List of Abbreviations
ISSVA – International Society for the Study of Vascular Anomalies
IH – infantile hemangioma (synonyms – childhood hemangioma); in English literature – "infantile hemangioma"
VHP – vascular hyperplasia
HHT — hereditary hemorrhagic telangiectasia
MFR – maxillofacial region
MRI – magnetic resonance imaging
US – ultrasound examination
VM – vascular malformation
VM – venous malformation
CM – capillary malformation
AVM – arteriovenous malformation
CVM – capillary-venous malformation
CLM – capillary-lymphatic malformation
CLVM – capillary-lymphatic-venous malformation
CAVM – cerebral arteriovenous malformations
LVM – lymphovenous malformation
CVAVM – capillary-venous-arteriovenous malformation
CLVAVM – capillary-lympho-venous-arteriovenous malformation
AVF – arteriovenous fistula
CAVA – capillary-arteriovenous malformation
CLAVM – capillary-lymphatico-arteriovenous malformation
SM – mixed malformation
RFA – radiofrequency thermal ablation
EEG – electroencephalogram
MSCT-AG – multislice computed tomography with angiography
Terms and Definitions
Blood vessel malformations (synonym: angiodysplasia) are vascular developmental anomalies that can affect capillaries, veins, arteries, or a combination thereof. Characteristically, these lesions "grow in parallel with the patient's growth".
Blood vessel hyperplasia (infantile hemangioma) — a reactive tumor-like tissue growth based on endothelial cell proliferation.
Nidus (central focus) – a developmental anomaly characterized by the formation of direct communications between vessels of various diameters or a reticular structure of tiny capillaries in patients with arteriovenous malformation.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Blood vessel malformations are vascular developmental anomalies that can affect capillaries, veins, arteries, or any combination of these vascular channels.
These are the consequences of dysembryogenesis of the circulatory system; patients predominantly present with localization of the pathology in the head and neck region, accompanied by various functional and aesthetic disorders. In the pathogenesis of the disease, the leading factor is the disruption of the anatomical integrity of the vascular wall and changes in their shape due to fluid dynamics. A characteristic feature of BVMs is hemodynamic disturbance. These lesions are characterized by the development of ectasia of vascular structures with an increase in their diameter.
Capillary malformation (CM) is a hemodynamically low-flow vascular disorder of the capillary network of the skin and mucous membranes, affecting the microvasculature and sometimes extending to the deeper layers, especially in the maxillofacial region. Normally, the diameter of the microvascular vessels ranges from 10 to 100 µm. In capillary malformations, their diameter varies from 100 to 2000 µm.
As part of combined forms, such as CM-AVM, CM-VM, the pathological process involves not only the skin vessels, but also the subcutaneous adipose, muscle, and bone tissues, as well as mucous membranes.
Arteriovenous malformation (AVM) is a hemodynamically active vascular pathology with a high blood flow velocity, developing as a result of a defect in the arterial and venous systems with the formation of direct communications between blood vessels of various calibers. The pathological "focus" (nidus) represents directly interconnected arteries and veins, bypassing the microvasculature. Arteriovenous malformations are rare and are usually located both superficially and viscerally. Most of them manifest from birth, but some become noticeable during puberty and never regress.
Venous malformation (VM) is a hemodynamically inactive, low-flow vascular malformation involving the collecting part of the vascular network. Venous malformation is the most common vascular anomaly. Venous malformation represents a disturbance in vascular morphogenesis characterized by dilated and deformed venous vessels, with defects in smooth muscle cells surrounded by a continuous layer of flat endothelial cells. Venous malformations in the head and neck region rank second in prevalence after the lower extremities, and arteriovenous malformations rank third after the upper and lower extremities. [1,2,3,13,36]
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
The etiology of BVMs currently remains incompletely understood. Certain progress has been achieved in the genetic study of a number of angiodysplasias, for which the presence of specific gene mutations has been proven. According to ISSVA, not only a number of syndromes including blood vessel malformation, but also sporadic forms of malformations have causative genes.
The critical period for the emergence of vascular malformations is the 4th to 20th week of the intrauterine period.
The normal circulatory system in the vascular bed is disrupted. Instead of the normal blood circulation sequence – artery, arterioles, precapillaries, and capillaries – a pathological blood shunt from the arterial system into the venous system occurs; depending on this, areas of altered blood circulation are formed: at the capillary level with slow blood flow, and at the arterial and venous level with high blood flow velocity.
Arteriovenous shunts determine the dynamics of blood alteration in accordance with the laws of fluid motion.
In most cases, BVMs are identified in the first weeks and months of life. Subsequently, the growth of the malformation occurs proportionally to the patient's growth. Intensive growth of the pathological vascular formation is usually typical of female patients during puberty and pregnancy. Rapid growth is also noted in the presence of provoking factors such as trauma, infection, and stressful situations. Most venous malformations appear sporadically. [3,4,6,31,36].
1.3 Epidemiology of the Disease or Condition
Blood vessel malformations in the head and neck region account for 5% to 14% of clinical observations of all vascular lesions. About 80% of all BVMs are lesions of small veins, venules, and capillaries. About 80% of all blood vessel malformations localized in the head and neck region are capillary malformations. [1-4]
1.4 Features of Coding the Disease or Condition
Blood vessel malformations (ICD-10 codes):
D10 Benign neoplasm of mouth and pharynx
D18 Hemangioma and lymphangioma of any site [AAG1]
Q25.8 Other congenital malformations of great arteries
Q25.9 Congenital malformation of great artery, unspecified
Q26.8 Other congenital malformations of great veins
Q26.9 Congenital malformation of great vein, unspecified
Q27 Other congenital malformations of peripheral vascular system
Q28.8 Other specified congenital malformations of circulatory system
Q28.9 Congenital malformation of circulatory system, unspecified [2-4]
1.5 Classification of the disease or condition
ISSVA 2018 Classification of Vascular Malformations:
1. Simple:
Capillary malformations (CM):
Simple nevus / salmon patch, "angel's kiss", "stork bite"
Cutaneous and/or mucosal CM (so-called port-wine stain)
Nonsyndromic CM
CM with CNS and/or ophthalmological anomaly (Sturge-Weber syndrome)
CM with bone and/or soft tissue hypertrophy
Diffuse CM with hypertrophy
Reticular CMs
CM due to [associated with] microcephaly
CM due to [associated with] megalencephaly and polymicrogyria
CM due to [associated with] CM-AVM (arteriovenous malformation)
Congenital cutis marmorata telangiectatica
Other
Telangiectasia
Hereditary hemorrhagic telangiectasia (HHT)
Other
Venous malformations
Simple VMs
Hereditary mucocutaneous VMs
Blue rubber bleb nevus syndrome (Bean syndrome)
Glomvenous malformation
Cerebral VM
Hereditary intraosseous vascular malformation
Verrucous VM (formerly called "verrucous hemangioma")
Other
Arteriovenous malformations (AVMs)
Sporadic
As part of HHT
As part of CM-AVM
Other
Arteriovenous fistula (AVF)
Sporadic
As part of HHT
As part of CM-AVM
Other
2. Combined
CVM, CLM, CAVM, LVM, CLVM, CLAVM, CVAVM, CLVAVM, Other
3. Anomalies of major specific vessels (so-called "canal-type" or "truncal" vascular malformations)
Affected:
Lymphatic vessels
Veins
Arteries
Anomalies:
Genesis [origin]
Bed [flow]
Number [quantity]
Length
Diameter (aplasia, hypoplasia, stenosis, ectasia/aneurysm)
Valves
Communication (AVF)
Persistence (embryonic vessels)
4. Vascular malformations associated with other anomalies
Klippel-Trenaunay syndrome
Parkes Weber syndrome
Servelle-Martorell syndrome
Sturge-Weber syndrome
Limb VM + congenital non-progressive limb hypertrophy
Maffucci syndrome
Macrocephaly-CM syndrome
Microcephaly-CM syndrome
CLOVES syndrome
Proteus syndrome
Bannayan-Riley-Ruvalcaba syndrome
CLAPO syndrome
Classification of blood vessel malformations (syndromic and non-syndromic)
(Roginsky V.V. et al., 2021).
By clinical course
- Stable
- Progressive
By affected vascular segment
- Capillary-venous
- Arterial
- Venous
- Mixed
- Arteriovenous fistulas (shunts)
By blood flow velocity
- Low-flow
- High-flow
1.6 Clinical presentation of the disease or condition
Vascular malformations are characterized by a wide variety of clinical manifestations, mainly presenting as a change in the color of the skin and mucous membranes ranging from pinkish-red to dark cherry, and less commonly as an increase in tissue volume. In some cases, they are accompanied by severe alterations in the local and general condition.
Symptoms of the disease manifest in the maxillofacial region and neck depending on the size and localization of the lesion. Lesions of the bones of the facial and cerebral skeleton, most commonly the jaws, are frequent. They can be isolated or combined with soft tissue involvement. The growth of the lesion is noted to be synchronous with the patient's growth. [1,2,31]
2. Diagnostics, medical indications and contraindications for diagnostic methods
The diagnosis of "vascular malformation" is clinical and is established on the basis of clinical and instrumental research methods: interview (patient life history, including the antenatal period [mother's obstetric history in pediatric patients] and disease history starting from the intranatal period, ascertaining the presence of vascular malformations in close relatives to identify hereditary forms and syndromes that include vascular malformations).
The history of the disease, visual inspection, physical examination data, and additional radiological and functional research methods make it possible to verify the nosology in patients.
One of the main clinical manifestations of blood vessel lesions is visible tissue deformation with altered facial symmetry, as well as changes in the color and structure of the skin and mucous membranes. [1-4,31]
2.1 Complaints and Anamnesis
It is recommended to consult a maxillofacial surgeon upon the appearance of the first signs of vascular pathology of the maxillofacial region and neck in patients of any age. [1,4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
It is recommended to ascertain from the patient or parents of a pediatric patient any changes in the color dynamics of the skin and mucous membranes, soft tissue configuration disorders, and bleeding episodes. [1-4,13,36]
GPR CLOE 5Grade of Recommendation C (Level of Evidence 5)
2.2 Physical Examination
The primary diagnostic link is a clinical examination of the patient to detect skin color changes, facial asymmetry, impairment of aesthetic parameters in the age aspect, and assessment of impaired nasal breathing, mastication, and occlusion.
It is recommended as part of the physical examination of patients with vascular malformations to assess the size, volume, and shape of the formation; examine the deformation of facial and neck contours, and facial aesthetic parameters. [1,4,31]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: at birth, capillary malformations and venous malformations are usually observed as a single phlebectasia, while arteriovenous malformations of blood vessels can mimic a capillary malformation in the first years of life with subsequent manifestation of the classical clinical picture.
It is recommended to pay attention to the color of the skin and mucous membranes in patients with vascular malformations. [1,4,31]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: malformations with a predominance of the venous component have a purple-bluish tint, whereas malformations with a predominance of the arterial component acquire a bright red skin color. In most cases, when the skin is affected or when the vascular malformation is superficially located, the skin has an uneven, bumpy surface. The presence of a bright red skin color of varying intensity may indicate a capillary form of malformation or a mixed CM-AVM form in the early stages. [1,4,7,31]
It is recommended to perform a palpation examination during patient inspection to detect soft tissue pulsation in the lesion area. [1-4,13,36]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: in patients with arteriovenous malformation, an increase in the volume of the affected area is noted, areas of hyperemia and elevated temperature are observed on the skin, and pulsation is noted upon palpation of the affected area.
In patients with vascular malformations with a pronounced arterial component, auscultation reveals a vascular murmur that coincides with the heart rate. For venous formations, a characteristic sign is an increase in soft tissue volume. The presence of phlebectasia in patients indicates the presence of venous malformations of blood vessels. Palpation of the lesion zone makes it possible to assess the condition of soft tissues and underlying bone structures, and the connection of the malformation with surrounding tissues. [1-4,36]
It is recommended in cases of extensive lesions and localization of vascular malformations in complex anatomical areas to use a multidisciplinary approach involving a team of specialists (pediatric surgeon, neurosurgeon, otorhinolaryngologist, endovascular diagnostics and treatment specialist, ophthalmologist, cardiovascular surgeon, ultrasound diagnostics physician, geneticist). [4,44]
GCP CLEL 5Level of Evidence C (Level of Likelihood of Evidence - 5).
Comments: following diagnostic studies, a treatment plan is drawn up by the team of specialists.
It is recommended to refer patients with vascular malformations combined with facial skeleton deformation and/or dysocclusion for a consultation with an orthodontist to resolve the issue of the need for orthodontic treatment. [1-4,13]
GCR CLEL 5 Grade of Recommendation C (Level of Evidence - 5).
2.3 Laboratory Diagnostics
It is recommended for adult patients with extensive vascular malformations during the preparation and planning of surgical intervention to perform laboratory tests to assess the hemostasis system (complete blood count, coagulation hemostasis test [prothrombin time, thrombin time, activated partial thromboplastin time, international normalized ratio]). [4]
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
2.4 Instrumental Diagnostics
It is recommended for all patients with vascular malformations to undergo duplex scanning of the vessels of the maxillofacial region with an assessment of the structure of the vascular formation, localization, and determination of the type and velocity of blood flow. [1,4,8,22]
GCP CLEL 5Level of Evidence C (Level of Likelihood of Evidence - 5).
Comments: According to the peak systolic blood flow velocity parameter in the nidus, a distinction is made between: "high-flow" vascular malformations (higher than or equal to 20 cm/s) and "low-flow" vascular malformations (less than 20 cm/s).
It is recommended to perform capillaroscopy in children with superficial blood vessel lesions. [1,4,13]
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
Comments: capillaroscopy allows high-resolution visualization of the capillary bed microvessels and helps differentiate VM from AVM (LM).
It is recommended to perform computed tomography angiography in adult patients with VM to visualize the angioarchitecture of the head and neck. [11, 18]
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
Comments: this study makes it possible to determine the degree of tissue vascularization based on quantitative X-ray density characteristics derived from contrast enhancement (in Hounsfield units). An X-ray density in the nidus >70 HU indicates "highly vascularized" VMs, whereas an X-ray density < 70 HU indicates "low-vascularized" VMs.
It is recommended to perform videolaryngoscopy, videorhinoscopy, and esophagogastroduodenoscopy in all patients with functional breathing and swallowing disorders to detect lesions in the laryngopharynx, pterygomandibular space, or neck. [20,37]
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
It is recommended to perform contrast-enhanced magnetic resonance imaging of soft tissues in all patients with extensive VM, AVM, or LM of blood vessels localized in complex anatomical and topographical regions to clarify the extent, localization, and detailed topography of the lesion. [1,4,7,13,18]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: general anesthesia is required when performing MRI in children under 5–6 years of age.
It is recommended to perform computed tomography angiography in patients with suspected intraosseous lesions (VM, AVM, LM) localized in complex anatomical and topographical regions. [4,36]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: general anesthesia is required when performing CT in children under 5–6 years of age.
It is recommended to perform preoperative selective angiography in patients with high-flow VMs. [1,2,13,35]
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: angiography allows the identification of the "feeding vessels" of the AVM.
It is recommended to perform brain magnetic resonance imaging when VMs involving half of the face and epileptic seizures are detected, to rule out or confirm Sturge-Weber-Krabbe syndrome. [4]
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
2.5 Other Procedures
It is recommended to perform ultrasound-guided diagnostic puncture of an oral cavity cyst when isolated bone VM is suspected. [4, 13]
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5).
Comments: isolated bone lesions of the jaws can mimic cysts (simple idiopathic, etc.). Obtaining blood under pressure suggests a VM and indicates the need for further evaluation.
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
Conservative treatment.
It is recommended to withhold treatment upon the initial presentation of parents with an infant who has a blood vessel lesion. [40]
GCP CLEL 5Level of Evidence C (Level of Likelihood of Evidence - 5).
Comments: a period of 2 weeks to 1 month is required during which the rate of lesion enlargement is recorded and differential diagnosis between IH and VM is performed. VMs demonstrate a normal endothelial growth rate and enlarge due to hypertrophy rather than proliferation, unlike IHs. Unlike IHs (infantile hemangiomas), vascular malformations do not undergo involution. Conservative treatment is indicated only for IHs.
Surgical treatment
Treatment for patients with head and neck VMs should be tailored taking into account the form of VM. Currently, the most rational treatment methods for patients with VMs include: surgical treatment, pulsed phototherapy, radiofrequency and laser ablation, endovascular embolization of arteriovenous malformation vessels, and combination therapy. The choice of treatment strategy depends on the size, form, and localization of the VM.
The treatment algorithm for patients depending on the extent and form of VM is presented in Appendix B.
It is recommended to perform excision of the arteriovenous malformation with preliminary endovascular occlusion of the AVM vessels in adult patients with AVM. [8,20]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comment: preoperative embolization reduces the rate of surgical complications, decreases intraoperative blood loss, and facilitates the identification of anatomical structures. Endovascular embolization without subsequent surgical treatment leads to active collateral formation and disease progression. Endovascular vessel embolization is performed 1–2 days prior to surgery. In case of delayed surgery, the effect of endovascular vessel embolization is significantly diminished. In venous malformations, endovascular embolization is neither advisable nor feasible.
It is recommended to perform vascular malformation excision with wound closure using local tissue in patients with venous malformation involving one or two anatomical regions. [1,2,4,13,20,41]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: if the venous malformation lesion is located in one or two anatomical regions, a single-stage surgical intervention is possible. Involvement of more than two anatomical regions involves staged excision or combined treatment (sclerotherapy + excision, ligation of afferent vessels + sclerotherapy, excision + muscle flap reconstruction).
It is recommended to repair the post-resection defect with a free revascularized flap in adult patients with venous malformation involving two or more anatomical regions. [1,2,4,20,31,33]
GCR CLOC 5Grade of Recommendation C (Level of Evidence 5)
It is not recommended to perform external carotid artery ligation as an independent treatment method for all types of VMs. [1,2,20,33]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: this method is associated with a high surgical risk, causes a compensatory increase in blood flow velocity on the contralateral side, and activates collateral formation.
It is not recommended for patients with CMS to undergo endovascular embolization in the presence of "feeding" vessels from the internal carotid artery basin due to the risk of embolus migration and the development of stroke. [4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
It is recommended in patients with CMS in the postoperative period, in the event of bleeding, to use the following techniques: packing, suturing, application of a pressure bandage, performing sclerotherapy, closed or open laser ablation. [20,35]
Level of recommendation evidence C (level of data validity - 5).
It is recommended for patients with localization of the arteriovenous malformation in a functionally and/or esthetically significant zone to perform partial excision, and to treat the remaining pathological tissues with one of the thermal ablation methods. [2,4,20,31]
Level of Evidence CGrade of Recommendation 5Level of recommendation C (level of evidence - 5).
It is recommended for patients with venous malformation to undergo sclerotherapy, either as a standalone method or as part of combination treatment (sclerotherapy + excision of the vascular malformation). [2,4,12,16,20,21,25]
RG CLE 5Recommendation Grade C (Level of Evidence 5).
Comment: sclerotherapy causes destruction of the vascular endothelium followed by inflammation, thrombotic occlusion, sclerosis, and reduction of the lesion size. An ideal sclerosant does not exist. Currently, the foam composition is the most effective. The effectiveness of the foam sclerosant is due to an increase in contact time and contact surface area. Sclerosing foam is prepared using: 15 mg of #Bleomycin** and 4 ml of 3% solution of #lauromacrogol 400 (polidocanol) (ready-made commercial solution) with the introduction of air according to the Tessari method. The procedure is performed under ultrasound guidance. Sclerosants do not have direct indications for the treatment of patients with maxillofacial pathology, but are used as off-label therapy.
It is recommended for adult patients with venous malformation to use a combination destruction method via suturing, sclerotherapy, and compression. [20,21]
RG CLE 5Recommendation Grade C (Level of Evidence 5).
Comment: the combination method allows achieving a reduction in the severity of the venous filling symptom, with a decrease in the volume of intraoperative blood loss and the prevention of secondary scar skin deformity. The volume of the injected sclerosant depends on the size of the lesion. Vein-sclerosing agents for local injections are used as sclerosants.
Laser treatment
It is recommended for patients with extensive capillary-type malformations to undergo laser therapy. [1,2,4,12,34,37,38,39]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: the active factor of laser radiation is a powerful luminous flux. High-intensity luminous flux causes a thermal effect upon interaction with tissues. Various types of lasers are used to treat patients with vascular lesions: currently, the dermatological laser device (pulsed dye laser) is recognized as the most effective. The main effects of laser treatment are vascular coagulation and rupture. Laser treatment is performed in several stages.
It is recommended to perform staged sessions of skin phototherapy for all patients with capillary malformations. [34,43]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: in pediatric patients, the procedure is most often performed under mask anesthesia. Pulsed phototherapy operates on the principle of "selective photothermolysis"—selective action on cells containing chromophores.
Laser and radiofrequency thermal ablations
It is recommended to perform radiofrequency thermal ablation in patients with extensive venous malformations in a complex anatomical area affecting deep layers. [1,4,17]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: this method can be used either as monotherapy or as part of combination treatment. The procedure must be performed under ultrasound guidance for clear visualization of the treatment site. Radiofrequency thermal ablation can be performed percutaneously and/or transmucosally, as well as in an open procedure.
It is not recommended to perform RFTA in patients with superficial VMS location due to the risk of thermal skin burns.[4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
It is recommended to perform laser ablation in patients with extensive VMS affecting superficial layers. [1,4,33]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
Comments: this procedure can be used as a monotherapy method and as part of combination treatment. The procedure is performed using lasers: neodymium-doped yttrium aluminum garnet (Nd:YAG) or holmium laser.
It is recommended for patients with vascular malformations to wear a pressure bandage for at least 10 days after radiofrequency thermal ablation or laser ablation. [4,18,20]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
4. Medical Rehabilitation and Sanatorium-Resort Treatment
General principles of rehabilitation for patients with VMS:
Comprehensive assessment of the patient's vascular lesion and formulation of a rehabilitation program;
Drawing up a plan of diagnostic and therapeutic measures necessary for rehabilitation;
Multidisciplinary principle of organizing rehabilitation care;
Monitoring the effectiveness of the ongoing therapy during the course of restorative treatment and at the end of the rehabilitation program.
It is recommended to carry out medical rehabilitation measures for the complete social and psycho-physical recovery of patients with vascular malformations of the head and neck. [4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
5. Prevention and Dispensary Observation
There is no specific prevention for the described pathology. If pathology is suspected during prenatal ultrasound screening, prevention consists of planned routine pregnancy management, a consultation with a maxillofacial surgeon after birth, and, in syndromic variants, a consultation with a medical geneticist.
It is recommended that all patients with VMS undergo follow-up examinations by a maxillofacial surgeon at 6 and 12 months after completion of treatment. [4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
It is recommended that patients with vascular malformations of the maxillofacial region undergo duplex scanning of the maxillofacial vessels 6 and 12 months after completion of treatment. [4]
GR CLEL 5 Grade of Recommendation C (level of evidence 5)
It is recommended that patients with vascular malformations undergo magnetic resonance imaging of the soft tissues of the head and/or neck 12 months after treatment. [4]
GPR CLOE 5Grade of Recommendation C (Level of Evidence 5)
It is recommended that patients with vascular malformations presenting with an intraosseous lesion undergo computed tomography of the facial skeleton 6 and 12 months after treatment. [4]
GRC CLEL 5Grade of Recommendation C (Level of Evidence 5)
6. Organization of Medical Care
Surgical interventions for patients with vascular malformations are performed in an inpatient setting.
Care for patients is provided by maxillofacial surgeons;
Hospitalization of patients is carried out on a scheduled basis.
Indications for hospitalization in a medical organization:
The need to perform instrumental diagnostic methods under anesthesia.
Need for surgical intervention to remove foci of vascular malformations and correct deformities resulting from previous stages of treatment.
Indications for discharge of the patient from the hospital:
Completion of scheduled diagnostic and therapeutic examinations.
Complete or partial removal of the lesion with improvement in the anatomical and functional state of the affected area.
Absence of signs of postoperative complications and deterioration of the patient's somatic status.
If necessary, transfer of the patient to another medical institution.
At the request of the patient or their legal representative. [2-4,13]
7. Additional Information
Negatively affects treatment outcomes:
non-compliance with the attending physician's recommendations;
development of infectious complications;
inconsistent tactics among specialist physicians at various stages of treatment;
patient non-compliance with activity restrictions and physical load limits following interventions.
Criteria for Evaluating the Quality of Medical Care
n/a | Quality criteria | Performance evaluation |
|---|---|---|
1 | A consultation with a maxillofacial surgeon was performed | Yes/No |
2 | The size, volume, and shape of the lesion were evaluated; facial and neck contour deformity and facial aesthetic parameters were examined. | Yes/No |
3 | Palpation was performed to detect soft tissue pulsation in the affected area | Yes/No |
4 | Duplex ultrasound scanning of the maxillofacial vessels was performed to assess the structure of the vascular lesion, localization, and to determine the type and velocity of blood flow | Yes/No |
5 | Computed tomography angiography was performed in adult patients with VCM | Yes/No |
6 | Videolaryngoscopy, videorinoscopy, and esophagogastroduodenoscopy were performed in patients with functional breathing and swallowing disorders to visually assess the laryngopharynx, pterygomaxillary space, and neck | Yes/No |
7 | Contrast-enhanced magnetic resonance imaging of soft tissues was performed in the presence of extensive VMs, AVMs, or blood vessel MVs localized in complex anatomical and topographical regions | Yes/No |
8 | Surgical removal of the arteriovenous malformation with preliminary endovascular occlusion of AVM vessels was performed in adult patients with AVM | Yes/No |
9 | Removal of the vascular malformation with local tissue flap wound closure was performed for lesions in one or two anatomical regions | Yes/No |
10 | Follow-up examinations by a maxillofacial surgeon were performed 6 and 12 months after completion of treatment | Yes/No |
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Appendix A1. Working Group Composition
Kulakov Anatoly Alekseevich - Chief Freelance Specialist in Maxillofacial Surgery of the Ministry of Health of the Russian Federation, Scientific Advisor of the National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia, Honored Scientist of the Russian Federation, Academician of the RAS, President of the All-Russian Public Organization "Society of Specialists in Maxillofacial Surgery".
Roginsky Vitaly Vladislavovich - Doctor of Medical Sciences, Professor, Head of the Department of Pediatric Maxillofacial Surgery and Dentistry, National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia, Honored Scientist of the Russian Federation.
Chkadua Tamara Zurabovna - Doctor of Medical Sciences, Deputy Director for Clinical and Research Work, Maxillofacial Surgeon, National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia.
Bolshakov Mikhail Nikolaevich - Candidate of Medical Sciences, Maxillofacial Surgeon, National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia.
Ageeva Lyudmila Vitalievna - Candidate of Medical Sciences, Senior Researcher at the Department of Pediatric Maxillofacial Surgery and Dentistry, National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia.
Gavelya Ekaterina Yuryevna - Candidate of Medical Sciences, Researcher at the Department of Pediatric Maxillofacial Surgery and Dentistry, National Medical Research Center "Central Research Institute of Dentistry and Maxillofacial Surgery" of the Ministry of Health of Russia.
No conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
Table 1.Scale for assessing 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 2.Level of Evidence (LoE) rating scale for preventive, therapeutic, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, therapeutic, and rehabilitation interventions)
LoE | Definitions |
1 | Systematic review of randomized controlled trials with meta-analysis |
2 | Individual randomized clinical trials and systematic reviews of studies of any design, excluding randomized clinical 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. Scale for assessing levels of reliability of recommendations (LRR) for methods of prevention, diagnosis, treatment, and medical rehabilitation, including those based on natural healing factors (preventive, diagnostic, therapeutic, and rehabilitative 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 proper quality evidence (all considered effectiveness criteria (outcomes) are unimportant, all studies have low methodological quality and their conclusions on the outcomes of interest are inconsistent)) |
Target audience for these clinical guidelines:
Maxillofacial surgeon
Pediatrician
General practitioner / Physician
Otorhinolaryngologist
Oral surgeon
Pediatric dentist
Pediatric surgeon
Procedure for updating clinical guidelines.
The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on 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
These clinical guidelines were developed taking into account the following regulatory legal documents:
1. Order of the Ministry of Health of Russia dated February 28, 2019, No. 103n "On Approval of the Procedure and Deadlines for the Development of Clinical Practice Guidelines, Their Revision, Standard Form of Clinical Practice Guidelines, and Requirements for Their Structure, Composition, and Scientific Validity of Information Included in Clinical Practice Guidelines"
2. Order of the Ministry of Health of the Russian Federation dated June 14, 2019, No. 422n "On Approval of the Procedure for Providing Medical Care in the Field of 'Maxillofacial Surgery'"
3. Decree of the Government of the Russian Federation dated October 12, 2019, No. 2406-r "On Approval of the List of Vital and Essential Drugs, as Well as Lists of Medicinal Products for Medical Use and the Minimum Assortment of Medicinal Products Required for Medical Care"
4. Article 76 of Federal Law of the Russian Federation No. 323-FZ dated November 21, 2011, "On the Fundamentals of Health Protection of Citizens in the Russian Federation", regarding the development and approval by medical professional non-profit organizations of clinical practice guidelines (treatment protocols) on the provision of medical care.
Appendix B. Physician Action Algorithms
Patient treatment algorithm for vascular malformations
Appendix C. Patient Information
Vascular malformation (VM) is a developmental defect of the circulatory system formed in utero. A vascular malformation is a congenital structural abnormality characterized by a normal growth rate and division count of endothelial cells. Vascular malformations grow in proportion to the patient's growth and never undergo spontaneous regression. Elements manifest at birth or in the first months of life. A characteristic sign of VM is hemodynamic disturbance. Clinical manifestations depend on the form of the pathology and the affected area.
The diagnosis of "vascular malformation" is clinical and established based on clinical and instrumental research methods, life history, and history of the disease. If parents discover a vascular lesion in the maxillofacial region of a child, they must consult a maxillofacial surgeon.
Timely diagnosis of VM accelerates the possibility of patient recovery, reduces the number and severity of facial skeletal deformities and associated pathologies. It is the patient's parents who are responsible for the timely detection of pathology symptoms and timely consultation with a specialist. All medical recommendations must be strictly followed. One should not fear staged rehabilitation for extensive lesions, since it is often the division of treatment into stages that achieves the best results in the shortest possible time. In the postoperative period, regular follow-up (at least once every 6 months) with specialists involved in the treatment process 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.