Preliminary evidence suggests that intrauterine myelomeningocele repair may benefit patients by reducing the both incidence of hydrocephalus and the severity of the Chiari malformation; however, this benefit remains unproved. Furthermore, the procedure entails substantial risks not associated with conventional therapy. A randomized controlled trial of intrauterine and conventional therapies is underway. This study should definitively establish the procedure related risks and benefits. Regardless of the outcome, it is clear that the risks of intrauterine intervention need to be reduced before myelomeningocele, or other congenital malformations, can be effectively treated prior to birth. To that end, studies are being conducted to assess the potential advantages of applying state-of-the-art endoscopic techniques to intrauterine therapy. If benefit can be proven and risks reduced, intrauterine myelomeningocele repair has the potential to become the preferred therapy for patients suffering from this debilitating disease.
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http://dx.doi.org/10.3171/foc.2004.16.2.3 | DOI Listing |
A A Pract
December 2024
From the Department of Anesthesiology, Uniformed Services University, Bethesda, Maryland.
Malignant hyperthermia (MH) is a potentially fatal disorder triggered by volatile anesthetics or succinylcholine, inducing a hypermetabolic crisis in susceptible patients. The caffeine-halothane contracture test (CHCT) remains a gold standard for MH detection. The authors describe a pregnant patient with a history of exertional rhabdomyolysis, who required urgent MH screening for administration of MH-triggering anesthetics.
View Article and Find Full Text PDFJ Neurosurg Pediatr
January 2025
1Department of Obstetrics and Gynecology, Baylor College of Medicine and Texas Children's Hospital Pavilion for Women, Houston, Texas.
Prenat Diagn
November 2024
Department of Fetal Medicine, Armand Trousseau Hospital, AP-HP, DMU ORIGYNE, National Reference Center for Rare Disease: Spin@, Sorbonne University, Paris, France.
Myelomeningocele is a birth defect whose clinical manifestations are due both to incomplete neural tube closure and the progressive destruction of exposed neuroepithelium during pregnancy. Two hypotheses have been formulated to explain the spinal cord damage in utero: mechanical trauma and chemical factors. The objective of this review was to summarize the current insights about the potential role of amniotic fluid in spinal cord damage in myelomeningocele.
View Article and Find Full Text PDFJ Clin Med
August 2024
Department of Obstetrics, University Hospital Zurich, Rämistrasse 100, 8091 Zurich, Switzerland.
Rev Assoc Med Bras (1992)
June 2024
Universidade Federal de São Paulo, Escola Paulista de Medicina, Department of Obstetrics - São Paulo (SP), Brazil.
Objective: The aim of this study was to understand the dynamics of families with children with myelomeningocele undergoing intrauterine fetal surgery.
Methods: A retrospective cohort pilot study was carried out with 11 mothers of children who had undergone intrauterine myelomeningocele repair. Participants in this study responded to an electronic questionnaire (via Google Forms), developed by the study authors, that consisted of 22 multiple-choice questions, of which 17 were closed-ended and 5 had a standardized format.
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