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PubMed1990

Established hip dislocations in children with cerebral palsy.

Hip dislocation in children with cerebral palsy is caused by a combination of factors, including spastic muscle imbalance, persistent fetal femoral geometry, acetabular dysplasia, and flexion-adduction contracture. The incidence of dislocation correlates with the severity of the spasticity, and the prevalence is close to 50% in neurologically immature, spastic quadriplegic children. Successful hip reductions improve muscular balance, provide satisfactory reduction of the femoral head, and establish good pelvic coverage. In 31 occurrences of established hip dislocation in 24 patients, the most successful operations used a combined procedure consisting of soft-tissue release, open reduction, femoral varus derotation and shortening osteotomy, and pelvic osteotomy.

Cerebral Palsy↗
PubMed2004

Sensory hypoinnervation in club foot.

We have compared the density of nerve fibres in the synovium in club foot with that of specimens obtained from the synovium of the hip at operations for developmental dysplasia. The study focused on the sensory neuropeptides substance P; calcitonin gene-related peptide; protein gene product 9.5, a general marker for mature peripheral nerve fibres; and growth associated protein 43, a neuronal marker for new or regenerating nerve fibres. In order to establish whether there might be any inherent difference we analysed the density of calcitonin gene-related peptide-positive nerve fibres in the hip and ankle joints in young rats. Semi-quantitative analysis showed a significant reduction in the number of sensory and mature nerve fibres in the synovium in club foot compared with the control hips. Calcitonin gene-related peptide (CGRP) positive fibres were reduced by 28%, substance P-positive fibres by 36% and protein gene product 9.5-positive fibres by 52% in club foot. The growth associated protein 43-positive fibres also seemed to be less in six samples of club foot. No difference in the density of CGRP-positive nerve fibres was observed in the synovium between ankle and hip joints in rats. The lack of sensory input may be responsible for the fibrosis and soft-tissue contractures associated with idiopathic club foot.

Animals↗
PubMed2005

Management of the bilateral congential dislocation of the hip and knee: a case report.

Congenital dislocation of the knee (CDK) is a rare disease and may be associated with other congenital and musculoskeletal disorders. We report our result in the treatment of a patient with CDK and DDH that treated with serial casting and Pavlik harness. Early diagnosis of the CDK is very important non operative treatment usually provides more stabile, greater range of motion and much more quadriceps strength than the surgical treatment. Early reduction of dislocation prevents formation of the knee contracture.

Casts, Surgical↗
PubMed1988

Risk factors associated with immobility.

In nursing homes, immobility and related complications are major problems with profound health care and financial implications. We conducted a retrospective study to identify risk factors associated with immobility. We compared factors in 34 nonambulatory residents with those in 12 independent ambulatory residents who served as controls. Factors associated with immobility included contractures, severe dementia, poor vision, and history of hip/leg fractures. Factors not associated with immobility included age, osteoarthritis, mild to moderate dementia, weight gain, and broad categories of selected medications. The immobile patients were further analyzed by subgrouping into those with and without contractures. Contractures were significantly associated with severe dementia. The finding that immobility was not identified on problem lists for 85% (29) of the immobile residents and that for 29% (10) reasons for immobility could not be ascertained suggest that immobility is frequently not documented as a major problem in medical records nor adequately evaluated.

Activities of Daily Living↗
PubMed2001

[Bone and joint revision surgery after limb salvage procedure of malignant bone tumor].

OBJECTIVE: To analyze the indications for revision surgery after limb salvage procedure of malignant bone tumor and summarize the experiences in revision surgery. METHODS: From January 1994 to December 1997, 8 cases were re-operated after primary limb salvage procedure. The average survival period with no-tumor occurrence was 8 years. The common causes for the revision were traumatic osteoarthritis, fracture, and bone resorption. The main difficulties in revision were soft tissue contracture and limb discrepancy from limb shortening. RESULTS: In this study, there was total hip replacement in 1 case, large segmental allograft for reconstruction of distal femur in 3 cases, total knee replacement upon composite of previously transplanted allograft in 3 cases, removing of intramedullary nail and re-internally fixed with intramedullary nail in 1 cases. The isotopic bone scan before the revision showed active bone metabolism in all 4 transplanted segmental allograft. The pathologic study of the transplanted allograft after revision confirmed new bone formation in allograft. The revision procedure reduced the pain, and improved the limb function. CONCLUSION: The main causes of revision surgery after limb salvage procedure of malignant bone tumor are fracture of transplanted allograft segment or devitalized tumor segment, and poor function of the affected joint. Constrained knee prostheses with rotating hinges or semi-constrained ball-axis resurfacing knee prostheses improve the function of knee joint postoperatively.

Adult↗
PubMed1986

Pelvic dysplasia associated with arthrogrypotic changes in the lower extremities. A new syndrome.

Two sisters exhibit arthrogrypotic changes in the lower extremities associated with a previously undescribed pelvic dysplasia. The pelvic dysplasia is characterized by: severe abnormalities of ossification with markedly widened triradiate cartilages; irregular, notched acetabulae; marked hypoplasia of the ilia; notching of the iliac wings; and delayed ossification of the capital femoral ossific nucleus. The elder girl has bilateral clubfeet, as well as flexion contractures of both knees. The younger girl has a right talipes equinovarus and left hip dislocation. Autosomal recessive inheritance is suggested. This distinctive syndrome should be considered in the differential diagnosis of the patient with arthrogryposis.

Arthrogryposis↗
PubMed2006

Muscle involvement and motor function in amyoplasia.

The most common form of arthrogryposis multiplex congenita (AMC) is amyoplasia. Diagnostic criteria are highly specific, with decreased muscle mass, typical contractures and limb positioning at birth. Intellectual development is normal. The aims of this study were to investigate how muscle strength and contractures affect motor function in amyoplasia, and to relate current functional status to joint positions at birth. Medical records were reviewed and a clinical examination was performed, with investigation of muscle strength, range of motion, and motor function. Thirty-five patients with amyoplasia participated and were divided into three functional groups: 11 community ambulators, 11 household ambulators, and 7 non-ambulators. Six children less than 2 years old were not categorized. Community ambulators had the best muscle strength and none had knee flexion contractures extending 20 degrees. Household ambulators had severe contractures in the lower limbs, but good muscle strength in the upper limbs. Non-ambulators had most severe contractures and most severely reduced muscle strength. Most of the non-ambulators were born with hips in severe abduction, flexion and external rotation. Good and very good correlations were found between muscle strength and motor function, and only moderate correlations between range of motion and motor function. We conclude that more attention should be paid to developing muscle strength, with early stimulation of active movement, and that periods of immobilization should be minimized. Further, ultrasound or MRI of muscle tissue in the newborn period would be useful to evaluate prerequisites for future development of muscle strength and thereby anticipate response to therapy.

Adolescent↗
PubMed2001

Factors influencing ambulation in myelomeningocele: a cross-sectional study.

A consecutive series of 53 children with myelomeningocele (mean age 7.6, range 3.2 to 11.4 years) was assessed in order to see if the children with motor paresis of the lower limbs achieved the expected level of ambulation, and if not, to identify possible causative factors. Methods used were clinical examination of orthopaedic and neurological status, information from medical reports, and documentation of orthoses use. Functional skills were documented and energy expenditure was examined. Thirty-one of 53 children had reached the expected ambulation considered possible according to their motor paresis, whereas 22 of the 53 performed worse than expected. Balance disturbances, occurrence of spasticity in knee and hip joints, and number of shunt revisions made differed significantly between the groups that achieved and did not achieve expected ambulation. Functional skills of mobility differed significantly between two muscle-function levels in children who had walking ability. Energy expenditure was higher in the non-achieving group than in the group who achieved expected ambulation in each of the muscle-function levels. Results show that children with similar muscle paresis exhibit different ambulatory function. This indicates the importance of a close analysis of other factors which may cause ambulation to deteriorate in order to predict future ambulation in children with myelomeningocele.

Child↗
PubMed1988

A syndrome of multiple congenital contractures: neuropathological analysis on five fetal cases.

We performed a neuropathological study on 5 fetuses with an autosomal recessive, lethal syndrome of congenital contractures diagnosed by fetal hydrops on ultrasonography. The fetuses showed a typical pattern of malpositioning of hips and knees with occasional pterygia of the neck and elbows. The muscles were hypoplastic and the spinal cords showed severe thinning, most markedly affecting the ventral half. A total loss of axons in the ventral and lateral funiculi, subtotal loss of anterior horn motor neurons with accompanying astrocytosis and astrogliosis, and similar but less severe changes at the brain stem level suggested a degenerative rather than a dysmorphogenetic mechanism. Sensory nuclei and pathways were distinctly less severely affected, if at all. The findings further delineate this condition as a genetically and pathoanatomically distinct autosomal recessive syndrome.

Abnormalities, Multiple↗
PubMedDate not supplied

Soft-tissue release for spastic hip subluxation in cerebral palsy.

Children with spastic hip subluxation secondary to cerebral palsy were treated with a standard protocol that focused on early detection of the subluxation using physical examination and anteroposterior pelvis radiographs. Using limited hip abduction of < or =30 degrees and subluxation of > or =25% migration percentage as indications, patients had open adductor and iliopsoas lengthenings with immediate postoperative mobilization and no abduction bracing. The protocol was applied to 74 children with a mean age of 4.5 years and had 147 hips surgically addressed. Of these hips initially, 20% were normal (migration percentage <25%), 52% were mildly subluxated (migration percentage 25-39%), 22% were moderately subluxated (migration percentage 40-59%), and 6% were severely subluxated (migration percentage > or =60%). At a final postoperative follow-up of 39 months, 54% of these hips were classified as good (migration percentage <25%), 34% were fair (migration percentage 25-39%), and 12% were poor (migration percentage > or =40%). Of this patient population, 69% were nonambulators and their outcomes were not statistically different from children who could walk. No child developed an abduction contracture or wide-based gait that required treatment. With early detection and applying this treatment algorithm, 80% of children with spastic hip disease should have good or fair outcomes. Longer follow-up will be required to determine how many children will need bony reconstruction to maintain stable and located hips at the conclusion of growth.

Cerebral Palsy↗
PubMed1994

Sonography and intracapsular pressure in Perthes' disease. 39 children examined 2-36 months after onset.

39 children with Legg-Calvé-Perthes Disease (LCPD) and with a radiographically unsatisfactory development were examined with sonography regarding synovitis and with intracapsular pressure recording and aspiration performed 15 (2-36) months after the onset of symptoms. The mean anterior sonographic capsular distension was 3.0 (1.0-7.0) mm greater than that of the contralateral, asymptomatic hip. The mean intracapsular pressure was 4.5 (0-11.5) kPa with the hips in extension and neutral rotation, 9.7 (1.3-27.3) kPa with the hips in extension and inward rotation and 0.9 (-0.8-4.7) kPa in 45 degrees of flexion. We conclude that these children have synovitis as diagnosed sonographically. This synovitis is probably symptomatically and prognostically important in LCPD due to increased intracapsular pressure, with pain, a decreased range of motion and, potential joint contracture.

Child↗
PubMed1979

Juvenile fibromatosis and other diseases of connective tissue.

There are a number of features which are common to such variable conditions as juvenile fibromatosis on the one hand and torticollis and congenital dislocation of the hip, and possibly scoliosis, on the other. Various pathological syndromes are presented, all of them characterized by disturbance and contracture of connective tissue. Surgery is carried out in severe cases when it is a practicable proposition. The administration of an enzyme inhibitor at cellular level has introduced a new dimension in the management of localized fibromatosis.

Birth Injuries↗