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Treatment of severe congenital diaphragmatic hernia by fetal tracheal occlusion: Clinical experience with fifteen cases

  • Alan W. Flake
    Affiliations
    Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, and the Departments of Surgery and Obstetrics, University of Pennsylvania School of Medicine. Philadelphia, Pennsylvania
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  • Timothy M. Crombleholme
    Affiliations
    Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, and the Departments of Surgery and Obstetrics, University of Pennsylvania School of Medicine. Philadelphia, Pennsylvania
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  • Mark P. Johnson
    Affiliations
    Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, and the Departments of Surgery and Obstetrics, University of Pennsylvania School of Medicine. Philadelphia, Pennsylvania
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  • Lori J. Howell
    Affiliations
    Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, and the Departments of Surgery and Obstetrics, University of Pennsylvania School of Medicine. Philadelphia, Pennsylvania
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  • N.Scott Adzick
    Affiliations
    Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, and the Departments of Surgery and Obstetrics, University of Pennsylvania School of Medicine. Philadelphia, Pennsylvania
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      Abstract

      Objective: Our purpose was to determine whether prenatal tracheal occllusion improves survival in a selected population of fetuses affected by severe congenital diaphragmatic hernia. Study Design: Fetuses with isolated congenital diaphragmatic hernia were selected as candidates for fetal intervention by specific criteria designed to predict a 90% mortality rate with conventional postnatal treatment. Results: Fifteen fetuses underwent tracheal occlusion with 5 survivors (33%). Two fetuses were lost to early preterm labor. In 13 mothers, postoperative gestation ranged from 19 to 68 days, with a mean duration of pregnancy after tracheal occlusion of 38 days. The 5 survivors were hospitalized for an average of 76 days. Despite dramatic lung growth in some fetuses after tracheal occlusion, intensive management was required, and most deaths were caused by respiratory insufficiency. Conclusion: Prenatal tracheal occlusion can result in impressive lung growth in a subset of fetuses with severe congenital diaphragmatic hernia. However, survival remains compromised by pulmonary functional abnormality and the consequences of prematurity. (Am J Obstet Gynecol 2000;183:1059-66.)

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      References

        • Harrison MR
        • Adzick NS
        • Flake AW
        Congenital diaphragmatic hernia: an unsolved problem.
        Semin Pediatr Surg. 1993; 2: 109-112
        • Flake A
        Fetal surgery for congenital diaphragmatic hernia.
        Semin Pediatr Surg. 1996; 5: 266-274
        • Harrison MR
        • Adzick NS
        • Flake AW
        • Jennings RW
        The CDH two-step: a dance of necessity.
        J Pediatr Surg. 1993; 28: 813-816
        • Harrison MR
        • Adzick NS
        • Bullard KM
        • Farrell JA
        • Howell LJ
        • Rosen MA
        • et al.
        Correction of congenital diaphragmatic hernia in utero. VII. A prospective trial.
        J Pediatr Surg. 1997; 32: 1637-1642
        • 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; 31: 1339-1348
        • 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; 33: 1017-1022
        • Metkus AP
        • Filly RA
        • Stringer MD
        • Harrison MR
        • Adzick NS
        Sonographic predictors of survival in fetal diaphragmatic hernia.
        J Pediatr Surg. 1996; 31: 148-151
        • Adzick N
        • Harrison M
        • Flake A
        • Glick P
        • Bottles K
        Automatic uterine stapling devices in fetal surgery: experience in a primate model.
        Surg Forum. 1985; 36: 479-481
        • Mychaliska GB
        • Bealer JF
        • Graf JL
        • Rosen MA
        • Adzick NS
        • Harrison MR
        Operating on placental support: the ex utero intrapartum treatment procedure.
        J Pediatr Surg. 1997; 32: 227-230
        • 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; 28: 1433-1439
        • 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; 29: 248-256
        • 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; 29: 612-617
        • Kitano Y
        • Yang EY
        • von Allmen D
        • Quinn TM
        • Adzick NS
        • Flake AW
        Tracheal occlusion in the fetal rat: a new experimental model for the study of accelerated lung growth.
        J Pediatr Surg. 1998; 33: 1741-1744
        • Kitano Y
        • Davies P
        • von Allmen D
        • Adzick NS
        • Flake AW
        Fetal tracheal occlusion in the rat model of nitrofen-induced congenital diaphragmatic hernia.
        J Appl Physiol. 1999; 87: 769-775
        • Harrison MR
        • Adzick NS
        • Estes JM
        • Howell LJ
        A prospective study of the outcome for fetuses with diaphragmatic hernia.
        JAMA. 1994; 271: 382-384
        • Lipshutz GS
        • Albanese CT
        • Feldstein VA
        • Jennings RW
        • Housley HT
        • Beech R
        • et al.
        Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia.
        J Pediatr Surg. 1997; 32: 1634-1636
        • Walsh DS
        • Hubbard AM
        • Olutoye OO
        • Crombleholme TM
        • Flake AW
        • Johnson MP
        • et al.
        Assessment of fetal lung volumes and liver herniation with magnetic resonance imaging in congenital diaphragmatic hernia [abstract 12].
        Am J Obstet Gynecol. 2000; 182: S16
        • Albanese CT
        • Lopoo J
        • Goldstein RB
        • Filly RA
        • Feldstein VA
        • Calen PW
        • et al.
        Fetal liver position and perinatal outcome for congenital diaphragmatic hernia.
        Prenat Diagn. 1998; 18: 1138-1142
        • 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; 30: 361-365
      1. Kitano Y, Kanai M, Davies P, von Allmen D, Yang EY, Kitano Y, et al. Lung growth induced by prenatal tracheal occlusion and its modifying factors: a study in the rat model of congenital diaphragmatic hernia. J Pediatr Surg. In press.

        • Benachi A
        • Chailley-Heu B
        • Delezoide AL
        • Dommergues M
        • Brunelle F
        • Dumez Y
        • et al.
        Lung growth and maturation after tracheal occlusion in diaphragmatic hernia.
        Am J Respir Crit Care Med. 1998; 157: 921-927
        • Bin Saddiq W
        • Piedboeuf B
        • Laberge JM
        • Gamache M
        • Petrov P
        • Hashim E
        • et al.
        The effects of tracheal occlusion and release on type II pneumocytes in fetal lambs.
        J Pediatr Surg. 1997; 32: 834-838
        • O’Toole SJ
        • Karamanoukian HL
        • Irish MS
        • Sharma A
        • Holm BA
        • Glick PL.
        Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment.
        J Pediatr Surg. 1997; 32: 407-410
        • Graf JL
        • Gibbs DL
        • Adzick NS
        • Harrison MR
        Fetal hydrops after in utero tracheal occlusion.
        J Pediatr Surg. 1997; 32: 214-215
        • Lund DP
        • Mitchell J
        • Kharasch V
        • Quigley S
        • Kuehn M
        • Wilson JM
        Congenital diaphragmatic hernia: the hidden morbidity.
        J Pediatr Surg. 1994; 29: 258-262
        • Schoeman L
        • Pierro A
        • Macrae D
        • Spitz L
        • Kiely EM
        • Drake DP
        Late death after extracorporeal membrane oxygenation for congenital diaphragmatic hernia.
        J Pediatr Surg. 1999; 34: 357-359
        • Bealer JF
        • Raisanen J
        • Skarsgard ED
        • Long SR
        • Wong K
        • Filly RA
        • et al.
        The incidence and spectrum of neurological injury after open fetal surgery.
        J Pediatr Surg. 1995; 30: 1150-1154
        • D’Agostino JA
        • Bernbaum JC
        • Gerdes M
        • Schwartz IP
        • Coburn CE
        • Hirschl RB
        • et al.
        Outcome for infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: the first year.
        J Pediatr Surg. 1995; 30: 10-15
        • McGahren ED
        • Mallik K
        • Rodgers BM
        Neurological outcome is diminished in survivors of congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation.
        J Pediatr Surg. 1997; 32: 1216-1220
        • Stolar CJ
        • Crisafi MA
        • Driscoll YT
        Neurocognitive outcome for neonates treated with extracorporeal membrane oxygenation: are infants with congenital diaphragmatic hernia different?.
        J Pediatr Surg. 1995; 30: 366-371
        • Benachi A
        • Dommergues M
        • Delezoide AL
        • Bourbon J
        • Dumez Y
        • Brunnelle F
        Tracheal obstruction in experimental diaphragmatic hernia: an endoscopic approach in the fetal lamb.
        Prenat Diagn. 1997; 17: 629-634
        • Deprest JA
        • Evrard VA
        • Van Ballaer PP
        • Verbeken E
        • Vandenberghe K
        • Lerut TE
        • et al.
        Tracheoscopic endoluminal plugging using an inflatable device in the fetal lamb model.
        Eur J Obstet Gynecol Reprod Biol. 1998; 81: 165-169
        • Luks FI
        • Gilchrist BF
        • Jackson BT
        • Piasecki GJ
        Endoscopic tracheal obstruction with an expanding device in a fetal lamb model: preliminary considerations.
        Fetal Diagn Ther. 1996; 11: 67-71
        • 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; 33: 299-303
        • Papadakis K
        • De Paepe ME
        • Tackett LD
        • Piasecki GJ
        • Luks FI
        Temporary tracheal occlusion causes catch-up lung maturation in a fetal model of diaphragmatic hernia.
        J Pediatr Surg. 1998; 33: 1030-1037