Foot deformities in systemic diseases: causes, symptoms, diagnosis, treatment

Deformities of the feet are characteristic manifestations of systemic diseases of the musculoskeletal system (SZODA).

ICD-10 codes

• Q77.5 Dystrophic dysplasia.
• Q77.7 Spondyloepiphysar dysplasia.
• Q77.8 Other osteochondrodysplasia with growth defects of tubular bones and spine.
• Q77.9 Osteochondrodysplasia with growth defects of tubular bones and spine, unspecified.
• Q79.6 Ehlers-Danlos Syndrome.

With multiple epiphyseal dysplasia, pseudoahondroplasia, late spondyloepiphysic dysplasia, congenital functionally significant deformities are rare.

With age, contractures increase due to deforming arthrosis of the ankle and foot joints, valgus or supination deformations are formed, the epiphyses flatten out. Conservative treatment includes massage, exercise therapy, physiotherapy treatment, medical therapy of arthrosis, orthopedic laying, use of orthoses, orthopedic footwear. With persistent deformities that cause functional disorders, surgical treatment is indicated.

With the syndromes of Ehlers-Danlos, Larsen, congenital spondyloepimetaphyseal dysplasia, congenital spondyloepiphysic dysplasia, dystrophic dysplasia, congenital deformities of the feet are observed in most patients. They are usually stiff and more pronounced than with isolated orthopedic pathology. Common for them - progressive bone deformities. High frequency of dislocations and subluxations in the joints of the foot, with epiphyseal dysplasia - flattening of the epiphyses. Typical acute osteoporosis, degenerative-dystrophic changes, rapidly growing during immobilization, the use of the Ilizarov apparatus. Atypical attachment of the tendon of the anterior tibial muscle to the diaphysis of I metatarsal bone, which provokes the development of relapse of anterior division, was noted in 30% or more patients. However, the phenotypic polymorphism of SZODA is extremely wide, there are children with minimal clinical manifestations and generally without deformities of the feet. Deformations with different DSM differ significantly not only from non-systemic, but also from each other and require a different approach to treatment. In this case, priority is given not to restoring the disturbed anatomical relationships and functions, but to restoring the foot support.

Clubfoot in dystrophic dysplasia

Characteristic signs.

• Underdevelopment of the fork of the ankle joint, discrepancy of its size with the dimensions of the talus bone (Fig. 110-9).
• Anterior subluxation (dislocation) in the ankle joint.
• Additional wedge-shaped bones, additional nuclei for their ossification; an increase in the size and deformation of the I sphenoid bone.
• Dislocation in I cuneate-metatarsal joint (up to 30%).
• Varus stop, caused not only by subluxations in the joints of Chopar and Lisfranc, but also by the progressive varus deformity of the metacarpal shafts with their thinning.
• Deltoid deformity of I metatarsal bone, thickening of the main phalanx of the 1st finger.
• Brahifalangia, symphalangism, clinodactyly.
• With age, subluxations and dislocations appear in the metatarsophalangeal joints and progress.

Treatment

Conservative treatment of severe deformities is ineffective. To keep the talus bone in the ankle fork, it is not possible to fix the dislocation in the I metatarsal-wedge joint. When using gradual cast plaster, aseptic necrosis of the talus is frequent.

With relatively easy deformations, early tenoligamentocapsulotomy is shown with the obligatory correction of the dislocation in the I metatarsophalangeal joint, modeling resection of the oblique joint surface of the first sphenoid bone, the movement of the tendon of the anterior tibial muscle. With more severe deformities, tenoligamentaktapsulotomy should be combined with the use of Ilizarov's apparatus, which postpones the possibility of performing it until the age of 2-2.5 years. Expressed bone deformities force to resort to early resections of the foot bones. Immobilization after surgery is carried out in the position of hypercorrection by 15-20 °. With anterior dislocation in the ankle and deformities of the talus, the operation of choice is early astragalectomy. To prevent relapses, in all cases corrective lavsanoplasty of ligaments along the external surface of the foot is shown. From the age of 9-10 years, it is advisable to use early arthrodesis of the subtalar joint and the joint of Chopar.

Clubfoot with Larsen's syndrome

Characteristic signs.

• The fusion of two nuclei, from which the heel bone is formed in the postnatal period with the outcome in its moderate deformation.
• Additional bones are tarsal and points of their ossification.
• Subluxations and dislocations in scaphoid-wedge-shaped and interclinical joints, Lisfranc's joint, not characteristic of other SZODA. The formation of a cavus deformation occurs due to a dislocation in the joint of Lisfranc.
• Lopatoid deformation of the distal phalanx of the 1st finger.
• Dislocations in the metatarsophalangeal joints.

Treatment

Early conservative treatment is effective in mild forms, without dislocations in the mid-foot joints, but increased risk of secondary deformities and aseptic necrosis of its bones. At an abnormal attachment of the anterior tibial muscle (detect with ultrasound) and dislocations, surgical treatment is indicated, in severe cases - using the Ilizarov apparatus. To prevent the recurrences of dislocations typical for Larsen syndrome after repositioning, stabilization of the joints with the formation of artificial ligaments is necessary.

Congenital spondyloepiphysic dysplasia, congenital spondyloepimetaphyseal dysplasia

With VSED and VSEMD, heavy and rigid clubfoot, congenital coarse bone deformities, rapidly progressing with age, are possible. Characteristic changes in the metaphysis of short tubular bones of the foot. However, in general, the distal parts of the skeleton are less affected by these diseases than the proximal ones. In some forms (pseudo-Morkio syndrome), there is a very sharp shortening of the metatarsal bones and phalanges of the fingers, the feet become square.

Treatment

Conservative treatment of gross deformities of the foot bones is not effective. When surgical treatment is combined TLCT, Ilizarov apparatus, modeling bone resection and transplantation of the anterior tibial muscle. Stabilization of the achieved result is shown by the formation of artificial ligaments, with deformities of the talus bone - astragalectomy. Lighter, functionally minor, deformations of the feet do not require surgical correction.

Clubfoot in the Ehlers-Danlos syndrome

A characteristic feature is the combination of rigid deformations in some joints of the foot and hypermobility in others. With multiple lesions of the joints, the clubfoot resembles arthrogryptic.

Treatment

Step plaster casts should be used from the first weeks of life. However, at the same time, there is a high risk of developing a secondary rocking foot, which can develop without treatment on the background of a static load with undisturbed deformation. As a result of this, plastering according to the Ponseti technique with surgical correction of the equinus component was shown. The first signs of the formation of secondary deformations require the cessation of conservative therapy and the conduct of surgical treatment. With pronounced hypermobility to lengthening of tendons, they are dosed, in some cases refuse capsulotomies, and postoperative immobilization is performed strictly at the middle position of the foot. Due to the inferiority of connective tissue, the risk of post-urticaria neuropathy, postoperative hematoma, is increased.

Foot-rocking chair with syndrome zelosa-danlos

The rocking-out foot practically does not succumb to conservative therapy. Stage plaster bandages (from the first month of life) allow to partially correct the withdrawal and rear flexion of the forefoot, reduce the tension of soft tissues and the risk of their necrosis in the course of early surgical treatment. It is shown either a one-stage open correction of the talus bone, or with the heaviest deformations the use of Ilizarov's apparatus. The risk of developing secondary deformities with a pronounced heel component is increased, which requires dispensary observation of the patient with examination at least once every 3 months, and correction of orthopedic footwear.

Stopped foot

Deformation is met mainly in the lightest subclinical variants of SZODA. Conservative treatment of it is shown in the absence of dislocations in the tarsal joints, atypical attachment of muscles and gross bone deformities. In other cases, early surgical treatment is indicated.

Taking into account the comparative rarity of the given foot in the SZODA and also its complexity and originality, which require surgical correction, the treatment is carried out in specialized institutions that have experience in the treatment of such patients. All patients before and after the operation are shown drug treatment of osteoporosis, use of chondroprotectors, angioprotectors, drugs that improve tissue metabolism. After the end of the gypsum immobilization and before the end of the foot growth, the use of orthopedic footwear, regular massage courses, exercise therapy, physiotherapy and spa treatment are shown.

Clinical follow-up is carried out with frequent examinations - at least 1 time in 6 months. This is especially important in hypermobile syndromes. When resolving the issue of surgical treatment of relapses, first of all, the connection of the functional insufficiency of the patient with deformations of other parts of the musculoskeletal system is taken into account. Moderate relapses, which do not cause significant difficulties in movement, as a rule, are not subject to prompt correction. It is completely impossible to cure a patient with SZODA. However, adequate treatment can largely correct the existing deformities and improve the patient's quality of life.

[1], [2], [3], [4], [5], [6]