Orthognathic Surgery - CAM 041

NOTE: This policy is used to determine coverage of orthognathic surgery for members whose plan documents indclude this benefit. Please review the individual plan document to determine if coverage is available.

Description:
Orthognathic surgery is a class of surgical procedures designed to realign the maxillofacial skeletal structures with each other and with the other craniofacial structures. This surgery usually involves the maxilla and/or mandible, but other bony components may be involved, as well. Clinical rationale for orthognathic surgery includes:

  1. Repair of congenital anomalies (cleft lip/palate and other similar anomalies)
  2. Repair of abnormalities resulting from trauma, tumors or infections
  3. Treatment of malocclusion that contributes significantly to temporomandibular joint syndrome symptoms
  4. Treatment of other medical problems (difficulty swallowing, speech abnormalities, malnutrition related to inability to masticate and intraoral trauma while chewing related to malocclusion)
  5. Treatment of significant malocclusion without current medical complications, that cannot be effectively corrected with orthodontic treatment alone
  6. Cosmetic enhancement of facial features
  7. Adjunctive treatment for obstructive sleep apnea (OSA) 

Because repositioning the maxilla and/or mandible also moves the teeth, orthognathic surgery is usually performed in conjunction with orthodontics (braces), so the teeth are in proper position after surgery. The complete process usually takes place in several phases over the course of one to two years or more.

Phase 1: Treatment planning
Preoperative treatment planning includes a photographic analysis and a complete orthognathic work-up involving cephalometric and panorex radiographs, dental impressions and models. This is done by the pedodontist/orthodontist in coordination with the maxillofacial surgeon. All findings are analyzed, and pre-surgical model surgery is performed to simulate the surgery and predict the results before actually performing the procedure. Additionally, the maxillofacial surgeon does pre-surgery computer analysis to simulate surgical results, thereby facilitating proper planning of the case.

Phase 2: Pre-surgical orthodontics
This phase involves alignment of the teeth into a stable relationship with the underlying jaw, which prepares the dental arches for the surgical repositioning. Phase two usually takes the longest (may take nine to 18 months depending on the patient’s age, cooperation and other factors). At this phase, the abnormal bite (malocclusion) may become more noticeable. After the pre-surgical orthodontic phase of the treatment has been completed, a new set of dental records will be obtained that will include a cephalometric film,
a panoramic film, as well as new models of the teeth in the upper and lower jaws. This information will aid the maxillofacial surgeon in finalizing the surgical movements, as well as creating a surgical splint, which will serve as a guide for proper intraoperative jaw positioning.

Phase 3: Surgery
Surgery is scheduled when the pre-surgical orthodontic phase is completed. Braces used to align teeth before surgery are left in place during the surgical procedure. They help in stabilizing the teeth and jaws after surgery. The operation may involve one or both jaws (maxilla and/or mandible).

Phase 4: Post-surgical orthodontics
The orthodontist will usually begin the post-surgical phase of orthodontic treatment four-six weeks after surgery. Orthodontic treatment is continued to achieve final alignment of the teeth and to retain them in their new position. Fixed or removable retainers may be required following removal of orthodontics
.

Policy:
Orthognathic surgery may be considered MEDICALLY NECESSARY for each of the following clinical indications when the guidelines listed below are met: Elements must be met under one of the following:  A, B, C, D or E.

A) Correction of significant congenital (apparent at birth) deformity. Specific procedures covered are noted in Guidelines. Also please refer to "Note" at the beginning of Policies section ("Abnormal growth of the jaws (resulting in maxillary and/or mandibular hypo- or hyperplasia) is NOT considered a congenital anomaly and in the absence of MEDICAL complications is not eligible for coverage").

OR

B) Restoration of function following treatment for significant accidental injury, infection or tumor.

OR

C) Treatment of malocclusion that contributes to recalcitrant temporomandibular (TMJ) syndrome symptoms. Medical necessity criteria for orthognathic surgery for TMJ syndrome symptoms include both one and two:

1) Signs and/or symptoms are present for at least four months. At least one sign and one symptom of TMJ disorder must be present:

(a) Symptoms must include at least one of the following:

(i) Painful chewing clearly related to the TMJ

(ii) Frequent and significant headaches clearly related to TMJ

(iii) Significant and persistent joint and/or muscle tenderness

AND

(b) Clinical signs must include at least one of the following and must be accompanied by X-rays, cephalometric diagrams and photos that support measurements:

(i) Class III or IV internal derangement of the TMJ, OR

(ii) Restricted range of motion, including at least one of the following:

  • Interincisal opening < 30 mm
  • Lateral excursive movement < 4mm
  • Protrusive excursive movement < 4mm

(iii) Significant malocclusion or dental misalignment characterized by one of the following:

  • For patients with mandibular excess or maxillary deficiency, a reverse overjet (ROJ) of at least 3 mm

OR

  • In mandibular deficiency an overjet (OJ) of at least 6 mm

OR

  • Open bite (OB) of at least 4 mm or deep bite (DB) of at least 7 mm

For these four conditions (ROJ, OJ, OB, DB) the measurement should be calculated without assuming the final results of the preoperative orthodontics or splinting.

AND

2) Symptoms are unresponsive to conservative measures for four months, including all of the following:

(a) elimination of aggravating factors (e.g., gum chewing, chewing hard or tough foods)

AND

(b) use of anti-inflammatory drugs unless contraindicated. Therapeutic level for at least six weeks,  AND

(c) Treatment with orthodontic and/or splint therapy (Note: In many cases orthodontic treatment alone cannot correct the abnormality. When it has been determined in advance by cephalometrics and clinical examination that no amount of orthodontic manipulation will achieve satisfactory results, then a failed course of orthodontic therapy will not be required for approval for surgery. Likewise, some patients [large open bite patients] cannot tolerate splints, as this actually aggravates the problem.)

OR

D) Treatment of malocclusion that contributes significantly to any one of the following (1, 2 or 3) and has failed > four months of non-operative therapy.

1) Speech Abnormality

Medically necessity criteria should include both of the following:

(a) Speech deficit is noticeable to a lay person or primary care physician and significantly impairs the patient’s ability to communicate (disturbance or impairment of sibilant sound class is not considered a significant functional impairment)

(b) The speech deficit cannot be resolved by speech therapy (requires speech therapy evaluation)

OR

2) Malnutrition related to choking, difficulty swallowing or an inability to masticate that results in:

(a) significant weight loss and/or failure to thrive documented in the records over four months; OR

(b) low serum albumin related to malnutrition

OR

3) Significant intraoral trauma while chewing related to malocclusion. Information should be supplied that indicates the severity and duration of the trauma and the extent of the interruption to daily activities. This may include recurrent damage to the soft tissues of the mouth during mastication, lower incisors injuring the soft tissue of the palate, cheek biting, lip biting, impingement or irritation of buccal or lingual soft tissues of the opposing arch. The injury or damage to soft tissues must be documented by objective findings in the medical record and supported by photos.

OR

E) Treatment of documented obstructive sleep apnea.

Maxillofacial surgery, including mandibular-maxillary advancement (MMA), may be considered medically necessary in patients with mandibular and maxillary deformities contributing to airway dysfunction when there is:

a. Clinically significant OSA (documented by a supervised polysomnography in a sleep laboratory with appropriate monitoring by skilled personnel); AND

b. Objective documentation of hypopharyngeal obstruction by physical examination; AND

c. Failure of non-surgical treatments, including a good faith effort at treatment with CPAP or BiPAP; AND

d. Expectation that orthognathic surgery will decrease airway resistance and improve breathing.

Orthognathic surgery will not be approved as the first surgical therapy for OSA unless otolaryngology evaluation has ruled out obstruction at a higher anatomic level.

The following are considered NOT MEDICALLY NECESSARY and are not covered:

1) Orthognathic surgery performed primarily for cosmetic purposes

2) Orthognathic surgery performed for malocclusion when the criteria listed above are not met.

3) Orthognathic surgery where significant risk of recurrence of symptoms or structural abnormalities exist. For treatment of mandibular excess, skeletal maturation must be documented by either:

(a) closure of the epiphyses at the wrist by radiography OR

(b) no change in mandibular or facial growth on serial cephalometric radiographs over six months

4) Orthognathic surgery performed to reshape or enhance the size of the chin to restore facial harmony and chin projection (e.g., genioplasty, mentoplasty chin augmentation, chin implants, mandibular osteotomies, ostectomies). Procedures to address genial hypoplasia, hypertrophy or asymmetry, when performed either as an isolated procedure or with other procedures, are considered cosmetic in nature.

5) Cosmetic augmentation of the mandibular angle or body is not covered. This procedure may be performed to add prominence and balance to the face.

Benefits Application:
BlueCard/National Account Issues:
This medical policy relates only to the services or supplies described herein. Please refer to the Member's Benefit Booklet for availability of benefits. Member's benefits may vary according to benefit design. Therefore, member benefit language should be reviewed before applying the terms of this medical policy.

Braces and any other orthodontic services are considered dental in nature and are not covered as a medical benefit. Some dental policies offer orthodontic services as part of the dental benefit.

Rationale:
Orthognathic surgery is the revision by ostectomy, osteotomy or osteoplasty of the upper jaw (maxilla) and/or the lower jaw (mandible) intended to alter the relationship of the jaws and teeth. These surgical procedures are intended (i) to correct skeletal jaw and cranio-facial deformities that may be associated with significant functional impairment and (ii) to reposition the jaws when conventional orthodontic therapy alone is unable to provide a satisfactory, functional dental occlusion within the limits of the available alveolar bone. Congenital or developmental defects can interfere with the normal development of the face and jaws.  These birth defects may interfere with the ability to chew properly, and may also affect speech and swallowing. In addition, trauma to the face and jaws may create skeletal deformities that cause significant functional impairment. Functional deficits addressed by this type of surgery are those that affect the skeletal masticatory apparatus such that chewing, speaking and/or swallowing are impaired.

There is limited evidence of the effectiveness of orthognathic surgery on temporomandibular disorders. Abrahamsson et al. (2007) examined if orthognathic surgery does affect the prevalence of signs and symptoms of temporomandibular disorders (TMDs). A literature survey in the PubMed and Cochrane Library electronic databases was performed and covered the period from January 1966 to April 2006. The inclusion criteria were controlled, prospective or retrospective studies comparing TMDs before and after orthognathic surgery in patients with malocclusion. There were no language restrictions, and three reviewers selected and extracted the data independently. The quality of the retrieved articles was evaluated by four reviewers. The search strategy resulted in 467 articles, of which three met the inclusion criteria. Because of few studies with unambiguous results and heterogeneity in study design, the scientific evidence was insufficient to evaluate the effects that orthognathic surgery had on TMD. Moreover, the studies had problems with inadequate selection description, confounding factors and lack of method error analysis. The authors concluded that to obtain reliable scientific evidence, additional well-controlled and well-designed studies are needed to determine how and if orthognathic surgery alters signs and symptoms of TMD.

Lindenmeyer et al. (2010) performed a systematic review of the best available research literature investigating the relation of oral and maxillofacial surgical procedures to the onset or relief of chronic painful TMD. A comprehensive review of the databases CINAHL, Cochrane Library, Embase, Medline, NHS Evidence-Oral Health, PsycINFO, Web of Knowledge and MetaLib was undertaken by two authors up to June 2009 using search terms appropriate to establishing a relation between orofacial surgical procedures and TMD.  The search was restricted to English-language publications. Of the 1,777 titles reviewed, 35 articles were critically appraised, but only 32 articles were considered eligible. These were observational studies that fell into two groups. Nine were seeking to establish a surgical cause for TMD. Of these, only two of a series of three claimed that there was a significant link, but this claim was based on weak data (health insurance records) and was abandoned in a subsequent report. Twenty-three studies were seeking to achieve relief by orthognathic surgical intervention. These were also negative overall, with seven articles showing varying degrees of mostly non-significant improvement, whereas 16 showed no change or a worse outcome. No published report on the putative effect of implant insertion was found. The authors concluded that these apparently contradictory approaches underline a belief that oral surgical trauma or gross malocclusion has a causative role in the onset of TMD. However, there was no overall evidence of a surgical causal etiology or orthognathic therapeutic value. This review emphasized that it is in the patients' best interest to carry out prospective appropriately controlled randomized trials to clarify the situation.

In a Cochrane review, Luther et al. (2010) examined the effectiveness of orthodontic intervention in reducing symptoms in patients with TMD (compared with any control group receiving no treatment, placebo treatment or reassurance) and investigated if active orthodontic intervention leads to TMD. The Cochrane Oral Health Group's Trials Register, CENTRAL, MEDLINE and EMBASE were searched. Hand-searching of orthodontic journals and other related journals was undertaken in keeping with the Cochrane Collaboration hand-searching program. No language restrictions were applied. Authors of any studies were identified, as were experts offering legal advice, and contacted to identify unpublished trials. Most recent search was April 13, 2010. All randomized controlled trials (RCTs), including quasi-randomized trials assessing orthodontic treatment for TMD, were included. Studies with adults aged equal to or above 18 years old with clinically diagnosed TMD were included. There were no age restrictions for prevention trials, provided the follow-up period extended into adulthood. The inclusion criteria required reports to state their diagnostic criteria for TMD at the start of treatment and for participants to exhibit two or more of the signs and/or symptoms. The treatment group included treatment with appliances that could induce stable orthodontic tooth movement. Patients receiving splints for eight to 12 weeks and studies involving surgical intervention (direct exploration/surgery of the joint and/or orthognathic surgery to correct an abnormality of the underlying skeletal pattern) were excluded. The outcomes were: how well were the symptoms reduced, adverse effects on oral health and quality of life. Screening of eligible studies, assessment of the methodological quality of the trials and data extraction were conducted in triplicate and independently by three review authors. As no two studies compared the same treatment strategies (interventions) it was not possible to combine the results of any studies. The searches identified 284 records from all databases. Initial screening of the abstracts and titles by all review authors identified 55 articles that related to orthodontic treatment and TMD. The full articles were then retrieved, and of these articles, only four demonstrated any data that might be of value with respect to TMD and orthodontics. After further analysis of the full texts of the four studies identified, none of the retrieved studies met the inclusion criteria, and all were excluded from this review. The authors concluded that there are insufficient research data on which to base clinical practice on the relationship of active orthodontic intervention and TMD. There is an urgent need for high quality RCTs  in this area of orthodontic practice.

There is a lack of evidence to support the use of condylar positioning devices in orthognathic surgery. Costa et al. (2008) stated that in the past few years, many devices have been proposed for preserving the pre-operative position of the mandibular condyle during bilateral sagittal split osteotomy. The authors stated that accurate mandibular condyle re-positioning is considered important to obtain a stable skeletal and occlusal result, and to prevent the onset of TMD. Condylar positioning devices (CPDs) have led to longer operating times, the need to keep inter-maxillary fixation as stable as possible during their application and the need for precision in the construction of the splint or intra-operative wax bite. The authors reviewed the literature concerning the use of CPDs in orthognathic surgery since 1990 and their application to prevent skeletal instability and contain TMD since 1995. They concluded that there is no scientific evidence to support the routine use of CPDs in orthognathic surgery.

Definitions and Descriptions:

  • Class I occlusion: Exists with the teeth in a normal relationship when the mesial-buccal cusp of the maxillary first permanent molar coincides with the buccal groove of the mandibular first molar.
  • Class II malocclusion: Occurs when the mandibular teeth are distal or behind the normal relationship with the maxillary teeth. This can be due to a deficiency of the lower jaw or an excess of the upper jaw, and, therefore, presents two types: (1) Division I is when the mandibular arch is behind the upper jaw with a consequential protrusion of the upper front teeth. (2) Division II exists when the mandibular teeth are behind the upper teeth, with a retrusion of the maxillary front teeth. Both of these malocclusions have a tendency toward a deep bite because of the uncontrolled migration of the lower front teeth upward.
  • Class III malocclusion: Occurs when the lower dental arch is in front of (mesial to) the upper dental arch. People with this type of occlusion usually have a strong or protrusive chin, which can be due to either horizontal mandibular excess or horizontal maxillary deficiency. Commonly referred to as an under bite.

Maxillary advancement is a type of orthognathic surgery that may be necessary to improve the facial contour and normalize dental occlusion when there is a relative deficiency of the midface region. This is done by surgically moving the maxilla with sophisticated bone mobilization techniques and fixing it securely into place.

Depending on the soft tissue profile of the face or the severity of an occlusal discrepancy, problems with the lower face may require surgery on the mandible. This can be done in conjunction with or separate from maxillary surgery. The mandible can be advanced, set back, tilted or augmented with bone grafts. A combination of these procedures may be necessary. Following any significant surgical movement of the mandible, fixation may be accomplished with mini-plates and screws or with a combination of interosseous wires and intermaxillary fixation (IMF). Rigid fixation (screws and plates) has the advantage of needing limited or no IMF. However, if interosseous wiring is used, IMF is maintained for approximately six weeks.

Anomaly: Deviation from normal.

Anteroposterior: From front to back.

Asymmetry: The lack of balance or symmetry.

Cephalometric: A scientific measurement of the head.

Cephalometrics: The interpretation of lateral skull X-rays taken under standardized conditions.

Dentoalveolar: Relating to a tooth and the part of the alveolar bone that immediately surrounds it.

Dysphagia: Difficulty swallowing.

Genioplasty: Plastic surgery of the chin.

Malformation: An abnormal shape or structure.

Malocclusion: Imperfect contact with the mandibular and maxillary teeth.

Mandible: The horseshoe-shaped bone forming the lower jaw.

Mastication: Biting and grinding food in the mouth so it becomes soft enough to swallow.

Maxilla: A paired bone that forms the skeletal base of the upper face, roof of the mouth, sides of the nasal cavity and floor of the orbit (contains the eye). The upper jaw.

Occlusion: Bringing the opposing surfaces of the teeth of the two jaws (mandible and maxilla) into contact with each other.

Orthodontics: The division of dentistry dealing with the prevention and correction of abnormally positioned or aligned teeth.

Panoramic radiograph: Radiograph of the maxilla and mandible extending from the left to right glenoid fossa. An X-ray image of a curved body surface, such as the upper and lower jaws, on a single film.

Radiograph: X-ray.

Supraeruption: The occurrence of a tooth continuing to grow out of the gum if the opposing tooth in the opposite jaw is missing.

Tomogram: An image of a tissue section produced by tomography.

Tomography: Imaging by sections or sectioning, through the use of any kind of penetrating wave.

Documentation Requirements
If coverage for orthognathic surgery is available, the following clinical documentation is required to support medical necessity for orthognathic surgery:

  • Medical history and physical examination with reference to symptoms related to the orthognathic deformity
  • Description of specific anatomic deformity present
  • Lateral and anterior-posterior cephalometric radiographs
  • Cephalometric tracings
  • Copy of medical records from treating physician documenting evaluation, diagnosis and previous management of the functional medical impairment(s)
  • Diagnostic quality (clear) photographs that fully demonstrate the dental occlusion

Molds may also be requested, depending on the individual circumstances of the case.

References:

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Coding Section

Codes Number Description
CPT 21120

Genioplasty; augmentation (autograft, allograft, prosthetic material)

  21121

Genioplasty; sliding osteotomy, single piece

  21122

Genioplasty; sliding osteotomies, two or more osteotomies (e.g., Wedge excision or bone wedge reversal for asymmetrical chin)

  21123

Genioplasty; sliding, augmentation with interpositional bone grafts (includes obtaining autografts)

  21125

Augmentation, mandibular body or angle; prosthetic material

  21141

Reconstruction midface, LeFort I; single piece, segment movement in any direction (e.g., for Long Face Syndrome), without bone graft

  21142

Reconstruction midface, LeFort I; two pieces, segment movement in any direction, without bone graft

  21143

Reconstruction midface, LeFort I; three or more pieces, segment movement in any direction, without bone graft

  21144

LeForte I maxillary osteotomy; single piece (Code deleted)

  21145

Reconstruction midface, LeFort I; single piece, segment movement in any direction, requiring bone grafts (includes obtaining autografts)

  21146

Reconstruction midface, LeFort I; two pieces, segment movement in any direction, requiring bone grafts (includes obtaining autografts) (e.g., ungrafted unilateral alveolar cleft)

  21147

Reconstruction midface, LeFort I; three or more pieces, segment movement in any direction, requiring bone grafts (includes obtaining autografts) (e.g., ungrafted bilateral alveolar cleft or multiple osteotomies)

  21150

Reconstruction midface, LeFort II; anterior intrusion (e.g., Treacher-Collins Syndrome)

  21151

Reconstruction midface, LeFortII; any direction, requiring bone grafts (includes obtaining autografts)

  21154

Reconstruction midface, LeFort III (extracranial), any type, requiring bone grafts (includes obtaining autografts); without LeFort I

  21155

Reconstruction midface, LeFort III (extracranial), any type, requiring bone grafts (includes obtaining autografts); with LeFort I

  21193

Mandibular ramus osteotomy (Horizontal, Vertical, C or L); without graft: right; left

  21194

Mandibular ramus osteotomy (Horizontal, Vertical, C or L); with graft: right; left

  21195

Mandibular sagittal split osteotomy; without rigid fixation: right; left

  21196

Mandibular sagittal split osteotomy; with rigid fixation: right; left

  21198

Mandibular segmental osteotomy

  21206

Osteotomy, maxilla, segmental (e.g., Wassmund or Schuchard)

  21208

Osteoplasty (facial bones); augmentation

  21209

Osteoplasty (facial bones); reduction

  21210

Graft, bone; nasal, maxillary or malar areas (include obtaining graft)

HCPCS  D7940

Osteoplasty – for orthognathic deformities

  D7944

Osteotomy – segmented or subapical – per sextant or quadrant

  D7946

LeFort I (maxilla - total)

  D7947

LeFort I (maxilla - segmented)

  D7948

LeFort II or LeFort III (osteoplasty of facial bones for midface hypoplasia or retrusion) - without bone graft

  D7949

LeFort II or LeFort III - with bone graft

  D7950

Osseous, osteoperiosteal or cartilage graft of the mandible or facial bones – autogenous or nonautogenous, by report

  D7995

Synthetic graft - mandible or facial bones, by report

  D7996

Implant - mandible for augmentation purposes (excluding alveolar ridge), by report

ICD-9 Diagnosis 253.0

Acromegaly

  346.8

Mobius syndrome

  520.5

Amelogenesis imperfecta

  524

Dentofacial anomalies

  524.00

Unspecified anomaly of jaw size

  524.01

Maxillary hyperplasia

  524.02

Mandibular hyperplasia

  524.03

Maxillary hypoplasia (Pfeiffer syndrome)

  524.04

Mandibular hypoplasia (Goldenhar syndrome/Hemifacial Microsomia; Hallermann-Streiff syndrome; Robin Complex/Pierre-Robin syndrome)

  524.05

Macrogenia

  524.06

Microgenia

  524.09

Other specified anomaly of jaw size

  524.1

Anomalies of relationship of jaw to cranial base

  524.10

Unspecified anomaly of relationship of jaw to cranial base

  524.11

Maxillary asymmetry

  524.12

Other jaw asymmetry

  524.19

Other specified anomaly

  524.2

Anomalies of dental arch relationship

  524.4

Dentofacial anomalies, including malocclusion; Malocclusion, unspecified

  524.5

Dentofacial anomalies, including malocclusion; Dentofacial functional abnormalities: Abnormal jaw closure; Malocclusion due to: abnormal swallowing, mouth breathing, tongue, lip or finger habits

  524.7

Dentoalveolar anomalies

  524.70

Unspecified alveolar anomaly

  524.71 Alveolar maxillary hyperplasia
  524.72

Alveolar hyperplasia 

  524.73

Alveolar maxillary hypoplasia 

  524.74

Alveolar mandibular hypoplasia 

  524.79

Other specified alveolar anomaly 

  526.89

Unilateral condylar hyperplasia or hypoplasia of mandible 

  738.1

Other acquired deformity of the head 

  738.10

Unspecified acquired deformity of head 

  738.11

Zygomatic hyperplasia 

  738.12

Zygomatic hypoplasia (Treacher-Collins syndrome/Mandibulofacial Dysostosis) 

  733.81

Malunion of fracture 

  733.82

Nonunion of fracture

  738.1

Other acquired deformity of the head

  738.10

Unspecified acquired deformity of head

  738.11

Zygomatic hyperplasia 

  738.12

Zygomatic hypoplasia (Treacher-Collins syndrome/Mandibulofacial Dysostosis) 

  738..19

Other specified acquired deformity of head

  744.9

Congenital anomalies of ear, face and neck; Unspecified anomalies of face and neck; Congenital: anomaly NOS, deformity NOS; of face (any part) or neck (any part) 

  748.1

Congenital anomalies of respiratory system; Other anomalies of nose, Absent nose, Accessory nose, Cleft nose, Congenital: deformity of nose, Congenital: notching of tip of nose, Congenital: perforation of wall of nasal sinus, Deformity of wall of nasal sinus 

  754.0

Hemifacial atrophy or hemifacial hypertrophy 

  455.55 Apert’s syndrome/Acrocephalosyndactyly
  755.59

Cleidocranial Dysplasia/Cleidocranial Dysotosis 

  758.83

Ehlers-Danlos syndrome 

  756.0

Crouzon’s syndrome/Craniofacial Synostosis 

  756.4

Achondroplasia 

  756.51

Osteogenesis Imperfecta 

  756.52

Osteopetrosis/Albers-Schonberg’s disease 

  756.55

Chondroectodermal Dysplasia/Ellis-Van Creveld syndrome 

  758.7

Klinefelter syndrome 

  759.89

Gorlin syndrome/Basal Cell Nevus syndrome 

  759.82

Marfan syndrome 

  780.53

Hypersomnia with sleep apnea 

ICD-10-CM (effective 10/01/15)  E22.0 

Acromegaly and pituitary gigantism 

  G47.30

Sleep apnea, unspecified 

  G51-G51.9 

Facial nerve disorders 

  K00-K00.9

Disorders of tooth development and eruption 

  M26-M26.9 

Dentofacial anomalies (including malocclusion) 

  M27-M27.9 

Other diseases of jaw 

  M85.2 

Hyperostosis of skull 

  M89.38

Hypertrophy of bone, other site 

  M89.8X8

Other specified disorders of bone, other site 

  M95.2

Other acquired deformity of head 

  Q18-Q1839

Other congenital malformations of face and neck 

  Q67.0

Congenital facial asymmetry 

  Q67.1

Congenital compression facies 

  Q67.2

Dolichocephaly 

  Q67.3

Plagiocephaly 

  Q67.4

Other congenital deformities of skull, face and jaw

  Q75.0 

Craniosynostosis 

  Q75.2

Hypertelorism 

  Q75.9

Congenital malformation of skull and face bones, unspecified 

  Q77.4

Achondroplasia 

  Q77.6

Chondroectodermal dysplasia 

  Q78.0

Osteogenesis imperfecta 

  Q78.2

Osteopetrosis 

  Q87.0

Congenital malformation syndromes predominantly affecting facial appearance 

  Q87.40

Marfan's syndrome, unspecified 

  Q98.4

Klinefelter syndrome, unspecified 

  Q99.8

Other specified chromosome abnormalities 

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive. 

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other nonaffiliated technology evaluation centers, reference to federal regulations, other plan medical policies and accredited national guidelines.

"Current Procedural Terminology © American Medical Association. All Rights Reserved" 

History From 2014 Forward     

07/20/2022 Annual review, no change to policy intent

07/01/2021 

Annual review, no change to policy intent 

07/01/2020 

Annual review, no change to policy intent. 

07/01/2019 

Annual review, no change to policy intent. 

07/09/2018 

Annual review, no change to policy intent. 

07/03/2017 

Annual review, no change to policy intent. 

07/01/2016 

Annual review, no change to policy intent. 

07/06/2015 

Annual review, no change to policy intent. Added coding. 

09/11/2014 

Added additional disclaimer regarding coverage being related to the individual's plan document. No change to intent of policy,

07/10/2014

Annual review, no changes made.

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