Obstructive Sleep Apnea Diagnosis and Treatment - part 5 pptx

174 Robert and Argaud
an individually made interface is now seldom needed even if it remains probably
the best interface (10–15). There are currently four different types of interfaces: nasal
mask, facial mask covering the nose and the mouth, nasal pillows, and mouthpieces.
Nasal masks are predominantly used (10,16). Mouthpieces are now essentially
indicated in the case of daytime ventilation (17,18). This may afford an excellent inter-
face to adjunct daytime ventilation in neuromuscular patients who are unable to main-
tain acceptable diurnal arterial blood gases without frequent intermittent periods of
assistance. The mouthpiece is positioned close to the patient’s mouth where it is inter-
mittently captured to take a few assisted breaths from the ventilator and subsequently
released. An advantage is to clear the face from a face-attached interface. Thus, the
patient needing assistance night and day may use a combination of interfaces.
Ventilator and Mode for Noninvasive Positive Pressure Ventilation
Ventilators use one of two basic methods: volume-preset and pressure-preset (10).
With volume-preset, the ventilator always delivers the tidal volume which is set by
the clinician, regardless of the patient’s pulmonary system mechanics (compliance,
resistance, and active inspiration). However, leaks at the skin–mask interface or
through the mouth when using a nasal mask, reduce the volume received by the
patient. Conversely, with pressure-preset, changes in pulmonary mechanics directly
influence the flow and the delivered tidal volume (lower or higher) since the venti-
lator delivers the set pressure all along inspiration. Then leaks augment the flow
and tend to maintain the tidal volume (19,20). It is important to understand that
NIPPV is dominated both by rapid variations of nonintentional leaks and of the
geometry and the resistance of the upper airway (21). Obviously, leaks and airway
resistance partly interact. Facing these continuous changes the respective advan-
tages and drawbacks of volume- and pressure-preset modes, which are opposite,
make a predictable effect difficult. The way to begin and end inspiration is either
initiated by the ventilator or in response to a patient effort to do so, allowing one to
define the main modes: (i) control (ii) assist-control (iii) assist or spontaneous (pos-
sible only with pressure-preset). Most of the home ventilators function uniquely in
volume or in pressure preset but modern ones may deliver inspiration according to

the two modes. Besides the classical circuitry including two valves (on the inspira-
tory and expiratory limbs) alternatively closing and opening, bilevel positive airway
pressure (BPAP) ventilators are simpler and therefore lend themselves to home
mechanical ventilation (22). Inspiratory and expiratory pressures are alternatively
established in a single circuit incorporating an intentional, calibrated leak located
close to the patient or even on the mask. The theoretical disadvantage with such a
circuit is the risk of a variable CO
2
rebreathing. Concern about the risk of CO
2

rebreathing is not definitively documented (23–25) even if the trend is to consider it
as negligible (26–28) provided positive expiratory pressure is applied in order to
eliminate CO
2
through the intentional leak (at least 2–4 cm H
2
O). Depending on the
ventilator, all the different modes and refined settings and even closed-loop modes
usually applied in the intensive care unit, are more or less available. Some ventilators
may analyze ventilation in an on-going manner, keep it in an internal memory and
provide the data for further assessment. The general objective is to provide many
possible capabilities in order to have enough tools to adapt and optimize patient–
machine synchronization. While conceptually attractive, sufficient studies have not
been performed to document or refute the advantages of such complexity in the
context of noninvasive home ventilation.
Noninvasive Positive Ventilation 175
Choice of the Ventilator and Mode
Many clinicians currently prefer a pressure-preset ventilator in assist mode as the
first choice with the view to offer the better synchronization (9). In fact, in the stud-

ies comparing volume- and pressure-preset ventilators no clear differences in the
correction of hypoventilation in short-term studies (29–37) and in long-term out-
comes (38–40) are shown. This is understandable since during NIPPV, leaks and
resistance changes alternate very quickly and when the pressure target does well,
the volume target does not do well, and conversely. However, it is important to
remain flexible by trying alternative approaches if problems occur with one or the
other type of ventilator. Besides, it should be noted that batteries are unavailable or
they offer a short autonomy for BPAP ventilators and this would limit security and
mobility of neuromuscular patients with hypoventilation and then drive the prefer-
ence to the volume ventilator.
CRITERIA TO DISCUSS NONINVASIVE POSITIVE
PRESSURE VENTILATION
Signs and Symptoms of Hypoventilation
The presence of clinical symptoms and/or physiologic markers of hypoventilation
are useful in identifying clinical severity as it relates to therapeutic decision-making
with regard to initiation of nocturnal NIPPV. In the course of a typical progressive
disease, two successive steps occur more or less rapidly: (i) nocturnal hypoventilation
reversible during wake associated with none or a few clinical symptoms; and (ii) noc-
turnal and daylight hypoventilation associated with clinical symptoms, which show
a low respiratory reserve and should be considered an unstable state with increased
susceptibility to life-threatening acute ventilatory failure that may be triggered by
what may otherwise be trivial additional factors (41,42). A sleep study continuously
recording CO
2
(end-tidal EtCO
2
or transcutaneous TcCO
2
) and/or oxygen saturation
(SpO

2
) is required to document nocturnal hypoventilation, which may occur through-
out all sleep stages but in some cases exclusively during rapid eye movement (REM)
sleep. Daytime hypoventilation is defined by an abnormally elevated

partial pressure
of arterial carbon dioxide (PaCO
2
), a high-serum bicarbonate level and a relatively
normal pH with associated reduction of partial pressure of arterial oxygen (PaO
2
).
Chronic daytime hypoventilation is an important indicator invariably associated with
sleep-related hypoventilation. Thus, in the presence of diurnal hypoventilation, the
reason for overnight recording is only to rule out obstructive or central apnea. Clinical
symptoms indicating consequences of hypoventilation (Table 1) must be carefully
evaluated since even when modest, they are important for the appreciation of disease
severity and prognosis and to indicate NIPPV. Pulmonary function tests help define
and quantify the ventilatory–respiratory disease but have low predictive values for
chronic sleep-related hypoventilation in individual patients except in those with neu-
romuscular disease. Indeed, in Duchenne muscular dystrophy, hypoventilation
appears only during REM sleep, all night, or during the daytime when supine inspira-
tory vital capacity is < 40%, < 25%, and < 12%, respectively (41). Similarly a peak
cough flow < 160 L/min, related to expiratory muscle deficit, means an increased risk
of accumulation of secretions that may worsen hypoventilation and trigger acute fail-
ure (18,43–46). It is crucial to notice that isolated reduced PaO
2
does not mean
hypoventilation but only a mismatching of ventilation and perfusion adequately
compensated or even overcompensated (low PaCO

2
), which will not require support
by mechanical ventilation but only by supplemental oxygen.
176 Robert and Argaud
Diseases That May Potentially Be Treated with
Noninvasive Positive Pressure Ventilation
The principal diseases which may be addressed using NIPPV therapy are shown in
Table 2. Except for those due to respiratory control or upper airway abnormalities,
all may become severe enough to cause alveolar hypoventilation during sleep and
daytime and eventually may impair quality of life and threaten life. In neuromuscular
disorders it is important to consider the progressiveness according to each type of
disease and the individual concerned.
Survival with Noninvasive Positive Pressure
Ventilation in Different Diseases
NIPPV efficacy in terms of survival compared to control treatment is major information
that one needs to adequately discuss NIPPV. Besides a few randomized control
trials (47–50), information comes from retrospective series compared to the usual
prognosis (14,40,51–58). In order to extend the analysis it is also possible to take into
TABLE 1 Clinical Features Frequently Associated with Alveolar Hypoventilation
Shortness of breath during activities of daily living in the absence of paralysis
Orthopnea in patients with disordered diaphragmatic dysfunction
Poor sleep quality: insomnia, nightmares, and frequent arousals
Nocturnal or early morning headaches
Daytime fatigue, drowsiness and sleepiness, loss of energy
Decrease in intellectual performance
Loss of appetite and weight loss
Appearance of recurrent complications: respiratory infections
Clinical signs of cor pulmonale
TABLE 2 Main Diseases That Can Benefit from Noninvasive Positive Pressure Ventilation,
Classified According to the Cause and Progressiveness of the Respiratory Impairment

Parietal disorders: (PFT abnormal:  VC,  FEV1, → FEV1/VC,  RV,  TLC)
a
Chest wall:
Kyphoscoliosis No worsening
Sequels of tuberculosis Slow worsening
Obesity hypoventilation syndrome Depends on obesity
Neuromuscular disorders:
Spinal muscular atrophy No worsening
Acid maltase deficit
Slow worsening (>15 years)
Duchenne muscular dystrophy Intermediate worsening (5–15 yrs)
Myotonic myopathy Intermediate worsening (5–15 yrs)
Amyotrophic lateral sclerosis Rapid Worsening (0–3 yrs)
Lung diseases: (PFT abnormal: → or  VC,  FEV1,  FEV1/VC,  RV,  TLC)
a
COPD Continuous worsening
Bronchiectasis, Cystic fibrosis Continuous worsening
Predominant ventilatory control abnormalities: (PFT normal)
Ondine’s curse Improvement (?)
Cheyne-Stokes breathing Depends on heart failure
Upper airway abnormalities: (PFT normal)
Pierre Robin No worsening
Obstructive sleep apnea No worsening
a
Symbols indicate actual compared to theoretical values:  or , decrease or increase; →, normal.
Abbreviations: COPD, chronic obstructive pulmonary disease; FEV1, forced expiratory volume in one second;
PFT, pulmonary function test; RV, residual volume; TLC, total lung capacity; VC, vital capacity.
Noninvasive Positive Ventilation 177
account the results obtained either with negative pressure ventilation (59) or with
tracheostomy (4,60). Their conclusions are informative enough and generally

accepted by the medical community even if these conclusions are refutable in terms
of evidence-based medicine. In neuromuscular disease, NIPPV always increases
survival. Approximate median prolongations of life depend on the age when starting
NIPPV and the comorbidities (including extended paralysis): very long (> 20 years)
in the sequelae of poliomyelitis; long (10 years) in spinal muscular atrophy (SMA)
type 2 and 3, Duchenne muscular dystrophy, acid maltase deficiency; short in
myotonic dystrophy (4 years); and very short in amyotrophic lateral sclerosis (ALS)
(one year). In chest-wall abnormalities NIPPV also prolongs life: in kyphosis
(15 years) and in the sequelae of tuberculosis (seven years). In lung diseases no data
support a positive effect on survival: in chronic obstructive pulmonary disease
(COPD) patients for whom randomized trials are negative (48,49,59) or in cystic
fibrosis or bronchiectasis patients for whom data are too scarce.
Circumstances and Indications for Noninvasive
Positive Pressure Ventilation
In clinical practice, NIPPV is initiated either electively or in the context of acute ven-
tilatory failure initially treated invasively with translaryngeal intubation or nonin-
vasively with facial interfaces (61). In the latter circumstances, the long-term
necessity for NIPPV should be re-evaluated after weeks or months during follow-up
since the indications for NIPPV may change as the clinical conditions improve or
not. In cases of chronic and stable awake hypoventilation, the cornerstone to foresee
use of NIPPV is an advanced severity with clinical symptoms of hypoventilation
plus a balance of several other issues: (i) the main primary process explaining the
hypoventilation: mechanical or lung deficit; (ii) the natural rate of progression
appreciated as a few years or dozens of years; (iii) the clinical severity at the time of
decision-making: actual symptoms and history of acute–subacute failure in the pre-
vious months; and (iv) the patient’s willingness, including the family and social
environment, to undertake this therapy. Indications are outlined in Table 3. NIPPV
is strongly indicated in patients with chest-wall and neuromuscular disorders in the
presence of clinical symptoms attributable to diurnal hypoventilation (18,62–67).
There are no validated values above which NIPPV is definitely indicated; however,

TABLE 3 Typical Indications for Nocturnal Noninvasive Positive Pressure Ventilation
According to Disease Process and Severity
Diseases
Symptoms
Day CO
2

a
Symptoms
Night CO
2

a
No symptoms
Day CO
2

a
Usual daily
duration of NIPPV
(hrs)
Scoliosis Yes Yes Perhaps
<12
Tuberculosis Yes Yes Perhaps
<12
Neuromuscular stable or slow Yes Perhaps Perhaps 18–24
Neuromuscular intermediate Yes Perhaps Perhaps 18–24
Neuromuscular rapid Yes Yes Yes 24
COPD Perhaps No No 12
Bronchiectasis, Cystic fibrosis Perhaps No No 18–24

Obesity-hypoventilation Perhaps Perhaps No
<12
a
, increase.
Abbreviations: COPD, chronic obstructive pulmonary disease; NIPPV, noninvasive positive pressure ventilation.
178 Robert and Argaud
many clinicians consider treatment in scoliosis and sequelae of tuberculosis with
awake PaCO
2
> 50–55 mmHg and a PaO
2
< 60 mmHg, and in neuromuscular dis-
ease, a PaCO
2
around 45–50 mmHg and a PaO
2
< 70 mmHg. In case of clear clinical
symptoms less severe values may be considered as an indication to start NIPPV
(65). Conversely, in COPD and probably in other lung diseases diurnal hypoventila-
tion do not support the unequivocal utility of NIPPV (68,69). Nevertheless, this
question remains open since the clinical trials are underpowered and secondary
parameters like some components of the quality of life or hospitalization days, may
have improved. Some observational series suggest better results (70–73). Presently,
we may admit NIPPV as an option in COPD patients with symptoms of hypoventi-
lation contributing to recurrence of acute–subacute failure, provided that long-term
oxygen and drug therapy have already been optimally adjusted. During early stages
with only isolated nocturnal hypoventilation, NIPPV is not mandatory but could be
optional in kyphoscoliosis (74,75) and in neuromuscular diseases (75). In the latter,
when worsening is both inevitable and rapid (e.g., ALS), NIPPV is valuable at an
early stage provided that this is an acceptable therapeutic option for the patient.

Other particular diseases may also deserve considerations related to NIPPV
use even if clinical experience remains nonconclusive. Obesity hypoventilation syn-
drome is dominated by morbid obesity impeding ventilation, frequent obstructive
apnea and more or less reversible decreased reactivity of the respiratory centers
(76). In acute–subacute as in chronic situations, NIPPV has been shown to reverse
hypoventilation (77–80). But, considering the high prevalence of obstructive apnea,
CPAP is also a simpler and efficient treatment. Cheyne-Stokes breathing with central
and obstructive apnea in the context of severe cardiac insufficiency has been shown
to negatively worsen the clinical situation and survival (81–83). Conventional NIPPV
or a new modality such as adaptive servo-ventilation has been shown to alleviate
apnea and improve cardiac function (84–89). Nevertheless, no conclusion about the
utility of nocturnal NIPPV in terms of survival and main outcomes is available. Even
more, a large study comparing O
2
and CPAP, which also alleviates apnea and improves
cardiac function does not prove the clinical superiority of CPAP in term of survival
even if apneas are significantly diminished (90). Pure obstructive apneas in the con-
text of OSA could be suppressed with NIPPV. Some authors have proposed NIPPV as
a second-line treatment in the case of CPAP failure. Such a possibility is not supported
with enough conclusive study to be recommended (91–93). Ondine’s curse, in chil-
dren, is characterized by the lack of metabolic response of the respiratory centers
during sleep and is responsible for severe nocturnal hypoventilation. The usual treat-
ment is tracheostomy and nocturnal ventilation. Some clinical experience suggests
that, after years, tracheostomy might be converted in some cases to nocturnal NIPPV.
Obviously such options must remain in the hand of specialized teams (94).
MANAGEMENT OF NONINVASIVE POSITIVE
PRESSURE VENTILATION
Initiation and Settings in the Case of Nocturnal Ventilation
The main goal of NIPPV, used at best uniquely during the night, includes the
provision of improvement in arterial blood gases nearly up to normal values with-

out discomfort and sleep disruption. The objective in case of a residual muscle
ability to breathe is to provide enough improvement to allow comfortable time off
the ventilator. Even if there is no absolute recommendation it is good general prac-
tice to proceed in three steps. The first step consists of selecting and adjusting the
ventilator settings while the patient is awake, insuring physiological adequacy,
Noninvasive Positive Ventilation 179
and patient comfort for at least one or two hours. One study, done on awake cystic
fibrosis patients, found that clinical observation is as efficient as the use of physio-
logical measurement including esophageal pressure in setting the ventilator param-
eters (95). Another study in patients with COPD and neuromuscular disease has
shown that using physiological measurement does not improve ventilation during
the day but improves ventilation and sleep quality during the night (96,97). In the
second step, the clinician should judge adequacy when sleeping during a nap and/
or nocturnal use. To complete this step, different options according to the resources
available in each center could be used. Arterial blood gas measurements would
seem ideal; however, one or few samples during the night do not represent the
rapid changes observed during several continuous hours of sleep, and the inva-
siveness of sampling have led most clinicians to noninvasively monitor different
parameters. Ideally, a complete polysomnogram recording SpO
2
and PtcCO
2
or
PEtCO
2
, airflow, tidal volume, airway pressure, rib cage and abdomen excursion,
and sleep staging permits a complete assessment (98). When resources are not
available to perform these detailed recordings, fewer measurements during over-
night recordings remain informative. However, the minimal requirement is to
overnight record SpO

2
in room air assessing that the normalization of SpO
2
goes
with the normalization, or at least the improvement of PaCO
2
. In addition, data
related to patient tolerance, comfort, sleep quality, and well-being should be
obtained. The third step consists in looking for reduction of PaCO
2
and augmenta-
tion of PaO
2
, without dyspnoea, during the day in free ventilation after several
NIPPV nights to confirm that the settings are adequate. This also gives information
about the necessity or not to add daylight hours of NIPPV (at first during the nap
and more when necessary). If the results are not satisfactory, alterations must be
made to the settings and possibly the mask and the ventilator, and their effects
checked again. In most cases, a few days are necessary to succeed.
If one uses assist pressure-preset ventilation, 10 cm H
2
O of inspiratory pressure
support is a suggested starting point. If necessary, the pressure level is progressively
increased to achieve evidence of improvement. Pressure support higher than 20 cm H
2
O is
rarely necessary. Nevertheless, one observational series reports good results in COPD
patients ventilated with higher (28 cm H
2
O) pressure (73). In COPD, the addition of an

expiratory positive pressure [positive end-expiratory pressure (PEEP) or expiratory
positive airway pressure], also necessary to decrease the rebreathing with BPAP venti-
lators, should conceptually improve patient triggering when intrinsic PEEP exists. But,
there is no long-term study proving its clinical usefulness (99,100). Depending on the
ventilator capabilities and observations made of how patient and ventilator do together,
more subtle settings concerning triggers, initial flow, and inspiratory time limit could be
tried. A backup frequency set close to the spontaneous frequency of the patient during
sleep is a reasonable substitute to avoid central apnea induced by transitory but
repeated hyperventilation overpassing the apnea threshold (101).
When employing a volume-preset ventilator, the initial suggested settings may be
established by adjusting the frequency of ventilator-delivered breaths so that it approxi-
mates the patient’s spontaneous breathing frequency during sleep, an inspiratory time/
total breathing cycle time between 0.33 and 0.5 and a relatively high tidal volume of
around 10 to 15 mL/kg to insure sufficient tidal volume in case of leaks (19).
Supplemental O
2
should be added into the ventilator circuit in those patients
requiring oxygen while awake due to lung parenchyma diseases (e.g., COPD, cystic
fibrosis, bronchiectasis). In the absence of parenchymal disease it is only after trying
to optimize all technical parameters that residual desaturation may justify addi-
tional O
2
bled into the ventilator circuit during sleep (102,103).
180 Robert and Argaud
Continuous Noninvasive Positive Pressure Ventilation
In neuromuscular (to a lesser degree in end-stage lung diseases) the ventilator
dependency may be total when starting NIPPV or may progressively increase
following the gradual worsening of the disease. In the case of continuous need for
ventilation, NIPPV could be used provided that the following techniques are
adapted: alternate interfaces night and day, and assisted coughing available

(18,104–106). Only a very well-trained team may take in charge of such an approach
in patients who are completely informed and conscious of the constraints and
dangers. Such application has been reported by different teams in stable neuro-
muscular patients, such as those with a sequelae of poliomyelitis, high-level spinal
cord injury or Duchenne muscular dystrophy (1,17,107). Alternatively, a tracheos-
tomy may be performed to facilitate ventilatory assistance and secretion removal.
There is no clear answer as to whether and beyond what duration a quite totally
ventilator-dependent patient is better or more safely ventilated by tracheostomy or
NIPPV (65,108–111). This debate will probably continue and, in the end, the
decision to indicate or to convert to tracheostomy is highly dependent on the phi-
losophy and capabilities of the clinical team as well as that of the patient and his/
her family environmental preferences. It is essential that discussion of such issues
be started as early as possible in the patient’s course, well before the imperative
arises. Besides, swallowing dysfunction, responsible for frequent and massive
aspirations and pneumonia, observed during the course of ALS (frequent and due
to bulbar origin) or of Duchenne muscular dystrophy (seldom and due to muscle
weakness), is an imperative indication for tracheostomy to prolong survival, but it
also raises major difficulties to communicate and to have enough personal
interactions (locked-in state) (110,112). From this point of view, NIPPV, which may
be easily stopped, could be a reasonable maximal option in case of rapidly devas-
tating diseases like ALS, and can be considered both by the patient and medical
team as a limitation of care or a palliative approach (113,114). This was confirmed
since NIPPV in ALS patients with bulbar symptoms do not survive longer than
controls (50).
Follow-up
Clinical follow-up and daytime arterial blood gas (ABG) measurements (or their
surrogates) should be conducted regularly (two times per year for example). When
possible, recordings during sleep on NIPPV, identical to those used for initiating
NIPPV are useful. At any time, when there are unsatisfactory results like recurrence
of clinical symptoms or hypoventilation on ABG, inadequate NIPPV must be sus-

pected and objective evaluation during sleep must be undertaken. At the very least,
overnight oximetry must be done. When NIPPV is determined to be suboptimal, a
change in ventilator modality or setting and a review of the mask fitting may be
indicated. Increasing the total duration of NIPPV use per day should also be considered,
particularly when the underlying disease has progressed. Masks have to be regularly
checked and changed or adapted as needed.
Management of Complications
Air Leaks During Noninvasive Positive Pressure Ventilation
To some degree, leaks are present when using nasal NIPPV during sleep in all
patients. The major potential adverse effects of such leaks are reduced efficiency of
ventilation and sleep fragmentation (115–118). A variety of measures, more or less
Noninvasive Positive Ventilation 181
efficacious, have been suggested to address problematic leaks. These include: pre-
venting neck flexion, reclining in a semi-recumbent position, discouraging the
mouth from opening by use of a chin strap (117) or a cervical collar, switching to
pressure-preset mode (19), decreasing the peak inspiratory pressure, increasing the
delivered volume (20), optimizing the interface (12,16), and possibly switching to
nasal pillows or a full-face mask (119). The effectiveness of these measures must be
confirmed during sleep recordings.
Nasal Dryness, Congestion, and Rhinitis
With reference to the CPAP literature, the side effects of nasal dryness, congestion,
and rhinitis are related to a defect of humidification promoted by air leaks (120). For
patients with nasal and mouth dryness, a cold passover or a heated humidifier (the
latter is more effective) (121) can be used. Heat/moisture exchangers are not well
adapted to the case of leaks since the “dry” flow from the ventilator is higher than
the “dampened” flow returning from the patient. In a large series, a minority of
patients needed humidifiers (10).
Aerophagia
Aerophagia, or swallowing air, is frequently reported by patients, but rarely
intolerable (122). Minor clinical signs are: eructation, flatulence, and abdominal dis-

comfort. Aerophagia is usually dependent on the level of inspiratory pressure and
is more commonly seen when using volume and/or mouthpiece ventilation and in
the care of patients with neuromuscular disease. The incidence decreases if the peak
inspiratory pressure is kept below 25 cm H
2
O pressure.
NONINVASIVE POSITIVE PRESSURE VENTILATION EFFECTS
(OTHER THAN SURVIVAL) AND RELATED MECHANISMS
During Ventilatory Assistance
As expected, when under NIPPV, ventilation and gas exchange are improved in all types
of disease (38,70,74,123–127), even if significant episodes of transient hypoventilation,
related to mouth leaks, may appear (115–118). Duration of sleep is augmented without
clear changes in its quality (115,116,128). Respiratory muscles are normally put at rest but
there are many exceptions due to air leaks and patient-ventilator asynchrony (129–131).
After Ventilation
When spontaneous ventilation exists and in the absence of major lung disease, gas
exchange remains improved. It may persist for hours and even days before reap-
pearance of hypoventilation (132,133). The improvement reported in many studies
is important in chest-wall and neuromuscular diseases but inconsistent in COPD
(123,125,134,135). Certainly, it explains improvements in clinical symptoms such as
general well-being, appetite, exercise capability, headaches, ankle edema, and resur-
gence of acute failure, as well as decreasing hospitalization, increasing quality of life
(136–138) and finally improving survival.
Three main explanations have been proposed: (i) improved respiratory muscle
strength; (ii) resetting of the chemoreceptors; and (iii) decrease of the ventilatory
load. The first hypothesis suggests that ventilatory assistance rests the respira-
tory muscles reversing fatigue. Indeed, inspiratory force [PI
max
(maximum insp-
iratory pressure) or P

es
(esophageal pressure) during sniff nasal pressure testing]
have been found significantly augmented in four studies (56,139–141), very close in
182 Robert and Argaud
one (142) and stable in one (143). One study in which a nonvolitional objective mea-
sure using bilateral anterolateral phrenic nerve magnetic stimulation was assessed
and found to be negative for improvement (142). One study in which respiratory
muscle endurance was measured showed significant improvement (139). The second
hypothesis suggests that, in response to chronic hypercapnia and hypoxia, the che-
moreceptors commanding the respiratory centers change their set point, which per-
petuates hypoventilation rather than attempting to generate nonsustainable
ventilatory muscle efforts (144–147). The resumption of better ventilation during
NIPPV would reset the centers to more normal values. The three studies that have
looked at the hypercapnic ventilatory response have actually found significant
improvement (140,142,143). It is interesting to consider that even a few hours of
NIPPV during daytime can have the same effect (72,148) indicating that the deter-
mining factor is to resume hypoventilation for a relatively short daily duration. The
third hypothesis suggests that an improvement of respiratory chest-wall and/or
lung compliance, under the effects of positive pressure ventilation, would reduce the
ventilatory load and increase the efficiency of the muscles. In the studies done on
scoliosis, vital capacity significantly increased (56,139–141); while in the other two
hypothesis, including also neuromuscular patients, the vital capacity remained
unchanged. In one study, chest-wall and lung compliance did not change even
though there was a nonsignificant trend toward an increase (142). In the three stud-
ies, periodic hyper-insufflation using higher inspiratory pressure during a few min-
utes in scoliosis (149,150) and ALS (151) patients, revealed an increase in compliance.
It seems probable that, even if the mechanisms which explain the efficacy of NIPPV
are imperfectly understood, it is likely that several factors, even if not individually
significant, change and interact together to improve alveolar ventilation. The mini-
mum mandatory duration of assistance is not clearly known. However, a relation-

ship between a decrease of PaCO
2
and the pressure to ventilate has been found (141).
Finally, one study reports a significant improvement of pulmonary arterial hyperten-
sion, which obviously favors clinical improvement (152).
In COPD patients, the absence of clinical results compared to scoliosis and
neuromuscular disease, even if resetting of the respiratory centers has been shown
(125,153), could be explained by the relatively low impairment of respiratory muscles
and the importance of the lesions of the lung itself and its progressiveness.
CONCLUSIONS
Chronic ventilatory support using NIPPV improves and stabilizes the clinical course
of many patients with chronic ventilatory failure. The results appear to be good in
patients with restrictive disorders and poor in COPD. Among the neuromuscular
disorders results are better in the slowly progressive ones. The benefit of NIPPV is
reflected by the improvements in survival, blood gas composition, and clinical sta-
bility. Due to its relative simplicity and its noninvasive nature, NIPPV permits long-
term mechanical ventilation to be an acceptable option to patients who otherwise
would not have been treated if tracheostomy were the only alternative. In this way,
nocturnal NIPPV represents a huge advance.
REFERENCES
1. Splaingard ML, Frates RC Jr, Harrison GM, et al. Home positive-pressure ventilation.
Twenty years’ experience. Chest 1983; 84:376–382.
Noninvasive Positive Ventilation 183
2. Baydur A, Layne E, Aral H, et al. Long term non-invasive ventilation in the community for
patients with musculoskeletal disorders: 46 year experience and review [see comments].
Thorax 2000; 55:4–11.
3. Duiverman ML, Bladder G, Meinesz AF, et al. Home mechanical ventilatory support in patients
with restrictive ventilatory disorders: a 48-year experience. Respir Med 2006; 100:56–65.
4. Robert D, Gerard M, Leger P, et al. Permanent mechanical ventilation at home via a tra-
cheotomy in chronic respiratory insufficiency. Rev Fr Mal Respir 1983; 11:923–936.

5. Bach JR, Alba AS, Bohatiuk G, et al. Mouth intermittent positive pressure ventilation in
the management of postpolio respiratory insufficiency. Chest 1987; 91:859–864.
6. Baydur A, Gilgoff I, Prentice W, et al. Decline in respiratory function and experience with long-
term assisted ventilation in advanced Duchenne’s muscular dystrophy. Chest 1990; 97:884–889.
7. Hill NS. Clinical applications of body ventilators. Chest 1986; 90:897–905.
8. Sullivan CE, Issa FG, Berthon-Jones M, et al. Reversal of obstructive sleep apnea by
continuous positive airway pressure applied the nares. Lancet 1981; 1:862–865.
9. Lloyd-Owen SJ, Donaldson GC, Ambrosino N, et al. Patterns of home mechanical venti-
lation use in Europe: results from the Eurovent survey. Eur Respir J 2005; 25:1025–1031.
10. Schonhofer B, Sortor-Leger S. Equipment needs for noninvasive mechanical ventilation.
Eur Respir J 2002; 20:1029–1036.
11. Hill NS. Saving face: better interfaces for noninvasive ventilation. Intensive Care Med
2002; 28:227–229.
12. Elliott MW. The interface: crucial for successful noninvasive ventilation. Eur Respir J 2004;
23:7–8.
13. Leger P, Jennequin J, Gerard M, et al. Home positive pressure ventilation via nasal mask
for patients with neuromuscular weakness or restrictive lung or chest-wall disease.
Respiratory Care 1989; 34:73–77.
14. Leger P, Bedicam JM, Cornette A, et al. Nasal intermittent positive pressure ventilation.
Long-term follow-up in patients with severe chronic respiratory insufficiency. Chest
1994; 105:100–105.
15. Fauroux B, Lavis JF, Nicot F, et al. Facial side effects during noninvasive positive pressure
ventilation in children. Intensive Care Med 2005; 31:965–969.
16. Leger SS, Leger P. The art of interface. Tools for administering noninvasive ventilation.
Med Klin 1999; 94:35–39.
17. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth
as an alternative to tracheostomy for 257 ventilators users. Chest 1993; 103:174–182.
18. Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular
dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004; 170:456–465.
19. Mehta S, McCool FD, Hill NS. Leak compensation in positive pressure ventilators: a

lung model study. Eur Respir J 2001; 17:259–267.
20. Tuggey JM, Elliott MW. Titration of non-invasive positive pressure ventilation in chronic
respiratory failure. Respir Med 2006; 100:1262–1269.
21. Jounieaux V, Aubert G, Dury M, et al. Effects of nasal positive-pressure hyperventilation
on the glottis in normal sleeping subjects. J Appl Physiol 1995; 79:186–193.
22. Strumpf DA, Carlisle CC, Millman RP, et al. An evaluation of the respironics BiPAP bi-level
CPAP device for delivery of assisted ventilation. Respir Care 1990; 35:415–422.
23. Ferguson GT, Gilmartin M. CO
2
rebreathing during BiPAP ventilatory assistance. Am J Respir
Crit Care Med 1995; 151:1126–1135.
24. Lofaso F, Brochard L, Touchard D, et al. Evaluation of carbon dioxyde rebreathing
during pressure support ventilation with airway management system (BiPAP) devices.
Chest 1995; 108:772–778.
25. Schettino GP, Chatmongkolchart S, Hess DR, et al. Position of exhalation port and mask
design affect CO
2
rebreathing during noninvasive positive pressure ventilation. Crit
Care Med 2003; 31:2178–2182.
26. Hill NS, Carlisle C, Kramer NR. Effect of a nonrebreathing exhalation valve on long-term
nasal ventilation using a bilevel device. Chest 2002; 122:84–91.
27. Hill N. What mask for noninvasive ventilation: is deadspace an issue? Crit Care Med 2003;
31:2247–2248.
28. Saatci E, Miller DM, Stell IM, et al. Dynamic dead space in face masks used with non-
invasive ventilators: a lung model study. Eur Respir J 2004; 23:129–135.
184 Robert and Argaud
29. Restrick LJ, Fox NC, Braid G, et al. Comparison of nasal pressure support ventilation
with nasal intermittent positive pressure ventilation in patients with nocturnal hypov-
entilation. Eur Respir J 1993; 6:364–370.
30. Meecham Jones DJ, Wedzichia JA. Comparison of pressure and volume preset nasal

ventilator systems in stable chronic respiratory failure. Eur Respir J 1993; 6:1060–1064.
31. Cinnella G, Conti G, Lofaso F, et al. Effects of assisted ventilation on the work of breath-
ing: volume-controlled versus pressure-controlled ventilation. Am J Respir Crit Care
Med 1996; 153:1025–1033.
32. Girault C, Richard JC, Chevron V, et al. Comparative physiologic effects of noninvasive
assist-control and pressure support ventilation in acute hypercapnic respiratory failure
[see comments]. Chest 1997; 111:1639–1648.
33. Tejeda M, Boix JH, Alvarez F, et al. Comparison of pressure support ventilation and assist-
control ventilation in the treatment of respiratory failure. Chest 1997; 111:1322–1325.
34. Lien TC, Wang JH, Huang SH, et al. Comparison of bilevel positive airway pressure and
volume ventilation via nasal or facial masks in patients with severe, stable COPD.
Chung Hua I Hsueh Tsa Chih (Taipei) 2000; 63:542–551.
35. Laserna E, Barrot E, Beiztegui A, et al. Non-invasive ventilation in kyphoscoliosis. A
comparison of a volumetric ventilator and a BIPAP support pressure device. Arch
Bronconeumol 2003; 39:13–18.
36. Tuggey JM, Elliott MW. Randomised crossover study of pressure and volume non-
invasive ventilation in chest wall deformity. Thorax 2005; 60:859–864.
37. Windisch W, Storre JH, Sorichter S, et al. Comparison of volume- and pressure-limited
NPPV at night: a prospective randomized cross-over trial. Respir Med 2005; 99:52–59.
38. Schonhofer B, Sonneborn M, Haidl P, et al. Comparison of two different modes for non-
invasive mechanical ventilation in chronic respiratory failure: volume versus pressure
controlled device. Eur Respir J 1997; 10:184–191.
39. Smith IE, Laroche CM, Jamieson SA, et al. Kyphosis secondary to tuberculosis osteomy-
elitis as a cause of ventilatory failure. Clinical features, mechanisms, and management.
Chest 1996; 110:1105–1110.
40. Janssens JP, Derivaz S, Breitenstein E, et al. Changing patterns in long-term noninvasive
ventilation: a 7-year prospective study in the Geneva Lake area. Chest 2003; 123:67–79.
41. Ragette R, Mellies U, Schwake C, et al. Patterns and predictors of sleep disordered
breathing in primary myopathies. Thorax 2002; 57:724–728.
42. Lo Coco D, Marchese S, Corrao S, et al. Development of chronic hypoventilation in

amyotrophic lateral sclerosis patients. Respir Med 2006; 100:1028–1036.
43. Bach JR. Mechanical insufflation, exsufflation. Comparison of peak expiratory flows with
manually assisted and unassisted coughing techniques. Chest 1993; 104:1553–1562.
44. Bach JR. Update and perspective on noninvasive respiratory muscle aids. Part 2: the
expiratory aids. Chest 1994; 105:1538–1544.
45. Bach JR, Saporito LR. Criteria for extubation and tracheostomy tube removal for patients
with ventilatory failure. A different approach to weaning. Chest 1996; 110:1566–1571.
46. Chaudri MB, Liu C, Hubbard R, et al. Relationship between supramaximal flow during
cough and mortality in motor neurone disease. Eur Respir J 2002; 19:434–438.
47. Pinto AC, Evangelista T, Carvalho M, et al. Respiratory assistance with a non-invasive
ventilator (BIPAP) in MND/ALS patients: survival rates in a controlled trial. J Neurol
Sci 1995; 129(suppl):19–26.
48. Clini E, Sturani C, Rossi A, et al. The Italian multicentre study on noninvasive ventilation
in chronic obstructive pulmonary disease patients. Eur Respir J 2002; 20:529–538.
49. Casanova C, Celli BR, Tost L, et al. Long-term controlled trial of nocturnal nasal positive
pressure ventilation in patients with severe COPD. Chest 2000; 118:1582–1590.
50. Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on sur-
vival and quality of life in patients with amyotrophic lateral sclerosis: a randomised
controlled trial. Lancet Neurol 2006; 5:140–147.
51. Jackson M, Smith I, King M, et al. Long term non-invasive domiciliary assisted ventilation
for respiratory failure following thoracoplasty. Thorax 1994; 49:915–919.
52. Simonds AK, Elliott MW. Outcome of domiciliary nasal intermittent positive pressure
ventilation in restrictive and obstructive disorders. Thorax 1995; 50:604–609.
53. Aboussouan LS, Khan SU, Meeker DP, et al. Effect of noninvasive positive-pressure ven-
tilation on survival in amyotrophic lateral sclerosis. Ann Intern Med 1997; 127:450–453.
Noninvasive Positive Ventilation 185
54. Kleopa KA, Sherman M, Neal B, et al. BIPAP improves survival and rate of pulmonary
function decline in patients with ALS. J Neurol Sci 1999; 164:82–88.
55. Nugent AM, Smith IE, Shneerson JM. Domiciliary-assisted ventilation in patients with
myotonic dystrophy. Chest 2002; 121:459–464.

56. Gonzalez C, Ferris G, Diaz J, et al. Kyphoscoliotic ventilatory insufficiency: effects of
long-term intermittent positive-pressure ventilation. Chest 2003; 124:857–862.
57. Chu CM, Yu WC, Tam CM, et al. Home mechanical ventilation in Hong Kong. Eur
Respir J 2004; 23:136–141.
58. Farrero E, Prats E, Povedano M, et al. Survival in amyotrophic lateral sclerosis with
home mechanical ventilation: the impact of systematic respiratory assessment and
bulbar involvement. Chest 2005; 127:2132–2138.
59. Shapiro SH, Ernst P, Gray-Donald K, et al. Effect of negative pressure ventilation in severe
chronic obstructive pulmonary disease [see comments]. Lancet 1992; 340:1425–1429.
60. Muir JF, Girault C, Cardinaud JP, et al. Survival and long-term follow-up of tracheos-
tomized patients with COPD treated by home mechanical ventilation. A multicenter
French study in 259 patients. French Cooperative Study Group. Chest 1994; 106:201–209.
61. Keenan SP, Sinuff T, Cook DJ, et al. Which patients with acute exacerbation of chronic
obstructive pulmonary disease benefit from noninvasive positive-pressure ventilation?
A systematic review of the literature. Ann Intern Med 2003; 138:861–870.
62. Robert D, Willig TN, Paulus J, et al. Long-term nasal ventilation in neuromuscular dis-
orders: report of a consensus conference. Eur Respir J 1993; 6:599–606.
63. Make BJ, Hill NS, Goldberg AI, et al. Mechanical ventilation beyond the intensive care
unit. Report of a consensus conference of the American College of Chest Physicians.
Chest 1998; 113:S289–S344.
64. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory
failure due to restrictive lung disease, COPD, and nocturnal hypoventilation—a con-
sensus conference report. Chest 1999; 116:521–534.
65. Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular
disorders. Eur Respir J 2002; 20:480–487.
66. Norregaard O. Noninvasive ventilation in children. Eur Respir J 2002; 20:1332–1342.
67. Simonds AK, Ward S, Heather S, et al. Outcome of paediatric domiciliary mask ventilation
in neuromuscular and skeletal disease. Eur Respir J 2000; 16:476–481.
68. Wijkstra PJ, Lacasse Y, Guyatt GH, et al. Nocturnal non-invasive positive pressure ven-
tilation for stable chronic obstructive pulmonary disease. Cochrane Database Syst Rev

2002:CD002878.
69. Wijkstra PJ, Lacasse Y, Guyatt GH, et al. A meta-analysis of nocturnal noninvasive posi-
tive pressure ventilation in patients with stable COPD. Chest 2003; 124:337–343.
70. Sivasothy P, Smith IE, Shneerson JM. Mask intermittent positive pressure ventilation in
chronic hypercapnic respiratory failure due to chronic obstructive pulmonary disease.
Eur Respir J 1998; 11:34–40.
71. Nava S, Fanfulla F, Frigerio P, et al. Physiologic evaluation of 4 weeks of nocturnal nasal
positive pressure ventilation in stable hypercapnic patients with chronic obstructive
pulmonary disease. Respiration 2001; 68:573–583.
72. Diaz O, Begin P, Andresen M, et al. Physiological and clinical effects of diurnal nonin-
vasive ventilation in hypercapnic COPD. Eur Respir J 2005; 26:1016–1023.
73. Windisch W, Kostic S, Dreher M, et al. Outcome of patients with stable COPD receiving
controlled noninvasive positive pressure ventilation aimed at a maximal reduction of
Pa(CO2). Chest 2005; 128:657–662.
74. Masa JF, Celli BR, Riesco JA, et al. Noninvasive positive pressure ventilation and not
oxygen may prevent overt ventilatory failure in patients with chest wall diseases. Chest
1997; 112:207–213.
75. Ward S, Chatwin M, Heather S, et al. Randomised controlled trial of non-invasive ven-
tilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease
patients with daytime normocapnia. Thorax 2005; 60:1019–1024.
76. Olson AL, Zwillich C. The obesity hypoventilation syndrome. Am J Med 2005; 118:
948–956.
77. Sullivan CE, Berthon-Jones M, Issa FG. Remission of severe obesity-hypoventilation
syndrome after short-term treatment during sleep with nasal continuous positive
airway pressure. Am Rev Respir Dis 1983; 128:177–181.
186 Robert and Argaud
78. Masa JF, Celli BR, Riesco JA, et al. The obesity hypoventilation syndrome can be treated
with noninvasive mechanical ventilation. Chest 2001; 119:1102–1107.
79. de Lucas-Ramos P, de Miguel-Diez J, Santacruz-Siminiani A, et al. Benefits at 1 year of
nocturnal intermittent positive pressure ventilation in patients with obesity-hypoventi-

lation syndrome. Respir Med 2004; 98:961–967.
80. Perez de Llano LA, Golpe R, Ortiz Piquer M, et al. Short-term and long-term effects
of nasal intermittent positive pressure ventilation in patients with obesity-hypoven-
tilation syndrome. Chest 2005; 128:587–594.
81. Bradley TD, Floras JS. Sleep apnea and heart failure. Part II: central sleep apnea.
Circulation 2003; 107:1822–1826.
82. Bradley TD, Floras JS. Sleep apnea and heart failure. Part I: obstructive sleep apnea.
Circulation 2003; 107:1671–1678.
83. Pepin JL, Chouri-Pontarollo N, Tamisier R, et al. Cheyne-Stokes respiration with central
sleep apnoea in chronic heart failure: proposals for a diagnostic and therapeutic strategy.
Sleep Med Rev 2006; 10:33–47.
84. Willson GN, Wilcox I, Piper AJ, et al. Noninvasive pressure preset ventilation for the
treatment of Cheyne-Stokes respiration during sleep. Eur Respir J 2001; 17:1250–1257.
85. Kohnlein T, Welte T, Tan LB, et al. Assisted ventilation for heart failure patients with
Cheyne-Stokes respiration. Eur Respir J 2002; 20:934–941.
86. Teschler H, Dohring J, Wang YM, et al. Adaptive pressure support servo-ventilation: a
novel treatment for Cheyne-Stokes respiration in heart failure. Am J Respir Crit Care
Med 2001; 164:614–619.
87. Pepperell JC, Maskell NA, Jones DR, et al. A randomized controlled trial of adaptive
ventilation for Cheyne-Stokes breathing in heart failure. Am J Respir Crit Care Med
2003; 168:1109–1114.
88. Philippe C, Stoica-Herman M, Drouot X, et al. Compliance with and effectiveness of
adaptive servo-ventilation versus continuous positive airway pressure in the treatment
of Cheyne-Stokes respiration in heart failure over a six month period. Heart 2006;
92:337–342.
89. Arzt M, Bradley TD. Treatment of sleep apnea in heart failure. Am J Respir Crit Care
Med 2006; 173:1300–1308.
90. Bradley TD, Logan AG, Kimoff RJ, et al. Continuous positive airway pressure for central
sleep apnea and heart failure. N Engl J Med 2005; 353:2025–2033.
91. Resta O, Guido P, Picca V, et al. Prescription of nCPAP and nBIPAP in obstructive sleep

apnea syndrome: Italian experience in 105 subjects. A prospective two centre study.
Respir Med 1998; 92:820–827.
92. Gay PC, Herold DL, Olson EJ. A randomized, double-blind clinical trial comparing con-
tinuous positive airway pressure with a novel bilevel pressure system for treatment of
obstructive sleep apnea syndrome. Sleep 2003; 26:864–869.
93. Han F, Chen E, Wei H, et al. Treatment effects on carbon dioxide retention in patients
with obstructive sleep apnea–hypopnea syndrome. Chest 2001; 119:1814–1819.
94. Trang H, Dehan M, Beaufils F, et al. The French Congenital Central Hypoventilation
Syndrome Registry: general data, phenotype, and genotype. Chest 2005; 127:72–79.
95. Fauroux B, Nicot F, Essouri S, et al. Setting of noninvasive pressure support in young
patients with cystic fibrosis. Eur Respir J 2004; 24:624–630.
96. Vitacca M, Nava S, Confalonieri M, et al. The appropriate setting of noninvasive pres-
sure support ventilation in stable COPD patients. Chest 2000; 118:1286–1293.
97. Fanfulla F, Delmastro M, Berardinelli A, et al. Effects of different ventilator settings on
sleep and inspiratory effort in patients with neuromuscular disease. Am J Respir Crit
Care Med 2005; 172:619–624.
98. Lofaso F, Quera-Salva MA. Polysomnography for the management of progressive neu-
romuscular disorders. Eur Respir J 2002; 19:989–990.
99. Nava S, Ambrosino N, Rubini F, et al. Effect of nasal pressure support ventilation and
external PEEP on diaphragmatic activity in patients with severe stable COPD. Chest
1993; 103:143–150.
100. Appendini L, Patessio A, Zanaboni S, et al. Physiologic effects of positive end expiratory
pressure and mask pressure support during exacerbation of chronic obstructive pulmonary
disease. Am J Respir Crit Care Med 1994; 149:1069–1076.
Noninvasive Positive Ventilation 187
101. Johnson KG, Johnson DC. Bilevel positive airway pressure worsens central apneas
during sleep. Chest 2005; 128:2141–2150.
102. Thys F, Liistro G, Dozin O, et al. Determinants of Fi, O2 with oxygen supplementation
during noninvasive two-level positive pressure ventilation. Eur Respir J 2002; 19:653–657.
103. Schwartz AR, Kacmarek RM, Hess DR. Factors affecting oxygen delivery with bi-level

positive airway pressure. Respir Care 2004; 49:270–275.
104. Bach JR. Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive
respiratory aids. Arch Phys Med Rehabil 1995; 76:828–832.
105. Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular
disease. Chest 2000; 118:1390–1396.
106. Dohna-Schwake C, Ragette R, Teschler H, et al. Predictors of severe chest infections in
pediatric neuromuscular disorders. Neuromuscul Disord 2006; 16:325–328.
107. Curran FJ, Colbert AP. Ventilator management in Duchenne muscular dystrophy and
postpoliomyelitis syndrome: twelve years’ experience. Arch Phys Med Rehabil 1989;
70:180–185.
108. Bach JR. Indications for tracheostomy and decannulation of tracheostomized ventilator
users. Monaldi Arch Chest Dis 1995; 50:223–227.
109. Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyo-
trophic lateral sclerosis. Chest 2004; 126:1502–1507.
110. Hayashi H, Oppenheimer E. ALS patients on TPPV totally locked-in state, neurologic
findings and clinical implications. Neurology 2003; 61:135–137.
111. Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral
sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation.
J Neurol Sci 1996; 139(suppl):123–128.
112. Tsara V, Serasli E, Voutsas V, et al. Burden and coping strategies in families of patients
under noninvasive home mechanical ventilation. Respiration 2006; 73:61–67.
113. Polkey MI, Lyall RA, Davidson AC, et al. Ethical and clinical issues in the use of home
non-invasive mechanical ventilation for the palliation of breathlessness in motor neurone
disease. Thorax 1999; 54:367–371.
114. Simonds AK. Ethics and decision making in end stage lung disease. Thorax 2003;
58:272–277.
115. Bach JR, Robert D, Leger P, et al. Sleep fragmentation in kyphoscoliotic individuals with
alveolar hypoventilation treated by NIPPV. Chest 1995; 107:1552–1558.
116. Meyer TJ, Pressman MR, Benditt J, et al. Air leaking through the mouth during noctur-
nal nasal ventilation: effect on sleep quality. Sleep 1997; 20:561–569.

117. Teschler H, Stampa J, Ragette R, et al. Effect of mouth leak on effectiveness of nasal
bilevel ventilatory assistance and sleep architecture. Eur Respir J 1999; 14:1251–1257.
118. Gonzalez J, Sharshar T, Hart N, et al. Air leaks during mechanical ventilation as a cause
of persistent hypercapnia in neuromuscular disorders. Intensive Care Med 2003;
29:596–602.
119. Mehta S, Hill NS. Noninvasive ventilation. Am J Respir Crit Care Med 2001; 163:540–577.
120. Richards GN, Cistulli PA, Ungar RG, et al. Mouth leak with nasal continuous positive
airway pressure increases nasal airway resistance. Am J Respir Crit Care Med 1996;
154:182–186.
121. Randerath WJ, Meier J, Genger H, et al. Efficiency of cold passover and heated humidi-
fication under continuous positive airway pressure. Eur Respir J 2002; 20:183–186.
122. Hill NS. Complications of noninvasive ventilation. Respir Care 2000; 45:480–481.
123. Strumpf DA, Millman RP, Carlisle CC, et al. Nocturnal positive-pressure ventilation via
nasal mask in patients with severe chronic obstructive pulmonary disease. Am Rev
Respir Dis 1991; 144:1234–1239.
124. Elliott MW. Noninvasive ventilation in chronic ventilatory failure due to chronic
obstructive pulmonary disease. Eur Respir J 2002; 20:511–514.
125. Meecham Jones DJ, Paul EA, Jones PW, et al. Nasal pressure support ventilation plus
oxygen compared with oxygen therapy alone in hypercapnic COPD. Am J Respir Crit
Care Med 1995; 152:538–544.
126. Barbé F, Quera-Salva MA, de Lattre J, et al. Long-term effects of nasal intermittent posi-
tive-pressure ventilation on pulmonary function and sleep architecture in patients with
neuromuscular diseases. Chest 1996; 110:1179–1183.
188 Robert and Argaud
127. Benhamou D, Muir JF, Raspaud C, et al. Long-term efficiency of home nasal mask ven-
tilation in patients with diffuse bronchiectasis and severe chronic respiratory failure.
Chest 1997; 112:1259–1266.
128. Schonhofer B, Kohler D. Effect of non-invasive mechanical ventilation on sleep and
nocturnal ventilation in patients with chronic respiratory failure. Thorax 2000;
55:308–313.

129. Brochard L. Noninvasive pressure support ventilation: physiological and clinical results
in patients with COPD and acute respiratory failure. Monaldi Arch Chest Dis 1997;
52:64–67.
130. Carrey Z, Gottfried SB, Levy RD. Ventilatory muscle support in respiratory failure with
nasal positive pressure ventilation. Chest 1990; 97:150–158.
131. L’Her E, Deye N, Lellouche F, et al. Physiologic effects of noninvasive ventilation during
acute lung injury. Am J Respir Crit Care Med 2005; 172:1112–1118.
132. Jimenez JFM, de Cos Escuin JS, Vicente CD, et al. Nasal intermittent positive pressure
ventilation. Analysis of its withdrawal. Chest 1995; 107:383–388.
133. Karakurt S, Fanfulla F, Nava S. Is it safe for patients with chronic hypercapnic respiratory
failure undergoing home noninvasive ventilation to discontinue ventilation briefly? Chest
2001; 119:1379–1386.
134. Gay PC, Hubmayr RD, Stroetz RW. Efficacy of nocturnal nasal ventilation in stable,
severe chronic obstructive pulmonary disease during a 3-month controlled trial. Mayo
Clin Proc 1996; 71:533–542.
135. Lin CC. Comparison between nocturnal nasal positive pressure ventilation combined
with oxygen therapy and oxygen monotherapy in patients with severe COPD. Am J
Respir Crit Care Med 1996; 154:353–358.
136. Windisch W, Freidel K, Matthys H, et al. Health-related quality of life (HRQL) in patients
receiving home mechanical ventilation. Pneumologie 2002; 56:610–620.
137. Markstrom A, Sundell K, Lysdahl M, et al. Quality-of-life evaluation of patients with
neuromuscular and skeletal diseases treated with noninvasive and invasive home
mechanical ventilation. Chest 2002; 122:1695–1700.
138. Domenech-Clar R, Nauffal-Manzur D, Perpina-Tordera M, et al. Home mechanical ven-
tilation for restrictive thoracic diseases: effects on patient quality-of-life and hospitaliza-
tions. Respir Med 2003; 97:1320–1327.
139. Schonhofer B, Wallstein S, Wiese C, et al. Noninvasive mechanical ventilation improves
endurance performance in patients with chronic respiratory failure due to thoracic
restriction. Chest 2001; 119:1371–1378.
140. Dellborg C, Olofson J, Hamnegard CH, et al. Ventilatory response to CO2 re-breathing

before and after nocturnal nasal intermittent positive pressure ventilation in patients
with chronic alveolar hypoventilation. Respir Med 2000; 94:1154–1160.
141. Budweiser S, Heinemann F, Fischer W, et al. Impact of ventilation parameters and duration
of ventilator use on non-invasive home ventilation in restrictive thoracic disorders.
Respiration 2006; 73:488–494.
142. Nickol AH, Hart N, Hopkinson NS, et al. Mechanisms of improvement of respiratory
failure in patients with restrictive thoracic disease treated with non-invasive ventilation.
Thorax 2005; 60:754–760.
143. Annane D, Quera-Salva MA, Lofaso F, et al. Mechanisms underlying effects of noctur-
nal ventilation on daytime blood gases in neuromuscular diseases. Eur Respir J 1999;
13:157–162.
144. Elliott MW, Mulvey DA, Moxham J, et al. Domiciliary nocturnal nasal intermittent posi-
tive pressure ventilation in COPD: mechanisms underlying changes in arterial blood
gas tensions. Eur Respir J 1991; 4:1044–1052.
145. Fernandez E, Weinert P, Meltzer E, et al. Sustained improvement in gas exchange after
negative pressure ventilation for 8 hours per day on 2 successive days in chronic airflow
limitation. Am Rev Respir Dis 1991; 144:390–394.
146. Hill NS, Eveloff SE, Carlisle C, et al. Efficacy of nocturnal nasal ventilation in patients
with restrictive thoracic disease. Am Rev Respir Dis 1992; 145:365–371.
147. Annane D, Chevrolet JC, Chevret S, et al. Nocturnal mechanical ventilation for chronic
hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane
Database Syst Rev 2000:CD001941.
Noninvasive Positive Ventilation 189
148. Schonhofer B, Geibel M, Sonneborn M, et al. Daytime mechanical ventilation in chronic
respiratory insufficiency. Eur Respir J 1997; 10:2840–2846.
149. Bergowsky EH. State of the art: respiratory failure in disorders of the thoracic cage. Am
Rev Respir J 1979; 119:643–669.
150. Simonds AK, Parker RA, Branthwaite MA. The effect of intermittent positive-pressure
hyperinflation in restrictive chest wall disease. Respiration 1989; 55:136–143.
151. Lechtzin N, Shade D, Clawson L, et al. Supramaximal inflation improves lung compliance

in subjects with amyotrophic lateral sclerosis. Chest 2006; 129:1322–1329.
152. Schonhofer B, Barchfeld T, Wenzel M, et al. Long term effects of non-invasive mechanical
ventilation on pulmonary haemodynamics in patients with chronic respiratory failure.
Thorax 2001; 56:524–528.
153. Elliott MW. Non-invasive ventilation—mechanisms of benefit. Med Klin (Munich) 1999;
94:2–6.

191
Upper Airway Surgery in the Adult
Donald M. Sesso
Department of Otolaryngology/Head and Neck Surgery, Stanford University
Medical Center, Palo Alto, California, U.S.A.
Nelson B. Powell and Robert W. Riley
Department of Otolaryngology/Head and Neck Surgery, Stanford University
Medical Center and Division of Sleep Medicine, Department of Behavioral
Sciences, Stanford School of Medicine, Palo Alto,
California, U.S.A.
Jerome E. Hester
Department of Otolaryngology/Head and Neck Surgery, Stanford University
Medical Center, Palo Alto, California, U.S.A.
INTRODUCTION
Sleep-disordered breathing (SDB) is a collective term, which encompasses snoring,
upper airway resistance syndrome (UARS), obstructive sleep apnea-hypopnea
syndrome, and obstructive sleep apnea (OSA). These terms describe a partial or
complete obstruction of the upper airway during sleep. Patency of the pharyngeal
airway is maintained by two opposing forces: negative intraluminal pressure and
the activity of the upper airway musculature. Anatomical or central neural abnor-
malities can disrupt this delicate balance with resultant compromise of the upper
airway. This reduction of airway caliber may cause sleep fragmentation and
subsequent behavioral derangements, such as excessive daytime sleepiness (EDS)

(1–3). Thus, medical and surgical therapy attempt to alleviate this obstruction and
increase airway patency.
Surgical management was the first therapeutic modality employed to treat
SDB. Kuhlo (4) described placement of a tracheotomy tube in an attempt to bypass
upper airway obstruction in Pickwickian patients. Although effective, tracheotomy
is not readily accepted by most patients and does not address the specific sites of
pharyngeal collapse. These regions include the nasal cavity/nasopharynx, oropharynx,
and hypopharynx. Often, multilevel obstruction is present. Consequently, the surgical
armamentarium has evolved to create techniques, which correct the specific anatom-
ical sites of obstruction. The objective of surgical intervention is to eliminate SDB. To
achieve this goal, it is necessary to alleviate all levels of obstruction in an organized
and safe protocol. Ultimately, it is the obligation of the surgeon to counsel the patient
regarding all surgical techniques, risks, complications, and alternative medical
therapies.
Medical management is often considered the primary treatment of SDB, how-
ever, there are exceptions. Treatment may consist of weight loss, avoidance of alcohol,
and sedating medications and manipulation of body position during sleep (5–9).
Currently, continuous positive airway pressure (CPAP) or bilevel positive airway
pressure devices are the preferred methods of treatment and the standard to which
other modalities are compared. The efficacy of CPAP has clearly been demonstrated
11
192 Sesso et al.
(10,11). Yet, a subset of patients struggle to comply with or accept CPAP therapy
(12,13). Consequently, patients who are unwilling or unable to comply with medical
treatment may be candidates for surgery.
RATIONALE FOR SURGICAL TREATMENT OF
SLEEP-DISORDERED BREATHING
The rationale for surgical treatment of the upper airway is to alleviate or minimize
the pathophysiologic and neurobehavioral derangements associated with upper
airway obstruction. The goal is to achieve outcomes that are equivalent to those of

medical management. Ideally, this would include an improved quality of life with a
reduction in cardiopulmonary and neurologic morbidity (14–17).
SURGICAL INDICATIONS
Indications for surgery are defined in Table 1. All patients require a comprehensive
evaluation to determine if they meet the criteria for surgery. Polysomnography as
well as a history and physical examination are essential to make this determination.
The subgroup of patients whose apnea-hypopnea index (AHI) is less than 20 may
still be candidates for surgery. Surgery is considered appropriate if these patients
have associated EDS, which results in altered daytime performance or comorbidi-
ties as recognized by the Center for Medicare and Medicaid Services (including
stroke and ischemic heart disease). For those patients whose EDS is not explained
by the severity of their sleep apnea or resolved with CPAP therapy, consideration
may be given to obtaining a multiple sleep latency test or the maintenance of wake-
fulness test to determine other etiologies of sleepiness (18,19). In these patients,
surgery is unlikely to be beneficial. Other factors exist, which could predict poor
surgical outcomes and consequently, render a patient to be unsuitable for surgery.
These factors are listed in Table 2.
PREOPERATIVE EVALUATION
Proper screening and selection of patients for surgery is paramount to achieve suc-
cessful outcomes and to minimize postoperative complications. The preoperative
evaluation requires a comprehensive medical history, head and neck examination,
polysomnography, fiberoptic nasopharyngolaryngoscopy, and lateral cephalomet-
ric analysis. A thorough review of this data can determine the extent of SDB severity,
TABLE 1 Surgical Indications
Apnea-hypopnea index ≥ 20
a
events per hour of sleep
Oxygen desaturation nadir < 90%
Esophageal pressure (P
es

) more negative than −10 cm H
2
O
Cardiovascular derangements (arrhythmia, hypertension)
Neurobehavioral symptoms (excessive daytime sleepiness)
Failure of medical management
Anatomical sites of obstruction (nose, palate, tongue base)
a
Surgery may be indicated with an AHI < 20 if accompanied by excessive
daytime fatigue.
Source: From Ref. 18.
Upper Airway Surgery in the Adult 193
uncover comorbidities, and assist in risk management. Furthermore, this systematic
approach will identify probable anatomic sites of obstruction. Armed with this
information, a safe, site-specific surgical protocol can be presented to the patient.
In addition, a surgeon must listen to the concerns and expectations of their
patients. Often, these may differ than those of the physician. Thus, educating a
patient about SDB and expected outcomes of surgery should be undertaken prior to
any intervention.
PHYSICAL EXAMINATION (SEE ALSO CHAPTER 1)
A complete physical exam with vital signs, weight and neck circumference should
be performed on every patient. Included in this evaluation is a detailed head and
neck examination. Specific attention is focused in the regions that have been well
described as potential sites of upper airway obstruction, such as the nose, palate,
and base of tongue (20–25). Nasal obstruction can occur as a result of alar collapse,
septal deviation, and turbinate hypertrophy or sinonasal masses. These can be iden-
tified on anterior rhinoscopy. The oral cavity should be examined for dental occlu-
sion, periodontal disease and any lesions, including torus mandibulae or torus
palatinus. Examination of the oropharyngeal and hypopharyngeal regions includes
a description of the tonsils, palate, lateral pharyngeal walls, and tongue base. A

variety of grading systems, such as Mallampati’s, have been developed to establish
a standard of describing the degree of obstruction caused by these structures (26,27).
However, it was Fujita who first proposed a classification system to define the levels
of upper airway obstruction in OSA patients (28,29). Laryngeal anatomy can be
evaluated by indirect laryngoscopy or fiberoptic exam (see Fiberoptic Nasophar-
yngolaryngoscopy section).
POLYSOMNOGRAM (SEE ALSO CHAPTER 3)
A polysomnogram (PSG) is a vital part of the preoperative evaluation. The study
must be carefully reviewed by the surgeon, with particular attention focused on the
AHI and oxygen desaturation nadir. These data will guide appropriate surgical
treatment, as well as preoperative and postoperative management. As with any
intervention, a postoperative PSG is needed to assess a patient’s response to sur-
gery. Failure to obtain this study may result in an inadequately treated patient.
FIBEROPTIC NASOPHARYNGOLARYNGOSCOPY
Obstruction of the nasal airway, pharynx, and larynx can all be visualized during
fiberoptic exam. In particular, the larynx can be more closely observed for such
TABLE 2 Poor Surgical Candidates
Severe pulmonary disease
Unstable cardiovascular disease
Morbid obesity
Alcohol or drug abuse
Psychiatric instability
Unrealistic expectations
Source: From Ref. 18.
194 Sesso et al.
abnormalities as an omega-shaped epiglottis, vocal fold paralysis or obstructing
lesions. Evaluation of the posterior airway space (PAs) is required to determine if
obstruction occurs at the palate and/or tongue base. Furthermore, the posterior
airway space is examined at rest as well as during provocative maneuvers. One
such technique, Müller’s maneuver, has been evaluated by Sher et al. (30) to identify

potential sites of obstruction and to predict surgical success. This test involves inspi-
ration against a closed oral and nasal airway, while keeping the glottis open. Photo
documentation of findings may prove useful for surgical planning and patient edu-
cation (Fig. 1).
Fiberoptic examination is not only important to evaluate the upper airway for
obstruction but aids in determining ease of intubation. These data can be invaluable
to both the surgeon and anesthesiologist, as they determine the best method to intu-
bate a patient.
CEPHALOMETRIC ANALYSIS
The lateral cephalogram is the most cost-effective radiographic study of the bony
facial skeleton and soft tissues of the upper airway. Using these landmarks, Riley
et al. (31) were first to describe anthropomorphic measurements, which can be
performed to ascertain skeletal facial abnormalities. Magnetic resonance imaging
(MRI) and computed tomography (CT) scanning have proven to be effective in
radiographic studies; however, these tools are often reserved for investigational
studies due to expense and time (32–34). Although not as detailed as CT scan or
MRI, the cephalogram allows measurement of the length of the soft palate, skeletal
proportions, posterior airway space, and hyoid position. Studies have shown the
cephalogram to be valid in assessing obstruction, and in fact, it compares favorably
to three-dimensional volumetric computed tomographic scans of the upper airway
(35). As with other tests, this modality may underestimate the degree of obstruction,
due to the fact that the study is not performed while the patient is sleeping.
SURGICAL TREATMENT SUMMARY
The aim of surgical treatment is to alleviate upper airway obstruction and its associ-
ated neurobehavioral symptoms and morbidities. No longer is a 50% reduction in the
FIGURE 1 (See color insert.) Fiberoptic laryngoscopy. Note that the entire vocal folds of the
normal larynx can be visualized (left). Müller’s maneuver results in velopharyngeal and hypopharyn-
geal collapse with airway obstruction. Vocal folds are obscured by soft tissue in this abnormal exam
(right). Abbreviation: OSA, obstructive sleep apnea.
Upper Airway Surgery in the Adult 195

AHI deemed acceptable (Table 3). Rather, the objective is to treat to cure (normalization
of respiratory events and elimination of hypoxemia). This goal can only be accom-
plished if a careful and systematic evaluation is performed on every patient. Since
obstruction may occur at multiple levels, it may be necessary to treat more than one
site. Failure to recognize or treat all anatomical levels will lead to persistent obstruc-
tion. Thus, the surgeon must be committed to treating the entire upper airway.
Once a surgical plan has been developed, this must be communicated to the
patient and our medical colleagues. Successful treatment of a SDB patient typically
requires a multidisciplinary team. This team will assist in the preoperative and post-
operative course to minimize risk and potential complications. Prior to surgical
treatment, a review of treatment options and risks must be discussed with the
patient. Only after the patient fully understands the process and has consented to
surgery can the treatment plan proceed.
The surgeon has a variety of procedures available within his or her armamen-
tarium to treat SDB. Selecting the appropriate surgery for a patient can be challeng-
ing. However, we have created a two-phase surgical protocol (Powell-Riley surgical
protocol) as a logically directed plan to treat the specific areas of upper airway
obstruction (36,37). This protocol (Table 4) as well as other contemporary surgical
techniques (Table 5) will be discussed in this chapter.
POWELL-RILEY TWO-PHASE SURGICAL PROTOCOL
This protocol consists of two distinct phases. The procedures included in each phase
are listed in Table 4. Developed to prevent unnecessary surgery, this method is a
conservative surgical approach to the SDB patient. Phase II surgery has documented
success rates exceeding 90%; however, a substantial number of patients may not
TABLE 3 Powell-Riley Definition of Surgical Responders
Apnea-hypopnea index < 20 events per hour of sleep
a
Oxygen desaturation nadir ≥ 90%
Excessive daytime sleepiness alleviated
Response equivalent to CPAP on full-night titration

a
A reduction of the apnea-hypopnea index by 50% or more is considered a cure if
the preoperative apnea-hypopnea index is less than 20.
Abbreviation: CPAP, continuous-positive airway pressure.
Source: From Ref. 18.
TABLE 4 Powell-Riley Protocol Surgical Procedures
Phase I
Nasal surgery (septoplasty, turbinate reduction, nasal valve grafting)
Tonsillectomy
Uvulopalatopharyngoplasty or uvulopalatal flap
Mandibular osteotomy with genioglossus advancement
Hyoid myotomy and suspension
Temperature-controlled radiofrequency
a
—turbinates, palate, tongue base
Phase II
Maxillomandibular advancement osteotomy
Temperature-controlled radiofrequency
a
—tongue base
a
Temperature-controlled radiofrequency is typically used as an adjunctive treatment.
Select patients may choose temperature-controlled radiofrequency as primary treatment.
196 Sesso et al.
need such extensive surgery (38,39). In fact, patients have a realistic chance to be
cured by phase I surgery alone. However, it is difficult to predict surgical outcomes
for an individual patient. Conservative surgery (phase I) is therefore recommended
initially with the plan to perform postoperative PSG to assess response to surgery.
Those patients who are incompletely treated would then be considered for phase II
surgery. As with any treatment protocol, exceptions may occur. There are select

cases in which phase II surgery may be the appropriate initial step, as in nonobese
patients with marked mandibular deficiency and normal palates (40).
Phase I surgery is directed towards the three potential sites of upper airway
obstruction (nose, palate, and tongue base). Essentially, these surgical approaches
treat the soft tissue of the upper airway. Neither dental occlusion nor the facial
skeleton is altered. Clinical response is determined by PSG. The PSG is obtained
four to six months following surgery to allow for adequate healing. Patients who
have persistent SDB are prepared for phase II surgery.
Phase II surgery refers to maxillomandibular advancement osteotomy (MMO)
or bimaxillary advancement. MMO helps clear hypopharyngeal obstruction, which
would be the only region incompletely treated by phase I. This is the only procedure
which physically creates more room for the tongue to be advanced anteriorly, thus
enlarging the posterior airway space.
SURGICAL OUTCOMES
As noted previously, simply reducing the AHI by 50% is no longer considered a cure
for SDB. Rather, surgical intervention aims to attain the results obtained by CPAP
therapy. Consequently, a more comprehensive criterion was established to determine
surgical success or cure (Table 3).
Clinical response to phase I surgery ranges from 42% to 75% (38,41–45). Our
published data have shown that approximately 60% of all patients are cured with
phase I surgery (38). Factors that portend less successful outcomes are a mean respiratory
disturbance index (RDI) greater than 60, oxygen desaturation below 70%, mandibular
deficiency (sella nasion point B < 75°) and morbid obesity [body mass index (BMI) >
33 kg/m
2
]. However, it is imprudent to forego phase I surgery in these patients, since
a reasonable percentage may not require more aggressive surgery (37).
Incomplete treatment by phase I surgery is primarily related to persistent
hypopharyngeal obstruction. Those patients who have failed treatment or who have
been incompletely treated would then be considered for phase II surgery (MMO).

MMO is a more aggressive surgery, which requires more intensive operative and
TABLE 5 Alternative Upper Airway Surgical Techniques
Pharyngeal obstruction
Temperature-controlled radiofrequency
Pillar
®
palatal implant system
Laser-assisted uvulopalatoplasty
Injection snoreplasty
Hypopharyngeal obstruction
Genial bone advancement trephine system (GBAT™)
Repose™ genioglossus advancement hyoid myotomy
Temperature-controlled radiofrequency
Midline glossectomy and lingualplasty
Airway bypass surgery
Tracheotomy
Upper Airway Surgery in the Adult 197
postoperative care. Patients must be prepared for a recovery period of four to six
weeks. Although the convalescence period may be extensive, documented success
rates exceed 90% (38,46–49).
SURGICAL PREPARATION
As with any surgery, ensuring that a patient is medically stable for the operative
procedure can reduce postoperative complications. This evaluation includes appro-
priate laboratory, cardiopulmonary, and radiographic testing. In patients with
existing comorbid medical conditions (diabetes, hypothyroidism, cardiovascular
disease, and pulmonary disease), consultation with the appropriate medical spe-
cialist should be sought.
Furthermore, for those patients who are tolerant of CPAP, they should be
encouraged to use this modality for at least two weeks prior to surgery. Preoperative
CPAP can alleviate the issues associated with sleep deprivation and may reduce the

risk of postobstructive pulmonary edema (50). In 1988, Powell et al. (51) recom-
mended the use of preoperative CPAP for all patients who have an RDI greater than
40 and an oxygen desaturation of 80% or less. According to this protocol, the surgeon
must consider insertion of a temporary tracheotomy for those patients with severe
OSA (RDI greater than 60 and/or SaO
2
less than 70%) who are intolerant of CPAP
therapy. Tracheotomy is rarely needed at our center and must be determined on a
case-by-case basis.
SURGICAL PROCEDURES—PHASE I
Nasal Reconstruction
Nasal obstruction can occur due to incompetent nasal valves, septal deviations or
chronically enlarged turbinates. A patent nasal airway is essential for normal respi-
ration and sleep. Obstruction can increase airway resistance and result in mouth
breathing. Opening of the mouth rotates the mandible posteriorly and inferiorly,
which in turn causes the tongue to prolapse into the posterior airway space. A pleth-
ora of techniques (septoplasty, alar grafting, and turbinate reduction) exist to treat
nasal obstruction. These techniques and their results have been well established in
the head and neck literature. The choice of procedure depends upon surgeon pre-
ference and experience.
Nasal reconstruction can improve quality of life and may improve OSA in
select patients (52,53). In addition, improvement of the nasal airway may improve a
patient’s tolerance of nasal CPAP (54). Rarely, however, will alleviating nasal
obstruction cure OSA.
Although most treatments of the nasal cavity are well established, treatment
of the turbinates is an evolving technique. Our preferred method is submucosal tur-
binoplasty with a radiofrequency probe. Submucosal turbinoplasty can be per-
formed with radiofrequency or a microdebrider. Radiofrequency is rarely associated
with complications such as bleeding or crusting. However, the ultimate goal of
reducing submucosal erectile tissue, while preserving the ciliated, surface mucosa is

the same (55,56).
Uvulopalatopharyngoplasty
/
Uvulopalatal Flap
Ikematsu (57) is credited with developing the uvulopalatopharyngoplasty (UPPP)
for the treatment of habitual snoring. This technique was later adapted to treat SDB
and snoring by Fujita et al. (29) in 1981. Since this time, multiple variations have
198 Sesso et al.
been developed to treat the obstructing tissues of the soft palate, lateral pharyngeal
walls, and tonsils.
UPPP is an excellent technique to alleviate isolated retropalatal (Table 6)
obstruction (Fujita Type I). Performed under general anesthesia, a portion of the
palate, uvula, lateral pharyngeal walls, and tonsils may be removed (Fig. 2). This is
conservative surgery, which an experienced surgeon can perform with ease. Results
vary depending upon the skill of the surgeon, the technique selected, and the
severity of disease. Unfortunately, there is often a stigma associated with UPPP due
to the intensity of postoperative pain and variable cure rates.
A meta-analysis of the cure rate of UPPP was performed by Sher et al. (46) in
1996. UPPP was found to have a success rate of 39% for curing OSA. Such a high
failure rate is most likely related to the fact that a large percentage of these patients
had coexisting, unrecognized hypopharyngeal obstruction. Undoubtedly, UPPP can
clear the palatal airway of excessive tissue and, if utilized appropriately, can improve
SDB. While capable of improving select patients, UPPP is seldom credited with
curing moderate or severe SDB. In fact, this procedure may be over-utilized as an
isolated surgical procedure to cure SDB by those who have failed to identify tongue
base obstruction. However, if UPPP is used appropriately or combined with
TABLE 6 Fujita Classification of Obstructive Regions
Type I: Palate (normal base of tongue)
Type II: Palate and base of tongue
Type III: Base of tongue (normal palate)

Source: From Ref. 28.
FIGURE 2 (See color insert.) Uvulopalatopharyngoplasty. (A) This patient demonstrates tonsillar
hypertrophy, an elongated uvula and redundant tissue of the lateral pharyngeal wall resulting in a
narrowed airway space. (B) Removal of the tonsils, lateral pharyngeal wall mucosa, and soft palate
mucosa has enlarged the airway. (C) Excised surgical specimen.