Abstract

MIR, Ramón et al. Tracheal Agenesis. a Rare Congenital Anomaly with High-Mortality. Pediatr. (Asunción) [online]. 2014, vol.41, n.3, pp.215-218. ISSN 1683-9803.

Introduction: Tracheal agenesis is a very rare congenital malformation that is incompatible with life. The anomaly consists of the partial or total absence of the trachea below the larynx that allows connection of the lower respiratory tract with the gastrointestinal tract via a distal tracheoesophageal fistula. Case Report: We present the case of a newborn with maternal history of polyhydramnios, born with respiratory distress, lack of crying, difficulty in orotracheal intubation, Apgar 4/8, who was referred to the NICU and given mechanically assisted ventilation. At 6 hs of life accidental extubation occurred, with re-intubation not being successful. Tomography of the neck and thorax showed tracheal agenesis with a distal tracheoesophageal fistula, but no other congenital malformations. Various palliative surgeries were performed. In spite of medical efforts, the patient died at 6 days of life due to airway collapse. Discussion: We report this case due to tracheal agenesis being a very rare malformation with few cases described in the literature. This was the first case seen at our hospital, and presents a complex clinical picture that required multidisciplinary management and various palliative surgeries prior to the patient’s death. The condition is generally fatal and presents a poor prognosis as no appropriate techniques for tracheal replacement have yet been developed.

Keywords : Tracheal agenesis; tracheoesophageal fistula; newborn.

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