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Myelomeningocele

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https://www.readbyqxmd.com/read/29132982/longitudinal-study-of-bladder-continence-among-people-with-spina-bifida-in-the-national-spina-bifida-patient-registry
#1
Tiebin Liu, Lijing Ouyang, Judy Thibadeau, John S Wiener, Jonathan C Routh, Heidi Castillo, Jonathan Castillo, Kurt A Freeman, Kathleen J Sawin, Kathryn Smith, Alexander Van Speybroeck, Rodolfo Valdez
PURPOSE: Achieving bladder continence among people with Spina bifida is a lifetime management goal. We investigated bladder continence status over time and factors associated with this status among people with SB. MATERIALS AND METHODS: We used National Spina Bifida Patient Registry data collected from 2009 through 2015 and applied generalized estimating equation models to analyze factors associated with bladder continence status. RESULTS: This analysis included 5,250 participants with SB in a large, multi-institutional patient registry who contributed 12,740 annual clinic visit records during the study period...
November 10, 2017: Journal of Urology
https://www.readbyqxmd.com/read/29125526/bowel-management-and-continence-in-adults-with-spina-bifida-results-from-the-national-spina-bifida-patient-registry-2009-15
#2
John S Wiener, Kristina D Suson, Jonathan Castillo, Jonathan C Routh, Stacy Tanaka, Tiebin Liu, Elisabeth Ward, Judy Thibadeau, David Joseph, National Spina Bifida Patient Registry
INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR)...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125525/early-postnatal-bladder-function-in-fetoscopic-myelomeningocele-repair-patients
#3
Gene O Huang, Michael A Belfort, William E Whitehead, Oluyinka O Olutoye, Jonathan Castillo, Heidi Castillo, Kathryn K Ostermaier, Chester J Koh, Duong D Tu
PURPOSE: Prenatal repair of myelomeningocele (MMC) via hysterotomy has demonstrated neurosurgical and motor benefits, when compared to postnatal repairs. Urologic benefits, however, remain to be seen. The purpose of this study was to review early postnatal bladder function in patients undergoing a novel endoscopic approach for MMC repair using an exteriorized uterus. METHODS: A prospective urologic assessment of patients undergoing fetoscopic MMC repair and receiving subsequent care at our facility, was performed...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125521/variation-in-surgical-management-of-neurogenic-bowel-among-centers-participating-in-national-spina-bifida-patient-registry
#4
Jonathan C Routh, David B Joseph, Tiebin Liu, Michael S Schechter, Judy K Thibadeau, M Chad Wallis, Elisabeth A Ward, John S Wiener
PURPOSE: Optimal management of neurogenic bowel in patients with spina bifida (SB) remains controversial. Surgical interventions may be utilized to treat constipation and provide fecal continence, but their use may vary among SB treatment centers. METHODS: We queried the National Spina Bifida Patient Registry (NSBPR) to identify patients who underwent surgical interventions for neurogenic bowel. We abstracted demographic characteristics, SB type, functional level, concurrent bladder surgery, mobility, and NSBPR clinics to determine whether any of these factors were associated with interventions for management of neurogenic bowel...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125519/advanced-skeletal-maturity-in-children-and-adolescents-with-myelomeningocele
#5
Ronald Roiz, Nicole M Mueske, Alexander Van Speybroeck, Deirdre D Ryan, Vicente Gilsanz, Tishya A L Wren
PURPOSE: Atypical skeletal development is common in youth with myelomeningocele (MM), though the underlying reasons have not been fully elucidated. This study assessed skeletal maturity in children and adolescents with MM and examined the effects of sex, age, sexual development, ethnicity, anthropometrics and shunt status. METHODS: Forty-three males and 35 females with MM, 6-16 years old, underwent hand radiographs for bone age determination. The difference between bone age and chronological age was evaluated using Wilcoxon sign rank tests...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125514/crowdsourcing-utility-estimation-for-spina-bifida-in-the-general-population
#6
Ruiyang Jiang, Brian M Inouye, Hsin-Hsiao S Wang, Rohit Tejwani, Jonathan C Routh
INTRODUCTION: Cost-utility analyses (CUA) are useful when the treatment conditions depend on patient preferences that are in turn dependent on health state utility value. Spina bifida (SB) is an example of such a preference-sensitive condition. Historically, the SB utility value for CUA has been gathered via a traditional face-to-face interview. However, due to funding and time constrains, utility estimation via online crowdsourcing has recently gained popularity. Our aim was to estimate the utility value for a generic SB health state using a validated online tool...
November 3, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125511/variation-in-bowel-and-bladder-continence-across-us-spina-bifida-programs-a-descriptive-study
#7
Kurt A Freeman, Heidi Castillo, Jonathan Castillo, Tiebin Liu, Michael Schechter, John S Wiener, Judy Thibadeau, Elisabeth Ward, Timothy Brei
PURPOSE: Continence is low in individuals with spina bifida, but published prevalence varies markedly across studies. The objective of this study was to examine bladder and bowel continence among patients served by multidisciplinary clinics participating in the National Spina Bifida Patient Registry and to examine whether variation in prevalence exists across clinics. METHODS: Data were obtained from patients 5 years and older from March 2009 to December 2012. Data were gathered at clinic visits using standardized definitions...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29125508/urologic-self-management-through-intermittent-self-catheterization-among-individuals-with-spina-bifida-a-journey-to-self-efficacy-and-autonomy
#8
Jonathan Castillo, Kathryn K Ostermaier, Ellen Fremion, Talia Collier, Huirong Zhu, Gene O Huang, Duong Tu, Heidi Castillo
PURPOSE: To describe the age of independence in intermittent self-catheterization (ISC) in a diverse patient population and identify factors associated with ISC in individuals with spina bifida. METHODS: Two hundred patients with myelomeningocele or lipomyelomeningocele, who were ⩾ 3 years of age and utilized catheterization for bladder management were included. Data regarding diagnosis, functional level of lesion, race, ethnicity, presence of shunt, method of catheterization, self-management skills, fine motor skills, and cognitive abilities were collected...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29122293/placental-mesenchymal-stromal-cells-seeded-on-clinical-grade-extracellular-matrix-improve-ambulation-in-ovine-myelomeningocele
#9
Sandra Kabagambe, Benjamin Keller, James Becker, Laura Goodman, Christopher Pivetti, Lee Lankford, Karen Chung, Chelsey Lee, Y Julia Chen, Priyadarsini Kumar, Melissa Vanover, Aijun Wang, Diana Farmer
PURPOSE: The purpose of this study was to investigate the effects of placental mesenchymal stromal cells (PMSCs) seeded on a clinical grade porcine small intestinal submucosa (SIS)-derived extracellular matrix (ECM) on hindlimb motor function in an ovine fetal repair model of myelomeningocele (MMC). METHODS: MMC defects were surgically created in 21 fetuses at median gestational age 78 (range 76-83) days. Fetuses were randomly assigned to repair 25days later with ECM only or PMSC-ECM...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29118540/total-endoscopic-and-anal-irrigation-management-approach-to-noncompliant-neuropathic-bladder-and-bowel-in-children-a-long-term-follow-up
#10
Naif Alqarni, Hamdan Alhazmi, Ossamah Alsowayan, Tamer Eweda, Khalid Fouda Neel
Purpose: To evaluate the long-term efficacy and durability of combined intradetrusor botulinum-A toxin (BTX-A), endoscopic treatment of vesicouerteral reflux and anal irrigation for stool incontinence (SI) via a total endoscopic and anal irrigation management (TEAM(®)) approach in patients with myelomeningocele and neuropathic bladder and bowel who did not respond to conservative measures. Materials and Methods: Fourteen myelomeningocele patients with at least 3 years follow-up were included in the study...
October 2017: Urology Annals
https://www.readbyqxmd.com/read/29104840/spinal-shortening-for-recurrent-tethered-cord-syndrome-via-a-lateral-retropleural-approach-a-novel-operative-technique
#11
Jeffrey A Steinberg, Arvin R Wali, Joel Martin, David R Santiago-Dieppa, David Gonda, William Taylor
Spine shortening via vertebral osteotomy (SSVO) for recurrent tethered cord syndrome (TCS) is a novel surgical technique that avoids the complication profile associated with revision detethering. While SSVO has previously been described via a posterior approach, we describe a lateral retropleural approach for SSVO in recurrent TCS in a 21-year-old female. Our patient presented with progressive lower extremity weakness, bowel and bladder incontinence, and back pain in the setting of childhood repair of myelomeningocele and two previous detethering procedures...
August 31, 2017: Curēus
https://www.readbyqxmd.com/read/29096888/fetal-surgical-repair-with-placenta-derived-mesenchymal-stromal-cell-engineered-patch-in-a-rodent-model-of-myelomeningocele
#12
Y Julia Chen, Karen Chung, Christopher Pivetti, Lee Lankford, Sandra K Kabagambe, Melissa Vanover, James Becker, Chelsey Lee, Josephine Tsang, Aijun Wang, Diana L Farmer
PURPOSE: The purpose of this study is to determine the feasibility of fetal surgical repair of myelomeningocele (MMC) in a rodent model using human placental mesenchymal stromal cells (PMSCs) seeded onto extracellular matrix (ECM) and to characterize the resulting changes in spinal cord tissue. METHODS: Fetal rodents with retinoic acid (RA) induced MMC underwent surgical repair of the MMC defect using an ECM patch on embryonic age (EA) 19 and were collected via caesarean section on EA 21...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29056972/methotrexate-and-valproic-acid-affect-early-neurogenesis-of-human-amniotic-fluid-stem-cells-from-myelomeningocele
#13
Vardine Sahakyan, Enrico Pozzo, Robin Duelen, Jan Deprest, Maurilio Sampaolesi
Myelomeningocele (MMC) is a severe type of neural tube defect (NTD), in which the backbone and spinal canal do not close completely during early embryonic development. This condition results in serious morbidity and increased mortality after birth. Folic acid significantly reduces, and conversely, folate antagonist methotrexate (MTX) and valproic acid (VPA) increase the occurrence of NTDs, including MMC. How these pharmacological agents exactly influence the early neurulation process is still largely unclear...
2017: Stem Cells International
https://www.readbyqxmd.com/read/29053056/intradural-spine-surgery-may-not-carry-an-increased-risk-of-shunt-revision-compared-with-extradural-spine-surgery-in-pediatric-patients-with-myelomeningocele
#14
Elizabeth N Kuhn, Betsy Hopson, Michael J Conklin, Jeffrey P Blount
OBJECTIVE Patients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele. METHODS The authors reviewed records of the National Spina Bifida Program Registry for Children's Hospital of Alabama...
October 20, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29042971/histological-evidence-of-reparative-activity-in-chorioamniotic-membrane-following-open-fetal-surgery-for-myelomeningocele
#15
Natalia S Carvalho, Antonio F Moron, Ramkumar Menon, Sergio Cavalheiro, Mauricio M Barbosa, Herbene J Milani, Marcia M Ishigai
An increased understanding of the reparative process in fetal membrane following surgical techniques may be helpful to decrease the risks to mother and fetus and avoid adverse pregnancy outcomes. The present study discusses histological evaluation of the fetal membrane following open fetal surgery. Chorioamniotic membranes (n=10) were obtained following birth from pregnancies that underwent open fetal surgery for myelomeningocele. The collagen distribution was quantified using picrosirius-polarization method comparing the suture site with non-suture site...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29042964/in-utero-repair-of-fetal-rat-myelomeningocele-affects-neuromuscular-development-in-the-bladder
#16
Liangfeng Tang, Haijun Zhong, Hong Chen, Jian Shen, Yunli Bi, Xianmin Xiao
Fetal repair of myelomeningocele (MMC) has been proven to be beneficial for the central nervous system development; however, the effect of fetal MMC repair on bladder function remains controversial. The objective of the present study was to establish an early timepoint for in utero MMC repair using a rat model, and to investigate the changes in bladder development subsequent to that intervention. Sprague Dawley rats were divided into the MMC, MMC repair and control groups. MMC rat fetus models were created by treating pregnant rats with all-trans retinoic acid...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29042329/rare-giant-prevertebral-thoracic-myelomeningocele
#17
Bryan D Choi, Katie P Fehnel, William E Butler
Here we report a 72-year-old man who presented with complaint of sudden onset weakness and impaired sensation in the left lower extremity. Radiographic evaluation revealed a congenital malformation with multiple formation defects including a giant thoracic prevertebral myelomeningocele. Following microsurgical dethetering of the spinal cord the patient recovered ambulation with assist. While thoracic myelomeningoceles are themselves rare, in this case the patient presented at a late age, and responded well to conservative management...
October 14, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29031539/fetal-surgery-for-myelomeningocele-after-the-management-of-myelomeningocele-study-moms
#18
REVIEW
Julie S Moldenhauer, N Scott Adzick
Myelomeningocele (MMC) is the most frequently occurring congenital abnormality of the central nervous system and leads to significant physical disabilities. Historically treatment involved postnatal closure with management of the associated sequelae including ventricular shunting. The mechanism of neurologic damage that begins with abnormal neurulation followed by continued injury over the course of gestation made MMC a plausible candidate for in-utero surgical repair. Animal and early human studies demonstrated the feasibility of fetal closure...
October 11, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29026989/the-pattern-peculiarities-and-management-challenges-of-spina-bifida-in-a-teaching-hospital-in-southwest-nigeria
#19
Edward O Komolafe, Chiazor Udochukwu Onyia, Ibironke O Ogunbameru, Oluwamuyiwa A Dada, Oluwafemi F Owagbemi, Fred S Ige-Orhionkpaibima, Oluseun A Olarewaju, Efosa O Obamwonyi, Muhammad I Abdullahi
PURPOSE: Spina bifida is a common congenital anomaly of the nervous system. It is frequently associated with significant morbidity and sometimes mortality in affected children. In this paper, we review the clinico-epidemiological pattern, peculiarities, and therapeutic challenges of this condition in our practice setting. METHODS: This is a retrospective study of all cases of spina bifida managed from July 2000 to March 2016 at a tertiary health facility located in the southwest region of Nigeria...
October 12, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28987842/post-natal-management-of-myelomeningocele-outcome-with-a-multidisciplinary-team-experience
#20
P A Beuriat, I Poirot, F Hameury, A Szathmari, C Rousselle, I Sabatier, F Di Rocco, C Mottolese
INTRODUCTION: Myelomeningocele (MMC) is a complex neural tube defect. Few studies report the results of modern post-natal management. The goal of this study was to report the long term outcome of a multidisciplinary approach of MMC patients. MATERIALS AND METHODS: Forty-six MMC were included. Clinical status was evaluated prospectively. RESULTS: Mean follow-up was 8,1 years. The level of the malformation was sacral or lower lumbar (≤ L4) in 27 cases, higher lumbar (between L1 and L3) in 5 cases and thoracic in 14 cases...
October 5, 2017: World Neurosurgery
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