Neural Tube Anomalies: Clinical Management by Open Fetal Surgery

Fetal Therapy ◽  
2019 ◽  
pp. 456-466
Author(s):  
N. Scott Adzick ◽  
Julie S. Moldenhauer ◽  
Gregory G. Heuer
2019 ◽  
Vol 28 (3) ◽  
pp. 476-487 ◽  
Author(s):  
JULIA A.E. RADIC ◽  
JUDY ILLES ◽  
PATRICK J. MCDONALD

Abstract:Open neural tube defects or myelomeningoceles are a common congenital condition caused by failure of closure of the neural tube early in gestation, leading to a number of neurologic sequelae including paralysis, hindbrain herniation, hydrocephalus and neurogenic bowel and bladder dysfunction. Traditionally, the condition was treated by closure of the defect postnatally but a recently completed randomized controlled trial of prenatal versus postnatal closure demonstrated improved neurologic outcomes in the prenatal closure group. Fetal surgery, or more precisely maternal-fetal surgery, raises a number of ethical issues that we address including who the patient is, informed consent, surgical innovation and equipoise as well maternal assumption of risk. As the procedure becomes more widely adopted into practice, we suggest close monitoring of new fetal surgery centers, in order to ensure that the positive results of the trial are maintained without increased risk to both the mother and fetus.


2016 ◽  
Vol 214 (1) ◽  
pp. S177-S178 ◽  
Author(s):  
Michael Belfort ◽  
William E. Whitehead ◽  
Alireza A. Shamshirsaz ◽  
Sundeep G. Keswani ◽  
Tim Lee ◽  
...  

Author(s):  
Nickie Niforatos ◽  
Adre J du Plessis

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
J. Safi ◽  
L. Joyeux ◽  
G. E. Chalouhi

Nutritional deficiencies are preventable etiological and epigenetic factors causing congenital abnormalities, first cause of infant mortality. Folate deficiency has a well-established teratogenic effect, leading to an increasing risk of neural tube defects. This paper highlights the most recent medical literature about folate deficiency, be it maternal or paternal. It then focuses on associated deficiencies as nutritional deficiencies are multiple and interrelated. Observational and interventional studies have all been consistent with a 50–70% protective effect of adequate women consumption of folates on neural tube defects. Since strategies to modify women’s dietary habits and vitamin use have achieved little progress, scientific as well as political effort is mandatory in order to implement global preventive public health strategies aimed at improving the alimentation of women in reproductive age, especially folic acid supplementation. Even with the recent breakthrough of fetal surgery for myelomeningocele, the emphasis should still be on prevention as the best practice rather than treatment of neural tube defects.


2012 ◽  
Vol 23 (3-4) ◽  
pp. 158-186 ◽  
Author(s):  
MASAYUKI ENDO ◽  
TIM VAN MIEGHEM ◽  
ELISENDA EIXARCH ◽  
PAOLO DE COPPI ◽  
GUNNAR NAULAERS ◽  
...  

The prevalence of neural tube defects (NTD) in Europe is around 9 per 10,000 births making it one of the most frequent congential anomalies affecting the central nervous system. NTD encompass all anomalies that are secondary to failure of closure of the neural tube. In this review, we will first summarize the embryology and some epidemiologic aspects related to NTDs. The review focuses on myelomeningocele (MMC), which is the most common distal closure defect. We will describe the secondary pathologic changes in the central and peripheral nervous system that appear later on in pregnancy and contribute to the condition's morbidity. The postnatal impact of MMC mainly depends on the upper level of the lesion. In Europe, the vast majority of parents with a fetus with prenatally diagnosed NTDs, including MMC, opt for termination of pregnancy, as they are apparently perceived as very debilitating conditions. Animal experiments have shown that prenatal surgery can reverse this sequence. This paved the way for clinical fetal surgery resulting in an apparent improvement in outcome. The results of a recent randomized trial confirmed better outcomes after fetal repair compared to postnatal repair; with follow up for 30 months. This should prompt fetal medicine specialists to reconsider their position towards this condition as well as its prenatal repair. The fetal surgery centre in Leuven did not have a clinical programme for fetal NTD repair until the publication of the MOMS trial. In order to offer this procedure safely and effectively, we allied to a high volume centre willing to share its expertise and assist us in the first procedures. Given the maternal side effects of current open fetal surgical techniques, we have intensified our research programmes to explore minimally invasive alternatives. Below we will describe how we are implementing this.


2021 ◽  
Author(s):  
Hadi Rahimian ◽  
Ramona Ana Maria Rahimian ◽  
Radu Vladareanu

Spina bifida and myelomeningocele, although frequent, present difficulties when it comes to diagnosis and clinical management. The recent developments in ultrasound and MRI technologies and software, allow for an easier and more precise diagnosis. As such, in the first part of our chapter we will present general information, such as etiology, pathophysiology and methods of diagnosis. Fetal surgery, open or fetoscopic, represents a cure in most cases of spina bifida and in other cases reduces the chances of major developmental issues in babies born with this affliction. In the second part of our chapter, we will present the surgical protocols for both procedures, the indications, and the statistics that we have acquired in the cases we have diagnosed and operated on in the Regina Maria Maternity Hospital, Bucharest, the only center in Romania where these procedures are available.


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