Hostname: page-component-745bb68f8f-mzp66 Total loading time: 0 Render date: 2025-01-11T12:11:25.601Z Has data issue: false hasContentIssue false
  • English
  • Français

Tracheal agenesis, a frightening scenario

Published online by Cambridge University Press:  07 January 2016

H Mohammed ,
K West ,
J Bewick  and
M Wickstead
H Mohammed*
Affiliation:
ENT Department, Norfolk and Norwich University Hospital, Norwich, UK
K West
Affiliation:
ENT Department, Norfolk and Norwich University Hospital, Norwich, UK
J Bewick
Affiliation:
ENT Department, Norfolk and Norwich University Hospital, Norwich, UK
M Wickstead
Affiliation:
ENT Department, Norfolk and Norwich University Hospital, Norwich, UK
*
Address for correspondence: Mr H Mohammed, ENT Department, Norfolk and Norwich University Hospital, Colney Lane, Norwich NR4 7UY, UK Fax: 01603 287211 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

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.

Methods:

A case series and a literature review.

Results:

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.

Conclusion:

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.

Keywords

TracheaAgenesisCongenital Defects
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 130 , Issue 3 , March 2016 , pp. 314 - 317
Copyright
Copyright © JLO (1984) Limited 2016 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Demircan, M, Aksoy, T, Ceran, C, Kafkasli, A. Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas. J Pediatr Surg 2008;43:e1–3CrossRefGoogle ScholarPubMed
2Wei, JL, Rodeberg, D, Thompson, DM. Tracheal agenesis with anomalies found in both VACTERL and TACRD associations. Int J Pediatr Otorhinolaryngol 2003;67:1013–17CrossRefGoogle ScholarPubMed
3Norfolk Insight. Demography Overview: 2014. In: http://www.norfolkinsight.org.uk/jsna/population [21 August 2015]Google Scholar
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;27:103–6CrossRefGoogle ScholarPubMed
5Bachiocco, V, Mondardini, MC. Julia's placebo effect. Pain 2010;150:582–5CrossRefGoogle ScholarPubMed
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;89:2019–22CrossRefGoogle Scholar
7Bhattarai, B, Pratap, A, Yadav, R, Pokharel, K. Anaesthetic management of tracheal agenesis. JNMA J Nepal Med Assoc 2008;47:142–4Google ScholarPubMed
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;14:774–7CrossRefGoogle ScholarPubMed
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;34:1541–3CrossRefGoogle Scholar
10Hiyama, E, Yokoyama, T, Ichikawa, T, Matsuura, Y. Surgical management of tracheal agenesis. J Thorac Cardiovasc Surg 1994;108:830–3CrossRefGoogle ScholarPubMed
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;71:821–3CrossRefGoogle ScholarPubMed
12Floyd, J, Campbell, DC Jr, Dominy, DE. Agenesis of the trachea. Am Rev Respir Dis 1962;86:557–60Google ScholarPubMed
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;74:422–37CrossRefGoogle ScholarPubMed
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:e1001170CrossRefGoogle ScholarPubMed
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:e29–32CrossRefGoogle ScholarPubMed
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;2:7212CrossRefGoogle ScholarPubMed
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;3:105CrossRefGoogle Scholar