Fetoscopic endoluminal tracheal occlusion and reestablishment of fetal airways for congenital diaphragmatic hernia

Lennart Van der Veeken, Francesca Maria Russo, Luc De Catte, Eduard Gratacos, Alexandra Benachi, Yves Ville, Kypros Nicolaides, Christoph Berg, Glenn Gardener, Nicola Persico, Pietro Bagolan, Greg Ryan, Michael A Belfort, Jan Deprest, Lennart Van der Veeken, Francesca Maria Russo, Luc De Catte, Eduard Gratacos, Alexandra Benachi, Yves Ville, Kypros Nicolaides, Christoph Berg, Glenn Gardener, Nicola Persico, Pietro Bagolan, Greg Ryan, Michael A Belfort, Jan Deprest

Abstract

Background: Congenital diaphragmatic hernia (CDH) is a congenital anomaly with high mortality and morbidity mainly due to pulmonary hypoplasia and hypertension. Temporary fetal tracheal occlusion to promote prenatal lung growth may improve survival. Entrapment of lung fluid stretches the airways, leading to lung growth.

Methods: Fetal endoluminal tracheal occlusion (FETO) is performed by percutaneous sono-endoscopic insertion of a balloon developed for interventional radiology. Reversal of the occlusion to induce lung maturation can be performed by fetoscopy, transabdominal puncture, tracheoscopy, or by postnatal removal if all else fails.

Results: FETO and balloon removal have been shown safe in experienced hands. This paper deals with the technical aspects of balloon insertion and removal. While FETO is invasive, it has minimal maternal risks yet can cause preterm birth potentially offsetting its beneficial effects.

Conclusion: For left-sided severe and moderate CDH, the procedure is considered investigational and is currently being evaluated in a global randomized clinical trial (https://www.totaltrial.eu/). The procedure can be clinically offered to fetuses with severe right-sided CDH.

Keywords: CDH; Congenital diaphragmatic hernia; FETO; Fetal endoluminal tracheal occlusion; Fetal surgery; Fetoscopy.

Conflict of interest statement

LVDV, FMR, LDC, and JD are in the Academic Department of Development and Regeneration, Woman and Child, Biomedical Sciences, and Clinical Department of Obstetrics and Gynaecology, KU Leuven, Leuven, Belgium. EG, AB, YV, KN, CB, GG, NP, PB, GR, MAB, and JD are members of the TOTAL (Tracheal Occlusion To Accelerate Lung Growth Trial) Consortium. EG is head of the Barcelona Center for Maternale Fetal and Neonatal Medicine (Hospital Clínic and Hospital Sant Joan de Déu), IDIBAPS, University of Barcelona, and Centre for Biomedical Research on Rare Diseases (CIBER-ER), Barcelona, Spain. AB is head of the Department of Obstetrics, Gynaecology and Reproductive Medicine, Hôpital Antoine-Béclère, University Paris Sud, Clamart, France. YV is head of the Fetal Medicine Unit, Obstetrics and Fetal Medicine Department, Necker-Enfants Malades Hospital, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. KN is head of the Fetal Medicine Center, Harris Birthright Centre, King’s College Hospital, London, UK. CB is head of the Division of Fetal Surgery, Department of Obstetrics and Prenatal Medicine, University of Bonn, and Department of Obstetrics and Gynecology, University of Cologne, Germany. GG is head of the Mater Health Services, Mater Research UQ, Brisbane, Australia. NP is in the Department of Obstetrics and Gynecology “L. Mangiagalli,” Fondazione IRCCS “Ca′ Granda” - Ospedale Maggiore Policlinico, Milan, Italy. PB is head of Neonatal Surgery Unit, Department of Medical and Surgical Neonatology, Bambino Gesù Children’s Hospital, IRCCS, Piazza S. Onofrio, 4, 00165, Rome, Italy. GR is head of the Fetal Medicine Unit, Mt. Sinai Hospital, and University of Toronto, Toronto, Canada. MAB is head of the Department of Obstetrics and Gynecology, Baylor College of Medicine and Texas Children’s Fetal Center, Houston, Texas, USA. AB, PB, and JD are members of the European Reference Network on Rare and Inherited Congenital Anomalies “ERNICA.”Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Fetal endoscopic tracheal occlusion (FETO): a schematic drawing showing access to uterus and the fetal trachea. Inserts: steps in balloon delivery. © UZ Leuven, UZ Leuven, Belgium, drawing Myrthe Boymans
Fig. 2
Fig. 2
The o/e LHR is calculated by taking the ratio of the lung area divided by the head circumference, compared to a reference value for that gestational age. a Head circumference. b Lung area and diameter measured in the plane of the four-chamber view, the lung is posterior to the heart. c Survival rates of fetuses with left-sided CDH expectantly managed during pregnancy, as a function of different o/e LHR and liver position. d Same, for fetuses with right-sided CDH. Yellow arrows indicate improved survival after FETO as reported by Jani et al. 2006 (left CDH) and DeKoninck et al. 2014 (right CDH) [11, 26]. Adapted, with permission, from Russo et al. 2017 [27]
Fig. 3
Fig. 3
Transabdominal trocar entry in the direction of the tip of the nose. © UZ Leuven, UZ Leuven, Belgium
Fig. 4
Fig. 4
Fetoscope, fetoscopic forceps, and stylet, courtesy of KARL STORZ Endoskope, Tuttlingen, Germany
Fig. 5
Fig. 5
Landmarks used for guidance from the tip of the nose to trachea. Up from left to right: tip of nose, upper lip, tongue, raphe palate, and uvula. Down from left to right: epiglottis, vocal cords, trachea with inwards bulging pars membranacea, trachea more expanded and also better visualization of the tracheal rings, and carina. © UZ Leuven, UZ Leuven, Belgium
Fig. 6
Fig. 6
a After detachment the balloon can be seen through the vocal cords which are in this case just above it. b On ultrasound the balloon is visible as a hypoechogenic area. Power Doppler can help to distinguish the balloon from the adjacent blood vessels. © UZ Leuven, UZ Leuven, Belgium
Fig. 7
Fig. 7
Schematic drawing of tracheoscopic removal on placental circulation under loco-regional anesthesia. © UZ Leuven, UZ Leuven, Belgium, drawing Myrthe Boymans

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