Perthes disease or Mayer's dysplasia. Dysplasia in children: causes, signs, treatment, prognosis for the future

- a large group of genetically heterogeneous skeletal dysplasias, which are united by a violation of the formation of the enchondral spongy bone in the area of the metaphyses and epiphyses of the bones, as well as (in some cases) the vertebral bodies. Symptoms of this condition vary depending on the type of disease, most often there is a curvature of the legs, anomalies of the joints of the limbs, and a decrease in growth. Diagnosis of epiphyseal dysplasia is carried out on the basis of X-ray data, a study of the patient's hereditary history, and molecular genetic analyzes. There is no specific therapy; palliative and symptomatic treatment is possible, including orthopedic measures and surgical correction.

General information

Epiphyseal dysplasia is one of the most common forms of congenital skeletal lesion, having a genetic nature with a diverse pattern of inheritance and clinical manifestations. Many variants of this pathology have been known for a long time, however, as a separate nosological unit, it was described in 1935 by the German doctor T. Feirbank, who was able to correctly determine the cause of the disease - a violation of the development of the epiphyses of the bones. Most forms of epiphyseal dysplasia are characterized by an autosomal dominant pattern of inheritance, however, penetrance and expressivity fluctuate over a very wide range, which affects the clinical course of the pathology. The incidence of a condition with an autosomal dominant pattern of inheritance is about 1:10,000, the frequency of a rare autosomal recessive form of the disease is unknown. Epiphyseal dysplasias of most types are equally likely to affect both men and women, but some varieties are slightly more common in males (the distribution is approximately 3:1).

Causes and classification of epiphyseal dysplasia

Epiphyseal dysplasias are characterized by pronounced genetic heterogeneity; this is the basis for the classification of various variants of this disease. In addition, there are certain phenotypic differences in the course of pathology and its prognosis. According to geneticists, more than half of all clinical cases of epiphyseal dysplasia are based on currently unknown molecular genetic disorders, so research on this disease continues. In total, more than 10 different forms of this pathology are currently known, the most common are types 1, 2, 3 and 4.

Epiphyseal dysplasia type 1 (Fairbank's disease) is an autosomal dominant disease and the most common type of this skeletal malformation. It is caused by mutations in the COMP gene, which is located on the 19th chromosome and encodes the oligomeric cartilage matrix protein, one of the proteins responsible for the metabolism and development of bone and cartilage tissue. In addition to type 1 epiphyseal dysplasia, mutations in this gene can lead to another well-known musculoskeletal disorder, pseudoachondroplasia. One of the reasons for the widespread occurrence of this pathology is the autosomal dominant nature of the inheritance of epiphyseal dysplasia.

Type 2 epiphyseal dysplasia is caused by a mutation in the COL9A2 gene, which encodes the alpha-2 chain of type 9 collagen, which is the most abundant in bone and cartilage. As a rule, missense mutations are noted in this gene, leading to a change in the structure of the encoded protein, which causes pathological changes.

Type 3 epiphyseal dysplasia is largely similar in its etiology to the previous variant of the disease, as it is caused by a mutation of the COL9A3 gene located on the 20th chromosome. It encodes another type 9 collagen chain, therefore, disturbances in its structure lead to the development of skeletal pathologies and complicate the formation of endochondral bones.

Epiphyseal dysplasia type 4 is caused by mutations in the SLC26A2 gene located on the 5th chromosome. It is an autosomal recessive disease. The expression product of this gene is considered to be a transmembrane protein of chondroblasts and osteoblasts, which is responsible for the transport of sulfate ions, which are essential for the formation of cartilage and bone tissue proteoglycans. Structural features of SLC26A2 lead to a relatively high frequency of development of defects in it; therefore, mutations in this gene are the cause of many hereditary diseases of the musculoskeletal system. In addition to multiple epiphyseal dysplasia, SLC26A2 defects are the cause of some types of achondrogenesis, atelosteogenesis, diastophic dysplasia.

There are a number of other types of epiphyseal dysplasia, and key genes have been identified for some. But they are much less common - there are forms of these diseases described in only a few families. In addition to the actual etiology, various forms of epiphyseal dysplasia can differ in clinical course - different age of onset of the disease, the presence or absence of other disorders (hearing loss, deafness, myopia, skin abnormalities). However, the nature of skeletal disorders in different forms is very similar and comes down to anomalies in the development of the joints, especially those that experience the greatest load - the hip, knee, ankle. The role of physical activity in the development of skeletal anomalies in epiphyseal dysplasia is quite large, therefore, various orthopedic methods of treatment can significantly improve the condition of patients.

Symptoms of epiphyseal dysplasia

Due to the pronounced heterogeneity of epiphyseal dysplasia, the onset of symptoms of this disease can begin at different ages, depending on the type of pathology. Some forms lead to skeletal anomalies that are already registered at the birth of the patient, a significant part of the varieties are characterized by the development of defects at the age of 2-3 years, some rare types of epiphyseal dysplasia are diagnosed in adolescence or even adulthood. The reason why this disorder usually begins in early childhood is due to the increased stress placed on bones and joints after walking and weight gain.

Many forms of epiphyseal dysplasia are characterized by the development of X- or O-shaped curvature of the legs, due to deformation of the epiphyses of the femur and tibia. In some cases, short stature is noted, caused both by a reduction in the length of the limbs (due to the shortening of long tubular bones) and a decrease in the body due to spinal deformity. Almost all types of epiphyseal dysplasia, to a greater or lesser extent, lead to hypoplasia of the vertebral bodies and the delayed formation of ossification points in them. This can cause a decrease in the length of the spinal column and its various curvatures (scoliosis, lordosis) - especially in the absence of orthopedic correction methods. A common symptom of many types of epiphyseal dysplasia is also increased mobility of a number of joints.

Separate forms of epiphyseal dysplasia, in addition to skeletal disorders, are accompanied by lesions of internal organs, eyes, auditory and endocrine systems. For example, the Walcott-Ralshson type is manifested by the early development of insulin-dependent diabetes mellitus and myopia, some other forms are combined with deafness. Varieties of epiphyseal dysplasia are described, which are also characterized by osteoporosis and skin atrophy. Intellectual development is not affected in most types of the disease, but in some forms, mental retardation of varying severity can be observed. Most often, epiphyseal dysplasia does not affect the life expectancy of patients, but concomitant disorders characteristic of some forms can lead to severe complications.

Diagnosis and treatment of epiphyseal dysplasia

The leading role in determining any type of epiphyseal dysplasia is played by x-ray studies, general examination of patients and molecular genetic analyzes. In some cases, the study of hereditary history is additionally used - its results may vary depending on the autosomal recessive or dominant nature of the inheritance of the pathology. On radiographs, depending on the form of epiphyseal dysplasia and the age of patients, a slowdown in the processes of ossification of the epiphyses, their deformation, and shortening of long tubular bones can be determined. Older patients often show enlargement and deformity of the knee and ankle joints. Many forms of epiphyseal dysplasia also lead to deformities of the vertebral bodies, their bone age often lags behind the actual one.

Molecular genetic diagnosis of epiphyseal dysplasia is possible for only some of the most common forms of the disease with reliably known etiology. As a rule, most laboratories and clinics provide this option for types of pathology caused by mutations in the COMP, COL9A2, COL9A3 and SLC26A2 genes. The most commonly used method is direct automatic sequencing of the above genes in order to detect mutations. To diagnose forms of epiphyseal dysplasia that are combined with other malformations (for example, deafness, eye and endocrine disorders), other research methods may be required - examination by a specialist of the appropriate profile, blood and urine tests.

At the moment, there is no specific treatment for epiphyseal dysplasia; a variety of methods of supportive and symptomatic therapy are used. It is especially important to prescribe orthopedic correction in a timely manner - wearing bandages and corsets to reduce the load on the spine and leg joints. This avoids severe deformities and thus improves the quality of life of patients with epiphyseal dysplasia. Some already developed curvature and defects can be corrected by surgical intervention. Symptomatic treatment is also indicated for disorders that accompany some forms of epiphyseal dysplasia - diabetes mellitus, myopia, osteoporosis.

Forecast and prevention of epiphyseal dysplasia

As a rule, the prognosis of most forms of epiphyseal dysplasia in terms of patient survival is favorable - limb deformities and short stature do not threaten life and do not shorten its duration. Only some severe forms of curvature of the spine can lead to violations of the internal organs, which aggravates the course of the disease. With the timely detection of epiphyseal dysplasia and the beginning of orthopedic treatment, disability of patients is relatively rare, many retain mobility and ability to work (albeit somewhat limited). The presence of concomitant disorders, especially from the endocrine system (diabetes mellitus), worsens the prognosis. Prevention of epiphyseal dysplasia is possible only within the framework of medical genetic counseling of parents before conceiving a child (with aggravated heredity) and prenatal diagnosis by molecular genetic methods.

Epiphyseal dysplasia of the femoral heads - Mayer's dysplasia.

Chechenova Fatima Valerievna

Epiphyseal dysplasia of the femoral heads - Mayer's dysplasia

Dissertations for a degree

Candidate of Medical Sciences

Moscow - 2009

The work was performed at the Federal State Institution Central Research Institute of Traumatology and Orthopedics named after A.I. N.N. Priorov Rosmedtekhnologii

Scientific adviser:

Doctor of Medical Sciences Mikhailova Lyudmila Konstantinovna

Official opponents:

Doctor of Medical Sciences Kozhevnikov Oleg Vsevolodovich

Doctor of Medical Sciences, Professor Kuznechikhin Evgeny Petrovich

Leading organization: Federal State Institution "Moscow Research Institute of Pediatrics and Pediatric Surgery of Rosmedtekhnologii".

The defense will take place at a meeting of the council for the defense of doctoral and master's theses D 208.112.01 at the Central Research Institute of Traumatology and Orthopedics. N.N. Priorov Rosmedtekhnologii.

Address: 127299, Moscow, st. Priorova, 10.

The dissertation can be found in the library of the FGU CITO.

Scientific Secretary of the Council for the Defense of Doctoral and Candidate's Dissertations S.S. Rodionova

The relevance of the work.

In the practice of a children's orthopedic clinic, there is often a need for diagnosis and differential diagnosis of diseases of the femoral head in preschool children. Sometimes the clinical and radiological picture of coxalgia is interpreted as the initial stage of Legg-Calve-Perthes disease and appropriate treatment is carried out (primarily prolonged immobilization), which, in case of an erroneous diagnosis, can lead to serious complications. At the same time, in preschool children, an orthopedist encounters a pathology that is diagnosed as isolated dysplasia of the femoral heads - a variant of epiphyseal dysplasia - Mayer's dysplasia.

So far, isolated dysplasia of the femoral heads - Mayer's dysplasia - has not been sufficiently studied. The first to identify epiphyseal dysplasia of the femoral head was S. Pedersen (1960). In his studies, he described such patients as: "patients with an atypical course of the Legg-Calve-Perthes disease."

J. Meyer (1964) believed that dysplasia of the epiphyses of the femoral heads initially manifests itself, and then, against its background, Legg-Calve-Perthes disease develops.

P. Maroteaux & Hedon (1981) wrote about 35 cases of isolated epiphyseal dysplasia of both hip joints in children under 6 years of age.

In the domestic literature, systematic data on Mayer's dysplasia are practically absent. At the same time, the timely and correct diagnosis of Mayer's dysplasia is extremely important for adequate and effective treatment.

Diagnosis of Mayer's dysplasia in children is extremely important, since the orthopedist must monitor the development of the epiphyses of the femoral heads as the child grows. Inadequate load (jumping, hypermobility), lack of vitamins, protein and mineral components in the child's diet can lead to impaired development of the epiphyses. First of all, Mayer's dysplasia should be differentiated from multiple epiphyseal dysplasia, Legg-Calve-Perthes disease and reactive synovitis of the hip joint.

Insufficient knowledge of Mayer's dysplasia leads to an inadequate approach to the treatment of patients who are contraindicated in long-term immobilization, since the treatment affects the degree of restoration of the anatomical and functional usefulness of the hip joint during the growth of a child with Mayer's dysplasia. All this determines relevance of this topic at the present stage of development of pediatric orthopedics.

Purpose of the study - to develop a detailed clinical and radiological characteristics and differential diagnostic criteria for Mayer's dysplasia in the age aspect.

Research objectives:

1. Formulate an algorithm for examining children with coxalgia and dysfunction of the hip joint aged 1 to 7 years.

2. To study the possibilities of clinical, radiological and laboratory research methods in the diagnosis of Mayer's dysplasia.

3. To develop differential diagnostic criteria for Mayer's dysplasia with Legg-Calve-Perthes disease, with multiple epiphyseal dysplasia, with reactive synovitis of the hip joint in preschool children.

4. Develop a set of therapeutic measures for Mayer's dysplasia. 5. To study the immediate results of treatment of children with Mayer's dysplasia.

Materials and research methods.

The work is based on the analysis of the results of a survey of 278 patients aged 1 to 7 years with clinical symptoms of hip joint pathology, who were examined and treated in the outpatient clinic at the CITO pediatric consultative clinic from January 2000 to July 2008. Of these, with a primary diagnosis of Perthes' disease - 106 patients, with a diagnosis of coxalgia - 55 children, with reactive synovitis of the hip joint - 84 children.

After examination and analysis of clinical and radiographic data of 278 patients, a group of children with Mayer's dysplasia was identified - 31 children, of which 23 were boys (74.2%) and 8 were girls (25.8%).

The following methods were used in the work: clinical examination with anthropometry and angulometry, radiography, radiometry, ultrasonography, computed tomography, magnetic resonance imaging, laboratory methods.

Scientific novelty.

For the first time, the clinical and radiological picture of Mayer's dysplasia has been studied in detail. An algorithm for the differential diagnosis of Mayer's dysplasia with multiple epiphyseal dysplasia, Legg-Calve-Perthes disease, and reactive synovitis of the hip joint in preschool children has been developed. A therapeutic tactic has been developed, the terms of dispensary observation for Mayer's dysplasia, depending on the presence or absence of complications (aseptic necrosis of the femoral head).

The X-ray picture of epiphyseal dysplasia of the femoral heads in children in the process of growth was traced. With Mayer's dysplasia, there was a decrease in the index of the epiphysis of the femoral heads on both sides compared with the index of the epiphysis of the femoral heads calculated in healthy children in similar age groups using radiometry.

It has been proven that during the growth of a child with Mayer's dysplasia, the proximal epiphyses of the femur remain reduced in height.

Practical significance.

The correct diagnosis and observation of patients with Mayer's dysplasia until the end of skeletal growth made it possible to maintain normal joint function with adequate physical activity and avoid unindicated treatment. Children with delayed development of the proximal epiphyses of the femur need to be observed until the end of growth with adequate correction of motor activity.

Provisions of the dissertation submitted for defense.

Mayer's dysplasia is an inherited skeletal disorder. Children with Mayer's dysplasia should be observed by an orthopedist during skeletal growth. Conservative treatment is indicated for patients with epiphyseal dysplasia of the femoral heads only in the case of aseptic necrosis of the femoral head.

Approbation of work.

The main provisions of the work were reported and discussed at the VIII Congress of Traumatologists and Orthopedists of Russia (Samara, June 6-8, 2006), at the XI Congress of Russian Pediatricians "Actual Problems of Pediatrics" (Moscow, February 5-8, 2007), at the Republican Scientific and Practical conference with international participation "Actual problems of pediatric traumatology, orthopedics and neurosurgery" (Tashkent, May 24, 2007), at the scientific and practical conference of pediatric traumatologists and orthopedists of Russia with international participation "Actual problems of pediatric traumatology and orthopedics" (Yekaterinburg, September 19-21, 2007 .), at the scientific and practical conference with international participation "Actual problems of bone pathology in children and adults" (Moscow, April 23-24, 2008), at the All-Russian symposium of pediatric orthopedic traumatologists with international participation "Improvement of traumatological and orthopedic care for children" ( Kazan September 16-18, 2008).

Publications and information about introduction into practice.

Scope and structure of work.

The dissertation material is presented on 126 pages of typewritten text, illustrated with 11 tables, 5 diagrams, 47 figures. It consists of an introduction, 5 chapters, a conclusion, conclusions, a list of references, including 168 sources, of which 68 are domestic and 100 are foreign.

This work is based on a study of the clinic, diagnosis and treatment of 278 patients of the children's consultative clinic of the FGU CITO named after. N.N. Priorov at the age of 1 to 7 years with clinical symptoms of hip joint pathology since January 2000. to July 2008 All children were on outpatient treatment and were examined in a children's polyclinic, radiology department and functional diagnostics department.

Patients were examined clinically, radiologically and laboratory, the maximum follow-up period was 9 years.

Septic arthritis was differentiated from transient synovitis on the basis of 4 clinical indicators: fever of the child more than 37.5°C, lack of support on the leg, ESR more than 20 mm/hour, and leukocytosis more than 12,000 cells/mm3. If septic arthritis of the hip joint was suspected, the children were hospitalized in specialized departments and were not included in our study.

With persistent synovitis of the hip joint that did not respond to therapy for 2-3 months, patients were referred for immunological examination to exclude a specific infection (cytomegalovirus, mycoplasma, toxoplasma, chlamydia, herpes, etc.). In our work, from a group of children with synovitis of the hip joint, synovitis persisted for 2.5 months in 3 patients during therapy. Upon further examination, chlamydial infection was diagnosed in 1 child, mycoplasma infection in combination with the herpes simplex virus in 1 girl. A 6-year-old boy had synovitis of both hip joints, which was difficult to treat, and at first the disease began on the right side, then, after 4 months, the pathological process was diagnosed in the left hip joint. A detailed examination of the child revealed a cytomegalovirus infection in the blood. After the specific therapy, no signs of synovitis of the hip joint were found in all three children with control ultrasonography.

From our observation, it can be seen that most often patients came with a primary diagnosis of Perthes disease (38%). However, after a survey of 278 patients under our control, Perthes' disease was confirmed in 28% of children. Reactive synovitis of the hip joint was diagnosed in 32% of patients, transient coxalgia was diagnosed in 10%. With tuberculous coxitis - 4 children and 2 patients with rheumatoid arthritis were not included in our further study. They were sent to specialized institutions for treatment and further observation.

42 patients (15%) came to us with complaints of gait disturbance, but did not appear for a second appointment. We contacted by phone the parents of 11 children who applied once, 11-24 months after the consultation at the CITO, and found out that there were no more gait disorders, and they are observed in the polyclinic at the place of residence.

It is noteworthy that the largest number of single appeals falls on the age group from 1 to 3 years. It can be assumed that this is exactly the age when the first complaints of the child associated with a possible pathology of the hip joint “slip”. It is very important to take such patients under dynamic observation until the end of skeletal growth.

To conduct a comparative characteristic of the height of the femoral heads in children with Mayer's dysplasia, in comparison with normal sizes, radiometry of normal hip joints was performed in 68 children aged 1 to 7 years, and the index of the epiphysis of the femoral head was calculated.

HEAD EPIPHYSIS INDEX = a: b,

where a- longitudinal size of the femoral head (height), b- the transverse dimension of the head.

Fig.1. X-ray of normal hip joints

Table number 1.

The average size of the epiphysis of the femoral head in healthy children aged 1 to 7 years.

Longitudinal dimension (height) of the femoral head (mm)

Transverse head size

hip(mm)

Femoral head epiphysis index (IE)

With age, this index of the epiphysis of the femoral head increases, while with Mayer's dysplasia in each age group, the index of the epiphysis is less than in the same group in healthy children.

To do this, we selected radiographs of children whose parents went to the children's polyclinic for an examination about incorrect posture and the hip joints were included in the survey radiography of the spine; for the purpose of preventive examination; children with unilateral dislocations (measurements were taken on the opposite side). The longitudinal and transverse dimensions of the epiphysis of the femur were determined on radiographs of the hip joints in direct projection and with the correct positioning of the child. Comparison of the obtained X-ray data made it possible to speak more accurately about the degree of decrease in the height of the femoral heads in children with Mayer's dysplasia.

After a clinical and radiological examination of 278 patients, we identified a group of children with Mayer's dysplasia - 31 (11.2%) people, including 23 boys and 8 girls.

All children with Mayer's dysplasia were divided into age groups: from 1 to 2 years - 7 patients (22.6%), from 2 to 3 years - 11 children (35.5%), from 3 to 4 years - 2 children ( 6.5%), 4-5 years old - 6 children (19.4%), 5-6 years old - 3 patients (9.7%), 6-7 years old - 2 patients (6.5%).

It should be noted that in the group of patients with Mayer's dysplasia, parents brought six children for the purpose of preventive examination; at the time of examination, no complaints were made. However, 2 of them had a history of gait disturbance episodes, 4 had hip dysplasia, manifested by the late appearance of ossification nuclei of the femoral heads (after 8 months), which was the reason for the appointment of repeated radiography of the hip joints. Parents of three children paid attention exclusively to the rapid fatigue of the child in comparison with their peers. 20 patients complained of gait disturbance - from periodic limping to lameness.

Careful collection of anamnesis of the development of the child's motor activity from parents revealed episodes of gait disturbance or complaints of short-term pain syndrome 2-3 months before treatment, which the parents associated with "stretching" of the muscles.

During a clinical examination of children with Mayer's dysplasia, 11 patients were diagnosed with limited hip abduction by 30°-40°, and hypotrophy of the gluteal muscles and thigh muscles on the synovitis side was detected in 7 children. The growth of children was within the age norm.

Often, dysplasia of the femoral heads is asymptomatic and is detected by chance during examination of the abdominal organs.

On radiographs of the hip joints in children with Mayer's dysplasia in different age groups, late appearance of ossification nuclei was revealed, after 8-10 months. The cartilaginous part of the epiphysis of the femoral head is reduced in size, a delayed appearance of single or multiple ossification nuclei is noted, i.e. multicentric ossification of the epiphyses (blackberry type) was revealed, which is often mistakenly diagnosed as aseptic necrosis of the femoral heads (H. Taybi, R. Lachman, 1996). This type of dysplasia is not associated with joint effusion and hip ultrasonography shows a flat cartilaginous surface of the femoral head. Children with femoral head dysplasia did not need an unloading regimen and special treatment, however, they are a “risk group” for the development of aseptic necrosis of the femoral heads and dynamic monitoring is necessary until the end of skeletal growth with recommendations to limit jumping, long-distance running, and sports loads .

Often, epiphyseal dysplasia of the femoral heads is complicated by aseptic necrosis of the femoral head with a corresponding clinical picture. Lameness, pain in the hip or knee joint joins, after 7-10 days, hypotrophy of the muscles of the affected limb appears. Therefore, we conducted a differential diagnosis of Mayer's dysplasia with diseases such as multiple epiphyseal dysplasia, Legg-Calve-Perthes disease, reactive synovitis of the hip joint, mainly to prescribe the correct, adequate treatment.

To exclude multiple epiphyseal dysplasia, radiographs of the knee joints were taken, as the most informative areas in case of multiple lesions of the epiphyses of other joints. On radiographs of the knee joints with multiple epiphyseal dysplasia, the epiphyses were reduced in size with uneven contours, an uneven structure, and sometimes with phenomena of fragmentation along the periphery. Whereas with Mayer's dysplasia, the knee joints are externally and radiographically without pathological changes. Multiple epiphyseal dysplasia must be ruled out, since with systemic skeletal damage, prolonged immobilization of even individual joints is contraindicated.

An aspect that needs to be clarified is whether Mayer's dysplasia is a variant of multiple epiphyseal dysplasia or an independent form of dysplasia. When examining families of children with Mayer's dysplasia in 16%, only the pathology of the hip joints was revealed, and the defeat of the distal epiphyses of the thighs was never detected.

When conducting a differential diagnosis of Mayer's dysplasia and Perthes' disease, it should be remembered that Mayer's dysplasia is a disease of early age (usually up to 4 years), while Perthes' disease, according to different authors, is diagnosed after 4 years. In Perthes' disease, the head on the opposite side is round, of normal size, and in Mayer's dysplasia, both heads are reduced in height. According to the clinical course and effectiveness of treatment, Mayer's dysplasia and Perthes' disease differ from each other. With Mayer's dysplasia, the clinic is less pronounced than with Perthes' disease, long-term immobilization of the joint is not required (on average from 3 weeks, but not more than 6 months) and the restoration of the structure of the femoral head occurs in a shorter time.

A number of authors (Pedersen, 1960, Monty, 1962, Wamoscher & Farhi, 1963, J. Meyer, 1964, G.A. Harrison, 1971, P. Beighton, 1988) noted in their works that in some children with epiphyseal dysplasia of the femoral heads, it is possible to attach Legg-Calve-Perthes disease, or singled out these patients as "an atypical course of Legg-Calve-Perthes disease".

A certain place in the study of Perthes' disease is occupied by dysplasia in the broad sense of this term (O.L. Nechvolodova et al., 1996, I.V. Popov et al., 1998, M.I. Timofeeva et al., 1989, J. Batory 1982). A.I. Krupatkin (2003), V.D. Sharpar (2004) found that the main cause of ischemic disorders in osteochondropathy of the femoral head is general neurovascular dysplasia, possibly of congenital origin.

Differential diagnosis with reactive synovitis of the hip joint is based on the data of ultrasonography and radiography of the hip joints, reactive synovitis requires a sparing regimen for 4-6 weeks and anti-inflammatory therapy.

Algorithm for examining children with coxalgia

Table number 2

Differential diagnosis of Mayer's dysplasia

With Legg-Calve-Perthes disease.

Clinical and radiological manifestations

Perthes disease

Mayer's dysplasia without aseptic necrosis

Mayer's dysplasia with aseptic necrosis

The first symptoms of the disease are usually

over 4 years old

may be asymptomatic

under 4 years old

Gait disturbance

Pain in the hip joint

Pain in the knee joint

hip abduction restriction

Limitation of internal rotation of the hip

Hypotrophy of the muscles of the lower limb

Leg length asymmetry

USG of the hip joints

Multicentric ossification, smooth cartilage of the femoral head

Synovitis, aseptic necrosis of the femoral head

Radiography of the hip joints

Changes in the area of the anterior-upper pole of the femoral head in various variants (according to the staging of S.A. Reinberg).

The contralateral head is of uniform structure, with normal height and smooth contours.

Late appearance of ossification nuclei of the femoral heads, their asymmetry or multicentric ossification. Reducing the height of the heads by 1/3.

Aseptic necrosis of the femoral head in combination with reduced height. The contralateral femoral head is reduced in height.

All patients with Mayer's dysplasia were divided into 3 groups:

group 1 - children with diagnosed aseptic necrosis of the femoral head (15 people);

group 2 - children with clinical signs of gait disturbance and limitation of abduction and internal rotation within 10º, but without signs of aseptic necrosis of the femoral head on radiographs of the hip joints made in dynamics (9 children);

group 3 - children with multicentric ossification of the femoral head, diagnosed by X-ray and ultrasonography (7 people).

The treatment of children of the 1st group included unloading the limb, physiotherapy and drug treatment aimed at relieving the pain symptom, improving blood circulation in the hip joint area, to maintain joint function while maintaining the physiological tone of the muscles of the lower extremities and general muscle tone, therapeutic exercises and massage courses were performed. A sparing regimen with limited axial load was prescribed for an average of 6 months, with its gradual expansion. After stopping the pain syndrome, swimming and cycling were recommended.

In the second group, the children were prescribed a sparing regimen with the exclusion of axial load on the lower limbs for a month, a course of physiotherapy and drug treatment. After a month of unloading the joint, therapeutic exercises were recommended to strengthen the muscles of the thigh and gluteal muscles, massage, swimming, and cycling. The children were under dynamic observation in order not to miss aseptic necrosis of the femoral head and to control the development of the hip joints.

For the third group of children, dynamic observation is recommended until the end of skeletal growth, especially during periods of so-called physiological "jumps" in growth. They are recommended to exclude long-distance running, jumping from a height, but general physical training classes are mandatory.

The results of treatment of children with Mayer's dysplasia were assessed in 22 patients out of 31. In 9 patients, it was not possible to evaluate the results of treatment, because after the verification of the diagnosis and the appointment of treatment for a second appointment, they did not come. These are 5 children from group 2 - children without aseptic necrosis of the femoral head, but with gait disturbance, limitation of abduction and internal rotation of the hip within 7º and 4 children from group 3 - children with multicentric ossification of the femoral heads.

The follow-up period ranged from 6 months to 9 years.

The main task in the treatment of children with Mayer's dysplasia was to restore the anatomical shape of the femoral head and restore the function of the hip joint. The result of treatment was evaluated during a comprehensive examination of the functional state of the hip joint, which included:

Subjective signs that assess the patient's condition according to the parents;

Clinical signs (gait, hip function);

Instrumental studies (ultrasound, radiation methods of research).

According to the results of treatment, patients were divided into groups with good, satisfactory and unsatisfactory results of treatment.

"Good" the result was considered when, during the examination, the children did not complain, the parents were satisfied with the function of the limb, movement in the joints without restrictions. Repeated ultrasound examination revealed no signs of synovitis of the hip joint (in the presence of synovitis at the onset of the disease). Radiographs show signs indicating residual effects of Mayer's dysplasia - a decrease in the height of the femoral head up to 2-3 mm compared with the height of the femoral head in healthy children in the same age group. X-ray restoration of the femoral heads occurred within 12-20 months from the start of treatment.

This group included 11 children (4 children from the group of children with Mayer's dysplasia with aseptic necrosis of the femoral head; 4 children from the 2nd group, without aseptic necrosis, but with impaired gait; and 3 children from the group with multicentric ossification of the femoral heads).

The outcome of the treatment was considered "satisfactory" at absence of complaints from parents (absence of pain syndrome, rhythmic, correct gait), movement in the hip joint in full, or there is a limitation of abduction within 5°-7°, with a decrease in the height of the femoral head by 3-5 mm, compared with height of the femoral head in healthy children in the same age group, X-ray reconstruction of the femoral heads occurred more than 24 months after the start of treatment.

A "satisfactory" result was noted in 9 (41%) children, all children from group 1 - Mayer's dysplasia with aseptic necrosis of the femoral head.

3. " unsatisfactory» was considered the result of treatment with periodic deterioration of the clinic in the form of recurrence of pain within 1 year, limitation of abduction and internal rotation of the thigh. X-ray picture of the hip joints without positive dynamics within 6 months. The reasons for the unsatisfactory outcomes of treatment in this group of patients are non-compliance with the doctor's recommendations and a gross violation of the regimen.

An “unsatisfactory” result was found in 2 (9%) patients from the group of children with aseptic necrosis of the femoral heads. Monitoring of all patients continues, children are already attending school.

Conclusions.

1. Children with late appearance and reduced size of the ossification nuclei of the femoral heads are at risk for Mayer's dysplasia. They need dispensary observation of an orthopedist until the end of skeletal growth and obligatory observance of the orthopedic regimen, since children with Mayer dysplasia may develop early arthrosis of the hip joints.

2. For the eligibility of the diagnosis of "Mayer's dysplasia", it is necessary to exclude the typical form of multiple epiphyseal dysplasia.

3. Mayer's dysplasia, complicated by aseptic necrosis of the femoral heads, requires differential diagnosis with Legg-Calve-Perthes disease.

4. Uncomplicated forms of Mayer's dysplasia (without aseptic necrosis and with multicentric ossification of the femoral heads) actually do not need treatment and require dynamic monitoring in compliance with the orthopedic regimen.

5. In Mayer's dysplasia with aseptic necrosis of the femoral head, in contrast to Legg-Calve-Perthes disease, long-term immobilization of the hip joints is contraindicated.

With improper development of the bone epiphyses and their compaction, they speak of the development of a disease such as epiphyseal dysplasia. In the early stages of the disease, hardening of the hip joints is observed. In this case, diseases of the spine can join. The symptomatic range varies depending on the form of the disease, but in most patients, growth is reduced and the limbs are bent. To make an accurate diagnosis, the patient undergoes a comprehensive examination. In addition, 4 main types of the episiary form of the disease are distinguished, and Mayer's dysplasia is also considered separately.

Reasons for the development of the disease

The disease is considered congenital, since before and during pregnancy, the mother's body is affected by various factors that can affect the development of the fetus.
The appearance of epiphyseal dysplasia is associated with a genetic predisposition, when the disease is transmitted to the baby at the gene level.
With an increase in the level of progesterone in the last stages of pregnancy, the process of muscle formation of the fetus changes, which leads to destabilization in the ligamentous apparatus.
The disease manifests itself if several factors act on the mother's body at the same time (lack of useful trace elements (vitamins, minerals).
Close contact with the wall of the uterus, limiting the mobility of the legs of the fetus;
Signs of the disease are observed when the child is tightly swaddled.

Varieties

According to ICD 10, the disease is divided into subspecies depending on the level of its development and the symptomatic range. Only with the help of ultrasound, the doctor determines the type of epiphyseal dysplasia. At the same time, all forms of deviation are characterized by poor joint mobility, constant pain manifestations and curvature of the limbs. Most often, patients are diagnosed with four main degrees.

Fairbank disease

With Fairbank's disease, the child has short limbs with dwarf growth.

The first stage is referred to as autosomal dominant diseases. It is one of the most common forms of epiphyseal dysplasia. It develops as a result of changes in the COMP gene. With the disease, patients experience congenital dwarfism, in which the limbs and necks of the hips are shortened, the joints of the fingers move strongly, the vertebral bodies take on an oval appearance, and the bones of the wrist harden longer during development.

Dysplasia type 2

Epiphyseal dysplasia begins to develop in children aged 2.5 to 6 years. At the same time, small patients constantly suffer from pain manifestations that spread to the knee and ankle joints. The joints of the knees and epiphyses, located in all joints of the body, are also deformed and increase in size. The disease appears when the COL9A2 gene changes.

Type 3 dysplasia

The disease is not very different from the second type of dysplasia. It appears in childhood or adolescence. Patients have a small stature, short upper limbs. In the process of life, their knee joints are deformed, but there are no diseases of the spine. In addition, people with this form of epiphyseal dysplasia waddle around, sometimes feeling severe pain.

Type 4 dysplasia

In type 4 pathology, children are diagnosed with diabetes.

It is an autosomal recessive disorder. Due to changes occurring in the SLC26A2 gene, hereditary diseases appear that affect the musculoskeletal system. At the same time, the patient has diabetes mellitus, short stature, short limbs, pain in the joints. In a few patients, renal function is impaired in parallel, they are often injured.

Mayer's syndrome

The disease is not characterized by pronounced symptoms, if there are no complications in the head of the joints of the pelvis or thigh. During the examination, the little patient looks pretty good, and the height and weight indicators fit into the normal range. But when a small one walks, it is noticeable how he can limp or an abnormal gait is manifested, accompanied by pain.

In the initial stages, the development of the child may be accompanied by necrosis of the femoral head. Because of this, lameness is observed and the lower limbs hurt. Mayer's dysplasia requires a thorough medical examination and treatment so that complications do not appear.

Symptoms of the disease

A symptom of such a disease can be x-shaped legs.

The disease is characterized by high mobility of certain joints. Some species are characterized by damage to the organs and systems of the body, for example, problems with the eyes, endocrine system, etc. appear. As a complication, osteoporosis or skin atrophy may occur, and the following symptoms are also observed:

In patients, the legs are bent, acquiring an X-shape. This is due to deformities of the hip joint in children.
People have short stature, shortened limbs. Since childhood, a person has a reduced torso.
There is lameness when walking.

Diagnostic methods

When a baby is born, doctors examine the baby. If there is a suspicion that epiphyseal dysplasia is developing, a small patient is shown to an orthopedist. During the first 3 months of life, ultrasound is used to determine the disease. X-rays are performed less frequently, because during the examination the body is irradiated, and the X-ray method itself cannot show the condition of the cartilaginous head of the femur bone. As an additional study of the disease, the doctor may prescribe a computer or magnetic resonance imaging, as well as send for a consultation with a geneticist.

Epiphyseal dysplasia of the femoral heads - Mayer's dysplasia.

Chechenova Fatima Valerievna

Epiphyseal dysplasia of the femoral heads - Mayer's dysplasia

Dissertations for a degree

Candidate of Medical Sciences

Moscow - 2009

The work was performed at the Federal State Institution Central Research Institute of Traumatology and Orthopedics named after A.I. N.N. Priorov Rosmedtekhnologii

Scientific adviser:

Doctor of Medical Sciences Mikhailova Lyudmila Konstantinovna

Official opponents:

Doctor of Medical Sciences Kozhevnikov Oleg Vsevolodovich

Doctor of Medical Sciences, Professor Kuznechikhin Evgeny Petrovich

Leading organization: Federal State Institution "Moscow Research Institute of Pediatrics and Pediatric Surgery of Rosmedtekhnologii".

Address: 127299, Moscow, st. Priorova, 10.

The dissertation can be found in the library of the FGU CITO.

Scientific Secretary of the Council for the Defense of Doctoral and Candidate's Dissertations S.S. Rodionova

The relevance of the work.

J. Meyer (1964) believed that dysplasia of the epiphyses of the femoral heads initially manifests itself, and then, against its background, Legg-Calve-Perthes disease develops.

P. Maroteaux & Hedon (1981) wrote about 35 cases of isolated epiphyseal dysplasia of both hip joints in children under 6 years of age.

Purpose of the study - to develop a detailed clinical and radiological characteristics and differential diagnostic criteria for Mayer's dysplasia in the age aspect.

Research objectives:

1. Formulate an algorithm for examining children with coxalgia and dysfunction of the hip joint aged 1 to 7 years.

2. To study the possibilities of clinical, radiological and laboratory research methods in the diagnosis of Mayer's dysplasia.

4. Develop a set of therapeutic measures for Mayer's dysplasia. 5. To study the immediate results of treatment of children with Mayer's dysplasia.

Materials and research methods.

Scientific novelty.

It has been proven that during the growth of a child with Mayer's dysplasia, the proximal epiphyses of the femur remain reduced in height.

Practical significance.

Provisions of the dissertation submitted for defense.

Approbation of work.

Publications and information about introduction into practice.

Scope and structure of work.

Patients were examined clinically, radiologically and laboratory, the maximum follow-up period was 9 years.

HEAD EPIPHYSIS INDEX = a: b,

where a- longitudinal size of the femoral head (height), b- the transverse dimension of the head.

Fig.1. X-ray of normal hip joints

Table number 1.

The average size of the epiphysis of the femoral head in healthy children aged 1 to 7 years.

Longitudinal dimension (height) of the femoral head (mm)

Transverse head size

hip(mm)

Femoral head epiphysis index (IE)

With age, this index of the epiphysis of the femoral head increases, while with Mayer's dysplasia in each age group, the index of the epiphysis is less than in the same group in healthy children.

After a clinical and radiological examination of 278 patients, we identified a group of children with Mayer's dysplasia - 31 (11.2%) people, including 23 boys and 8 girls.

Often, dysplasia of the femoral heads is asymptomatic and is detected by chance during examination of the abdominal organs.

Algorithm for examining children with coxalgia

Table number 2

Differential diagnosis of Mayer's dysplasia

With Legg-Calve-Perthes disease.

Clinical and radiological manifestations

Perthes disease

Mayer's dysplasia without aseptic necrosis

Mayer's dysplasia with aseptic necrosis

The first symptoms of the disease are usually

over 4 years old

may be asymptomatic

under 4 years old

Gait disturbance

Pain in the hip joint

Pain in the knee joint

hip abduction restriction

Limitation of internal rotation of the hip

Hypotrophy of the muscles of the lower limb

Leg length asymmetry

USG of the hip joints

Multicentric ossification, smooth cartilage of the femoral head

Synovitis, aseptic necrosis of the femoral head

Radiography of the hip joints

Changes in the area of the anterior-upper pole of the femoral head in various variants (according to the staging of S.A. Reinberg).

The contralateral head is of uniform structure, with normal height and smooth contours.

Late appearance of ossification nuclei of the femoral heads, their asymmetry or multicentric ossification. Reducing the height of the heads by 1/3.

Aseptic necrosis of the femoral head in combination with reduced height. The contralateral femoral head is reduced in height.

All patients with Mayer's dysplasia were divided into 3 groups:

group 1 - children with diagnosed aseptic necrosis of the femoral head (15 people);

group 3 - children with multicentric ossification of the femoral head, diagnosed by X-ray and ultrasonography (7 people).

The follow-up period ranged from 6 months to 9 years.

Subjective signs that assess the patient's condition according to the parents;

Clinical signs (gait, hip function);

Instrumental studies (ultrasound, radiation methods of research).

According to the results of treatment, patients were divided into groups with good, satisfactory and unsatisfactory results of treatment.

A "satisfactory" result was noted in 9 (41%) children, all children from group 1 - Mayer's dysplasia with aseptic necrosis of the femoral head.

Conclusions.

2. For the eligibility of the diagnosis of "Mayer's dysplasia", it is necessary to exclude the typical form of multiple epiphyseal dysplasia.

3. Mayer's dysplasia, complicated by aseptic necrosis of the femoral heads, requires differential diagnosis with Legg-Calve-Perthes disease.

5. In Mayer's dysplasia with aseptic necrosis of the femoral head, in contrast to Legg-Calve-Perthes disease, long-term immobilization of the hip joints is contraindicated.

Dystrophy of bone and cartilage tissue during the period of embryogenesis provokes the development of dysplasia. This is a congenital anomaly recorded in patients of different age categories. In pediatrics, hip dysplasia (dysgenesis, DTS) is one of the most common pathologies. Approximately 60% of cases revealed dysplasia of the left hip joint. What is it connected with?

The fact is that the left limb of the fetus, as a rule, is pressed against the wall of the uterus. Right joint dysgenesis is diagnosed in about 20% of cases. Bilateral dysplasia in adults is a very rare occurrence. The female sex is more susceptible to the development of the disease.

As a rule, hip dysplasia is a congenital anomaly, however, sometimes this ailment can manifest itself not only in children, but also in adults. Against the background of the development of the disease, there is a limitation of the functions of the affected limb.

If your baby has been diagnosed with hip dysplasia, don't despair - it's still possible to change. Remember that only early detection and proper treatment will help to avoid complications!

Children's dysplasia is dangerous because, with illiterate or untimely treatment, secondary symptoms of the disease are formed, namely:

curvature of the spine (lordosis, kyphosis);
violation of the anatomical shape of the pelvis;
osteochondrosis;
subluxations and dislocations of the opposite joint;
dysplastic coxarthrosis.

All of the above complications can lead the patient to disability.

Hip dysplasia is a common malformation that threatens the normal life of a child from the very first days of life.

Hip dysplasia is a pathology that requires immediate treatment, since there is a high risk of patient disability

Classification of joint dysplasia

Taking into account the anatomical structure of the damaged organ, the following types of TDS are distinguished:

epiphyseal (Mayer);
acetabular (articular fossa);
rotary.

Mayer's dysgenesis

Epiphyseal dysplasia is characterized by punctate ossification of cartilaginous tissues. Dystrophic processes provoke the development of pain and deformity of the legs. Pathological changes are detected in the proximal part of the thigh. Epiphyseal TDS is classified into several types:

type I Fairbank;
multiple, type II;
multiple, type III;
multiple, Walcott-Ralshson type;
multiple with myopia and conductive hearing loss;
familial epiphyseal type Bakes;
macroepiphyseal with osteoporosis;
epiphyseal hemimelic.

Acetabular dysgenesis

Acetabular hip dysplasia affects the articular cavity. Pathology manifests itself in several stages. First, a pre-dislocation develops, in which the femur is set aside from the acetabular fossa by a small distance. Further, subluxation develops, in this case the distance increases, and, finally, dislocation - here the epiphyseal part of the thigh is already completely out of the glenoid fossa.

Residual childhood dysplasia is observed with involuntary self-reduction of a dislocated joint. This phenomenon is sometimes recorded in adult patients; in women, pregnancy or childbirth can act as a catalyst for this process.

Rotational dysgenesis

There is a deviation from the norm in the location of the bone relative to the articular fossa. All of the above factors provoke the development of clubfoot. There are three degrees of joint damage:

predislocation (I degree);
subluxation (II degree);
dislocation (III degree).

Light form

Congenital predislocation is characterized by underdevelopment of cartilage and bone tissue. It should be noted that the ligamentous-muscular apparatus is not involved in the pathological process. This allows him to securely fix the femoral epiphysis in an anatomically correct position, that is, in the articular cavity.

Middle form

At this stage, the synovial capsule is stretched, which contributes to the displacement of the head of the hip joint. Thus, the epiphysis of the femur comes out of the glenoid fossa.

Severe form

A serious pathology characterized by complete prolapse of the femoral epiphysis from the articular cavity. Without appropriate therapy, the pathology progresses, which leads to a significant stretching of the joint capsule, the cavity of the acetabular fossa is gradually filled with adipose and connective tissue. Thus, the so-called pseudo or false joint is formed.

The main causes provoking the development of the disease

There is an opinion among the people that dysplasia is the result of the negligence of health workers who take birth (gynecologists, obstetricians, orderlies), during which they could injure the limbs of a newborn baby. This is not a correct opinion, because joint dysplasia is a polyetiological congenital pathology, therefore its development can be provoked by various endo- and exogenous factors. The main ones include the following:

childbirth in old age;
infectious pathologies of the mother during pregnancy;
genetic predisposition;
dysfunction of the endocrine glands in the mother;
birth and postpartum trauma;
harmful working conditions of the mother;
protracted childbirth;
large size of the fetus;
technogenic zone (unfavorable ecological situation);
medical preservation of the child with the threat of miscarriage;
rapid childbirth;
taking certain drugs during the period of bearing a baby;
intrauterine entanglement with the umbilical cord and breech presentation of the fetus;
elevated levels of the hormone progesterone in the third trimester of pregnancy;
gynecological pathologies in a pregnant woman;
the weight of the born baby is up to 2500 g;
multiple births;
underdevelopment of the spinal cord and spinal column;
sharp "pulling out" of the baby during caesarean section;
gestosis of the first half of pregnancy.

There are a number of predetermining factors that provoke the development of the disease:

lack of mineral elements (phosphorus, selenium, magnesium, calcium, iron, iodine, zinc, cobalt, manganese) and vitamins (tocopherol, retinol, thiamine, riboflavin) in the mother's diet;
oligohydramnios and hypertonicity of the myometrium;
female baby.

In some cases, childhood dysgenesis is combined with other pathologies of embryonic development (myelodysplasia, hydrocephalus, etc.).

Symptoms

Dysplasia in babies is detected in the perinatal period. The earliest sign of childhood dysgenesis is hip abduction restriction. This pathology can only be diagnosed by a highly qualified specialist. If the baby is healthy, then the abduction of the joints will be complete. The asymmetry of the folds on the hips of the baby is the second symptom of the disease, which you need to pay attention to. Obvious manifestations of the disease can also include a symptom of shortening of one limb of the baby in relation to the other.

When examining a child, a "click" symptom may also be detected, when the epiphyseal part of the thigh pops out of the articular cavity. Dysplasia in newborns should be diagnosed in the early stages of its development. With timely therapy, this disease disappears by 6-8 months. Additional symptoms of TDS include:

torticollis;
unconditioned reflexes are weakly expressed;
softness of the bones of the skull;
varus or valgus setting of the foot.

The characteristic signs of the disease in children older than a year are:

lameness on a sore leg;
"duck" gait;
increase in lumbar lordosis;
clinical manifestations identified in the postembryonic period are more pronounced.

If the above symptoms are found, the child must be shown to a qualified doctor.

Diagnostic methods

On the basis of only the above clinical manifestations, it is impossible to make an accurate diagnosis of hip dysplasia. For this, it is necessary to conduct additional research methods: radiography, ultrasonography, computed, magnetic resonance imaging. It is somewhat more difficult to diagnose the pathology presented above in babies older than three months of age, since the only obvious symptom can only be less mobility of the affected joint.

Sonography is the basic method for diagnosing childhood dysgenesis. X-ray diagnostics is used less frequently. Using ultrasound, you can monitor the effectiveness of treatment. Magnetic resonance or computed tomography is used when there is a question about the operation. Gives a more complete, structured picture. Arthrography and arthroscopy are invasive diagnostic methods, so they are not so popular these days.

How to differentiate dysgenesis

TDS must be differentiated from the following pathologies:

English disease (rickets);
injuries and fractures;
congenital viral deformity of the femoral necks;
pathological and paralytic dislocation of the hip after paralysis;
arthrogryposis;
tumors of the femoral neck;
chondrodystrophy;
osteomyelitis;
physiological and spastic hypertonicity of the thigh muscles;
chondrodysplasia and epiphysiolysis of the femoral head of various etiologies;
deformity after Perthes disease;
epiphyseal osteodysplasia.

Dangerous consequences of pathology

An undiagnosed or untreated hip dysplasia in an infant can cause many problems for the patient in adulthood. As a rule, patients with such a pathology develop coxarthrosis of the hip joint. This disease is accompanied by the development of necrotic processes in the epiphysis of the thigh, joint atrophy, unbearable pain, localized in the joints. Ultimately, the disease for the patient often ends in disability.

Therapeutic methods for eliminating the disease

If the doctor confirms the diagnosis of childhood hip dysgenesis, treatment is started immediately. With the insufficiency of therapeutic measures with the growth of the child, the progression of the disease is observed. Over time, a mild form of dysplasia is complicated by subluxation and dislocation. Parents will have to be patient, as the treatment of childhood dysgenesis is a rather lengthy and exhausting process. The effectiveness of therapy largely depends on the degree of damage to the organ, and, of course, the age of the patient himself.

Conservative methods of therapy

In the early stages of the development of the disease, doctors prescribe wide swaddling, physiotherapy and physiotherapy exercises. The complex of recreational gymnastics contributes to the gradual formation of the articular fossa. As you can see, therapy is aimed at fixing the joints in an anatomically correct position. In addition, pediatricians also recommend exercise therapy. Beneficial swimming. Exercise should not be carried out in a crib, but on a hard surface.

Good results are achieved when prescribing to a baby:

mud therapy;
amplipulse therapy;
hyperbaric oxygenation;
ultrasound;
acupuncture;
manual therapy;
massage;
electrophoresis;
magnetic laser therapy.

Previously, plaster casts were used to correct dysplasia, as well as rigid orthopedic structures. In the treatment of children in the first months of life, only soft elastic constructions are used. Modern medicine for the treatment of childhood dysgenesis recommends the use of a variety of orthopedic techniques and devices: Becker's panties, Volkov's, Vilensky's abduction splints, Freik's pillows, Pavlik's stirrups.

Correctly chosen tactics of functional treatment of dysplasia in neonatal and infancy can significantly reduce the treatment time, avoid possible complications and adapt the child to early axial load.

Operative methods of treatment of pathology

Surgery is indicated in the following cases:

ineffective conservative treatment;
late diagnosis of the disease (in this case, minimally invasive methods of treatment are unlikely to be beneficial).

The choice of surgery depends on many factors. Perform open reduction of dislocation, reconstruction of the acetabulum, tenotomy, osteotomy of the pelvis. The later the surgical intervention is carried out, the greater the risk of disability, and the less likely the 100% cure.

Forecast

With timely prescribed therapy (children under 3 months), the prognosis is conditionally favorable. It is possible to achieve 100 percent recovery without surgical intervention in most cases.

Correctly holding a newborn baby in your arms is a very important point. The baby should clasp the legs of an adult. Health workers do not recommend carrying the baby sideways. To ensure the correct position of the child, you can purchase a special device - a sling carrier.

In the process of changing clothes, the baby needs to perform short sessions of recreational physical education. Before and after exercise, you need to perform a light relaxing massage.

You can do the following exercises at home:

breeding of the lower extremities in the prone position;
rotation of the baby's hips with light pressure on the knees;
abduction of bent legs.

Prevention of dysplasia

Prevention of joint dysgenesis is aimed at preventing severe pregnancies, since lesions associated with disorders of early intrauterine development are the least susceptible to therapeutic effects. In this regard, the expectant mother during the period of bearing a child should monitor the diet and pay increased attention to her health. It is very important to listen to the advice and recommendations of the gynecologist who will manage the pregnancy and plan the birth.

During pregnancy, the expectant mother should include in her diet foods rich in minerals and vitamins (fruits, vegetables, dietary meat, nuts, cottage cheese, cabbage, cheese, eggs, fish).

Women with congenital pathology of the joints are recommended to have a caesarean section, that is, childbirth should take place in the most gentle mode.

For optimal development of the musculoskeletal system, orthopedists do not recommend swaddling newborns. In countries (Germany, the United States of America, France, Africa, Great Britain, Italy, etc.), in which it is not customary to make tight swaddling, congenital anomalies of the joints are practically not recorded.