Ex utero intrapartum treatment in the management of giant cervical teratomas

Pablo Laje, Mark P. Johnson, Lori J. Howell, Michael W. Bebbington, Holly L. Hedrick, Alan W. Flake, N. Scott Adzick

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84 Scopus citations

Abstract

Purpose: The purpose of this study is to present the outcome and technical details of the Ex Utero Intrapartum Treatment (EXIT) procedure performed in the management of the fetus with a giant cervical teratoma. Methods: A retrospective review of the medical records of patients undergoing the EXIT procedure between September 1995 and September 2010 was performed. Results: Eighty-seven EXIT procedures were performed. In 20% of cases (17/87), the indication was giant cervical teratoma. There were 10 females and 7 males. Polyhydramnios was present in 82%. Median gestational age at EXIT was 35 weeks (range, 30-39 weeks). Median birth weight was 2.5 kg (range, 1.7-3.7 kg). Access to the airway under placental support was established in all cases via direct laryngoscopy/ bronchoscopy in 8 patients (47%) and via surgical exploration (tracheostomy or retrograde intubation) in 9 patients (53%). The mortality rate under placental support was zero. Seven patients had the tumors resected immediately after the EXIT, 6 patients had the resection later, and 4 patients died before resection. The neonatal mortality rate was 23% (4/17 patients). Patients who died had severe pulmonary hypoplasia that resulted from the upward traction by the giant cervical mass on the airway and compression of the lungs against the thoracic apex. Conclusions: We conclude that the EXIT procedure continues to be the optimal delivery strategy for patients with prenatally diagnosed giant cervical teratomas and potential airway obstruction at birth. A thorough evaluation of the prenatal images and an experienced multidisciplinary team are key factors for an effective approach to the obstructed fetal airway.

Original languageEnglish
Pages (from-to)1208-1216
Number of pages9
JournalJournal of Pediatric Surgery
Volume47
Issue number6
DOIs
StatePublished - Jun 2012

Keywords

  • Cervical teratoma
  • EXIT
  • Ex Utero Intrapartum Treatment
  • Tracheostomy

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