Reversal of Hindbrain Herniation after Maternal-fetal Surgery for Myelomeningocele Subsequently Impacts on Brain Stem Function

The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
Neuropediatrics (Impact Factor: 1.1). 12/2009; 39(6):359-62. DOI: 10.1055/s-0029-1202835
Source: PubMed

ABSTRACT The aim of our study was to delineate whether the reversal of hindbrain herniation (HH) following fetal myelomeningocele (fMMC) closure subsequently reduces the incidence and severity of HH-associated brainstem dysfunction (BSD). Prior to the NIH-sponsored Management of Myelomeningocele Study (MOMS) trial, 54 children underwent fMMC closure at our institution. Forty-eight (89%) families participated in a structured survey focusing on HH-associated BSD (e.g., apnea, neurogenic dysphagia [ND], gastro-esophageal reflux disease [GERD], neuro-ophthalmologic disturbances [NOD]). Median age at follow-up was 72 months (range: 46-98). Fifty-percent required shunting. HH-related symptoms were completely absent in 15 (63%) non-shunted and 10 (42%) shunted children (P=0.15). No HH-related death occurred and none developed severe persistent cyanotic apnea. ND was reported in 2 (8%) non-shunted and 9 (38%) shunted infants (P=0.03). Mild GERD (medically managed) developed in 2 (8%) without and 6 (25%) with shunt placement (P=0.24). NOD was found in 6 (25%) and 13 (54%) of non-shunted and shunted children, respectively (P=0.07). The majority of fMMC children developed no or only mild BSD at follow-up. Our data support the hypothesis that neurodevelopmental deficits associated with MMC are at least partially acquired and that reversal of HH following fMMC surgery may help to reduce the incidence and severity of BSD.

1 Follower
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Abstract Objective: The aim of the study was a randomized comparison of the outcomes of intrauterine myelomeningocele repairs (IUMR) in type II Chiari malformation (II CM) fetuses with clinical data of newborns and infants operated on postnatally. Methods: The study group (SG) comprised of 46 pregnant women whose type II CM children underwent IUMR, while 47 pregnant women whose type II CM children were operated on postnatally constituted the control group (CG). A total of 24 SG and 20 CG patients reached the endpoint of the study. Results: High incidence of PROM (24 (52.2%), CI 3.74 (1.69-8.26) (p<0.001) was noted in the group of prenatal surgeries as compared to controls. The need for VP shunt implantation was statistically significantly lower (p<0.008) in the group of children after IUMR as compared to controls (5 (27.8%) and 16 (80%), respectively, CI 0.35 (0.16-0.75). None of the postnatally treated CG children can walk without adaptive equipment. In contrast, two children from the study group (2 (11.1%) CI 1.86 (1.00-3.48) p<0.048) are able to walk independently. Conclusions: Prenatal MMC closure significantly lowers further adverse evolution of the type II Chiari malformation. Further studies are needed, especially on preventive measures for preterm labor and IPPROM in the postoperative course of IUMR.
    The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 10/2013; 27(14). DOI:10.3109/14767058.2013.858689 · 1.21 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Formerly, the disastrous cluster of neurologic deficits and associated neurogenic problems in patients with myelomeningocele (MMC) was generally thought to solely result from the primary malformation, i.e., failure of neurulation. Today, however, there is no doubt that a dimensional additional pathogenic mechanism exists. Most likely, it contributes much more to loss of neurologic function than non-neurulation does. Today, there is a large body of compelling experimental and clinical evidence confirming that the exposed part of the non-neurulated spinal cord is progressively destroyed during gestation, particularly so in the third trimester. These considerations gave rise to the two-hit-pathogenesis of MMC with non-neurulation being the first and consecutive in utero acquired neural tissue destruction being the second hit. This novel pathophysiologic understanding has obviously triggered the question whether the serious and irreversible functional loss caused by the second hit could not be prevented or, at least, significantly alleviated by timely protecting the exposed spinal cord segments, i.e., by early in utero repair of the MMC lesion. Based on this intriguing hypothesis and the above-mentioned data, human fetal surgery for MMC was born in the late nineties of the last century and has made its way to become a novel standard of care, particularly after the so-called “MOMS Trial”. This trial, published in the New England Journal of Medicine, has indisputably shown that overall, open prenatal repair is distinctly better than postnatal care alone. Finally, a number of important other topics deserve being mentioned, including the necessity to work on the up till now immature endoscopic fetal repair technique and the need for concentration of these extremely challenging cases to a small number of really qualified fetal surgery centers worldwide. In conclusion, despite the fact that in utero repair of MMC is not a complete cure and not free of risk for both mother and fetus, current data clearly demonstrate that open fetal–maternal surgery is to be recommended as novel standard of care when pregnancy is to be continued and when respective criteria for the intervention before birth are met. Undoubtedly, it is imperative to inform expecting mothers about the option of prenatal surgery once their fetus is diagnosed with open spina bifida.
    Pediatric Surgery International 07/2014; 30(7). DOI:10.1007/s00383-014-3524-8 · 1.06 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The aims of this study were to describe a new ultrasonographic technique to assess the normal level of the cerebellum and the brainstem in the posterior fossa in normal foetuses and to compare in pathologic cases. We propose a new line cross between the dens cervical and the inferior portion of occipitum (occipitum-dens line-ODL). In a cross-sectional study, a single observer with experience in foetal neurosonography evaluated 54 foetuses (40 normal and 14 with open neural tube defect) between 20 and 28 weeks of gestation. The reference points for the ODL are principally the lower portion of the occipital bone (occipitum) and odontoid process of the second cervical vertebra (dens). The line was considered the level zero (near level of foramen magnum). Structures above it had a positive measurement and below it had a negative measurement. Moreover, in most foetuses with open neural tube defect (93 %), the end portion of cerebellum was below the ODL associated with different degrees of ventriculomegaly. The proposed innovation aims to bring to the ultrasound the most likely anatomical parameters of evaluation in normal foetuses and in foetuses with spinal dysraphism.
    Child s Nervous System 02/2015; DOI:10.1007/s00381-015-2621-x · 1.16 Impact Factor