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Myelomeningocele

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https://www.readbyqxmd.com/read/29333813/body-composition-and-energy-expenditure-in-a-population-of-children-and-adolescents-with-myelomeningocele
#1
Carolina Caminiti, Carola Saure, Julieta Weglinski, Fernanda de Castro, Liliana Campmany
INTRODUCTION: Myelomeningocele is a congenital defect that occurs when the neural tube fails to close completely. It causes body composition alterations and a high prevalence of obesity. It is difficult to detect the most adequate indicator for a nutritional diagnosis due to the impossibility of recording accurate anthropometric measurements. OBJECTIVE: To describe body composition, resting energy expenditure and metabolic disorders in a population of patients with myelomeningocele managed at "Hospital Garrahan" by comparing obese patients with myelomeningocele and a control population with multifactorial obesity...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29321944/myelomeningocele-with-unilateral-right-renal-agenesis-a-case-report
#2
Hajime Maeda, Hayato Go, Jun Sakuma, Takashi Imamura, Maki Sato, Nobuo Momoi, Mitsuaki Hosoya
Congenital anomalies of the spine may occur with malformations of the central nervous, cardiovascular, gastrointestinal, respiratory, and genitourinary systems. This is a case of myelomeningocele with unilateral right renal agenesis in a newborn. The patient suffered complications of cerebrospinal fluid leak and meningitis, but was successfully treated and discharged on day 86. In this case, unilateral right renal agenesis represented a significant surgical risk because failure of the remaining kidney could result in renal failure...
January 2018: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29319575/duplex-ultrasonography-assisted-freestyle-pedicled-perforator-flaps-for-the-repair-of-myelomeningocele-defects
#3
Atsushi Imaizumi
BACKGROUND: Perforator flaps have unique advantages that may overcome the shortcomings of conventional approaches to myelomeningocele reconstruction. However, identifying and dissecting tiny perforators in neonates is arduous. We have overcome these obstacles with a freestyle flap approach that uses duplex ultrasonography to locate perforator vessels, allowing for limited superficial dissection. This report describes the duplex ultrasonography-assisted freestyle pedicled perforator flap technique for closure of myelomeningocele defects, with long-term clinical outcomes...
January 9, 2018: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/29316543/the-use-of-external-ventricular-drainage-to-reduce-the-frequency-of-wound-complications-in-myelomeningocele-closure
#4
Michael M McDowell, Philip S Lee, Kimberly A Foster, Stephanie Greene
INTRODUCTION: Myelomeningocele (MMC) is an open neural tube defect routinely surgically closed within 48 h of birth to prevent secondary infection. Up to 18% of patients experience wound complications, and 85% require shunting for hydrocephalus. We hypothesized that wound complications could be reduced by cerebrospinal fluid (CSF) diversion at the time of closure. METHODS: Institutional review board approval was obtained to review records of the 88 patients who underwent MMC closure between January 2005 and June 2016 at the Children's Hospital of Pittsburgh...
January 10, 2018: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29314321/percutaneous-fetoscopic-closure-of-large-open-spina-bifida-using-a-bilaminar-skin-substitute
#5
Denise A Lapa Pedreira, Gregório L Acacio, Rodrigo T Gonçalves, Renato Augusto M Sá, Reynaldo A Brandt, Ramen Chmait, Eftichia Kontopoulos, Ruben A Quintero
OBJECTIVE: We have previously described our percutaneous fetoscopic technique for the treatment of open spina bifida (OSB). However, approximately 20-30% of OSB defects are too large to allow primary skin closure. We hereby describe a modification of our standard technique using a bilaminar skin substitute to allow closure of such large spinal defects. The aim of this study was to report our clinical experience with the use of a bilaminar skin substitute and a percutaneous fetoscopic technique for the prenatal closure of large spina bifida defects...
January 4, 2018: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29313712/energy-efficiency-in-children-with-myelomeningocele-during-acute-use-of-assistive-devices-a-pilot-study
#6
Jennifer K Sansom, Beverly D Ulrich
Due to increased metabolic demands during walking, ∼50% of children with myelomeningocele transition to wheelchair use during adolescence/early adulthood. The purpose of our pilot study involving children with myelomeningocele was to determine: (a) energy expenditure needs during acute use of common assistive devices and (b) if walking poles are a feasible assistive device. Oxygen uptake was recorded for eight (5-12 years old) children in four conditions: independent, walker, crutches, and poles. Acute pole use did not significantly differ from independent walking net energy consumption or cost...
January 9, 2018: Adapted Physical Activity Quarterly: APAQ
https://www.readbyqxmd.com/read/29302380/a-case-of-fatal-pulmonary-hypoplasia-with-congenital-diaphragmatic-hernia-thoracic-myelomeningocele-and-thoracic-dysplasia
#7
Ai Ito, Hideshi Fujinaga, Sachiko Matsui, Kumiko Tago, Yuka Iwasaki, Shuhei Fujino, Junko Nagasawa, Shoichiro Amari, Masao Kaneshige, Yuka Wada, Shigehiro Takahashi, Keiko Tsukamoto, Osamu Miyazaki, Takako Yoshioka, Akira Ishiguro, Yushi Ito
Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29284599/spinal-arteriovenous-vascular-malformations-in-patients-with-neural-tube-defects
#8
E Giordan, C Bortolotti, G Lanzino, W Brinjikji
BACKGROUND AND PURPOSE: Neural tube defects, such as tethered cord, intradural lipoma, or myelomeningocele may coexist with spinal vascular malformations. The coexistence of these 2 rare entities is suggestive of a causal relationship between them, which may lead to further understanding of their pathogenesis. We present a series of 6 patients with epidural spinal arteriovenous fistulas associated with neural tube defects. MATERIALS AND METHODS: We retrieved cases of spinal vascular malformations associated with neural tube defects seen at our institution...
December 28, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29249073/medical-and-socioeconomic-predictors-of-quality-of-life-in-myelomeningocele-patients-with-shunted-hydrocephalus
#9
Brij S Karmur, Abhaya V Kulkarni
INTRODUCTION: Children with myelomeningocele (MMC) often develop hydrocephalus, and the combination of these conditions can lead to psychosocial, cognitive, and physical health issues that decrease their health-related quality of life (HRQOL). The goal of the present study was to understand the QOL in patients with MMC and shunted hydrocephalus. METHODS: Data from the Toronto Hydrocephalus QOL Database was acquired between 2004 and 2009 using parent-completed questionnaires and, when appropriate, child-completed questionnaires: Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index...
December 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29248276/pre-operative-risk-factors-for-stomal-stenosis-with-malone-antegrade-continence-enema-procedures
#10
A J Rensing, J F Koenig, P F Austin
INTRODUCTION: The Malone antegrade continence enema (MACE) procedure remains an effective tool in providing independence to patients with refractory constipation. Appendiceal, ileal and colonic segments are all used depending upon the patient's individual anatomy and surgeon's preference. Unfortunately, MACE stomal stenosis (SS) remains a frustrating complication. It was hypothesized that SS is associated with the type of efferent limb used for MACE construction. OBJECTIVE: To perform a retrospective risk analysis of the pre-operative factors that correlated with postoperative stomal stenosis...
December 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29246577/the-management-of-myelomeningocele-study-full-cohort-30-month-pediatric-outcomes
#11
Diana L Farmer, Elizabeth A Thom, John W Brock, Pamela K Burrows, Mark P Johnson, Lori J Howell, Jody A Farrell, Nalin Gupta, N Scott Adzick
BACKGROUND: Previous reports from the Management of Myelomeningocele Study (MOMS) demonstrated that prenatal repair of myelomeningocele reduces hindbrain herniation, the need for cerebrospinal fluid shunting, and improves motor function in children with myelomeningocele. The trial was stopped for efficacy after 183 patients had been randomized, but 30-month outcomes were only available at the time of initial publication in 134 mother-child dyads. Data from the complete cohort for the 30-month outcomes are presented here...
December 12, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29243972/endoscopic-third-ventriculostomy-and-choroid-plexus-cauterization-in-infant-hydrocephalus-a-prospective-study-by-the-hydrocephalus-clinical-research-network
#12
Abhaya V Kulkarni, Jay Riva-Cambrin, Curtis J Rozzelle, Robert P Naftel, Jessica S Alvey, Ron W Reeder, Richard Holubkov, Samuel R Browd, D Douglas Cochrane, David D Limbrick, Tamara D Simon, Mandeep Tamber, John C Wellons, William E Whitehead, John R W Kestle
OBJECTIVE High-quality data comparing endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) to shunt and ETV alone in North America are greatly lacking. To address this, the Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study of ETV+CPC in infants. Here, these prospective data are presented and compared to prospectively collected data from a historical cohort of infants treated with shunt or ETV alone. METHODS From June 2014 to September 2015, infants (corrected age ≤ 24 months) requiring treatment for hydrocephalus with anatomy suitable for ETV+CPC were entered into a prospective study at 9 HCRN centers...
December 15, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29236131/trends-in-incidence-and-long-term-outcomes-of-myelomeningocele-in-british-columbia
#13
Taylor North, Alexander Cheong, Paul Steinbok, Julia Ae Radic
PURPOSE: Myelomeningocele is typically a disabling condition that results in neurologic, orthopedic, and urologic morbidity. The aim of this study was to examine the trends over time in both incidence and outcomes of myelomeningocele (MMC) in British Columbia (BC). METHODS: A retrospective chart review was performed of all children with MMC followed in the British Columbia Children's Hospital (BCCH) Spinal Cord Clinic between 1971 and 2016. The incidence of new MMC cases and the long-term outcomes of MMC were compared between two 10-year cohorts...
December 13, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29229221/intravesical-electromotive-botulinum-toxin-type-a-administration-for-management-of-urinary-incontinence-secondary-to-neuropathic-detrusor-overactivity-in-children-long-term-follow-up
#14
Seyedeh-Sanam Ladi-Seyedian, Lida Sharifi-Rad, Abdol-Mohammad Kajbafzadeh
OBJECTIVES: To investigate the long-term efficacy and success rate of intravesical electromotive botulinum toxin type A (BoNTA) "Dysport®" administration in myelomeningocele (MMC) patients who had urinary incontinence due to neuropathic detrusor overactivity (NDO). METHODS: Twenty four MMC patients (mean age: 9± 3.6 years, range: 3-16) were included in the study and followed up for six years. Using an electrode bladder catheter, 10 IU/kg of Dysport® (BoNTA) was inserted into the bladder for electromotive drug administration (EMDA) without anesthesia on outpatient basis...
December 8, 2017: Urology
https://www.readbyqxmd.com/read/29222684/how-much-do-plastic-surgeons-add-to-the-closure-of-myelomeningoceles
#15
Rhian Bevan, Nicholas Wilson-Jones, Imran Bhatti, Chirag Patel, Paul Leach
PURPOSE: This study reviews the outcomes of children undergoing myelomeningocele (MMC) repair in the paediatric neurosurgical department in Cardiff. These procedures are historically performed by paediatric neurosurgeons with occasional support from plastic surgeons for the larger lesions. We reviewed the postoperative outcomes over a 9-year period to assess the efficacy of having a plastic surgeon present at all MMC closures. METHODS: Analysis of a prospectively collected database of all MMC closures performed at University Hospital Wales from April 2009 to August 2017 was used...
December 8, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29221695/sleep-disordered-breathing-among-newborns-with-myelomeningocele
#16
Renée A Shellhaas, Payal V Kenia, Fauziya Hassan, John D E Barks, Niko Kaciroti, Ronald D Chervin
In a matched cohort study, we report that the apnea-hypopnea index is significantly higher in neonates with myelomeningocele (34 ± 22) compared with age-matched controls (19 ± 11; P = .021). Assessment of newborns with myelomeningocele for sleep-disordered breathing may facilitate early treatment; the impact on long-term neurodevelopment is unknown.
December 6, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29196068/incidental-discovery-of-adenocarcinoma-of-an-augmented-bladder-in-a-patient-with-myelomenigocele-undergoing-cystolithotomy
#17
Majdee M Islam, Amanda F Saltzman, Alonso Carrasco, Ty Higuchi
Bladder malignancy in patients with congenital bladder anomalies who have undergone bladder augmentation is a rare but well recognized condition. These patients present with locally advanced or metastatic disease and have poor survival. We report a case of a patient with myelomeningocele who was incidentally found to have a high grade intestinal type adenocarcinoma of her bladder augment at the time of cystolithotomy. This case highlights the need to continue to follow patients with congenital bladder anomalies and questions if there are better screening methods available...
November 28, 2017: Urology
https://www.readbyqxmd.com/read/29190654/prenatal-folic-acid-use-associated-with-decreased-risk-of-myelomeningocele-a-case-control-study-offers-further-support-for-folic-acid-fortification-in-bangladesh
#18
Vijaya Kancherla, Md Omar Sharif Ibne Hasan, Rezina Hamid, Ligi Paul, Jacob Selhub, Godfrey Oakley, Quazi Quamruzzaman, Maitreyi Mazumdar
Neural tube defects contribute to severe morbidity and mortality in children and adults; however, they are largely preventable through maternal intake of folic acid before and during early pregnancy. We examined the association between maternal prenatal folic acid supplement intake and risk of myelomeningocele (a severe and common type of neural tube defect) in the offspring. We performed secondary analysis using data from a case-control study conducted at Dhaka Community Hospital, Bangladesh between April and November of 2013...
2017: PloS One
https://www.readbyqxmd.com/read/29170734/the-prevalence-and-distribution-of-spina-bifida-in-a-single-major-referral-center-in-malaysia
#19
Adibah Sahmat, Renuka Gunasekaran, Siti W Mohd-Zin, Lohis Balachandran, Meow-Keong Thong, Julia P Engkasan, Dharmendra Ganesan, Zaliha Omar, Abu Bakar Azizi, Azlina Ahmad-Annuar, Noraishah M Abdul-Aziz
Background: The aim of this study is to review the medical history of patients with spina bifida, encompassing both aperta and occulta types born between the years 2003 until 2016, spanning a 13-year time period. We assessed each patient and maternal parent information, details of the defects, and conditions associated with the primary defect. We also include information on patients' ambulation and education level (where available). Methods: Data from the Department of Patient Information University of Malaya Medical Centre (UMMC), Malaysia was captured from spina bifida patients (ICD10: Q05 spina bifida)...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29159427/shunt-revision-rates-in-myelomeningocele-patients-in-the-first-year-of-life-a-retrospective-study-of-52-patients
#20
Ibrahim Alatas, Gokhan Canaz, Nesrin Akkoyun Kayran, Nursu Kara, Huseyin Canaz
PURPOSE: Shunt placement indications are stringent and require confirmation of clinical and radiological evidence of hydrocephalus (HC). The aim of this study was to determine the rate of shunting and discuss the outcome in the first year of life in patients with myelomeningocele (MMC) on the basis of review of the literature. METHODS: All patients who underwent postnatal repair of MMC at our institution between March 2014 and March 2015 were evaluated. Patients were only included if they underwent both MMC repair and ventriculoperitoneal (VP) shunt insertion at our institution and were followed up for at least 12 months...
November 20, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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