Minimally invasive intraluminal tracheal occlusion in a human fetus with left congenital diaphragmatic hernia at 27 weeks' gestation via direct fetal laryngoscopy

R. A. Quintero, W. J. Morales, P. W. Bornick, M. H. Allen, P. K. Johnson

Research output: Contribution to journalArticle

19 Scopus citations

Abstract

Congenital diaphragmatic hernia (CDH) occurs in approximately 1/3000 pregnancies and is associated with a 40-60% perinatal mortality rate due to pulmonary hypoplasia. In utero therapy is currently indicated in patients with liver herniation or a low lung-to-head ratio (LHR). Therapy consists of occluding the trachea to allow fetal lung growth and maturation. However, current methods of extraluminal tracheal occlusion have been associated with significant fetal and maternal morbidity. We report a case of CDH with a low LHR treated at 27 weeks with minimally invasive intraluminal tracheal occlusion (MITO). A direct fetal laryngoscopy was performed percutaneously, and the trachea was accessed. An intratracheal device was deployed under ultrasound guidance without complications. The intratracheal device remained in place for approximately 2 weeks, after which it became dislodged. The patient delivered vaginally at term a 2540-g infant. Severe pulmonary hypoplasia was diagnosed requiring extracorporeal membrane oxygenation support. Repair of the CDH was performed on the 10th day of life, at which time the diaphragm was noted to be virtually absent. Neonatal demise from severe pulmonary hypoplasia occurred on day 24 of life. Our case shows the feasibility of performing MITe via direct fetal laryngoscopy to treat fetuses with severe CDH; MITO should facilitate the in utero treatment of severe CDH by reducing maternal and fetal morbidity and hopefully result in improved perinatal outcome.

Original languageEnglish (US)
Pages (from-to)134-140
Number of pages7
JournalPrenatal and Neonatal Medicine
Volume5
Issue number2
StatePublished - Jan 1 2000

Keywords

  • Congenital diaphragmatic hernia
  • Fetal therapy
  • Operative fetoscopy
  • Ultrasound

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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