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Int Urol Nephrol 2022 May 24. Epub 2022 May 24.

Department of Urology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Tzu Chi University, 707, Section 3, Chung-Yang Road, Hualien, Taiwan.

Purpose: Neurogenic lower urinary tract dysfunction (NLUTD) is common among children with myelomeningocele (MMC). If NLUTD is not appropriately managed, recurrent urinary tract infection (UTI) can persist and may affect upper urinary tract function. This study investigated the usefulness of videourodynamic study (VUDS) in the urological management of MMC. Read More

Childs Nerv Syst 2022 May 19. Epub 2022 May 19.

Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA.

Objective: Chiari I malformation is treated with suboccipital craniectomy with cervical laminectomy, a procedure which has been associated with the possibility of pre-existing or iatrogenic occipitocervical instability. The long-term risk of subsequent spinal deformity and need for occipito cervical fusion after standard Chiari decompression in pediatric patients has not yet been characterized.

Methods: We queried our institutional electronic database for patients aged 18 and under, with at least 5 years of follow-up, that underwent surgical decompression for Chiari I malformation. Read More

Pediatr Surg Int 2022 May 14. Epub 2022 May 14.

Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, 302004, India.

Purpose: Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described.

Methods: Six cases of HT managed at two tertiary care teaching hospitals over the last 26 years are described. Read More

Acta Paediatr 2022 May 14. Epub 2022 May 14.

Lund University, Skåne University Hospital, Department of Clinical Sciences Lund, Orthopedics, Lund, Sweden.

Aim: To investigate the occurrence of pressure injuries (PIs) in children with myelomeningocele (MMC) and to investigate the association between PIs and orthoses use by disability-specific variables.

Methods: Population-based registry study including participants in the Swedish multidisciplinary follow-up program for MMC. Risks of PIs were investigated by birth cohort, country of birth, sex, type of MMC, muscle function level (MFL), and continence status. Read More

J Am Acad Orthop Surg Glob Res Rev 2022 May 1;6(5). Epub 2022 May 1.

From the Department of Orthopaedic Surgery, Mayagüez Medical Center, Mayagüez, PR (Dr. Ramirez); the Department of Orthopaedic Surgery, University of Puerto Rico, San Juan, PR (Olivella, and Dr. Torres-Lugo); the Department of Orthopaedic Surgery, Riley Children's Health, Indianapolis, IN (Dr. Fitzgerald); the Department of Orthopedics, Primary Children's Medical Center, Salt Lake City, UT (Dr. Smith); the Division of Orthopaedic Surgery, Cincinnati Children's Hospital, Cincinnati, OH (Dr. Sturm); the Department of Orthopaedic Surgery, Johns Hopkins University, Baltimore, MD (Dr. Sponseller); the Department of Orthopaedic Surgery, Boston Children's Hospital, Boston, MA (Dr. Karlin); the Department of Orthopaedic Surgery, Washington University School of Medicine, St. Louis, MO (Dr. Luhmann); and the Pediatric Spine Foundation, Valley Forge, PA (Dr. St. Hilaire).

Introduction: Early-onset scoliosis (EOS) is a well-known orthopaedic manifestation in patients with myelomeningocele. The rib-based growing system (RBGS) has been proposed as an alternative for these individuals because of the poor outcomes with traditional surgical techniques. We aimed to describe the effect of RBGS in patients with nonambulatory EOS myelomeningocele. Read More

Acta Neurol Belg 2022 May 10. Epub 2022 May 10.

Department of Neurosurgery, Tianjin Medical University General Hospital, Tianjin, 300000, China.

Fetal Pediatr Pathol 2022 May 10:1-7. Epub 2022 May 10.

Patología Quirúrgica y Citopatológica del Centro, Tuxtla Gutiérrez, Chiapas, Mexico.

Lipofibromatosis is a relatively new entity, considered in the differential diagnosis of soft tissue tumors in children, involving mainly the extremities. Most cases can be completely resected without recurrence. Atypical forms have been described and their highly infiltrative capability can lead to severe clinical impairment. Read More

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

Ann Plast Surg 2022 Apr 2. Epub 2022 Apr 2.

Department of Plastic and Reconstructive Surgery, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Republic of Korea.

Introduction: Myelomeningocele is the most common form of neural tube anomalies. Early reliable skin coverage should be achieved to reduce central nervous system infections. The keystone flaps are gaining popularity for myelomeningocele defect reconstruction. Read More

Acta Ortop Mex 2021 Sep-Oct;35(5):399-404

Ortopedia y Traumatología. Hospital Italiano de Buenos Aires, Argentina.

Objective: The aim of the study was to evaluate the outcomes and complications in patients with myelomeningocele who have undergone spinal fusion for neuromuscular scoliosis.

Material And Methods: Retrospective study of 40 consecutive patients with myelomeningocele with neuromuscular scoliosis who underwent spinal arthrodesis, treated at our center between July 1991 and July 2028.

Results: Mean follow up was 10 years. Read More

Birth Defects Res 2022 Apr 19. Epub 2022 Apr 19.

Department of Medical Genetics, Osaka Women's and Children's Hospital, Osaka, Japan.

Background: Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period.

Case: A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Read More

J Pediatr Urol 2022 Mar 5. Epub 2022 Mar 5.

Division of Urology, Department of Surgery, Duke University School of Medicine, Durham, NC, USA. Electronic address:

Introduction: Spina bifida is the most common permanently disabling birth defect in the United States and requires lifelong, multi-specialty care. The cost of such care has the potential to result in financial toxicity - the 'objective financial burden' and 'subjective financial distress' which can negatively impact clinical outcomes. While this concept has been extensively studied in other areas of medicine, particularly oncology, financial toxicity has not yet been examined in pediatric urology or in individuals with spina bifida and their families/caregivers. Read More

Surg Neurol Int 2022 4;13:75. Epub 2022 Mar 4.

Department of Neurosurgery, Health Sciences Center, Federal University of Espírito Santo, Brazil.

Background: The most commonly used technique in the management of hydrocephalus is a neurosurgical procedure, known as ventriculoperitoneal shunt (VPS). Several complications of the distal end of a VPS catheter have been described in the literature, although migration and erosive bladder perforation of this shunt are extremely uncommon.

Case Description: We relate a rare pediatric case of a 4-year-old boy, with long-term dysuria, intermittent incontinence, and chronic abdominal pain developed during a myelomeningocele postoperative period, without other symptoms. Read More

J Perinatol 2022 Apr 7. Epub 2022 Apr 7.

Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

In our response to, "Parental request for non-resuscitation in fetal myelomeningocele repair: an analysis of the novel ethical tensions in fetal intervention" by Wolfe and co-authors, we argue that parental authority should guide resuscitation decision-making for a fetus at risk for preterm delivery as a complication of fetal myelomeningocele (fMMC) repair. Due to the elevated morbidity and mortality risks of combined myelomeningocele, extreme prematurity, and fetal hypoxia, parents' values regarding the acceptability of possible outcomes should be elicited and their preferences honored. Ethical decision-making in these situations must also consider the broader context of the fetal-maternal dyad. Read More

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

Int Braz J Urol 2022 Jul-Aug;48(4):672-678

Departamento de Urologia, CACAU-NUPEP, São Paulo, Brasil.

Objectives: High-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup.

Patients And Methods: From our prospective cohort of in utero MMC we have identified patients in the high-risk group. Read More

Ultrasound Obstet Gynecol 2022 Mar 30. Epub 2022 Mar 30.

My FetUZ Fetal Research Center, Department of Development and Regeneration, Biomedical Sciences, KU Leuven, Leuven, Belgium.

Objectives: the MOMS randomized trial has demonstrated that prenatal spina bifida aperta (SBA) repair via open approach is safe and effective for both mother and fetus, yet half of the infants have incomplete reversal of hindbrain herniation and no improvement in neuromotor function. One contributing factor may be the incompleteness of the neurosurgical repair causing persistent in utero leakage of cerebrospinal fluid (CSF) and exposure of the spinal cord to the amniotic fluid until birth. We aim to investigate the neurostructural and neurofunctional efficacy of the watertightness of prenatal SBA repair. Read More

BMJ Case Rep 2022 Mar 29;15(3). Epub 2022 Mar 29.

Radiology, Mahatma Gandhi Mission's Institute of Health Sciences, Navi Mumbai, Maharashtra, India.

Diastematomyelia is the asymmetric or symmetric lateral duplication of the spinal cord into two hemicords. Pang divided it into three types: type-1, type-2 and composite split cord malformation (SCM). Composite SCMs are uncommon and are defined by the coexistence of multiple types of split cord with normal cord in between. Read More

J Pediatr Urol 2022 Feb 19. 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

Sci Rep 2022 03 10;12(1):3950. Epub 2022 Mar 10.

Department of Biomedical Engineering, University of Cincinnati, Cincinnati, OH, USA.

Open spina bifida or myelomeningocele (MMC) is a devastating neurologic congenital defect characterized by primary failure of neural tube closure of the spinal column during the embryologic period. Cerebrospinal fluid leak caused by the MMC spinal defect in the developing fetus can result in a constellation of encephalic anomalies that include hindbrain herniation and hydrocephalus. The exposure of extruded spinal cord to amniotic fluid also poses a significant risk for inducing partial or complete paralysis of the body parts beneath the spinal aperture by progressive spinal cord damage in-utero. Read More

Fetal Diagn Ther 2022 10;49(3):125-137. Epub 2022 Mar 10.

National Clinician Scholars Program, Yale School of Medicine and Yale School of Nursing, New Haven, Connecticut, USA.

Background/purpose: Although maternal-fetal surgery to treat fetal anomalies such as spina bifida continues to grow more common, potential health disparities in the field remain relatively unexamined. To address this gap, we identified maternal-fetal surgery studies with the highest level of evidence and analyzed the reporting of participant sociodemographic characteristics and representation of racial and ethnic groups.

Methods: We conducted a systematic review of the scientific literature using biomedical databases. Read More

SICOT J 2022 7;8. Epub 2022 Mar 7.

Department of Orthopedic Surgery, Faculty of Medicine, Ain Shams University, 11591 Cairo, Egypt.

Study Design: Prospective case series.

Purpose: To describe a new technique for anterior column reconstruction after kyphectomy in myelomeningocele patients using titanium mesh cage and to evaluate outcomes and complications.

Methods: Sixteen patients with severe dorsolumbar kyphosis 2 to myelomeningocele were enrolled with a mean age of 10. Read More

Cureus 2022 Jan 29;14(1):e21718. Epub 2022 Jan 29.

Department of Orthopaedics, Himalayan Institute of Medical Science, Dehradun, IND.

Neuropathic arthropathy is the painless destruction of weight-bearing bones and joints that is caused by a neurosensory deficit. Minimally displaced neuropathic fractures may be managed non-operatively. However, larger displacements often require surgical fixation. Read More

J Neurosurg Pediatr 2022 Mar 4:1-10. Epub 2022 Mar 4.

3Division of Pediatric Neurosurgery, Oklahoma Children's Hospital, Department of Neurosurgery, The University of Oklahoma College of Medicine, Oklahoma City, Oklahoma.

Objective: Tethered cord release (TCR) is the gold standard treatment for tethered cord syndrome (TCS); however, there are significant shortcomings including high rates of retethering, especially in complex and recurrent cases. Spinal column shortening (SCS) is an alternative treatment for TCS intended to avoid these shortcomings. Early studies were limited to case reports and smaller case series; however, in recent years, larger case series and small cohort studies have been conducted. Read More

J Neurosurg Pediatr 2022 Mar 4:1-7. Epub 2022 Mar 4.

2Department of Neurosurgery, University of North Carolina School of Medicine, Chapel Hill, North Carolina.

Objective: Relatively few women undergo open maternal-fetal surgery (OMFS) for myelomeningocele (MMC) despite the potential to reverse hindbrain herniation, reduce the rate of infant shunt-dependent hydrocephalus, and improve ambulation. These benefits have the potential to significantly reduce morbidity and lifetime medical care. In this study, the authors examined demographics and socioeconomic variables of women who were offered and opted for OMFS for MMC versus postnatal MMC surgery, with the purpose of identifying variables driving the disparity between these two patient populations. Read More

Pan Afr Med J 2021 21;40:248. Epub 2021 Dec 21.

School of Medical Laboratory Sciences, Institute of Health, Jimma University, Jimma, Ethiopia.

Introduction: prenatal development could be considered normal or abnormal. Abnormal development occurs because of interference of normal development from genetic disorders, environmental factors, and multifactorial inheritances during the critical period of embryogenesis. The present study was aimed at evaluating the prevalence and patterns of birth defects among newborns in southwestern Ethiopia. Read More

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

Clin Perinatol 2022 03 21;49(1):267-277. Epub 2022 Jan 21.

Division of Pediatric Surgery, Children's Wisconsin, 999 North 92nd Street, Suite C320, Milwaukee, WI 53226, USA. Electronic address:

Fetal surgery is a constantly evolving field that showed noticeable progress with the treatment of myelomeningocele (MMC) using prenatal repair. Despite this success, there are ongoing questions regarding the optimal approach for fetal myelomeningocele repair, as well as which patients are eligible. Expansion of the inclusion and exclusion criteria is an important ongoing area of study for myelomeningocele including the recent Management of Myelomeningocele Plus trial. Read More

Spec Care Dentist 2022 Feb 24. Epub 2022 Feb 24.

Department of Pedodontics and Preventive Dentistry, M.A. Rangoonwala College of Dental Sciences and Research Centre, Pune, Maharashtra, India.

Background: Myelomeningocele (MMC) is a severe manifestation of spina bifida. Children with MMC have motor disability, hydrocephalus, skeletal abnormalities, and mental retardation. These individuals are more susceptible to caries due to poor oral hygiene, carbohydrate-rich diet, prolonged use of sugar-containing medications and limited motor abilities. Read More

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