BioMed CentralJournal of Medical Case Reports
ss
Open AcceCase report
Tracheal agenesis as a rare cause of difficult intubation in a newborn
with respiratory distress: a case report
Raja Ahmad*1, Kahairi Abdullah1, Lukman Mokhtar2 and Ahmad Fadzil3
Address: 1Department of Otolaryngology - Head & Neck Surgery, Faculty of Medicine, International Islamic University Malaysia, Kuantan 25200,
Malaysia, 2Department of Anaesthesiology and Intensive Care, Faculty of Medicine, International Islamic University Malaysia, Kuantan 25200,
Malaysia and 3Department of Pediatric, Tengku Ampuan Afzan Hospital, Kuantan 25710, Malaysia
Email: Raja Ahmad* - XXXXXXXXXX; Kahairi Abdullah - XXXXXXXXXX; Lukman Mokhtar - XXXXXXXXXX;
Ahmad Fadzil - XXXXXXXXXX
* Co
esponding author
Abstract
Introduction: Tracheal agenesis is a very rare congenital airway anomaly. It may pose a great
challenge to the first attending physician both in diagnosis and in establishing the airway during the
first day of life.
Case presentation: We report a newborn Malay baby boy with trachea agenesis (type III by
Floyd's classification) who presented with severe respiratory distress immediately after birth.
Clinical diagnosis in this case was not straightforward, as it started with difficulty in intubation
followed by an unsuccessful emergency tracheostomy in the neonatal intensive care unit. Urgent
surgical neck exploration with endoscopic examination in the general operating theatre revealed
the final diagnosis. The authors present a short description of the em
yopathology and diagnostic
criteria of the abnormality.
Conclusion: We hope this case presentation will be valuable in increasing the awareness of
physicians about this rare cause of tracheal obstruction or difficult intubation.
Introduction
Tracheal agenesis is a rare congenital airway anomaly.
There were 116 cases of tracheal agenesis reported in the
literature between 1900 and (September) 2004 [1]. Air-
way management in this abnormality poses a great chal-
lenge to otolaryngologists, anaesthetists and
pediatricians. This condition is incompatible with life. At
present there is no specific surgical management tech-
nique that is associated with survival of tracheal agenesis.
The newborn with tracheal agenesis usually presents with
immediate and severe respiratory distress, and the only
way to provide ventilation is through the esophagus. Per-
forming endotracheal intubation or tracheostomy does
not normally help. Bag-mask ventilation and intubation
of the esophagus may allow ventilation of the lungs. Neck
exploration during the tracheostomy and endoscopic
evaluation will establish the diagnosis. The ex-utero intra-
partum treatment (EXIT) procedure is an excellent
method developed for use in anticipation of possible air-
way compromises in newborn babies at birth.
Case presentation
A 2.4 kg Malay baby boy was delivered after 37 weeks of
gestation by spontaneous vaginal delivery. The pregnancy
was complicated by polyhydramnios. The baby developed
Published: 4 November 2009
Journal of Medical Case Reports 2009, 3:105 doi:10.1186/ XXXXXXXXXX
Received: 27 October 2009
Accepted: 4 November 2009
This article is available from: http:
www.jmedicalcasereports.com/content/3/1/105
© 2009 Ahmad et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http:
creativecommons.org/licenses
y/2.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Page 1 of 3
(page number not for citation purposes)
http:
www.jmedicalcasereports.com/content/3/1/105
http:
creativecommons.org/licenses
y/2.0
http:
www.biomedcentral.com
http:
www.biomedcentral.com/info/about/charte
Journal of Medical Case Reports 2009, 3:105 http:
www.jmedicalcasereports.com/content/3/1/105
immediate respiratory distress at birth with an Apgar score
of 1 at 1 min, 5 at 5 min, and 6 at 10 minutes of life. He
was initially resuscitated with bag-mask ventilation, and
subsequently transfe
ed to the neonatal intensive care
unit (NICU). Multiple oral endotracheal intubations were
attempted in the NICU with no success. Bag-mask ventila-
tion was continued and an otolaryngologist was con-
sulted for emergency tracheostomy. Oxygen saturation
was successfully maintained at above 85% with bag-mask
ventilation. It was possible to pass a Ryle's tube through
oth nostrils.
During the surgical procedure, the trachea could not be
identified. The baby was transfe
ed to the operating the-
atre for neck exploration, and a complete endoscopic
examination was performed to evaluate airway patency.
During the neck exploration, it was noted that the larynx
ended blindly at the cricoid level (Figure 1), while the tra-
chea was absent. A laryngoscopic evaluation without mus-
cle relaxant disclosed a cleft larynx with bilateral
immobile vocal cords (Figure 2). A
onchoscope could
not be passed below the vocal cords. An esophagoscopy
was performed which revealed two openings at the distal
portion of the esophagus, which communicated with the
left and right
onchus, respectively (Figure 3).
At the end of the surgical procedure, an endotracheal tube
was inserted into the esophagus and effective ventilation
confirmed by visualization of normal chest expansion
and good oxygen saturation. The baby also had persistent
ductus arteriosus, dysplasia of the right radius and the
ight thumb, and an imperforated anus. A diagnosis of tra-
cheal agenesis was made and the family members were
counseled about the grim prognosis. Life support was dis-
continued with the agreement of the parents, and the
aby was allowed to die.
Discussion
At present, there is no specific surgical management that
allows survival in cases of tracheal agenesis. Normally, a
newborn with tracheal agenesis presents with immediate
espiratory distress and an absent or very weak cry. This
are congenital anomaly might confound the attending
doctor in the delivery room or operating theatre.
Neck exploration revealed a normal larynx that ended in a blind pouch at the level of cricoid (black a
ow)Figure 1
Neck exploration revealed a normal larynx that
ended in a blind pouch at the level of cricoid (black
a
ow). The dome-shaped cricoid occludes the lumen.
Laryngoscopy examination revealed a cleft larynx with immo-bile vocal cords bilaterally in midline positionFigure 2
Laryngoscopy examination revealed a cleft larynx
with immobile vocal cords bilaterally in midline posi-
tion.
Esophagoscopy view showing one of the openings into the left
onchus at the distal end of the esophagusFigure 3
Esophagoscopy view showing one of the openings
into the left
onchus at the distal end of the esopha-
gus.
Page 2 of 3
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:105 http:
www.jmedicalcasereports.com/content/3/1/105
The em
yopathology resulting in this abnormality
occurs during the first eight weeks of gestation. The tra-
cheo-pulmonary complex develops from the respiratory
diverticulum at the ventral aspect of the primitive foregut.
A compromised vascular supply to the developing trachea
during this stage may cause tracheal agenesis or tracheal
stenosis with complete tracheal ring [2]. According to
Merei et al [3], the point of bifurcation between the devel-
oping trachea at ventral and developing esophagus at dor-
sal foregut remains fixed in relation to the cervical
verte
a. Caudally, the respiratory diverticulum will
develop into the carina and
oncho-pulmonary tree. The
cephalic aspect of the respiratory diverticulum will be
elongated to form the trachea and the infra-glottic struc-
ture. Tracheal agenesis results when this normal elonga-
tion process fails to take place [1]. This anomaly is
associated with relatively normal supra-glottic structures
and pulmonary development, as seen in this case. The
congenital abnormality is only limited to the region of the
developing trachea. The severity of tracheal agenesis was
described in detail by Floyd and colleagues and classified
into three types [4]. In Type I, a short segment of the tra-
chea fails to elongate to fuse with the larynx. In Type II,
the respiratory diverticulum fuses in midline to form the
carina but tracheal elongation does not take place. And in
type III, the respiratory diverticula does not fuse in the
midline, resulting in two fistula opening at the lower part
of esophagus. The present case is compatible with a type
III abnormality. Type II remains the most common abnor-
mality (61%), followed by type III (23%) and type I
(11%) [1].
Tracheal agenesis is commonly associated with other con-
genital anomalies such as verte
al defects, anal atresia,
tracheoesophageal fistula, esophageal atresia, cardiovas-
cular defects, limb defects, duodenal atresia and renal
defects. Tracheal agenesis can be a manifestation of sev-
eral syndromes such as VATER (verte
ae, anus, trachea,
esophagus, and renal), also known as VACTERL, and
TARCD (total alkaloids from rhizoma corydalis decum-
eutis).
A high index of suspicion is required to diagnose tracheal
atresia. Antepartum features that would co
oborate such
suspicion are the presence of polyhydramnios with multi-
ple fetal anomalies. During birth, the baby may not cry o
may have a weak cry. An acute severe respiratory distress
develops and multiple attempts at intubations fail. Laryn-
goscopy will reveal immobile vocal cords lying in the mid-
line position. Other findings are a cleft between the
arytenoids, as well as associated congenital anomalies.
Good oxygenation may be maintained with bag-mask
ventilation or esophageal intubation. The diagnosis is
made through neck exploration during emergency trache-
ostomy and an endoscopic evaluation of the larynx and
esophagus. A pre-delivery procedure with three-dimen-
sional ultrasound or fetal magnetic resonance imaging
allows a complete evaluation of this upper airway abnor-
mality. The ex-utero intrapartum treatment (EXIT) proce-
dure can be planned based on the imaging results. EXIT
procedure can reduce the risk of respiratory distress imme-
diately after birth [5].
Conclusion
Tracheal agenesis should be suspected in a newborn baby
who presents with immediate respiratory distress, as well
as extremely weak cry and failed intubation despite ade-
quate ventilation with facemask. The establishment of air-
way after an insertion of endotracheal tube in the
oesophagus will further enhance the index of suspicion
efore the definitive endoscopic evaluation.
Consent
Written informed consent could not be obtained in this
case since the patient is now lost to follow-up. We believe
that this case report contains a worthwhile clinical lesson
which could not be made as effectively in any other way.
We expect that the patient and her family would not
object to the publication since every effort has been made
so that she remains anonymous.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
All authors of this paper have participated directly in the
planning, execution, or analysis of this report, and have
ead and approved the final version submitted.
References
1. Lander TA, Schauer G, Bendel-Stenzel E, Sidman JD: Tracheal agen-
esis in newborns. Laryngoscope 2004, 114: XXXXXXXXXX.
2. Holinger LD, Lusk RP, Green CG: Pediatric Larngology Bronchoe-
sophagology Volume 6. Philadelphia: Lippincott-Raven Publishers; 1997.
3. Merei JM, Hutson JM: Em
yogenesis of tracheoesophageal
anomalies: a review. Pediatr Surg Int 2002, 18:319-326.
4. Floyd J, Campbell DC, Dominy DE: Agenesis of the trachea. Am
Rev Respir Dis 1962, 86:557-560.
5. Peng SS, Hsu WC, Chou HC, Peng SS, Chen LK, Chang YL, Hsieh FJ:
Prenatal three-dimensional ultrasound and magnetic reso-
nance imaging evaluation of a fetal oral tumor in preparation
for the ex utero intrapartum treatment (EXIT) procedure.
Ultrasound Review of Obstetrics and Gynecology 2005, 1(25):76.
Page 3 of 3
(page number not for citation purposes)
http:
www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids= XXXXXXXXXX
http:
www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids= XXXXXXXXXX
http:
www.ncbi