Tracheal agenesis

A rare lethal congenital abnormality characterized by complete interruption or absence of the trachea. The affected male to female ratio is 2:1 and half of cases are associated with POLYHYDRAMNIOS or premature delivery.

Also Known As:

Networked: 29 relevant articles (1 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tracheomalacia
2.	Tracheobronchomalacia
3.	Scimitar Syndrome
4.	Respiratory Insufficiency (Respiratory Failure)
5.	Fistula

Experts

1.	Kawahara, Hisayoshi: 2 articles (12/2015 - 10/2008)
2.	Kubota, Akio: 2 articles (12/2015 - 10/2008)
3.	Okuyama, Hiroomi: 2 articles (12/2015 - 10/2008)
4.	Saka, Ryuta: 2 articles (12/2015 - 10/2008)
5.	Ueno, Takehisa: 2 articles (12/2015 - 10/2008)
6.	Beasley, S W: 2 articles (01/2001 - 01/2001)
7.	Qi, B Q: 2 articles (01/2001 - 01/2001)
8.	Engelhardt, Thomas: 1 article (02/2022)
9.	Johansen, Mathias: 1 article (02/2022)
10.	Veyckemans, Francis: 1 article (02/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Tracheal agenesis:

1.	Doxorubicin (Adriamycin)FDA LinkGeneric 01/01/1999 - "Pathohistological study of adriamycin-induced tracheal agenesis in the fetal rat." 05/01/2007 - "The adriamycin rat model (ARM) has proved to be a valuable model of the VACTERL anomalies, illustrating the dysmorphogenesis of oesophageal atresia and tracheal agenesis. " 01/01/2001 - "Exposure to adriamycin resulted in a range of anatomical defects including OA/TOF (47%) and tracheal agenesis (TA) (41%). " 01/01/2001 - "The relationship of the Nt to the Fg mesenchyme determines the severity of the abnormality induced by adriamycin: extensive adherence produces tracheal agenesis and EA."
2.	Anesthetics (Anesthetic Agents)IBA 12/01/2017 - "We describe the anesthetic considerations for a patient with tracheal agenesis undergoing reconstruction." 12/01/2017 - "Anesthetic Management of an Infant With Postnatally Diagnosed Tracheal Agenesis Undergoing Tracheal Reconstruction: A Case Report." 02/01/2022 - "This educational review will focus on the pathophysiology, clinical manifestations, surgical approaches, and anesthetic management of congenital anomalies of the large intrathoracic airways: congenital tracheal stenosis, tracheal agenesis, tracheal diverticulum, bronchial anomalies (tracheal, esophageal, or bridging bronchus), congenital lung malformations, lung sequestrations and Scimitar syndrome, lobar emphysema, Williams-Campbell syndrome, and pleuropulmonary blastoma. "
3.	Type I Bone Morphogenetic Protein ReceptorsIBA 03/01/2011 - "Here, we show that conditional inactivation of BMP type I receptor genes Bmpr1a and Bmpr1b (Bmpr1a;b) in the ventral endoderm leads to tracheal agenesis and ectopic primary bronchi. "
4.	Surface-Active Agents (Surfactants)IBA 12/01/2001 - "The authors studied surfactant protein expression and ultrastructure of pulmonary epithelial cells in fetal hyperplastic lungs associated with congenital tracheal agenesis. "
5.	Proteins (Proteins, Gene)FDA Link 12/01/2001 - "The authors studied surfactant protein expression and ultrastructure of pulmonary epithelial cells in fetal hyperplastic lungs associated with congenital tracheal agenesis. "
6.	Midazolam (Versed)FDA LinkGeneric 09/01/2010 - "We describe the case of an infant affected by a tracheal agenesis who underwent a series of operative and diagnostic bronchoscopies for which she received midazolam and fentanyl. "
7.	Fentanyl (Sublimaze)FDA LinkGeneric 09/01/2010 - "We describe the case of an infant affected by a tracheal agenesis who underwent a series of operative and diagnostic bronchoscopies for which she received midazolam and fentanyl. "
8.	mesoglycanIBA 10/01/2017 - "Using the keywords "tracheal atresia", "tracheal agenesis" and "tracheal hypoplasia" a search through Embase and Pubmed databases was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. "

Therapies and Procedures

1.	Mechanical Ventilators (Ventilator) 01/01/2023 - "We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age.The" 01/01/2023 - "We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age. " 12/01/2015 - "None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. "
2.	Tracheostomy 01/01/2012 - "Esophageal endoscopy and CT scan were performed postoperatively and their findings revealed Floyd's type II tracheal agenesis in which tracheostomy was impossible. " 08/01/1990 - "Infants having type I tracheal agenesis may benefit from immediate tracheostomy." 08/01/2005 - "25 patients presenting during the same period for other tracheal pathologies including esophagotracheal cleft syndrome (n = 7), tracheomalacia (n = 4), total tracheal agenesis (n = 3), or for placement of a tracheostomy (n = 11) due to other diseases were excluded from this study. " 11/01/2002 - "The prognosis in tracheal agenesis is universally fatal but cases with laryngeal atresia and subglottic stenosis may be saved with prompt tracheostomy and later surgical reconstruction."
3.	Airway Management 01/01/2018 - "We describe a procedure of airway management applied in two newborns with suspected tracheal agenesis. " 01/01/2017 - "Two case reports of unexpected tracheal agenesis in the neonate: 3 C's beyond algorithms for difficult airway management." 07/01/2003 - "In this report, we describe the types of tracheal agenesis and discuss initial airway management." 01/01/2000 - "We present a case of complete tracheal agenesis without tracheo nor broncho-oesophageal fistula (type II by Floyd's classification) - the diagnosis of which was prenatally suspected - and discuss the important features of the airway management of this condition."
4.	Resuscitation 12/01/2010 - "Tracheal agenesis: a rare cause of unsuccessful tracheal intubation during resuscitation." 07/17/2009 - "Failed resuscitation of a newborn due to congenital tracheal agenesis: a case report." 02/01/1991 - "However, in most cases of tracheal agenesis with esophagotracheal communication primary resuscitation is possible by esophageal intubation and by application of continuous positive pressure ventilation. " 12/01/2006 - "Interesting information on tracheal agenesis was gathered, the differential diagnosis of respiratory insufficiency of the newborn is summarised and a modified algorithm of the current newborn resuscitation guidelines of the American Heart Association is presented."
5.	Therapeutics 10/01/2013 - "Tracheal agenesis with tracheoesophageal fistulae: fetal MRI diagnosis with confirmation by ultrasound during an ex utero intrapartum therapy (EXIT) delivery and postdelivery MRI." 04/01/1991 - "We think that tracheal agenesis, a rare cause of respiratory distress in the newborn, has no suitable therapy today. "