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Isolated Agnathia-Otocephaly Complex Diagnosed Prenatally for Ex-Utero Intrapartum Treatment: A Case Report.
- Source :
-
American Journal of Case Reports . 5/11/2023, Vol. 24, pe939016-1-e939016-7. 7p. - Publication Year :
- 2023
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Abstract
- Objective: Congenital defects/diseases Background: Agnathia-otocephaly complex (AOC) is a rare congenital malformation due to a first-branch arch disorder and has been considered lethal. However, milder variants of the isolated type of AOC have been reported as non- lethal. The ex-utero intrapartum treatment (EXIT) procedure is basically indicated for a fetus with a high risk of airway obstruction immediately after birth; it is not indicated for all AOC cases but is chosen to treat cases until the airway can be evaluated to achieve a better prognosis. Case Report: A 37-year-old woman was referred with reported fetal facial deformity and polyhydramnios at 27 weeks of gestation. Our fetal ultrasound scans showed agnathia, microstomia, and synotia, but not holoprosencepha- ly. Isolated AOC was diagnosed prenatally. Magnetic resonance imaging and microbubble tests revealed de- layed fetal lung maturation, although it was not completely unmatured. With patient agreement, an emergen- cy cesarean section with EXIT was performed because of clinical chorioamnionitis at 35 weeks of gestation. Tracheostomy was attempted for 16 min during EXIT and was completed 4 min after delivery. Despite this, the neonate died 12 h after delivery from severe respiratory failure and a tension pneumothorax caused by a hy- poplastic lung. Conclusions: There is controversy surrounding the non-lethality of all isolated AOC cases and the non-contraindication of EXIT procedures. Our case was estimated as the milder variant, and the EXIT procedure was indicated; howev- er, the neonate died of the hypoplastic lung. The evaluation methods of lung maturation are inconsistent, and the indication of the invasive EXIT procedure must be carefully considered. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 19415923
- Volume :
- 24
- Database :
- Academic Search Index
- Journal :
- American Journal of Case Reports
- Publication Type :
- Academic Journal
- Accession number :
- 163675300
- Full Text :
- https://doi.org/10.12659/AJCR.939016