Management of tracheal agenesis

Abstract

Complete tracheal agenesis is a very rare congenital anomaly that is only compatible with life in some cases with associated tracheo-oesophageal or broncho-oesophageal fistula. In most cases, concomitant congenital anomalies of the heart, digestive tract or genitourinary tract are present. It should be suspected in any neonate with a history of hydramnios, absent crying, respiratory distress and difficulty in intubation. The possibility for surgical correction or palliation rests on the extent of atresia present. We present a case of complete tracheal agenesis without tracheo nor broncho-oesophageal fistula (type II by Floyd's classification) - the diagnosis of which was prenatally suspected - and discuss the important features of the airway management of this condition.

Tracheal agenesis with unique anatomy

“…Occasionally, the diagnosis is made antenatally by fetal magnetic resonance imaging [4]. Maternal polyhydramnios is often present, as is prematurity.…”

“…There is a 2:1 male predominance. Other anomalies are often present, which include cardiac defects, genitourinary tract, and limb abnormalities [3][4][5][6]. This has led some authors to suggest that TA may be one of the components of the VACTERL association which includes vertebral anomalies, anal atresia, cardiac defects, TOF or OA, and renal and limb malformations [7].…”

Neonatal airway: challenging endotracheal intubation in infants with tracheal malformations at birth

“…Prenatal diagnosis of TA is rare and only possible in the absence of TEF, as the patient would present with classical congenital high airway obstruction syndrome (CHAOS), characterised by enlarged hyperechogenic lungs, fluid-filled dilated trachea and bronchi, and an absent flow in the trachea during fetal breathing. As phospholipids are secreted into the amniotic fluid by lungs, the absence of it in cases of CHAOS may support the diagnosis of TA 7. CHAOS should be suspected in pregnancies with a history of oligohydramnios in early pregnancy and/or polyhydramnios later on in pregnancy due to compression of oesophagus resulting in decreased fetal swallowing 8.…”

“…In TA with TEF, alternative management lines include intubating the oesophagus and ventilating the infant using the fistula as a conduit, even though there will be cycles of stabilisation and deterioration as observed in case 2. Chest movement should be observed if this manoeuvre is useful 7. If oesophageal intubation can stabilise the infant, oesophageal ligation distal to the site of TEF can be considered to ventilate through the fistula and a gastrostomy is needed to feed the infant 14.…”

Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature

“…If uncertainty remains, fetal MRI can provide additional information [28]. If CHAOS is suspected, absence of amniotic phospholipids in amniotic fluid may support the diagnosis of TA because normally phospholipids are secreted into the amniotic fluid by the respiratory tract [8]. If TA is prenatally suspected, an EXIT procedure can be planned in a tertiary hospital as described by Vaikunth et al [38].…”