Fetal Surgery for Congenital Diaphragmatic Hernia

After appropriate counseling has been completed; the risks, benefits, and potential complications have been thoroughly reviewed; and it has been established that the family wishes to proceed, preparations are made for the fetal bronchoscopy and tracheal occlusion procedure.

The technique was originally described by Harrison et al^[15]and subsequently outlined in detail by Deprest et al.^[34]Briefly, after appropriate anesthesia is achieved, the uterine and fetal anatomy are carefully surveyed with ultrasonography (US). The placenta is avoided, and a laparoscopic trocar (usually a disposable central venous introducer sheath) is inserted by means of the Seldinger technique.

The fetoscope is introduced and maneuvered into the oropharynx, through the glottis, and into the trachea. The detachable balloon catheter is passed through the side-channel of the fetoscope, the balloon is inflated and detached, and then the fetoscope is withdrawn. Appropriate positioning of the balloon in the trachea is confirmed by means of US. (See the video and image below).

The fetoscope enters the oropharynx and passes through the glottis into the trachea. The mainstem bronchi are seen, and then the detachable balloon is passed through a side-channel of the fetoscope into the trachea, inflated, and detached in an appropriate position. The fetoscope is then removed and ultrasound confirms the balloon position in the trachea. Current practice is to repeat fetoscopy and remove the balloon after 4-6 weeks. Video courtesy of Division of Pediatric Surgery, University of California, San Francisco, School of Medicine.

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Fetoscopic images of landmarks during percutaneous fetal tracheal balloon occlusion. From top left to right bottom: epiglottis, vocal cords, trachea, carina, inflated and detached balloon, vocal cords about to close over the balloon. Image courtesy of Division of Pediatric Surgery, University of California, San Francisco, School of Medicine.

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Reversal Procedure

Since the development of the fetal tracheal occlusion technique, reversal of the occlusion has been recognized as an important part of the therapy, both to avoid the necessity of an ex-utero intrapartum treatment (EXIT) procedure^{[35, 36]} for delivery and to allow the lungs to recover from the distention forces that result in lung growth.^[37]

Reversal is achieved through repeat fetoscopy and balloon removal 4-6 weeks after the initial procedure. In the future, more sophisticated tracheal occlusion devices may allow for more physiologic lung growth and development.^[38]

Chiu PP, Sauer C, Mihailovic A, Adatia I, Bohn D, Coates AL, et al. The price of success in the management of congenital diaphragmatic hernia: is improved survival accompanied by an increase in long-term morbidity?. J Pediatr Surg. 2006 May. 41(5):888-92. [QxMD MEDLINE Link].
Cortes RA, Keller RL, Townsend T, Harrison MR, Farmer DL, Lee H, et al. Survival of severe congenital diaphragmatic hernia has morbid consequences. J Pediatr Surg. 2005 Jan. 40(1):36-45; discussion 45-6. [QxMD MEDLINE Link].
McHoney M, Hammond P. Role of ECMO in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed. 2018 Mar. 103 (2):F178-F181. [QxMD MEDLINE Link].
Harrison MR, Adzick NS, Longaker MT, Goldberg JD, Rosen MA, Filly RA, et al. Successful repair in utero of a fetal diaphragmatic hernia after removal of herniated viscera from the left thorax. N Engl J Med. 1990 May 31. 322(22):1582-4. [QxMD MEDLINE Link].
Harrison MR, Adzick NS, Flake AW, Jennings RW, Estes JM, MacGillivray TE, et al. Correction of congenital diaphragmatic hernia in utero: VI. Hard-earned lessons. J Pediatr Surg. 1993 Oct. 28(10):1411-7; discussion 1417-8. [QxMD MEDLINE Link].
Clifton MS, Wulkan ML. Congenital Diaphragmatic Hernia and Diaphragmatic Eventration. Clin Perinatol. 2017 Dec. 44 (4):773-779. [QxMD MEDLINE Link].
Wilson JM, DiFiore JW, Peters CA. Experimental fetal tracheal ligation prevents the pulmonary hypoplasia associated with fetal nephrectomy: possible application for congenital diaphragmatic hernia. J Pediatr Surg. 1993 Nov. 28(11):1433-9; discussion 1439-40. [QxMD MEDLINE Link].
DiFiore JW, Fauza DO, Slavin R, Peters CA, Fackler JC, Wilson JM. Experimental fetal tracheal ligation reverses the structural and physiological effects of pulmonary hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg. 1994 Feb. 29(2):248-56; discussion 256-7. [QxMD MEDLINE Link].
Hedrick MH, Estes JM, Sullivan KM, Bealer JF, Kitterman JA, Flake AW, et al. Plug the lung until it grows (PLUG): a new method to treat congenital diaphragmatic hernia in utero. J Pediatr Surg. 1994 May. 29(5):612-7. [QxMD MEDLINE Link].
Adzick NS, Outwater KM, Harrison MR, Davies P, Glick PL, deLorimier AA, et al. Correction of congenital diaphragmatic hernia in utero. IV. An early gestational fetal lamb model for pulmonary vascular morphometric analysis. J Pediatr Surg. 1985 Dec. 20(6):673-80. [QxMD MEDLINE Link].
Harrison MR, Adzick NS, Flake AW, VanderWall KJ, Bealer JF, Howell LJ, et al. Correction of congenital diaphragmatic hernia in utero VIII: Response of the hypoplastic lung to tracheal occlusion. J Pediatr Surg. 1996 Oct. 31(10):1339-48. [QxMD MEDLINE Link].
Skarsgard ED, Meuli M, VanderWall KJ, Bealer JF, Adzick NS, Harrison MR. Fetal endoscopic tracheal occlusion ('Fetendo-PLUG') for congenital diaphragmatic hernia. J Pediatr Surg. 1996 Oct. 31(10):1335-8. [QxMD MEDLINE Link].
Bealer JF, Skarsgard ED, Hedrick MH, Meuli M, VanderWall KJ, Flake AW, et al. The 'PLUG' odyssey: adventures in experimental fetal tracheal occlusion. J Pediatr Surg. 1995 Feb. 30(2):361-4; discussion 364-5. [QxMD MEDLINE Link].
Harrison MR, Mychaliska GB, Albanese CT, Jennings RW, Farrell JA, Hawgood S, et al. Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion. J Pediatr Surg. 1998 Jul. 33(7):1017-22; discussion 1022-3. [QxMD MEDLINE Link].
Harrison MR, Albanese CT, Hawgood SB, Farmer DL, Farrell JA, Sandberg PL. Fetoscopic temporary tracheal occlusion by means of detachable balloon for congenital diaphragmatic hernia. Am J Obstet Gynecol. 2001 Sep. 185(3):730-3. [QxMD MEDLINE Link].
Harrison MR, Keller RL, Hawgood SB, Kitterman JA, Sandberg PL, Farmer DL, et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med. 2003 Nov 13. 349(20):1916-24. [QxMD MEDLINE Link].
Keller RL, Hawgood S, Neuhaus JM, Farmer DL, Lee H, Albanese CT, et al. Infant pulmonary function in a randomized trial of fetal tracheal occlusion for severe congenital diaphragmatic hernia. Pediatr Res. 2004 Nov. 56(5):818-25. [QxMD MEDLINE Link].
Ruano R, Yoshisaki CT, da Silva MM, Ceccon ME, Grasi MS, Tannuri U, et al. A randomized controlled trial of fetal endoscopic tracheal occlusion versus postnatal management of severe isolated congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 2012 Jan. 39(1):20-7. [QxMD MEDLINE Link].
Deprest J, De Coppi P. Antenatal management of isolated congenital diaphragmatic hernia today and tomorrow: ongoing collaborative research and development. Journal of Pediatric Surgery Lecture. J Pediatr Surg. 2012 Feb. 47(2):282-90. [QxMD MEDLINE Link].
Deprest JA, Nicolaides K, Gratacos E. Fetal surgery for congenital diaphragmatic hernia is back from never gone. Fetal Diagn Ther. 2011. 29(1):6-17. [QxMD MEDLINE Link].
Persico N, Fabietti I, Ciralli F, Gentilino V, D'Ambrosi F, Boito S, et al. Fetoscopic Endoluminal Tracheal Occlusion in Fetuses with Severe Diaphragmatic Hernia: A Three-Year Single-Center Experience. Fetal Diagn Ther. 2017. 41 (3):215-219. [QxMD MEDLINE Link].
Grivell RM, Andersen C, Dodd JM. Prenatal interventions for congenital diaphragmatic hernia for improving outcomes. Cochrane Database Syst Rev. 2015 Nov 27. CD008925. [QxMD MEDLINE Link].
Al-Maary J, Eastwood MP, Russo FM, Deprest JA, Keijzer R. Fetal Tracheal Occlusion for Severe Pulmonary Hypoplasia in Isolated Congenital Diaphragmatic Hernia: A Systematic Review and Meta-analysis of Survival. Ann Surg. 2016 Dec. 264 (6):929-933. [QxMD MEDLINE Link].
Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg. 1996 Jan. 31(1):148-51; discussion 151-2. [QxMD MEDLINE Link].
Büsing KA, Kilian AK, Schaible T, Debus A, Weiss C, Neff KW. Reliability and validity of MR image lung volume measurement in fetuses with congenital diaphragmatic hernia and in vitro lung models. Radiology. 2008 Feb. 246(2):553-61. [QxMD MEDLINE Link].
Lazar DA, Ruano R, Cass DL, Moise KJ Jr, Johnson A, Lee TC, et al. Defining "liver-up": does the volume of liver herniation predict outcome for fetuses with isolated left-sided congenital diaphragmatic hernia?. J Pediatr Surg. 2012 Jun. 47(6):1058-62. [QxMD MEDLINE Link].
Ruano R, Yoshisaki CT, da Silva MM, Ceccon ME, Grasi MS, Tannuri U. A randomized controlled trial of fetal endoscopic tracheal occlusion versus postnatal management of severe isolated congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 2012 Jan. 39(1):20-7. [QxMD MEDLINE Link].
Jani JC, Nicolaides KH, Gratacós E, Valencia CM, Doné E, Martinez JM, et al. Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol. 2009 Sep. 34(3):304-10. [QxMD MEDLINE Link].
Araujo Júnior E, Tonni G, Martins WP, Ruano R. Procedure-Related Complications and Survival Following Fetoscopic Endotracheal Occlusion (FETO) for Severe Congenital Diaphragmatic Hernia: Systematic Review and Meta-Analysis in the FETO Era. Eur J Pediatr Surg. 2017 Aug. 27 (4):297-305. [QxMD MEDLINE Link].
Jani J, Valencia C, Cannie M, Vuckovic A, Sellars M, Nicolaides KH. Tracheal diameter at birth in severe congenital diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Prenat Diagn. 2011 Jul. 31(7):699-704. [QxMD MEDLINE Link].
Enriquez G, Cadavid L, Garcés-Iñigo E, Castellote A, Piqueras J, Peiró JL. Tracheobronchomegaly following intrauterine tracheal occlusion for congenital diaphragmatic hernia. Pediatr Radiol. 2012 Aug. 42(8):916-22. [QxMD MEDLINE Link].
Speggiorin S, Fierens A, McHugh K, Roebuck DJ, McLaren CA, Mok Q. Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution. J Pediatr Surg. 2011 May. 46(5):e1-3. [QxMD MEDLINE Link].
McHugh K, Afaq A, Broderick N, Gabra HO, Roebuck DJ, Elliott MJ. Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia. Pediatr Radiol. 2010 May. 40(5):674-80. [QxMD MEDLINE Link].
Deprest J, Nicolaides K, Done' E, Lewi P, Barki G, Largen E. Technical aspects of fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia. J Pediatr Surg. 2011 Jan. 46(1):22-32. [QxMD MEDLINE Link].
Hirose S, Farmer DL, Lee H, Nobuhara KK, Harrison MR. The ex utero intrapartum treatment procedure: Looking back at the EXIT. J Pediatr Surg. 2004 Mar. 39(3):375-80; discussion 375-80. [QxMD MEDLINE Link].
Van der Veeken L, Russo FM, De Catte L, Gratacos E, Benachi A, Ville Y, et al. Fetoscopic endoluminal tracheal occlusion and reestablishment of fetal airways for congenital diaphragmatic hernia. Gynecol Surg. 2018. 15 (1):9. [QxMD MEDLINE Link]. [Full Text].
Flageole H, Evrard VA, Piedboeuf B, Laberge JM, Lerut TE, Deprest JA. The plug-unplug sequence: an important step to achieve type II pneumocyte maturation in the fetal lamb model. J Pediatr Surg. 1998 Feb. 33(2):299-303. [QxMD MEDLINE Link].
Jelin EB, Etemadi M, Encinas J, Schecter SC, Chapin C, Wu J, et al. Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia. J Pediatr Surg. 2011 Jun. 46(6):1150-7. [QxMD MEDLINE Link].

Media Gallery

Schematic of fetal tracheal balloon occlusion, using a catheter-based detachable balloon that is placed through the side channel of the fetoscope.
The fetoscope enters the oropharynx and passes through the glottis into the trachea. The mainstem bronchi are seen, and then the detachable balloon is passed through a side-channel of the fetoscope into the trachea, inflated, and detached in an appropriate position. The fetoscope is then removed and ultrasound confirms the balloon position in the trachea. Current practice is to repeat fetoscopy and remove the balloon after 4-6 weeks. Video courtesy of Division of Pediatric Surgery, University of California, San Francisco, School of Medicine.
Fetoscopic images of landmarks during percutaneous fetal tracheal balloon occlusion. From top left to right bottom: epiglottis, vocal cords, trachea, carina, inflated and detached balloon, vocal cords about to close over the balloon. Image courtesy of Division of Pediatric Surgery, University of California, San Francisco, School of Medicine.