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The Journal of Urology Aug 2011
Topics: Child; Humans; Meningomyelocele; Urodynamics
PubMed: 21683414
DOI: 10.1016/j.juro.2011.03.177 -
Child's Nervous System : ChNS :... Sep 2013The prevention of neural tube defects (NTDs) is now an issue of major public health concern. Myelomeningocele, the most common NTD, often results in severe disabilities... (Review)
Review
BACKGROUND
The prevention of neural tube defects (NTDs) is now an issue of major public health concern. Myelomeningocele, the most common NTD, often results in severe disabilities and may be life threatening. In Africa, there is little awareness of the NTDs, and most African countries lag behind in the global efforts at preventing them.
PURPOSE
Following a review of global literature, we discuss the burden of myelomeningocele on the society and the current state of its prevention with an emphasis on Africa within the global context. The realization that folate supplementation reduces the occurrence of NTDs by as much as 85 % offers an opportunity to promote global reduction in the incidence of NTDs through (1) effective folate supplementation strategies combined with (2) secondary prevention using prenatal detection and termination of NTD pregnancies within respective national legal frameworks. We call attention to the poor state of understanding of NTDs in Africa and the alarming rarity of policies to prevent the condition in the continent.
CONCLUSIONS
The understanding of the contributions of folate deficiency to the causation of NTDs has enabled appropriate, though still inadequate, preventive measures to be taken in several countries. We call on African governments and the international community to rapidly promote policies aimed at making fortification of wheat (and or other substitute staple foods) with folic acid universally available.
Topics: Africa; Cost of Illness; Folic Acid; Folic Acid Deficiency; Humans; Meningomyelocele
PubMed: 24013323
DOI: 10.1007/s00381-013-2126-4 -
Seminars in Perinatology Dec 1999Myelomeningocele is a common birth defect that is associated with significant lifelong morbidity. Despite improvements in technology and overall patient care, little... (Review)
Review
Myelomeningocele is a common birth defect that is associated with significant lifelong morbidity. Despite improvements in technology and overall patient care, little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord and preventing infection. Numerous interventions for ventricular shunts, tethered cord, scoliosis, incontinence, urologic complications, and extremity anomalies are frequently required. Although myelomeningocele is a nonlethal fetal anomaly, the limitations with current postnatal treatment strategies has led to extensive investigation of prenatal treatment options. This article outlines the rationale for fetal intervention and discusses the preliminary experience with human fetal myelomeningocele surgery.
Topics: Female; Fetal Diseases; Gestational Age; Humans; Meningomyelocele; Pregnancy; Surgical Procedures, Operative; Treatment Outcome
PubMed: 10630542
DOI: 10.1016/s0146-0005(99)80025-1 -
Przeglad Lekarski 1998Neural tube defects are the most common anomalies of the CNS in the group of dysraphic syndromes. They occur on both encephalic and spinal levels. According to the title... (Review)
Review
Neural tube defects are the most common anomalies of the CNS in the group of dysraphic syndromes. They occur on both encephalic and spinal levels. According to the title of this article we discuss some characteristic examples of intracranial, encephalic changes occurring in cases of meningomyeloceles. The special position has to be attributed to Arnold-Chiari or Chiari malformation, being currently under a nosological discussion. It is often restricted to its most typical form-Chiari II syndrome. This is characterized by the neural tube defect and the posterior fossa and brain-stem-cerebellum malformation with a displacement into the foramen magnum. The compression of the fourth ventricle and even secondary necrotic changes are also seen. Clinical symptoms depend on the topography of pathological changes. Chiari II malformation coincides in the majority of cases with meningomyeloceles and sometime with other spinal cord malformations. Meningomyeloceles coincide often with anomalies within cerebral hemispheres. Among them hydrocephalus deserves particular attention, occurring in the majority of Chiari II cases and not being rare in cases of myeloceles. The anomalies and/or secondary lesions of encephalic structures, particularly of the cortical mantle are observed, depending upon the severity of hydrocephalus and the onset of its development. Meningomyeloceles can also be accompanied by cerebral hemispheres anomalies arising in the other pathomechanism and developmental period than dysraphic syndromes. The midline malformations and anomalies of commissural system are not rare among them. In conclusion we emphasize that each case of CNS malformations is unique. The observed anomalies have to be assessed whether they form a part of a leading syndrome or occur together with dysraphic changes.
Topics: Abnormalities, Multiple; Brain; Brain Diseases; Humans; Hydrocephalus; Meningomyelocele; Spinal Dysraphism
PubMed: 9656737
DOI: No ID Found -
American Journal of Medical Genetics Aug 1993
Topics: Amniocentesis; Female; Fetal Death; Fetal Diseases; Humans; Male; Meningomyelocele; Pregnancy; Ultrasonography, Prenatal; Waardenburg Syndrome
PubMed: 8368244
DOI: 10.1002/ajmg.1320470130 -
Child's Nervous System : ChNS :... Apr 2020Fetal repair of myelomeningocele has been increasingly offered to mothers of children with myelomeningocele after the seminal Management of Myelomeningocele (MOMs)...
Fetal repair of myelomeningocele has been increasingly offered to mothers of children with myelomeningocele after the seminal Management of Myelomeningocele (MOMs) trial, which demonstrated decreased reliance on ventriculoperitoneal shunt following fetal closure. We present the case of a fetus diagnosed with a lumbar myelomeningocele in utero whose mother refused in utero closure and who was subsequently born with a skin-covered defect. A fetal MRI was obtained on a mother with a male fetus diagnosed with open neural tube defect at 20 weeks of gestation. The child demonstrated spinal dysraphism extending from L2 to L5 and associated Chiari II malformation with lateral and third ventriculomegaly. Based on our institutional criteria and the criteria of the MOMs trial, the parents were offered fetal repair of the myelomeningocele; however, they declined because of concerns about risks to the mother. At birth, the patient was found to have a skin-covered meningocele. He underwent elective repair of his occult meningocele and detethering of his spinal cord. Intraoperative findings demonstrated spinal nerve roots attached to the arachnoid within the defect, and a closed, tubularized neural placode. This represents a unique case in which a fetus with a clinical picture consistent with open spinal defect was found to have a lesion more consistent with meningocele on postnatal operative interrogation. Knowledge that this can occur should be taken into consideration when discussing fetal closure, although the frequency of this occurrence is not known. Additionally, identification of this case sheds light on the mechanism by which occult myelomeningoceles form.
Topics: Child; Female; Fetus; Humans; Hydrocephalus; Infant, Newborn; Male; Meningomyelocele; Spinal Dysraphism; Ventriculoperitoneal Shunt
PubMed: 32025870
DOI: 10.1007/s00381-019-04454-z -
Neurosurgery Jan 1985Terminal myelocystoceles constitute approximately 5% of skin-covered lumbosacral masses and are especially common in patients with cloacal exstrophy. Pathologically,...
Terminal myelocystoceles constitute approximately 5% of skin-covered lumbosacral masses and are especially common in patients with cloacal exstrophy. Pathologically, terminal myelocystocele consists of (a) a skin-covered lumbosacral spina bifida (b) an arachnoid-lined meningocele that is directly continuous with the spinal subarachnoid space; and (c) a low-lying, hydromyelic spinal cord that traverses the meningocele and then expands into a large terminal cyst. The terminal cyst bulges into the extraarachnoid compartment caudal to the meningocele and forms a distal sac that does not communicate with the subarachnoid space. The terminal cyst is lined by ependyma and dysplastic glia, is directly continuous with the dilated central canal of the cord, and probably represents a ballooned terminal ventricle. Patients with terminal myelocystocele have normal intellectual potential and are usually born without neurological deficit, so these defects must be identified and repaired early, before the onset or progression of lower extremity pareses.
Topics: Female; Humans; Infant, Newborn; Meningomyelocele; Myelography; Spinal Cord; Tomography, X-Ray Computed; Ultrasonography
PubMed: 3883218
DOI: No ID Found -
Journal of Neurosurgery. Pediatrics Jul 2017OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in... (Review)
Review
OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.
Topics: Comorbidity; Humans; Meningomyelocele; Scoliosis
PubMed: 28452655
DOI: 10.3171/2017.2.PEDS16641 -
Lancet (London, England)
Comparative Study
Topics: Humans; Infant, Newborn; Meningomyelocele
PubMed: 2867411
DOI: 10.1016/s0140-6736(85)92582-6 -
The Western Journal of Medicine Oct 1974
Topics: Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Meningomyelocele; Pregnancy; Spinal Dysraphism
PubMed: 4608838
DOI: No ID Found