List of Abbreviations
SD – Scheuermann's disease
COMT – computer optical topographer
CT – computed tomography
MRI – magnetic resonance imaging
MSCT – multislice computed tomography
PCF – pedicle screw fixation
Terms and Definitions
Kyphosis – alteration in the shape of the spinal segment in the sagittal plane, resulting in a deformity with the convexity directed dorsally; abnormal kyphosis.
Ventral angle of kyphosis – the angle formed by the intersection of lines tangent to the anterior surface of the vertebral bodies of the upper and lower limbs of the kyphosis.
Hyperkyphosis – exceeding the limits of normal parameters of the thoracic spine in the sagittal plane (>40°).
Gibbus (gibbus) – sharp-peaked angular kyphosis.
Vertebral growth zones – a) vertebral body endplates – ensure the growth of the vertebral body in height and width; b) growth zones of the spinous, transverse, and articular processes determine the growth of the posterior elements of the vertebra; c) the cartilaginous plate located between the vertebral body and the pedicle (neurocentral cartilage) plays a decisive role in the formation of the spinal canal. Normally, all growth zones develop harmoniously.
Transitional kyphosis – kyphotic deformity in the transitional thoracolumbar spine. Normally, the Th12-L2 segment is straight or slightly lordotic (3° measured by Cobb). Therefore, any kyphosis in this zone is considered pathological, which must be taken into account when planning corrective intervention, especially in King type II deformities.
Vertebral ring apophyses – cartilaginous plates located on the cranial and caudal endplates of the vertebral body. They ensure the height growth of the vertebral body.
Schmorl's node (Ch.G. Schmorl, German pathologist) – protrusion of the nucleus pulposus of the disc into the vertebral body through the endplate.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Scheuermann's disease (SD) – a genetically determined condition whose main clinical manifestation is a rigid, smooth thoracic kyphosis and pain syndrome that progressively intensifies over the years.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
H. Scheuermann [1] was the first to suggest that the pathogenesis of the disease is based on avascular necrosis of the ring apophyses of the vertebral body. With the development of necrosis, the growth of the ventral parts of the vertebral bodies is suppressed, and kyphosis develops.
G. Schmorl [2] suggested the possible herniation of intervertebral disc tissue through the growth plates, which leads to kyphosis. According to his view, changes begin as a protrusion of the disc in the nucleus pulposus zone, predominantly due to developmental disorders. Through congenital or traumatic defects of the endplates, part of the disc tissue is extruded into the cancellous bone, leading to a decrease in disc height. Impaired endochondral growth initiates the development of kyphosis.
A familial form of Scheuermann's disease was also described. A. Findlay, A. Conner, and J. Connor [3] suggested an autosomal dominant inheritance pattern. Lundine et al. [4] identified the Duffy and COL1A2 genes in the examined familial series. Familial kyphoses were also noted by F. Halal, R. Gledhill, and F. Fraser [5], and F. Rathke [6]. These authors found many families with a high incidence of Scheuermann's disease among relatives, while at the same time, any spine surgeon has seen a mass of such patients whose families had neither kyphosis nor scoliosis.
E.V. Kalashnikova [7] concluded that SD follows a Mendelian model: autosomal dominant inheritance with complete penetrance of the mutant gene in males and incomplete penetrance (50%) in females, due to which the disease develops in only a portion of female carriers of the pathological gene. The development of specific manifestations of the disease is due to the expression of the pathological major gene.
1.3 Epidemiology of the Disease or Condition
Scheuermann's disease occurs in the general population with a frequency of 0.4–8.3%, depending on whether the diagnosis is based on clinical presentation or radiographic findings [7]. It can be assumed that such a high frequency of the disease is explained by the inclusion in this group of so-called atypical forms, which in our country are more often interpreted as juvenile osteochondroses. In a school screening examination initiated in 1972, E. Ascani et al. [8] described a disease frequency approaching 1%. The male-to-female ratio among patients is 1:1.4. According to D. Bradford et al. [9], this ratio is 2:1. Determining the age of onset is difficult because typical radiographic changes are rarely identified before 10–11 years of age. However, by 12–13 years of age, these changes, including wedging and kyphosis, are usually present.
1.4 Features of Coding the Disease or Condition
M40.0 – Positional kyphosis
M40.1 – Other secondary kyphoses
M40.2 – Other and unspecified kyphoses
M42.0 – Juvenile osteochondrosis of spine
Q76.4 – Other congenital malformations of spine, not associated with scoliosis
Q77.8 – Other osteochondrodysplasia with defects of growth of tubular bones and spine.
1.5 Classification of the disease or condition
There are three radiographic stages of the disease [8]. The first stage is characterized by heterogeneity of the shadows of the vertebral body apophyses, which appear loosened and segmented, with tortuous contours. The contours of the vertebral body endplates adjacent to the apophyses undergo similar changes. The apophyseal line separating the apophyses from the vertebral bodies also becomes uneven.
The main sign of the second stage is wedge-shaped deformity of the vertebral bodies, with the greatest deformity occurring in the vertebral bodies located at the apex of the kyphotic deformity. The radiographic picture of Schmorl's nodes is formed – sclerotic semi-oval rims, which represent the reaction of the bone tissue to the elements of the intervertebral disc that have invaded its thickness.
In the third stage, the apophyses fuse with the vertebral bodies. With large kyphotic deformities, the anterior corners of the vertebral bodies become somewhat pointed and elongated ventrally. In fact, the kyphotic deformity itself is formed by several wedge-shaped deformed vertebrae located at the apex. Starting from the third decade of life, many patients develop signs of a degenerative process (osteochondrosis, spondylarthrosis). E.V. Kalashnikova [7] suggests considering this qualitatively new condition as stage IV of the disease – the stage of long-term consequences.
In the available literature, we were unable to find a unified clinical and radiological classification of kyphotic deformities in SD.
K. Sorensen [10] described two clinical forms of Scheuermann's kyphosis: thoracic (classical, type I), where the apex of the deformity is located at the Th7–Th9 level, and thoracolumbar (type II) with the apex between Th10–Th12. A third form (type III)—lumbar—has been reported, which occurs exclusively in male adolescent athletes (Wenger D. [11]). In addition, painful and painless forms are distinguished.
1.6 Clinical presentation of the disease or condition
The primary clinical manifestation is a pronounced smooth kyphosis of the thoracic spine, as well as a progressive pain syndrome over time. [1] [2] [3] Other clinical manifestations do not occur in all patients and depend on the severity and duration of the established kyphotic deformity: deterioration of neurological status (decreased sensory and motor function below the level of deformity), impaired internal organ function, and manifestations corresponding to osteoarthritis of the hip and knee joints.
2. Diagnostics, medical indications and contraindications for diagnostic methods
Criteria for establishing the diagnosis/condition: the diagnosis of Scheuermann's disease is established based on the characteristic clinical presentation (see section 1.6) and instrumental diagnostic data (see section 2.4).
2.1 Complaints and Anamnesis
It is recommended to perform an evaluation in all patients for diagnostic purposes:
information on the severity of complaints from the patient and their parents (for children);
time of onset and dynamics of changes in complaints;
history of prior treatment;
presence of comorbid pathology;
presence of allergies and drug intolerance [12, 24].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: Scheuermann's kyphosis usually develops in adolescents aged 10–15 years. Detecting the disease in its initial stage of development is impossible. As a rule, parents consult a doctor only when an increase in kyphosis becomes visible. In rare cases, pain between the shoulder blades is observed in the early stages (usually this sign appears when the deformity is already pronounced). The disease progresses slowly. In the late stages, the back becomes rounded or hyperkyphosis develops. Spinal rigidity and pain increase gradually.
2.2 Physical Examination
It is recommended in all patients for diagnostic purposes:
- to conduct a general clinical examination of the patient with an assessment of orthopedic status and skin condition;
- to identify or rule out signs of infectious foci;
- in the presence of comorbid pathology, to consult a relevant specialist physician;
- to photograph the patient from three viewpoints before and after surgery, as well as at subsequent follow-up stages [7].
GCR CLOC 5Grade of Recommendation C (Level of Evidence – 5)
Comments: The main clinical manifestation of Scheuermann's disease is a smooth kyphotic deformity of varying severity. The apex of the kyphosis is most typically located in the lower thoracic spine, although mid-thoracic and upper lumbar deformities also occur. During the Adam's forward bend test, the increase in thoracic kyphosis may become more prominent in this position. In 30% of cases, Scheuermann's kyphosis is combined with scoliosis. Neurological symptoms in Scheuermann's disease usually do not develop.
2.3 Laboratory Diagnostics
Currently, specific laboratory tests for diagnosing Scheuermann's disease have not been developed, and genetic testing to verify this pathology is not available in routine vertebrological practice; furthermore, it also requires a geneticist consultation.
All patients diagnosed with Scheuermann's disease for whom surgical treatment is planned are recommended to undergo the following laboratory tests:
- complete blood count (CBC);
- urinalysis;
- general clinical blood biochemistry analysis;
- determination of main blood groups by the ABO system and determination of the D antigen of the Rhesus system (Rh factor);
- determination of hepatitis B virus surface antigen (HBsAg) in the blood;
- determination of antibodies to hepatitis C virus (Hepatitis C virus) in the blood;
- determination of antibodies to Treponema pallidum in the blood;
- determination of antibodies of classes M and G (IgM, IgG) to human immunodeficiency virus HIV-1/2 and p24 antigen (Human immunodeficiency virus HIV 1/2 + Agp24) in the blood;
- coagulogram (screening assessment of the hemostatic system) [7, 21].
Level of Evidence 5Grade of Recommendation C (Level of Evidence – 5).
2.4 Instrumental Diagnostics
It is recommended in all patients:
- at all stages of patient follow-up, to perform imaging of spinal pathology (using one or more methods at the discretion of the attending physician): radiography of the thoracic and thoracolumbar spine, computed tomography of the spine (one segment) (thoracic spine, thoracolumbar spine), magnetic resonance imaging of the spine (one segment) (thoracic spine, thoracolumbar spine), computer-assisted optical spine topography;
- as part of the standard preoperative evaluation, to perform chest radiography, electrocardiography, echocardiography, comprehensive ultrasound of the abdominal organs, pulmonary function testing (unforced respiratory volumes and flows), and esophagogastroduodenoscopy for adults and children over 12 years of age;
- when indicated, to perform neurophysiological studies, myelography, and duplex scanning of lower extremity vessels (arteries and veins) [7].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5).
2.5 Other Procedures
It is recommended to administer questionnaires to patients who have undergone surgical treatment in order to assess the dynamics of outcomes [8].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comment. The SRS-22 questionnaire can be used for assessment (Appendix G1).
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
It is recommended in all patients with no indications for surgical treatment (see section 3.2 Surgical Treatment) to use bracing for spinal pathology (thoracic, lumbar) both as a standalone treatment and to curb the progression of spinal deformity until skeletal maturation is optimally completed from a surgical perspective [15].
GPP BLOE 3Grade of Recommendation B (Level of Evidence – 3).
It is recommended in all patients to conduct comprehensive non-pharmacological conservative treatment, regardless of bracing and surgical planning, in order to prevent further progression of the deformity, strengthen the muscular corset, and improve external respiration function and cardiovascular status [12].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
It is recommended in all patients to monitor follow-up and treatment by other medical specialists in order to achieve somatic status compensation [12].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
3.2 Surgical Treatment
It is recommended to perform surgical treatment in all patients under the following conditions:
- failure of conservative therapy, rapid progression of spinal deformity, and presence of initially severe deformities;
- application of differentiated surgical tactics with minimal involvement of the posterior spinal elements in children with immature bone tissue (first years of life);
- use of metal implants approved in the Russian Federation;
- preoperative planning;
- availability of a specialized orthopedic department with the necessary human and material-technical resources [15].
GPP BLOE 3Grade of Recommendation B (Level of Evidence – 3).
It is not recommended to perform surgical treatment in children and adolescents if conditions are lacking for:
- multi-stage and revision procedures;
- patient follow-up and rehabilitation monitoring [7].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
It is recommended to use pedicle screw fixation (A16.04.042 correction of spinal deformity) in patients undergoing surgical treatment in order to achieve the best clinical outcome [13].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
It is not recommended to use laminar fixation (when pedicle screw fixation is feasible) in patients undergoing surgical treatment in order to achieve the best clinical outcome [13].
Level CLevel 5Level of recommendation evidence C (reliability level of evidence – 5).
It is recommended to perform surgical treatment in patients diagnosed with Scheuermann's disease who have one or more of the following indications: deformity angle of 70–75° or greater; pronounced pain syndrome; neurological deficit; compression of the cardiopulmonary complex and its functions due to deformity; unsatisfactory subjective assessment of appearance by the patient, which prompts them to seek treatment [14].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
It is recommended in all patients to choose a surgical intervention (except for thoracoscopic ones) performed via a posterior approach rather than a two-stage approach (anterior and posterior), unless objective indications/contraindications require a different approach or approaches [15], [16].
GCR BLOE 2Grade of Recommendation B (Level of Evidence – 2).
Comments: Interventions performed via a posterior approach are characterized by comparable radiological parameters (including loss of correction during long-term follow-up) to those performed via two approaches (anterior and posterior), but they have more favorable parameters for intraoperative blood loss, operative time, and the incidence of adjacent segment disease [16]. According to [15] such interventions have a lower complication rate.
It is recommended in all patients undergoing surgical treatment, for the prevention of distal adjacent segment disease, to select as the distal fixation level the vertebra that corresponds to the plumb line passing through the posterior edge of the sacrum preoperatively in the standing position (sagittal stable vertebra) [17].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
It is recommended that all patients, given the appropriate technical equipment and qualified specialists, undergo deformity correction using thoracoscopic anterior release (A16.04.008.001 intervertebral disc excision using video-endoscopic technologies)
- with dorsal fixation to achieve the best clinical outcome [18], [19].
GPP CLOE 4Grade of Recommendation C (Level of Evidence – 4).
It is recommended that patients with kyphotic deformity of the thoracic or thoracolumbar spine caused by Scheuermann's disease undergo Ponte osteotomy as the primary surgical treatment method (if thoracoscopic anterior release is not feasible) to achieve the best clinical outcome [15].
GPP BLOE 3Grade of Recommendation B (Level of Evidence – 3).
It is recommended that patients with severe rigid kyphotic deformity and one or more of the following manifestations: severe pain syndrome; neurological symptoms caused by the curvature and compression of neural structures by deformed vertebral structures—perform posterior vertebral column resection (if technically feasible) for the most complete correction of the deformity and relief of clinical symptoms [15].
GPP BLOE 3Grade of Recommendation B (Level of Evidence – 3).
Comment: The listed symptoms and clinical manifestations are not specific to Scheuermann's disease; however, in cases where they accompany rapid disease progression, vertebral column resection (VCR) may be justified. If technical feasibility or sufficient qualifications of the operating surgeon are lacking, referral for consultation and/or a telemedicine consultation with leading orthopedic and trauma medical centers is justified.
It is recommended to use (if technically feasible) intraoperative neuromonitoring during surgical correction of kyphotic deformity in all patients with Scheuermann's disease to minimize the risk of nerve structure damage [12].
GCR CLOE 5Grade of Recommendation C (Level of Evidence – 5).
Comment: Currently, no studies have been published that allow recommending the use of intraoperative neuromonitoring specifically in the treatment of kyphosis in Scheuermann's disease. Nevertheless, according to literature data, neuromonitoring significantly reduces the risk of intraoperative nerve injury during surgery to correct severe deformities (kyphotic, kyphoscoliotic, scoliotic) regardless of their etiology.
3.3 Pain management
It is recommended that patients presenting with pain be prescribed nonsteroidal anti-inflammatory and antirheumatic drugs (at the discretion of the attending physician, in accordance with indications and in the absence of contraindications), as well as other analgesics (N02) at appropriate dosages and taking into account the allergy history [12].
GCR CLOE 5Grade of Recommendation C (Level of Evidence – 5).
Comments: Pain management in the surgical treatment of Scheuermann's disease does not require any treatment regimens unique to this condition; therefore, standard pain management protocols for spine surgery in general are followed.
3.4 Dietary Therapy
No specific dietary therapy has been developed.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
It is recommended to draw up a medical rehabilitation program for all patients discharged from the hospital aiming at early motor activation of the patient [12].
GCR CLOE 5Grade of Recommendation C (Level of Evidence – 5).
It is recommended:
- to monitor at all stages of follow-up the presence and adherence to a set of orthopedic prescriptions and non-drug treatments to maintain the muscular corset in an optimal state;
- in the presence of comorbid pathology, to conduct timely specialist consultations and monitor the implementation of their prescriptions;
- to use technologies in the postoperative period that promote timely healing of the surgical wound and prevent the development of infectious complications;
- to avoid significant loads on the spine in the long-term postoperative period (prolonged sitting, standing, walking, lifting heavy objects, bending and twisting the torso, contact sports) [13].
GCR CLOE 5Grade of Recommendation C (Level of Evidence – 5).
Comments: In the early postoperative period (7–14 days after radical spine surgeries), rehabilitation measures are carried out to achieve early motor activation of the patient, relieve pain, and stimulate reparative processes in the wound defect area.
Recovery of motor activity begins on the first day using breathing exercises and passive exercises in bed. Verticalization and gait restoration measures are carried out, including the use of special therapeutic exercises, verticalizers, and subsequently – balance systems.
Rehabilitation using non-drug therapies is carried out in combination with nonsteroidal anti-inflammatory and antirheumatic drugs; other drug groups are prescribed according to indications (N02 Analgesics).
In the late postoperative period (from 2 to 8 weeks after radical spine surgeries), the goal of rehabilitation measures is the full restoration of motor activity, the formation of the muscular corset, and the stimulation of metabolic processes in the spinal tissues to prevent the recurrence of pain.
Of paramount importance in this period is physical therapy aimed at strengthening the muscles of the back, interscapular region, abdominal wall, and buttocks, forming correct posture skills and proper movement stereotypes, and providing post-isometric muscle relaxation. Special therapeutic gymnastics classes are supplemented with specialized exercise machines (physical therapy using apparatus and exercise machines for spinal disorders) and training on a stabilometric platform. Physical therapy classes are combined with physiotherapy methods.
Rehabilitation measures in the late postoperative period are carried out on an outpatient basis or in a sanatorium. The main types of treatment at the outpatient stage are physical therapy for spinal disorders with a selection of exercises designed for the treatment of kyphosis, massage for spinal disorders, and pool sessions (therapeutic swimming in the pool). Outpatient courses of rehabilitation treatment are recommended 4 times a year.
It is recommended that all patients during and for the purpose of planning rehabilitation measures take into account the reduction in pulmonary ventilation parameters, which requires special monitoring when the kyphotic deformity angle is greater than 75 degrees [12].
GCR CLOE 5Grade of Recommendation C (Level of Evidence – 5).
5. Prevention and Dispensary Observation
It is recommended to carry out follow-up evaluations at 6, 12, 24, and 36 months after the final stage of surgical treatment at the surgical site, including vertical spinal radiography, examination by an orthopedic trauma surgeon and a neurologist, computer-assisted optical spinal topography, photography, and completion of the SRS-22 questionnaire [13] (Appendix G1).
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: It is quite difficult to talk about prevention, but timely detection and initiation of conservative treatment help avoid the development of severe, neglected spinal deformities, neurological complications, and disability. In addition, proper conservative orthopedic management of the patient in some cases removes the question of indications for surgical treatment. Dispensary observation of such patients is carried out by a pediatric orthopedic – trauma surgeon once a year from the moment the disease is detected until 18 years of age (the end of the active growth period), and thereafter as indicated. If pain syndrome develops, the patient is monitored and receives outpatient treatment from a neurologist.
It is recommended that all patients, in order to prevent complications, continue conservative treatment to maintain the muscular corset in an optimal state throughout their lives, regardless of the use of surgical techniques; [1]-[3], [10], [13].
LEr CQoE 4Level of Evidence Recommendation C, Quality of Evidence – 4
It is recommended that all patients, in order to prevent complications, take into account the topographical and anatomical features of the affected segment, correctly select instruments, implants, and types of surgical intervention, and observe the rules of asepsis and antisepsis at all stages of treatment to prevent surgical complications [1]–[3], [10], [13].
LEr CQoE 4Level of Evidence Recommendation C, Quality of Evidence – 4
Comments: Information on the risks associated with the lack of consistent treatment for this pathology is provided in the "Additional Information" section.
6. Organization of Medical Care
Indications for hospital admission to a medical organization:
Presence of verified outpatient Scheuermann's disease.
Fact of seeking medical care for the specified disease, presenting with complaints of kyphotic deformity and/or pain syndrome caused by the specified disease.
Indications for patient discharge from a medical organization
Achieved improvement regarding deformity correction and sagittal balance, and reduction of the pre-treatment pain syndrome.
Absence or, if they occur, resolution of septic, inflammatory, and other complications caused by the treatment.
Patient mobilization (verticalization), ability to perform self-care.
7. Additional Information
Information on the risks associated with the lack of sequential treatment for Scheuermann's disease and its manifestations
With the progression of untreated spinal deformity, there is a high risk of developing severe neurological complications and deterioration of somatic status, up to profound disability. Even minimal trauma can result in the spinal cord being "transected" by the bony structures of the destabilized spine.
In surgical treatment, along with the risks associated with the quality of execution and the effectiveness of the surgical intervention, one must consider the possibility of complications that may require re-operation. They can be divided into three groups: implant-related complications; non-implant-related surgical complications; and infectious complications. The most commonly anticipated complications include:
a. Dural tear, postoperative cerebrospinal fluid (CSF) leakage;
b. Neurological complications, including worsening of limb paresis, hypoesthesia, urinary retention due to spinal cord circulatory disorders at the conus-epiconus level;
c. Postoperative edema of the spinal nerve roots;
d. Epidural hematoma;
e. Implant-related complications;
f. Infectious processes at the surgical site;
g. Bleeding, hematoma due to ineffective postoperative wound drainage or ongoing hemorrhage;
h. Pleural injuries.
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | Performance evaluation |
|---|---|---|
1 | Spinal imaging was performed (using one or more methods at the discretion of the attending physician): radiography of the thoracic and lumbosacral spine, computed tomography of the spine (one segment) (thoracic region, lumbosacral region), magnetic resonance imaging of the spine (one segment) (thoracic region, lumbosacral region), computer-assisted optical spinal topography. | yes / no |
2 | Patients presenting with pain were prescribed nonsteroidal anti-inflammatory and antirheumatic drugs (at the discretion of the attending physician, according to indications and in the absence of contraindications), as well as other analgesics (N02) in appropriate dosages and taking into account the allergy history. | yes / no |
3 | Surgical treatment was performed in the presence of indications and absence of contraindications. | yes / no |
4 | A medical rehabilitation program was drawn up for patients discharged from the hospital. | yes / no |
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Appendix A1. Working Group Composition
Mikhailovsky Mikhail Vitalievich – MD, Professor, The Y.L. Tsivyan Novosibirsk Research Institute of Traumatology and Orthopaedics of the Russian Ministry of Health, Member of the Interregional Public Organization "Association of Spine Surgeons";
Sorokin Artem Nikolaevich – PhD in Medicine, Traumatologist-Orthopedist, The Y.L. Tsivyan Novosibirsk Research Institute of Traumatology and Orthopaedics of the Russian Ministry of Health, Member of the Interregional Public Organization "Association of Spine Surgeons";
Novikov Vyacheslav Viktorovich – MD, The Y.L. Tsivyan Novosibirsk Research Institute of Traumatology and Orthopaedics of the Russian Ministry of Health, Board Member of the Interregional Public Organization "Association of Spine Surgeons";
Kolesov Sergey Vasilievich – MD, Professor, National Medical Research Center of Traumatology and Orthopedics named after N.N. Priorov of the Russian Ministry of Health, President of the Interregional Public Organization "Association of Spine Surgeons"; Member of ASTR.
Gorbatyuk Dmitry Sergeevich – Traumatologist-Orthopedist, National Medical Research Center of Traumatology and Orthopedics named after N.N. Priorov of the Russian Ministry of Health, Member of ASTR.
Zhelnov Pavel Viktorovich – Traumatologist-Orthopedist, National Medical Research Center of Traumatology and Orthopedics named after N.N. Priorov of the Russian Ministry of Health.
Conflict of interest: the authors declare no conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience for these clinical guidelines:
- neurologists;
- neurosurgeons;
- traumatologists-orthopedists;
- chief physicians (heads) of a medical organization;
- deputy heads (chiefs) of a medical organization;
- heads (chiefs) of a structural subdivision (department, unit, laboratory, office, squad, etc.) of a medical organization – medical specialists;
- heads (chief physicians, chiefs) of a structural subdivision carrying out medical activities in another organization.
Material for the clinical guideline was collected by analyzing relevant publications known to the members of the working group.
The level of scientific evidence supporting the recommendation statements was assessed according to Tables 1 and 2, and the strength of the recommendations was determined according to Table 3 (Appendix No. 2 to the Requirements for the Structure of Clinical Guidelines, Composition and Scientific Validity of Information Included in Clinical Guidelines, approved by Order of the Ministry of Health of the Russian Federation No. 103n dated February 28, 2019).
Table 1. Levels of Evidence (LoE) grading scale 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. Scale for assessing the levels of evidence (LE) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative 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 3. Scale for grading levels of recommendation certainty (LRC) for prevention, diagnostic, treatment, and rehabilitation methods (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 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 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
Order of the Ministry of Health of the Russian Federation No. 919n dated November 15, 2012, "On Approval of the Procedure for Providing Medical Care to the Adult Population in the Profile 'Anesthesiology and Reanimatology';"
Order of the Ministry of Health of the Russian Federation No. 909n dated November 12, 2012, "On Approval of the Procedure for Providing Medical Care to Children in the Profile 'Anesthesiology and Reanimatology';"
Order of the Ministry of Health of the Russian Federation No. 901n dated November 12, 2012, "On Approval of the Procedure for Providing Medical Care to the Population in the Profile 'Traumatology and Orthopedics';"
Order of the Ministry of Health of the Russian Federation No. 562n dated October 31, 2012, "On Approval of the Procedure for Providing Medical Care in the Profile 'Pediatric Surgery';"
Appendix B. Physician Action Algorithms
Appendix 1. Algorithm for patient management in the provision of primary care to patients with suspected kyphosis
Appendix 2. Algorithm for patient management in the provision of specialized, including high-tech, medical care
Appendix C. Patient Information
Scheuermann's disease is a progressive kyphotic spinal deformity (kyphosis = hunchback) that occurs during a period of rapid child growth.
The exact causes of Scheuermann's disease are unknown. The most likely cause is considered to be a genetic predisposition. Another possible cause is trauma to the growth plates of the vertebral bodies. The potential pathological impact of abnormal development of the back muscles is also considered.
The first signs of Scheuermann's disease appear in children during puberty. In the early stage of the disease, a correct diagnosis can be made exclusively based on spinal radiography. Diagnosis is frequently delayed because patients present to the doctor with a noticeable spinal curvature and complaints of mid-back pain. As the disease progresses, the kyphotic curve increases, along with back pain.
Diagnostics
The basis of diagnosis is radiographic examination of the spine (or multislice computed tomography), which allows accurate determination of the severity of pathological changes in the vertebral growth plates and the magnitude of the kyphotic spinal deformity. If necessary, the physician prescribes magnetic resonance imaging and electroneuromyography.
Treatment Methods
Patient follow-up by a traumatologist-orthopedist is carried out every 12 months. Treatment for Scheuermann's kyphosis can be surgical or conservative. The specific method is determined by the attending physician. Most commonly, this condition is treated conservatively using physical therapy procedures, massage for spinal disorders, and therapeutic exercise techniques. Bracing is sometimes necessary. Surgical intervention is resorted to only in extreme cases, as surgery carries a certain degree of risk.
Indications for surgery in kyphosis include:
- persistent pain syndrome not relieved by conventional conservative treatment;
- kyphosis angle greater than 65 degrees;
- impairment of respiratory and circulatory functions.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Appendix G1. Postoperative Questionnaire of the Scoliosis Research Society – SRS-22
Russian title: Postoperative Questionnaire of the Scoliosis Research Society – SRS-22.
Original title: Scoliosis Research Society Outcomes Instrument-22.
Source (official developer website): https://www.srs.org/Research/Patient-Outcome-Questionnaires; validation study in children [18] (for the SRS-24 questionnaire, which is an earlier version of the SRS-22 questionnaire [19]; no validation studies of the Russian-language version of SRS-22 in children have been published), validation study in adults [20].
Source of the Russian-language version: [20].
Type: questionnaire
Purpose: assessment of the patient's subjective state and quality of life
Content:
Patient's full name: _______________________________ Date of birth: ____________________
Date of completion: ______________ Have you had spinal surgery and when: _______________
Instructions: We have carefully evaluated the condition of your back. It is important that you answer all questions independently. Please circle the single most appropriate answer option for each question.
1. How would you describe the amount of pain you have experienced in the past 6 months?
5 – none; 4 – mild; 3 – moderate; 2 – moderate to severe; 1 – severe.
2. How would you describe the amount of pain you have experienced in the past month?
5 – none; 4 – mild; 3 – moderate; 2 – moderate to severe; 1 – severe.
3. Have you been a very nervous person in the past 6 months?
5 – none of the time; 4 – a little bit of the time; 3 – some of the time; 2 – a good bit of the time; 1 – all of the time.
4. How would you feel if you had to spend the rest of your life with the appearance of your back as it is now?
5 – very satisfied; 4 – somewhat satisfied; 3 – neither satisfied nor dissatisfied; 2 – somewhat dissatisfied; 1 – very dissatisfied.
5. What is your level of daily activity?
1 – bedridden; 2 – mostly inactive; 3 – light work and light sports;
4 – moderate work and moderate sports; 5 – full activity without restrictions.
6. How do you look in clothes?
5 – very well; 4 – well; 3 – satisfactory; 2 – poorly; 1 – very poorly.
7. In the past 6 months, have you felt downhearted and blue, with nothing able to cheer you up?
1 – very often; 2 – often; 3 – sometimes; 4 – rarely; 5 – never.
8. Do you experience back pain at rest?
1 – very often; 2 – often; 3 – sometimes; 4 – rarely; 5 – never.
9. What is your level of work/study performance?
5 – 100% of normal; 4 – 75% of normal; 3 – 50% of normal; 2 – 25% of normal; 1 – 0% of normal.
10. What does your trunk look like, excluding the head and limbs?
5 – very well; 4 – well; 3 – satisfactory; 2 – poorly; 1 – very poorly.
11. How often and what medications do you take to reduce back pain?
5 – none; 4 – pain relievers weekly or less often; 3 – pain relievers daily;
2 – strong pain relievers weekly or less often (tramadol, tramal);
1 – strong pain relievers daily (tramadol, tramal).
12. Does your spine limit you in housework?
5 – never; 4 – rarely; 3 – sometimes; 2 – often; 1 – very often.
13. Have you felt calm and peaceful in the past 6 months?
5 – all of the time; 4 – a good bit of the time; 3 – some of the time; 2 – a little bit of the time; 1 – none of the time.
14. Do you think your condition affects your relationship with loved ones?
5 – no; 4 – slightly; 3 – insignificantly; 2 – moderately; 1 – severely.
15. Do you and/or your family experience financial difficulties due to the condition of your spine?
1 – severe; 2 – moderate; 3 – minor; 4 – slight; 5 – no.
16. Over the past 6 months, have you felt depressed or downcast?
5 – never; 4 – rarely; 3 – sometimes; 2 – often; 1 – very often.
17. Have you taken sick leave due to back pain (if working)?
Was your working capacity in daily activities limited due to back problems?
5 – 0 days; 4 – 1 day; 3 – 5–10 days; 2 – 10–14 days; 1 – more than 14 days.
18. Does your back condition restrict you from meeting with family/friends?
5 – never; 4 – rarely; 3 – sometimes; 2 – often; 1 – very often.
19. Do you consider yourself attractive at present?
5 – yes, very; 4 – yes, somewhat; 3 – neither yes nor no; 2 – no, not really; 1 – absolutely not.
20. Have you felt happy over the past 6 months?
1 – never; 2 – a little bit of the time; 3 – some of the time; 4 – a good bit of the time; 5 – all of the time.
21. Are you satisfied with the outcome of your spinal treatment?
5 – very satisfied; 4 – satisfied; 3 – neither yes nor no; 2 – dissatisfied; 1 – very dissatisfied.
22. Would you undergo the same treatment again given the same back condition?
5 – definitely yes; 4 – probably yes; 3 – not sure; 2 – probably no; 1 – definitely no.
Scoring key for the SRS-22 questionnaire
Domain | Questions | Sum of answers (A) | Number of answered questions (B) | Mean score (A/B) |
|---|---|---|---|---|
Function | 5, 9, 12, 15, 18 | |||
Pain | 1, 2, 8, 11, 17 | |||
Self-image | 4, 6, 10, 14,19 | |||
Mental health | 3, 7, 13, 16, 20 | |||
Satisfaction with management | 21, 22 | |||
Total |
Unanswered questions should be excluded from the sum of answered questions.
Remove questions with more than one answer.
The domain (subject area) cannot be assessed if fewer than three questions are answered.