Evaluation of Fetal Vocal Cords to Select Candidates for Successful Fetoscopic Treatment of Congenital High Airway Obstruction Syndrome: Preliminary Case Series

Abstract

Objective: Congenital high airway obstruction syndrome (CHAOS) normally has a dire prognosis due to laryngeal atresia and association with other anomalies. However, rare cases with an isolated tracheal membrane have been described. We assessed systematically the larynx and vocal cords in fetuses with CHAOS, with the goal of identifying such cases and attempting fetoscopic deobstruction. Methods: Between 2008 and 2012, 7 cases of CHAOS were referred to our institution. The ultrasonographic aspect of the larynx and trachea was assessed. We report the ultrasound findings, necropsy findings of 6 cases and perinatal outcome in 1 case undergoing therapy. Results: In 6 cases, laryngeal atresia was presumed by ultrasound and confirmed by necropsy after termination of pregnancy. In 1 case, normal appearance of the vocal cords led to suspicion of a possible isolated tracheal web completely occluding the trachea. Fetoscopy confirmed the finding, thus the membrane was perforated. Lung size normalized and hydrops resolved. A normal fetus was delivered at term, requiring no respiratory support. At 9 months of age, the infant showed a normal laryngoscopy and was developing well. Conclusions: Systematic ultrasound assessment of the vocal cords can be performed in fetuses with CHAOS. We report the first case of long-term intact survival after fetoscopic airway deobstruction.

Fetal Endoscopic Tracheal Intubation: A New Fetoscopic Procedure to Ensure Extrauterine Tracheal Permeability in a Case with Congenital Cervical Teratoma

“…However, it has been demonstrated that in experienced hands, fetal tracheoscopy for the treatment of fetuses with congenital diaphragmatic hernia can be achieved in a median surgical time of 10 min [15]. Similarly, we and others have demonstrated the feasibility of fetal airway endoscopy for cases with congenital high airway obstruction syndrome [16,17], bronchial atresia [18] and congenital microcystic adenomatoid malformation [19,20]. In this regard, it would be expected that fetal tracheoscopy in cases with neck masses may be more challenging due to severe distortion of the glottis, laryngeal and tracheal anatomy.…”

Laser Ablation of Posterior Urethral Valves by Fetal Cystoscopy

“…Further experience and technological improvements might help achieving better technical results. Obviously, the rarity of PUV will render the technique here described as an exceptional procedure for most fetal therapy units, being very likely used only in the context of experienced multidisciplinary fetal surgery teams, such as in other highly uncommon fetal surgeries [33,34]. In spite of technical success, prediction of complex forms of renal dysplasia will probably remain highly challenging, and a proportion of survivors will develop renal failure postnatally.…”

Treatment of a Fetal Tracheal Obstruction by Fetoscopy and Laser

“…However, in the series reported by Mong et al [4], 7 fetuses out of 10 died in utero. In the surviving cases, the quality of lung tissue was poor and rarely allowed normal respiratory function [5]. Fetoscopy should be an option for fetuses of less than 24 weeks because of the poor prognostic of tracheal obstruction in this situation.…”

“…Martinez et al [5] recently described a similar case of fetoscopic treatment of airway obstruction. They systematically assessed the larynx and vocal cords by a sonographic coronal view of the fetal neck and the passage of fluids through the trachea by color Doppler in every patient referred for CHAOS.…”