1,651 results match your criteria Spinal Dysraphism Myelomeningocele


PREVALENCE AND RISK FACTORS FOR SCOLIOSIS IN ADULTS WITH CLOSED AND OPEN SPINA BIFIDA: A LARGE, CROSS-SECTIONAL STUDY.

Ann Phys Rehabil Med 2022 Jun 15:101685. Epub 2022 Jun 15.

Department of Physical Medicine and Rehabilitation, Rennes University Hospital, 35033 Rennes, France; Centre de référence maladies rares Spina Bifida, site constitutif du centre de référence C-MAVEM, CHU Rennes, 35000 Rennes, France; Unité Empenn (ex-Visages) U1228 INSERM-INRIA, IRISA UMR CNRS 6074, Campus de Beaulieu, 35042 Rennes Cedex, France.

Background: Scoliosis develops in a proportion of children with myelomeningocele; however, little is known about scoliosis in adulthood and in other forms of spina bifida (SB).

Objectives: The aims of this study were to describe the prevalence of scoliosis and identify risk factors for its development in a large cohort of adults with open and closed SB.

Methods: This was a cross-sectional study of data from 580 adults with SB attending their first consultation at a French multidisciplinary referral centre for SB. Read More

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Progressive-tension sutures in reconstruction of posterior trunk defects in pediatric patients: A prospective series.

Plast Reconstr Surg 2022 Jun 9. Epub 2022 Jun 9.

From: Craniofacial Center, Division of Plastic and Craniofacial Surgery.

Following neurosurgical repair of spinal dysraphism defects, soft tissue reconstruction is often required to obtain robust coverage of the dura. Layered closure utilizing local muscle and muscle fascia have proven reliable for this purpose, but often result in significant dead space necessitating closed suction drainage. Progressive tension sutures have been reported as an alternative to drains for prevention of fluid collection in a number of other procedures. Read More

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Repair of refractory postoperative cerebrospinal fluid leakage using a reversed dermis flap in a pediatric lipomyelomeningocele patient.

Childs Nerv Syst 2022 Jun 10;38(6):1185-1188. Epub 2022 May 10.

Department of Neurosurgery, Hyogo Prefectural Kobe Children's Hospital, 1-6-7 Minatojima-minamimachi, Chuo-ku, Kobe, Hyogo, 650-0047, Japan.

Introduction: Cerebrospinal fluid (CSF) leak and pseudomeningocele are common complications after surgery for spinal dysraphism.

Case Report And Technique: We report a 6-month-old girl with a lumbosacral lipomyelomeningocele and accessory lower limb who developed a refractory cerebrospinal fluid leak and pseudomeningocele after lipomyelomeningocele repair and removal of the accessory limb. The pseudomeningocele was successfully repaired using a reversed dermis flap made from excess skin that covered the meningocele. Read More

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National trends and outcomes in the use of intravesical botulinum toxin and enterocystoplasty among patients with myelomeningocele.

Urology 2022 May 3. Epub 2022 May 3.

Division of Urology, Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah. Electronic address:

Objectives: To compare trends in the treatment of patients with myelomeningocele receiving intravesical Botulinum (IVB) toxin and enterocystoplasty.

Methods: We identified patients with myelomeningocele in a commercial insurance database from 2008-2017 and stratified them into adult and pediatric samples. Index procedure was identified as either IVB toxin injection or enterocystoplasty. Read More

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Dural substitutes for spina bifida repair: past, present, and future.

Childs Nerv Syst 2022 05 4;38(5):873-891. Epub 2022 Apr 4.

Center for Fetal and Placental Research, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 11025, Cincinnati, OH, USA.

Purpose: The use of materials to facilitate dural closure during spina bifida (SB) repair has been a highly studied aspect of the surgical procedure. The overall objective of this review is to present key findings pertaining to the success of the materials used in clinical and pre-clinical studies. Additionally, this review aims to aid fetal surgeons as they prepare for open or fetoscopic prenatal SB repairs. Read More

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A pilot study assessing average detrusor pressure garnered from area under a urodynamic curve: Evaluation of clinical outcomes.

J Pediatr Urol 2022 Jun 19;18(3):325.e1-325.e9. Epub 2022 Feb 19.

Ege University Department of Pediatric Surgery Division of Pediatric Urology, Turkey. Electronic address:

Introduction: The management of neurogenic bladder mostly relies on urodynamic studies; however, several studies have shown low interobserver reproducibility. The aim of this study was to evaluate if a new objective cystometric parameter was superior to other cystometric measures.

Patients And Methods: A new parameter (pressure adjusted area under curve ratio, PAUC) was formulated from the ratio of area under the cystometry curve to a DLPP-adjusted total area (A). Read More

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Retained Medullary Cord Associated with Terminal Myelocystocele and Intramedullary Arachnoid Cyst.

Pediatr Neurosurg 2022 8;57(3):184-190. Epub 2022 Mar 8.

Department of Neurosurgery, Harasanshin Hospital, Fukuoka, Japan.

Introduction: The retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during the last phase of secondary neurulation. The terminal myelocystocele (TMC) is an unusual type of closed spinal dysraphism, characterized by localized cystic dilatation of the terminal part of the central canal that then herniates through a posterior spinal bifida. The co-occurrence of RMC and TMC is extremely rare. Read More

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Team Approach: The Management of Adolescents and Adults with Scoliosis and Spina Bifida.

JBJS Rev 2022 03 1;10(3). Epub 2022 Mar 1.

Meyer Center for Developmental Pediatrics, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

»: Globally, the prevalence of myelomeningocele, the most common subtype of spina bifida, is 0.2 to 6.5 per 1,000 live births. Read More

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Outcomes of late open fetal surgery for intrauterine spina bifida repair after 26 weeks. Should we extend the Management of Myelomeningocele Study time window?

Prenat Diagn 2022 04 25;42(4):495-501. Epub 2022 Feb 25.

Department of Anesthesiology, Hospital de Especialidades del Niño y la Mujer Dr. Felipe Núñez Lara, Querétaro, México.

Objective: To evaluate maternal and perinatal outcomes of late open fetal repair for open spina bifida (OSB) between 26 -27  weeks.

Methods: A cohort of fetuses with OSB who underwent open surgery in two fetal surgery centers (Argentina and Mexico). Two groups were defined based on the gestational age (GA) at intervention: Management of Myelomeningocele Study (MOMS) time window group: GA 19 -25 , and late intervention group: GA 26 -27 . Read More

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Spinal Dysraphisms: A New Anatomical-Clinicoradiological Classification.

Indian J Radiol Imaging 2021 Oct 11;31(4):809-829. Epub 2022 Jan 11.

Department of Radiodiagnosis, Government Stanley Medical College, Chennai, Tamil Nadu, India.

 Spinal dysraphisms refer to the congenital abnormalities of the spine and spinal cord due to aberrations in the processes of gastrulation, primary neurulation, and secondary neurulation. Embryology of many complex spinal dysraphisms are yet poorly understood and there is no agreeable anatomical-clinicoradiological classification with inclusion of recently documented and complex spinal dysraphisms.  The main objective of this study was to review the imaging features of spinal dysraphisms with a better understanding of embryological abnormalities and propose a new classification inclusive of all complex and unusual dysraphisms based on anatomical and clinicoradiological correlation. Read More

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October 2021

Hydrocephalus in Spina Bifida.

Neurol India 2021 Nov-Dec;69(Supplement):S367-S371

Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, Al. USA 35233, USA.

Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have required treatment with a shunt but recent advances including intra-uterine myelomeningocele closure and ETV-CPC are reducing this burden. The expression of hydrocephalus differs between patients and across the life span. Read More

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February 2022

Increased medial pedicle angulation of the lumbar spine as a function of the posterior osseous defect in patients with myelomeningocele.

Spine Deform 2022 05 30;10(3):709-716. Epub 2022 Jan 30.

Department of Orthopedics, Division of Spine and Scoliosis Surgery, Nemours Children's Health/Alfred I.duPont Hospital for Children, Delaware, 1600, Rockland Road, Wilmington, DE, 19899, USA.

Purpose: Bony anatomy in patients with immature spines has been previously characterized. Alterations in lumbosacral morphology in patients with myelodysplasia, demonstrating increasing medial pedicle angulation (MPA) from L1-S1, have been examined; however, MPA related to size of the defect in myelomeningocele patients has not been defined. We aimed to establish that magnitude of posterior vertebral arch defects determines the extent of MPA in the lumbar spine, with larger posterior defects associated with increased MPA. Read More

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Development of a core outcome set for the orthopaedic management of spinal dysraphism : a study protocol.

Bone Jt Open 2022 Jan;3(1):54-60

St George's Healthcare NHS Trust, London, UK.

Aims: The aim of this study is to define a core outcome set (COS) to allow consistency in outcome reporting amongst studies investigating the management of orthopaedic treatment in children with spinal dysraphism (SD).

Methods: Relevant outcomes will be identified in a four-stage process from both the literature and key stakeholders (patients, their families, and clinical professionals). Previous outcomes used in clinical studies will be identified through a systematic review of the literature, and each outcome will be assigned to one of the five core areas, defined by the Outcome Measures in Rheumatoid Arthritis Clinical Trials (OMERACT). Read More

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January 2022

The Absence of the Neuronal Component in Limited Dorsal Myeloschisis: A Case Report.

Pediatr Neurosurg 2022 17;57(2):127-132. Epub 2022 Jan 17.

Department of Neurosurgery, Maastricht University Medical Centre, Maastricht, The Netherlands.

Introduction: The presence of neuroglial tissue is considered a hallmark in limited dorsal myeloschisis (LDM). However, several reports have indicated that the presence of neuroglial tissue in LDM cannot always be demonstrated. Here, we present such a case of LDM and provide an alternative hypothesis for lacking the neuronal component. Read More

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Comparing Cystatin C- and Creatinine-Estimated Glomerular Filtration Rates in Patients With Thoracic Versus Sacral Motor Levels of Spina Bifida.

Am J Phys Med Rehabil 2022 02;101(2):139-144

From the Department of Pediatric Rehabilitation Medicine, Kennedy Krieger Institute, Baltimore, Maryland (AKM, SAK); Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland (AKM, SAK); Department of Rehabilitation Services, Mt Washington Pediatric Hospital, Baltimore, Maryland (AKM); Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland (TAZ); and Department of Urology, Johns Hopkins University School of Medicine, Baltimore, Maryland (JD).

Objective: Patients with myelomeningocele-type spina bifida are at increased risk of developing kidney disease from neurogenic bladder. Differences between creatinine- and cystatin C-estimated glomerular filtration rates were examined in patients with thoracic versus sacral level myelomeningocele given presumed differences in muscle mass.

Design: A retrospective chart review (2005-2018) was performed on 57 adults with myelomeningocele [thoracic n = 44 (77%); sacral n = 13 (23%)]. Read More

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February 2022

Nephro-urological outcomes of a proactive management of children with spina bifida in their first 5 Years of life.

J Pediatr Urol 2022 Apr 10;18(2):181.e1-181.e7. Epub 2021 Dec 10.

Urology Department, Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina.

Introduction: The proactive management of spina bifida (SB), especially of its severe form, myelomeningocele (MMC), has contributed to decreasing chronic kidney disease (CKD). The objective of this study is to present the evolution of 5-year-old patient with MMC followed from birth with a proactive approach.

Material And Methods: This retrospective study included 55 cases with MMC of up to 5 years of age. Read More

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Fetal-Maternal Surgery for Spina Bifida in a HIV-Infected Mother.

Fetal Diagn Ther 2022 6;49(1-2):25-28. Epub 2022 Jan 6.

Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland.

Introduction: In select cases, in utero surgery for myelomeningocele (MMC) leads to better outcomes than postnatal repair. However, maternal HIV infection constitutes a formal exclusion criterion due to the potential of vertical HIV transmission. Encouraged by a previous case of a successful fetal spina bifida repair in a Hepatitis Bs antigen-positive woman, a plan was devised allowing for fetal surgery. Read More

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Characteristics and first-year mortality, by lesion level, among infants with spina bifida in the New York State Birth Defects Registry, 2008-2017.

Birth Defects Res 2022 01 25;114(2):62-68. Epub 2021 Dec 25.

Department of Epidemiology and Biostatistics, University at Albany School of Public Health, Rensselaer, New York, USA.

Background: Spina bifida is the most common neural tube defect. It has been associated with increased mortality, disability, and may require lifelong medical care. Higher-level lesions have been shown to be associated with increased mortality in infants with spina bifida. Read More

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January 2022

Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics.

J Neurosurg Pediatr 2022 Apr 24;29(4):371-378. Epub 2021 Dec 24.

1Department of Neurosurgery, University of Kansas Medical Center, Kansas City, Kansas.

Objective: As the care of patients with spina bifida continues to evolve, life expectancy is increasing, leading to a critical need for transition planning from pediatric-based to adult-based care. The burden of neurosurgical care for adults with spina bifida remains unknown. In this study, the authors sought to use a large national data set to estimate the prevalence of neurosurgical interventions in adults with spina bifida. Read More

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What is the Role of Scoliosis Surgery in Adolescents and Adults with Myelomeningocele? A Systematic Review.

Clin Orthop Relat Res 2022 04;480(4):773-787

Developmental Pediatrics, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.

Background: Life expectancy of individuals with spina bifida has continued to improve over the past several decades. However, little is known about the longitudinal course of scoliosis in individuals with myelomeningocele (MMC), a spina bifida subtype, across their lifespan. Specifically, it is not known whether management during or after the transition years from adolescence to adulthood is associated with comorbidities in adulthood nor if these individuals benefit from scoliosis treatment later in life. Read More

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Systems biology analysis of human genomes points to key pathways conferring spina bifida risk.

Proc Natl Acad Sci U S A 2021 12;118(51)

Center for Neurogenetics, Feil Family Brain and Mind Research Institute, Weill Cornell Medicine, New York, NY 10021;

Spina bifida (SB) is a debilitating birth defect caused by multiple gene and environment interactions. Though SB shows non-Mendelian inheritance, genetic factors contribute to an estimated 70% of cases. Nevertheless, identifying human mutations conferring SB risk is challenging due to its relative rarity, genetic heterogeneity, incomplete penetrance, and environmental influences that hamper genome-wide association studies approaches to untargeted discovery. Read More

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December 2021

Spina bifida care, education, and research: A multidisciplinary community in a global context.

J Pediatr Rehabil Med 2021 ;14(4):569-570

Spina Bifida Association, Arlington, VA, USA.

Worldwide neural tube defects, such as encephalocele and spina bifida (SB), remain a substantial cause of the global burden of disease; and in the US, Latinos consistently have a higher birth prevalence of SB compared with other ethnic groups. From limited access and fragmented care, to scarcely available adult services, many are the challenges that besiege those living with SB. Thus, to provide inclusion and active involvement of parents of children and adults with SB from all communities, innovative approaches will be required, such as community-based participatory research and culturally competent learning collaboratives. Read More

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February 2022

Scope of care in the first four years of life for individuals born with myelomeningocele: A single institution experience1.

J Pediatr Rehabil Med 2021 ;14(4):667-673

Department of Neurosurgery, Division of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA.

Purpose: Children with myelomeningocele are known to be consumers of substantial healthcare resources, with many early hospital encounters. The purpose of this study was to survey the extent of medical and surgical care that patients with myelomeningocele receive during the first four years of life.

Methods: Clinical and demographic data were collected on newborn infants with open myelomeningocele from the Children's of Alabama Spina Bifida Web Tracker, a prospective, comprehensive spina bifida database. Read More

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February 2022

A qualitative interview study on successful pregnancies in women with spina bifida.

J Pediatr Urol 2022 02 12;18(1):3.e1-3.e7. Epub 2021 Nov 12.

Department of Physical Medicine and Rehabilitation, University of Alabama at Birmingham, United States.

Background: Improvements in antenatal medicine and surgical management for conditions associated with spina bifida such as hydrocephalus have extended the lifespan for individuals with spina bifida (SB) into adulthood. Decisions and education regarding reproductive care and pregnancies for patients with spina bifida are increasingly important. Pregnancy in these patients can be particularly challenging due to physical limitations, previous abdominal surgery for urinary or bowel management and presence of a ventriculoperitoneal shunt. Read More

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February 2022

The Myelic Limited Dorsal Malformation: Prenatal Ultrasonographic Characteristics of an Intermediate Form of Dysraphism.

Fetal Diagn Ther 2021 29;48(9):690-700. Epub 2021 Oct 29.

Service de Radiopédiatrie, Hôpital Armand-Trousseau, AP-HP, Médecine Sorbonne Université, Paris, France.

Objectives: The aim of the study was to report a subtype of dysraphism designated as myelic limited dorsal malformation (MyeLDM) and to describe its characteristics at prenatal ultrasound (US).

Methods: It was a retrospective study from 2014 to 2020 based on second-line US evaluation of patients referred to our institution for myelomeningocele (MMC). Magnetic resonance imaging and acetylcholine esterase evaluation in the amniotic fluid were also offered. Read More

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November 2021

[Effectiveness and safety of sacral neuromodulation on neurogenic bladder and bowel dysfunction in patients with spina bifida].

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2021 Nov;35(11):1374-1379

Department of Urology, Beijing Boai Hospital, China Rehabilitation Research Center, School of Rehabilitation Medicine, Capital Medical University, Beijing, 100068, P.R.China.

Objective: To evaluate the effectiveness and safety of sacral neuromodulation (SNM) in the treatment of neurogenic bladder and bowel dysfunction in patients with spina bifida.

Methods: The clinical data of 33 patients with neurogenic bladder and bowel dysfunction caused by spina bifida treated with SNM between July 2012 and May 2021 were retrospectively analyzed. There were 19 males and 14 females, with an average age of 26. Read More

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November 2021

Site, frequency, and duration of pain in young children with spina bifida.

J Pediatr Rehabil Med 2021 ;14(4):571-582

Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, UT, USA.

Purpose: To investigate the: (1) percent of children with spina bifida (SB) complaining of pain, (2) frequency, duration, and cause of pain by sex, level of lesion type of SB, and ambulation status, (3) body sites reported to hurt, by variables in objective 2, and (4) associations between physical and mental/emotional health between caregiver and child.

Methods: Cross-sectional study of 101 caregivers of children (3 to 6 years old) with SB. Survey data and information from medical records were included. Read More

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February 2022

Open surgery for in utero repair of spina bifida: Microneurosurgery versus standard technique - A systematic review.

Prenat Diagn 2021 Dec 28;41(13):1615-1623. Epub 2021 Oct 28.

Unidad de Cirugía Fetal, Hospital de Especialidades del Niño y la Mujer, y Centro de Diagnóstico Prenatal Avanzado y Cirugía Fetal, Medicina Fetal México, Querétaro, México.

Background/objectives: Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery). Read More

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December 2021