Surgical Airway Management in Pierre Robin Sequence: Is There a Role

for Tongue-Lip Adhesion?

RICHARD E. KIRSCHNER, M.D.
DAVID W. LOW, M.D.
PETER RANDALL, M.D,
SCOTT P. BARTLETT, M.D.
DONNA M. MCDONALD-MCGINN, M.S.
PATRICIA J. SCHULTZ, R.N., B.S.N.
ELAINE H. ZACKAI, M.D.
DON LAROSSA, M.D.

Objective: The purpose of this study was to examine the efficacy of tongue-
lip adhesion (TLA) in the management of clinically significant airway obstruc-
tion associated with Pierre Robin sequence.
Design: The records of all children admitted to The Childrens Hospital of
Philadelphia with a diagnosis of Pierre Robin sequence were reviewed. Charts
were reviewed for birth data, diagnosis, preoperative airway management
methods, and surgical intervention. Records of infants undergoing TLA were
analyzed for timing of surgery, operative technique, postoperative complica-
tions, length of hospital stay, and treatment outcome.
Results: Over the 28-year period 1971 to 1999, 107 patients (47 boys, 60 girls)
meeting the criteria for Pierre Robin sequence were admitted for treatment. Of
these, 74 (69.2%) were successfully managed by positioning alone. Surgical
management of the airway was performed in the remaining 33 (30.8%) patients,
29 of whom underwent TLA and 4 of whom underwent tracheostomy. Dehis-
cence of the adhesion occurred in five patients (17.2%), two of whom subse-
quently required tracheostomy. Within the group of patients who underwent
mucosal adhesion alone, the dehiscence rate was 41.6%. When the adhesion
included muscular sutures, however, dehiscence was not observed in any pa-
tient. Of the 24 patients in whom primary TLA healed uneventfully, airway ob-
struction was successfully relieved in 20 (83.3%). Failure of a healed TLA to
relieve the airway obstruction resulted in conversion to a tracheostomy in four
patients. Six patients who underwent TLA (20.7%) ultimately required a trache-
ostomy; five of these patients (83.3%) were syndromic. Of patients requiring
preoperative intubation, 42.9% ultimately required tracheostomy.
Conclusion: TLA successfully relieves airway obstruction that is unresponsive
to positioning alone in the majority of patients with Pierre Robin sequence and
should therefore play an important role in the management of these infants.

KEY WORDS: airway obstruction, Pierre Robin sequence, tongue-lip adhesion

Pierre Robin sequence (PRS) describes the clinical triad of both immediately after birth or during the following weeks of
microretrognathia, glossoptosis, and upper airway obstruction life. Untreated, many of these infants may suffer significant
(Robin, 1923, 1934; Randall, et al., 1965). Infants with PRS complications, including failure to thrive, chronic hypoxemia,
may present with airway obstruction, feeding difficulties, or carbon dioxide retention, and cor pulmonale (Robin, 1934;

Dr. Kirschner is Assistant Professor of Surgery, Dr. Low and Dr. Bartlett are
Associate Professors of Surgery, and Dr. Randall is Emeritus Professor of Sur-
gery, Division of Plastic Surgery, University of Pennsylvania School of Med-
icine and Attending Surgeon, The Childrens Hospital of Philadelphia. Ms.
McDonald-McGinn is Instructor of Pediatrics, University of Pennsylvania
School of Medicine and Associate Director of Clinical Genetics, Childrens
Hospital of Philadelphia. Ms. Schultz is Nurse Coordinator, Cleft Lip and Palate
Program, Childrens Hospital of Philadelphia. Dr. Zackai is Professor of Pe-
diatrics, University of Pennsylvania School of Medicine and Director of Clin-
ical Genetics, Childrens Hospital of Philadelphia. Dr. LaRossa is Professor of
Surgery, University of Pennsylvania School of Medicine, Chief, Division of
Plastic Surgery, and Director, Cleft Lip and Palate Program, Childrens Hospital
of Philadelphia, Philadelphia, Pennsylvania.
Presented at the annual meeting of the American Society of Plastic Surgeons;
Los Angeles, California; October 16, 2000; and at the 9th International Con-
gress on Cleft Palate and Related Craniofacial Anomalies; Gothenberg, Sweden;
June 2001.
Submitted September 2001; Accepted February 2002.
Address correspondence to: Richard E. Kirschner, M.D., Division of Plastic
Surgery, The Childrens Hospital of Philadelphia, 34th and Civic Center Blvd.,
First Floor, Wood Building, Philadelphia, PA 19104. E-mail kirschner@email.
chop.edu.

13
14 Cleft PalateCraniofacial Journal, January 2003, Vol. 40 No. 1
FIGURE 1 A. Design of lingual and labial mucosal flaps for tongue-lip adhesion. B. Suturing the lingual flap in place.
Pashayan and Lewis, 1984; Dykes et al., 1985). Although
deaths related to PRS are not nearly as frequent as were re-
ported in the early 20th century, modern mortality rates may
still be as high as 20% (Dykes et al., 1985; Benjamin and
Walker, 1991).
The infant with PRS often presents the clinician with a dif-
ficult diagnostic and therapeutic challenge, the severity and the
consistency of airway obstruction dictating the technique of
airway management. Frequently airway obstruction associated
with PRS may be adequately managed by prone positioning
alone. When respiratory or feeding problems persist despite
positioning maneuvers; however, surgical airway management
may be indicated. Several surgical options for the management
of airway obstruction associated with PRS have been de-
scribed, including tongue-lip adhesion, genioglossus release,
and tracheostomy. Recently some have advocated early man-
dibular lengthening in infants with PRS by distraction osteo-
genesis (Denny et al., 2001), although the indications for its
use remain poorly defined. To date, there remain no reliable
guidelines for establishing when surgical intervention is war-
ranted in the infant with PRS. Moreover, the most appropriate
procedure to perform in this setting remains the subject of
considerable disagreement.
Douglas described treatment of upper airway obstruction in
PRS by tongue-lip adhesion in 1946. Since that time, there has
been little consensus regarding the role of tongue-lip adhesion
in the management of infants with PRS. In a recent survey of
pediatric otolaryngology fellowship programs (Myer et al.,
1998), only 13% reported using tongue-lip adhesion. Nearly
two-thirds of the programs reported that they considered glos-
sopexy to be an inappropriate management technique under
any circumstances. Respondents to the survey noted several
disadvantages to tongue-lip adhesion, including dehiscence,
persistent airway obstruction, feeding difficulties, scar defor-
mity, and injury to Whartons ducts. With these responses in
mind and with the availability of mandibular distraction, we
may ask the question, Is there still a role for tongue-lip ad-
hesion in the management of infants with PRS? The purpose
of this study, therefore, was to examine the efficacy of tongue-
lip adhesion in the management of clinically significant airway
obstruction associated with PRS.
METHODS
The records of all children admitted to The Childrens Hos-
pital of Philadelphia with a diagnosis of PRS over a 28-year
period (1971 to 1999) were reviewed. Inclusion criteria were
the presence of microretrognathia, glossoptosis, and obstruc-
tive apnea. In all nonintubated patients, obstructive apnea was
confirmed preoperatively by polysomnography. Charts were
reviewed for birth data, diagnosis, preoperative airway man-
agement methods, and surgical intervention. The presence or
absence of syndromic diagnoses was determined by evaluation
by a geneticist.
Records of infants undergoing tongue-lip adhesion were an-
alyzed for timing of surgery, operative technique, postopera-
tive complications, length of hospital stay, and treatment out-
come. Glossopexy was performed by one of six staff plastic
surgeons, the details of the surgical technique varying slightly
among surgeons. In all cases, tongue-lip adhesion was per-
formed by apposition of a proximally based mucosal flap from
the ventral tongue and a superiorly based mucosal flap from
the posterior lower lip (Fig. 1). In some cases, the muscularis
propria of the tongue was sutured to the orbicularis oris muscle
(Fig. 2); in others, a mucosal adhesion alone was performed.
Retention sutures were placed in all patients; the sutures were
tied over polypropylene buttons placed at the posterior tongue
and beneath the chin (Fig. 3).
RESULTS
A total of 107 patients (47 boys, 60 girls) with PRS were
admitted for treatment during the study period. Of these, 74
(69.2%) were successfully managed by positioning alone. Sur-
gical management of the airway was performed in the remain-
ing 33 patients (30.8%), 29 of whom underwent tongue-lip
adhesion and 4 of whom underwent tracheostomy.
Of the 29 patients (13 boys, 16 girls) who underwent

FIGURE 2 Intermuscular suture joining the muscularis propria of the
tongue to the orbicularis oris.
FIGURE 3 Senior authors technique for retention suture placement.
Kirschner et al., TONGUE-LIP ADHESION 15
TABLE 1 Syndromic Pierre Robin Patients Managed by
Tongue-Lip Adhesion (n 5 14)
Syndrome # of Patients
Stickler 7
Fetal alcohol 1
Oral-facial-digital 1
Mobius 1
Provisionally unique 4
tongue-lip adhesion, 25 were full-term infants; four were born
prematurely. The mean birth weight was 3183 g (range 1730
to 4029 g). Fourteen patients (48.3%) were nonsyndromic. In
the remainder, PRS was associated with a syndromic diagnosis,
the most common of which was Stickler syndrome (Table 1).
Preoperative management of patients undergoing tongue-lip
adhesion included supplemental oxygen in 15 (44.8%), naso-
pharyngeal tube placement in 7 (24.1%), and endotracheal in-
tubation in 7 (24.1%). The mean age at surgery was 26.3 days
(range 2 to 129 days). Mucosal adhesion alone was performed
in 13 patients (44.8%). In the remaining 16 patients (55.2%),
the mucosal adhesion was reinforced by suturing the muscu-
laris propria of the tongue to the orbicularis oris. Concomitant
16 Cleft PalateCraniofacial Journal, January 2003, Vol. 40 No. 1
TABLE 2 Failed Tongue-Lip Adhesion (TLA) Requiring
Tracheostomy
Reason for Tracheostomy # of Patients
TLA dehiscence 2
Persistent glossoptosis 1 (Moyson, 1961; Hawkins and Williams, 1976; Wetmore et al.,
Laryngomalacia 1 1982; Line et al., 1986; Gianoli et al., 1990). The results of
Laryngeal granulation tissue 1
Failed later elective intubation 1
release of the mandibular insertion of the genioglossus was
performed in three patients (10.3%). Retention sutures were
removed at a mean of 13 days postoperatively.
After tongue-lip adhesion, patients remained intubated for a
mean of 4.8 days (range 0 to 12 days). Average length of
hospital stay for patients undergoing tongue-lip adhesion was
26.9 days (range 8 to 70 days). Dehiscence of the adhesion
occurred in five patients (17.2%), two of whom subsequently
required tracheostomy (Table 2), two of whom were thereafter
successfully managed by prone positioning alone and one of
whom underwent repeat tongue-lip adhesion. Within the group
of patients who underwent mucosal adhesion alone, the dehis-
cence rate was 41.6%. When the adhesion included muscular
sutures, however, dehiscence was not observed in any patient.
Of the 24 patients in whom primary tongue-lip adhesion
healed uneventfully, airway obstruction was successfully re-
lieved in 20 (83.3%). Failure of a healed tongue-lip adhesion
to relieve the airway obstruction resulted in conversion to a
tracheostomy in four patients (Table 2). Thus, six patients who
underwent tongue-lip adhesion (20.7%) ultimately required a
tracheostomy; five of these patients (83.3%) were syndromic.
Of patients requiring preoperative intubation, 42.9% ultimately
required tracheostomy.
No patient demonstrated an exacerbation of feeding diffi-
culties postoperatively, and most demonstrated improved feed-
ing associated with relief of upper airway obstruction. Naso-
gastric tube feedings were required in 93.1% of patients pre-
operatively and in 72.4% postoperatively. Sixty-two percent of
these infants were successfully weaned off nasogastric feed-
ings within 6 months of surgery. Patients who underwent suc-
cessful tongue-lip adhesion underwent takedown of the adhe-
sion at a mean of 9.3 months of age. No patient demonstrated
obstructive apnea after takedown of the tongue-lip adhesion,
and no patient suffered significant cosmetic or functional im-
pairment secondary to scar formation. Palate repair was suc-
cessfully performed in this group of patients at a mean age of
13.7 months. There were no deaths in the series.
DISCUSSION
Infants with PRS requiring surgical airway management
should be treated with the least morbid procedure that will
provide relief of their upper airway obstruction. In a recent
survey, 91% of pediatric otolaryngology fellowship programs
cited tracheostomy as the safest and most reliable surgical
method for long-term airway management in infants with PRS
(Myer et al., 1998). However, complications related to pedi-
atric tracheostomy, including cannula obstruction and acciden-
tal decannulation, have been reported to range from 19% to
49% and tracheostomy-related mortality from 2% to 8.5%
this series indicate that the overwhelming majority of infants
who fail to respond to positioning alone can be safely and
effectively managed by a properly performed tongue-lip ad-
hesion.
There are significant benefits to the use of tongue-lip ad-
hesion over both mandibular distraction and tracheostomy,
benefits that justify its use as first-line treatment of severe air-
way obstruction associated with PRS. Glossopexy requires no
long-term nursing care and may be safely taken down prior to
1 year of age in most infants. In contrast, tracheostomy may
be required for a comparatively longer period of time, neces-
sitating chronic nursing care and often long-term speech and
language therapy to promote the appropriate development of
communication skills. Whereas infants who have undergone
tracheostomy may demonstrate significant delays in both
speech production and language development (Kaslon and
Stein, 1985; Kamen, 1991), those treated by tongue-lip adhe-
sion suffer minimal or no adverse effect on their speech de-
velopment (LeBlanc and Golding-Kushner, 1992). Although
some have cited feeding difficulties as a potential drawback of
glossopexy, the majority of infants will demonstrate a signif-
icant improvement in feeding following surgery because of
relief of their upper airway obstruction (Singer and Sidoti,
1992).
Scarring that results from tongue-lip adhesion is usually mi-
nor and of no aesthetic or functional consequence. In contrast,
tracheostomy often results in significant external scarring after
decannulation and is associated with a risk of subglottic ste-
nosis. Mandibular distraction usually results in external scar-
ring as well. Although such scars may be minimized or later
revised, they can not be removed. Moreover, little is yet known
about the long-term effects of neonatal mandibular distraction
on inferior alveolar nerve function, the development and erup-
tion of the primary and secondary dentition, or long-term man-
dibular growth. Mandibular distraction should therefore be re-
served for the small minority of infants with PRS who dem-
onstrate persistent obstructive apnea despite tongue-lip adhe-
sion and reserve tracheostomy for those infants who are not
candidates for either procedure, such as those with severe lar-
yngomalacia. Of the 107 patients with PRS in this series, tra-
cheostomy was ultimately required in just 10 patients, two of
whom were found to have laryngeal abnormalities that con-
tributed to their airway obstruction. Therefore, only eight pa-
tients (7.5%) would be considered candidates for mandibular
distraction.
Although glossopexy was described as early as 1911 (Shu-
kowsky), the concept of tongue-lip adhesion for relief of ob-
structive apnea associated with PRS was first popularized by
Douglas in 1946. The Douglas procedure involved the excision
of mucosa from a rectangular area on the undersurface of the

tongue, extending onto the floor of the mouth, alveolus, and
lower lip. The tongue was then brought forward and the lateral
edges of the mucosal incision sutured to one another, thus cre-
ating a broad-based adhesion of the tongue to the lip, alveolus,
and floor of mouth. A retention suture was then placed from
the dorsum of the tongue to the chin. Despite some success,
the Douglas procedure was associated with significant com-
plications, including dehiscence, tongue lacerations, injuries to
Whartons ducts, and cicatricial ankyloglossia. In 1960, Rou-
tledge reported on a modification the tongue-lip procedure that
avoided many of these pitfalls. Transverse incisions were made
along the anterior tongue and the posterior lower lip, the ad-
hesion fashioned by suturing the wound margins to one an-
other and supported by a retention suture from the dorsum of
the tongue to the chin.
Several modifications of the Routledge procedure have since
been described. In 1977, Randall described moving the reten-
tion suture to the posterior aspect of the tongue, thereby pro-
viding for better support of the tongue base during healing.
Argamaso (1992) later described the use of an internal reten-
tion suture looped around the mandible and sutured to the mus-
cularis propria of the tongue. He and others have stressed the
importance of releasing the genioglossus muscle from its man-
dibular insertion to allow for improved anterior displacement
of the tongue (Delorme et al., 1989; Argamaso, 1992). Al-
though release of the genioglossus from its mandibular origin
may be a useful adjunct in some patients, we have not found
its routine use necessary to the success of airway management
by tongue-lip adhesion. Furthermore, our data suggest that use
of a circummandibular suture to prevent tongue-lip adhesion
dehiscence may be unnecessary provided that the mucosal ad-
hesion is supported by underlying sutures between the mus-
cularis propria of the tongue and the orbicularis oris. The ad-
dition of muscle sutures completely prevented the complica-
tions of tongue laceration and glossopexy dehiscence.
In our series, as in others (Pashayan and Lewis, 1984;
Caouette-Laberge et al., 1994; Tomaski et al., 1995), the over-
whelming majority of infants with airway obstruction associ-
ated with PRS were successfully managed by positioning
alone. Although most of the infants manifesting persistent air-
way obstruction despite positioning were successfully man-
aged by tongue-lip adhesion, more aggressive management by
tracheostomy was required more frequently in patients with
PRS associated with multiple anomaly syndromes than in those
in whom PRS was an isolated finding. A poorer prognosis for
infants with syndromic PRS sequence has been reported by
others as well (Pashayan and Lewis, 1984; Caouette-Laberge
et al., 1994; Tomaski et al., 1995) and likely reflects the pres-
ence of additional underlying airway anomalies, neurodevel-
opmental abnormalities, or both. These results underscore the
importance of an early comprehensive clinical evaluation of
all infants with PRS, including thorough assessment of the
upper airway, complete physical examination, and genetics
consultation.
It has been our practice to take down the tongue lip adhesion
several months prior to cleft palate repair. Although some sur-
Kirschner et al., TONGUE-LIP ADHESION 17
geons routinely maintain the glossopexy to protect the airway
after palatoplasty, we have not found this to be necessary in
our patients. Reversal of the tongue-lip adhesion at an earlier
age allows for hygiene of the lower central incisors and pro-
vides some assurance that the infant will successfully maintain
upper airway patency after palate repair. Finally, the majority
of infants in our series were successfully weaned off gavage
feedings shortly after tongue-lip adhesion, a reflection of the
fact that upper airway obstruction is the primary cause of feed-
ing problems in infants with PRS. Although none of the infants
in our series underwent gastrostomy, such may be considered
in infants who demonstrate persistent feeding difficulties de-
spite successful airway management.
CONCLUSION
Based on our experience, we offer the following algorithm
for the management of upper airway obstruction associated
with PRS:
laryngotracheal anomaly
assess airway treat anomaly
6 tracheostomy
| no laryngothracheal anomaly
successful
prone or decubitus positioning observe
| persistent upper airway obstruction
successful
tongue-lip adhesion observe
| persistent upper airway obstruction
mandibular distraction
Tongue-lip adhesion can safely relieve airway obstruction that
has proved refractory to positioning alone in the majority of
patients with PRS and should therefore play an important role
in the management of these infants. In all cases, successful
airway management is dependent on accurate diagnosis, in-
cluding both an adequate assessment of the mechanism of air-
way obstruction and associated congenital anomalies.
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