List of Abbreviations
EEG - electroencephalography
OH — orbital hypertelorism
IOD — interorbital distance
CT — computed tomography
MRI — magnetic resonance imaging
Ultrasound — ultrasound examination
УДД – levels of evidence certainty
RCT – randomized controlled trial
УУР – levels of recommendation consensus
Terms and Definitions
Plagiocephaly (from Greek plagio - oblique + kephale - head) is a descriptive term meaning cranial asymmetry or an oblique, distorted skull shape regardless of etiology. This skull shape occurs in unilateral coronal synostosis, fetal head deformation due to intrauterine compression without suture synostosis, infant head deformation due to a forced head position during sleep or neuromuscular dysfunctions, and compensatory frontal plagiocephaly resulting from lambdoid suture synostosis.
Trigonocephaly (from Greek trigonos – triangular) is a descriptive term denoting the characteristic triangular shape of the anterior skull vault resulting from premature fusion of the metopic suture.
Scaphocephaly (from Greek scapho – boat) is a term used to describe a skull shape with characteristic narrowing in the parietal and temporal regions, occurring upon premature fusion of the sagittal suture. In specialized literature, the term "dolichocephaly" is also used to define this type of craniosynostosis. Dolichocephaly (from Greek dolicho – long) is a head shape variant characterized by a significant predominance of its longitudinal dimensions over transverse ones.
Brachycephaly (from Greek brachy – short) is a head shape variant characterized by a significant predominance of its transverse dimensions over longitudinal ones. As applied to craniosynostoses, the term is used to describe skull deformation resulting from premature fusion of both coronal sutures.
Acrocephaly (from Greek acro – high) is a term used to describe a high skull shape resulting from premature synostosis of the coronal and lambdoid sutures.
Oxycephaly (from Greek oxys – sharp) is a term used to describe a sharp skull deformity resulting from premature synostosis of the coronal, sagittal, and sometimes lambdoid sutures.
Turricephaly is a term used to describe a skull shape with a characteristic tower-like elevation of the frontal region and its overhang over the facial skeleton, resulting from premature fusion of the coronal and sagittal sutures.
Macrocephaly (from Greek makros – large) is a general term used to denote any of a number of pathological conditions characterized by excessive enlargement of the head size.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Dysplasia is a general term for the consequences of improper formation of individual body parts, organs, or tissues during embryogenesis and the postnatal period; alterations in the size, shape, and structure of cells, tissues, or organs.
Craniosynostosis is the process of premature fusion of the cranial sutures. Craniostenosis is the final result of this process. Craniosynostoses are divided into simple, when a single suture is affected, and complex, when several sutures are affected simultaneously. If all cranial sutures are affected, it is referred to as pansynostosis.
The diagnosis of craniostenosis is clinical and established based on visual examination, anthropometric, and radiological data. The abnormal skull shape (trigonocephaly, scaphocephaly, plagiocephaly, brachycephaly, oxycephaly) is determined by the type of craniosynostosis and depends on which specific sutures underwent premature synostosis. At the same time, synostosis of identical sutures occurring at different times may cause various types of skull deformations. Craniosynostoses are divided into isolated or non-syndromic (deformations of the neurocranium) and syndromic (along with craniostenosis, other morphogenesis defects are present).
Treacher Collins syndrome is a type of complex craniofacial dysostosis characterized by hypoplasia of the lower and middle facial zones and craniosynostosis. Alternative names: Franceschetti syndrome, Franceschetti-Klein syndrome, Franceschetti-Zwahlen-Klein syndrome, mandibulofacial dysostosis.
Hemifacial microsomia (HFM) is a term used to identify facial deformations associated with impaired development of the first and second pairs of branchial arches, characterized by hypoplasia of one half of the face. Alternative names: otocraniostenosis, craniofacial microsomia, lateral facial dysplasia, first and second branchial arch syndrome, oculo-auriculo-vertebral dysplasia, or Goldenhar syndrome.
Orbital hypertelorism (OH) is a term denoting craniofacial dysplasia characterized by an abnormally wide distance between the orbits due to enlargement of the ethmoid labyrinth elements.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
Etiological factors leading to dysostoses affect the fetus in the 2nd to 3rd month of embryonic life. Damaging teratogenic factors, metabolic disorders, hematological disorders, and malformations affect the formation of the neurocranial cord, from which, in addition to the brain, elements of the midface and the I and II branchial arches are formed. The cause of suture closure is attributed to vascular, hormonal, genetic, mechanical, and local factors. However, the exact cause of suture closure remains unclear [1, 2].
Syndromic craniosynostoses comprise a group of dyscrania in which various malformations and ossifications of the facial and neurocranium bones are combined with anomalies of other organs, tissues, and systems. The exact etiology remains unclear; the occurrence of syndromic craniosynostoses is associated with specific gene mutations.
The etiology of Treacher Collins syndrome and hemifacial microsomia is unknown [3, 4].
1.3 Epidemiology of the Disease or Condition
The incidence of craniosynostoses is 1–4 per 10,000 newborns. Non-syndromic craniosynostoses account for about 90% of this number, while syndromic craniosynostoses comprise more than 150 different syndromes.
The most common simple craniosynostosis is sagittal suture synostosis. It accounts for 54–58% of all craniosynostoses. Hemicoronal synostosis (synostotic frontal plagiocephaly) occurs with a frequency of 0.4–1 case per 1,000 newborns. Torticollis and deformational frontal plagiocephaly occur together in 1 out of every 300 newborns. Premature closure of the lambdoid suture and compensatory frontal plagiocephaly occur in 1% to 9.4% of cases.
Metopic suture synostosis accounts for 10% to 17% of all forms of craniosynostoses. Fusion of multiple sutures simultaneously occurs in approximately 7% of cases. The incidence of syndromic craniosynostoses, according to various authors, can reach up to 11% [1, 5, 6].
Orbital hypertelorism occurs in 33.4% of cases with frontonasal dysplasia, in 28.9% of cases with various syndromic pathologies, in 20% of cases with craniofrontonasal dysplasia, in 11% of cases with paramedian craniofacial clefts, and in 6% of cases with encephaloceles [7].
1.4 Features of Coding the Disease or Condition
Q75.0 Craniosynostosis
Q67.2 Dolichocephaly
Q67.3 Plagiocephaly
Q75.3 Macrocephaly
Q75.1 Craniofacial dysostosis
Q75.4 Mandibulofacial dysostosis
Q87.0 Syndromes with congenital anomalies predominantly affecting facial appearance
Q75.5 Oculomandibular dysostosis
Q75.8 Other specified malformations of skull and face
Q75.9 Congenital malformation of skull and facial bones, unspecified
Q67.0 Facial asymmetry
Q67.1 Compressed face
Q67.4 Other congenital deformities of skull, face and jaw
Q75.2 Hypertelorism
1.5 Classification of the disease or condition
Anatomical and topographical classification of craniostenoses (according to Tessier) [1]
A. Isolated dysmorphism of the skull vault
B. Symmetric orbito-cranial dysmorphism
1. Trigonocephaly
2. Acrocephaly
3. Brachycephaly without teleorbitism
4. Brachycephaly with euryprosopia and teleorbitism
C. Asymmetric orbito-cranial dysmorphism (plagiocephaly)
1. Simple vertical orbital dystopia
2. Plagiocephaly without teleorbitism
3. Plagiocephaly with teleorbitism
D. Saethre-Chotzen group
E. Crouzon group
1. Typical Crouzon
2. Upper Crouzon
3. Lower Crouzon
4. Cloverleaf skull Crouzon
F. Apert group
1. Hyperacrocephaly Apert
2. Hyperbrachycephaly Apert
3. Pfeiffer
4. Cloverleaf skull Apert
5. Carpenter
The diagnosis of orbital hypertelorism is clinical and established based on visual examination, anthropometric, and radiological data. The objective quantitative assessment of orbital hypertelorism, used to determine its degree, is the measurement of the interorbital distance (IOD). The type of orbital hypertelorism is determined by the difference in measurements between the IOD at the level of the upper third of the orbits and the IOD between the midpoints of the anterior lacrimal crests [1, 7].
Table 1. Classification of orbital hypertelorism [1]
|
Degree and type |
Measurement parameters |
||
|
Grade I oxycephaly, type 1 |
IOD between the midpoints of the anterior lacrimal crests: 30–34 mm IOD between the inner orbital margins at the level of the upper third of the orbits: no more than 34 mm |
||
|
Grade I oxycephaly, type 2 |
IOD between the midpoints of the anterior lacrimal crests: 30–34 mm IOD at the level of the upper third of the orbits: 35–39 mm |
||
|
Grade II oxycephaly, type 1 |
IOD between the midpoints of the anterior lacrimal crests: 35–39 mm IOD at the level of the upper third of the orbits: no more than 39 mm |
||
|
Grade II oxycephaly, type 2 |
IOD between the midpoints of the anterior lacrimal crests: 35–39 mm IOD at the level of the upper third of the orbits: 40–44 mm |
||
|
Grade III oxycephaly, type 1 |
IOD between the midpoints of the anterior lacrimal crests: 40 mm or more; IOD at the level of the upper third of the orbits exceeds this value by no more than 4 mm |
||
|
Grade III oxycephaly, type 2 |
IOD between the midpoints of the anterior lacrimal crests: 40 mm and more; IOD at the level of the upper third of the orbits exceeds the first value by 5 mm or more |
||
1.6 Clinical presentation of the disease or condition
The main clinical manifestations of congenital craniofacial bone anomalies, as well as congenital musculoskeletal head and face deformations, are: altered facial and cranial configuration, strabismus, restriction of extraocular eye movement, difficulty with jaw movement, nasal breathing difficulties, malocclusion, developmental delay, and—in cases of increased intracranial pressure—headaches, anxiety, crying, and other complaints depending on the rate of disease progression.
2. Diagnostics, medical indications and contraindications for diagnostic methods
2.1 Complaints and Anamnesis
It is recommended to check for complaints regarding altered facial and cranial configuration, strabismus, restriction of extraocular eye movement, difficulty with jaw movement, developmental delay, and—in cases of increased intracranial pressure—headaches, anxiety, crying, and other complaints depending on the rate of disease progression. These conditions are congenital, and symptoms are usually observed starting from the first month of life.
2.2 Physical Examination
To identify clinical symptoms, the attending physician is recommended to begin the examination by assessing the symmetry of the neurocranium and viscerocranium, evaluating the occlusion, performing anthropometric measurements, and measuring the patient's head circumference and body mass [8].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: One of the main clinical manifestations of craniofacial dysostoses is external deformation and gross asymmetry of the face, the assessment of which forms the primary stage of examination. Anthropometric measurements allow for an objective evaluation of the degree of deformation and determine whether the head dimensions correspond to the child's age. Assessing the patient's body mass is important for the initial evaluation of operative and anesthetic risks associated with potentially necessary invasive surgery involving blood loss.
It is recommended that the patient be examined by a maxillofacial surgeon in order to establish a diagnosis and determine a treatment plan [8].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: The maxillofacial surgeon establishes the diagnosis, determines the treatment plan, specifies the need for additional diagnostic methods and consultations with related specialists, and provides treatment.
It is recommended that the patient be examined by a neurosurgeon to identify neurosurgical pathology and determine the need for additional neurosurgical evaluation [9]
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: Co-management of the patient by a maxillofacial surgeon and a neurosurgeon allows for a comprehensive assessment of all aspects of the pathology and surgical intervention involving both the facial skeleton and the neurocranium. If necessary, the neurosurgeon determines the indications for lumbar puncture, which allows for diagnostic evaluation of the cerebrospinal fluid system and assessment of intracranial pressure, while placement of a lumbar drain enables the management of intracranial pressure levels. Prior to surgery, lumbar puncture may be used in older children to reduce intracranial pressure. In such cases, the anatomy of the foramen magnum must be studied in detail to rule out Chiari malformation, as brain herniation may otherwise occur. In the postoperative period, lumbar punctures may be performed in children with compensated hydrocephalus in cases of intraoperative dural tear to facilitate better healing.
It is recommended that the patient be examined by an ophthalmologist to assess the condition of the visual system and ocular adnexa [10]
GSR CLEL 4Grade of Recommendations C (Level of Evidence: 4)
Comments: Fundus examination is a non-invasive method for detecting signs of intracranial hypertension. The ophthalmologist identifies signs of optic atrophy, papilledema, and visual impairments.
It is recommended that the patient be examined by a neurologist to assess neurological status [1].
GSR CLEL 5Grade of Recommendations C (Level of Evidence: 5)
Comments: The neurologist evaluates the neurological status, the degree of mental and psychological development of the child, and determines the indications for neurophysiological studies, EEG, and video-EEG monitoring.
It is recommended that the patient be examined by an otorhinolaryngologist to assess the condition of the ENT organs [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: The otorhinolaryngologist assesses the condition and prevents the development of complications related to the ENT organs.
It is recommended that the patient be examined by a pediatrician to assess the patient's general somatic status [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: The pediatrician assesses the patient's general condition, height-to-weight ratios, and identifies any concomitant pathology. The overwhelming majority of surgical interventions for the described conditions are elective; therefore, the patient must be thoroughly evaluated and any concomitant pathology must be compensated.
It is recommended that the patient be examined by an anesthesiologist-reanimatologist to assess the patient's preoperative condition and evaluate the risks of anesthesia [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: Prior to any surgical intervention, the degree of anesthetic risk is assessed. Most surgeries for the described pathologies are highly invasive, involve bone sawing and repositioning, affect a large volume of soft tissues, and are accompanied by massive blood loss, which requires blood transfusion. The latter is recommended to be initiated from the start of the surgery rather than waiting for blood parameters to drop.
It is recommended that the patient be examined by an orthodontist to assess the condition of the bite [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: A large number of craniofacial dysostoses 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 evaluates jaw positioning and provides orthodontic correction both before and after surgery.
It is recommended that the patient be examined by a geneticist to identify genetic pathology [11].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: This is performed to identify the etiological cause of the disease, potential associated syndromes, and to plan future pregnancies.
Table 2. Differential diagnosis of plagiocephaly according to etiology [1]
|
Type of plagiocephaly |
Etiology |
||
|
Synostotic (hemicoronal synostosis) |
Unilateral synostosis of the coronal, frontoethmoidal, and frontosphenoid sutures. |
||
|
Compensatory (hemilambdoid synostosis) |
Premature closure of the lambdoid suture and compensatory growth and bulging of the forehead on the side opposite to the lambdoid synostosis. |
||
|
Deformational |
Intrauterine compression or postnatal compression (forced head position of the infant during sleep). |
||
Table 3. Differential diagnosis of synostotic and deformational forms of plagiocephaly [1]
|
Anatomical landmarks |
Synostotic |
Deformational |
||
|
Forehead |
Flattened |
Flattened |
||
|
Supraorbital margin |
Displaced superiorly |
Displaced inferiorly |
||
|
Auricle |
Forward and upward |
Backward and downward |
||
|
Zygomatic bone |
Forward |
Back |
||
|
Palpebral fissure |
Widened |
Narrowed |
||
|
Root of nose |
Displaced ipsilaterally |
Not displaced |
||
|
Pogonion |
Displaced contralaterally |
Displaced ipsilaterally |
||
Table 4. Differential diagnosis of deformational and compensatory forms of plagiocephaly [1]
|
Features |
Deformational |
Compensatory |
||
|
Contralateral posterior bulging |
Occipital |
Parietal |
||
|
Frontal bulging |
Ipsilateral |
Contralateral |
||
|
Ipsilateral occipitomastoid bulging |
Absent |
Present |
||
|
Auricle |
Anteriorly |
Posteriorly (anteriorly) and inferiorly |
||
|
Skull base and face |
Not tilted |
Tilt ipsilateral and downward |
||
|
Ridge along the lambdoid suture |
Absent |
Present |
||
|
Head shape: superior view |
Parallelogram |
Trapezoid |
||
|
Head shape: occipital projection |
Normal |
Parallelogram |
||
|
Condition of the lambdoid suture |
Open |
Closed |
||
Table 5. Differential diagnosis of cranial vault deformity depending on affected sutures [1]
|
Cranial vault shape |
Synostosed sutures |
|||||||
|
Lambdoid |
Coronal |
Sagittal |
Frontoethmoidal |
Frontosphenoid |
Zygomaticosphenoid |
|||
|
Oxycephaly |
+/- |
+ |
+ |
+ |
+ |
+ |
||
|
Acrocephaly |
+/- |
+ |
- |
+ |
+ |
+ |
||
|
Turricephaly |
- |
+ |
+/- |
+ |
+ |
+ |
||
|
Brachycephaly |
- |
+ |
- |
+ |
+ |
+ |
||
+ indicates that the suture is prematurely synostosed
- indicates that the suture is unaffected
+/- indicates that the suture may be synostosed
2.3 Laboratory Diagnostics
The laboratory diagnostic panel includes tests prescribed by a geneticist, as well as a preoperative panel of tests for elective surgery. No other specific laboratory diagnostics are provided.
2.4 Instrumental Diagnostics
Computed tomography of the skull with multiplanar and three-dimensional reconstruction by a radiologist is recommended to visualize head structures [12, 13, 14].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: diagnosis of any of the described diseases is impossible without this imaging method, which is fundamental for these patients. CT allows a detailed study of the anatomy of the cranial bones and sutures, and the mechanism of deformation. It is recommended to perform multidetector computed tomography of the entire skull (both facial and neurocranium) with a resolution of at least 1 mm.
Magnetic resonance imaging of the brain in venography mode and computed tomography angiography by a radiologist are recommended to visualize the vascular structures of the brain [1, 15].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: these studies allow a detailed assessment of the brain, the cerebrospinal fluid system, and the anatomy of the cranial vascular system in order to plan surgical intervention and reduce the risk of intraoperative complications, including hemorrhage.
Endoscopic endonasal revision of the nasal cavity and nasopharynx is recommended [16].
LoR CGoE 3Level of Recommendation C (Level of Evidence - 3)
Comments: allows examination of the nasal cavity and nasopharynx to identify potential anatomical abnormalities and features that may impede anesthesia administration.
Electroencephalography, and in the presence of changes on the encephalogram, electroencephalography with activation procedures and/or video-EEG monitoring by a functional diagnostics physician is recommended to assess brain activity [1, 17, 18].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: electroencephalography allows the detection of functional brain changes and epileptiform activity. The results must be interpreted by a neurologist, and video-EEG monitoring should be prescribed if necessary.
It is recommended that an audiologist perform audiometry to assess hearing [1,17,19].
GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)
Comments: allows for an objective assessment of hearing status and hearing impairment.
It is recommended that an ophthalmologist perform dacryocystorhinography to assess the lacrimal drainage system if there is a clinical presentation of lacrimal drainage disorders [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: the study provides the most complete data on the condition of the lacrimal drainage pathways.
2.5 Other Procedures
Section 2.4 describes diagnostic methods that most comprehensively cover all aspects of the described diseases; however, the pathology is so diverse, and the combination of symptoms in each specific case is so specific, that additional methods may be applied depending on the clinical manifestations. No other specific diagnostics not described above are provided.
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
- Conservative treatment is recommended only for deformational frontal plagiocephaly at the age of 6-18 months and consists of custom-molded helmets. In all other cases, conservative treatment is not used [1,20,21,22,23].
GPR CLEL 4Grade of Recommendation C (Level of Evidence - 4
Comments: the child's head circumference in the first 6 months of life increases by 8.3 cm due to brain growth. After 18 months, brain growth slows down and the bones of the cranial vault thicken. Helmet therapy for children with deformational frontal plagiocephaly is not recommended under the age of six months, as the helmet may restrict rapid brain growth. After 18 months, helmet therapy will not yield positive results, as brain growth during this period has already slowed. Between 6 and 18 months, slow brain growth increases the head circumference by approximately 0.6 cm per month. This is the safest time to use a molding helmet. The weak brain growth potential during this period does not exert a major impact on the cranial walls, so helmet therapy can be effective precisely due to the slow brain growth during this period.
3.2 Surgical Treatment
- It is recommended to include a maxillofacial surgeon and a neurosurgeon in the surgical team, and an ophthalmologist and otolaryngologist if simultaneous interventions on the visual or auditory organs are necessary [1].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
- In all cases of the described pathologies, it is recommended to eliminate the deformity and create conditions for the normal growth of the brain, neurocranium, and facial skeleton [1,23,24,25].
GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)
Comments: currently, there is no single universal method for eliminating a given deformity. Individual surgical planning is necessary in each specific case, depending on the patient's age and the type of deformity. When dealing with all cases where there is a threat to normal facial growth, the maxillofacial surgeon has the right to combine known methods of neurocranium and facial skeleton reconstruction.
- Regardless of the degree of external deformity, when initiating treatment, it is recommended to assess the involvement of all parts of the neurocranium and facial skeleton [1,24,25,26].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: in all cases, pathology of both the neurocranium and the facial skeleton is present.
It is recommended to perform surgical interventions at the earliest possible age of the patient [27,28].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: other things being equal and given satisfactory anthropometric parameters of the operated area, the best treatment outcomes can be achieved by performing surgeries at the earliest possible age of the patient.
- In patients under one year of age, removal of fixed metal implants is recommended no later than 2 months after surgery [1,29,30].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: during this period, bone fragments fuse in the correct position, whereas longer retention of the plates will have a growth-restricting effect on the skull
- In patients over one year of age, removal of fixed metal implants is recommended no later than 6 months after surgery [1,29,30].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
Comments: during this period, bone fragments fuse in the correct position, whereas longer retention of the plates will have a growth-restricting effect on the skull
- In both syndromic and nonsyndromic forms of craniosynostosis, linear craniectomies, remodeling, and repositioning of the bones of the cranial vault and base are recommended to eliminate craniostenosis and neurocranial and facial deformities [1,2,31,32,33,34,35,36].
GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)
Comments: elimination of craniosynostosis using only craniectomies is possible only in patients with isolated sagittal suture synostosis under 3 months of age. In all other cases, reconstructive intervention via craniectomies, remodeling, and bone fragment repositioning must be performed.
- In syndromic forms of craniosynostosis, advancement of the midface bones (Le Fort III and/or Le Fort I) and contour plasty of the nasal dorsum using a cranial vault autograft are recommended to correct midface hypoplasia [1,2,37,38,39].
GPR CLEL 4Grade of Recommendation C (Level of Evidence - 4
- In syndromic forms of craniosynostosis, remodeling and/or reconstruction of the mandible and step genioplasty are recommended for contouring the lower facial skeleton [1,2,40].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- In syndromic forms of craniosynostosis, orthognathic surgeries are recommended from 10-14 years of age [1,2,41,42].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- In syndromic forms of craniosynostosis, contour plasty is recommended to eliminate soft-tissue facial deformities [1,2,43].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- Correction of eyelid colobomas and lacrimal drainage disorders is recommended via blepharoplasty and dacryocystorhinostomy starting at 1-2 years of age [1,2,44].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- Correction of deformities caused by underdevelopment of the midface and mandible bones is recommended via supporting-contour plasty of the zygomatic-orbital complex and genioplasty starting at 5-6 years of age [1,2,45,46].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- Reconstructive ear surgeries to treat hearing-related pathology are recommended starting at 6-7 years of age [1,2,47,48].
GPR CLEL 4Grade of Recommendation C (Level of Evidence - 4
- Orthognathic surgical interventions are recommended starting at 10-14 years of age [1,2,49].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- When orbital hypertelorism is combined with premature synostosis of the cranial vault and base sutures leading to plagiocephaly, brachycephaly, or scaphocephaly, correction of orbital hypertelorism is recommended along with the correction of various forms of craniostenosis [1,2,50,51].
GSR CLEL 5Grade of Recommendations C (Level of Evidence: 5)
- The method of craniofacial reconstruction and orbital translocation should be chosen based on the degree of orbital hypertelorism, its type, and the depth of prolapse of the cribriform plate of the ethmoid bone [1,52].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
- Surgical treatment of patients with orbital hypertelorism and associated deformities is recommended in children starting from 3-6 months of life [1,2,53].
GSR CLEL 5Grade of Recommendations C (Level of Evidence: 5)
- To correct grade 1, type 1 orbital hypertelorism, the following surgical techniques are recommended: Converse-Smith orbital osteotomy; osteoectomy of the inner orbital regions; bilateral medial transnasal canthopexy; exenteration of ethmoid labyrinth air cells; excision of encephalocele, dura mater closure; skull base defect repair using cranial vault bone autografts; nasal dorsum contouring using a cranial vault autograft; facial skin plastic surgery with local tissues [1,2,54].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- To correct grade 1, type 2, and grade 2, types 1 and 2 orbital hypertelorism, the following surgical techniques are recommended: superior U-shaped osteotomy, orbital translocation; craniotomy; circular orbitotomy, orbital translocation; cranialization of the frontal sinuses; frontonasal duct plugging; bone grafting of skull defects using cranial vault autografts; temporalis muscle transposition; bilateral medial transnasal canthopexy; exenteration of ethmoid labyrinth air cells; excision of encephalocele, dura mater closure; skull base defect repair using cranial vault bone autografts; nasal dorsum contouring using a cranial vault autograft; facial skin plastic surgery with local tissues [1,2,55].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
- To correct grade 3, types 1 and 2 orbital hypertelorism, the following surgical techniques are recommended: craniotomy; circular orbitotomy, orbital translocation; cranialization of the frontal sinuses; frontonasal duct plugging; bone grafting of skull defects using cranial vault autografts; temporalis muscle transposition; bilateral medial transnasal canthopexy; exenteration of ethmoid labyrinth air cells; excision of encephalocele, dura mater closure; skull base defect repair using cranial vault bone autografts; nasal dorsum contouring using a cranial vault autograft; facial skin plastic surgery with local tissues [1,2,56].
GPR CLEL 5Grade of Recommendation C (Level of Evidence - 5
3.3 Other Treatment
No other specific treatment is provided for patients with the described pathology.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
- All patients who have undergone surgical treatment are recommended to participate in rehabilitation measures aimed at complete social and physical recovery [57].
GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)
Comments: surgical treatment of patients with craniofacial dysostoses is fundamental, but only comprehensive, timely, and systematically implemented specialized care for patients with congenital malformations of the cranial bones ensures optimal anatomical and functional treatment outcomes and full 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 full rehabilitation of patients with craniosynostosis, coordinated teamwork of specialists is required: maxillofacial surgeon, neurosurgeon, neurologist, ophthalmologist, pediatrician, otorhinolaryngologist, geneticist, orthodontist, anesthesiologist, speech therapist. Rehabilitation measures are carried out based on specific clinical symptoms (neurological, ophthalmological, speech therapy, etc.) and are subject to consideration in the relevant clinical guidelines.
5. Prevention and Dispensary Observation
5.1 Prevention
- If symptoms of craniofacial bone anomalies as well as musculoskeletal head deformations appear, consultation with a maxillofacial surgeon is recommended to determine the management and treatment tactics in order to prevent disease progression and the development of complications [58].
GCR CLEL 4Grade of Recommendation C (Level of Evidence - 4)
Comments: there is no specific prevention for the described pathologies. In case of prenatal detection of pathology, prevention consists of planned rational pregnancy management, and in case of identified cases of diseases, examination by a geneticist.
5.2 Dispensary monitoring
- As a minimum postoperative follow-up protocol, regular examinations (at least once every 6 months) by a maxillofacial surgeon, neurologist, ophthalmologist, and pediatrician are recommended [1, 2, 59].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
- If there is suspicion of recurrence, secondary deformation, deterioration of the patient's local, neurological, ophthalmological, and other status, a CT scan of the entire skull, brain MRI, EEG, and other studies are recommended based on the clinical picture [1, 2, 59].
LoR CGoE 5Level of Recommendation C (Grade of Evidence - 5)
6. Organization of Medical Care
Indications for hospital admission to a medical organization:
- The need to perform instrumental diagnostic methods under anesthesia.
- Necessity of surgical intervention to correct a craniofacial anomaly.
Indications for discharging the patient from the hospital:
- Performance of scheduled diagnostic examinations.
- Correction or improvement of the craniofacial anomaly.
- No signs of postoperative complications.
7. Additional Information
- Surgical intervention on the bones of the neurocranium, upper facial zone, and upper half of the middle facial zone performed at an age older than 1–1.5 years negatively affects treatment outcomes and prognosis.
- The use of linear craniectomies alone for the treatment of craniosynostosis (except for sagittal suture synostosis under the age of 3 months) leads to unfavorable outcomes and complications.
- When treating sagittal suture synostosis by craniectomy, excising an excessively wide defect in the area of the sagittal suture (more than 3 cm) leads to unfavorable outcomes, secondary deformations, and complications.
Criteria for Evaluating the Quality of Medical Care
Table 10. Quality of medical care assessment criteria.
|
№ |
Quality criteria |
Level of evidence |
Grade of Recommendation |
|
1 |
Craniofacial region analysis was performed, existing anatomical changes were identified |
3 |
C |
|
2 |
Surgical intervention was performed, during which an improvement in the patient's appearance was achieved. |
3 |
C |
|
3 |
Surgical intervention was performed, resulting in conditions for ensuring normal growth and development of the brain and skull in the postoperative period. |
3 |
C |
References
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Appendix A1. Working Group Composition
- Belchenko Viktor Alekseevich – Professor, Doctor of Medical Sciences, Chief Freelance Specialist in Maxillofacial Surgery of the Moscow Healthcare Department, Chief Physician of the State Budgetary Healthcare Institution "Maxillofacial Hospital for War Veterans of the Moscow Healthcare Department", Board Member of the All-Russian Public Organization "Society of Specialists in Maxillofacial Surgery".
- Glazer Igor Semenovich – Maxillofacial Surgeon, State Budgetary Healthcare Institution "Morozov Children's City Clinical Hospital of the Moscow Healthcare Department", Member of the All-Russian Public Organization "Society of Specialists in Maxillofacial Surgery".
- Golovanev Pavel Sergeevich – Maxillofacial Surgeon, State Budgetary Healthcare Institution "Scientific and Practical Center for Specialized Medical Care for Children Named after V.F. Voyno-Yasenetsky of the Moscow Healthcare Department", Member of the All-Russian Public Organization "Society of Specialists in Maxillofacial Surgery".
There is no conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience of these clinical guidelines:
- Maxillofacial surgeons
- Neurosurgeons
Table 11. Scale for grading levels of evidence (LE) 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 standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled 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 12. Scale for grading levels of evidence (LE) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, rehabilitation interventions)
|
LoE |
Definition |
|
1 |
Systematic review of RCTs with meta-analysis |
|
2 |
Individual RCTs and systematic reviews of studies of any design except RCTs, 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 13. Scale for grading levels of recommendation (LR) for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, rehabilitation interventions)
|
GoR |
Definition |
|
A |
Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
|
B |
Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on 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) |
Procedure for updating clinical guidelines.
The clinical guidelines update mechanism provides for their 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 in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months
Appendix A3. Reference Materials
"Procedure for the provision of medical care in the profile of 'maxillofacial surgery', approved by the Ministry of Health of the Russian Federation dated June 14, 2019, No. 422n (registered by the Ministry of Justice of the Russian Federation on August 30, 2019, registration No. 55783).
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
Surgical treatment for craniofacial dysostoses, craniosynostoses, and hypertelorism is a dangerous procedure, yet one that has no alternative. The invasiveness of surgical interventions is extremely high; in almost all cases, they are accompanied by blood component and substitute transfusions, and all interventions carry high surgical risks. For this very reason, the patient's parents must strictly follow all pre- and postoperative instructions of the attending physician. One should not fear staged treatment or undergoing multiple surgeries, as this is most frequently the only option to fully help the child. It must be understood that the ultimate goal should not be performing as few operations as possible, but rather providing the most complete care possible to the child. In the postoperative period, regular follow-up (at least once every 6 months) with specialists involved in the treatment process (maxillofacial surgeon, neurosurgeon, neurologist, ophthalmologist, pediatrician) is mandatory.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
These clinical practice guidelines do not provide for rating scales, questionnaires, or other patient status assessment tools.