Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566

Airway considerations in craniofacial patients

Susanna Leighton, BSc, FRCS(ORL-HNS)a,b,@, Amelia F. Drake, MDc,*
a
Department of Pediatric Otolaryngology, Great Ormond Street Hospital, London, UK
b
Department of Surgery, Institute of Child Health, London, UK
c
Department of Otolaryngology, University of North Carolina, CB #7070, Chapel Hill, NC 27599, USA

Sleep apneas are recognizable interruptions of Central apnea and hypopnea

breathing and are classified as central, obstructive, or
mixed. Central apneas are characterized by a cessa-
tion of airflow at the nose and mouth with no
apparent respiratory effort and are the consequence of
a cessation in the phasic central neural drive to
breathe. In contrast, obstructive apneas are seen when
there is a cessation of airflow at the nose and mouth
but continued respiratory effort; they are particularly
likely to occur during rapid eye movement sleep. The
respiratory efforts during an obstructive apnea are
rendered ineffective by the loss of upper airway
patency because of collapse. It is also possible to
have partial airway obstruction when the airway
patency is reduced. This condition is characterized
by increased respiratory effort but diminished airflow
in response to increased upper airway resistance.
Mixed apneas are described when elements of cen-
tral and obstructive apneas are seen in association.
The length of apnea considered abnormal varies
with age. Premature infants, for example, may exhibit
central apneas of up to 20 seconds’ duration before
they are considered abnormal, whereas older infants
and children may exhibit shorter periods of clinically
significant obstructive apnea. Its association with
significant hypoxia, hypercapnia, or bradycardia may
be a measure of the significance of an apnea.
* Corresponding author.
E-mail address: amelia_drake@med.unc.edu
(A.F. Drake).
@
Deceased.
Central apneas are characterized by a cessation in
airflow at the nose and mouth with no discernible
respiratory effort. They are considered clinically
significant if they are associated with significant
changes in blood gases (SaO2<92%, end-tidal partial
pressure of carbon dioxide>55mmHg) or significant
bradycardia. Central apneas are frequently associated
with arousals that cause sleep fragmentation and poor
sleep quality, which may have detrimental effects on
daytime behavior and growth.
Craniofacial patients may be considered at high
risk for central respiratory control problems for two
reasons. First, the abnormal anatomy of the cranial
base may lead to a mechanical distortion of the brain
stem, wherein lie the respiratory control centers.
Second, the intracranial hypertension, which is often
seen in these patients, may cause anomalies of
respiratory control because of increased pressure
exerted on the brain stem structures. In extreme
cases, the brain stem may herniate through the
foramen magnum of the skull base, which may be
visualized on MRI scan.
The first description of chronic hindbrain hernia-
tion in a patient with syndromic craniosynostosis was
in an infant with Pfeiffer syndrome [1]. It has been
described subsequently in patients with clover-leaf
skull malformation and Crouzon and Apert syn-
dromes [2 – 5]. The caudal displacement of the
cerebellar tissue through the foramen magnum and
into the cervical canal may be associated with
anatomic changes in the brain stem, with lesions of
the cerebellum and compression of the medulla. From

1042-3699/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved.
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556 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
a respiratory point of view, herniation of the hind-
brain may affect brain stem respiratory centers and
the cranial nerve nuclei and roots of the nerves that
innervate upper airway musculature. Malfunction of
these structures may lead to feeding difficulties,
stridor, breath-holding spells, upper airway obstruc-
tion, and central sleep apnea or hypoventilation.
Patients with Arnold-Chiari malformation are
susceptible to acute cord compression syndromes,
particularly under circumstances in which there may
be acute changes in intracranial pressure (eg, obstruc-
tive sleep apnea with swings in PaCO2). If the brain
stem becomes compressed, these individuals may
develop central alveolar hypoventilation or central
apneas. Under these circumstances, they may require
mechanical ventilation continuously or intermittently.
Gonsalez et al [6] studied a group of 13 children
with syndromic craniosynostosis and hindbrain her-
niation (confirmed on MRI). They underwent over-
night cardiorespiratory sleep studies without sedation.
Clinically significant central apneas with falls in SaO2
to less than 90% were found in only two cases. In
contrast, 10 of the patients showed a degree of upper
airway obstruction that ranged in severity from mild
to severe. Two additional patients had previously
undergone tracheostomy to alleviate severe obstruc-
tive sleep apnea. The authors concluded that in a
selected group of children with syndromic cranio-
synostosis and hindbrain herniation, the predominant
sleep-related breathing problem was obstructive
apnea and that despite expectations, central apneas
and mixed apneas were seen only infrequently.
Brain stem compression also may be associated
with an increased risk of airway obstruction. Patients
with Arnold-Chiari malformation are at risk of
developing bilateral vocal fold paralysis [7 – 9]. This
condition is most frequent in infants and commonly
presents with airway symptoms between 1 and
12 months of age. Affected infants develop progres-
sive inspiratory stridor, which may become worse
during sleep. The stridor may progress to constant
stridor with CO2 retention and respiratory failure.
Normal respiratory control involves a coordinated
dilatation of the upper airway musculature to main-
tain upper airway patency during the imposition of
negative intraluminal pressures during inspiration.
During expiration, the vocal folds occupy their
normal relaxed partially open position; during inspi-
ration, the folds are normally abducted, which opens
the airway lumen. The recurrent laryngeal branch of
the vagus nerve controls vocal fold abduction.
Malfunction can be brought on because of traction
on the nerve roots or by compression or stretching of
the medulla itself [10].
Obstructive apneas and hypopneas
A patent upper airway is a prerequisite for suc-
cessful respiration. The upper airway is a collapsible
muscular tube that includes the nasopharynx, oro-
pharynx, and laryngopharynx. Its patency is deter-
mined by its diameter and the tonic activity of the
pharyngeal dilators and their phasic contraction in
response to the negative upper airway pressure
generated during inspiration. During sleep, reduced
tone of these dilators and diminished reflex dilatation
lead to physiologic airway narrowing, especially
during rapid eye movement sleep. This physiologic
narrowing can result in sleep-disordered breathing
(SDB), especially if the craniofacial morphology is
abnormal, which results in an airway of less-than-
average diameter at any point. Craniofacial abnor-
malities, such as the syndromic craniosynostoses,
Treacher Collins syndrome [11], and Pierre Robin
sequence [12], predispose individuals to SDB.
Nasal obstruction can contribute to SDB. The
likely mechanism is that high nasal resistance to
airflow necessitates the generation of high negative
intrathoracic pressures. Opening the mouth to aug-
ment the airway causes the tongue to move pos-
teriorly, which narrows the airway and further
contributes to the conditions necessary for collapse.
Large tonsils and adenoids are associated with SDB
[13,14] but there is no strong evidence of a cor-
relation between their size and the severity of airway
obstruction [15]. It seems to be their relative size com-
pared with the size of the airway that is important.
In the syndromic craniosynostoses, although the
membranous bones of the facial skeleton fail to grow
normally, the cartilaginous components are un-
affected. Facial dysplasia is severe, with the maxilla
grossly hypoplastic in all dimensions and the nose
and mandible relatively prominent. Foreshortening of
the anterior and posterior cranial base occurs, and the
maxilla is retrognathic in relation to the former. This
results in a reduction in pharyngeal depth, height, and
width. There is also a reduction in the height and
width of the posterior choanae leading to choanal
stenosis, which is often misdiagnosed clinically as
choanal atresia in these patients [16].
A long velum and a short mandibular body
contribute to pharyngeal crowding in three dimen-
sions. The mandibular hypoplasia displaces the
tongue posteriorly, and obstruction may occur at
tongue base level [17]. The palate is typically narrow
and high arched. In Apert and Crouzon syndromes
there are lateral palatal swellings caused by soft-
tissue deposits of mucopolysaccharides lying along
the lateral arches of the alveolus [18]. These are
 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
separated by a narrow median groove that may be
misdiagnosed as a cleft palate. The swellings often
become more prominent with maturity.
Children with Pfeiffer, Crouzon, and Apert
syndromes are likely to need airway support for
symptoms or complications of SDB 20% to 30% of
the time at some stage during infancy and childhood
(S. Leighton, unpublished data). A proportion of chil-
dren with Apert syndrome have a cleft palate, which
is associated with a reduced need for airway inter-
vention. Individuals with Antley-Bixler and Saethre-
Chotzen syndromes do not seem to have more airway
problems in childhood than the general population.
Obstructive apneas are defined as periods of
cessation of airflow measured at the nose and mouth
during which respiratory efforts continue and may
even increase or crescendo. The apneic period is
associated with detrimental changes in blood gases
(falling SaO2, rising PaCO2) and possibly reflex
bradycardia. During the apnea there is a progressive
increase in the drive to breathe, which leads to
increasing respiratory efforts against the closed
airway, and progressively greater negative intratho-
racic pressures, which results in respiratory-related
dips in blood pressure (pulsus paradoxus). The apnea
is terminated by an arousal that is probably triggered
by the increasing levels of afferent input from the
respiratory chemoreceptors and by increased activity
from mechanoreceptors within the lung, airway and
chest wall. Arousal serves to increase the level of
drive to the musculature supporting the upper airway,
which causes airway dilatation and allows effective
respiration to resume. Arousal is also associated with
a reflex increase in heart rate and a surge in blood
pressure because of a sudden increase in sympathetic
drive. The patient typically takes three or four large
effective breaths, sufficient to restore blood gases,
before returning to a deeper sleep state, at which
point airway tone is again lost and the obstructive
cycle begins again.
There is no universally accepted definition of
obstructive sleep apnea, and this is especially true for
children. In adults, the definition proposed by
Guilleminault et al is widely accepted [19]. They
defined sleep apnea as 30 or more apneic episodes
that last more than 10 seconds each that occur within
an 8-hour sleep period. Patients with significant
symptoms (eg, daytime hypersomnolence, impaired
daytime function), however, commonly have ap-
neic episodes that last considerably longer than
10 seconds and are associated with significant
oxygen desaturation, and they have several hundred
episodes per night. In children, the criteria for
diagnosis of obstructive sleep apnea based on dura-
557
tion and frequency of apneic events are even more
poorly established.
There is a clinical spectrum of SDB in children
that ranges from primary snoring through upper
airway resistance syndrome and obstructive hypo-
ventilation syndrome to obstructive sleep apnea syn-
drome (OSAS) as severity increases. Primary snoring
refers to the noise generated by turbulent airflow in
the pharynx; there is no associated apnea, hypopnea,
hypoxemia, or sleep fragmentation because of
arousals. In children, it is most commonly caused
by physiologic adenotonsillar hypertrophy. The peak
incidence is in the 3- to 6-year age group, when it
affects up to 10% of normal children [20]. In upper
airway resistance syndrome, snoring is associated
with partial upper airway obstruction during sleep
and increased inspiratory effort [21]. There is sleep
fragmentation and there may be daytime symptoms,
but there are no gas exchange abnormalities. In
obstructive hypoventilation syndrome there is con-
tinuous partial upper airway obstruction during sleep,
with at least 50% reduction in airflow, which causes
mild oxygen desaturation [22]. There also may be a
degree of hypercapnia. Sleep fragmentation and its
consequences are observed. OSAS is characterized
by episodic complete upper airway obstruction during
sleep with increased respiratory effort, paradoxic
respiratory movements, and sleep fragmentation with
reduced arterial oxygen saturation with or without
hypercapnia [19]. Two percent to 3% of normal
children may be affected by OSA, which, like pri-
mary snoring, peaks in the 3- to 6-year age group
[22]. A diagnosis of SDB in a child may be more
difficult than in an adult, and it is not necessarily
based solely on ‘‘objective’’ sleep study indices.
A combination of sleep study findings and direct
clinical observations of the sleeping child can
help a clinician grade the degree of nocturnal
airway obstruction.
Obstruction (partial or complete) is generally
considered significant if associated with hypoxemia
(falls in SaO2 to less than 90%) and hypercapnia
(PaCO2>45 mm Hg) or if sleep-related breathing
difficulties and sleep fragmentation or deprivation
result in clinically significant effects, such as behav-
ioral problems, failure to thrive, or cor pulmonale. A
history of disordered sleep patterns is often the first
indication of SDB in a child. More detailed ques-
tioning of the parents, possibly with the use of a sleep
diary to record nocturnal events of certain period (eg,
a week), may reveal other signs and symptoms of
sleep apnea. Sleep laboratory recordings help estab-
lish the presence and severity of sleep breathing
disturbances and their effects on overall sleep quality.
558 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
A recent study confirmed that the use of a
structured questionnaire for the parents, together with
a formal sleep study, formed the best basis for the
diagnosis of SDB [24]. The use of questionnaire and
clinical history in the absence of a formal sleep study
was found to give an accurate assessment of the
severity of sleep breathing problems in only 42% of
cases. In 17% of cases, the clinician underestimated
the severity of SDB when subsequently assessed by
sleep study, whereas in 16% of cases the clinician
overestimated the severity. In 10% of cases, the
clinical impression was markedly different from the
sleep study assessment.
Clinical features of obstructive sleep apnea
syndrome
Nocturnal symptoms and signs of OSAS usually
include heavy snoring, restlessness, and sweating.
There may be a history of nightmares or night terrors.
Intermittent apneas and increased respiratory effort
are often noted. Children may adopt unusual sleeping
positions to optimize the airway—typically with a
hyperextended neck and mouth-open posture or
prone with the knees tucked under the chest and the
head turned to the side. There may be associated
enuresis [25].
Daytime symptoms and signs include nasal
obstruction, mouth breathing, and hyponasal speech.
The complex cumulative physiologic effects of re-
peated upper airway obstruction during sleep also
may have adverse effects on cardiac function, somatic
growth, and neurobehavioral function [26], including
daytime hyperactivity, aggression, social withdrawal,
hypersomnolence [13,14,27 – 29], reduced capacity
for attention, impaired memory and cognitive func-
tion with developmental delay [13,30], and, in older
children, problems with academic performance [31].
These symptoms are believed to be caused by
arousals that disrupt the functions of the sleeping
brain. Although there is much anecdotal evidence,
however, currently there are relatively few data from
well-controlled studies.
The ability to remain at a task and attend to
external stimuli plays an important role in learning
and social and academic development. Recent studies
have provided the first limited data on the detrimental
effects of SDB on attention capacity in children
[32,33]. They suggest that children with sleep-related
breathing disorders are less reflective and more
impulsive and show poorer sustained and selective
attention. Importantly, two further studies have
suggested that these effects are reversible with
successful treatment of the upper airway obstruction
during sleep [21,34]. Two studies using standard
psychometric tests have shown significantly reduced
memory performance in children with sleep-related
breathing disorders [33,35]. Children with moderate
to severe OSA were shown to perform in the
intellectually deficient ranges compared with controls
who produced scores within the average range,
whereas children with mild SDB tended to fall in
the low normal range. These results suggest a dose-
dependent effect of SDB on memory capacity. Only
three published studies have examined intelligence
(IQ) in school-aged children with sleep-related
breathing disorders. Two studies found significantly
reduced IQ scores [33,35], whereas the third did not
[34]. Further studies are needed to clarify this
relationship. It can be shown that children with
SDB show diminished academic performance
[36,37]. Conversely, it has been shown that poor
academic achievers include a high preponderance of
children with SDB [31,38,39]. Importantly, research
also has demonstrated that treatment of these children
with sleep-related breathing problems by tonsillec-
tomy or adenotonsillectomy has been followed by an
improvement in academic performance [21,38].
Questionnaires for parents and teachers suggest
that behavioral problems are common in children
with sleep-related breathing disorders. Children have
been found to show increased levels of internalized
problematic behaviors (eg, shyness, withdrawal,
anxiety) [28,30] and externalized behaviors (eg,
impulsivity, hyperactivity, aggression) [20,34,36,37].
Inattention hyperactivity is frequently found, with a
reported prevalence rate of 20% to 42% [30,31,36].
Conversely, children with inattention hyperactivity
show a high prevalence of SDB [31,40]. Treatment
of nocturnal breathing problems has been shown
to improve daytime behavior [21,40].
Unlike adults with OSAS, excessive daytime
sleepiness is not usually a feature in children [41];
this is believed to be because arousals in children are
usually movement arousals rather than EEG or
behavioral arousals, as they are in adults. The diag-
nosis of excessive daytime sleepiness in children is
difficult because tiredness may manifest as hyper-
activity [40]. Daytime sleepiness is reported in some
children, however [20,28,33,37].
Intracranial hypertension (>15 mm Hg) is frequent
in children with syndromic craniosynostosis. Its eti-
ology is multifactorial, but OSAS may be an impor-
tant cause [42]. It may be asymptomatic, or children
may notice headache or failing vision. Like SDB,
it also can have an effect on behavior.
 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
There may be poor growth and failure to thrive
[13,41,43] caused by the increased energy expendi-
ture necessary to breathe during sleep and reduced
growth hormone secretion as a result of sleep frag-
mentation [44,45]. Successful treatment, however,
results in catch-up growth. Unlike adults, obesity [46]
and systemic hypertension [47] are uncommon
features in children.
Cardiorespiratory complications occur because of
recurrent severe hypoxia, hypercarbia, and acidosis,
including dysrhythmias, pulmonary hypertension, and
cor pulmonale [13,19,48,49]. These complications
are reversible within 48 hours of treatment of
OSAS [50]. Acute cardiorespiratory failure may be
triggered by an upper respiratory tract infection and
result in sudden death [51]. Such complications are
rare with recognition of OSAS and earlier diag-
nosis and treatment. The long-term cardiorespiratory
effects of mild SDB are unknown; there may be
an increased risk of systemic hypertension in later
life [52].
Diagnosis
The diagnosis of SBD in children is based on the
history from the parents, clinical findings on exami-
nation, and the results of investigations. The gold
standard investigation for the diagnosis of OSAS is
polysomnography. Other investigations that are
advocated include radiology, cephalometry (in which
standardized distances and angles from identifiable
bony landmarks are measured), fluoroscopy, endos-
copy, CT, and MRI. They may provide information
about the level of obstruction, but their indications
are not standardized and their value is unclear.
Investigations in the awake child may not provide
information relevant to the status of the airway during
sleep, and paradoxically, sedation may not mimic
normal sleep either. Pulse oximetry, if it confirms a
pattern of cyclic desaturation, can be helpful, but
there is a high false-negative rate for OSAS and it
cannot diagnose upper airway resistance syndrome or
obstructive hypoventilation syndrome [53]. The
reference technique for the diagnosis of upper airway
resistance syndrome is esophageal manometry, which
shows wide swings in pressure in this condition;
pulse transit time correlates with these pressure
changes and can be used as a clinical measure [54].
Research has shown that clinical histories from
parents tend to overestimate the significance of mild
airway symptoms and underestimate the severity of
serious problems [23,55], perhaps because of chro-
nicity. In syndromic children, respiratory problems
559
may be overshadowed by other apparently more
severe problems, such as cosmesis or developmental
delay, and may be discounted by the parents.
Expectations of children with multiple problems
may be low, and developmental delays may be
attributed to the syndrome itself rather than other
potentially remediable causes, such as SDB, hearing
impairment, or visual problems.
Clinical examination confirms the abnormal cra-
niofacial morphology. Patency of the nasal airways,
status of the palate, and size of the tonsils and
adenoids should be assessed, as should the general
condition of the child, to look for any signs of chronic
respiratory distress. If the clinician suspects cardio-
respiratory complications, an electrocardiogram and
a chest radiograph should be examined for evidence
of right ventricular hypertrophy. Measurement of
height and weight and comparison with standardized
growth charts may confirm failure to thrive, espe-
cially if the child is monitored over time. Feeding
difficulties are common in children with syndromic
craniosynostoses, and failure to thrive may be at-
tributed to this cause alone, but SDB is also an im-
portant cause and should be considered.
Respiratory sleep studies are not usually per-
formed before adenotonsillectomy for normal chil-
dren with symptoms and signs of OSAS or upper
airway resistance syndrome, but they are recom-
mended by the American Thoracic Society [56] for
several more complex situations, including cranio-
facial anomalies. It has been estimated that as many
as 85% of children with syndromic craniosynostosis
who have not had corrective surgery have some
degree of upper airway obstruction during sleep, and
in 61% of these children it is clinically significant
(moderate to severe) [42]. Formal sleep studies
should be used on a regular basis to evaluate the
function of the airway for this group of children.
Respiratory sleep studies
There is general agreement that sleep-related
breathing disorders must be documented objectively,
but controversy remains over the type of investigation
needed, what to look for, and what degree of
abnormality constitutes a problem worth treating.
Traditional polysomnography typically includes
documentation of the following:
! gross sleep architecture and arousals derived from
neurophysiologic recordings (EEG, EOG, EMG)
! respiratory patterns discerned from recordings
of oronasal airflow (eg, apnea, hypopnea), and
560 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
from ribcage and abdominal movements (respi-
ratory effort)
! ECG to give information about heart rate
changes (eg, increased heart rate with arousal,
reflex bradycardia with hypoxia) and dysrhyth-
mias (possibly stimulated by hypoxia)
! upper airway obstruction, as evidenced by
snoring, monitored by direct sound recordings
or from characteristic patterns on the oronasal
flow tracing
! changes in arterial blood gases (SaO2 and
noninvasive arterial PCO2), which give mea-
sures of the adequacy of ventilation
! posture, which may be important because upper
airway obstruction may be exacerbated in
certain positions
! leg movements documented from EMG record-
ings from an anterior leg muscle Because
periodic leg movements are an example of a
nonrespiratory cause of sleep fragmentation
The analysis of respiratory events is time consum-
ing and requires considerable skill and experience.
International recommendations
The American Thoracic Society has published two
official statements on cardiorespiratory sleep studies
in children [56,57]. In relation to craniofacial
patients, sleep studies are recommended in the
following circumstances:
! to differentiate benign/primary snoring (ie,
snoring not associated with apnea or hypoventi-
lation) from pathologic snoring (associated with
partial or complete airway collapse, dipping
SaO2 and sleep disruption)
! to investigate disturbed sleep patterns, excessive
daytime sleepiness, cor pulmonale, failure to
thrive, and unexplained polycythemia, espe-
cially in the presence of snoring
! to investigate a child with observed snoring and
clinically significant airway obstruction (eg,
apnea, retractions, paradox), either observed or
on video record, to confirm the diagnosis of
OSAS or optimize treatment
! to determine if the level of OSAS is severe
enough to warrant surgery or anticipate that the
child may need intensive postoperative care after
adenotonsillar or pharyngeal surgery; there is an
increased surgical risk in children younger than
2 years old, children with a respiratory distur-
bance index of more than 10 events per hour,
and children with craniofacial anomalies, par-
ticularly midfacial hypoplasia, retrognathia, and
micrognathia [58 – 61]
! to recommend follow-up sleep studies in chil-
dren previously diagnosed with OSA in whom
symptoms persist after therapy; a sleep study
should be delayed at least 4 weeks after any
surgery to allow postoperative edema to resolve
! to follow-up and review patients with OSAS who
were treated with continuous positive airway
pressure (CPAP) or a nasopharyngeal airway
! to ensure that children with mild to moderate
OSAS with complete resolution of snoring and
disturbed sleep patterns after therapy do not
require follow-up sleep studies; however, for
children with severe OSAS or who are younger
than 1 year of age, follow-up sleep studies can
assure resolution of clinically significant abnor-
malities [62]. Regardless of whether a sleep
study is ordered, the parents and primary care-
givers should understand the signs and symp-
toms of a recurrence of OSAS. The child should
undergo routine clinical assessments to ensure
early detection of the recurrence of OSAS.
Simpler alternatives to full polysomnography
Historically, respiratory sleep studies grew out of
neurophysiologic sleep studies. Although still con-
troversial, there is increasing support for the idea
of simplified sleep studies for the assessment of
respiratory problems. The idea is attractive from
practical and financial points of view and for the time
involved. In the pediatric field, the idea of simpler,
less invasive studies is attractive. The question is how
simple a study can be for it to retain the sensitivity
and specificity of the gold standard polysomnogram.
In the real world, a balance is needed between that
which is ideal and that which is feasible, practical,
and clinically acceptable. Clinically, a test must iden-
tify correctly the individuals whose disease severity
warrants treatment and can be used to follow-up these
patients after therapy to confirm the effectiveness of
therapy. In reality, there is little agreement between
sleep centers as to what constitutes the ideal limited
sleep study. Most researchers would agree, however,
that the aims of any limited sleep study should allow
the assessment of three factors: (1) respiratory effort
(ribcage and abdominal movements, snoring, or
possibly a high-quality video recording), (2) respira-
tory efficacy (eg, SaO2 or end-tidal PCO2), and
 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
(3) sleep fragmentation (EEG, or acute heart rate
rises, acute blood pressure rises, or gross body
movement from leg EMG, pressure-sensitive mattress
or high-quality video recording).
Oximetry alone, which measures SaO2 and pulse
rate, is an unreliable tool in the investigation of SDB.
It may miss patients with significant upper airway
obstruction who readily arouse. These individuals
may have all the symptoms of OSAS, including
fragmented sleep and daytime consequences, but do
not show abnormalities from these limited sleep
studies. A study that compared the analysis of
oximetry alone with full polysomnography showed
that a SaO2 study that shows significant periods of
hypoxemia during sleep is clinically significant. The
absence of hypoxemia cannot be used to exclude
OSAS, however [53]. Many symptomatic children do
not desaturate, although they may demonstrate
hypercapnia, partial upper airway obstruction, and
marked sleep disturbance on polysomnography.
Follow-up studies to monitor progress or response
to treatment may be possible using oximetry alone.
Interpretation
The interpretation of a sleep study involves the
integration of the patterns seen in the sleep study
recording with the information available from the
patient’s sleep history and physical examination.
OSAS is not an all-or-nothing condition; rather, it
represents a continuum that ranges from normality
through different degrees of severity. A system of
classification that categorizes the patient, based on
clinical observations of the sleeping child, can be
used together with the sleep study results. Using these
two measures, one can classify an individual as
having normal breathing during sleep, mild OSA,
moderate OSA, or severe OSA [42].
Although the use of rigid indices of severity of
OSA (eg, apnea/hypopnea index or SaO2 dip/h) with
arbitrary cut offs between normality and disease (eg,
normal less than one obstructive apnea/h [56] or three
or fewer SaO2 dip/h [36]) may be appropriate in
describing patient groups in research studies, they are
probably oversimplistic when dealing with individual
patients. Although age-related normative data exist
for numerous respiratory variables [56,57,63], inter-
pretation of clinical sleep studies requires a more
flexible, intuitive approach. Researchers who ana-
lyze, interpret, and report the sleep studies need
considerable experience and, preferably, knowledge
of individual patients.
561
Treatment
Treatment of symptomatic SDB in children
depends on the cause and severity, the age of the
patient, family and social circumstances, and patient
compliance with treatment. Intervention may be
mandatory in severe cases in which there is risk of
cardiopulmonary complications. It also may be help-
ful for snoring children with mild abnormalities on
sleep study when significant daytime symptoms that
may be the result of nocturnal upper airway
obstruction coexist. The challenge for the individual
patient is in comparing the risks and complications
of any planned intervention against the risks of
leaving the condition untreated.
In adults, weight reduction and pharmacotherapy
are therapeutic options, but this is not the case with
children. Treatment attempts to relieve or bypass the
obstruction. Theoretically, available options include
nasopharyngeal airways, adenotonsillectomy, uvulo-
pharyngopalatoplasty, splitting of the soft palate with
or without resection of the posterior palate margin,
nasal CPAP, tracheostomy and LeFort III osteotomy,
and maxillary advancement or monobloc advance-
ment to increase the diameter of the pharynx. After
surgical correction of the obstructed airway, close
postoperative monitoring in the first 24 to 48 hours is
essential because the correction of the blood oxygen
and carbon dioxide levels may affect respiratory
control centers, which results in respiratory arrest.
A nasopharyngeal airway can be useful in the first
few months of life, when an infant is an obligate nasal
breather, if there is significant choanal stenosis. It
requires regular suction to remain patent and regular
changing. It is associated with feeding difficulties,
especially nasal regurgitation of milk. The older child
does not tolerate such an intervention. Occasionally,
however, the nasopharyngeal may be useful on a
temporary basis after cleft palate repair until post-
operative edema has settled. It also may help on a
longer term basis to bypass obstruction at the level of
the tongue base, particularly if inserted only at night,
thus avoiding daytime cosmetic and feeding issues
(S. Leighton, unpublished data). The ideal position of
the airway is with its tip lying just above the epi-
glottis. There is a positive correlation between the
length of the tube and the crown/heel length, but clini-
cal assessment can confirm optimal placement [64].
Adenotonsillectomy is the standard treatment for
childhood SDB generally; it results in decreases in
respiratory disturbance and the number of arousals
[65]. Although the pathophysiology of SDB in
children with syndromic craniosynostosis is multi-
factorial, adenotonsillectomy may be beneficial
562 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566

regardless of whether adenotonsillar hypertrophy is lance with regular suction and tube changes. There
present. Even a small increase in pharyngeal airway are adverse effects on feeding, speech and language
diameter may alter the airflow dynamics and raise the development, education, and socialization. Children
critical closing pressure. In affected children, SDB with syndromic craniosynostosis already may have
may persist after adenotonsillectomy but may be less significant handicaps in these areas. We endeavor to
severe (S. Leighton, unpublished data). Factors that avoid long-term tracheostomy in this group of
predict which children may benefit from adenoton- patients; however, it is sometimes unavoidable. Ten
sillectomy remain to be established. of 147 children with a diagnosis of Apert, Crouzon,
Uvulopharyngopalatoplasty is not widely used or Pfeiffer syndrome underwent tracheostomy in the
to treat childhood OSAS, although it has been Great Ormond Street Hospital series (S. Leighton,
successful in children with neuromuscular disorders unpublished data). This compares favorably with
[66]. Palate splitting with or without resection of the other series, in which rates as high as 48% have been
posterior palate margin is not used routinely either, reported [16]. Older children may require temporary
although it has been reported in syndromic cranio- tracheostomies for intraoperative and postoperative
synostosis [67]. It carries the potential for increasing airway management if intubation is difficult. Trache-
the problem of middle ear effusion and later speech
disorders and velopharyngeal incompetence, espe-
cially after maxillary advancement. Theoretically,
later closure of the pseudocleft, with or without Box 1. Anatomic sites of airway
pharyngoplasty, could help to correct these problems. obstruction in the craniofacial patient
Nasal CPAP is beneficial for patients who have
not received sufficient benefit from adenotonsillec- Nasal and nasopharyngeal
tomy or who are not suitable candidates for surgery
[68]. Positive pressure is used to stent the collapsing Nasal piriform aperture stenosis/
airway. The level of pressure required to eliminate holoprosencephaly
obstructive apneas and correct nocturnal ventilation Choanal atresia/CHARGE association
and oxygenation is determined in the pediatric sleep Midfacial hypoplasia
laboratory and must be serially evaluated and
adjusted as a child grows. Complications from nasal Apert syndrome
CPAP include irritation over the nasal bridge from Crouzon syndrome
the mask, nasal congestion, and noncompliance. Pfeiffer syndrome
Compliance has improved with advances in equip-
ment and mask design and can be further improved Oropharyngeal and hypopharyngeal
by intensive behavioral intervention [69]. The advan-
tages of nasal CPAP are that it is noninvasive and that Macroglossia
it is nocturnal, worn only during sleep. It may be
ineffective if the adenoids are obstructive or signifi- Beckwith-Wiedemann syndrome
cant choanal stenosis is present, which results in
insufficient airway patency. Adenoidectomy should Tonsillar hypertrophy
be considered before a trial of nasal CPAP. The pres- Mandibular hypoplasia
ence of a cleft palate or a submucous cleft with bifid
uvula is a relative contraindication to adenoidectomy Pierre Robin sequence
because of the risk of precipitating velopharyngeal Treacher Collins syndrome
incompetence; in these cases, a ‘‘limited’’ adenoid- Stickler syndrome
ectomy is sometimes recommended.
Tracheostomy is an effective method of bypassing Laryngeal
multilevel obstruction and always relieves OSAS.
It has been advocated as the first step in the Laryngeal atresia/web
management of all patients with severe craniofacial Laryngeal cleft (Opitz-Frias or
anomalies and breathing problems, regardless of G syndrome)
planned subsequent treatment [70]. Long-term pedi- Vocal cord paralysis (Mobius
atric tracheostomy carries significant risk in terms syndrome)
of morbidity and mortality, however. The tube is Congenital subglottic stenosis
subject to obstruction and requires constant vigi-
 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566 563

Hx & Exam
Sleep Study

Normal Mild Abnormality Mild Abnormality Moderate/Severe

Thriving Not Thriving Abnormality Raised ICP

Follow up Nasendoscopy/Laryngoscopy/Bronchoscopy
(to determine site of obstruction)

Adenotonsillectomy

Resolved/Mild Moderate Severe

Follow up No Rx CPAP

Review Nasopharyngeal Airway

Surgery

Co-morbidity Tracheostomy Osteotomies

Fig. 1. Guidelines for airway management in syndromic craniosynostosis.

ostomy is, however, mandatory if interdental wiring Guidelines

is being undertaken or if halo distraction is performed
in children on nasal CPAP.
Surgical correction of the midface by Le Fort III
osteotomies and maxillary advancement or monobloc
advancement may improve airway caliber, but the
benefit for SDB is unpredictable and may not be
maintained [71]. Conventional techniques using bone
grafts and titanium plates are associated with an
increased risk for revision surgery, which is often
necessary in adolescence for children who have had
early surgery, because growth is disordered and
relapse may occur. Modern techniques using dis-
traction osteogenesis may be equally or more
effective in correcting deformity without increasing
the risk of later surgery, however, and the early re-
sults for airway improvement are encouraging
(S. Leighton, unpublished data). The most successful
results for facial form and occlusion occur only after
facial growth is completed. It is our belief that early
craniofacial surgery ideally benefits children with at
least two functional problems rather than children
with airway problems alone.
Box 1 provides a list of common craniofacial
conditions and frequent sites of airway compromise.
Fig. 1 presents a suggested algorithm for diagnosing
and managing a child with a craniofacial condition.
These guidelines are a compilation of the clinical
practice at our respective institutions. The practitioner
for the individual patient can and should modify them
to fit the situation. Likely they will evolve, as will the
practice of this exciting field.
Summary
Airway obstruction in children with craniofacial
abnormalities can cause significant morbidity and
mortality. The status of the airway should be assessed
as part of the initial and follow-up clinical evalua-
tions. When abnormalities exist, an approach to
effective management can follow rational and effec-
tive guidelines.
564 S. Leighton, A.F. Drake / Oral Maxillofacial Surg Clin N Am 16 (2004) 555 – 566
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