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Journal of Pediatric Rehabilitation... 2022This study aimed to describe health care use by type of health providers and care settings visited by children with spina bifida (SB) and to compare this use between...
PURPOSE
This study aimed to describe health care use by type of health providers and care settings visited by children with spina bifida (SB) and to compare this use between children with and without a shunt.
METHODS
Health care use data were extracted from a larger study on the health and functioning of children with SB aged 3-6 years. The present study focused on the medical information subsection of a parent-reported survey related to SB care, general care, specialty care (e.g., neurosurgery), emergency care, and complications related to SB and shunts.
RESULTS
Parents of 101 children with SB participated. Most of the children were male with myelomeningocele and had a shunt. They visited a health care provider for SB care an average of 7.4 times and a specialist an average of 11.9 times in the previous 12 months. Most visited a multidisciplinary clinic for SB-related care and a private physician's office for general care. Children with a shunt had more SB-related medical visits, more visits to a specialist, and a greater number of different types of specialists than those without it. Frequency of emergency room visits did not differ between the two groups. Health providers informed parents about headaches, vomiting, and fever as signs of complications, and some parents did report shunt-related complications.
CONCLUSION
SB is a complex medical condition requiring that children receive medical care from various medical specialists, especially for children with a shunt. Findings on health care use suggest high levels of monitoring and care coordination that parents navigate to care for their child.
Topics: Child; Humans; Male; Child, Preschool; Female; Spinal Dysraphism; Meningomyelocele; Parents; Surveys and Questionnaires; Delivery of Health Care
PubMed: 36530103
DOI: 10.3233/PRM-220027 -
Early Human Development Nov 2013Until about forty years ago, the womb shielded the fetus from observation and therapy. The rapid changes in the diagnosis and treatment of human fetal anatomical... (Review)
Review
Until about forty years ago, the womb shielded the fetus from observation and therapy. The rapid changes in the diagnosis and treatment of human fetal anatomical abnormalities are due to improved fetal imaging studies as well as fetal sampling techniques (e.g. amniocentesis, chorionic villus sampling), and a better understanding of fetal pathophysiology derived from laboratory animals. Fetal therapy is the logical culmination of progress in fetal diagnosis. In other words, the fetus is now a patient. The fetal surgical treatment of the most severe form of spina bifida - myelomeningocele (MMC) - will be used as a paradigm to illustrate progress in and future prospects for fetal surgery. This review will focus on the rationale for in utero repair in the context of pathologic observations and animal models of MMC, outcomes from human fetal MMC repair including the recently completed Management of Myelomeningocele Study (MOMS trial), and future research directions.
Topics: Animals; Disease Models, Animal; Female; Fetal Therapies; Fetus; Humans; Meningomyelocele; Pregnancy
PubMed: 24094456
DOI: 10.1016/j.earlhumdev.2013.09.010 -
Pediatric Nephrology (Berlin, Germany) Aug 2008The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50%... (Review)
Review
The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate with age, especially in those with thoracic lesions. Those who walk in childhood have a 20-50% chance of becoming wheelchair dependent as adults. Immobility, poor respiratory reserve, obesity, latex allergy and worsening kyphoscoliosis contribute to the increased risks of surgery. It is essential that safe and manageable urine drainage is established in childhood: the bladder never improves with time, and surgical reconstruction becomes progressively more difficult. Independence in adult life will only be possible with intense preparation in childhood. Children must be allowed to join in with family chores and events. Education, both academic and practical, must be encouraged. Skills such as driving, shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocephalus, is limited (but not absent). However, by adult life, about two thirds will have established a regular partnership. All females and those males who are naturally potent are likely to be fertile. There is a high risk of neural tube defects in their offspring unless the female partner takes prophylactic folic acid for 3 months before pregnancy and for first trimester.
Topics: Adaptation, Psychological; Child; Education, Medical, Continuing; Humans; Meningomyelocele; Quality of Life; Treatment Outcome; Urinary Bladder, Neurogenic
PubMed: 18200450
DOI: 10.1007/s00467-007-0663-3 -
Clinics in Perinatology Jun 2012Myelomeningocele (MMC) is a congenital neural tube defect that occurs in approximately 1 in 2900 live births in the United States. It is a devastating disability with... (Review)
Review
Myelomeningocele (MMC) is a congenital neural tube defect that occurs in approximately 1 in 2900 live births in the United States. It is a devastating disability with significant morbidity and mortality within the first few decades of life. MMC was the first nonlethal disease to be considered and studied for fetal surgery and is now the most common open fetal surgery performed. The recently completed MOMS randomized controlled trial has shown that fetal repair for MMC can improve hydrocephalus and hindbrain herniation, can reduce the need for vetriculoperitoneal shunting, and may improve distal neurologic function in some patients.
Topics: Female; Fetal Diseases; Fetoscopy; Fetus; Humans; Hydrocephalus; Meningomyelocele; Pregnancy; Pregnancy Complications; Treatment Outcome; Ventriculoperitoneal Shunt
PubMed: 22682379
DOI: 10.1016/j.clp.2012.04.003 -
Journal of Orthopaedic Surgery and... Aug 2021Myelomeningocele (MMC) is the most common and severe form of spina bifida and imposes a significant burden on patients and the healthcare system. Recently, the... (Review)
Review
BACKGROUND
Myelomeningocele (MMC) is the most common and severe form of spina bifida and imposes a significant burden on patients and the healthcare system. Recently, the multidisciplinary management of MMC has become popular. Herein, we aimed to review the orthopedic management, outcomes, and complications of the of patients with MMC eyeing a multidisciplinary approach.
METHODS
We searched PubMed and EMBASE to find relevant studies published before August 2020. All studies that included clinical management of MMC patients and published earlier than 2000 were considered for review on the condition that they reported at least one orthopedic intervention and the rate of complications. We excluded review articles, case reports, case series, letters, commentaries, editorials, and conference abstracts. The primary and secondary goals of our review were to report the outcomes and complication rates of multidisciplinary management for MMC patients.
RESULTS
Twenty-six studies included data for the management of 229,791 patients with MMC and were selected. Sixteen studies reported multidisciplinary management in addition to orthopedic management. From those, 11 (42.31%) included urologic management, 13 (50%) neurosurgical management, 11 (42.31%) neurologic management, and 5 (19.23%) gastrointestinal management. All studies included postnatal operations and related management. No randomized clinical trial was found in our search.
CONCLUSION
Orthopedic approaches play a key role in MMC management by alleviating spinal deformities, particularly scoliosis, and hip, foot, and ankle complications. However, the most appropriate management, whether surgical or non-surgical, may vary for different patients, given disease severity and the age of patients.
Topics: Humans; Meningomyelocele; Orthopedics; Scoliosis
PubMed: 34389028
DOI: 10.1186/s13018-021-02643-8 -
The Western Journal of Medicine Feb 1975
Topics: Female; Genetic Counseling; Humans; Infant, Newborn; Meningomyelocele; Pregnancy; Prenatal Diagnosis; Spinal Dysraphism
PubMed: 1090092
DOI: No ID Found -
Proceedings of the Royal Society of... Jun 1963
Topics: Humans; Hydrocephalus; Meningomyelocele; Postoperative Care; Spinal Dysraphism; Urinary Incontinence
PubMed: 13937597
DOI: No ID Found -
Developmental Disabilities Research... 2010A cognitive phenotype is a product of both assets and deficits that specifies what individuals with spina bifida meningomyelocele (SBM) can and cannot do and why they... (Review)
Review
A cognitive phenotype is a product of both assets and deficits that specifies what individuals with spina bifida meningomyelocele (SBM) can and cannot do and why they can or cannot do it. In this article, we review the cognitive phenotype of SBM and describe the processing assets and deficits that cut within and across content domains, sensory modality, and material, including studies from our laboratory and other investigations. We discuss some implications of the SBM cognitive phenotype for assessment, rehabilitation, and research.
Topics: Achievement; Attention; Child; Cognition Disorders; Cues; Educational Status; Humans; Language Disorders; Mathematics; Meningomyelocele; Neuropsychological Tests; Phenotype; Severity of Illness Index; Space Perception; Spinal Dysraphism; Time Factors
PubMed: 20419769
DOI: 10.1002/ddrr.89 -
Journal of Orthopaedics and... Sep 2010In patients with myelomeningocele (MMC), a high number of fractures occur in the paralyzed extremities, affecting mobility and independence. The aims of this...
BACKGROUND
In patients with myelomeningocele (MMC), a high number of fractures occur in the paralyzed extremities, affecting mobility and independence. The aims of this retrospective cross-sectional study are to determine the frequency of fractures in our patient cohort and to identify trends and risk factors relevant for such fractures.
MATERIALS AND METHODS
Between March 1988 and June 2005, 862 patients with MMC were treated at our hospital. The medical records, surgery reports, and X-rays from these patients were evaluated.
RESULTS
During the study period, 11% of the patients (n = 92) suffered one or more fractures. Risk analysis showed that patients with MMC and thoracic-level paralysis had a sixfold higher risk of fracture compared with those with sacral-level paralysis. Femoral-neck z-scores measured by dual-energy X-ray absorptiometry (DEXA) differed significantly according to the level of neurological impairment, with lower z-scores in children with a higher level of lesion. Furthermore, the rate of epiphyseal separation increased noticeably after cast immobilization. Mainly patients who could walk relatively well were affected.
CONCLUSIONS
Patients with thoracic-level paralysis represent a group with high fracture risk. According to these results, fracture and epiphyseal injury in patients with MMC should be treated by plaster immobilization. The duration of immobilization should be kept to a minimum (<4 weeks) because of increased risk of secondary fractures. Alternatively, patients with refractures can be treated by surgery, when nonoperative treatment has failed.
Topics: Adolescent; Age Distribution; Analysis of Variance; Bone Plates; Casts, Surgical; Chi-Square Distribution; Child; Child, Preschool; Cross-Sectional Studies; Female; Femoral Neck Fractures; Follow-Up Studies; Fracture Fixation, Internal; Fracture Healing; Humans; Immobilization; Incidence; Injury Severity Score; Logistic Models; Lower Extremity; Male; Meningomyelocele; Radiography; Retrospective Studies; Risk Assessment; Sex Distribution; Tibial Fractures; Time Factors; Treatment Outcome
PubMed: 20721596
DOI: 10.1007/s10195-010-0102-2 -
PloS One 2012The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic... (Review)
Review
BACKGROUND
The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic review on urological outcome in adult SB patients is available and no follow-up schemes exist.
OBJECTIVES
To systematically summarize the evidence on outcome of urinary tract functioning in adult SB patients.
METHODS
A literature search in PubMed and Embase databases was done. Only papers published in the last 25 years describing patients with open SB with a mean age >18 years were included. We focused on finding differences in the treatment strategies, e.g., clean intermittent catheterization and antimuscarinic drugs versus early urinary diversion, with regard to long-term renal and bladder outcomes.
RESULTS
A total of 13 articles and 5 meeting abstracts on urinary tract status of adult SB patients were found describing a total of 1564 patients with a mean age of 26.1 years (range 3-74 years, with a few patients <18 years). All were retrospective cohort studies with relatively small and heterogeneous samples with inconsistent reporting of outcome; this precluded the pooling of data and meta-analysis. Total continence was achieved in 449/1192 (37.7%; range 8-85%) patients. Neurological level of the lesion and hydrocephalus were associated with incontinence. Renal function was studied in 1128 adult patients. In 290/1128 (25.7%; range 3-81.8%) patients some degree of renal damage was found and end-stage renal disease was seen in 12/958 (1.3%) patients. Detrusor-sphincter dyssynergy and detrusor-overactivity acted as adverse prognostic factors for the development of renal damage.
CONCLUSIONS
These findings should outline follow-up schedules for SB patients, which do not yet exist. Since renal and bladder deterioration continues beyond adolescence, follow-up of these individuals is needed. We recommend standardization in reporting the outcome of urinary tract function in adult SB patients.
Topics: Adult; Humans; Meningomyelocele; Prognosis; Risk Factors; Urinary Tract
PubMed: 23119003
DOI: 10.1371/journal.pone.0048399