Skip to main content Accessibility help

Tracheal agenesis, a frightening scenario

  • H Mohammed (a1), K West (a1), J Bewick (a1) and M Wickstead (a1)



This paper discusses three cases of tracheal agenesis that presented within a six-week period to the Norfolk and Norwich University Hospital. By reviewing the available literature on tracheal agenesis, the report aims to outline a protocol for future prenatal and postnatal management.


A case series and a literature review.


Three cases of tracheal agenesis presented in the classical manner, with respiratory distress and unsuccessful intubation following delivery. A literature review confirmed that prenatal diagnosis requires future innovation; survival is rare and is predominately reliant on intubation of the oesophagus when a patent tracheoesophageal fistula is present. In most cases, tracheal agenesis represents part of the ‘VATER’ association: vertebral defects, anal atresia, tracheoesophageal fistula with oesophageal atresia, and radial or renal dysplasia. Complex, multiple-stage surgical procedures have been described; however, no survival to adolescence is documented.


There is a call for improved prenatal diagnosis to allow both adequate counselling of parents and preparation for multi-specialty management at delivery. In addition, these cases highlight the ongoing need for improved congenital anomaly data within the UK, with currently only 49 per cent of England's births being registered.


Corresponding author

Address for correspondence: Mr H Mohammed, ENT Department, Norfolk and Norwich University Hospital, Colney Lane, Norwich NR4 7UY, UK Fax: 01603 287211 E-mail:


Hide All
1Demircan, M, Aksoy, T, Ceran, C, Kafkasli, A. Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas. J Pediatr Surg 2008;43:e13
2Wei, JL, Rodeberg, D, Thompson, DM. Tracheal agenesis with anomalies found in both VACTERL and TACRD associations. Int J Pediatr Otorhinolaryngol 2003;67:1013–17
3Norfolk Insight. Demography Overview: 2014. In: [21 August 2015]
4Fuchimoto, Y, Mori, M, Takasato, F, Tomita, H, Yamamoto, Y, Shimojima, N et al. A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction. Pediatr Surg Int 2011;27:103–6
5Bachiocco, V, Mondardini, MC. Julia's placebo effect. Pain 2010;150:582–5
6Usui, N, Kamiyama, M, Tani, G, Takama, Y, Soh, H, Uehara, S et al. Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis. Ann Thorac Surg 2010;89:2019–22
7Bhattarai, B, Pratap, A, Yadav, R, Pokharel, K. Anaesthetic management of tracheal agenesis. JNMA J Nepal Med Assoc 2008;47:142–4
8Baroncini-Cornea, S, Fae, M, Gargiulo, G, Gentili, A, Lima, M, Pigna, A et al. Tracheal agenesis: management of the first 10 months of life. Paediatr Anaesth 2004;14:774–7
9Soh, H, Kawahawa, H, Imura, K, Yagi, M, Yoneda, A, Kubota, A et al. Tracheal agenesis in a child who survived for 6 years. J Pediatr Surg 1999;34:1541–3
10Hiyama, E, Yokoyama, T, Ichikawa, T, Matsuura, Y. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830–3
11Sankaran, K, Bhagirath, CP, Bingham, WT, Hjertaas, R, Haight, K. Tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula: report of two cases and review of literature. Pediatrics 1983;71:821–3
12Floyd, J, Campbell, DC Jr, Dominy, DE. Agenesis of the trachea. Am Rev Respir Dis 1962;86:557–60
13Que, J, Choi, M, Ziel, JW, Klingensmith, J, Hogan, BL. Morphogenesis of the trachea and esophagus: current players and new roles for noggin and Bmps. Differentiation 2006;74:422–37
14Jurado, S, Smyth, I, van Denderen, B, Tenis, N, Hammet, A, Hewitt, K et al. Dual functions of ASCIZ in the DNA base damage response and pulmonary organogenesis. PLoS Genet 2010;6:e1001170
15De Luca, D, De Carolis, MP, Capelli, A, Gallini, F, Draisci, G, Pinto, R et al. Tracheal agenesis without esophageal fistula: genetic, resuscitative, and pathological issues. J Pediatr Surg 2008;43:e2932
16Dijkman, KP, Andriessen, P, van Lijnschoten, G, Halbertsma, FJ. Failed resuscitation of a newborn due to congenital tracheal agenesis: a case report. Cases J 2009;2:7212
17Ahmad, R, Abdullah, K, Mokhtar, L, Fadzil, A. Tracheal agenesis as a rare cause of difficult intubation in a newborn with respiratory distress: a case report. J Med Case Rep 2009;3:105


Tracheal agenesis, a frightening scenario

  • H Mohammed (a1), K West (a1), J Bewick (a1) and M Wickstead (a1)


Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed