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KEY WORDS: cleft palate, nasopharyngeal airway, Pierre Robin sequence, up-
per airway obstruction
In 1923 Pierre Robin published a description of neonates be seen in a position between the palatal shelves with the uvula
with micrognathia, glossoptosis, and respiratory distress and lying at each side. Retroaction of the tongue is aggravated
in a later publication added cleft palate as an aggravating factor when the baby is distressed. In some cases respiratory obstruc-
(Robin, 1934). These features combined are known as Pierre tion is severe enough to be immediately life threatening; in
Robin sequence. Pierre Robin sequence (PRS) can occur in most cases, however, it is less severe. Tongue position and cleft
isolation or in association with other congenital anomalies or palate together also lead to feeding difficulties. In cases of mild
syndromes (Benjamin and Walker, 1991; Shprintzen, 1992). respiratory obstruction, most of the babys energy may be
Neonates with PRS suffer from two main problems, airway spent on breathing and together with the feeding difficulties
obstruction and feeding difficulties. Both are closely related this results in failure to thrive (Shprintzen, 1992). By the age
and the severity of each is variable in each baby. Airway ob- of about 6 months, most babies outgrow these difficulties
struction is thought to be due mainly to the tongue falling (Benjamin and Walker, 1991) because of maturation of the
backward in the upper pharynx (Robin, 1923; Shprintzen, neuromuscular control of the tongue and growth of the man-
1992), although other causes have been suggested, and it may dible (Pasyayan and Lewis, 1984; Sher, 1992).
be a multifactorial problem (Sher, 1992). The tongue often can Babies with PRS have been treated by various methods,
ranging from nursing in the prone position with or without the
Miss S. Wagener is a Trust Doctor, Pediatric Plastic Surgery, Mr. Rayatt is
help of different appliances (Pasyayan and Lewis, 1984; Sa-
a Specialist Registrar, Plastic Surgery, Dr. Tatman is a Consultant Anesthetist, dewitz, 1992) and endotracheal intubation (Benjamin and
Mr. Gornall is a Consultant Pediatric Surgeon, and Miss Slator is a Consultant Walker, 1991) to invasive surgical treatment such as glosso-
Plastic Surgeon, Birmingham Childrens Hospital, Birmingham, England. pexy (Douglas, 1946; Argamaso, 1992), mandibular distraction
Presented at the Conference of the British Association of Plastic Surgeons; (Wada et al., 1983), subperiosteal release of the floor of the
Birmingham, England; June 7, 2000.
mouth (Caouette-Laberge et al., 1996), or tracheostomy (Bath
Submitted March 2001; Accepted April 2002.
Address correspondence to: Miss R. Slator, Consultant Plastic Surgeon, Bir- and Bull, 1997). The use of a nasopharyngeal airway (NPA)
mingham Childrens Hospital, Steelhouse Lane, Birmingham B4 6NH, Great as treatment to relieve respiratory distress in babies with PRS
Britain. E-mail rona.slator@blueyonder.co.uk. was first described in a case report (Axtrup, 1963). In 1982 a
180
Wagener et al., MANAGEMENT OF PIERRE ROBIN SEQUENCE 181
,2.5 7 3
2.53.5 7.5 3.5
.3.5 8 4
tracheostomy (Sher, 1992; Singer and Sidoti, 1992), which still information in future studies of babies with PRS but would
have significant complications (Augarten et al., 1990; Freezer involve more complex care of the babies.
et al., 1990; Argamaso, 1992; Donnelly et al., 1996). The ma- We believe that attention to feeding is the second important
jor criticisms against conservative management have been the factor in treating babies with PRS. Although it is known that
difficulty in clinically monitoring the airway and the prolonged weight gain improves after insertion of an NPA (Heaf et al.,
hospital stay in an environment that precludes normal inter- 1982), most of the babies in this study needed careful super-
action between parents and baby (Bull et al., 1990; Singer and vision of their nutrition. Feeding via an NG tube reduced the
Sidoti, 1992). In some reports it has been suggested that NPAs amount of energy needed and was introduced in all babies.
failed to effectively relieve airway obstruction (Augarten et Seventeen babies required extra calories as well. It is also im-
al., 1990; Freezer et al., 1990). This is more likely to occur portant to stimulate the tongue and encourage sucking to im-
with the use of NPAs with traditional connectors. In 1998 a prove neuromuscular coordination. Small amounts of bottle-
method of stabilizing the NPA with an individually constructed feeding were therefore introduced as early as possible. Parents
nasal splint was published (Smyth, 1998), and recently the use were encouraged to stroke their babies tongue from the first
of a modified NPA, which successfully relieved airway ob- day, and sucking on a dummy was encouraged. Apart from a
struction in all six babies with PRS treated (Masters et al., baby with intrauterine growth retardation, all babies without
1999; Chang et al., 2000), has been described. associated anomalies in this study were thriving satisfactorily
It has been shown that significant hypoxia in babies with on the described regimen including the premature babies. All
PRS can be present without apparent clinical signs (Bull et al., but one baby were discharged home at least partially bottle-
1990). It is therefore necessary to assess the neonates besides feeding. Top-up NG feeds were required for a variable period
clinically. In the recent series mentioned above, using a mod- of time but at the latest were stopped at the time of palate
ified NPA (Masters et al., 1999; Chang et al., 2000) polysom- repair without a problem.
nography was performed in a sleep laboratory to assess the Grouping of the babies involved in the study according to
respiratory status, and lateral neck radiographs were used to the characteristics of their initial assessment developed over
check the position of the NPA. In a different report, endoscopy the early period of the study, and babies were allocated to
was used for this purpose (Olson et al., 1990). groups retrospectively. Assessment of the progress of the ba-
In this series the NPA described relieved airway obstruction bies in groups 2 through 4 suggests some differences among
the groups. Clearly those in group 4 required immediate in-
effectively in all babies. This was confirmed by the continuous
sertion of an NPA, whereas the time to insertion of the NPA
oxygen saturation monitoring. The NPA used is similar to the
increased through groups 3 and 2. Babies in group 2 tended
modified NPA described by Masters et al. (1999), and is fixed
to be slightly more premature than those in groups 3 and 4.
in a similar way. It does not require any connectors or splints.
Group 2 babies also needed the NPA for a shorter length of
The size and length can easily be determined by the weight of
time and consequently needed a shorter inpatient stay than
the baby. Using too long a tube relieves airway obstruction
those in groups 3 and 4. Group 2 babies required the NG tube
but can aggravate gastroesophageal reflux. We found that if
for longer than babies in groups 3 and 4, however. All babies
the size of the tube was determined by the weight of the baby,
were a similar weight at discharge. We believe this suggests
this problem did not occur. Occasionally NPAs became ob-
that group 2 may represent babies whose problems are less
structed with secretions, but this was always detected early by
clearly caused by an obstructed airway and possibly more re-
the nursing staff; no severe oxygen saturation drops occurred lated to mechanisms of coordination and control of feeding
as a result. The size of NPA used did not seem to influence and respiration. Babies in group 4 would then be at the other
the occurrence of blockage. Changing the NPA was straight- end of the spectrum with problems that are most clearly the
forward and performed by the nursing staff as necessary. result of anatomical upper airway obstruction. However, the
Assessment of the airway with continuous oxygen saturation numbers of patients in each group is small, and no conclusions
monitoring as used in this study was noninvasive, safe, easy can be drawn without further prospective investigation to look
to carry out and interpret, and showed intermittent brief re- at such differences in more detail.
spiratory obstruction. It can be used in the normal neonatal One baby developed nostril stenosis, which probably could
ward environment, allowing continuous assessment before and have been avoided by use of the smaller self-cut NPA. Three
after insertion of the NPA and over a prolonged period. Once babies developed chest infections. The baby who required
the NPA is inserted and the baby comfortable, normal contact bronchial lavage was transferred to our pediatric intensive care
between parents and baby is possible. Parents can cuddle, feed, unit after aspiration occurred in a different hospital. The NPA
and change their babies, admittedly in the hospital but other- in place at transfer was too long (11.5 cm) and as a result may
wise in a normal manner. No polysomnographic studies were have contributed to aspiration. The other two babies had no
performed in this series, and central apneas could not be iden- evidence of aspiration. No other complications occurred, and
tified. Polysomnography is a more sophisticated method of as- no baby needed surgical treatment to relieve airway obstruc-
sessing respiratory patterns and may highlight differences be- tion.
tween babies not identified by oxygen saturation monitoring. A disadvantage of the treatment described in this article was
Polysomnography would therefore provide valuable additional the long hospital stay involved. Home management of babies
Wagener et al., MANAGEMENT OF PIERRE ROBIN SEQUENCE 185
with NPA has been reported (Olson et al., 1990; Chang et al., floor of the mouth in Pierre Robin sequence: experience with 12 cases. Cleft
Palate Craniofac J. 1996;33:468472.
2000), and we are in the process of revising our guidelines to
Chang AB, Masters IB, Williams GR, Harris M, ONeil MC. A modified na-
include early discharge of babies from the hospital with an sopharyngeal tube to relieve high upper airway obstruction. Pediatr Pul-
NPA. So far, four babies have been managed at home with an monol. 2000;29:299306.
NPA and NG feeding without difficulties. Donnelly MJ, Lacey PD, Maguire AJ. A twenty year (19711990) review of
This is the largest reported number of babies with PRS treat- tracheostomies in a major paediatric hospital. Int J Pediatr Otorhinolaryn-
gol. 1996;35:19.
ed safely with NPA. We think that the assessment and treat- Douglas B. The treatment of micrognathia with obstruction by a plastic pro-
ment described is safe and avoids the need for surgery in a cedure. Plast Reconstr Surg. 1946;1:300308.
condition the babies outgrow. It can be used on a neonatal Freezer NJ, Beasley SW, Robertson CF. Tracheostomy. Arch Dis Child. 1990;
ward, and normal contact between parents and baby is possi- 65:123126.
Heaf DP, Helms PJ, Dinwiddie R, Matthew DJ. Nasopharyngeal airways in
ble. This treatment would be further improved by establishing Pierre Robin syndrome. J Pediatr. 1982;100:698703.
early home management. Masters IB, Chang AB, Harris M, ONeil MC. Modified nasopharyngeal tube
for upper airway obstruction. Arch Dis Child. 1999;80:186187.
Olson YS, Kearns DB, Pransky SM, Seid AB. Early home management of
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