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Case Reports
. 2019;46(1):75-80.
doi: 10.1159/000500455. Epub 2019 Jun 25.

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

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Case Reports

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

Clara T Nicolas et al. Fetal Diagn Ther. 2019.

Abstract

We present a case of prenatal hydrops secondary to congenital high airway obstruction syndrome (CHAOS) that was treated with fetoscopy-assisted needle decompression. A 22-year-old G3P2 woman presented after a 21-week ultrasound demonstrated CHAOS. The fetus developed hydrops at 25 weeks, characterized by abdominal ascites, pericardial effusion, and scalp edema. Fetal MRI showed complete obstruction of the glottis and subglottic airway, suggestive of laryngeal atresia. At 27 weeks, due to the progression of the hydrops, operative fetoscopy was proposed and performed. Fetal laryngoscopy confirmed fusion of the vocal cords and laryngeal atresia. The atretic segment was a solid cartilaginous block, preventing intubation. Using the fetoscope to stabilize the fetal head and neck, we performed ultrasound-guided percutaneous needle drainage of the cervical trachea through the anterior fetal neck. We removed 17 mL of viscous fluid from the lower trachea, resulting in immediate lung decompression. Two weeks later, ultrasound confirmed hydrops resolution. The patient was delivered and tracheostomy performed at 30 weeks via an ex utero intrapartum treatment (EXIT) procedure after progression of preterm labor. At 27 days of life, the infant was stable on minimal ventilator support. To our knowledge, this is the first successful report of an ultrasound-guided percutaneous tracheal decompression through the anterior neck of a fetus with CHAOS secondary to laryngeal atresia.

Keywords: CHAOS; Congenital high airway obstruction syndrome; EXIT; Ex utero intrapartum treatment; Fetal surgery; Fetoscopy; Laryngeal atresia.

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