Management of Infants With Pierre Robin Sequence

SILKE WAGENER, M.R.C.S.

SUKH S. RAYATT, F.D.S., F.R.C.S.
ANDY J. TATMAN, M.R.C.P., F.R.C.A.
PETER GORNALL, F.R.C.S. (ENG.)
RONA SLATOR, D.PHIL., F.R.C.S. (PLAST.)

Objective: Several methods of treating babies with Pierre Robin sequence

have been described since the condition itself was first documented in 1923.
The main aim of treatment has been to relieve upper airway obstruction. Treat-
ment methods used range from positioning of the baby to invasive surgery.
The aim of this article was to describe the assessment, treatment, and moni-
toring methods used for babies referred with Pierre Robin sequence (PRS).
Setting/Patients: From December 1995 to May 2000, 22 consecutive patients
were admitted to Birmingham Childrens Hospital with PRS. Their airway and
nutritional status were assessed and continuously monitored.
Interventions: Treatment concentrated on the relief of airway obstruction
with a nasopharyngeal airway (NPA) and nutritional support of the babies until
they grew out of their respiratory and feeding difficulties.
Main Outcome Measures: Outcome measures were oxygen saturation,
growth of the babies, and the need for surgery.
Results: All babies were managed successfully with an NPA and nutritional
support. No baby required surgery, and the majority showed good weight gain.
Conclusion: Relieving airway obstruction by NPA is an effective and safe
treatment for babies with PRS until they have grown out of their respiratory
and feeding difficulties. It avoids the need for surgery and can be used on
neonatal wards using the monitoring described.

KEY WORDS: cleft palate, nasopharyngeal airway, Pierre Robin sequence, up-
per airway obstruction

In 1923 Pierre Robin published a description of neonates
with micrognathia, glossoptosis, and respiratory distress and
in a later publication added cleft palate as an aggravating factor
(Robin, 1934). These features combined are known as Pierre
Robin sequence. Pierre Robin sequence (PRS) can occur in
isolation or in association with other congenital anomalies or
syndromes (Benjamin and Walker, 1991; Shprintzen, 1992).
Neonates with PRS suffer from two main problems, airway
obstruction and feeding difficulties. Both are closely related
and the severity of each is variable in each baby. Airway ob-
struction is thought to be due mainly to the tongue falling
backward in the upper pharynx (Robin, 1923; Shprintzen,
1992), although other causes have been suggested, and it may
be a multifactorial problem (Sher, 1992). The tongue often can
Miss S. Wagener is a Trust Doctor, Pediatric Plastic Surgery, Mr. Rayatt is
a Specialist Registrar, Plastic Surgery, Dr. Tatman is a Consultant Anesthetist,
Mr. Gornall is a Consultant Pediatric Surgeon, and Miss Slator is a Consultant
Plastic Surgeon, Birmingham Childrens Hospital, Birmingham, England.
Presented at the Conference of the British Association of Plastic Surgeons;
Birmingham, England; June 7, 2000.
Submitted March 2001; Accepted April 2002.
Address correspondence to: Miss R. Slator, Consultant Plastic Surgeon, Bir-
mingham Childrens Hospital, Steelhouse Lane, Birmingham B4 6NH, Great
Britain. E-mail rona.slator@blueyonder.co.uk.
be seen in a position between the palatal shelves with the uvula
lying at each side. Retroaction of the tongue is aggravated
when the baby is distressed. In some cases respiratory obstruc-
tion is severe enough to be immediately life threatening; in
most cases, however, it is less severe. Tongue position and cleft
palate together also lead to feeding difficulties. In cases of mild
respiratory obstruction, most of the babys energy may be
spent on breathing and together with the feeding difficulties
this results in failure to thrive (Shprintzen, 1992). By the age
of about 6 months, most babies outgrow these difficulties
(Benjamin and Walker, 1991) because of maturation of the
neuromuscular control of the tongue and growth of the man-
dible (Pasyayan and Lewis, 1984; Sher, 1992).
Babies with PRS have been treated by various methods,
ranging from nursing in the prone position with or without the
help of different appliances (Pasyayan and Lewis, 1984; Sa-
dewitz, 1992) and endotracheal intubation (Benjamin and
Walker, 1991) to invasive surgical treatment such as glosso-
pexy (Douglas, 1946; Argamaso, 1992), mandibular distraction
(Wada et al., 1983), subperiosteal release of the floor of the
mouth (Caouette-Laberge et al., 1996), or tracheostomy (Bath
and Bull, 1997). The use of a nasopharyngeal airway (NPA)
as treatment to relieve respiratory distress in babies with PRS
was first described in a case report (Axtrup, 1963). In 1982 a

180

FIGURE 1 Trend analysis of the continuous oxygen saturation monitor-
ing, showing the percentage of time the baby spent at each level of satu-
ration over the previous 24 hours.
report on a series of 12 babies with PRS treated with NPAs
was published (Heaf et al., 1982). Clinically observed im-
provements in airway obstruction and cyanotic episodes were
confirmed by arterial blood gases, and in five babies lung me-
chanics were measured to demonstrate the benefits of treatment
with NPAs. Since then NPAs have been used but as a definitive
treatment only in mild cases for up to 8 weeks or as temporary
treatment before surgery (Argamaso, 1992; Shprintzen, 1992;
Singer and Sidoti, 1992). More recently the use of a modified
NPA as definitive treatment in a series of 10 babies with upper
airway obstruction, including six with PRS has been described
(Masters et al., 1999; Chang et al., 2000). Polysomnography
sleep studies were used to monitor improvement.
Since December 1995 we have successfully treated all ne-
onates referred with PRS with an NPA and nasogastric (NG)
feeding and continuous pulse oximetry monitoring. The aim
has been to provide and monitor airway and nutritional support
in the least invasive manner until the babies outgrow their
respiratory and feeding difficulties after the first few critical
months. This article describes the methods of assessment and
treatment used and presents the outcome of 22 babies treated.
PATIENTS AND METHODS
Patients
Between December 1995 and May 2000, 22 neonates with
Pierre Robin sequence were referred to Birmingham Childrens
Hospital (BCH) from the West-Midlands. All these babies pre-
sented with the four clinical features: micrognathia, glossop-
tosis, respiratory distress, and cleft palate. The degree of re-
spiratory distress varied widely. One baby was transferred ven-
tilated and three babies had an NPA in situ at the time of
transfer.
Assessment
On arrival at BCH, the babies underwent assessment of their
airway and nutritional status. The airway was assessed both
clinically and with continuous oxygen saturation monitoring.
Clinically signs of respiratory distress (recessions, tracheal tug,
stridor) were recorded with the baby cradled in an upright
position and then gently lowered into a supine position while
Wagener et al., MANAGEMENT OF PIERRE ROBIN SEQUENCE 181
FIGURE 2 Different patterns of saturation found at continuous satura-
tion monitoring. A. Normal pattern of saturation. B. Chronic mild desat-
urations. C. Deep desaturations.
sleeping. If no obstruction occurred, the procedure was re-
peated while bottle-feeding.
Oxygen saturation was monitored continuously with a Sym-
phony 3000 pulse oximeter (Nellcor Inc., Haywood, CA). The
measurements from a period of 24 to 36 hours were down-
loaded and analyzed with Sat Master (EMG Scientific, Beverly
Hills, CA). This analysis allowed inspection of two different
aspects. First, the trend analysis showed the percentage of time
the baby spent at each level of oxygen saturation (Fig. 1). We
considered oxygen saturations of less than 90% for more than
5% of the time as significant and unsatisfactory. Second, dif-
ferent patterns of desaturations could be identified (Fig. 2A
through 2C). Deep desaturations below 80% were considered
a sign of severe intermittent airway obstruction.
The nutritional status of the neonates was assessed with dai-
ly weight measurements. A weight gain of 20 to 30 g/day was
considered satisfactory.
During the period of time of this study, we found that the
babies could be grouped into four categories according to the
results of the initial assessment. In the first group, babies had
clinically relatively mild signs of respiratory obstruction but
satisfactory oxygen saturation monitoring and gained weight
steadily over a period of 5 to 7 days. In the second group,
babies also had minimal clinical signs of respiratory obstruc-
tion but over a period of time either unsatisfactory oxygen
saturation monitoring or failed to gain weight. Babies in the
third group had clear evidence of clinical respiratory obstruc-
tion that was usually worse in the supine position and when
182 Cleft PalateCraniofacial Journal, March 2003, Vol. 40 No. 2

TABLE 1 The Size and Length of the Nasopharyngeal Airway

Used Determined by the Babys Weight

Weight, kg Length, cm Diameter, mm

,2.5 7 3
2.53.5 7.5 3.5
.3.5 8 4

Management of the airway depended on the outcome of the

initial assessment. Two babies did not require any airway in-
tervention (group 1) and were weaned from their NG tube
FIGURE 3 Oxygen saturation reading (above) and pulse rate (below) of before discharge home. They were discharged home when sat-
a baby with severe respiratory obstruction before and after insertion of an isfactory weight gain was established and the parents could
NPA at 2200 hours (10:00 PM). manage feeding.
All other babies were considered to need airway support
following assessment (groups 2 through 4) and were treated
feeding. These babies all had unsatisfactory oxygen saturation with a nasopharyngeal airway. Where respiratory obstruction
monitoring and did not gain weight. A fourth group of babies was severe, the NPA was inserted immediately and before fur-
presented with severe respiratory obstruction at rest and re- ther assessment (group 4). In groups 2 and 3, the NPA was
quired immediate insertion of a nasopharyngeal airway. Figure inserted after varying lengths of time (maximum 8 days), de-
3 shows the oxygen saturation in a baby with respiratory ob- pending on the progress of the baby as determined by oxygen
struction before and after insertion of an NPA. saturation monitoring and weight gain.
After insertion of the NPA, babies in groups 2 through 4
Treatment were treated similarly. Progress was monitored and regularly
reviewed regarding their airway and weight gain. When a tar-
All babies were initially fed with continuous NG feed (180 get weight of 3.5 kg was achieved and their oxygen saturation
mL/kg per day). When vomiting occurred, the feed was thick- monitoring remained satisfactory, the babies were gradually
ened. If weight gain was poor and oxygen saturation monitor- weaned from the NPA. The NPA was initially removed for 4
ing satisfactory, high-calorie supplements were added. It is hours/day and the airway and weight gain observed for a pe-
thought that growth can be impaired in babies with low sodium riod of 4 to 5 days. If all remained satisfactory, the amount of
and improved by sodium supplementation (Al-Dahan et al.,
1984). This may occur even if serum sodium is normal and
the only finding is a low urinary sodium (Bower et al., 1988).
Babies with NPAs or NG tubes lose sodium in their saliva.
Therefore, urine sodium was measured, and if it was low
(, 10 mmol), sodium supplements were given.
When airway obstruction was diagnosed by the criteria de-
scribed in the initial assessment, it was treated with an NPA.
The airways used were endotracheal tubes (Portex, SIMS Por-
tex, Hythe UK). The tube was cut to a size and length deter-
mined by the weight of the baby (Table 1). The end of the
tube was cut into two strips that were fixed on the babys face
with adhesive tape over a DuoDerm base dressing (DuoDerm
extra thin, ConvaTec Ltd., Deeside UK) as shown in Figure 4.
The NPA was changed regularly and adjusted in size according
to growth. Alternate nostrils were used.
Progress was monitored by assessing continuous oxygen sat-
uration and weight gain. As soon as weight gain was satisfac-
tory, continuous NG feeds were initially changed to NG bolus
feeds following which oral feeding (Mead Johnson Cleft Lip/
Palate Nurser; Mead Johnson Nutritionals, Evansville, IN) was
introduced. Initially bottles were offered twice a day for 10
minutes. This was gradually increased if oxygen saturation
monitoring and weight gain remained satisfactory. Babies were
encouraged to suckle on a dummy or finger from their arrival FIGURE 4 Baby with a fixed nasopharyngeal airway in the right and a
in the hospital. nasogastric tube in the left nostril.
 Wagener et al., MANAGEMENT OF PIERRE ROBIN SEQUENCE
FIGURE 5 Weight chart of a baby before and after insertion of a na-
sopharyngeal airway.
time without NPA was increased by 4 hours every 4 to 5 days
until it was no longer required. Discharge criteria were having
the NPA out for 4 days, satisfactory weight gain, and parental
confidence with feeding. All babies were discharged home ei-
ther on full bottle-feeds or with top-up feeds via an NG tube.
RESULTS
Of 22 neonates (14 girls, 8 boys) with PRS referred to BCH,
17 babies presented with isolated PRS, four of which were
premature (31 to 36 weeks of gestation). Two babies developed
cardiac problems (abnormal pulmonary vein drainage in a pre-
mature baby, dilated cardiomyopathy) but had no known syn-
drome. Three babies had PRS as part of a syndrome: Stickler
syndrome, craniocostomandibular syndrome (premature baby)
and partial duplication of chromosome 1 with an atrioseptal
defect (ASD) and a ventriculoseptal defect (VSD).
The mean gestational age was 38 weeks (range 31 to 42
weeks, median 38 weeks) and the mean birth weight was 2.8
kg (range 1.42 to 4.08 kg, median 2.9 kg). The mean age at
arrival at BCH was 13 days (range 1 to 69 days, median 3
days).
None of the babies required surgical intervention.
Two babies with isolated PRS had satisfactory results at
assessment (group 1) and were discharged home after feeding
was established. Both were fully bottle-fed. They stayed in the
hospital for 10 and 15 days, respectively.
Twenty babies required an NPA by our criteria. Of these,
one baby was transferred ventilated and three babies had an
NPA in situ at transfer. The NPA was required for a mean
duration of 44 days (range 16 to 104 days, median 40 days).
After insertion of the NPA and starting of NG feeds, the
weight gain of all babies improved, in some very markedly
(Fig. 5, for example). During their hospital stay, 18 babies
grew along the percentile of their birth weight or a higher
percentile. This included the four premature babies with iso-
lated PRS, the premature baby with abnormal pulmonary vein
183
drainage, and two syndromic babies. Four babies dropped their
weight significantly (one percentile band or more). One of
these was born with intrauterine growth retardation, one had
persistent fetal circulation and a dysplastic kidney, one baby
developed dilated cardiomyopathy, and one had a partial du-
plication of chromosome 1 with ASD and VSD.
The mean hospital stay for the babies treated with NPAs
was 60 days (range 25 to 162 days, median 54 days). Three
babies were fully bottle-fed on discharge, 16 were discharged
home partially bottle-feeding with NG top-up, and one baby
was discharged on full NG bolus-feeds. The length of time
NG feeding was required at home is known in 15 of the 17
babies who were discharged with NG feeding. One baby was
followed up elsewhere and in one baby the time was not re-
corded, but NG feeding had been stopped before palate repair
at 7 months. The baby with dilated cardiomyopathy still re-
quires NG feeds. For the remaining 14 babies, the mean du-
ration of NG feeding was 4 months (range 1 week to 11
months, median 4 months). This included two babies who had
NG top-up feeds until their palate repair. Following surgery
these babies NG tubes were removed without any problem.
Complications occurred in four neonates. Three babies de-
veloped chest infections in the hospital. Two responded to an-
tibiotic treatment and one required bronchoscopic washout.
One baby developed right nostril stenosis. This baby was ini-
tially ventilated at referral and a manufactured NPA (NPA with
Linder airguide; Polamedco, Marina del Rey, CA) was used.
To obtain the required length with this make of tube, a bigger
diameter than usual (4.0 mm) had to be used. In addition, the
baby suffered from left choanal atresia so that only the right
nostril could be used.
So far, 18 babies have had their cleft palate repaired at a
mean age of 9 months (range 6 to 17 months, median 8
months). Of these babies, two initially lost weight before dis-
charge from the hospital. Sixteen babies were growing along
or above the percentile of their birth weight during their initial
hospital stay, and 10 of these had maintained their weight per-
centile at the time of admission for cleft palate repair. Six
babies dropped their weight by one percentile band or more
between their initial discharge from hospital and readmission
for palate repair.
Two babies still await palate repair, and the baby with di-
lated cardiomyopathy is not well enough for surgery. One baby
was followed up and treated in a different hospital after dis-
charge and no further data were available.
DISCUSSION
Neonates with PRS have been treated in many different
ways. The oldest established method, prone nursing, does not
relieve airway obstruction in all babies (Sher, 1992). Oral feed-
ing is difficult and normal contact (cuddling) between parents
and baby is prevented. Additionally, it can be difficult to assess
the baby clinically while prone so that significant respiratory
obstruction could be missed (Singer and Sidoti, 1992). The
other well-established treatment modalities are glossopexy and
184 Cleft PalateCraniofacial Journal, March 2003, Vol. 40 No. 2
tracheostomy (Sher, 1992; Singer and Sidoti, 1992), which still
have significant complications (Augarten et al., 1990; Freezer
et al., 1990; Argamaso, 1992; Donnelly et al., 1996). The ma-
jor criticisms against conservative management have been the
difficulty in clinically monitoring the airway and the prolonged
hospital stay in an environment that precludes normal inter-
action between parents and baby (Bull et al., 1990; Singer and
Sidoti, 1992). In some reports it has been suggested that NPAs
failed to effectively relieve airway obstruction (Augarten et
al., 1990; Freezer et al., 1990). This is more likely to occur
with the use of NPAs with traditional connectors. In 1998 a
method of stabilizing the NPA with an individually constructed
nasal splint was published (Smyth, 1998), and recently the use
of a modified NPA, which successfully relieved airway ob-
struction in all six babies with PRS treated (Masters et al.,
1999; Chang et al., 2000), has been described.
It has been shown that significant hypoxia in babies with
PRS can be present without apparent clinical signs (Bull et al.,
1990). It is therefore necessary to assess the neonates besides
clinically. In the recent series mentioned above, using a mod-
ified NPA (Masters et al., 1999; Chang et al., 2000) polysom-
nography was performed in a sleep laboratory to assess the
respiratory status, and lateral neck radiographs were used to
check the position of the NPA. In a different report, endoscopy
was used for this purpose (Olson et al., 1990).
In this series the NPA described relieved airway obstruction
effectively in all babies. This was confirmed by the continuous
oxygen saturation monitoring. The NPA used is similar to the
modified NPA described by Masters et al. (1999), and is fixed
in a similar way. It does not require any connectors or splints.
The size and length can easily be determined by the weight of
the baby. Using too long a tube relieves airway obstruction
but can aggravate gastroesophageal reflux. We found that if
the size of the tube was determined by the weight of the baby,
this problem did not occur. Occasionally NPAs became ob-
structed with secretions, but this was always detected early by
the nursing staff; no severe oxygen saturation drops occurred
as a result. The size of NPA used did not seem to influence
the occurrence of blockage. Changing the NPA was straight-
forward and performed by the nursing staff as necessary.
Assessment of the airway with continuous oxygen saturation
monitoring as used in this study was noninvasive, safe, easy
to carry out and interpret, and showed intermittent brief re-
spiratory obstruction. It can be used in the normal neonatal
ward environment, allowing continuous assessment before and
after insertion of the NPA and over a prolonged period. Once
the NPA is inserted and the baby comfortable, normal contact
between parents and baby is possible. Parents can cuddle, feed,
and change their babies, admittedly in the hospital but other-
wise in a normal manner. No polysomnographic studies were
performed in this series, and central apneas could not be iden-
tified. Polysomnography is a more sophisticated method of as-
sessing respiratory patterns and may highlight differences be-
tween babies not identified by oxygen saturation monitoring.
Polysomnography would therefore provide valuable additional
information in future studies of babies with PRS but would
involve more complex care of the babies.
We believe that attention to feeding is the second important
factor in treating babies with PRS. Although it is known that
weight gain improves after insertion of an NPA (Heaf et al.,
1982), most of the babies in this study needed careful super-
vision of their nutrition. Feeding via an NG tube reduced the
amount of energy needed and was introduced in all babies.
Seventeen babies required extra calories as well. It is also im-
portant to stimulate the tongue and encourage sucking to im-
prove neuromuscular coordination. Small amounts of bottle-
feeding were therefore introduced as early as possible. Parents
were encouraged to stroke their babies tongue from the first
day, and sucking on a dummy was encouraged. Apart from a
baby with intrauterine growth retardation, all babies without
associated anomalies in this study were thriving satisfactorily
on the described regimen including the premature babies. All
but one baby were discharged home at least partially bottle-
feeding. Top-up NG feeds were required for a variable period
of time but at the latest were stopped at the time of palate
repair without a problem.
Grouping of the babies involved in the study according to
the characteristics of their initial assessment developed over
the early period of the study, and babies were allocated to
groups retrospectively. Assessment of the progress of the ba-
bies in groups 2 through 4 suggests some differences among
the groups. Clearly those in group 4 required immediate in-
sertion of an NPA, whereas the time to insertion of the NPA
increased through groups 3 and 2. Babies in group 2 tended
to be slightly more premature than those in groups 3 and 4.
Group 2 babies also needed the NPA for a shorter length of
time and consequently needed a shorter inpatient stay than
those in groups 3 and 4. Group 2 babies required the NG tube
for longer than babies in groups 3 and 4, however. All babies
were a similar weight at discharge. We believe this suggests
that group 2 may represent babies whose problems are less
clearly caused by an obstructed airway and possibly more re-
lated to mechanisms of coordination and control of feeding
and respiration. Babies in group 4 would then be at the other
end of the spectrum with problems that are most clearly the
result of anatomical upper airway obstruction. However, the
numbers of patients in each group is small, and no conclusions
can be drawn without further prospective investigation to look
at such differences in more detail.
One baby developed nostril stenosis, which probably could
have been avoided by use of the smaller self-cut NPA. Three
babies developed chest infections. The baby who required
bronchial lavage was transferred to our pediatric intensive care
unit after aspiration occurred in a different hospital. The NPA
in place at transfer was too long (11.5 cm) and as a result may
have contributed to aspiration. The other two babies had no
evidence of aspiration. No other complications occurred, and
no baby needed surgical treatment to relieve airway obstruc-
tion.
A disadvantage of the treatment described in this article was
the long hospital stay involved. Home management of babies

with NPA has been reported (Olson et al., 1990; Chang et al.,
2000), and we are in the process of revising our guidelines to
include early discharge of babies from the hospital with an
NPA. So far, four babies have been managed at home with an
NPA and NG feeding without difficulties.
This is the largest reported number of babies with PRS treat-
ed safely with NPA. We think that the assessment and treat-
ment described is safe and avoids the need for surgery in a
condition the babies outgrow. It can be used on a neonatal
ward, and normal contact between parents and baby is possi-
ble. This treatment would be further improved by establishing
early home management.
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