Congenital and acquired anomalies and deformities of the dentofacial system and facial skeleton: clinical guidelines

Applies toadults2023 editionK07.0, K07.1, K07.4, Q18

Clinical Guidelines of the Russian Ministry of Health, adults. 2023 edition, ICD-10 codes: K07.0, K07.1, K07.4, Q18.

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Brief for Practice

WhiteCoat Pro editorial summary of the clinical guideline text, 2023 edition. Each point links to the original section — consult it before prescribing.

Congenital and acquired anomalies of the dentofacial system and facial skeleton are associated with impaired growth and shape of the jaws, their interrelations, and symmetry; causes include genetic, traumatic, inflammatory, neoplastic, and external factors. The guideline covers diagnostics, orthodontic-surgical treatment planning, postoperative rehabilitation, and patient follow-up.

Key points

Diagnostics

  1. Take the medical history and clarify complaints regarding changes in facial and jaw shape or asymmetry, tooth loss, impaired mastication and mandibular movements, clicking, crepitus, or pain in the temporomandibular joint (TMJ), and impaired nasal breathing and speech (LRE C, LTD 5). → in the text
  2. During the examination, assess facial and facial skeleton symmetry, bite, and intermaxillary relations; perform anthropometric measurements and evaluate dental casts (LRE C, LTD 5). → in the text
  3. Perform dental and general somatic assessments; in case of psychological instability, additionally assess the neuropsychiatric status. → in the text
  4. Perform CT of the facial skull — CBCT or MSCT, with or without 3D spatial reconstruction; the recommended slice thickness is no more than 0.5 mm (LRE C, LTD 5). → in the text
  5. Perform TMJ MRI to assess the position and integrity of the articular disc and other soft tissue structures (LRE C, LTD 5). → in the text
  6. During preoperative preparation, perform laboratory tests to rule out comorbid conditions (LRE C, LTD 5). → in the text
  7. All patients with malocclusion pathology are recommended consultations with an orthodontist, geneticist, prosthodontist, and otorhinolaryngologist; prior to surgery — consultations with an anesthesiologist-resuscitator and general dentist (LRE C, LTD 4–5). → in the text
  8. In case of breathing disorders, assess the state of ENT organs and external respiration function using PARM, acoustic rhinometry, and/or anterior rhinomanometry. → in the text

Treatment

When to refer and how to monitor

Frequently asked questions

What investigations are mandatory before treating malocclusion pathology?

All patients with malocclusion pathology are recommended CT of the facial skull — CBCT or MSCT, with or without 3D spatial reconstruction, and TMJ MRI (LRE C, LTD 5). → in the text

Is surgery always necessary for skeletal anomalies?

Surgery is the primary treatment method for skeletal dentofacial anomalies. Tactics are determined individually; in severe decompensated diseases, surgery is postponed until condition improvement. → in the text

How is Skeletal Class III treated?

BSSO is performed for mandibular overgrowth. Le Fort I osteotomy is used for maxillary hypoplasia, and operations on both jaws are performed when disorders are combined (LRE C, LTD 5). → in the text

Is it possible to do without preoperative orthodontics?

Preoperative orthodontic preparation and postoperative correction are usually performed. In certain cases, a SURGERY FIRST protocol without prior orthodontic preparation is possible. → in the text

How should the patient be monitored postoperatively?

Following surgical treatment, examinations by a maxillofacial surgeon and orthodontist are conducted regularly, at least once every 6 months. If relapse, secondary deformity, or clinical deterioration is suspected, total skull CT, brain MRI, EEG, and other investigations are prescribed according to the clinical picture (LRE C, LTD 4–5). → in the text

Status
Applied · approved by the Scientific and Practical Council of the Russian Ministry of Health
Database ID
779, revision 1 (ID 779_1)
Publication date
December 29, 2023
Age category
adults
ICD-10 codes
K07.0K07.1K07.4Q18
Specialties
Dentistry and Maxillofacial Surgery
Developers
LLC "Association of Maxillofacial Surgeons"
Official text
Russian Ministry of Health Rubricator — 779_1

List of Abbreviations

TMJ — temporomandibular joint

OPG — orthopantomogram

cephalogram — lateral cephalometric radiogram

MSCT — multislice computed tomography

CBCT — cone-beam computed tomography

CG — clinical guidelines

MRI — magnetic resonance imaging

AAR — active anterior rhinomanometry

AR — acoustic rhinometry

BSSO —  Bilateral Sagittal Split Osteotomy

3D — three-dimensional reconstruction

Terms and Definitions

Dentofacial anomaly (deformity) is a developmental disproportion (ratio mismatch) of the jaws characterized by functional and aesthetic disorders. Anomalies and deformities may involve the upper and lower jaws, zygomatic bones, nasal structures, and other facial bones.

Orthognathic surgery (from ortho- — Lat. straight, gnatos- — Lat. jaw) is a branch of maxillofacial surgery dedicated to the normalization of the shape, size, position, and proportion of the jaws.

Genioplasty — osteotomy of the mandibular symphysis region to alter chin contours, performed for aesthetic purposes.

Orthognathic surgery includes various types of procedures: single-jaw osteotomy (maxillary — Le Fort I type, or mandibular — sagittal split / intercortical osteotomy), maxillary segmental osteotomy, surgical expansion of the hard palate, bimaxillary osteotomy, genioplasty, zygomatic bone osteotomy, marginal mandibular resection, and surgical techniques aimed at preventing nasal breathing impairment.

Anomaly (from Greek ανωμαλία) — a congenital deviation from the norm or general pattern.

Deformity (from Latin deformatio — distortion) — a change in the size, shape, and configuration of a bone resulting from external or internal factors.

Occlusion — the manner in which the dental arches meet.

Distal occlusion — a type of malocclusion characterized by the posterior displacement of the lower dental arch relative to the upper, incisor disocclusion, and disturbed sagittal relationships of the posterior teeth.

Bite (Occlusion) — the relationship of the jaws in the position of central occlusion.

Mesial occlusion — a type of malocclusion caused by the anterior displacement of the mandible relative to the maxilla.

Vertical incisor disocclusion — a type of malocclusion caused by the absence of occlusal contacts between the incisors.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Developmental anomalies of the dentofacial system are a symptom of impaired development of the entire organism, such as hormonal dysregulation or genetically determined anomalies of human skeletal development. Pathologies of jaw size and shape occur with a hereditary predisposition and congenital anomalies of the maxillofacial region (congenital cleft lip, alveolar ridge, and palate, hemifacial microsomia, Robin and Goldenhar syndromes, etc.), developmental disorders of the temporomandibular joints, jaw trauma with growth zone damage, as well as after rickets and diseases associated with metabolic disorders, particularly calcium metabolism and endocrinopathies [1,2,5,9]. Less significant, yet possible, causes may include: nasal breathing impairment, macroglossia, improper breastfeeding in infancy, caries complications, insufficient chewing load associated with a disproportion in the size of teeth and individual jaw segments [3,6,8].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

According to modern concepts, causes of dentofacial anomalies may include genetic diseases, trauma, inflammatory and tumor processes, environmental factors, parafunctions, and bad habits [11].

All of the above can, to a varying degree, influence jaw growth and thus lead to the development of a dentofacial anomaly.

1.3 Epidemiology of the Disease or Condition

According to international statistics, about 45% of the population has a malocclusion characterized by an absence or such minor jaw relationship disturbance that it can be corrected solely by orthodontic treatment [10].

Nevertheless, 5% to 15% of people have a dentofacial anomaly characterized by a significant disproportion in jaw growth, requiring combined treatment incorporating the principles of orthognathic surgery [9].

1.4 Features of Coding the Disease or Condition

Q18 Other congenital malformations [birth defects] of the face and neck

K07.0 Major anomalies of jaw size

  • Hyperplasia of mandible or maxilla

  • Hypoplasia of mandible or maxilla

  • Macrognathia of mandible or maxilla

  • Micrognathia of mandible or maxilla

K07.1 Anomalies of jaw-cranial base relationship

  • Asymmetry of jaw

  • Prognathia of mandible or maxilla

  • Retrognathia of mandible or maxilla

K07.4 Malocclusion, unspecified (idiopathic root resorption)

1.5 Classification of the disease or condition

Currently, LLC "Specialists in Maxillofacial Surgery" uses the following working classification of skeletal dentofacial anomalies:

  • By the type of dental arch occlusion anomaly:

- sagittal throughout: distal occlusion / mesial occlusion;

- transversal throughout: crossbite;

- vertical in the anterior segment: vertical incisor disocclusion.

  • By the type of intermaxillary relationship based on occlusion:

- Skeletal Class II (corresponds to distal bite type);

- Skeletal Class III (corresponds to mesial bite type);

- jaw constriction (corresponds to crossbite).

  • By symmetry:

- symmetrical;

- asymmetrical / non-symmetrical.

1.6 Clinical presentation of the disease or condition

The clinical presentation of dentofacial anomalies is diverse. This is explained by the complexity of the anatomical structure and functioning of the dentofacial apparatus. Clinical symptoms and syndromes in adult patients with dentofacial anomalies are often significantly more complex than in children [1].

This is due to the addition of tooth loss, dental arch and jaw deformities, functional periodontal overload, and occlusal disorders to the underlying condition [8].

2. Diagnostics, medical indications and contraindications for diagnostic methods

Diagnosis of congenital and acquired anomalies and deformities of the dentofacial system and facial skeleton is established based on history taking, clinical patient evaluation, dental cast evaluation, anthropometric analysis, and radiological examination data (OPG, lateral cephalometry, CBCT or MSCT, MRI).

Required differential diagnostic examinations:

  • History taking;

  • Assessment of local status;

  • Assessment of dental status;

  • Radiological examination (OPG, lateral cephalometry, CBCT or MSCT, MRI);

  • Assessment of somatic status;

  • Assessment of neuropsychiatric status (in case of labile psyche);

  • Assessment of ENT organ status in case of breathing function impairment (assessment of external respiration function: AAR, acoustic rhinometry and/or anterior rhinomanometry);

2.1 Complaints and Anamnesis

It is recommended to elicit complaints regarding changes in jaw or facial configuration, asymmetry of one or both jaws, facial asymmetry, partial tooth loss, difficulty moving the lower jaw, chewing food, clicking, popping, or pain in the area of one or both temporomandibular joints, impaired nasal breathing, and less commonly, speech defects, etc. [1,2,3,40].

GCP CLEL 5Grade of Recommendation – C (Level of Evidence – 5)

2.2 Physical Examination

The attending physician is recommended to begin the examination by assessing the symmetry of the facial skeleton and the skull as a whole, determining the bite (occlusion), and performing anthropometric measurements of the face [7].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: one of the main clinical manifestations of congenital or acquired anomalies and deformities of the dentofacial system and facial skeleton is visible deformity and impaired facial symmetry, as well as malocclusion and altered intermaxillary relationships. Assessment of these parameters is the primary stage of examination. Anthropometric measurements allow for an objective evaluation of the severity of the anomaly or deformity. Evaluation of plaster dental casts makes it possible to assess the state of occlusion [2].

2.3 Laboratory Diagnostics

  • Laboratory diagnostic tests are recommended for all patients with bite pathologies during the preparation and planning stages of surgical intervention to rule out comorbid conditions [6].

GCP CLEL 5Grade of Recommendation – C (Level of Evidence – 5)

2.4 Instrumental Diagnostics

  • All patients with bite pathologies are recommended to undergo computed tomography of the facial skull, with or without 3D spatial image reconstruction [4].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: the diagnosis of any of the described congenital or acquired anomalies or deformities of the dentofacial system and facial skeleton is impossible without CBCT or MSCT, which are fundamental for this category of patients. These imaging methods allow for a detailed study of the anatomy of the facial bones and the nature and developmental features of the anomaly or deformity mechanism. It is recommended to perform CBCT or MSCT with a scanner step/slice thickness of no more than 0.5 mm.

  • All patients with bite pathologies are recommended to undergo magnetic resonance imaging (MRI) of the temporomandibular joint (TMJ) [4, 5].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: this study allows for a detailed examination of the soft tissue structures of the temporomandibular joint (evaluating the integrity and position of the articular disc, as well as its spatial orientation within the joint).

2.5 Other Procedures

  • All patients with bite pathologies are recommended to consult an orthodontist [1, 2, 3].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: the vast majority of congenital or acquired anomalies and deformities of the dentofacial system are accompanied by malposition of the jaws and malocclusion. Surgical treatment of such patients is always accompanied by an orthodontic phase, during which the orthodontist corrects tooth positions, performs preoperative orthodontic preparation, and provides postoperative orthodontic correction.

  • All patients with bite pathologies are recommended to consult a geneticist [41].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: performed to identify the etiological cause of the disease when a dentofacial anomaly is suspected to be part of a syndromic symptom complex or associated with bone metabolism disorders.

  • All patients with bite pathologies are recommended to consult an anesthesiologist-resuscitologist prior to surgical treatment [42].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

Comments: the degree of anesthetic risk is assessed prior to any surgical intervention. Most surgeries for congenital or acquired anomalies and deformities of the dentofacial system and facial skeleton are highly invasive, involve osteotomy and bone repositioning, engage a large volume of soft tissues, and can be accompanied by significant blood loss, which may necessitate blood transfusion.

  • All patients with bite pathologies are recommended to consult a general dentist prior to surgical treatment [37, 42].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

Comments: evaluates the condition of the teeth and performs necessary procedures to prepare the patient for surgical treatment and subsequent rehabilitation.

  • All patients with bite pathologies are recommended to consult a prosthodontist [1, 2].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: evaluates the presence of dental arch defects and occlusal relationships, and performs necessary procedures to prepare the patient for surgical treatment and subsequent rehabilitation.

  • All patients with bite pathologies are recommended to consult an otolaryngologist [2, 6].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: evaluates the condition and prevents the development of complications involving ENT organs.

The diagnostic methods described above cover all aspects of the described conditions comprehensively; however, the pathology is so diverse, and the combination of symptoms in each specific case is so specific, that additional methods may be employed depending on the clinical manifestations. No other specific diagnostics beyond those described above are provided.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

Indications: surgery is the recommended method of treatment for skeletal dentofacial anomalies [1, 23].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Contraindications: surgical interventions are not recommended in the presence of severe decompensated diseases of various organs and systems. If a patient's condition can be improved through prior treatment, surgery becomes feasible [2, 36, 6].

GCR CLoE 5Level of recommendation grading – C (level of evidence – 5

Comments: performing surgery in patients under 18 years of age should be coordinated with an orthodontist, taking into account individual patterns of growth cessation to prevent relapses in the postoperative period.

3.1 Surgical Treatment

Currently, there is no single universal method for correcting a particular congenital or acquired anomaly or deformity of the dentofacial system and facial skeleton. Individual planning of the scope of surgical treatment is required in each specific case, depending on the type and severity of the anomaly or deformity, as well as the patient's ethnicity and age. Given the diversity and variability of anomalies and deformities, maxillofacial surgeons are advised to apply established surgical techniques to restore the function, shape, and symmetry of the dentofacial system and facial skull.

Key principles of surgical treatment for patients.

  1. All surgical treatment methods should aim to correct the anomaly or deformity while creating conditions for the normal functioning of the dentofacial system [1,3,5,9,24].

  2. Regardless of the degree of external anomaly or deformity, treatment initiation requires evaluating the parameters of the entire facial skeleton and applying a comprehensive approach [22].

  3. All patients with bite pathologies are recommended to plan the scope and techniques of the surgical intervention prior to operation to ensure higher-quality treatment [11,13].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

  • All patients with bite pathologies are recommended to use specialized computer software for modeling jaw repositioning and facial soft tissue changes prior to surgery to ensure higher-quality treatment [14,15,45].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

  • All patients with bite pathologies are recommended to use rigid fixation of bone structures (titanium plates and screws) during orthognathic surgery to achieve a stable postoperative outcome and significantly reduce the risk of relapse [35].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with bite pathologies are recommended to undergo preoperative orthodontic preparation and postoperative orthodontic correction to achieve a successful surgical outcome [21].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Comments: in some cases, the "SURGERY FIRST" approach can be used, in which preoperative orthodontic preparation is omitted.

Surgical treatment of patients with Class III skeletal malocclusion accompanied by mandibular overgrowth

  • All patients with Class III skeletal malocclusion are recommended to undergo bilateral sagittal split osteotomy (BSSO) of the mandible to correct its excessive dimensions [16, 34].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with an excessively large chin (mental) region of the mandible are recommended to undergo reduction genioplasty for aesthetic indications [17, 42, 43].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

Surgical treatment of patients with Class III skeletal malocclusion accompanied by maxillary hypoplasia

(normal or hypodivergent facial skeletal growth pattern)

  • All patients are recommended to undergo Le Fort I maxillary osteotomy (segmental osteotomy is also possible) [1,18, 20].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with an excessively large chin (mental) region of the mandible are recommended to undergo reduction genioplasty for aesthetic indications [2,19, 42, 43].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Surgical treatment of patients with Class III skeletal malocclusion accompanied by mandibular overgrowth and maxillary hypoplasia

  • All patients are recommended to undergo Le Fort I maxillary osteotomy (segmental osteotomy is possible) to increase its dimensions [19, 33].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to undergo bilateral sagittal split osteotomy (BSSO) of the mandible to reduce its dimensions [2,3].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with an excessively large chin of the mandible are recommended to undergo reduction genioplasty for aesthetic indications [1].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Surgical treatment of patients with skeletal Class II malocclusion accompanied by excessive maxillary development (hyperdivergent growth pattern) and mandibular retrognathia.

  • All patients are recommended to undergo Le Fort I maxillary osteotomy (segmental osteotomy is possible) to reduce its dimensions [1,2,3,32].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with a prominent gummy smile are recommended to perform a counterclockwise rotation of the maxilla to improve smile aesthetics [23,46].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

  • All patients are recommended to undergo bilateral sagittal split osteotomy (BSSO) of the mandible to increase its size [18]. Level of recommendation C (Level of evidence - 4)

Comments: genioplasty is performed for aesthetic indications.

Surgical treatment of patients with skeletal Class II malocclusion accompanied by mandibular retrognathia

  • All patients are recommended to undergo bilateral sagittal split osteotomy (BSSO) of the mandible to increase its dimensions [16,17].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to undergo Le Fort I maxillary osteotomy [31,46].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

  • All patients with an insufficiently sized chin of the mandible are recommended to undergo augmentation genioplasty for aesthetic indications [5,6].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Surgical treatment of patients with maxillary constriction and pronounced crossbite

  • All patients are recommended to assess their age and the degree of fusion of the palatal suture to determine further treatment tactics [1,2,7,4,5].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to consult an orthodontist regarding the possibility and feasibility of orthodontic expansion of the maxilla and dental arch [30].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients with fusion of the palatal suture or the impossibility of orthodontic dental arch expansion are recommended to undergo Le Fort I maxillary osteotomy and midline palatal suture osteotomy with fixation of a distraction device. The amount of distraction (maxillary expansion) is determined by the orthodontist [1,2].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Surgical treatment of patients with vertical anterior open bite

  • All patients are recommended to undergo segmental maxillary osteotomy with rotation of the anterior segment including four or six teeth to prevent postoperative relapse [15,29,46,48].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to undergo special orthodontic preparation consisting in creating spaces (tremas) between teeth where subsequent jaw osteotomy is planned in order to prevent damage to the tooth roots [4,5, 38].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to undergo Le Fort I maxillary osteotomy, bilateral sagittal split osteotomy (BSSO) of the mandible, and genioplasty [14,48].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

Surgical treatment of patients with asymmetric anomalies and deformities

  • All patients are recommended to use CBCT or MSCT data with specialized computer software to assess the potential occurrence of postoperative facial skull deformity [1,2,6,28, 43].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to perform Le Fort I maxillary osteotomy, bilateral sagittal split osteotomy (BSSO) of the mandible, genioplasty (for aesthetic indications); single-jaw surgery is possible according to indications (in case of symmetrical position of the upper or lower jaw) [13, 43, 48].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

  • All patients are recommended to use additional surgical techniques (options for marginal jaw resection or augmentation) to eliminate visible asymmetry after the main jaw repositioning and establishment of correct intermaxillary relations [27, 43].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

3.2 Other Treatment

No other specific treatment is provided for patients with congenital or acquired anomalies and deformities of the dentofacial system and facial skeleton.

  • For TMJ treatment, arthrocentesis, arthrolavage, and diagnostic arthroscopy are recommended [2,5].

LoR CLoE 5Level of recommendation C (Level of evidence - 5

  • Patients with impaired nasal breathing are recommended to undergo surgical correction of internal nasal structures according to indications [26,42].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

  • All patients are recommended to evaluate indications for the removal of subcutaneous adipose tissue (liposuction), plastic surgery of subcutaneous adipose tissue by autologous microfat transfer (lipofilling), platysmaplasty, the use of peripheral muscle relaxants (botulinum therapy), as well as the full arsenal of plastic surgery (facial contouring and rejuvenation) to improve aesthetic parameters — [12, 24].

LoR CLoE 5Level of recommendation C (Level of evidence - 5

4. Medical Rehabilitation and Sanatorium-Resort Treatment

  • All patients who have undergone surgical treatment are recommended to undergo rehabilitation measures aimed at the complete social and physical recovery of the patient [44].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

Comments: surgical treatment of patients with congenital or acquired anomalies and deformities of the dentofacial system and facial skeleton is fundamental, but only comprehensive, timely, and systematically implemented specialized care for this group of patients ensures an optimal functional and aesthetic treatment outcome and complete rehabilitation. Regular patient follow-up allows monitoring the quality of treatment throughout the rehabilitation period and adjusting the management plan in accordance with emerging changes.

No specific rehabilitation is provided for the described pathologies. To provide comprehensive care and ensure complete rehabilitation of patients with congenital or acquired anomalies and deformities of the dentofacial system and facial skeleton, coordinated teamwork of specialists is required: a maxillofacial surgeon, an orthodontist, and an anesthesiologist-intensivist. In some cases, other specialists may be additionally involved: a neurologist, an ophthalmologist, an otorhinolaryngologist, a geneticist, and a speech-language pathologist. Rehabilitation measures are carried out based on specific clinical symptoms (type and severity of the dentofacial anomaly) and are subject to consideration in the relevant clinical guidelines.

5. Prevention and Dispensary Observation

5.1 Prevention

ï All patients showing symptoms of craniofacial bone anomalies and facial bone-musculoskeletal deformities are recommended a consultation with a maxillofacial surgeon to determine patient management and treatment tactics to prevent disease progression and complication development [42].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

Comments: there is no specific prophylaxis for the described pathologies. Prophylaxis consists of planned rational pregnancy management, and in case of detected diseases, examination by an orthodontist.

5.2 Dispensary monitoring

ï All patients after surgical treatment for malocclusion are recommended regular (at least once every 6 months) examinations by a maxillofacial surgeon and an orthodontist [42,43].

GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)

ï If there is suspicion of relapse, secondary deformity, or deterioration of the patient's local, neurological, ophthalmological, or other statuses, CT of the entire skull, brain MRI, EEG, and other studies are recommended based on the clinical presentation [4,43,48, 49].

LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)

6. Organization of Medical Care

orthognathic surgeries in patients with dentofacial anomalies are performed in an inpatient setting;

• care for patients is provided by maxillofacial surgeons;

• hospitalization of patients is planned.

Indications for elective hospitalization:

• if it is necessary to perform orthognathic surgeries or if outpatient orthodontic correction of a dentofacial anomaly is impossible;

• inability to perform diagnostic or therapeutic procedures in an outpatient setting;

Indications for patient discharge:

• in the absence of indications for further inpatient treatment;

• if the patient needs to be transferred to another medical institution;

• at the request of the patient or their legal representative;

• in cases of patient non-compliance with prescriptions or hospital internal regulations, provided this does not threaten the patient's life or the health of others.

7. Additional Information

Negative factors affecting treatment outcomes include:

1. Development of infectious complications.

2. Non-compliance with local contact cryotherapy.

2. Non-compliance with personal oral hygiene.

3. Non-compliance with the prescribed diet and masticatory activity restrictions.

4. Non-compliance with recommendations for wearing intermaxillary elastics.

5. Non-compliance with physical therapy recommendations in the postoperative period.

6. Failure to attend follow-up examinations.

7. Non-compliance with other recommendations of the attending physician.

Criteria for Evaluating the Quality of Medical Care

Table 1

Quality of care assessment criteria for patients with congenital and acquired anomalies and deformations of the dentofacial system and facial skeleton

№

Quality criteria

Level of evidence

Grade of Recommendation

2

Computed tomography of the facial skeleton and magnetic resonance imaging of the temporomandibular joint were performed

5

C

3

Anthropometric analysis was performed

5

C

4

Consultation with a prosthodontist was performed

5

C

5

Consultation with an orthodontist was performed

5

C

6

Surgical intervention was performed, resulting in the achievement of conditions to ensure normal growth and development of the dentofacial system in the postoperative period.

4

C

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Appendix A1. Working Group Composition

  1. Kulakov, A. A. – Academician of the Russian Academy of Sciences, MD, Professor, President of the Association of Maxillofacial Surgery Specialists LLC.

  2. Drobyshev Alexey Yuryevich — Professor, Doctor of Medical Sciences, Vice-President of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

  3. Chausskaya Irina Yuryevna — Maxillofacial Surgeon, Candidate of Medical Sciences, Member of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

  4. Miterev Andrey Alexandrovich — Maxillofacial Surgeon, Candidate of Medical Sciences, Member of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

  5. Bayrikov Ivan Mikhailovich — Professor, Doctor of Medical Sciences, Corresponding Member of the RAS, Member of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

  6. Dibirov Timur Magomedbegovich — Maxillofacial Surgeon, Candidate of Medical Sciences, Member of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

  7. Klipa Igor Alexandrovich — Maxillofacial Surgeon, Candidate of Medical Sciences, Member of the All-Russian Public Organization "Association of Maxillofacial Surgery Specialists".

There is no conflict of interest.

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience of these clinical guidelines:

1. Maxillofacial surgeons

2. Orthodontists

3. Prosthodontists

4. Oral surgeons

5. Mid-level medical personnel

6. Healthcare organizers

7. Medical insurance expert physicians (including when conducting medical and economic expertise)

8. Medical students, residents, postgraduate students.

In these clinical guidelines, all data are ranked by level of reliability (evidence) depending on the quantity and quality of studies on the issue.

Table 1

Scale for assessing levels of reliability of evidence (LRE) for diagnostic methods (diagnostic interventions)

LoE

Definition

1

Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis

2

Individual studies with reference method control or individual randomized clinical trials and systematic reviews of studies of any design, except for randomized clinical trials with meta-analysis

3

Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies

4

Non-comparative studies, case report

5

Only justification of mechanism of action or expert opinion is available

Table 2

Rating scale for levels of evidence (LE) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)

LoE

Definition

1

Systematic review of randomized controlled trials with meta-analysis

2

Individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis

3

Non-randomized comparative studies, including cohort studies

4

Non-comparative studies, case reports or case series, case-control studies

5

Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available

Table 3

Rating scale for grades of recommendation (GR) for prevention, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)

Level of Evidence / Strength of Recommendation

Definition

A

Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high satisfactory methodological quality, and their conclusions regarding the outcomes of interest are consistent)

C

Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high satisfactory methodological quality and/or their conclusions regarding the outcomes of interest are inconsistent)

C

Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent)

The clinical guideline update mechanism provides for systematic revision at least once every three years, as well as upon the emergence of new evidence-based data regarding the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or when justified additions/comments to previously approved clinical guidelines arise, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

Order of the Russian Ministry of Health dated June 14, 2019, No. 422n

"On Approval of the Procedure for Providing Medical Care in the Field of "Maxillofacial Surgery"

Appendix B. Physician Action Algorithms

Appendix B. Algorithms of physician actions — Congenital and acquired anomalies and deformations of the dentofacial system and facial skull

Appendix C. Patient Information

A malocclusion occurs when the upper and lower jaws do not match in size, shape, and relative position, resulting in improper closing of the dental arches. A patient's smile with a malocclusion may look quite aesthetic, but such a pathology can subsequently cause serious functional disorders of the dentofacial apparatus.

Often, malocclusion manifests after 30 years of age, when clicking, crunching, or pain in the temporomandibular joints appear, breathing is impaired, tooth roots become exposed, increased tooth wear and premature tooth loss occur, and patients complain of aesthetic discomfort and other symptoms.

According to various authors, in approximately 20-30% of patients with malocclusion, the discrepancy between the jaws is so pronounced that its correction exclusively through conservative orthodontic methods is impossible.

At the present stage, treatment of patients with skeletal dentofacial anomalies typically involves a three-stage approach: the first stage consists of orthodontic preparation for surgical treatment, the second stage is the orthognathic surgery itself, and the third is the final stage of orthodontic treatment.

In some cases, with pronounced skeletal dentofacial anomalies, a two-stage treatment is performed, where orthognathic surgery is carried out already at the first stage, and the final orthodontic correction constitutes the second stage.

Reconstructive jaw surgery requires general anesthesia. The surgical intervention must be performed in an inpatient setting. The average length of hospitalization usually ranges from several days to two weeks, depending on the course of the immediate postoperative period.

Usually, on the first day after surgery, intermaxillary elastic elastics are applied to the patient, completely eliminating the possibility of mouth opening, to maintain the jaws in the correct position and ensure optimal conditions for bone fusion.

On the first day after surgery, local application of cold is recommended for the prevention of edema. The patient undergoes a course of antibiotic therapy, anti-inflammatory/anti-edematous therapy, and analgesia.

In the first few days after surgery, drains may be left in the postoperative wounds. Drains are removed on days 1–3 post-surgery, as the wound discharge decreases.

The patient is advised to maintain daily personal oral hygiene.

After discharge from the hospital, the patient is followed up either on an outpatient basis or in a day hospital setting.

Restriction of mouth opening after maxillary surgery is typically 2 weeks, and approximately 3 weeks following mandibular orthognathic surgeries.

After removing the intermaxillary elastic traction, to prevent limitation of mouth opening, the patient is recommended to engage in therapeutic exercises and physical therapy. To gradually increase the mouth-opening amplitude, the patient is advised to open and close the mouth in front of a mirror, and perform forward-backward and lateral movements of the mandible. The patient can increase the mouth-opening amplitude using their index and thumbs placed on the cutting edges of the upper and lower incisors.

As a rule, after 3–4 weeks post-surgery, the patient is referred to an orthodontist for the final stage of appliance-based orthodontic correction.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

not applicable

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