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[Spasm of the adductor muscles, pre-dislocations and dislocations of the hip joints in children and adolescents with cerebral palsy. Clinical observations on aetiology, pathogenesis, therapy and rehabilitation. Part II. The importance of the iliopsoas tendon, its tenotomy, of the coxa valga antetorta, and correction through osteotomy turning the hip into varus (author's transl)].

The following factors besides spasm and contraction of the adductor muscles contribute to the occurrence of dislocations of the hip in spastic paralysis: Spasm and contraction of the iliopsoas muscle and enhanced valgus position and antetorsion. The author holds the opinion that in case of malformation of the proximal end of the femur, it is not only the indirect action of the spastic musculature via the proximal femur-epiphyseal cartilage which is responsible for this phenomen in accordance with the law on functional adaption through longitudinal growth (Pauwels), but also the direct traction of the iliopsoas tendon. A clue in this direction is the often very pronounced elongation or enlargement of the trochanter minor. The author demonstrates the pathogenetic importance of iliopsoas contracture and malpositioning of the neck of the femur by means of analyses of the course in two patients. The following principles of treatment are postulated for spastic dislocation of the hip: Elimination of the pathogenetic factors through myotenotomy of the adductor muscles and complete resection of the obturator nerve, with observation of strict aftertreatment criteria, tenotomy of the iliopsoas, repositioning and osteotomy with turning into varus. Osteotomy without previous elimination of the pathogenetically acting muscular forces does not appear useful. Likewise, permanent re-positioning by means of muscle-relaxing operation cannot be sufficiently safe-guarded without additional osteotomy once the dislocation has taken place. In twelve patients with spastic dislocation of the hip, treated in accordance with these guidelines (two without osteotomy) aged 6 6/12 and 19 5/12 years, a roentgenologically good result was obtained in half of the cases, whereas the functional result was satisfactory not only with these patients but also with part of the other patients. If surgical treatment is instituted early enough, and if the experiences described here are taken into consideration, it is to be expected that the results will be even more satisfactory. The corset supporting the seated patient, developed by us, has been found very useful during the aftertreatment stage. A definite stand is taken against the therapeutic nihilism which leaves treatment of spastic dislocations to physiotherapy. It is also pointed out that indication for treatment is not represented only by the target of learning how to walk, but also by providing an overall improvement of the life situation of the patient, by either enabling him, or improving his ability, to sit or by "merely" improving the care of the perineum.

Adolescent↗

Compliant positioning of total hip components for optimal range of motion.

Impingement between femoral neck and endoprosthetic cup is one of the causes for dislocation in total hip arthroplasty (THA). Choosing a correct combined orientation of both components, the acetabular cup and femoral stem, in manual or computer-assisted implantation will yield a maximized, stable range of motion (ROM) and will reduce the risk for dislocation. A mathematical model of a THA was developed to determine the optimal combination of cup inclination, cup anteversion, and stem antetorsion for maximizing ROM and minimizing the risk for cup-neck impingement. Single and combined hip joint motions were tested. A radiographic definition was used for component orientation. Additional parameters, such as stem-neck (CCD) angle, head-neck ratio, and the design of the acetabular opening, were also considered. The model showed that a maximized and safe ROM requires compliant, well-defined combinations of cup inclination, cup anteversion, and stem antetorsion depending on the intended ROM. Radiographic cup anteversion and stem antetorsion were linearly correlated. Additional internal rotation reduced flexion, and additional external rotation reduced extension, abduction and adduction. The articulating hemispheric surface of acetabular cups should be oriented between 40 degrees and 45 degrees of radiographic inclination, between 20 degrees and 28 degrees of radiographic cup anteversion, and should be combined with stem antetorsion so that the sum of cup anteversion plus 0.7 times the stem antetorsion equals 37 degrees. Final component orientation must also consider cup containment, implant impingement with bone and soft tissue, and preoperative skeletal contractures or deformities to achieve the optimal compromise for each patient.

Arthroplasty, Replacement, Hip↗

A randomized study of combined botulinum toxin type A and casting in the ambulant child with cerebral palsy using objective outcome measures.

It is recognized that objective gait analysis is of great value in planning a multilevel botulinum toxin type A (BTX-A) treatment. After BTX-A treatment, objective outcome measures can provide new and interesting information for each individual child with cerebral palsy (CP). Moreover, by studying group results, we may evaluate our treatment hypotheses. The present prospective study attempts to document the effect of integrated multilevel BTX-A treatment on objective gait parameters and to define the optimal strategy for the combined treatment of BTX-A with casting in children with cerebral palsy. Objective three-dimensional gait analysis (3DGA) data were collected pre- and 2 months post-treatment, in two randomized patient groups: a first group of 17 children treated with lower leg casting prior to BTX-A injections, and a second group of 17 patients who received casting immediately after injections. The present study demonstrates that improved gait can be achieved after a multilevel BTX-A treatment, combined with casting, using a set of 90 gait parameters. The most pronounced improvement was seen at the ankle joint. The results in the knee, hip and pelvis imply that multilevel treatment of the child with CP should start at an early age, in order to prevent development of muscle contractures. Slightly more pronounced benefits, mainly in the proximal joints, were seen for the children who were casted after injections as compared to the children who were casted before injections.

Biomechanical Phenomena↗

Findings in post-poliomyelitis syndrome. Weakness of muscles of the calf as a source of late pain and fatigue of muscles of the thigh after poliomyelitis.

The purpose of this study was to identify overuse of muscles and other alterations in the mechanics of gait in twenty-one patients who had muscular dysfunction as a late consequence of poliomyelitis. All of the patients had good or normal strength (grade 4 or 5) of the vastus lateralis and zero to fair strength (grade 0 to 3) of the calf, as determined by manual testing. Dynamic electromyography was used, while the patients were walking, to quantify the intensity and duration of contraction of the inferior part of the gluteus maximus, the long head of the biceps femoris, the vastus lateralis, and the soleus muscles. Patterns of contact of the foot with the floor, temporal-spatial parameters, and motion of the knee and ankle were recorded. The principal mechanisms of substitution for a weak calf muscle fell into three groups: overuse of the quadriceps (twelve patients) or a hip extensor (the inferior part of the gluteus maximus in eight patients and the long head of the biceps femoris in four), or both; equinus contracture (twelve patients); and avoidance of loading-response flexion of the knee (five patients). Most patients used more than one method of substitution. These observations support the theory that post-poliomyelitis syndrome results from long-term substitutions for muscular weakness that place increased demands on joints, ligaments, and muscles and that treatment--based on the early identification of overuse of muscles and ligamentous strain--should aim at modification of lifestyle and include use of a brace.

Ankle Joint↗

Surgical management of musculoskeletal involvement in hemophilic patients.

Since the establishment of a Hemophilic Center at the National Taiwan University Hospital in 1984, all hemophilic patients have been registered. They have received regular follow-ups by specialists in appropriate fields. Orthopedic surgeons are assigned to evaluate and handle the complications of the musculoskeletal system. From January 1984 to April 1988, 20 of these patients were referred for various reconstructive procedures. Seventeen had a moderate to severe depletion of factor VIII and 2 had a severe factor IX deficiency. The indications for surgery include pain, swelling, frequent bleeding, and flexion contracture of the involved joints. Knee joints outnumbered the others. The operative procedures included: synovectomy in 7 patients, total knee replacement in 3 patients, total hip replacement in 1 patient, tendon reconstruction and supracondylar osteotomy of the femur in 3 patients, excision of a pseudotumor or cyst in 4 patients and amputation in 2 patients. Postoperatively, joint pain was relieved greatly, recurrent bleeding became rare and walking ability was significantly improved. To prevent a decrease in the arc of motion of the operated knee joint, exercise was initiated early using a continuous passive motion machine with promising results. Postoperative complications were minimal. Indications for various surgical procedures, and the details of hematological handling are discussed.

Adolescent↗

Medial-approach open reduction of hip dislocation in amyoplasia-type arthrogryposis.

In 95 children with amyoplasia-type arthrogryposis multiplex congenita, 40 hip dislocations in 26 patients were found. In 16 of these 26 patients, bilateral (nine patients) and unilateral (seven patients) dislocations were reduced by a medial-approach open reduction. The mean age at the time of surgery was 8.9 months. Acetabular development was satisfactory. Complications included one early redislocation, two hips with stiffness, and four of 25 hips with avascular necrosis (types 1 and 2). Overall 80% (five of seven unilateral, 15 of 18 bilateral hips) were rated good and 12% fair, and 8% (one of seven unilateral, one of 18 bilateral) were poor. Stiffness or asymmetry was not observed in the nine bilateral cases. This study suggests that dislocations in infants with amyoplasia may be successfully reduced by medial-approach open reduction. Bilateral reduction and concurrent correction of other lower limb contractures may be accomplished during the same surgical session.

Arthrogryposis↗

Normal ranges of popliteal angle in children.

Measurement of the popliteal angle is used to assess hamstring contracture in children with cerebral palsy. The popliteal angle in 482 normal children, 1-10 years of age, was measured. Using a 360 degrees goniometer, the popliteal angle was measured with the hip held at 90 degrees flexion to indicate hamstring muscle tightness. Between the ages of 1 and 3 years, the mean angle was 6 degrees (range, 0-15). At age 4, the angle rose to 17 degrees in girls and 27 degrees in boys (range, 5-45). At greater than or equal to 5 years the mean angle was 26 degrees with little change (range, 0-50). A popliteal angle of greater than 50 degrees in the above age groups indicates abnormal hamstring tightness.

Cerebral Palsy↗

Longterm followup of hip and knee soft tissue release in juvenile chronic arthritis.

Longterm results of soft tissue release for hip and knee flexion deformity in 27 patients with juvenile chronic arthritis (JCA) were evaluated. A total of 53 tenotomies were reviewed: 22 hips and 31 knees. Mean age at surgery was 12.1 years, with a mean underlying disease duration of 6.3 years and a postoperative followup of 5 years. Patients were evaluated at 3-6 months, then at one, 3 and 5 years. Results showed a marked drop in flexion contracture and an improvement in joint range up to the first 3 years postsurgery, but beyond this period benefits achieved were gradually partially lost. Only 37% of patients were able to walk before surgery. At one year followup, 81% were able to do so. Soft tissue release thus seems a beneficial therapeutic alternative to preserve both alignment and function in hip and knee flexion deformity affecting patients with JCA.

Adolescent↗

Southern hip exposure.

The efficacy of the posterior approach to the hip was analyzed in a retrospective study of 168 consecutive procedures performed by the author. Surgical technique is redefined historically and described in detail. Results are studied in two subgroups of hip surgery patients: endoprosthetic replacement (98) and total joint replacement (70). The factors evaluated were surgical time and blood loss, morbidity (dislocation, infection, pain, and limp), mortality, and complications of surgery. The posterior approach to the hip allowed excellent exposure without transecting tissue planes and creating dead spaces. Operative time averaged less than one hour for all cases, and blood loss, less than 500 cc. The four dislocations that occurred postoperatively were in patients with pre-existing flexion contractures. One superficial infection was noted but there was no increase in morbidity due to pain, limp, or loss of motion. Two total joint replacements were complicated by hematoma formation but occurred in patients who had extensive capsule resection and acetabuloplasty. Excellent and rapid exposure with minimal soft tissue destruction or blood loss coupled with few postoperative complications should encourage orthopedic surgeons to include the posterior approach in their treatment of hip problems.

Adult↗

Severe Phenotype in an Indian Family With Progressive Pseudorheumatoid Arthropathy of Childhood.

Progressive pseudorheumatoid arthropathy of childhood (PPAC) is a rare autosomal recessive progressive condition that affects the cartilage of joints and bones. The symptoms of PPAC include stiffness of the joints, bony swelling of the toes and fingers, short stature, kyphosis, and muscle weakness. The radiologic manifestations are often mistaken for juvenile rheumatoid arthritis and include platyspondyly, widened metaphyses, flattened epiphyses, and large femoral heads. The disease is caused by variants in the WISP3 (also known as CCN6) gene, which encodes a member of the WNT1 inducible signaling pathway (WISP) protein subfamily, which belongs to the connective tissue growth factor (CTGF) family. We describe three affected female siblings in an Indian family with PPAC, all of whom manifest a severe phenotype of the disease. The girls all walked with a crouching gait from severe joint contractures. Radiographic findings included generalized periarticular osteopenia and widened metaphyses. The radiographs of the hands and feet showed similar changes with narrow joint spaces, widened metaphyses, and flattened epiphyses. Elbows and knees revealed gracile bones, and contractures with severe muscle atrophy. Spine films were significant for marked beaking, lumbar vertebrae anterior narrowing, irregular end plates and rotational scoliosis. X-rays of the hips revealed deformed femurs, coxa vara, and large flat epiphyses. All affected individuals were compound heterozygotes for two pathogenic variants in the WISP3/CCN6 gene, a novel nonsense variant (c.172A>T [p.Lys58*]) and a 2-base pair deletion (c.740_741delGT [p.Cys247Leufs*31]). This combination of a nonsense and frameshift variant has not previously been reported and may be the explanation for the severe clinical manifestation of PPAC in this family. Further investigation into the mechanism of the disease may provide promising therapies to modify disease progression.

WISP3↗

Thigh compartment syndrome secondary to intertrochanteric hip fracture in a quadriplegic patient: case report.

Compartment syndromes in the thigh are rare and the diagnosis may be difficult in the light of subtle early physical findings in the patient with spinal cord injury. Clinical awareness of the impending compartment syndrome is important to provide timely proper treatment and avoid disabling deformities. A compartment syndrome should not be ignored in the paraplegic, because the potential for late fibrosis and contractures may limit the independence of such patients. Greater awareness and index of suspicion are needed to successfully recognize and promptly treat the compartment syndrome in this patient population.

Accidental Falls↗

Low-load, prolonged stretch in the treatment of knee flexion contractures in nursing home residents.

BACKGROUND AND PURPOSE: The purpose of this study was to do a pilot test of the effectiveness of prolonged stretch (use of splint) in reducing knee flexion contractures more than a traditional program of passive range of motion (PROM) in a group of nursing home residents. SUBJECTS: The subjects were 28 nursing home residents with 10 degrees or more of knee flexion contracture bilaterally. Eighteen subjects completed the study. METHODS: Data were collected prior to the initiation of the intervention and at monthly intervals during the 6 months of treatment. Repeated measurements were made of hip, knee, and ankle range of motion (ROM); the torque required to maintain passive impairment. knee extension; knee pain; several indicators of function; and cognitive impairment. Both legs of each subject received PROM and manual stretching twice a week; in addition, one leg was given a prolonged stretch (use of a splint) five times a week. RESULTS: There were no differences in knee ROM between the side that received prolonged stretching and the side that received only PROM and manual stretching at the beginning of the study. No differences in ROM or torque measurements existed between the side that received prolonged stretching and the side that received only PROM and manual stretching at any interval, nor in ROM or torque over time for either side. Because of the low statistical power of the study, the results should be viewed with caution. CONCLUSION AND DISCUSSION: Physical therapists need to question whether prolonged stretch for nursing home residents with knee flexion contractures greater than 10 degrees is of any greater benefit than PROM and manual stretching. Investigations of other treatment protocols and treatment doses are needed, including work in the area of prevention of knee flexion contractures. For the pilot group of nursing home residents studied, gains in knee extension did not occur with the use of prolonged stretch for 3 hours a day, 5 days per week. [Steffen TM, Mollinger LA. Low-load, prolonged stretch in the treatment of knee flexion contractures in nursing home residents.

Activities of Daily Living↗

Mobility aids for children with high-level myelomeningocele: parapodium versus wheelchair.

The optimal method for providing mobility for children with myelomeningocele remains controversial. 39 children using a parapodium were compared with 29 children in wheelchairs. There were no significant differences between the two groups for medical complications, use of health-care services or activities of daily living, although there were differences in the patterns of complications. Children using the parapodium were more likely to develop lesions of the lower extremities, to have dislocated hips, to be more obese and to watch more television; children using wheelchairs were more likely to develop lesions of the gluteal region, to have knee-flexion contractures and to have fewer fecal accidents. The parapodium was judged by families to be less effective as a mobility aid; however, the upright posture it allows was considered extremely advantageous. A combined approach allowing upright posture and wheeled mobility would appear to be optimal.

Activities of Daily Living↗

Postoperative heterotopic ossification in children: a comparison of children with spina bifida and with cerebral palsy.

The authors reviewed 250 consecutive myotomies and tenotomies performed on the hips of 186 children. Two groups were studied: 162 children with cerebral palsy and 24 children with spina bifida. In total 19 children were found to have heterotopic ossification postoperatively. The incidence of heterotopic ossification was 21% in the spina bifida group and 8.6% in the cerebral palsy group. The most severe cases were in children with thoracic myelomeningocele. Only one child with lumbar myelomeningocele had ossification, and it was mild. This difference was statistically significant. Two children with thoracic spina bifida had severe ossification and abduction contractures requiring resection of the bony mass. Recurrence was successfully prevented by a course of radiotherapy.

Cerebral Palsy↗

Fiber density in congenital muscle fiber type disproportion. II. Congenital muscle hypotonia and hip dislocation.

Our previous paper presenting electromyographic findings in patients with congenital fiber type disproportion myopathy, confirmed the myogenic character of the disease process. That group of patients was however fairly heterogenous regarding both the clinical features and the morphological changes in muscle fibers (e.g. cases with central cores). In the present study we have examined 13 children with hypotonia and muscle fiber type disproportion operated on in childhood for congenital hip dislocation. In all cases CNEMG and SFEMG with FD estimation was performed in biceps brachii and quadriceps femoris muscles. In all muscles examined either slight EMG changes indicative of myopathy or a normal EMG pattern was found. None of the patients demonstrated an evident increase in FD values. Normal FD and the recruitment pattern proportional to the force of contractures indicate that the normal number of motoneurons is preserved. Accordingly, our present findings confirm the conclusions of our previous paper.

Adolescent↗

A cerebral cause of arthrogryposis: unilateral cerebral hypoplasia.

The authors describe a case of predominantly right-sided arthrogryposis in an infant with contralateral congenital cerebral hypoplasia and associated unilateral hydrocephalus. Diagnosis was made by prenatal ultrasonography and confirmed by postnatal cerebral CT-scanning. In addition to a variety of congenital neuromuscular disorders, arthrogryposis may be caused by disorders of the central nervous system. Hence, in the diagnostic approach of newborns with congenital contractures, cerebral malformations must be considered.

Abnormalities, Multiple↗

Total hip replacement: a comparison between the transtrochanteric and posterior surgical approaches.

It can be argued with good justification, that the trochanteric complications which stimulated this study could have been avoided by strict adherence to Charnley's technique regarding trochanteric advancement and re-attachment. Should trochanteric reattachment be required, it should be done with strict attention to the details outlined by Charnley, or by use of some other device such as the Volz bolt. Trochanteric removal is usually unnecessary. The transtrochanteric approach to total hip replacement has inherent technical problems that increase the potential for complications. These problems include: painful trochanteric bursitis, displacement of trochanter, non-union of trochanter, broken wires and, an increase in dislocation rate (12% as compared to 4%). The posterior approach reduces operative time, blood loss, hospitalization, and shortens the period of time to unprotected weight bearing. Should certain conditions such as external rotation contracture, acetabular protrusion, or relative lengthening of the operated limb indicate trochanteric osteotomy, it can be accomplished readily by way of the posterior exposure.

Arthroplasty↗