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J Neurosurg Pediatr 2021 May 7:1-7. Epub 2021 May 7.

8Developmental Behavioral Pediatrics, Seattle Children's Hospital and University of Washington College of Medicine, Seattle, Washington.

Objective: The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics.

Methods: The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Read More

J Neurosurg Pediatr 2021 May 7:1-10. Epub 2021 May 7.

1Section of Pediatric Neurosurgery, Department of Neurological Surgery, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis.

Objective: Tethered cord syndrome (TCS) is a clinical and radiographic diagnosis of pathological stretching of the spinal cord leading to progressive loss of neurological function. The gold standard treatment for TCS is a tethered cord release. However, detethering involves significant risks of spinal cord injury and high rates of retethering. Read More

Acta Chir Orthop Traumatol Cech 2021 ;88(2):95-100

Oddělení ortopedie a traumatologie, AGEL Středomoravská nemocniční a.s., Nemocnice Přerov.

PURPOSE OF THE STUDY The study aimed to evaluate the intraoperative and early postoperative response to simultaneous bilateral femoral osteotomy usually accompanied by soft tissue release of hip joints, or open reduction, capsuloplasty, pelvic osteotomy or extraarticular shelf procedure. MATERIAL AND METHODS A bilateral surgery was performed in 16 children. Twelve children suffered from (spastic) cerebral palsy and there was one case of paralytic dislocation in a patient with myelomeningocele, while the remaining patients suffered from chromosome I aberrations, Dandy-Walker syndrome and merosin-deficient muscular dystrophy. Read More

Am J Obstet Gynecol 2021 May 3. Epub 2021 May 3.

Baylor College of Medicine Department of Obstetrics and Gynecology, Division of Fetal Therapy and Surgery, and Texas Children's Hospital Fetal Center, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine.

Background: In-utero closure of meningomyelocele using an open hysterotomy approach is associated with preterm delivery and its attendant adverse neonatal outcomes.

Objectives: This study compares neonatal outcomes between in-utero closure of meningomyelocele using a 2-port, exteriorized uterus, fetoscopic approach and the conventional open hysterotomy approach.

Study Design: This retrospective cohort study included all consecutive patients who underwent in-utero meningomyelocele closure using open hysterotomy (n=44) or a 2- port, exteriorized uterus, fetoscopic approach (n=46) at a single institution, between 2012 and 2020. Read More

Hinyokika Kiyo 2021 Mar;67(3):103-107

The Department of Urology, enpan Community Health-Care Organization Hoshigaoka Medical Center.

A 38-year-old woman with neurogenic bladder caused by myelomeningocele,who had been on clean intermittent self-catheterization for years,complained of gross hematuria. Computerized tomography, urinary cytology and cystoscopy failed to reveal causative lesions and the hematuria ceased spontaneously. Because gross hematuria recurred 2 years later,we examined the patient again and detected calcification of the urinary bladder. Read More

J Pediatr Neurosci 2020 Oct-Dec;15(4):393-401. Epub 2021 Jan 19.

Department of Neurology, Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey.

Objective: Myelomeningocele is the most severe and the most frequent form of spina bifida. Most of the myelomeningocele patients undergo operations in new-born age. In terms of life quality and rehabilitation, follow-up's of these patients in the growth and development period after the operation is critical. Read More

J Pediatr Rehabil Med 2021 Apr 29. Epub 2021 Apr 29.

Department of Physical Therapy, Tel Aviv University, Tel Aviv, Israel.

Purpose: To assess hand dexterity in children with myelomeningocele (MMC) and to explore factors related to hand dexterity in these children.

Methods: Ninety-four children with myelomeningocele, aged 4 to 18 years, were assessed. Demographic characteristics, disease factors, visual perception (Beery test of Visual Motor Integration), cognition (WeeFunctional Independence Measure), and self-care (Pediatric Evaluation of Disability Inventory) were assessed in relation to the Nine-Hole Peg Test (9HPT) for hand dexterity using Spearmen correlations and linear regressions. Read More

Neuroradiology 2021 May 1. Epub 2021 May 1.

School of Biomedical Engineering & Imaging Sciences (BMEIS), King's College London, London, UK.

Purpose: A retrospective study was performed to study the effect of fetal surgery on brain development measured by MRI in fetuses with myelomeningocele (MMC).

Methods: MRI scans of 12 MMC fetuses before and after surgery were compared to 24 age-matched controls without central nervous system abnormalities. An automated super-resolution reconstruction technique generated isotropic brain volumes to mitigate 2D MRI fetal motion artefact. Read More

J Pediatr Surg 2021 Apr 15. Epub 2021 Apr 15.

Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.

Introduction: Intraamniotic microparticle injection is a novel technique for the treatment of myelomeningocele (MMC) in which microparticles are delivered in-utero in a minimally invasive fashion to bind to and protect the exposed spinal cord. This technique could offer earlier intervention and greater access to prenatal treatment of MMC. Here we demonstrate progress on the engineering of the microparticles to promote binding to the MMC defect. Read More

Arch Gynecol Obstet 2021 May 1. Epub 2021 May 1.

Disciplina de Obstetricia, Departamento de Obstetricia e Ginecologia, Hospital das Clinicas, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Avenida Dr. Eneas de Carvalho Aguiar, 255 - 10° andar - Cerqueira Cesar, Sao Paulo, CEP 05403-000, Brazil.

Purpose: Myelomeningocele (MMC) is an open neural tube defect that causes great morbidity. Prenatal open repair is the standard treatment; however, there are many complications related to the procedure. This study reports preliminary findings of open in utero repair of MMC in a public tertiary hospital in Brazil and describes factors that could be associated with increased surgical morbidity. Read More

Folia Med (Plovdiv) 2021 Apr;63(2):272-276

Medical University of Plovdiv, Plovdiv, Bulgaria.

Mermaid syndrome or sirenomelia is an extremely rare congenital malformation with an incidence between 1.5 and 4.2 per 1,000,000 pregnancies. Read More

J Gynecol Obstet Hum Reprod 2021 Apr 26:102155. Epub 2021 Apr 26.

Sorbonne University, AP-HP, Trousseau Hospital, DMU ORIGYNE, Department of Fetal Medicine, 26 Avenue du Dr Arnold Netter, 75012 Paris, France.; National Reference Center for Rare Disease: Vertebral and Spinal Cord Anomalies (MAVEM Center), AP-HP, Trousseau Hospital, 26 Avenue du Dr Arnold Netter, 75012 Paris, France.

Introduction: - Open fetal myelomeningocele (MMC) surgery is currently the standard of care option for prenatal MMC repair. We described the population referred to our center and reviewed outcome after open fetal MMC repair.

Material And Methods: - All patients referred to our center for MMC were reviewed from July 2014 to June 2020. Read More

J Korean Neurosurg Soc 2021 Apr 29. Epub 2021 Apr 29.

Neuro-oncology Clinic, Center for Rare Cancers, National Cancer Center, Goyang, Korea.

Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Read More

Ultrasound Obstet Gynecol 2021 Apr 20. Epub 2021 Apr 20.

Fetal Medicine Research Institute, King's College Hospital, London, UK.

Background: A trial comparing prenatal with postnatal open spina bifida (OSB) repair established that prenatal surgery was associated with better postnatal outcome. However, in the trial the fetal surgery was carried out through hysterotomy. Minimally invasive approaches are being developed to mitigate the risks of open maternal fetal surgery. Read More

Dev Neurorehabil 2021 Apr 19:1-14. Epub 2021 Apr 19.

Division of Pediatric Neurology, Duke University Health System, Durham, United States.

: Though much research has been done on the cognitive profiles of children, the abilities of patients with SBM as they age into adulthood are not well understood.: Determine if adults with SBM have impairments in overall cognition, attention, executive function, and memory compared to typically developing adults or a standardized population mean.: A medical librarian composed a search of spina bifida, adults, and cognitive function. Read More

Int Braz J Urol 2021 Feb 28;47. Epub 2021 Feb 28.

Service of Urology, Italian Hospital, Buenos Aires, Argentina.

Introduction: Defective closure of the neural tube affects different systems and generates sequelae, such as neurogenic bladder (NB). Myelomeningocele (MMC) represents the most frequent and most severe cause of NB in children. Damage of the renal parenchyma in children with NB acquired in postnatal stages is preventable given adequate evaluation, follow-up and proactive management. Read More

Fetal Diagn Ther 2021 Apr 14:1-8. Epub 2021 Apr 14.

Department of Anesthesiology, Perioperative and Pain Medicine,Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

Background: The fetoscopic approach to the prenatal closure of a neural tube defect (NTD) may offer similar advantages to the newborn compared to prenatal open closure of a NTD, with a reduction in maternal risks. Enhanced recovery after surgery (ERAS) protocols have been applied to different surgical procedures with documented advantages. We modified the perioperative care of patients undergoing in utero repair of myelomeningocele with the goal of enhancing the recovery. Read More

Pediatr Neurosurg 2021 13;56(3):229-238. Epub 2021 Apr 13.

Department of Neurosurgery, Children's Hospital of Pittsburgh University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

Objective: We sought to compare our large single-institution cohort of postnatal myelomeningocele closure to the 2 arms of the Management of Myelomeningocele Study (MOMS) trial at the designated trial time points, as well as assess outcomes at long-term follow-up among our postnatal cohort.

Methods: A single-institutional retrospective review of myelomeningocele cases presenting from 1995 to 2015 at Children's Hospital of Pittsburgh was performed. We compared outcomes at 12 and 30 months to both arms of the MOMS trial and compared our cohort's outcomes at those designated time points to our long-term outcomes. Read More

Oper Neurosurg (Hagerstown) 2021 Apr 2. Epub 2021 Apr 2.

The Daniel and Jane Och Spine Hospital, New York Presbyterian/Columbia University Medical Center, New York, New York, USA.

Background And Importance: Lumbar hyperlordosis in ambulatory children is an uncommon but potentially problematic spinal deformity, and the operative management has not been comprehensively described.

Clinical Presentation: We report the case of a 14-yr-old girl presenting with severe progressive lumbar hyperlordosis (-122°) and sagittal imbalance (-6 cm). She had multiple prior surgeries, including myelomeningocele repair at 10 d old, midlumbar meningioma resection at 8 mo old, and posterior lumbar instrumented spinal fusion at 5 yr old. Read More

Homo 2021 Apr 6. Epub 2021 Apr 6.

Department of Human Biology, Józef Piłsudski University of Physical Education in Warsaw Marymoncka St. 34, 00-968 Warsaw, Poland.

The aim of this cross-sectional study was to evaluate the relationship between the condition of bone mineral density and body composition and the adapted physical activity, the number of past fractures and selected somatic characteristics of girls with myelomeningocele (MMC). The study examined 37 girls with myelomeningocele (MMC) at the age of 11.9 ± 1. Read More

J Perinat Med 2021 Apr 5. Epub 2021 Apr 5.

Hospital Israelita Albert Einstein, Fetal Therapy Program, São Paulo, SP, Brazil.

Skin-over-biocellulose for Antenatal Fetoscopic Repair (SAFER) is a recently developed technique for fully percutaneous fetoscopic repair of myelomeningocele. The formation of a neo-dura mater triggered by the use of a biocellulose patch over the placode obviates the need of primary repair of fetal dura mater, and seems to further improve neurodevelopmental outcome. Insufflation of humidified carbon dioxide into the amniotic cavity requires proper training and a different equipment set from those used in the classic "under-water" fetoscopy. Read More

Children (Basel) 2021 Mar 10;8(3). Epub 2021 Mar 10.

Department of Clinical Sciences, Pediatrics, Lund University, 22185 Lund, Sweden.

Background: The primary aim was to describe patient-reported morbidity from neurogenic bladder and bowel dysfunction in a cohort of children with spina bifida. The secondary aim was to describe the overall surgical burden in these children.

Methods: Children with meningocele or myelomeningocele, born between 2000-2016, and followed by a tertiary spina bifida center were evaluated in a cross-sectional cohort study using data from charts and a prospective national follow-up program. Read More

Pediatr Neurosurg 2021 30;56(3):261-267. Epub 2021 Mar 30.

Department of Neurosurgery, AIIMS, New Delhi, India.

Introduction: Intraspinal epidermoid cysts are congenital or acquired in origin; whereas intraspinal neurenteric cysts (NECs) are of congenital origin. Their individual association with spinal dysraphism and vertebral segmentation anomalies is very well known.

Case Presentation: We hereby report a case of concurrent intradural extramedullary epidermoid and NEC at adjacent vertebral levels in a spinal dysraphism child, not reported in English Literature till now. Read More

Case Rep Anesthesiol 2021 5;2021:6679845. Epub 2021 Mar 5.

Division of Pediatric Surgery, Hasbro Children's Hospital, The Warren Alpert Medical School of Brown University, Providence, RI 02903, USA.

Effective tocolysis is essential after fetal myelomeningocele repair and is associated with the development of pulmonary edema. The increased uterine activity in the immediate postoperative period is commonly treated with magnesium sulfate. However, other tocolytic agents such as nitroglycerine, nifedipine, indomethacin, terbutaline, and atosiban (outside the US) have also been used to combat uterine contractility. Read More

Stem Cell Rev Rep 2021 Mar 19. Epub 2021 Mar 19.

Pediatric Urology and Regenerative Medicine Research Center, Section of Tissue Engineering and Stem Cells Therapy, Children's Hospital Medical Center, Tehran University of Medical Sciences, No. 62, Dr. Gharib's Street, Keshavarz Boulevard, Tehran, 1419433151, Iran.

Neural tube defects (NTDs) are among the most common congenital defects during neurulation. Spina bifida is a type of NTD that can occur in different forms. Since myelomeningocele (MMC) is the most severe form of spina bifida, finding a satisfactory treatment for MMC is a gold standard for the treatment of spina bifida. Read More

Br J Surg 2021 Mar 15. Epub 2021 Mar 15.

Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, UK.

Fetal medicine is a super-specialty enterprise and a technology-driven field. The growth and interest in fetal surgery can be largely attributed to advances in fetal imaging and bespoke instruments for in utero intervention. Previously fatal fetal conditions are now being treated using open surgery, minimally invasive procedures, and percutaneous fetal technologies. Read More

J Pediatr Urol 2021 Feb 23. Epub 2021 Feb 23.

Department of Urology, Biruni University, Istanbul, Turkey. Electronic address:

Objective: This study aimed to determine the effectiveness of intradetrusor injections of onabotulinum toxin-A (BoNT-A) in pediatric patients with neurogenic bladders (NB) due to myelomeningocele (MMC). The factors predicting success were also evaluated.

Study Design: We retrospectively identified 62 patients with NB due to MMC who underwent intravesical BoNT-A injection (100-300 U) between May 2013 and December 2018. Read More

Childs Nerv Syst 2021 Mar 11. Epub 2021 Mar 11.

Dipartimento Scienze della salute della donna, del bambino e di sanità pubblica - Unità Operativa Spina Bifida e Uropatie Malformative, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Purpose: A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and completed a neuropsychological battery in order to evaluate their cognitive, personality, and behavior profile.

Methods: Enrolled children underwent cognitive assessment by means of the Weschler WISC-IV cognitive test and assessment of the attention sustained through the LEITER test. In addition, parents were asked, in order to obtain a personality and behavior profile of the children, to fill in a "CBCL 6-18 years" questionnaire and to fill in a Barthel Index questionnaire. Read More

Pediatr Cardiol 2021 Mar 10. Epub 2021 Mar 10.

Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, 6651 Main St, Houston, TX, E192077030, USA.

Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require significant operations shortly after birth. Few studies have examined the impact of these combined lesions. We sought to examine the incidence of CHD in patients with MMC, and to evaluate length of stay (LOS), hospital charges, and mortality. Read More

Eur J Obstet Gynecol Reprod Biol 2021 Apr 16;259:185-190. Epub 2021 Feb 16.

Department of Obstetrics and Gynecology, Baylor College of Medicine and Texas Children's Hospital Pavilion for Women, Houston, TX, USA. Electronic address:

Objective: To estimate the significance of the association between mid-trimester maternal serum alpha-fetoprotein (MSAFP) level and fetal neuroanatomic findings in cases of open neural tube defect (ONTD).

Methods: Retrospective study of patients referred for prenatal ONTD repair between 2012 and 2018. Cases were classified into three groups based on their MSAFP level: 1)High MSAFP (>3. Read More