Neonatal Respiratory Distress and Airway Emergency: Report of Two Cases.

Lorenzo Bresciani,Amerigo Santoro,Gaetano Chirico,Carla Baronchelli,Roberta Bosio,Paola Grazioli,Luca O Redaelli De Zinis,Cesare Zambelloni

doi:10.3390/children8040255

Lorenzo Bresciani, Amerigo Santoro + Show 6 more

Open Access

https://doi.org/10.3390/children8040255

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Journal: Children	Publication Date: Mar 25, 2021
Citations: 2	License type: CC BY 4.0

Affiliation: Spedali Civili di Brescia, University of Brescia

Abstract

We discuss two cases of congenital airway malformations seen in our neonatal intensive care unit (NICU). The aim is to report extremely rare events characterized by immediate respiratory distress after delivery and the impossibility to ventilate and intubate the airway. The first case is a male twin born at 34 weeks by emergency caesarean section. Immediately after delivery, the newborn was cyanotic and showed severe respiratory distress. Bag-valve-mask ventilation did not relieve the respiratory distress but allowed for temporary oxygenation during subsequent unsuccessful oral-tracheal intubation (OTI) attempts. Flexible laryngoscopy revealed complete subglottic obstruction. Postmortem analysis revealed a poly-malformative syndrome, unilateral multicystic renal dysplasia with a complete subglottic diaphragm, and a tracheo-esophageal fistula (TEF). The second case is a male patient that was vaginally born at 35 weeks. Antenatally, an ultrasound (US) arose suspicion for a VACTERL association (vertebral defects, anal atresia, TEF with esophageal atresia and radial or renal dysplasia, plus cardiovascular and limb defects) and a TEF, and thus, fetal magnetic resonance (MRI) was scheduled. Spontaneous labor started shortly thereafter, before imaging could be performed. Respiratory distress, cyanosis, and absence of an audible cry was observed immediately at delivery. Attempts at OTI were unsuccessful, whereas bag-valve-mask ventilation and esophageal intubation allowed for sufficient oxygenation. An emergency tracheostomy was attempted, although no trachea could be found on cervical exploration. Postmortem analysis revealed tracheal agenesis (TA), renal dysplasia, anal atresia, and a single umbilical artery. Clinicians need to be aware of congenital airway malformations and subsequent difficulties upon endotracheal intubation and must plan for multidisciplinary management of the airway at delivery, including emergency esophageal intubation and tracheostomy.

Highlights

Congenital high airway obstruction syndrome (CHAOS) is a rare and frequently fatal disorder caused by complete or near-complete obstruction of the fetal upper airway [1]
Prenatal diagnosis is desirable, even though difficult, in order to plan for ex-utero intrapartum treatment (EXIT) [2,3,4,5]
We present two cases of tracheal agenesis (TA) and Laryngeal atresia (LA) observed in our neonatal intensive care unit (NICU) in a four-month period

Summary

Introduction

Congenital high airway obstruction syndrome (CHAOS) is a rare and frequently fatal disorder caused by complete or near-complete obstruction of the fetal upper airway [1]. Laryngeal atresia (LA) and tracheal agenesis (TA) are two of the rare causes of intrinsic airway obstruction in CHAOS [4,5]. Laryngeal atresia (LA) and tracheal agenesis (TA) are two of the rare causes of intrinsic airway tohbosutgruhcdtiioffniciunltC, HinAoOrdSe[r4t,5o].plan for ex-utero intrapartum treatment (EXIT) [2,3,4,5]. LA is characterized by complete or near-complete laryngeal obstruction and is classified according to either the anatomic site of obstruction or the presence of an associated TEF and the following clinical presentations, as shown in Table 1 [2,12]. The aim of this paper is to discuss rare congenital airway malformations which present as emergencies at delivery if not identified during pregnancy, and which carry an overall poor prognosis despite intensive treatment [10,13,14]

Case One

Case Two

Findings

Conclusions