Pessoa de Meia-Idade , Disrafismo Espinal/complicações , Disrafismo Espinal/cirurgia
11.
Pediatr Clin North Am ; 67(1): 45-57, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31779836

RESUMO

Neuromuscular disorders are pathologies that can severely affect the quality of life as well as longevity of patients. The most common disorders include cerebral palsy and myelodysplasia. The orthopedic manifestations of these disorders can be treated operatively or nonoperatively. Both focus on the prolongation of mobility and preservation of ambulatory capacity for patients.


Assuntos
Doenças Musculoesqueléticas/etiologia , Doenças Neuromusculares/complicações , Paralisia Cerebral/complicações , Criança , Humanos , Meningomielocele/complicações , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/terapia , Disrafismo Espinal/complicações
12.
Neoreviews ; 20(12): e711-e724, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31792158

RESUMO

Spinal dysraphism, which includes conditions such as myelomeningocele and sacral agenesis, is one of the most common causes of congenital lower urinary tract dysfunction. Early evaluation of the neurogenic bladder serves to minimize renal damage, and the main goals of management include preserving renal function, achieving acceptable continence, and optimizing quality of life. The survival of patients with such conditions has improved to greater than 80% reaching adulthood, owing to advances in diagnostic and therapeutic modalities. The result is a real, and unfortunately often unmet, need for successful transitional care in this complex patient population. Clinicians must be able to identify the unique challenges encountered by patients with neurogenic bladder as they shift through different stages of their life.


Assuntos
Inibidores da Liberação da Acetilcolina/uso terapêutico , Toxinas Botulínicas Tipo A/uso terapêutico , Antagonistas Colinérgicos/uso terapêutico , Bexiga Urinaria Neurogênica/terapia , Cateterismo Urinário/métodos , Anormalidades Múltiplas/diagnóstico por imagem , Terapias Fetais , Humanos , Hidronefrose/etiologia , Hidronefrose/prevenção & controle , Meningocele/complicações , Meningocele/diagnóstico por imagem , Meningomielocele/complicações , Meningomielocele/diagnóstico por imagem , Meningomielocele/cirurgia , Região Sacrococcígea/anormalidades , Região Sacrococcígea/diagnóstico por imagem , Espinha Bífida Oculta/complicações , Espinha Bífida Oculta/diagnóstico por imagem , Disrafismo Espinal/complicações , Disrafismo Espinal/diagnóstico por imagem , Disrafismo Espinal/cirurgia , Transição para Assistência do Adulto , Ultrassonografia Pré-Natal , Bexiga Urinária , Bexiga Urinaria Neurogênica/diagnóstico , Bexiga Urinaria Neurogênica/fisiopatologia , Infecções Urinárias , Urodinâmica , Urologia , Refluxo Vesicoureteral , Conduta Expectante
13.
Curr Urol Rep ; 20(11): 76, 2019 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-31734847

RESUMO

PURPOSE OF REVIEW: Advancements in the care of patients affected by myelomeningocele have flourished in recent years especially with respect to renal preservation and continence. Involvement of urologists both prenatally and early in life has driven many developments in preventative care and early intervention. As of yet, however, the ideal management algorithm that offers these patients the least invasive diagnostic testing and interventions while still preserving renal and bladder function remains ill defined. RECENT FINDINGS: In a shift from prior years where the use of surgical intervention and intermittent catheterization were more liberally employed, some providers have more recently advocated for monitoring patients in a more conservative manner with a variety of diagnostic tests until radiographic or clinical changes are discovered. The criteria used to define the need for catheterization and the timing to initiate CIC or more invasive interventions is disparate across pediatric urology and there is published data to support several approaches. This review presents some of these criteria for use of CIC and some newer evidence to support different approaches along with supporting the trend toward individualized medicine and use of risk stratification in developing clinical treatment algorithms.


Assuntos
Cateterismo Uretral Intermitente , Disrafismo Espinal/terapia , Conduta Expectante , Humanos , Rim/fisiopatologia , Medição de Risco , Disrafismo Espinal/complicações , Disrafismo Espinal/cirurgia , Bexiga Urinaria Neurogênica/etiologia , Bexiga Urinaria Neurogênica/fisiopatologia
14.
J Pediatr Rehabil Med ; 12(4): 361-368, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31744028

RESUMO

BACKGROUND: Ethnic disparities in continence rates in spina bifida (SB) have been studied regionally but not nationally. National SB Patient Registry (NSBPR) data were analyzed to explore differences in prevalence of bowel and bladder continence and interventions between Hispanics/Latinos and others. METHODS: Participants 5 to 21 years were categorized into Hispanic/Latino and non-Hispanic/non-Latino. Bladder/bowel continence was defined as dry/no involuntary stool leakage during the day or none/⩽ monthly incontinence. Chi-square test, Wilcoxon Two Sample Test, and generalized estimating equation (GEE) were used for statistical analysis. RESULTS: Twenty-five percent of the 4,364 patients were Hispanic/Latino. At their most recent clinic visit, Hispanics/Latinos demonstrated lower rates of urinary continence (38.6% vs. 44.9%; p= 0.0003), bowel continence (43.9% vs. 55.8%, p< 0.0001), private insurance (p< 0.0001), bowel (p< 0.0001) or bladder surgeries (p= 0.0054), and more vesicostomies (p= 0.0427) compared to others. In multiple GEE models, Hispanic/Latino participants demonstrated lower odds of bowel continence as compared to non-Hispanic/non-Latino participants (estimated odds ratio, 0.82, 95% CI, 0.72-0.94, p= 0.0032). CONCLUSIONS: After controlling for covariates, Hispanics/Latinos with SB are less likely to report bowel continence. Clinicians are encouraged to consider the risk of negative health disparities for Hispanic patients with SB and work to mitigate this risk.


Assuntos
Incontinência Fecal/epidemiologia , Incontinência Fecal/etiologia , Disrafismo Espinal/complicações , Incontinência Urinária/epidemiologia , Incontinência Urinária/etiologia , Adolescente , Criança , Pré-Escolar , Grupos Étnicos , Feminino , Humanos , Masculino , Prevalência , Adulto Jovem
15.
J Pediatr Rehabil Med ; 12(4): 369-374, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31744035

RESUMO

PURPOSE: Spina Bifida (SB) is a congenital defect of the neural tube resulting in motor and sensory defects and frequently includes insensate skin on the buttocks and feet which are innervated by sacral nerves. For those living in hot climates such as the Southwestern United States, environmental surfaces reach temperatures throughout June-September that cause thermal burns. The objective of this novel case series was to evaluate the circumstances associated with environmental buttocks burns in children with SB who attend the District Medical Group Children's Rehabilitative Services Myelomeningocele (MM) Planning clinic in Phoenix, AZ. METHODS: Nine children and adolescents with SB and a history of environmental buttocks burns were identified through participation in the National Spina Bifida Patient Registry at District Medical Group Children's Rehabilitative Services Myelomeningocele Planning Clinic. Parents and patients were interviewed about the burn circumstances, charts were reviewed, and information was gathered from the NSBPR including level of function and ambulation status. RESULTS: Most of the patients were found to be of emerging independent ages (average age 10 years) and were not with their parents at the time of the burn. All had myelomeningocele (MM), almost all were community ambulators, and all had mid lumbar, low lumbar or sacral level function. There were no non-ambulators. In addition, the majority were wearing incontinence products. CONCLUSIONS: Children and adolescents with MM who are community ambulators, of an emerging independent age, and live in hot climates are at risk of environmental buttocks burns. Enhanced education in environmental thermal burn prevention is important. This education should be particularly focused on the child as they spend more time in settings without their parents. In addition, this enhanced education should extend to those who support the child or teen such as teachers, coaches, family and friends.


Assuntos
Queimaduras/etiologia , Nádegas/lesões , Meio Ambiente , Meningomielocele/complicações , Disrafismo Espinal/complicações , Adolescente , Criança , Feminino , Humanos , Masculino , Sudoeste dos Estados Unidos
16.
Urology ; 134: 84-89, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31585199

RESUMO

OBJECTIVE: To investigate computed tomography (CT) texture analysis of the bladder wall as a predictor of urodynamics findings in adult patient with spina bifida. METHODS: A single-center prospective trial was conducted from March 2015 to March 2017 including all consecutive adult spina bifida patients seen for urodynamic testing. A contrast-enhanced abdominal CT was systematically performed in all patients during the same visit. Texture features of the bladder wall related to the gray-level histogram and gray-level co-occurrence were evaluated on CT images. Multivariate analysis was performed to identify independent predictors of poor bladder compliance and detrusor overactivity among clinical and texture parameters. RESULTS: Fourty patients were included. The Lasso penalized logistic regression analysis identified 2 texture parameters as potential predictors of poor bladder compliance: Skewness (coefficient weight, -1.81) and S.1.1.SumVarnc (coefficient weight, -3.52). Multivariate logistic regression analysis confirmed skewness (odds ratio [confidence interval 95%] = 0.40 [0.14, 0.97], P = .04) as an independent predictor of poor bladder compliance. The Lasso penalized logistic regression analysis identified one texture parameters as potential predictor of detrusor overactivity: Kurtosis (coefficient weight, -3.52), which was confirmed in multivariate logistic regression analysis (odds ratio [confidence interval 95%] = 1.12 [1.01, 1.55], P = .02). CONCLUSION: Our findings demonstrate that CT texture analysis of the bladder wall might be an interesting tool to identify spina bifida patients with high risk urodynamic features.


Assuntos
Disrafismo Espinal , Tomografia Computadorizada por Raios X/métodos , Bexiga Urinária Hiperativa , Bexiga Urinária , Urodinâmica , Adulto , Feminino , França , Humanos , Masculino , Estudos Prospectivos , Intensificação de Imagem Radiográfica/métodos , Disrafismo Espinal/complicações , Disrafismo Espinal/diagnóstico , Disrafismo Espinal/fisiopatologia , Bexiga Urinária/diagnóstico por imagem , Bexiga Urinária/fisiopatologia , Bexiga Urinária Hiperativa/diagnóstico , Bexiga Urinária Hiperativa/etiologia , Bexiga Urinária Hiperativa/fisiopatologia
17.
Neurourol Urodyn ; 38(8): 2151-2158, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31486131

RESUMO

AIMS: To assess the predictive values of six urinary markers (nerve growth factor [NGF], brain-derived neurotrophic factor [BDNF], matrix metalloproteinase 2 [MMP-2], tissue inhibitor metalloproteinase 2 [TIMP-2], transformation growth factor ß-1 [TGF-B1], and prostaglandin 2 [PGE2]) for adverse urodynamic features and for upper urinary tract damage in adult patients with spina bifida. MATERIALS AND METHODS: A single-center prospective trial was conducted from March 2015 to March 2017 including all consecutive adult patients with spina bifida seen for urodynamic testing. The urine was collected and stored at -80°C. A urodynamic and an upper urinary tract were systematically performed. At the end of the inclusion period, urines were defrosted and urinary nerve growth factor, BDNF, TIMP-2, and TGF-B1 were assessed using validated ELISA kits. The urinary markers levels were adjusted on the urinary creatinine level. Urinary MMP-2 levels were assessed by zymography. RESULTS: Fourty patients were included. Only TIMP-2 and MMP-2 were significantly associated with poor bladder compliance (P = .043 and P = .039, respectively). TIMP-2 was also the only urinary marker significantly associated with upper urinary tract damage on imaging (OR = 19.81; P = .02). Of all urodynamic parameters, bladder compliance and maximum detrusor pressure were the only ones associated with upper urinary tract damage on imaging (P = .01 and P = .02), The diagnostic performances of urinary TIMP-2 for upper urinary tract damage were slightly superior to PdetMax and bladder compliance with an area under the curve of 0.72. CONCLUSION: Urinary TIMP-2 and MMP-2 were significantly associated with poor bladder compliance and urinary TIMP-2 was significantly associated with upper urinary tract damage. These findings support a pathophysiological role of extracellular matrix remodeling in poor bladder compliance of adult patients with spina bifida.


Assuntos
Disrafismo Espinal/fisiopatologia , Bexiga Urinaria Neurogênica/urina , Adulto , Atrofia , Biomarcadores/urina , Fator Neurotrófico Derivado do Encéfalo/urina , Complacência (Medida de Distensibilidade)/fisiologia , Dinoprostona/urina , Feminino , Humanos , Hidronefrose/diagnóstico por imagem , Rim/diagnóstico por imagem , Rim/patologia , Masculino , Metaloproteinase 2 da Matriz/urina , Pessoa de Meia-Idade , Fator de Crescimento Neural/urina , Estudos Prospectivos , Disrafismo Espinal/complicações , Inibidor Tecidual de Metaloproteinase-2/urina , Fator de Crescimento Transformador beta1/urina , Bexiga Urinaria Neurogênica/etiologia , Bexiga Urinaria Neurogênica/fisiopatologia , Urodinâmica , Adulto Jovem
18.
J Neurosurg Pediatr ; 24(2): 105-114, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31370010

RESUMO

The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.


Assuntos
Fetoscopia/métodos , Meningomielocele/cirurgia , Disrafismo Espinal/cirurgia , Técnicas de Fechamento de Ferimentos , Adolescente , Desenvolvimento do Adolescente , Animais , Derivações do Líquido Cefalorraquidiano , Criança , Desenvolvimento Infantil , Cistos/etiologia , Modelos Animais de Doenças , Fetoscopia/efeitos adversos , Humanos , Hidrocefalia/terapia , Recém-Nascido , Recém-Nascido Prematuro , Ilustração Médica , Estudos Multicêntricos como Assunto , Defeitos do Tubo Neural/etiologia , Posicionamento do Paciente , Fotografação , Complicações Pós-Operatórias , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Disrafismo Espinal/complicações , Resultado do Tratamento
19.
Med J Malaysia ; 74(4): 281-287, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31424034

RESUMO

INTRODUCTION: The average incidence of spina bifida (SB) in Malaysia is 0.43 among 1,000 live births. The burden of the disease and its impact on the overall development and health though tremendously improved, remains significant. Therefore, current patient management strategies must include quality of life (QOL) measures. METHODS: This was a prospective, cross-sectional study on spina bifida children aged 5-20 years, attending the paediatric spina bifida clinics of Universiti Kebangsaan Malaysia Medical Centre Kuala Lumpur and Hospital Tuanku Jaanku Seremban. Scores were obtained using the validated disease specific Parkin QOL questionnaire. Univariate and multivariate analysis were used to investigate factors that were determinants for these outcomes. Results were expressed as beta coefficient and 95% confidence intervals (95%CI). RESULTS: A total of 54 children and adolescents aged between 5-20 years completed the questionnaires. Presence of neurogenic bowel (p=0.003), neurogenic bladder (p=0.041), shunt (p=0.044), non-ambulators (p=0.007) and being the only child in the family (p=0.037) were associated with lower QOL scores. Multivariate analysis showed presence of neurogenic bowel (ß=0.375, 95%CI: 0.00, 0.15) and being the only child in the family (ß=0.250, 95%CI: 0.04, 0.17) explained 22.1% of the variance in the QOL mean percentage scores. CONCLUSION: Being a single child in the family was the only socio-demographic variable associated with lower QOL scores. Although several clinical factors appeared to contribute significantly to QOL in spina bifida children, the presence of neurogenic bowel had the greatest impact.


Assuntos
Qualidade de Vida/psicologia , Disrafismo Espinal/psicologia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Indicadores Básicos de Saúde , Humanos , Modelos Lineares , Malásia , Masculino , Intestino Neurogênico/etiologia , Intestino Neurogênico/psicologia , Filho Único/psicologia , Estudos Prospectivos , Disrafismo Espinal/complicações , Disrafismo Espinal/fisiopatologia , Disrafismo Espinal/terapia , Adulto Jovem
20.
J Am Podiatr Med Assoc ; 109(3): 180-186, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31268782

RESUMO

BACKGROUND: Cole osteotomy is performed in patients having a cavus deformity with the apex of the deformity in the midfoot. Correction of the deformity at this midfoot level improves foot and ankle stability by creating a plantigrade foot. We retrospectively reviewed the clinical and radiographic results of six feet (five patients) that underwent Cole midfoot osteotomy (2011-2015). METHODS: The patients had different etiologies (spastic cerebral palsy, burn sequelae, spina bifida, and Charcot-Marie-Tooth disease). Dorsal and slightly laterally based transverse wedge osteotomy through the navicular bone medially and the cuboid bone laterally was performed. Patients were under routine clinical follow-up. We evaluated clinical and radiographic results. RESULTS: Mean clinical follow-up was 15.7 months (range, 6-36 months). The mean preoperative and postoperative talo-first metatarsal angles on lateral radiographs were 29.9° and 8.7°, respectively (P < .05) and on anteroposterior radiographs were 30.3° and 8.6° (P < .05). The mean preoperative talocalcaneal angle on anteroposterior radiographs increased from 19.2° to 29.8° postoperatively (P < .05). The mean postoperative calcaneal pitch angle change was 10.8° on the lateral radiograph (P < .05). At final follow-up, all five patients were independently active, had plantigrade feet, and were able to wear conventional shoes. The mean American Orthopaedic Foot and Ankle Society questionnaire score was 38.8 preoperatively and 79.5 postoperatively (P < .05). Only one patient did not have full bony union. Achilles tightness was seen in one patient. CONCLUSIONS: Cole midfoot osteotomy is a laboring procedure to correct adult pes cavus deformity with the apex in midfoot, although having some complication risks.


Assuntos
Osteotomia/métodos , Pé Cavo/cirurgia , Ossos do Tarso/cirurgia , Adulto , Queimaduras/complicações , Paralisia Cerebral/complicações , Doença de Charcot-Marie-Tooth/complicações , Contraindicações de Procedimentos , Feminino , Seguimentos , Humanos , Masculino , Radiografia , Estudos Retrospectivos , Disrafismo Espinal/complicações , Pé Cavo/diagnóstico por imagem , Pé Cavo/etiologia , Adulto Jovem
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