Article

Use of computed tomography to categorize the type of tracheal agenesis.

Department of Pediatrics, Strong Children's Research Center, University of Rochester Medical Center, Rochester, NY 14642, USA.
Journal of Pediatric Surgery (impact factor: 1.45). 06/2009; 44(5):1044-6. DOI:10.1016/j.jpedsurg.2009.01.051 pp.1044-6
Source: PubMed

ABSTRACT We report a case of type I tracheal agenesis with vertebral defects, anal atresia, tracheoesophageal fistula and/or esophageal atresia, radial dysplasia, renal defects, and cardiovascular defects association in which computed tomography was helpful in categorizing the diagnosis. This rare and fatal condition should always be considered when confronted with a neonate impossible to resuscitate at birth.

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    Article: Tracheal agenesis: approach towards this severe diagnosis. Case report and review of the literature.
    Maurike D de Groot-van der Mooren, Monique C Haak, Phillis Lakeman, Titia E Cohen-Overbeek, J Patrick van der Voorn, Jochen H Bretschneider, Ruurd M van Elburg
    [show abstract] [hide abstract]
    ABSTRACT: 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.
    European Journal of Pediatrics 09/2011; 171(3):425-31. · 1.88 Impact Factor
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Keywords

anal atresia
 
cardiovascular defects association
 
categorizing
 
fatal condition
 
tracheoesophageal fistula
 
vertebral defects