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J Pediatr Surg. 2013 Oct;48(10):2005-10. doi: 10.1016/j.jpedsurg.2013.02.031.

Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach.

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1
Department of Surgery, Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Abstract

PURPOSE:

To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT).

METHODS:

We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012.

RESULTS:

Four patients were included in the series, three females and one male. The diagnoses were epignathus (n=2) and congenital epulis (n=2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185 min (range: 166-281) and median maternal operative time was 99 min (range: 85-153). Median maternal blood loss was 550 ml (range: 350-2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18-66). Mean fetal birth weight was 2.7 kg (range: 2.4-3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities.

CONCLUSIONS:

We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities.

KEYWORDS:

Congenital epulis; Congenital oropharyngeal tumors; Epignathus; Ex utero intrapartum treatment

PMID:
24094948
DOI:
10.1016/j.jpedsurg.2013.02.031
[Indexed for MEDLINE]
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