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https://www.readbyqxmd.com/read/29031539/fetal-surgery-for-myelomeningocele-after-the-management-of-myelomeningocele-study-moms
#1
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
#2
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
#3
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
https://www.readbyqxmd.com/read/28980329/a-case-of-prenatally-diagnosed-limited-dorsal-myeloschisis-with-good-prognosis
#4
Anne Sophie Lafitte, Marie Blouet, Frédérique Belloy, Alin Borha, Guillaume Benoist
Diagnosis of fetal spinal dysraphism is a challenge. It is difficult to distinguish between a meningocele, myelomeningocele, and a recently described entity called limited dorsal myeloschisis (LDM). Although myelomeningocele is associated with a poor prognosis, LDM can have a good outcome. We present a case of prenatally diagnosed LDM. Because sonographic examination revealed a round, cystic, septated cervical mass without associated cerebral anomalies, the lesion was initially considered an isolated meningocele...
October 5, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28971794/bowel-preparation-prior-to-reconstructive-urologic-surgery-in-pediatric-myelomeningocele-patients
#5
Nicholas J Farber, Rachel B Davis, Gwen M Grimsby, Brian Shinder, Glenn M Cannon, Micah A Jacobs, Michael C Ost, Francis X Schneck, Heidi A Stephany, Patricio C Gargollo, Moira E Dwyer
INTRODUCTION: Mechanical bowel preparation (MBP) has historically been the standard of care for patients undergoing reconstructive urologic surgery, including urinary diversion. To date, several studies have examined the role of mechanical bowel preparation in postoperative outcomes in pediatric patients undergoing augmentation cystoplasty. However, these patient populations have been heterogeneous in nature, with no studies dedicated to examining the role of MBP prior to reconstructive urologic surgery in pediatric patients with myelomenginoceles...
October 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28948692/mutations-in-folate-transporter-genes-and-risk-for-human-myelomeningocele
#6
Tina O Findley, Joy C Tenpenny, Michelle R O'Byrne, Alanna C Morrison, James E Hixson, Hope Northrup, Kit Sing Au
The molecular mechanisms linking folate deficiency and neural tube defect (NTD) risk in offspring remain unclear. Folate transporters (SLC19A1, SLC46A1, SLC25A32, and FOLH1) and folate receptors (FOLR1, FOLR2, and FOLR3) are suggested to play essential roles in transporting folate from maternal intestinal lumen to the developing embryo. Loss of function variants in these genes may affect folate availability and contribute to NTD risk. This study examines whether variants within the folate transporter and receptor genes are associated with an increased risk for myelomeningocele (MM)...
September 26, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28935364/urological-surveillance-and-medical-complications-among-ault-spina-bifida-medicare-beneficiaries
#7
Yahir Santiago-Lastra, Anne P Cameron, Julie Lai, Christopher Saigal, J Quentin Clemens
OBJECTIVE: To evaluate urologic follow-up and prevalence of medical complications among adult myelomeningocele (MMC) patients in the United States who are Medicare beneficiaries. METHODS: We performed a retrospective study using a 5% Medicare sample from 2007-2010. We defined acceptable minimum follow-up criteria as patients receiving all of the following: serum creatinine, upper urinary tract imaging and a urologist evaluation within a 2-year period. We queried associated diagnoses and relevant complications using ICD-9 codes, graded based on clinical impact...
September 18, 2017: Urology
https://www.readbyqxmd.com/read/28918465/congenital-lumbar-kyphosis-with-skin-ulceration-and-osteomyelitis-in-a-myelomeningocele-child-a-case-report
#8
Benoit Vibert, Marco Turati, Pierre-Yves Rabattu, Marco Bigoni, Ahmad Eid, Aurélien Courvoisier
INTRODUCTION: Kyphosis is a frequent problem in children with spina bifida, and this deformity may cause different complications as respiratory insufficiency, bowel dysfunction, and skin ulcers. CASE REPORT: We report on a 13-year-old myelomeningocele male with a lumbar kyphoscoliosis associated to a septic skin ulceration that resulted in an acute sepsis. An X-ray revealed a kyphosis of 110° and a scoliosis of 25° between T9 and L5. The wound and blood cultures showed Staphylococcus aureus colonization, and an appropriate antibiotic therapy was started...
September 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28910784/fetal-surgery-for-myelomeningocele-a-systematic-review-and-meta-analysis-of-outcomes-in-fetoscopic-versus-open-repair
#9
Sandra K Kabagambe, Guy W Jensen, Yue Julia Chen, Melissa A Vanover, Diana L Farmer
BACKGROUND/OBJECTIVES: The Management of Myelomeningocele (MMC) Study (MOMS) showed that prenatal repair of MMC resulted in improved neurological outcomes but was associated with high rates of obstetrical complications. This study compares outcomes of open and fetoscopic MMC repair. DATA SOURCES: PubMed and Embase studies reporting outcomes of fetal MMC repair published since the completion of the MOMS. RESULTS: We analyzed 11 studies and found no difference in mortality or the rate of shunt placement for hydrocephalus...
September 15, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28904628/tibial-derotational-osteotomies-in-two-neuromuscular-populations-comparing-cerebral-palsy-with-myelomeningocele
#10
R M Thompson, S Ihnow, L Dias, V Swaroop
PURPOSE: To review the outcomes of tibial derotational osteotomies (TDOs) as a function of complication and revision surgery rates comparing a cohort of children with myelodysplasia to a cohort with cerebral palsy (CP). METHODS: A chart review was completed on TDOs performed in a tertiary referral centre on patients with myelodysplasia or CP between 1985 and 2013 in patients aged > 5 years with > 2 years follow-up. Charts were reviewed for demographics, direction/degree of derotation, complications and need for re-derotation...
August 1, 2017: Journal of Children's Orthopaedics
https://www.readbyqxmd.com/read/28904581/split-notochord-syndrome-a-rare-variant
#11
Vidhu Dhawan, Kanchan Kapoor, Balbir Singh, Suman Kochhar, Alka Sehgal, Rima Dada
Split notochord syndrome represents an extremely rare and pleomorphic form of spinal dysraphism characterized by a persistent communication between the endoderm and the ectoderm, resulting in splitting or deviation of the notochord. It manifests as a cleft in the dorsal midline of the body through which intestinal loops are exteriorized and even myelomeningoceles or teratomas may occur at the site. A rare variant was diagnosed on autopsy of a 23(+4)-week-old fetus showing a similar dorsal enteric fistula and midline protruding intestinal loops in thoracolumbar region...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28901576/theory-of-mind-in-spina-bifida-relationship-with-intellectual-and-executive-functioning
#12
Jan Stubberud
This article investigates emotion recognition ability, a central aspect of Theory of Mind (ToM), in a group of individuals with spina bifida myelomeningocele (SBM) experiencing executive function deficits, and examine associations between emotion recognition, and intellectual and executive functioning. A total of 38 adult subjects with SBM were included in this study, participating in a randomized controlled trial evaluating the effects of a cognitive rehabilitation intervention for executive dysfunction. Reading the Mind in the Eyes Test (RMET) was used as a measure of emotion recognition, and neuropsychological tests and questionnaires were utilized as executive function measures...
October 2017: Scandinavian Journal of Psychology
https://www.readbyqxmd.com/read/28888508/functional-independence-of-children-with-myelomeningocele-is-it-associated-with-the-informal-caregivers-burden
#13
Karen Negrão Cavalari, Pedro Tadao Hamamoto Filho, Silvia Maria Caldeira, Helio Rubens de Carvalho Nunes, Fernanda Maria Alves Lima, Marla Andréia Garcia de Avila
PURPOSE: This study aims to evaluate the association between the functional independence of children after postnatal spinal dysraphism correction and informal caregivers' burden. DESIGN AND METHODS: This is a cross-sectional study conducted in a child neurosurgery clinic at a University hospital. We included informal caregivers of children between six months and seven-and-a-half years old who were operated on for spinal dysraphism correction due to myelomeningocele...
September 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28885096/infant-hydrocephalus-in-sub-saharan-africa-the-reality-on-the-tanzanian-side-of-the-lake
#14
Maria M Santos, Derick K Rubagumya, Imani Dominic, Amos Brighton, Soledad Colombe, Philip O'Donnell, Micaella R Zubkov, Roger Härtl
OBJECTIVE Infant hydrocephalus is estimated to affect more than 100,000 new infants each year in sub-Saharan Africa (SSA). Bugando Medical Centre (BMC), a government-funded and patient cost-shared referral center, serves over 13 million people in the Lake and Western regions of Tanzania. The goals of this study were to characterize the infant population affected by hydrocephalus who presented to BMC and were treated with a ventriculoperitoneal shunt (VPS) to determine the rate of early complications associated with this surgical procedure and to assess its potential risk factors...
September 8, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28879597/prenatal-congenital-vertical-talus-rocker-bottom-foot-a-marker-for-multisystem-anomalies
#15
Eva I Rubio, Nimisha Mehta, Anna R Blask, Dorothy I Bulas
BACKGROUND: Congenital vertical talus is a rare foot anomaly characterized by a prominent calcaneus and rigid forefoot dorsiflexion. While congenital vertical talus has been associated with anomalies such as trisomy 18, myelomeningocele and arthrogryposis, postnatal series have reported cases of isolated congenital vertical talus. OBJECTIVE: The purpose of our study was to determine the incidence of isolated congenital vertical talus prenatally and identify the most common anomalies associated with this finding...
September 6, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28876688/management-of-myelomeningocele-in-the-province-of-kwazulu-natal-south-africa
#16
M N Mnguni, B Enicker, T E Madiba
BACKGROUND: Spina bifida is the most common of congenital anomalies of the central nervous system that are compatible with life. The most frequent form is myelomeningocele. METHOD: Setting: Neurosurgery Unit, Inkosi Albert Luthuli Central Hospital. Patients: Consecutive children with diagnosis myelomeningocele who were managed at from January 2006 to December 2014. Design: Retrospective analysis of children with myelomeningocele. Multiple logistic regression analysis identified clinical, demographic and surgical variables that were associated with outcome...
June 2017: South African Journal of Surgery. Suid-Afrikaanse Tydskrif Vir Chirurgie
https://www.readbyqxmd.com/read/28873371/modified-hysterotomy-closure-technique-for-open-fetal-surgery
#17
Michael V Zaretsky, Kenneth W Liechty, Henry L Galan, Nicholas J Behrendt, Shane Reeves, Ahmed I Marwan, Corbett Wilkinson, Michael Handler, Megan Lagueux, Timothy M Crombleholme
OBJECTIVE: We reviewed our experience with open fetal surgical myelomeningocele repair to assess the efficacy of a new modification of the hysterotomy closure technique regarding hysterotomy complication rates at the time of cesarean delivery. METHODS: A modification of the standard hysterotomy closure was performed on all patients undergoing prenatal myelomeningocele repair. The closure consisted of an interrupted full-thickness #0 polydioxanone (PDS) retention suture as well as a running #0 PDS suture to re-approximate the myometrial edges, and the modification was a third imbricating layer resulting in serosal-to-serosal apposition...
September 6, 2017: Fetal Diagnosis and Therapy
https://www.readbyqxmd.com/read/28867320/complete-reversibility-of-the-chiari-type-ii-malformation-following-post-natal-repair-of-myelomeningocele
#18
P A Beuriat, A Szathmari, C Rousselle, I Sabatier, F di Rocco, C Mottolese
INTRODUCTION: It was believed that Chiari type II malformation (CM-II) was always present in a myelomeningocele (MMC). In fact, it is associated in about 80 % of cases. Improvement of the hindbrain herniation after prenatal closure of MMC has challenged the idea that this condition was irreversible. Only two studies report ascent of the cerebellar tonsil after post-natal closure. This work aimed to study a large group of MMC patients who benefitted from a post-natal repair to evaluate the rate of long-term total reversibility of CM-II...
August 31, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28836807/health-self-management-transition-readiness-and-adaptive-behavior-in-persons-with-cerebral-palsy-or-myelomeningocele
#19
Seth Warschausky, Jacqueline N Kaufman, William Schutt, Michael Evitts, Edward A Hurvitz
PURPOSE/OBJECTIVE: This study was conducted to examine the associations between generic and condition-specific health self-management and levels of adaptive behavior in 2 groups of transition-age youth with congenital neurodevelopmental conditions. METHOD: The sample included 43 adolescents/young adults diagnosed with cerebral palsy (CP) and 36 with spina bifida/myelomeningocele (SBM), ages 13-29, mean age 18.96 (4.77), 51.9% female. Health self-management was assessed with the Transition Readiness Assessment Questionnaire (TRAQ) completed by the child, and the Kennedy Krieger Independence Scales-Spina Bifida (KKIS-SB) completed by the parent/guardian...
August 2017: Rehabilitation Psychology
https://www.readbyqxmd.com/read/28832491/committee-opinion-no-720-maternal-fetal-surgery-for-myelomeningocele
#20
(no author information available yet)
Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies...
September 2017: Obstetrics and Gynecology
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