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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4421_Библиотеки_им_академика_М_И_Перельмана.pdf
X
- •Contents
- •Contributors
- •1.6 Mixed Disorders
- •1.7 Isolated Symptoms
- •1.7.1 Snoring
- •1.7.2 Catathrenia
- •1.8 Summary
- •References
- •1.1 Introduction
- •1.2 Obstructive Sleep Apnea
- •1.2.1 Obstructive Sleep Apnea, Adult
- •1.2.2 Obstructive Sleep Apnea, Pediatric
- •1.3 Central Sleep Apnea
- •1.3.5 Primary Central Sleep Apnea
- •1.5 Sleep-Related Hypoxemia Disorder
- •2.7 Summary
- •References
- •3: Health Consequences of Obstructive Sleep Apnea
- •3.1 Cardiovascular Consequences
- •3.1.1 Chronic Heart Failure
- •3.1.2 Systemic Hypertension
- •3.1.3 Coronary Heart Disease
- •3.1.4 Arrhythmias
- •3.1.5 Cerebrovascular Disease
- •3.2 Respiratory Consequences
- •3.2.1 Asthma
- •3.2.3 Pulmonary Embolism
- •3.2.4 Pulmonary Hypertension
- •3.3.1 Diabetes Mellitus
- •3.3.2 Metabolic Syndrome
- •3.3.3 Sexual Dysfunction
- •3.4 Gastrointestinal Consequences
- •3.4.2 Nonalcoholic Fatty Liver Disease
- •3.5 Obstetric Outcomes
- •3.5.2 Gestational Diabetes
- •3.5.4 Maternal Surgical Complications
- •3.6 Perinatal Outcomes
- •3.6.1 Impaired Fetal Growth
- •3.6.2 Preterm Birth
- •3.6.4 Stillbirth
- •3.6.5 NICU Admission
- •3.7 Perioperative Outcomes
- •3.8 Accident-Related Consequences
- •3.9 Cancer-Related Outcomes
- •3.10 Survival Outcomes
- •3.10.1 Overall Mortality
- •3.10.2 Cardiovascular Death
- •3.10.4 Perioperative Mortality
- •References
- •4.1 Patient Case
- •4.2 Introduction
- •4.3 History
- •4.4.1 Oxygen
- •4.4.2 Vascular
- •4.4.3 Endocrine
- •4.6.1 Attention & Executive Function
- •4.6.4 Visual-Spatial
- •4.7 Summary
- •References
- •5.1 Introduction
- •5.2 Obesity
- •5.3 Hypertension
- •5.4 Diabetes Mellitus
- •5.5 Fatty Liver Disease
- •5.6 Conclusions
- •References
- •6.1 Background
- •6.2 History Taking
- •6.3 Physical Examination
- •6.4 Conclusion
- •References
- •Further Reading
- •7.1 Background
- •7.2.2 Screening Tools
- •7.2.3 Diagnostic Tests
- •7.2.7 Clinical Guidelines
- •7.3 Home Sleep Apnea Test (HSAT)
- •7.3.1 Advantages
- •7.3.2 Disadvantages
- •7.3.3 Patient Selection
- •7.3.4 Data Obtained
- •7.3.8 Recommended Follow-Up
- •7.3.9 Clinical Outcomes
- •7.4 Polysomnography (PSG)
- •7.4.1 Patient Selection
- •7.4.4 Follow-Up
- •7.5 Conclusions
- •Further Reading
- •8.1 Introduction
- •8.4 CBCT and OSA
- •8.5.1 CPAP
- •8.5.2 Oral Appliances
- •8.5.3 Maxillomandibular Advancement
- •8.6 Upper Airway Stimulation
- •8.7 Summary
- •References
- •9.1.1.1 Cranial Base Lengthening
- •9.1.1.2 Cranial Base Flexion
- •9.1.5.3 Tongue Growth
- •References
- •10.2.1.1 Cranial Base
- •10.2.1.2 Facial Height
- •10.2.1.4 Pharyngeal Airway Space
- •10.2.1.6 Hyoid Bone Position
- •10.3.1 Maxillary Expansion
- •10.3.1.4 RME for OSA
- •References
- •11.2 Pathophysiology
- •11.3 Clinical Exam
- •11.5 Treatment
- •11.6 Summary
- •References
- •12.1 Introduction
- •12.5 Mask Options
- •12.6.1 Dry Mouth
- •12.6.2 Tangled Tubing
- •12.6.3 Condensation
- •12.6.4 Headgear Problems
- •12.6.6 Ramp
- •12.6.7 Cleaning Equipment
- •12.6.8 Skin Irritation
- •12.6.9 Nasal Congestion
- •12.6.10 Aerophagia
- •12.7 Cleaning Equipment
- •12.7.1 Travel Options
- •References
- •13: Oral Appliance Therapy
- •13.1 Introduction
- •13.2 Terminology
- •13.3.2 Device Designs
- •13.4 Methodology
- •13.7.2 Device Design
- •13.7.5 Non-anatomical Traits
- •13.7.6 Disease Severity
- •13.7.7 Supine Dependency
- •13.12.3 Adherence
- •13.12.4 Mean Disease Alleviation
- •13.13 Long-Term Outcomes
- •13.16 Guidelines
- •References
- •14.1 Introduction
- •14.2 Positional Therapy
- •14.2.1 Weight Loss
- •14.2.2 Nasal EPAP Therapy
- •14.2.3 Oral Pressure Therapy
- •14.2.4 Hypoglossal Nerve Stimulation
- •References
- •15.1 Introduction: Background Information
- •15.4 Preoperative Assessment
- •15.4.1 Physical Examination
- •15.4.2 Polysomnography
- •15.4.3 Clinical History
- •15.5 Preoperative Consent
- •15.6 Preoperative Assessment
- •15.6.1 Surgical Setting
- •15.8 Instrumentation
- •15.8.1 Tonsillectomy
- •15.8.2 Adenoidectomy
- •15.9 Postoperative Management
- •15.9.1 Pain
- •15.9.2 Diet
- •15.9.3 Follow-Up
- •15.10 Expected Outcomes by Population
- •15.10.1 General Population
- •15.10.2 Complex Children
- •15.10.2.1 Obese Children
- •15.10.2.2 Down Syndrome
- •15.10.2.3 Craniofacial Syndromes
- •15.10.2.4 Synchronous Airway Lesion
- •15.11.3 Cardiovascular Parameters
- •15.13 Conclusion
- •References
- •Further Reading
- •16.1 Introduction
- •16.3.1 Anatomic Factors
- •16.8 Summary
- •References
- •17: Palatal Surgery for OSA Patients
- •17.1 Introduction
- •17.2.2 Nasopharyngeal Endoscopy
- •17.2.3 Cephalometrics
- •17.3.1.1 Success Rate of UPPP
- •17.3.1.2 Limitations of UPPP
- •17.3.1.3 Impact of UPPP
- •17.3.1.4 Complications of UPPP
- •17.3.2.2 Z-Palatopharyngoplasty
- •17.3.2.3 Expansion Sphincter Pharyngoplasty
- •References
- •18: Hypopharyngeal Surgery for OSA Patients
- •18.1 Introduction
- •18.2 Historical Perspective
- •18.3 Patient Selection
- •18.4 Physical Exam
- •18.5 Imaging I
- •18.5.1 Imaging
- •18.6 Drug-Induced Sedated Endoscopy
- •18.7 Treatment Algorithm
- •18.8 Procedures
- •18.8.1 Transoral Robotic Surgery
- •18.8.2 Radiofrequency Ablation (RFA)
- •18.8.3 Genioglossus Advancement
- •18.8.4 Tongue Base Suspension
- •18.8.5 Hyoid Suspension
- •18.8.7 Hypoglossal Nerve Stimulators
- •18.9 Future Directions
- •References
- •Suggested Reading
- •19.1.1 Imaging
- •19.2.1.1 Pierre Robin Sequence
- •19.2.1.2 Craniofacial Microsomia
- •19.2.2.1 Crouzon’s Syndrome
- •19.2.2.2 Apert Syndrome
- •19.2.3.1 Treacher Collins Syndrome
- •19.2.3.2 Goldenhar Syndrome
- •19.3 Surgical Correction
- •Bibliography
- •20.1 Introduction
- •20.4.2 Surgical Technique (DOME)
- •20.4.4 Consolidation Phase
- •20.6 Discussion
- •References
- •21.3.3 Maxillomandibular Setback
- •References
- •22.1 Introduction
- •22.3 Results
- •22.3.1 Success Rate
- •22.4 Cases
- •22.5 Discussion
- •22.6 Conclusion
- •References
- •23.1 Patient Evaluation
- •23.1.1 Patient Concerns
- •23.1.4 Facial Evaluation
- •23.1.5 Lateral View
- •23.1.6 Oral Examination
- •23.1.7 Periodontal Evaluation
- •23.1.8 Tongue Assessment
- •23.1.9 Temporomandibular Joint
- •23.1.10 The Nose
- •23.1.11 Oropharyngeal Airway Assessment
- •23.2 Radiographic Evaluation
- •23.2.2 Lateral Cephalometric Radiograph
- •23.2.5 Cephalometric Analysis
- •23.3 Dental Model Analysis
- •23.3.1 Arch Length Measurements
- •23.3.2 Tooth Size Analysis
- •23.3.3 Tooth Position
- •23.3.4 Arch Width Analysis
- •23.3.6 Cuspid-Molar Position
- •23.3.7 Tooth Arch Symmetry
- •23.3.10 Ankylosed Teeth
- •23.4 Summary
- •References
- •24.1 TMJ Articular Disc Displacement
- •24.3 Reactive Arthritis (ReA)
- •24.5 Trauma
- •24.6 TMJ Ankylosis
- •24.7 Other End-Stage TMJ Conditions
- •24.8 Summary
- •References
- •25.1 Background
- •25.2 Treatment Planning Maxillary Surgery
- •25.2.1 Bone Anatomy
- •25.2.2 Vascular Anatomy
- •25.5 Adjunct Procedures
- •25.6 Complications
- •References
- •26: Mandibular Surgical Procedures
- •26.1 Genioplasty Procedures
- •26.2 Osseous Genioplasty
- •26.2.1 Anteroposterior Augmentation
- •26.2.2 Surgical Procedure
- •26.2.3 Anteroposterior Reduction
- •26.2.4 Vertical Augmentation (Downgraft)
- •26.2.5 Vertical Reduction
- •26.3 Alloplastic Augmentations
- •26.3.1 Surgical Procedure
- •26.4 Genioplasty Complications
- •26.5 Mandibular Subapical Procedures
- •26.5.3 Possible Complications
- •26.6 Mandibular Body Surgery
- •26.7.1 Nonunion or Malunion
- •26.7.3 Infections
- •26.7.4 Periodontal Defects
- •26.7.5 Nerve Damage
- •26.8 Mandibular Ramus Surgery
- •26.9 Vertical Ramus Osteotomy
- •26.11.1 Early Relapse
- •26.11.2 Condylar Sag
- •26.11.4 Unfavorable Splits or Fractures
- •26.11.6 Periodontal Defects
- •26.11.8 Nerve Injury
- •26.11.9 Infections
- •26.11.10 Nonunion
- •26.11.11 Bleeding Problems
- •References
- •27.1 Occlusal Plane Alteration
- •27.1.1 History
- •27.2 Corrected Frankfort Horizontal Plane
- •27.3 High Occlusal Plane (HOP) Facial Type
- •27.3.6 MRI Evaluation
- •27.3.7 TMJ Disc Displacement
- •27.3.9 Reactive Arthritis
- •27.3.11 Other End-Stage TMJ Pathologies
- •27.6 Summary
- •References
- •28: Maxillomandibular Advancement
- •28.1.1 Symptoms
- •28.1.3.1 Noninvasive Treatments
- •28.1.3.2 Surgical Interventions
- •28.4.1 Preoperative Medical Assessment
- •28.5 Procedure
- •28.5.1.2 Plates Vs. Screws
- •28.7 Post-MMA Follow-Up Care
- •28.8 Conclusion
- •References
- •29.2.1 CASS Adoption Widespread
- •29.2.2 Overall CASS Accuracy
- •29.2.2.1 Soft-Tissue Prediction Simulators
- •29.2.3 Cost
- •29.4.1 Overall CASS Process
- •29.4.1.1 Step 1: Patient Referral
- •29.4.1.7 Step 7: Procedure
- •29.4.4 Case 3
- •29.5 Conclusion
- •References
- •30.1 Introduction
- •30.2 Preoperative Considerations
- •30.2.1 Surgical Facility
- •30.2.2 Medical Clearance
- •30.2.3 Anesthesia Considerations
- •30.3 Inpatient Postoperative Management
- •30.3.1 Immediate Postoperative Course
- •30.3.2 Acute Pain Management
- •30.3.5 DVT Prophylaxis
- •30.3.6 Nutrition
- •30.3.7 Antibiotics
- •30.4.1 Follow-Up Regimen
- •30.4.2 Postoperative Occlusal Guidance
- •30.5 Conclusion
- •References
- •31.1 Paradigm
- •31.2 Preoperative
- •31.3 Acute Post-surgical
- •31.4 Long-Term Post-surgical
- •References

278
P. Yalamanchi and P. T. Hoff
. Fig. 18.10 Tongue base suspension procedure
Tongue base suspension
Hyoid suspension
18
Thyrohyoidpexy
. Fig. 18.11 Hyoid suspension procedures
advancement and/or with palatal repositioning procedures for obstructive sleep apnea, . Fig.18.11. The thyrohyoidpexy procedure advances the hyoid bone over
the thyroid lamina which is then secured with permanent sutures placed through the superior portion of the
thyroid cartilage. Anterior repositioning of the hyoid
bone by attaching it to the thyroid cartilage expands the
airway. While there is variable success with hyoid
advancement, the procedure is generally well tolerated
with surgical risks limited to infection, seroma formation, and dysphagia.
The hyoid can also be suspended to the posterior surface of the mandible, which pulls the tongue base in an
anterior-superior direction. The hyoid bone is xed to
the mandible with anchor sutures placed through a sub-
Hyoidmandibulopexy
mental incision. This is a minimally invasive technique
and works well for patients with a large muscular tongue
base without lymphoid hypertrophy.
Suspension procedures have been shown to be modestly effective when performed in isolation; the best
results are obtained when performed in conjunction
with palatal surgery.
18.8.6 Maxillomandibular Advancement
(MMA)
Maxillomandibular advancement increases the retropalatal and retrolingual airway by advancing the maxilla and
mandible through Le Fort I maxillary and sagittal- split

Hypophar yngeal Surgery forOSA Patients
279
a
18
b
. Fig. 18.12 Inspire hypoglossal nerve stimulator: a Inspire pulse generator, sensing lead, and stimulating lead, b Inspire layout, c intra-
operative image of hypoglossal nerve dissection with red vessel loop around inclusion branches of the nerve
mandibular osteotomies. Typically, this procedure is per-
18.9 Future Directions
formed when other surgical interventions have been
unsuccessful. Potential complications include malocclusion, nonunion, or malunion temporomandibular joint
problems and nerve paresthesia. Despite the risks and
relative morbidity of the procedure, the success rate of this
procedure has been reported to be between 80% and 90%.
Increasingly, neurostimulation treatment strategies are
viewed as a preferred alternative to bony or soft tissue
surgical interventions for treatment of OSA in CPAPintolerant patients. These procedures have a lower risk
prole and are associated with signicantly reduced
postoperative pain and recovery time. While strict inclusion criteria and cost are currently the primary barriers
18.8.7 Hypoglossal Nerve Stimulators
for increasing adoption of hypoglossal nerve stimulation,
novel stimulation strategies are being designed and
HNS therapy has been shown to signicantly reduce AHI
in moderate-to-severe OSA patients with strict inclusion
criteria including BMI <32, an AHI between 15 and 65,
and favorable pattern of upper airway obstruction during
DISE. The hypoglossal nerve stimulator system involves
three main components: the implantable pulse generator
(IPG), the sensing lead, and the stimulator lead,
.
Fig. 18.12. The IPG is surgically implanted into an
infraclavicular subcutaneous pocket supercial to the pectoralis major and produces electrical impulses. The sensing
and stimulation leads are tunneled subcutaneously from
the IPG, respectively, to the lower ribs and hypoglossal
nerve. Closed-loop stimulating systems produce impulses
with inhalation through the IPG to the hypoglossal nerve
via a tripolar electrode that wraps around the nerve resulting in opening of the retroglossal airway as well as the retropalatal airway due to mechanical coupling of the
palatoglossal and genioglossus musculature, . Fig.18.13.
invigorating the eld of sleep medicine. Additional clinical trials are underway assessing the efcacy of external
HNS stimulation devices.
Patient selection is an area of active research. It is
now widely recognized that surgeons must address both
the anatomic and nonanatomical parameters such as
loop gain (a measure of ventilatory stability) and arousal
threshold using a physiology-based model to better predict outcomes after upper airway surgery for obstructive
sleep apnea.
Additionally, recent research has indicated that efferent motor pathways may be stimulated through recruitment of reex afferent input to respiratory and upper
airway motor control centers. Compared with direct
unilateral hypoglossal nerve stimulation, recent studies
in animal models has suggested esophageal distention,
electrical auricular stimulation, sciatic nerve stimulation, and pulsed nasal insufation of heated, humidied
c

280
P. Yalamanchi and P. T. Hoff
18
. Fig. 18.13 Effects of
hypoglossal nerve stimulation.
(Courtesy of Inspire Medical
Systems)
Inspire hypoglossal nerve stimulation eects
No stimulation Mild stimulation
Base of tontgue Base of tontguePalate Palate
air may activate respiratory brainstem motor nuclei to
initiate a more coordinated brainstem response involving several cranial nerves and upper airway muscles to
improve airway patency during sleep. The eld of neurostimulation for treatment of OSA is exciting and nascent
as promising new approaches for activating efferent and
afferent motor pathways are currently in early stage
development.
References
1. Stuck BA, Leitzbach S, Maurer JT. Effects of continuous
positive airway pressure on apnea-hypopnea index in obstructive sleep apnea based on long-term compliance. Sleep Breath.
2012;16(2):467–71.
2. Fujita S. Obstructive sleep apnea syndrome: pathophysiology, upper airway evaluation and surgical treatment. Ear Nose
Throat J. 1993;72(1):67–72. 5–6
3. Riley RW, Powell NB, Guilleminault C.Obstructive sleep apnea
syndrome: a review of 306 consecutively treated surgical patients.
Otolaryngol Head Neck Surg. 1993;108(2):117–25.
4. Weaver TE, Laizner AM, Evans LK, Maislin G, Chugh DK, Lyon
K, Smith PL, Schwartz AR, Redline S, Pack AI, Dinges DF.An
instrument to measure functional status outcomes for disorders of
excessive sleepiness. Sleep. 1997;20(10):835–43.
5. Thaler ER, Rassekh CH, Lee JM, Weinstein GS, O’Malley BW
Jr. Outcomes for multilevel surgery for sleep apnea: obstructive
sleep apnea, transoral robotic surgery, and uvulopalatopharyngoplasty. Laryngoscope. 2015;126:266.
6. Vicini C, Dallan I, Canzi P, Frassineti S, Nacci A, Seccia V, etal.
Transoral robotic surgery of the tongue base in obstructive sleep
Apnea-Hypopnea syndrome: anatomic considerations and clinical experience. Head Neck. 2012;34(1):15–22.
7. Kezirian EJ.Nonresponders to pharyngeal surgery for obstructive sleep apnea: insights from drug-induced sleep endoscopy.
Laryngoscope. 2011;121(6):1320–6.
8. Vicini C, Dallan I, Canzi P, Frassineti S, La Pietra MG, Montevecchi F.Transoral robotic tongue base resection in obstructive
sleep apnoea-hypopnoea syndrome: a preliminary report. ORL
J Otorhinolaryngol Relat Spec. 2010;72(1):22–7.
9. Blumen MB, Coquille F, Rocchicioli C, Mellot F, Chabolle
F. Radiofrequency tongue reduction through a cervical
approach: a pilot study. Laryngoscope. 2006;116:1887–93.
10. Steward DL, Weaver EM, Woodson BT.Multilevel temperaturecontrolled radiofrequency for obstructive sleep apnea: extended
follow-up. Otolaryngol Head Neck Surg. 2005;132:630–5.
11. Woodson BT, Steward DL, Weaver EM, Javaheri S.A randomized trial of temperature controlled radiofrequency, continuous
positive airway pressure, and placebo for obstructive sleep apnea
syndrome. Otolaryngol Head Neck Surg. 2003;128:848–61.
12. Lin HS, Rowley JA, Badr MS, Folbe AJ, Yoo GH, Victor L,
et al. Transoral robotic surgery for treatment of obstructive
sleep apnea-hypopnea syndrome. Laryngoscope. 2013;123(7):
1811–6.
13. Vicini C, Montevecchi F, Campanini A, Dallan I, Hoff PT,
Spector ME, et al. Clinical outcomes and complications
associated with TORS for OSAHS: a benchmark for evaluating an emerging surgical technology in a targeted application for benign disease. ORL J Otorhinolaryngol Relat Spec.
2014;76(2):63–9.
Suggested Reading
Friedman M, Hamilton C, Samuelson CG, Kelley K, Taylor D,
Pearson-Chauhan K, et al. Transoral robotic glossectomy for
the treatment of obstructive sleep apnea-hypopnea syndrome.
Otolaryngol Head Neck Surg. 2012;146(5):854–62.

Management ofObstructive
Sleep Apnea (OSA)
inCraniofacial Patients
MikhailDaya andJasonE.Portnof
Contents
19.1 Diagnosis andManagement of Childhood Obstructive
Sleep Apnea Syndrome – 282
19.1.1 Imaging – 282
19.2 Role ofSleep Upper Airway Endoscopy intheDiagnosis
ofOSA – 282
19.2.1 Mandibular Deciency – 282
19.2.2 Mid-Face Deciency – 283
19.2.3 Both Mid-Face andMandibular Deciency – 284
281
19
19.3 Surgical Correction – 284
Bibliography – 290
© Springer Nature Switzerland AG 2021
K. B. Kim et al. (eds.), Management of Obstructive Sleep Apnea, https://doi.org/10.1007/978-3-030-54146-0_19

282
M. Daya and J. E. Portnof
19
19.1 Diagnosis andManagement of
Childhood Obstructive Sleep Apnea
Syndrome
Many screening tools and questionnaires have been
described for the evaluation of pediatric patients with
suspicion of obstructive sleep apnea (OSA). These
screening tools could not be relied upon in patients with
craniofacial syndromes since they commonly present
with other manifestations such as cognitive and hearing
decits and ocular decits, among others.
Advanced imaging has shown to be powerful in the
assessment of structural abnormalities and in helping to
locate the anatomical location of the obstruction along
the upper airway. Many software tools incorporate
measuring tools to assess the airway, providing threedimensional measurements.
Polysomnography has proven to be the gold standard
for diagnosis of OSA in pediatric population. However,
other alternatives have been proposed in the past for
screening and diagnosis of this condition.
19.1.1 Imaging
Plain lm radiographs including lateral cephalometric
radiographs, AP/PA cephalometric radiographs, and
panorex radiographs have been used in the past as an
efcient and inexpensive diagnostic tool in the assessment of dentofacial deformities since they are readily
available at orthodontists and oral surgeons’ ofces.
These X-rays are used to diagnose bony anatomy, position of the maxilla and mandible, length of the soft
palate, and position of the hyoid bone. The major disadvantage of pain lms is the inability to study soft tissues.
Computer tomography (CT) and cone-beam CT
(CBCT) signicantly improve the soft tissue contrast
and detail. In addition, 3D studies aid in examining the
airway in all pains as compared to plain lms allowing for precise measurement of minimal cross sectional
area and volumetric assessment of the airway. CT scans
also provide with detailed anatomical information of
the obstruction site, this is relevant in planning upper
airway surgery when necessary. However, controversy
still exists in the usefulness of 3D imaging in awake nonsupine patients, since the true anatomy of asleep patient
cannot be assessed accurately.
Comparison of CBCT before and after surgery
determines the difference in airway volume. However,
no guidelines have been established regarding minimal
cross sectional area and airway volume to diagnose OSA
based on these ndings.
Magnetic resonance imaging with compared to
CBCT and CT scans offers various advantages including lack of ionized radiation and better analysis of the
soft tissue structures. The lack of ionized radiation
makes MRI the imaging technique in children with OSA
syndrome.
The application of these imagine technique in craniofacial patients can be challenging to the lack of cooperation by the patient. Sedation may be required for the
patient to tolerate these studies without moving while
acquiring the imaging. On the other hand, obtaining the
images with the patient sedated in a supine position may
replicate the airway size and position during sleep, but
this is still controversial and no advantages have been
proved.
19.2 Role ofSleep Upper Airway Endoscopy
intheDiagnosis ofOSA
Sleep endoscopy has been used since 1991 to examine
upper airway during pharmacologically induced sleep.
Even though the goal standard up to date remains polysomnographic studies, other studies such as sleep endoscopy aid in identifying the level and degree of upper
airway collapse. This diagnostic tool can aid in the development of a treatment plan guided toward obstruction.
Multiple grading systems have been developed to
grade the severity of OSA. Berchard etal. described a
system that focuses on ve anatomical sites including
nose and nasopharynx (N), palatine plane, uvula or tonsils (P), tongue (T), larynx (L), and hypopharynx (H).
Furthermore, the obstruction was categorized as partial
(1) or complete (2) for each one of the above. So, if the
patient has partial obstruction at the nose and tonsils
and complete obstruction at the tongue, this would be
labeled as N1P1T2 and would be assigned a value of 4
(1+ 1 +2). A score range of 0–2 was associated with
mild OSA, 3–4 with moderate, and more than 4 with
severe.
19.2.1 Mandibular Deciency
19.2.1.1 Pierre Robin Sequence
Pierre Robin Sequence (PRS) is a congenital malformation occurring in 1in 30,000 live births. This condition is characterized by the triad of severe mandibular
hypoplasia, glossoptosis, and cleft palate. Many of these
patients present with airway obstruction.
Sher et al. described four types of airway obstruction in patients with PRS based on exible beroptic
nasopharyngoscopy ndings. The most common form,
TypeI, is dened by obstruction due to posterior movement of the tongue against the posterior pharyngeal
wall. Type II obstruction is due to posterior and superior
displacement of the tongue, causing obstruction from
the tongue, the velum, and the pharyngeal wall in the

Management ofObstructive Sleep Apnea (OSA) inCraniofacial Patients
283
19
superior oropharynx. Type III obstruction is caused by
prolapse of the medial pharyngeal wall. Type IV obstruction is due to constriction of the pharynx circumferentially by lateral pharyngeal walls and the tongue.
Syndromes presenting along with this condition
include Stickler syndrome, Nager syndrome, Treacher
Collins, and velocardiofacial syndrome. Al-Samakri
etal. showed up to 60% of Pierre Robin sequence cases
present in combination with other syndromes; 80 to 90%
of these patients also present with cleft lip and palate.
The prevalence of OSA in this population ranges
from 46% to 100% across the literature. This is due to
upper airway obstruction related to micrognathia, posterior position of the tongue, and cleft palate.
Management of OSA in patients with Pierre Robin
sequence involves treating the upper airway by treating
cleft palate, stabilizing the pharyngeal wall, and widening the hypopharynx. Emergent tracheostomy is necessary in cases where airway is severely affected. Other
necessary treatments include mandibular distraction
and application of oral devices to widen the palate and
the upper airway.
19.2.1.2 Craniofacial Microsomia
Craniofacial microsomia is condition characterized by
structures derived from the rst and second branchial
arches, including maxillomandibular complex, facial
nerves, ears, and soft tissue. The incidence of craniofacial microsomia ranges from 1in 3500 to 1in 20,000 live
births in the literature and it is the second most common congenital facial defect, after cleft lip and palate.
Craniofacial microsomia can be unilateral, known as
hemifacial microsomia, or bilateral.
The mandible is commonly affected in craniofacial
microsomia. Mandibular and maxillary hypoplasia,
along with adenotonsillar hypertrophy and glossoptosis
are important contributing factors for obstructive sleep
disorders in this patient population.
The orbits, mandible, ears, nerve, soft tissue
(O.M.E.N.S) classication has been widely used to
describe the different anatomical variations and severity
of this condition. Later, the word Plus was added to the
classication to describe any alterations outside of the
craniomaxillofacial complex. The OMENS-Plus score is
calculated grading each anatomical abnormality from 0
to 3. Where 0 is normal, 1 is abnormal size, 2 is abnormal
position, and 3 for the combination of both. Pruzansky
graded the mandible, and this classication was later
modied by Kaban. Mandibular grading assesses the
mandibular ramus and condyle [0=normal; 1=small
mandible, short ramus; 2 = abnormally shaped ramus
and condyle] with (a) glenoid fossa anatomically acceptable compared to the contralateral side, (b) temporomandibular joint (TMJ) displaced anteriorly, medially,
or inferiorly with hypoplastic condyle; and 3=complete
absence of ramus and fossa.
The prevalence of OSA in patient with craniofacial
microsomia varies signicantly in the literature from 7%
to 67% taking into consideration the severity of the anatomical defects as well as the denition of OSA in the
different studies.
19.2.2 Mid-Face Deciency
19.2.2.1 Crouzon’s Syndrome
Crouzon’s syndrome is an autosomal dominant condition. It is considered a syndromic craniosynostosis
with birth prevalence of 1 in 60,000. This condition
can present as an isolated entity or in combination with
other malformations. Important clinical characteristics
of this condition include bilateral coronal craniosynostosis, exorbitism with hypertelorism, and maxillary
hypoplasia, among other variations. The combination
of skull and maxillary growth disturbance often results
in increased intracranial pressure and obstructive sleep
apnea; 40 to 85% of these patients are diagnosed with
obstructive sleep apnea at some point and the severity
is usually determined by the anatomy of the airway and
the degree of mid-face deciency.
Treatment of obstructive sleep apnea for these
patients is different from treatment of adults with just
OSA.Children with Crouzon’s syndrome often present
severe mid-face deciency, increased scleral show, and
other craniofacial deformities. These deformities and
skeletal deciencies causing obstructive sleep apnea
must be address simultaneously. Many surgical options
have been proposed for treatment of OSA in children
with Crouzon’s syndrome depending on the severity of
the case. Severe emergent cases may require tracheostomy for immediate airway protection. Other treatment
modalities include LeFort III osteotomies for severe midface deciency, LeFort I osteotomies, distraction osteogenesis (DO), and orthodontic/orthognathic surgery.
Conservative methods such as CPAP application have
been recommended for less severe cases.
19.2.2.2 Apert Syndrome
Apert syndrome is a rare condition that affects 1in every
65,000 births. It is an autosomal dominant transmitted
disorder that results in the abnormal development of the
skull and face due to the premature closure of sutures,
primarily the coronal sutures resulting in brachycephaly
and turricephaly (Omar Breik).
This syndrome is characterized by craniosynostosis,
mid-facial hypoplasia, syndactyly of the hands and feet,
and other malformations of the limbs (Omar Breik). In
this syndrome, the maxilla is also involved in the synostosis. Therefore, up to 75% of these patients present with
cleft palate or bid uvula.
The causes of OSA in patients with Apert syndrome
are similar to those mentioned in Crouzon syndrome. OSA

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M. Daya and J. E. Portnof
19
is often multi-level problem in these children. However,
since mid-face deciency is the primary cause for airway
constriction, monoblock or LeFort III advancement with
distraction is usually the treatment of choice (Doerga).
19.2.3 Both Mid-Face andMandibular
Deciency
19.2.3.1 Treacher Collins Syndrome
Treacher Collins syndrome is an autosomal dominant
condition that affects 1in 25,000 to 1in 50,000 births.
This craniofacial syndrome is characterized by craniomaxillofacial soft tissue and skeletal hypoplasia of the
rst and second branchial arches. The major features
include mandibular micrognathia, conductive hearing
loss, malar deciency, and down-slanting eyes and in
33% of the cases with cleft palate.
The treatment of this patient population is carried
through the different growth phases. First, to support
the airway when the child is born, then during early
childhood to support feeding and speech, and denitive treatment is performed when growth has been completed to address the facial defects.
19.2.3.2 Goldenhar Syndrome
Goldenhar syndrome is a congenital disorder that
affects the rst and second branchial arches and it
affects 1 of 5600 live births. Unlike microal microsomia, Goldenhar syndrome also affects ears, eyes, and
vertebrae. Cardiac and neurologic conditions are associated with this disorder; however, they are not necessary
for the nal diagnosis.
Obstructive sleep apnea affects 11.6% of patients
with Goldenhar syndrome. The etiology of the obstructions is multifactorial, including anatomical and neurological alterations. Unlike patients with hemifacial
microsomia, patients with this syndrome show increase
in CO2. This additional nding may correlate with dysfunction of the regulation of respiration during sleep
caused by neurological impairment.
Treatment of OSA in patients with Goldenhar syndrome like in other syndromes varies depending on the
severity of OSA as well as the anatomical area in the
obstruction. Severe cases require more aggressive interventions such as tracheostomy to protect the airway and
improve oxygenation levels while denitive treatment is
performed.
19.3 Surgical Correction
In the newborn emergency setting, tongue–lip adhesion
and/or tracheostomy may be required to secure and maintain the airway in craniofacial syndromes with profound
airway obstruction and respiratory distress. Tracheostomy
is a bypass procedure that can be used as a temporary
measure to maintain the airway while other procedures to
improve the airway are planned and performed.
These immediate procedures, performed shortly
after birth to address the airway, are then revised. Once
facial growth is adequate and life-threatening obstruction is treated, release of the tongue–lip adhesion and a
reversal of tracheostomy are performed.
Tonsillectomy/adenoidectomy and palatal procedures
such as uvulopalatopharyngoplasty (UPPP) address
obstructions of the oropharynx. Adenotonsillectomy
is the procedure of choice for surgical management of
OSA in children.
Procedures such as hyoid suspension, partial glossectomy, and radiofrequency ablation of the tongue base
are soft tissue procedures that will address obstruction
of the hypopharynx.
Timing of surgery can be during active phases of
growth or after growth cessation.
It is possible that surgery performed during active
phases of growth is susceptible to relapse and there is need
for additional surgical procedures including repeat of the
surgical procedure to obtain the desired functional result.
Ilizarov described his concept of distraction osteogenesis (DO) for limb reconstruction in 1952 and McCarthy
described the application of DO to the human craniomaxillofacial skeleton in 1989. As described, the principle
includes a surgical osteotomy after subperiosteal dissection, a latency period of 4–5 days, a distraction period
of a rate of sometimes greater than 1.0mm per day, and
then a consolidation period. Commonly used distraction
protocols are based on a clinical goal of 20% overcorrection. The overall treatment time for distraction of the craniofacial skeleton can be less than 3months. Successful
maxillary, mid-face, zygomatic, orbital, mandibular, and
cranial bone distraction have all been described.
Distraction osteogenesis is a viable treatment option
for obstructive sleep apnea in craniofacial syndromic
patients. Depending on the anatomic location of the
obstruction, the patient may be a candidate for mandibular or mid-face distraction (see .
Fig. 19.1). Virtual
surgical planning based on medical grade computed
tomography scans or cone-beam CT scans (CBCT) can
be helpful to predict the surgical design.
Mid-face distraction osteogenesis can be performed
intraorally or extraorally with a rigid external distraction (RED) device. With proper patient selection, RED
can be utilized from age 5 through adulthood (see
Fig. 19.2). Similarly, mandibular distraction can be
.
performed with an internal or external device.
Unilateral or bilateral costochondral graft (CCG)
reconstruction of the mandible can be an option for
craniofacial patients with severe mandibular deciency
involving the ramus and condyle unit (see . Fig.19.3).
It is recommended that patients who receive mandibular reconstruction with either distraction osteogenesis

Management ofObstructive Sleep Apnea (OSA) inCraniofacial Patients
. Fig. 19.1 Graphic description of extra-oral mandibular distractor (left) and rigid external distractor (RED) device (right)
285
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. Fig. 19.2 Mid-face deciency in teenager patient with history of cleft lip and palate treated with distraction osteogenesis with rigid exter-
nal distractor (RED) device (left). Postoperative lateral cephalogram with RED device (right)

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M. Daya and J. E. Portnof
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. Fig. 19.3 Patient with Pierre Robin Sequence and severe mandibular hypoplasia treated with bilateral condylectomies and costochondral
graft. Top picture shows virtual surgical planning and bottom pictures postoperative panoramic X-ray

Management ofObstructive Sleep Apnea (OSA) inCraniofacial Patients
287
19
or costochondral graft follow a similar protocol as has
been described by Kaban etal. for the management of
TMJ ankylosis in children, which includes the following:
1. Lining of joint with temporalis fascia
2. Rigid xation
3. Early mobilization of jaw
(a) if DO is used to reconstruct, mobilize day of
surgery
(b) if CCG is used, early mobilization with minimal
maxillomandibular xation (not to exceed
10days)
4. Aggressive physiotherapy
Among the many therapies offered for OSA, maxillomandibular advancement (MMA) is recognized as a
powerful technique for relieving upper airway obstruction. This orthognathic surgical procedure generally
involves LeFort I maxillary and sagittal split mandibular osteotomies (see .
Fig.19.4). It is possible that in
the craniofacial patient, a sagittal split osteotomy would
be insufcient to allow for the large mandibular discrepancy that can oftentimes be greater than 1cm. In these
patients, extra-oral inverted L osteotomies with bone
graft augmentation may be necessary (see . Fig.19.5).
MMA surgery will simultaneously enlarge the pha-
ryngeal airway dimension at the nasopharynx, oropharynx, and hypopharynx. The facial skeletal framework is
expanded, with benecial airway effects based on improved
positioning of the pharyngeal soft tissues and the tongue.
In addition to maxillomandibular advancement, an
advancement genioplasty can be performed with a simple anterior mandibular horizontal osteotomy (AMHO)
and this technique will allow to advance the genioglossus
attachment further opening the airway (see .
Fig.19.6).
However, genioglossus advancement may compromise facial aesthetics by over-projecting the chin prominence. A modication of the AMHO genioplasty was
described by Heggie etal. with a design involving a rotational repositioning that allows for advancement of the
genioglossus attachments while avoiding excessive projection of pogonion.
In the genial tubercle advancement, a rectangular
bone window of the anterior mandible that incorporates the genial tubercle is osteotomized. The bone
fragment is advanced, rotated 90 degrees, and stabilized. The bony advancement stretches the genioglossus muscle and addresses hypopharyngeal soft tissue
obstruction. During this operation, the chin point is
not changed, and it can be used in patients with an
orthognathic prole without a negative impact on
facial aesthetics.
Surgical treatment planning for correction of OSA
in patients with craniofacial syndromes will often be a
component of a multimodality approach.
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