Nasopharyngeal Airway for Management of Airway Obstruction in Infants

With Micrognathia
Nooshin Parhizkar, M.D., Babette Saltzman, Ph.D., Kellyn Grote, Jacqueline Starr, Ph.D., Michael Cunningham,
M.D., Ph.D., Jonathan Perkins, D.O., Kathleen Sie, M.D.
Objective: Describe airway management using nasopharyngeal airway in
infants.
Design: Retrospective case series (1996 to 2006).
Setting: Tertiary pediatric hospital.
Patients, Participants: The craniofacial database of Seattle Childrens
Hospital was searched to identify patients with one of the following diagnoses:
micrognathia, secondary cleft palate, branchial arch anomalies, Pierre Robin
sequence (PRS), or velocardiofacial syndrome. Thirty-five (10.9%) of the 320
infants born between January 1, 1996, and March 31, 2006, identified using the
criteria listed above were managed with nasopharyngeal airway (NPA) during
infancy.
Interventions: Use of NPA.
Main Outcome Measure: Summary statistics describing the distribution of
the infants demographic characteristics, duration, and timing of their NPA
placement, need for tracheotomy, feeding interventions, and death.
Results: Of the 35 patients included in this case series, 60% (21) were male.
Eighteen (51.4%) patients had the diagnosis of PRS, 13 (37.1%) had secondary
cleft palate and other craniofacial anomalies, and four (11.4%) had branchial
anomalies and micrognathia (nonsyndromic or syndromic). Thirty-one children
(88.6%) were born at term. Mean and median age at initial NPA placement was
3.2 and 1.3 weeks, respectively; median duration of NPA was 8.0 weeks. Nine
children received tracheotomies. Feeding tubes were required in 85.7% of
patients. Two children died; however, neither death was attributed to airway
obstruction or the use of NPA.
Conclusions: NPA is one option in the management of patients with
craniofacial anomalies and airway obstruction. The majority of nonsyndromic
PRS patients treated with NPA during infancy did not require airway
intervention beyond NPA.
KEY WORDS: airway management, nasopharyngeal airway, Pierre Robin
sequence

Infants with craniofacial anomalies (CFA) may experience airway obstruction and feeding difficulties requiring
medical and surgical intervention. It has been reported that
up to 65% of this population will require some form of
airway intervention (Perkins et al., 1997). The range of
airway interventions includes positioning, nasopharyngeal
airway (NPA), endotracheal intubation, tongue lip adhesion, mandible distraction, and tracheotomy (Wagener et
al., 2003). The type of CFA may have an impact on causes
and treatments of airway obstruction.
This is an investigation of children with (1) secondary
cleft palate, with micrognathia or syndromic features; or (2)
branchial arch anomalies and syndromic or nonsyndromic
micrognathia.
Infants with these diagnoses are at risk for airway
obstruction, often related to the position of the tongue.
Airway management is directed at relieving the area of
obstruction. Nonsurgical management of mild-to-moderate

Dr. Parhizkar, Pediatric ENT DivisionChildrens Hospital & Research

Center Oakland, Oakland, California. Dr. Saltzman, Craniofacial Center,
Seattle Childrens Hospital, Seattle, Washington. Kellyn Grote, School of
Nursing, University of Washington, Seattle, Washington. Dr. Starr,
Craniofacial Center, Seattle Childrens Hospital, Departments of Pediatrics and Epidemiology, University of Washington, Seattle, Washington.
Dr. Cunningham, Craniofacial Center and Department of Pediatrics,
Seattle Childrens Hospital, Department of Pediatrics, University of
Washington, Seattle, Washington. Drs. Perkins and Sie, Division of
Pediatric Otolaryngology, Seattle Childrens Hospital, Department of
OtolaryngologyHead and Neck Surgery, University of Washington,
Seattle, Washington.
Presented in part at the American Society of Pediatric Otolaryngology,
May 2009.
Submitted April 2010; Accepted August 2010.
Address correspondence to: Dr. Kathleen Sie, Seattle Childrens
Hospital, Division of OtolaryngologyHead and Neck Surgery, 4800
Sand Point Way NE / Mailstop W-7729, Seattle, WA 98105-0371. E-mail
kathleen.sie@seattlechildrens.org.
DOI: 10.1597/09-263
478

Parhizkar et al., MANAGEMENT OF AIRWAY OBSTRUCTION WITH NASOPHARYNGEAL AIRWAY

obstruction includes positioning, oral airway, and/or the

NPA. Surgical measures, such as tongue lip adhesion and
early mandibular distraction, are also options for these
patients. There is some controversy about the most
appropriate management for infants with airway obstruction related to micrognathia. Although these measures can
be adequate as definitive management in some patients,
others will require further intervention such as mandibular
distraction osteogenesis and tracheotomy (Leighton and
Drake, 2004).
At the Seattle Childrens Hospital, NPA has been used to
manage patients with airway obstruction related to tongue
position (glossoptosis). The main goal of using NPA is to
offer a nonsurgical means of airway management that will
allow for the infant to be discharged home safely. NPA
involves placement of a modified endotracheal tube into
one nasal passage, with the tip positioned in the distal
oropharynx. Flexible nasopharyngoscopy through the tube
can be performed to visualize the position of the distal tip
of the NPA above the level of the glottis or distal
oropharynx.
The parents are trained to maintain patency of the tube
with application of saline drops and suction and to replace
the tube; this training on behalf of the parents allows for
outpatient management.
The infants feeding status is monitored as part of the
assessment of the NPA. Once an NPA is placed, the infant
is allowed to feed by mouth. If the infant requires
nutritional supplementation, a feeding tube is placed in
the contralateral nasal passage. Once adequate training,
family comfort, feeding status, and community resources
are established, the patient is discharged home for the
duration of the airway intervention.
This is a retrospective review of airway management
using NPA in infants with craniofacial anomalies resulting
in upper airway obstruction. Our protocol for the use of the
NPA to stabilize the airway in children with CFA is
presented. We present this series to demonstrate the
feasibility of NPA for inpatient and outpatient management of infants with micrognathia and airway obstruction.
METHODS
The Seattle Childrens Hospital Craniofacial Center
database was queried to identify children with (1)
secondary cleft palate with micrognathia (Pierre Robin
sequence) or other syndromic features and (2) branchial
arch anomalies (with micrognathia or with other cranial
abnormalities) whose birth dates fell between January 1,
1996, and March 31, 2006, and who were treated during the
period of 1996 to 2006. This study received approval of the
institutional review board. Three hundred twenty subjects
were identified. Their medical records were reviewed for
sex, gestational age, patient diagnosis, age at time of NPA
placement and removal, need for tracheotomy and feeding
tube placements, and morbidity and mortality.

479

Forty of the 320 children had NPAs placed; 35 had their

initial NPA placed before 15 weeks of age, and five children
had their first NPA after 15 weeks of age. The five subjects
who had their initial NPA placed after 15 weeks of age were
excluded because their indication for placement appeared
to be different than the younger children; specifically, these
patients had NPA for postsurgical airway management as
opposed to general airway management.
The procedure to create and secure the NPA was stable
throughout the duration of the study period. The patients
underwent flexible fiberoptic nasopharyngoscopy at the
bedside to determine the site of airway obstruction. With
endoscopic confirmation of glossoptosis in the infant with
airway obstruction, NPA was recommended.
A modified endotracheal tube was used as the NPA. The
proximal end of the endotracheal tube was trimmed so that
the distal end of the tube would be positioned in the distal
oropharynx, beyond the area of glossoptosis. This position
was verified by placing the endoscope within the endotracheal tube and directly visualizing the position of the tube.
The length of the tube was documented in the medical
record and the tube secured. This process did not require
anesthesia.
The modified endotracheal tube was then secured to the
cheeks. A small safety pin was placed through the
endotracheal tube with care to minimize obstruction of
the lumen of the tube to allow for suctioning. Small-caliber
tracheotomy ties were placed through both ends of the
safety pin. These ends were covered with tape to prevent
inadvertent opening of the safety pin. DuoDERM was
placed on the cheeks to protect the skin. The ties were
secured to the DuoDERM with a clear adhesive dressing.
In this manner, the ties could be positioned to avoid
pressure on the nasal rim.
Families were trained to place saline drops in the NPA
and suction as needed prior to discharge. The appropriate
depth of suction corresponded to the length of the tube.
Arrangements were made for the families to have suction
and oxygen available at home. The families were also
trained to change the NPA and were advised to change
sides, if possible, once every week. Prefabricated tubes were
provided to the families.
Timing of NPA removal was based upon clinical
assessment of the infant. Infants with NPA were generally
seen in the Craniofacial Center once or twice a month.
Parents were often able to provide a history suggesting that
the infant had fewer airway symptoms while the NPA was
out for the routine tube change. Alternatively, the
physicians seeing the patient in clinic might notice
significant improvement in respiratory effort and suggest
a trial of NPA removal. If it was unclear whether the
patient would tolerate NPA removal, observation in the
hospital for 1 to 2 days was recommended.
Descriptive statistics were calculated using STATA
(version 9.0 for windows; Stata Corp., College Station,
TX).

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Cleft PalateCraniofacial Journal, July 2011, Vol. 48 No. 4

TABLE 1 Characteristics of Infants Requiring NPA Treatment at

Seattle Childrens Hospital (n = 35)
Characteristic

n (%)

Sex
Male

21 (60.0)

Syndrome
Secondary CP with micrognathia, nonsyndromic PRS
Syndromic secondary CP 6 other craniofacial anomalies*
Branchial anomalies and syndromic micrognathia{

18 (52.9)
13 (11.8)
4 (11.4)

Gestational age
3436 weeks
3741 weeks
Unknown

3 (8.6)
31 (88.6)
1 (2.9)

Events
Death
Tracheotomy
No tracheotomy
Tracheotomy placed same day as NPA
Tracheotomy placed after NPA trial
Feeding tube
No feeding tube
Feeding tube placed before NPA
Feeding tube placed same day as NPA
Feeding tube placed after NPA

2 (5.7)
26 (74.3)
1 (2.9)
8 (22.9)
5
10
6
14

(14.3)
(28.6)
(17.1)
(40.0)

* Includes Miller, multiple malformation, and auriculocondylar syndromes.

{ Includes diastrophic dysplasia; campomelic dysplasia; Peters anomaly; and Stickler,
van der Woude, Smith-Lemli-Opitz, Coffin-Siris, and Marshall-Smith syndromes.

RESULTS
Characteristics of the 35 patients included in this case
series are described in Table 1. Twenty-one (60%) patients
were male. Eighteen (51.4%) patients had the diagnosis of
Pierre Robin sequence (PRS) and 13 (37.1%) had secondary
palatal clefts and associated syndromes including diastrophic dysplasia; campomelic dysplasia; Peters anomaly;
and Stickler, van der Woude, Smith-Lemli-Opitz, CoffinSiris, and Marshall-Smith syndromes. Four (11.5%) children had syndromic micrognathia including Miller, multiple malformation, and auriculocondylar syndromes. Thirty-one of the 35 (88.6%) children were born between 37 and
41 weeks of gestational age.
The mean and median age of first NPA placement was
3.2 and 1.3 weeks, respectively. The median time between
first placement and final removal of NPA was 8.0 weeks.
The median age at time of final NPA removal was
15.0 weeks (Table 2). Frequently, NPAs are removed for
some duration (ranging from hours to weeks) and then
replaced if a child appears to have recurrent difficulty
breathing with positional therapy alone. Among the 35
children with NPAs described in this report, five had their
NPAs removed for longer than 1 week (if NPA free-time
was shorter than 1 week, the sequential NPA placements
were considered to be one continuous placement); these
NPA-free durations ranged from 2.4 to 78.0 weeks, with a
median duration of 13.6 weeks.
Tracheotomy was performed in nine patients (25.7%;
Table 3); one tracheotomy was placed on the same day as a

failed attempt at NPA, and eight were placed at least 1 day

after placement of the initial NPA. Feeding tube placement
was required in 85.7% of all patients managed with NPA.
No soft tissue complications were noted. Intranasal
examination was not routinely performed, and therefore we
were unable to assess the incidence of intranasal complications.
Two of the 35 children managed with an NPA died.
Neither death was attributed to airway obstruction or the
NPA. One infant suffered from a severe pulmonary
infection unrelated to his craniofacial condition and passed
away at home at the age of 19 months, more than 1 year
after any airway management was required; the second
infant had a chromosomal translocation 7;22 with trisomy
7q as well as multiple and complex medical conditions
including cardiopulmonary disease.
DISCUSSION
Management of children born with airway and feeding
difficulties secondary to craniofacial anomalies remains
challenging. The Craniofacial Center at Seattle Childrens
Hospital is a regional center that manages infants born with
craniofacial anomalies from Washington, Wyoming,
Alaska, Montana, and Idaho (WWAMI), as well as
patients outside the WWAMI region. Our series describes
our experience with NPA for airway management in infants
with upper airway obstruction related to craniofacial
anomalies.
The type of CFA can impact the location of airway
obstruction. Therefore, we focused this investigation on
children with secondary cleft palate with other craniofacial
anomalies, and branchial anomalies with isolated or
syndromic micrognathia whose NPA was placed before
the 15th week of life. These children are at risk for airway
obstruction related to tongue position, mandibular hypoplasia, and occasionally other airway malformations.
One group of patients in which NPA has been described
is children born with PRS (mandibular hypoplasia,
glossoptosis, and U-shaped cleft palate) (Benjamin and
Walker, 1991; Tomaski et al., 1995). Patients with isolated
PRS may be successfully managed with positioning and
NPA with a range of 1 to 6 months of intervention
(Benjamin and Walker, 1991; Singer and Sidoti, 1992;
Tomaski et al., 1995; Meyer et al., 2008). Most of the
studies that have looked at the use of the NPA have
focused on children with isolated PRS, and not at
syndromic patients with micrognathia.
Wagener et al. reviewed the use of NPA in 20 neonates
with PRS and respiratory distress; their airways were
successfully managed without the need for further surgical
intervention to relieve airway obstruction (Wagener et al.,
2003). In their group, mean duration of NPA was 44 days,
with a range of 16 to 104 days; however, all of these
children were in the hospital for the duration of their NPA.
In a recent study by Meyer et al. (2008), airway

Parhizkar et al., MANAGEMENT OF AIRWAY OBSTRUCTION WITH NASOPHARYNGEAL AIRWAY

TABLE 2

481

Statistics Describing Timing and Duration of NPA in Weeks

Percentiles
n (%)

Age at initial NPA placement

Age at removal{
Time between initial NPA placement and removal{
Duration of NPA-free intervals{

35
33
33
5

(100)
(94.3)
(94.3)
(14.3)

Mean*

Minimum

10th

50th

90th

Maximum

3.2
n/a
n/a
n/a

0
0
0
2.4

0
2.6
0
2.4

1.3
15.0
8.0
13.6

10.6
89.6
27.2
78.0

14.7
97.9{
30.6{
78.0{

* n/a 5 not applicable.

{ Two children were missing an NPA removal date.
{ Total duration of NPA-free intervals between the first and last placement among five children who did not have continuous NPA placement.

management in 74 nonsyndromic and syndromic infants

with PRS was reviewed. In their study, 29 of 38 (76.3%)
patients were treated at some point with the NPA; however
the rest of this cohort of PRS infants required surgical
airway intervention.
Other techniques are also used to treat infants with CFA
and airway obstruction. One method is tongue lip adhesion
(TLA), a surgical procedure in which the tongue is
temporarily attached to the lower lip, bringing the base of
the tongue forward. Several months after the adhesion is
performed, a second surgery is required to release the
tongue.
There has been minimal consensus regarding the role of
TLA in the management of infants with PRS. Some centers
have moved away from the use of glossopexy because of
concern for risk of wound dehiscence, persistent airway
obstruction, feeding difficulties, scarring, and injury to
Whartons ducts. However, Kirschner et al. (2003) reported
a favorable experience with TLA in 29 patients, particularly
when the technique included deep sutures to provide additional support to the mucosal sutures. This modification

TABLE 3 Selected Characteristics of Children Managed With

NPA Requiring Tracheotomies (n = 9)
Characteristic

n (%)

Sex
Male

5 (55.6)

Syndrome
Secondary CP with PRS
Syndromic secondary CP 6 other craniofacial anomalies*
Branchial anomalies and syndromic micrognathia{

1 (11.1)
6 (55.6)
2 (33.3)

Gestational age
3436 weeks
3741 weeks
Unknown

1 (11.1)
7 (77.8)
1 (11.1)

Events
Death
Feeding tubes
No feeding tube
Feeding tube placed before NPA
Feeding tube placed same day as NPA
NPA placed before feeding tube

2 (22.2)
0 (0)
2 (22.2)
1 (11.1)
6 (66.7)

* Includes diastrophic dysplasia; campomelic dysplasia; Peters anomaly; and Stickler,

Coffin-Siris, and Marshall-Smith syndromes.
{ Includes auriculocondylar syndrome.

obviated the need for retention sutures or release of the

genioglossus musculature. They noted no significant
change in feeding issues after TLA, though most patients
required tube feeding even after TLA. The average age of
TLA was 24 days and the average age at takedown of TLA
was 9.3 months (Kirschner et al., 2003).
It needs to be determined preoperatively that glossoptosis is the definitive reason for airway obstruction. It is
absolutely essential to determine glossoptosis as the
definitive etiology for airway obstruction in a PRS child
prior to TLA (Qaqish and Caccamese Jr., 2009).
Dehiscence is the most commonly reported complication,
occurring in 4.2% to 17.2% of patients undergoing TLA
(Qaqish and Caccamese Jr., 2009), but it is notable that
TLA does require patients to undergo general anesthesia
and to remain intubated for an average of nearly 5 days
postoperatively. Furthermore, patients will require an
additional procedure to divide the adhesion, though this
has been coordinated with cleft palate repair in some cases.
Although long-term sequelae can be minimal, it is
important to note that TLA even with appropriate patient
selection will require at least two procedures and hospitalization (Kirschner et al., 2003).
Distraction osteogenesis of the mandible is another
technique that has recently gained favor in treating
micrognathia and its associated airway obstruction (Meyer
et al., 2008). Denny et al. (2001) showed that mandibular
advancement using distraction osteogenesis can improve
airway dimensions and result in decannulation in tracheotomy-dependent children. Monasterio et al. (2002) also
reported excellent results in avoiding tracheotomy in
neonates and children with mandibular hypoplasia using
mandibular distraction. Meyer et al. (2008) also describe
favorable outcomes with distraction osteogenesis in patients
with PRS who failed NPA.
Although some have described success with mandibular
distraction, the potential complications are important to
consider when making treatment recommendations. Distraction device placement can lead to complications, such
as injury to the marginal mandibular nerve and inferior
alveolar nerve and tooth root injury. In a recent review of
over 2000 mandibular distraction cases, the incidence of
injury was 3.6%, 1.9%, and 0.4% for inferior alveolar nerve,
marginal mandibular, and tooth root injury, respectively

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Cleft PalateCraniofacial Journal, July 2011, Vol. 48 No. 4

(Franco and Carstens, 2009). Infection is the most

commonly reported complication, at a rate of 18.5% in a
review of over 3000 cases by Mofid et al. (2001). The
majority were local infections related to the pin site; these
were resolved with local wound care and oral antibiotics.
One percent of infections involved bone. Hardware failure
and dislodgement has been reported at a rate of 7.5%.
Malocclusion can also occur due to initial improper vectors
of the device. External scars and the need for revision
surgery of the scar are also well-known complications
(Franco and Carstens, 2009).
NPA is an appealing nonsurgical measure to definitively
manage airway obstruction during infancy in some patients
with micrognathia. The majority of children treated with
NPA at our institution were younger than 15 weeks of age.
They were full term with almost equal sex distribution.
Furthermore, only 53% had isolated PRS. At our center,
we have preferentially used the NPA to manage patients
with airway obstruction related to the position of the
tongue base (glossoptosis) in whom positioning alone was
insufficient. The main advantages of NPA are that it can be
placed without anesthesia, and these patients may be
discharged home and followed as outpatients. Furthermore, the NPA can be removed without anesthesia, and the
duration of intervention is relatively short. Disadvantages
are the additional home care required of the parents, the
cosmetic appearance of the NPA, and the possible negative
impact on oral feeding.
In our series, tracheotomy was performed in nine (25%)
patients after a failed NPA trial. There was equal sex
distribution, and a majority of these patients were full-term
infants. Only one of the nine children (11%) who failed the
NPA trial had isolated PRS. Six patients (55%) who
required tracheotomies had secondary cleft palate and
other craniofacial anomalies (including diastrophic dysplasia; campomelic dysplasia; Peters anomaly; and Stickler,
Coffin-Siris, and Marshall-Smith syndromes), while two
(33%) had brachial anomalies and/or micrognathia (isolated and syndromic, including auriculocondylar syndrome).
There are several limitations of this study. This is an
observational descriptive report of the experience using
NPA at a single center using existing data. We were not
able to compare the outcomes of NPA with any other
management. Furthermore, for this project we were unable
to collect information about other characteristics such as
weight gain, oxygen saturation, and bicarbonate levels that
may shed light on the efficacy of the NPA as a treatment
for children with CFA.

CONCLUSION
NPA is a nonsurgical option for management of infants
with craniofacial anomalies and upper airway obstruction
in the pharynx. In our patient population, infants born with
PRS, secondary cleft palate, and other craniofacial
anomalies, and branchial anomalies with isolated or
syndromic micrognathia were candidates for a trial with
NPA for airway management as a noninvasive intervention. There were no complications attributable to NPA in
this series, and the infants were able to be discharged from
the hospital with NPA in place. Most patients who had
NPA also required feeding intervention. NPA is a
reasonable noninvasive airway intervention that may be
considered prior to surgical intervention.
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