Tracheal Agenesis in Newborns

Lander, Timothy A.; Schauer, Galen M.; Bendel-Stenzel, Ellen; Sidman, James D.

doi:10.1097/00005537-200409000-00024

Cited by 24 publications

(18 citation statements)

References 13 publications

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“…This is the first reported case, to our knowledge, of a successful salvage at birth of a baby with CHAOS due to complete tracheal agenesis. Until presently, tracheal agenesis has not been prenatally diagnosed, which has prevented the option for the EXIT strategy [4][5][6] . However, this case demonstrates that if the correct diagnosis can be made and the pregnant mother can be transferred to an institution with appropriate experience, EXIT offers the potential for salvage of these babies [7][8][9] .…”

Section: Discussionmentioning

confidence: 99%

“…Ninety-four percent of the reported cases of tracheal agenesis have an associated tracheoesophageal fistula that would decompress into the tracheobronchial tree and prevent the development of CHAOS [4] . Infants with this condition survive the initial few hours of life by ventilating through a bronchoesophageal fistula [6,12] . Infants with tracheal agenesis or atresia and a tracheoesophageal fistula without CHAOS may also benefit from the EXIT procedure.…”

Section: Discussionmentioning

confidence: 99%

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Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Vaikunth¹,

Morris²,

Polzin

et al. 2009

Fetal Diagn Ther

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Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition characterized by complete blockage of the fetal airways associated with hydrops. We present a case of CHAOS due to the rare cause of complete tracheal agenesis. The ex utero intrapartum therapy (EXIT) strategy was employed to allow for neck and mediastinal exploration. Thymectomy allowed dissection to the level of the carina demonstrating the confluence of dilated mainstem bronchi but no trachea and no connection to the esophagus. A 2.5 endotracheal tube was inserted into the right mainstem bronchus and secured to the left clavicle. At 3 months of age, the infant succumbed to sepsis from Enterobacter mediastinitis due to friction between the tracheostomy tube and the nasogastric tube resulting in erosion of the esophagus. Complete tracheal agenesis, as seen in this case, is consistent with the failure of normal tracheal elongation as suggested by newer theories of foregut development. This case illustrates the most severe form of tracheal atresia causing CHAOS ever salvaged by the EXIT procedure at birth. The subsequent postnatal course highlights the need for early tracheal replacement in this particularly challenging form of CHAOS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Vaikunth¹,

Morris²,

Polzin

et al. 2009

Fetal Diagn Ther

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“…Tracheal agenesis represents a devastating congenital anomaly that typically presents with severe respiratory distress and an absent cry [1]. The incidence of this rare anomaly is reported to be less than 1:50,000 with a male predominance [2].…”

Section: Discussionmentioning

confidence: 99%

“…Type 2 occurs if the respiratory diverticula fuse to form the carina but tracheal elongation does not occur. If the respiratory diver- ticula fail to fuse, separate bronchoesopahgeal fistulae persist and type 3 tracheal agenesis results [1]. Various other abnormalities have been described in association with tracheal agenesis.…”

Section: Discussionmentioning

confidence: 99%

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Tracheal agenesis: Two case reports

Agarwal

John

Latta

et al. 2006

International Journal of Pediatric Otorhinolaryngology

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Characterization of a biologically derived rabbit tracheal scaffold

Lange

Shah

Birchall

et al. 2016

J. Biomed. Mater. Res.

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There is a clinical need to provide replacement tracheal tissue for the pediatric population affected by congenital defects, as current surgical solutions are not universally applicable. A potential solution is to use tissue engineered scaffold as the framework for regenerating autologous tissue. Rabbit trachea were used and different detergents (Triton x-100 and sodium deoxycholate) and enzymes (DNAse/RNAse) investigated to create a decellularization protocol. Each reagent was initially tested individually and the outcome used to design a combined protocol. At each stage the resultant scaffold was assessed histologically, molecularly for acellularity and matrix preservation. Immunogenicity of the final scaffold was assessed by implantation into a rat model for 4 weeks. Both enzymes and detergents were required to produce a completely acellular (DNA content 42.78 ng/mg) scaffold with preserved collagen and elastin however, GAG content were reduced (8.78 ± 1.35 vs. 5.5 ± 4.8). Following in vivo implantation the scaffold elicited minimal immune response and showed significant cellular infiltration and vasculogenesis. The luminal aspect of the implanted scaffold showed infiltration of host derived cells, which were positive for pan cytokeratin. It is possible to create biologically derived biocompatible scaffolds to address specific pediatric clinical problems. © 2016 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 105B: 2126-2135, 2017.

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Tracheal Agenesis in Newborns

Abstract: Although tracheal agenesis is rare, it may be more common than previously thought. The diagnosis is not straightforward, and the prognosis is grim. The embryology of the trachea and other foregut derivatives is closely related, and associated birth defects are common.

Cited by 24 publications

References 13 publications

Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Congenital High Airway Obstruction Syndrome due to Complete Tracheal Agenesis: An Accident of Nature with Clues for Tracheal Development and Lessons in Management

Tracheal agenesis: Two case reports

Characterization of a biologically derived rabbit tracheal scaffold

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