International Journal of Pediatric Otorhinolaryngology 78 (2014) 1348–1351

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Tracheo-innominate artery fistula with severe motor and intellectual

disability: Incidence and therapeutic management
Makoto Kurose a , Kenichi Takano a, *, Hiroaki Mitsuzawa b , Tetsuo Himi a
a
Department of Otolaryngology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8556, Japan
b
Department of Otolaryngology, Hokkaido Medical Center for Child Health andRehabilitation, Japan

A R T I C L E I N F O A B S T R A C T

Article history: Objective: Tracheo-innominate artery fistula (TIF) is a rare but life-threatening complication following
Received 13 March 2014 tracheostomy or tracheoesophageal diversion (TED). Although successful surgical intervention for TIF has
Received in revised form 18 May 2014 been reported, few studies have been performed in patients with severe motor and intellectual disability
Accepted 20 May 2014
(SMID). Therefore, we aimed to analyze TIF in patients with SMID to clarify the clinical variables
Available online 2 June 2014
predicting the occurrence and adequate management for lifesaving of TIF.
Methods: We retrospectively reviewed the records of patients with SMID undergoing surgical
Keywords:
tracheostomy and TED between 2006 and 2012 and identified those with TIF. When TIF occurred, we
Tracheo-innominate artery fistula
Tracheostomy
obtained the clinical status and emergency management.
Tracheoesophageal diversion Results: Of 70 patients who underwent tracheostomy or TED during the study period, three patients had
Severe motor and intellectual disability TIFs; in one case, TIF was avoided by ligation of the innominate artery before TED. The incidence of TIF in
those undergoing tracheostomy and TED was 2.3% and 7.4%, respectively. The interval between
tracheostomy and TIF was 14–50 months.
Conclusions: Patients with SMID may have an increased risk of TIF. Prompt diagnosis and surgical
intervention to control the bleeding is the only effective management at present.
ã 2014 Elsevier Ireland Ltd. All rights reserved.

1. Introduction the definitive therapeutic procedure when managing their

Tracheo-innominate artery fistula (TIF) is an unusual but
fatal complication of tracheostomy occurring when various
factors cause an erosion through the tracheal wall into the
adjacent arterial wall. Although its incidence is low, varying
from 0.3% to 0.8% [1], patients with TIF rapidly deteriorate
because of massive tracheal hemorrhage and asphyxiation from
blood aspiration. Consequently, the survival rate is low at 7–25%
[2,3].
TIF is generally considered to be an acute complication because
most occur within the first three weeks of a tracheostomy.
However, because of improvements in post-tracheostomy man-
agement, TIF can now occur years after the primary surgery and
represents a feared late complication [4]. Patients with severe
motor and intellectual disability (SMID), which is a heterogeneous
group of disorders involving severe physical disability and
profound mental retardation [5], frequently have a variety of
respiratory complications. Therefore, tracheostomy is considered
* Corresponding author. Tel.: +81 116112111; fax: +81 116155405.
E-mail address: kent@sapmed.ac.jp (K. Takano).
respiratory problems. In addition, patients with SMID often have
anomalies of the larynx, trachea, and esophagus, which increase
the frequency of TIF compared with other groups [6,7]. In this
study, we retrospectively reviewed our records to identify patients
with SMID and TIF to provide a basis for discussing TIF as a late
complication of tracheostomy or tracheoesophageal diversion
(TED) in patients with SMID.
2. Materials and methods
We identified patients with SMID undergoing surgical trache-
ostomy and TED between 2006 and 2012 at the Department of
Otolaryngology, Hokkaido Medical Center for Child Health and
Rehabilitation, Sapporo, Japan. A retrospective review of the
medical records was performed on all patients with SMID who had
long-term tracheostomy tubes. We subsequently evaluated the
clinical features of those patients, including the sternocervical
spine distance (SCD), and reviewed their emergency and thera-
peutic management at the occurrence of TIF.
The present study was conducted according to the Declaration
of Helsinki and approved by the Ethics Committee of Hokkaido
Medical Center for Children Health and Rehabilitation and Sapporo

http://dx.doi.org/10.1016/j.ijporl.2014.05.027
0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
 M. Kurose et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1348–1351 1349

Table 1
Patient characteristics.

Tracheostomy Tracheo-esophageal diversion

Patients, No. 43 27
Gender (M:F) 22:21 16:11
Median age 1 year 11 months 4 years 4 months
Mean age 5 years 6 months 7 years 6 months
TIF (%) 1 (2.3) 2 (7.4)

Medical University. Informed consent was obtained from all

patients before surgery.

3. Results

3.1. Descriptive statistics

A total of 70 patients with SMID and long-term tracheostomy

tubes were identified (Table 1). All patients were bedridden and
had severe mental retardation and spastic tetraplegia. Tracheos-
tomy and TED were performed in 43 (22 male, 21 female) and 27
(16 male, 11 female) patients, respectively; the corresponding
median ages were 1 year 11 months and 4 years 4 months. We
identified three patients for whom TIF was visually confirmed and
one patient for whom TIF was avoided by ligation of the
innominate artery before TED. The incidences of TIF in the
tracheostomy and TED groups were 2.3% and 7.4%, respectively.
Table 2 summarizes the data obtained from patients with TIF.
Primary diseases were neonatal asphyxia in two patients and
meningitis in one patient. Tracheostomy or TED were performed at
the ages of 6 years and 5 months, 6 years and 4 months, and 12
years and 1 month. TIF subsequently occurred at the ages of 9 years
and 3 months, 11 years and 2 months, and 13 years and 3 months,
respectively. The interval between tracheostomy and TIF was in the
range 14–50 months.
3.2. Case reviews
Fig. 1. A computed tomography scan from the neck to the chest showed the tip of
tracheostomy tube (arrow) was juxtaposed with the innominate artery (arrow
head).
3.2.2. Patient 2
At the age of 6 years 4 months, this female patient underwent
TED for the treatment of intractable aspiration pneumonia. The
goal was to direct aspirated saliva/gastric juice into the cervical
esophagus via an end-to-side tracheoesophageal anastomosis, as
reported by Lindeman [8]. The procedure provided good control for
aspiration. When TIF occurred, her CT scan suggested that the
innominate artery was situated above(Fig. 2). Therefore, we made a
suprasternal incision as described in patient 1, contrary to the
standard procedure. We identified the trachea and the overriding
innominate artery and successfully ligated the artery proximally
and distally to the trachea. The postoperative course was
uneventful with no sign of further bleeding, new-onset neurologi-
cal symptoms, or blood pressure reductions of the right upper arm.

3.2.1. Patient 1 3.2.3. Patient 3

In patient 1 who had an episode of massive acute hemoptysis,
we identified a large amount of bleeding in the larynx and trachea.
A computed tomography (CT) scan of the chest revealed that the tip
of the tracheostomy tube was juxtaposed with the innominate
artery (Fig. 1). To secure the airway, we advanced the intubation
tube deeper and commenced mechanical ventilation. We subse-
quently consulted the thoracic surgery team for ligation of the
innominate artery under thoracotomy. However, because of a large
quantity of blood and associated respiratory dysfunction, we
abandoned the thoracotomy and made a suprasternal incision.
Although the innominate artery was visualized, the proximal
portion of the artery could not be identified in surgical field.
Unfortunately, the patient exsanguinated and could not be
resuscitated. She expired during the surgery.
Approximately 10 months after his birth, this infant contracted
meningitis; he subsequently required TED at the age of 12 years
and 1 month to manage chronic pulmonary aspiration. When TIF
occurred, a CT scan showed that the innominate artery was
situated below the sternal notch. In addition, his systemic
condition was severe because a large quantity of blood flowed
into the lung; therefore, ligation of the artery under thoracotomy
was impossible and endovascular coiling was selected. After
securing the airway and temporarily controlling the bleeding,
emergency coil embolization was performed using a percutaneous
approach through the right common femoral artery. An angiogram
obtained during cuff inflation showed an intact innominate artery.
A total of five coils were placed in the innominate artery, and a
subsequent angiogram demonstrated successful occlusion (Fig. 3).

Table 2
Findings related to tracheo-innominate artery fistulas.

Case No. Primary disease Age of tracheostomy or Age of TIF Interval Management Prognosis SCD (mm)
TED
1 Neonatal 6 years 5 months 9 years 3 months 2 years 10 Ligation of the innominate artery Death 9.5
asphyxia months
2 Neonatal 6 years 4 months 11 years 2 4 years 10 Ligation of the innominate artery Alive 18.9
asphyxia months months
3 Meningitis 12 years 1 month 13 years 3 1 years 2 months Endovascular embolization Alive 9.9
months
Avoided West syndrome 4 years 4 months – – Ligation of the innominate artery before Alive 15.6
TIF TED
1350 M. Kurose et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1348–1351
Fig. 2. Three-dimensional computed tomography image demonstrating that the
innominate artery was situated above the sternal notch.
After surgery, there were no complications; bleeding did not recur
and there was no blocking of blood flow.
3.2.4. The Patient who avoided TIF
We experienced a single case in which we avoided TIF by
ligation of the innominate artery before undergoing TED. As shown
in Fig. 4, preoperative CT findings showed that the innominate
artery strongly compressed the trachea, and the risk of TIF by TED
was considered to be high. Therefore, we ligated the innominate
artery under thoracotomy, and the patient was underwent TED
four months later.
4. Discussion
TIF is perhaps the most feared complication of both tracheos-
tomy and TED [9], with an overall incidence of 0.3–0.8% following
tracheostomy [1]. However, the rate of TIF in children undergoing
tracheostomy has been reported to be 0.4–0.5% [10,11], with a
higher rate in patients with SMID (4.0–6.2% [6,7]). In the present
study, TIF occurred in 3 of 70 (4.3%) patients who underwent these
procedures; therefore, SMID should be considered as a risk factor
for TIF. General risk factors for TIF include excessive movement of
the tracheostomy, the use of a high-pressure cuff, and tube
Fig. 3. (A) An angiogram of the innominate artery showed the tip of the tracheostomy tube compressing the innominate artery (arrow). (B) The innominate artery was
successfully embolized using coils (arrow head).
Fig. 4. A computed tomography image before tracheoesophageal diversion
indicated that the innominate artery strongly compressed the trachea.
placement that is extremely low [4]. Patients with SMID have
additional risk factors, including the need for long-term tracheos-
tomy tubes and the presence of deformations of the trunk or
thorax. Although TIF usually occurs during the first three weeks
after tracheostomy [3], all cases in this study occurred over a year
after the initial procedure, which implies that it can present as a
late complication. This may represent a shift in presentation due to
improved management of TIF risk factors such as low positioning
of the stoma, pressure necrosis, and infections.
It has been reported that if the distance between the posterior
surface of the sternum and the anterior surface of the cervical
spine (the SCD; Fig. 5) was shorter than 2 cm in length, the risk of
TIF was higher [12]. In each of our patients, SCD was shorter than
2 cm; in patients 1 and 3, SCDs were extremely short at 9.5 mm
and 9.9 mm, respectively. However, we examined SCD in the other
patients with SMID who did not develop TIF, and identified that
the SCD in these patients was typically shorter than 2 cm.
 M. Kurose et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1348–1351
Fig. 5. The sterno-cervical spine distance was defined as the distance between the
posterior surface of the sternum and the anterior surface of the cervical spine.
Therefore, patients with SMID should be considered to have a
high risk for TIF.
At our institution, surgical tracheostomy is performed by a
simply vertical slit made across two tracheal rings between stay
sutures that are passed through the tracheal wall on either side of
the midline. This technique is commonly used by pediatric
otolaryngologists, and we do not consider the procedure itself
as being responsible for TIF.
Prompt diagnosis and surgical control of bleeding is the only
recognized management for TIF. Without surgical management,
the mortality is almost 100% following acute massive tracheal
hemorrhage [13]. Therefore, the possibility of TIF must always be
considered if local hemorrhage occurs in a patient with tracheos-
tomy. A previous report proposed the following three emergency
steps to control bleeding before definitive surgery [14]: (1) flexible
fibroscopy through the tracheostomy to clear and secure the
airway, (2) over-inflate the tracheostomy tube cuff, and (3) the
application of digital compression around the tracheostomy.
Another study recommended avoiding fibroscopy (step 1) in
suspected TIF because it can precipitate fatal bleeding [6].
Various methods have been reported to manage the involved
artery, including ligation of innominate artery, direct suture with
resection, graft interposition [15], endovascular stent grafting [16],
and endovascular embolization [17]. Most authors agree that
ligation of the innominate artery is the treatment of choice, and we
agree that this procedure is recommended in patients with or
without SMID. A suprasternal neck approach is a quick and
minimally invasive surgical procedure; however, this approach is
limited with a high-lying innominate artery, as in patient 2 in this
report. Although median sternotomy is often chose first, this
1351
approach is highly invasive with a high risk of surgical
complications. The endovascular approach that we used in patient
3 is only available at limited institutions; however, it represents a
new effective therapeutic tool for TIF. Flow interruption of the
innominate artery in not expected to yield significant neurological
or vascular complications because collateral blood flow develops
through the external carotid artery, thyrocervical trunk, and
vertebral vessels [3,18]. In our study, there were no neurological
complications after either ligation or embolization, and long-term
survival was observed in all patients.
5. Conclusion
When bleeding occurs from a TIF, prompt surgery aimed at
interrupting the innominate artery is necessary once temporary
hemostasis is secured. In this report, all patients with SMID who
underwent tracheostomy or TED were at high risk of TIF.
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