Abstract:Tracheal agenesis (TA), aplasia or total atresia of the trachea are congenital anomalies which are still incompatible with life. Despite the many attempts of different interventions, there are yet no promising, long-term methods of treatment. Only with sufficient proportion of the proximal or distal trachea available, it is possible to place a tracheostomy, which also opens up new vistas of life for the affected child. In most cases the seldom deformation, trachealagenesis, does not get recognised before the c… Show more
“…Forty-nine cases of TA, including our case, were found [1, 2, 4–7, 9–11, 14, 17, 18, 20, 23, 24, 26, 29–33, 35–38, 40, 42]. The perinatal characteristics, diagnostic investigations, and management are summarized (Table 1) .…”
Tracheal agenesis (TA) is a severe congenital disorder with often an unexpected emergency presentation. There is complete or partial absence of the trachea below the larynx, with presence or absence of a tracheoesophageal fistula (TOF). A neonate with TA is described, and another 48 cases found in literature are reviewed. Due to absence of a TOF, five cases were diagnosed prenatally because of congenital high airway obstruction syndrome (CHAOS). When a TOF is present, polyhydramnion and several other congenital malformations seen on the ultrasound examination should alert clinicians of potential tracheal problems. Prenatal magnetic resonance imaging (MRI) may provide a definitive diagnosis. Postnatal diagnosis is based on recognition of specific clinical signs in the newborn with TA: respiratory distress with breathing movement without appropriate air entry, no audible cry, and failed endotracheal intubation. Despite progress in surgical interventions, mortality remains high. Prenatal diagnosis of TA is possible, but only if a TOF is absent resulting in CHAOS. Prenatal diagnosis of polyhydramnion and other congenital malformation should alert clinicians of potential tracheal problems. Prenatal MRI may provide a definitive diagnosis.
“…Forty-nine cases of TA, including our case, were found [1, 2, 4–7, 9–11, 14, 17, 18, 20, 23, 24, 26, 29–33, 35–38, 40, 42]. The perinatal characteristics, diagnostic investigations, and management are summarized (Table 1) .…”
Tracheal agenesis (TA) is a severe congenital disorder with often an unexpected emergency presentation. There is complete or partial absence of the trachea below the larynx, with presence or absence of a tracheoesophageal fistula (TOF). A neonate with TA is described, and another 48 cases found in literature are reviewed. Due to absence of a TOF, five cases were diagnosed prenatally because of congenital high airway obstruction syndrome (CHAOS). When a TOF is present, polyhydramnion and several other congenital malformations seen on the ultrasound examination should alert clinicians of potential tracheal problems. Prenatal magnetic resonance imaging (MRI) may provide a definitive diagnosis. Postnatal diagnosis is based on recognition of specific clinical signs in the newborn with TA: respiratory distress with breathing movement without appropriate air entry, no audible cry, and failed endotracheal intubation. Despite progress in surgical interventions, mortality remains high. Prenatal diagnosis of TA is possible, but only if a TOF is absent resulting in CHAOS. Prenatal diagnosis of polyhydramnion and other congenital malformation should alert clinicians of potential tracheal problems. Prenatal MRI may provide a definitive diagnosis.
“…M ö glicherweise werden sich so auch leichter neue Therapiema ß nahmen entwickeln lassen, um bisher noch als fatal anzusehende Fehlbildungen von Ö sophagus und / oder Trachea (z. B. Trachealagenesie) erfolgreich zu behandeln [5] . Die Effi zienz derartiger diagnosebezogener klinischer Register steht au ß er Frage [8] , ihr Dilemma ist die ungekl ä rte l ä ngerfristige Finanzierung [6] .…”
Section: Rehabilitation Of the Benign Oesophageal Stenosis In Childhoodunclassified
“…Obwohl bei 86 % der Neugeborenen eine Ö sophagusatresie ( Ö A) des Typs Vogt III b mit oberem Blindsack und unterer tracheo ö sophagealer Fistel (T Ö F) vorliegt, sind stets seltene Varianten zu bedenken [1,9] .…”
This report highlights the diagnostic and therapeutic challenges which occurred in a newborn with oesophageal atresia of the type Vogt III c in association with a tubular supracarinal tracheal stenosis.
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