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Myelomeningocele

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https://www.readbyqxmd.com/read/28617430/physician-views-regarding-the-benefits-and-burdens-of-prenatal-surgery-for-myelomeningocele
#1
R M Antiel, C A Collura, A W Flake, M P Johnson, N E Rintoul, J D Lantos, F A Curlin, J C Tilburt, S D Brown, C Feudtner
OBJECTIVE: Examine how pediatric and obstetrical subspecialists view benefits and burdens of prenatal myelomeningocele (MMC) closure. STUDY DESIGN: Mail survey of 1200 neonatologists, pediatric surgeons and maternal-fetal medicine specialists (MFMs). RESULTS: Of 1176 eligible physicians, 670 (57%) responded. Most respondents disagreed (68%, 11% strongly) that open fetal surgery places an unacceptable burden on women and their families. Most agreed (65%, 10% strongly) that denying the benefits of open maternal-fetal surgery is unfair to the future child...
June 15, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28600017/comparison-of-prenatal-and-postnatal-management-of-patients-with-myelomeningocele
#2
REVIEW
Sergio Cavalheiro, Marcos Devanir Silva da Costa, Antonio Fernandes Moron, Jeffrey Leonard
Myelomeningocele (MMC) is a costly lifetime disease with many comorbidities, including sensory and motor lower limb disability, bladder/bowel dysfunction, scoliosis, club foot, and hydrocephalus. MMC treatment options have changed over time because routine use of fetal ultrasonography and MRI has provided prenatal diagnosis and the potential for fetal surgery. There is still no consensus on how to treat the MMC diagnoses prenatally, mainly related to the infrastructure required to operate on pregnant patients...
July 2017: Neurosurgery Clinics of North America
https://www.readbyqxmd.com/read/28591652/fetal-therapy-model-of-myelomeningocele-with-three-dimensional-skin-using-amniotic-fluid-cell-derived-induced-pluripotent-stem-cells
#3
Kazuhiro Kajiwara, Tomohiro Tanemoto, Seiji Wada, Jurii Karibe, Norimasa Ihara, Yu Ikemoto, Tomoyuki Kawasaki, Yoshie Oishi, Osamu Samura, Kohji Okamura, Shuji Takada, Hidenori Akutsu, Haruhiko Sago, Aikou Okamoto, Akihiro Umezawa
Myelomeningocele (MMC) is a congenital disease without genetic abnormalities. Neurological symptoms are irreversibly impaired after birth, and no effective treatment has been reported to date. Only surgical repairs have been reported so far. In this study, we performed antenatal treatment of MMC with an artificial skin using induced pluripotent stem cells (iPSCs) generated from a patient with Down syndrome (AF-T21-iPSCs) and twin-twin transfusion syndrome (AF-TTTS-iPSCs) to a rat model. We manufactured three-dimensional skin with epidermis generated from keratinocytes derived from AF-T21-iPSCs and AF-TTTS-iPSCs and dermis of human fibroblasts and collagen type I...
June 6, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28586935/endoscopic-management-of-arnold-chiari-malformation-type-i-with-or-without-syringomyelia
#4
Shailendra Ratre, Nishtha Yadav, Yad Ram Yadav, Vijay Singh Parihar, Jitin Bajaj, Yatin Kher
Introduction Several different surgical techniques have been used in the treatment of patients with symptomatic Arnold-Chiari malformation type 1 (ACM-1) with or without syrinx. Endoscope-assisted decompression of the posterior fossa has been found to be safe and effective. We report our initial experience of endoscopic management of ACM-I. Material and Methods This was a prospective study of 15 symptomatic patients. Pre- and postoperative clinical status and computed tomography and magnetic resonance imaging findings were recorded...
June 6, 2017: Journal of Neurological Surgery. Part A, Central European Neurosurgery
https://www.readbyqxmd.com/read/28583853/lipomyelomeningocele-for-the-urologist-should-we-view-it-the-same-as-myelomeningocele
#5
E B Yerkes, C Halline, G Yoshiba, T A Meyer, I Rosoklija, R Bowman, D McLone, E Y Cheng
INTRODUCTION: The primary urologic objectives for lipomyelomeningocele (LMM) and myelomeningocele (MM) are preserving renal integrity and achieving continence. Due to this common ground, LMM and MM are urologically treated the same. However, unlike MM, LMM may present with no evident functional concerns. Indications for and timing of tethered cord release (TCR) in LMM are therefore controversial. Long-term urologic outcomes are not well defined. OBJECTIVE: Expectations for continence and potential for intermittent catheterization (CIC) following TCR in LMM are important for realistically counseling families regarding future needs...
May 22, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28578005/isolation-characterization-and-differentiation-of-multipotent-neural-progenitor-cells-from-human-cerebrospinal-fluid-in-fetal-cystic-myelomeningocele
#6
Mario Marotta, Alejandra Fernández-Martín, Marc Oria, Cesar G Fontecha, Carles Giné, Vicente Martínez-Ibáñez, Elena Carreras, Michael A Belfort, Gloria Pelizzo, Jose L Peiró
Despite benefits of prenatal in utero repair of myelomeningocele, a severe type of spina bifida aperta, many of these patients will still suffer mild to severe impairment. One potential source of stem cells for new regenerative medicine-based therapeutic approaches for spinal cord injury repair is neural progenitor cells (NPCs) in cerebrospinal fluid (CSF). To this aim, we extracted CSF from the cyst surrounding the exposed neural placode during the surgical repair of myelomeningocele in 6 fetuses (20 to 26weeks of gestation)...
May 15, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28569523/mastery-motivation-and-executive-functions-as-predictors-of-adaptive-behavior-in-adolescents-and-young-adults-with-cerebral-palsy-or-myelomeningocele
#7
Seth Warschausky, Jacqueline N Kaufman, Michael Evitts, William Schutt, Edward A Hurvitz
PURPOSE/OBJECTIVE: To examine mastery motivation and executive functions or behaviors as predictors of adaptive behavior in adolescents and young adults with congenital neurodevelopmental conditions. METHOD: Participants were 2 groups of adolescents and young adults, ages 13-29, including 43 with cerebral palsy and 36 with myelomeningocele living with a parent or caregiver. Participants completed measures of mastery motivation, executive functions or behaviors, and a measure of adaptive behavior...
June 1, 2017: Rehabilitation Psychology
https://www.readbyqxmd.com/read/28559064/endoscopic-third-ventriculostomy-for-the-treatment-of-hydrocephalus-in-a-paediatric-population-with-myelomeningocele
#8
Joana Rei, Josué Pereira, Carina Reis, Sérgio Salvador, Rui Vaz
BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although Endoscopic Third Ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiological outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 paediatric patients with myelomeningocele submitted to ETV from 1998 to 2015, at the Centro Hospitalar S...
May 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28555310/antenatal-management-of-fetal-neurosurgical-diseases
#9
Sergio Cavalheiro, Marcos Devanir Silva da Costa, Jardel Nicacio Mendonça, Patricia Alesssandra Dastoli, Italo Capraro Suriano, Mauricio Mendes Barbosa, Antonio Fernandes Moron
The advance in the imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Myelomeningocele is the most recognized disease that can be treated during pregnancy with a high rate of success. Additionally, this field can be extended to other conditions such as hydrocephalus and encephaloceles. However, each one of these diseases has nuances in the diagnostic evaluation that should fit the requirements to perform the fetal procedure and overbalance the benefits to the patients...
May 29, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28553378/dermoid-of-the-posterior-fossa-in-chiari-ii-malformation-the-first-reported-case
#10
R Shane Tubbs, Ketan Verma, Sarah N Mirahsani, Martin M Mortazavi, Randle A Umeh, Brandon Rocque, Jeffrey B Blount
Dermoid cysts are rare lesions, particularly in children. Chiari II malformations are seen in patients with myelomeningocele. Here, we present a child with Chiari II malformation who, during a Chiari II decompression, was found to have a dermoid cyst. To the best of our knowledge, this is the first such case ever reported.
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28550526/fetoscopic-patch-coverage-of-experimental-myelomenigocele-using-a-two-port-access-in-fetal-sheep
#11
Lucie Guilbaud, Nathalie Roux, Stéphanie Friszer, Charles Garabedian, Ferdinand Dhombres, Bettina Bessières, Catherine Fallet-Bianco, Federico Di Rocco, Michel Zerah, Jean-Marie Jouannic
PURPOSE: This study aims to assess the feasibility and the effectiveness of a fetoscopic myelomeningocele (MMC) coverage using a sealed inert patch through a two-port access, in the sheep model. METHODS: Forty-four fetuses underwent surgical creation of a MMC defect at day 75 and were divided into four groups according to the MMC repair technique, performed at day 90. Group 1 remained untreated. Group 2 had an open surgery using suture of the defect. Groups 3 and 4 underwent defect coverage using a Gore®-polytetrafluoroethylene patch secured with surgical adhesive (Bioglue®), with an open approach (group 3) and a fetoscopic one (group 4)...
May 26, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28548634/predictors-of-permanent-disability-among-adults-with-spinal-dysraphism
#12
Matthew C Davis, Betsy D Hopson, Jeffrey P Blount, Rachel Carroll, Tracey S Wilson, Danielle K Powell, Amie B Jackson McLain, Brandon G Rocque
OBJECTIVE Predictors of permanent disability among individuals with spinal dysraphism are not well established. In this study, the authors examined potential risk factors for self-reported permanent disability among adults with spinal dysraphism. METHODS A total of 188 consecutive individuals undergoing follow-up in an adult spinal dysraphism clinic completed a standardized National Spina Bifida Patient Registry survey. Chi-square tests and logistic regression were used to assess bivariate relationships, while multivariate logistic regression was used to identify factors independently associated with self-identification as "permanently disabled...
May 26, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28547209/anesthesia-for-myelomeningocele-surgery-in-fetus
#13
Juan Carlos Devoto, Juan Luis Alcalde, Felipe Otayza, Waldo Sepulveda
BACKGROUND: Administering anesthesia for prenatal repair of myelomeningocele reveals several issues that are unique to this new form of treatment. This includes issues such as fetal well-being, surgical conditions and monitoring, among others. Exploring, analyzing, and understanding the different variables that are involved will help us reduce the high level of risk associated with this surgery. OBJECTIVE: This review provides a systematic approach to the issues that are faced by anesthesiologists during fetal surgery...
May 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28536839/maternal-fetal-surgery-for-myelomeningocele-some-thoughts-on-ethical-legal-and-psychological-issues-in-a-western-european-situation
#14
Frank Van Calenbergh, Luc Joyeux, Jan Deprest
BACKGROUND: The results of the Management of Myelomeningocele Study (MOMS) randomized controlled trial have demonstrated that maternal-fetal surgery (MFS) for myelomeningocele (MMC) compared to postnatal MMC repair has clear neurological benefits for the child at 12 and 30 months of age. Level I evidence nevertheless does not provide answers to many questions in this delicate field. Since the beginning of 2012, our fetal center has been offering MFS for spina bifida aperta (SBA) to patients from different European and non-European countries, in a societal context where termination of pregnancy is the option chosen by most patients when being informed of this diagnosis...
May 23, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28516217/prenatal-surgery-for-myelomeningocele-review-of-the-literature-and-future-directions
#15
Gregory G Heuer, Julie S Moldenhauer, N Scott Adzick
Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations. Historically, this condition has been treated with closure of the MMC defect shortly after birth. The goal of postnatal closure is to cover the exposed spinal cord and prevent infection. However, postnatal surgery does not reverse or prevent the neurologic injury seen in MMC, reverse hindbrain herniation, or prevent hydrocephalus. The neurologic defects result from primary incomplete neurulation and secondary chronic prenatal damage to the exposed neural elements through mechanical and chemical trauma...
May 17, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28516216/the-role-of-intrauterine-magnetic-resonance-in-the-management-of-myelomenigocele
#16
Denise Trigubo, Mercedes Negri, Rosana Mabel Salvatico, Gustavo Leguizamón
OBJECTIVE: To assess the role of magnetic resonance imaging (MRI) in the management of myelomenigocele. BACKGROUND: Spinal dysraphism or neural tube defects (NTD) encompass a heterogeneous group of congenital spinal anomalies that result from the defective closure of the neural tube early in gestation. Myelomeningocele is the most common type of NTD that is compatible with life, with high survival rates but lifelong physical impairments. CONCLUSION: MRI is an important adjunct to ultrasound in assessing NTD, as it pertains to pre-surgical planning and perinatal management...
May 17, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28512689/long-term-results-of-fibular-achilles-tenodesis-westin-s-tenodesis-for-paralytic-pes-calcaneus-is-hypercorrection-avoidable-a-longitudinal-retrospective-study
#17
Helder Henzo Yamada, Patricia Maria Moraes de Barros Fucs
PURPOSE: The purpose of this study was to review all cases of patients submitted to Westin's tenodesis, who had calcaneus feet secondary to myelomeningocele sequel, in order to evaluate the anatomical change provided by surgery and also to verify, in a long-term follow-up, the inversion of the deformity depending on the patient's age. METHODS: In this longitudinal retrospective study, all medical records of patients with myelomeningocele sequelae submitted to Westin's tenodesis from 1993 to 2013 in a public university hospital were reviewed...
May 16, 2017: International Orthopaedics
https://www.readbyqxmd.com/read/28510071/surgical-techniques-for-open-fetal-repair-of-myelomeningocele
#18
Nalin Gupta
The current standard for fetal repair of myelomeningocele is an open exposure of the gravid uterus with control of the amniotic membranes, and then a multi-layer closure of the fetal defect. The nature of the surgical repair has evolved and requires a series of steps that take into account the delicate nature of the fetal tissues. The individual surgical steps are described including potential pitfalls.
May 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28510070/sonographic-detection-of-open-spina-bifida-in-the-first-trimester-review-of-the-literature
#19
César Meller, Horacio Aiello, Lucas Otaño
In the beginnings, sonographic diagnosis of open spina bifida (OSB) relied on the meticulous scanning of the fetal vertebrae for abnormalities but many defects were missed. After the mid-1980s, however, with the description of the intracranial findings in the second trimester (the "lemon sign" and the "banana sign"), the prenatal diagnosis of OSB was enhanced. In the last 2 decades, there has been widespread uptake of routine ultrasound examination in the first trimester of pregnancy with the purpose of the measurement of fetal crown-rump length to determine gestational age, to screen for trisomy 21 and other aneuploidies, mainly with the nuchal translucency, and for diagnosis of many major abnormalities...
May 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28503628/visceral-hyperalgesia-when-to-consider-gabapentin-use-in-neonates-case-study-and-review
#20
Joseph Asaro, Christine A Robinson, Philip T Levy
Visceral hyperalgesia refers to increased pain sensation in response to gastrointestinal sensory stimulus. In neonates with neurological impairments, gabapentin has been successfully used as a treatment for visceral hyperalgesia in neonates. The authors describe a preterm infant with myelomeningocele and persistent neuropathic pain that manifested as irritability, hypertonicity, poor weight gain, and feeding intolerance. After exclusion of other etiologies, the diagnosis of visceral hyperalgesia was suspected and the infant was treated with gabapentin...
January 2017: Child neurology open
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