A Case of Congenital High Airway Obstruction Syndrome Managed by Ex Utero Intrapartum Treatment: Case Report and Review of the Literature

Abstract

Congenital high airway obstruction syndrome (CHAOS) has been reported to be fatal. Ten cases of CHAOS that underwent ex utero intrapartum treatment (EXIT) procedure to secure the fetal airway have been reported. A 36-year-old woman (gravida 3, para 2) was referred to our hospital at 22 weeks of gestation. Sonography revealed large echogenic lungs, flattened diaphragm, and marked hydrops. Magnetic resonance imaging confirmed the diagnosis of CHAOS. Polyhydramnios and fetal skin edema were improved and the fetal ascitic fluid was regressed gradually. At 36 weeks of gestation, an EXIT procedure was undertaken. Fetal laryngoscopy and bronchoscopy showed complete laryngeal obstruction, and a tracheostomy was performed immediately. The infant was discharged from hospital at 6 weeks of age. Thereafter, he developed well both physically and mentally. A laryngoplasty was performed at 20 months of age using silicon sheet as a patent airway. The child has a tracheostomy, is able to phonate but does not speak, and is awaiting decannulation. Use of the EXIT procedure in CHAOS cases offers the potential for salvage and excellent long-term outcome of these fetuses that otherwise would not survive. However, management of the airway, particularly with regard to long-term reconstruction in children with CHAOS, remains challenging.

Predicting the severity of congenital high airway obstruction syndrome

“…Understanding the natural history of the disease is essential. Although a few fetuses with CHAOS have been saved by emergency tracheostomy immediately after delivery, use of the EXIT procedure has most dramatically improved survival with CHAOS [3,[5][6][7][8][9][10][11][12][13][14]. The EXIT approach preserves the uteroplacental circulation to the fetus while an airway is established.…”

Tracheal agenesis without esophageal fistula: genetic, resuscitative, and pathological issues

“…Three children have survived after this experimental procedure, and this is the largest available population of surviving babies [11]. More recent data confirm that survival may be enabled by means of EXIT [12]. To our knowledge, only one case survived for a long time without EXIT procedure, undergoing several corrective surgeries [13].…”

Fetoscopy-Assisted Percutaneous Decompression of the Distal Trachea and Lungs Reverses Hydrops Fetalis and Fetal Distress in a Fetus with Laryngeal Atresia

“…CHAOS is fatal without intervention and only a small number of case reports exist that describe successful delivery of these patients on uteroplacental bypass [5-9]. Delayed cord clamping has also been proposed as an alternative method to manage CHAOS in cases where the EXIT procedure is not possible [10].…”