title: Fetoscopic and ultrasound-guided decompression of the fetal trachea in a human fetus with Fraser syndrome and congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia.
abstract: Congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia bears a poor prognosis for hydropic fetuses owing to cardiac failure. We attempted percutaneous fetoscopic and ultrasound-guided tracheal decompression in a hydropic human fetus with CHAOS associated with Fraser syndrome. Percutaneous fetoscopic and ultrasound-guided tracheal decompression was performed using three trocars under general materno-fetal anesthesia at 19 + 5 weeks of gestation. Abnormal fetoplacental blood flow normalized within hours as a result of the intervention. Furthermore, a normalization of lung : heart size and lung echogenicity was observed within days. Resolution of hydrops was complete within 3 weeks. Premature rupture of membranes and premature contractions prompted emergency delivery of the fetus by ex-utero intrapartum treatment (EXIT) at 28 + 2 weeks of gestation. Following delivery, the lungs could be ventilated at low pressures and ambient oxygen concentration. Weaning from ventilation was achieved at 18 days of postnatal life. Our experience indicated that percutaneous fetoscopic and ultrasound-guided decompression of the fetal trachea is feasible and may permit normalization of hemodynamics in hydropic human fetuses with CHAOS from laryngeal atresia. The procedure may also result in normalization of heart : lung size and provide the time needed to regain the function of the overstretched diaphragm in this grave fetal condition.
authors.affiliation: German Center for Fetal Surgery & Minimally-Invasive Therapy, Department of Obstetrics & Prenatal Medicine, University of Bonn, Bonn, Germany.|Department of Cardiology, University Children's Hospital Münster, Münster, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Kohl
authors.affiliation: Department of Anesthesiology, University of Bonn, Bonn, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Hering
authors.affiliation: Department of Internal Medicine II-Cardiology, University of Bonn, Bonn, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Bauriedel
authors.affiliation: German Center for Fetal Surgery & Minimally-Invasive Therapy, Department of Obstetrics & Prenatal Medicine, University of Bonn, Bonn, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Van de Vondel
authors.affiliation: Department of Neonatology, University of Bonn, Bonn, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Heep
authors.affiliation: Clinic for ENT, Head and Neck Surgery, University of Bonn, Bonn, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Keiner
authors.affiliation: Department of Neonatology, University of Bonn, Bonn, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Müller
authors.affiliation: Department of Neonatology, University of Bonn, Bonn, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Franz
authors.affiliation: Department of Neonatology, University of Bonn, Bonn, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Bartmann
authors.affiliation: German Center for Fetal Surgery & Minimally-Invasive Therapy, Department of Obstetrics & Prenatal Medicine, University of Bonn, Bonn, Germany.
authors.forename: U
authors.initials: U
authors.lastname: Gembruch
mesh_terms: D000402:Airway Obstruction / Q000601:surgery*; D019299:Decompression, Surgical; D005260:Female; D005315:Fetal Diseases / Q000601:surgery*; D005332:Fetoscopy / Q000379:methods; D006801:Humans; D007830:Larynx / Q000002:abnormalities*; D011247:Pregnancy; D013577:Syndrome; D018084:Ultrasonography, Interventional / Q000379:methods; D016216:Ultrasonography, Prenatal / Q000379:methods*