In our opinion, fetoscopic treatment for myelomeningocele is a promising intervention that should be the subject of further research and should be promoted. Our assessment of the results is not based on outdated data, and we did not turn the results on their head, as Kohl says in his letter. The reference (e19) is from 2012 and therefore as up to date as it can be. The author of a commentary to this article (1) expresses grave concerns—namely, that valid statements about improved neurological and muscular function can only be made when the children are rather older, and that the proportion of children with dyspnea syndrome (12 out of 13) was very high. We do not share the view expressed by Shurtleff (1), that intrauterine endoscopic correction of myelomeningocele in human fetuses is still unethical, but we do see the urgent need for a randomized study. In this context it would be of particular interest to compare a group of babies who had intrauterine surgery with a group of babies who were operated on postnatally, where the pregnancy was ended in the 33rd gestation week by elective cesarean. This is the mean gestational age at birth in the studies on intrauterine surgery that have been published to date. It may be that shortening the term of the pregnancy—thereby shortening the exposure of nerve fibers to the toxic effects of amniotic fluid—is sufficient to improve the neurological results. The results mentioned by Kohl, which have improved thanks to greater and growing experience, have so far been published only in abstract form in conference proceedings. We look forward with some excitement to the publication of these data in the shape of original, peer-reviewed, articles.
Randomized studies are also urgently needed to evaluate the therapy of severe congenital diaphragmatic hernia. Comparing the results of observational studies from individual centers is not conclusive, owing to different selection criteria and small case numbers (2). We agree with Schaible that the treatment of fetuses and neonates with CDH should be given in highly specialized perinatal centers. It is hard to assess whether using extracorporeal membrane oxygenation (ECMO) significantly improves outcomes as no randomized studies have been undertaken so far. A follow-up study from two Dutch centers (Nijmegen and Rotterdam) showed the worst results for CHD, compared with other indications for ECMO: mortality 42%, 16% of surviving babies had severe handicaps, and only 37.5% had normal neurological test results (3). A multicenter randomized study of intrauterine tracheal occlusion with a balloon (www.totaltrial.eu) is currently being conducted in Leuven, headed up by J Deprest. In Germany, the prenatal medicine department at Bonn University Medical Center and our own group are participating.
We thank Reuss for reminding us that the PLUTO study was stopped early. Only 31 of 200 planned pregnancies were randomized, and data are available for a further 46 non-randomized cases (www.pluto.bham.ac.uk). The data are currently being analyzed. We fundamentally agree with Reuss in her critical assessment of the prognosis in infravesical obstruction, but we do not think that total therapeutic nihilism is appropriate (4).
Footnotes
Conflict of interest statement
The authors of all contributions declare that no conflict of interest exists
References
- 1.Shurtleff D. Fetal endoscopic myelomeningocele repair. Dev Med Child Neurol. 2012;54:4–5. doi: 10.1111/j.1469-8749.2011.04141.x. [DOI] [PubMed] [Google Scholar]
- 2.Berdan EA, Saltzman DA. Right- versus left-sided diaphragmatic hernia—can we trust the data? Pediatr Crit Care Med. 2012;13:103–104. doi: 10.1097/PCC.0b013e31823886ee. [DOI] [PubMed] [Google Scholar]
- 3.Nijhuis-van der Sanden MWG, van der Cammen-van Zijp MHM, Janssen AJWM, et al. Motor performance in five-year-old extracorporeal membrane oxygenation survivors: a population-based study. Critical Care. 2009;13 doi: 10.1186/cc7770. R47 (doi:10.1186/cc7770) [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Morris RK, Malin GL, Khan KS, Kilby MD. Systematic review of the effectiveness of antenatal intervention for the treatment of congential lower utinary tract obstruction. BJOG. 2010;117:382–390. doi: 10.1111/j.1471-0528.2010.02500.x. [DOI] [PubMed] [Google Scholar]
- 5.Diemert A, Diehl W, Glosemeyer P, Deprest J, Hecher K. Intrauterine Surgery—Choices and Limitations. Dtsch Arztebl Int. 2012;109(38):603–608. doi: 10.3238/arztebl.2012.0603. [DOI] [PMC free article] [PubMed] [Google Scholar]