Tracheal agenesis without esophageal fistula: genetic, resuscitative, and pathological issues

Luca, Danièle De; Carolis, Maria Pia De; Capelli, Arnaldo; Gallini, Francesca; Draisci, Gaetano; Pinto, R.; Arena, Vincenzo

doi:10.1016/j.jpedsurg.2007.10.004

Cited by 11 publications

(8 citation statements)

References 13 publications

(19 reference statements)

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“…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) .…”

Section: Resultsmentioning

confidence: 98%

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

et al. 2011

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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.

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Section: Resultsmentioning

confidence: 98%

“…In another case, CCAM was also suspected, but fetal MRI was not carried out because of a non-reassuring cardiotocogram. The child died shortly after birth [9]. All five prenatal diagnosed cases of TA had no TOF; therefore, they presented as a classic CHAOS.…”

Section: Resultsmentioning

confidence: 99%

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

et al. 2011

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“…In presence of an oesophageal-bronchial fistula the infant can be stabilized by bag mask-ventilation and oesophageal intubation or insertion of a laryngeal mask ([ 15 ] Vanzati). In some cases of tracheal agenesis, emergency tracheotomy has been performed, but the procedure is seldom and complex ([ 16 ] Krause, [ 17 ] De Luca). Overall, tracheal agenesis has a very poor prognosis, even though cases of surviving patients with experimental therapies have been reported in the literature ([ 18 ] Hiyama, [ 19 ] Soh, [ 20 ] Tazuke).…”

Section: Discussionmentioning

confidence: 99%

Two case reports of unexpected tracheal agenesis in the neonate: 3 C’s beyond algorithms for difficult airway management

et al. 2017

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BackgroundHandling neonates with postnatal respiratory failure due to congenital airway malformations implies knowledge about emergency management of unexpected difficult airway. In these stressful situations both technical and communication skills of the caretakers are essential.Case presentationTwo cases with prenatally unknown tracheal agenesis are reported.ConclusionIn the presented cases, airway malformation and subsequent difficulties upon endotracheal intubation were not adequately communicated between caretakers. We discuss the aspects of culture, communication, and capnography.

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“…1,2 Severe cases of congenital tracheal stenosis resulting in functional atresia of the trachea are frequently combined with other organ anomalies; they can present with or without esophageal fistula. Without a fistula these cases are usually not treatable 3 although novel in-utero treatments are currently being exploited to address the issue. 4,5 If initial emergent control of the airway is successful, definitive surgical correction has to be planned.…”

Section: Introductionmentioning

confidence: 99%

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 without esophageal fistula: genetic, resuscitative, and pathological issues

Cited by 11 publications

References 13 publications

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

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

Two case reports of unexpected tracheal agenesis in the neonate: 3 C’s beyond algorithms for difficult airway management

Characterization of a biologically derived rabbit tracheal scaffold

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