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[Treatment of spasticity in cerebral palsy with botulinum toxin].

OBJECTIVE: A review of the pathophysiological and developmental basis, measurement scales and the usefulness of botulinum toxin A injections in selected muscles for the treatment of spasticity in children with cerebral palsy. DEVELOPMENT: Cerebral palsy is the most common cause of spasticity in children. The increase in muscle length is achieved through the addition of sarcomeres in series at the level of the muscle tendinous junction. The regulation of the number of sarcomeres seems to be determined by the lengthening of the muscle. The muscle contracture is a shortening of the length of a muscle as a result of a decrease in the number of sarcomeres. Spasticity and motor function assessment scales used in children with cerebral palsy: a) Modified Ashworth scale for the assessment of spasticity; b) modified Tardieu scale for the assessment of dynamic muscle length; c) muscle spasms frequency scale; d) modified Medical Research Council scale for muscle strength; e) hip adductor muscle tone scale; f) global pain scale with affective facial expression represented in a drawing; g) goniometric measurement of the joint range of movement; h) Palisano gross motor function measure; i) observational video gait analysis scale. Recommended guidelines for dosing the botulinum toxin A: 1. Total maximum dose administered per visit up to 15 U/kg or a total of 400 U; 2. Dose range of large muscles 3 to 6 U/kg per visit; 3. Dose range of small muscles 1 to 3 U/kg per visit; 4. Maximum dose per injection site: 50 U dividing the total planned unit dose/muscle into equal amounts/injection site; 5. Frequency: no more than one injection every 3 months, frequently once every 6 or more months. CONCLUSION: Botulinum toxin A injection is a well tolerated, safe and effective procedure in the treatment of children with spastic cerebral palsy.

Botulinum Toxins, Type A↗

[Analyses of survival time of joints with dysplastic arthrosis after intertrochanteric femoral osteotomy].

METHODS: Survivorship analysis was performed up to 13 years after intertrochanteric femoral osteotomies in 136 dysplastic hip joints with osteoarthritis. Varus-valgus and rotational osteotomies were evaluated. The criterium limiting survival was the recurrence of pain postoperatively. RESULTS: Joints with severely pathological Sharp Angles (> 48 degrees) showed a diminution within 10 years to 50%. Moderately pathological joints (< 49 degrees) needed 13 years to diminish to 50%. 3 groups of the CE angle showed a similar picture with somewhat more difference between the groups, but not statistically significant during this time. This was different when evaluating the 3 grades of arthrosis. Joints with grade 1 were free of pain after 13 years in only 51.2%, joints of grade 2 already after 10 years in only 37% (p < 0.05). The final values of grade 3 joints were found after only 5 years. This demonstrates that varus osteotomy is only of limited value in dysplastic joints with osteoarthritis. Operations correcting the acetabulum have to be compared. CONCLUSION: In 21 patients rotational osteotomies to correct external rotation contracture had a marked effect on pain in 90.4%, valgus osteotomies with rotation in 75%, varusosteotomies with rotation in 65%. Without rotation a marked reduction of pain was found in varus osteotomy in 61.6% and in valgus osteotomy in only 55%. So far, patients with diminished anteversion of the femur were not separated and treated specially. But diminished anteversion of the femur and/or acetabulum leads to osteoarthritis too.

Adult↗

[Treatment of ununited fractures and pseudarthrosis of long bones of the lower limbs complicated by osteomyelitis].

123 patients with ununited diaphyseal fractures and false joints of the shin bones (93) and the hip (30), complicated by purulent infection, were treated in the clinic for pyogenic complications and consequences of locomotor traumas of the CITO from 1988 to 1994 years. Most patients were men (110 patients). The posttraumatic period varied from several months to 12 years. Many patients before their admittance to the clinic were not once unsuccessfully operated 69 (56.1%) patients had osseous defects of the thigh and the shin, all patients contractures of joints of the lower extremities as well as substantial trophic disturbances and atrophy of the muscles. 12 patients underwent closed transosseous osteosynthesis by the apparatus of an external fixation. One stage surgery was carried out in 111 patients, which included radical fistulosequestrenecretomy (and when indicated--extended segmental sequestrectomy) and transosseous osteosynthesis by the apparatus of external fixation with the use of the needles, rods, or their combination. The feature of the osteosynthesis depended on the extent of bone defect, which has been developed after sequestrectomy, in the defect up to 4 cm monolocal osteosynthesis was used, in the defect from 5 to 8 cm--bilocal and if it were over 8 cm--multilocal osteosynthesis was preferable. One stage treatment resulted in favourable outcomes in 95.9% of patients: the osteomyelitis was eliminated, the integrity of the bone was restored and in a great majority of cases--the length of the extremity was preserved. The treatment duration was 1.5-2 times less than in commonly used methods.

Adolescent↗

[3 case reports of lumbosacral agenesis; role of the lumbo-iliac bone graft].

From 3 cases (8,2 and 16 years) the two first patients have been followed since birth, and in reviewing the 124 cases published in the literature, the authors analyze: the possible causes with in first the mother's diabetes, the possibility of antenatal diagnosis with ultrasound, the clinical symptoms: paraplegia with stiff joints and deformities of lower limbs, finally the therapeutic possibilities to correct the deformities of lower limbs and find the compromise between straighten the patient to authorize the possible standing and keep mobility between trunk and pelvis to allow sitting. In this case, the lumbo-iliac bone graft can be used if necessary and if the mobility of the hips make that possible. This bone graft was realized once in January 1979 (follow up 5 1/2 years). All the references are in Dr Barnetche's thesis (Bordeaux 1984, n degrees 135).

Adolescent↗

Profiles of neuromuscular diseases. Becker's muscular dystrophy.

Fifteen subjects with Becker's muscular dystrophy (BMD) were studied prospectively over a 10-yr period to provide a profile of impairment and disability. Proximal lower extremity musculature (particularly the hip and knee extensors), ankle dorsiflexors, and neck flexors showed significantly early weakness. Extensor muscle groups were weaker than flexor muscles at the elbow and knee. The mean manual muscle test (MMT) strength grade for all muscle groups combined was 3.7 +/- 0.8 MMT units. There was a slowly progressive decline in strength, only -0.31 MMT units per decade, and the decline was relatively equal in all muscle groups. There was not side dominance. Severe contractures did not appear to be a problem until after transition to a wheelchair, and scoliosis was rare. Restrictive lung disease occurred as a late complication in a small percentage of cases; however, maximal expiratory pressure was significantly reduced early in the disease. Only two individuals (19%) had severe restrictive lung disease and a history of significant respiratory complications. There was a slight but significant decline in forced vital capacity and maximal expiratory pressure with age and disease duration. The cardiomyopathy in BMD appeared to be disproportionately severe in some cases. Eleven individuals (73%) had abnormal electrocardiograms, and five (25%) had a history of significant cardiovascular complication, with no age or disease duration effect. Functional evaluations and timed motor performance tests showed only mild disability in most individuals. Mean intellectual and neuropsychologic function was within normal limits, but with a large variability in intelligence quotient scores. This report and others suggest a tremendous heterogeneity of severity among males with BMD.

Adolescent↗

The musculoskeletal manifestations of epidermolysis bullosa: an analysis of 19 cases with a review of the literature.

Epidermolysis bullosa encompasses a group of rare disorders characterized by marked skin fragility and blister formation. In patients with dystrophic epidermolysis bullosa, skeletal and soft-tissue abnormalities are an important feature. An analysis of the musculoskeletal manifestations in 19 patients is presented. In the hands and feet, features included generalized osteoporosis, wedge-shaped thinning and hooking of distal phalanges, overconstricted bones, acro-osteolysis, flexion contractures, metatarsal and metacarpal subluxation, distal trophic changes, webbing of digits, encasement of the whole extremity in a pouch of skin, soft-tissue calcification and retarded skeletal maturity. Previously undescribed findings in the hands and feet are bony ankylosis of the proximal interphalangeal joints, resorption of the metatarsal and metacarpal heads, shortened metatarsal bones, carpal and tarsal fusion and destruction, and cystic changes of the distal radius and ulna. In the remainder of the skeleton, hip dysplasia with premature osteoarthritis, knee joint bony ankylosis and thoracic and thoraco-lumbar scoliosis are other undescribed findings.

Adolescent↗

[Ehlers-Danlos syndrome--clinico-pathology and orthopedic treatment].

Eight cases of Ehlers-Danlos syndrome (EDS) were surveyed for clinical manifestations and orthopaedic treatment. Cases of EDS were clinically subdivided into three groups. The first group was characterized by multiple congenital contractures in the extremities, so that it was very difficult to distinguish it from arthrogryposis multiplex congenita in early infancy. Since laxity of the soft tissue coexists with contracture in this group, over-correction of deformity caused much more disability than under-correction, especially in clubfoot. Care should therefore be taken to avoid this. The third group was characterized by marked laxity of the soft tissue from birth. Diagnosis in this group was easily made by the obvious triad of EDS. Lengthy immobilization in an appropriate position was necessary to maintain a corrected position for the treatment of any deformity in the hip, foot or hand. In the second group, deformity and disability were clinically so mild that no orthopaedic management was generally required.

Child, Preschool↗

Nonspinal tuberculous osteomyelitis in children.

Tuberculous osteomyelitis is uncommon in children but its incidence seems to have increased in our hospital recently. From April 1984 to December 1994, we treated 12 children with this problem. Three cases were diagnosed in the first 7 years and nine cases in the latter 4 years. Primary spinal lesion was excluded. There were seven boys and five girls, with an average age of 2.4 years (range 1-4.5). Anatomic sites of involvement were distal femur in three patients, proximal tibia in three, foot in three, proximal femur in one, proximal humerus in one, proximal radius in one, distal radius in one, and rib in one. Except for one case of hip lesion, the most common symptom was localized swelling. Delay in diagnosis was 6.6 months on average. The diagnosis was confirmed bacteriologically or histologically in all patients after surgical debridement and curettage. Anti-tuberculosis (TB) drugs were administered for 6 months postoperatively. All patients were followed-up for an average of 3.8 years (range 2-8) with satisfactory healing of their tuberculous lesions. Two cases were complicated with physeal bar formation. One patient had residual flexion contracture of the knee. Although the mortality from TB has decreased in Taiwan, the frequently reported late cases of TB deserve attention. Physicians should be aware that TB remains an important cause of bone lesions. These lesions can be healed by combined therapy with surgical debridement and anti-TB medication for 6 months.

Age of Onset↗

Lower limb surgery in Duchenne muscular dystrophy.

Two hundred and thirteen of 428 patients with Duchenne muscular dystrophy (DMD) of a prospective and open study were operated on bilaterally with hip and knee release, aponeurectomy of the iliotibial band and Achilles tendon lengthening. In 87 patients the operation was carried out during early restrictions of the lower limb joint mobility at an average age of 6.56 years (4.02-8.26, SD 1.42). The follow-up was on average 5.4 years (0.25-9.01, SD 2.7). This group was compared to a control group (natural history) consisting of 100 non-operated DMD patients. A significant (P < 0.001) release of the contractures could be obtained. Loss of walking ability occurred in the control group at an average of 9.29 years (5.85-13.63, SD 1.98) and in the operated group at an average of 10.55 years (8.17-14.39, SD 1.76). This shows that early lower limb surgery leads to a prolongation of independent ambulation of 1.25 years on average. In contrast to the patients of the control group all treated patients between ages 6 and 8 years could walk independently. The positive influence of early lower limb surgery could also be shown by the development of Hammersmith motor ability score, CIDD (Council of Investigation of Duchenne Dystrophy) grading and Vignos scale. Nevertheless, in consideration of the well-known course of DMD not only the prolongation of ambulation but also the achieved prolongation of assisted standing ability with no or mild contractures are aims of lower limb surgery. Since no improvement of muscle strength could be observed after lower limb surgery, further studies have to investigate if additionally administered steroids can prolong ambulation after early lower limb surgery.

Adolescent↗

Total knee replacement with a variable axis knee prosthesis.

The Variable Axis Knee Prosthesis was designed to accommodate a wide range of problems in the knee and to minimize the complication of loosening. The results of the series, which now spans over seven years at this institution, have confirmed the effectiveness of the prosthesis in providing stability and firm fixation. Patellar alignment remains critical for optimum results. A wide variety of abnormalities, including failed prostheses, severe flexion contractures, arthrodesed knees, and severely osteoporotic bone, can be effectively managed utilizing this device. As with any other total knee prosthesis, the use of this prosthesis should be restricted to those knees with definite radiographic evidence of joint impairment in addition to significant symptoms. Relative contraindications include previous sepsis, neuropathic joint disease, and gross ligamentous incompetency. If these indications and limitations are adhered to, the results from knee replacement can be expected to equal or exceed those from total hip replacement over the long term.

Arthritis, Rheumatoid↗

Walking ability in patients with diastrophic dysplasia: a clinical, electroneurophysiological, treadmill, and MRI analysis.

Patients with diastrophic dysplasia have walking difficulties of obscure etiology; some are even wheelchair-bound. To explore the problem, physical examination, treadmill, magnetic resonance imaging (MRI), and electroneurophysiologic studies were performed on 87 patients (56 females, 31 males) with an average age of 31 (range 3-56) years. Mobility of the spine, hips, knees, and feet was diminished. Some of the patients were obese (mean body mass index 27.0 kg/m). In the treadmill study, patients were able to walk an average of 638 m (range 0-1,618 m). On MRI, five patients showed compression of neural structures; one of them also had clinical symptoms. Somatosensory evoked potentials and electroneuromyography revealed evidence of compression of neural structure in three (3%) and two (3%) patients, respectively. The walking difficulties seem to have a multifactorial etiology: flexion contractures of the knees, early and rapid osteoarthrosis, equinus or equinovarus foot deformities, and obesity, but only rarely spinal stenosis.

Adolescent↗

Pathologic gait.

The diagnosis of gait deviations and their causes requires three levels of knowledge. First is an awareness of the functional limitations caused by each type of abnormality (contracture, muscle weakness, pain, and spasticity). Second is familiarity with normal function. Third is having a trained eye or gait analysis system that is capable of identifying the gait deviations. Once diagnosis is complete, a fourth requirement is the ability to use the findings to set up an appropriate treatment regimen.

Ankle Joint↗

Assessment of the short-term effect of antispastic positioning on spasticity.

BACKGROUND: This study was performed in order to investigate the effect of antispastic positioning on spasticity by using different assessment methods. METHODS: A total of 16 patients (11 males [68.75%] and five females [31.25%]), diagnosed as spastic diplegic and referred to the School of Physical Therapy and Rehabilitation, Paediatric Rehabilitation Unit for treatment, were included in this study. The mean age of patients was 6.43 +/- 1.99 years (range, 4-13 years). Passive dorsi-flexion movement was measured by using goniometer and intensity of spasticity was determined by using Modified Ashworth Scale (MAS) and electromyography. For the objective measurement of severity of spasticity, Hoffman reflex (H) and Hoffman reflex/motor response (H/M) ratios were used. Children were placed in an antispastic position for 20 min. The patient was placed in a sitting position, by a physiotherapist, with hips abducted at a 45 degrees angle and externally rotated, knees extended, and ankles placed in a neutral position. RESULTS: The decrease in H responses, H/M ratios, MAS values and the increase in goniometric measurement values were found statistically significant after antispastic positioning (P < 0.05). CONCLUSION: Our study supports that antispastic positioning can be used with neurodevelopmental treatment approaches when it is required. Antispastic positioning may help exercises to be performed more easily, and also has importance in a home exercise program to prevent muscle contractures and joint limitation in children with long-term spastic diplegia.

Cerebral Palsy↗

Heterotopic ossification: a review.

Heterotopic ossification is defined as the presence of lamellar bone at locations where bone normally does not exist. The condition must be distinguished from metastatic calcifications, which mainly occur in hypercalcaemia, and dystrophic calcifications in tumours. It is a frequent complication following central nervous system disorders (brain injuries, tumours, encephalitis, spinal cord lesions), multiple injuries, hip surgery and burns. In addition to this acquired form, hereditary causes also exist, such as fibrodysplasia ossificans progressiva, progressive osseous heteroplasia and Albright's hereditary osteodystrophy. Although these conditions are extremely rare, they can provide useful information on the physiopathology of heterotopic ossification, and thus lead to novel and causal treatment modalities. Heterotopic ossification is no trivial complication. A limitation of the range of joint motion may have serious consequences for the daily functioning of people who are already severely incapacitated because of their original lesion. Increased contractures and spasticity, pressure ulcers and increasing pain further compromise the patient's capabilities. Consequently, we feel that attention should be paid to the pathogenesis and particularly the prevention and treatment of this disorder.

Burns↗

Multiple joint replacement in chronically neglected polyarthritic patients: Two case reports.

Multiple joint replacement is a viable option for rehabilitation of young polyarthritic patients with unsalvageable joints. Young polyarthritic patients in this part of the world suffer from chronic neglect because of ignorance, apathy and low socio-economic status. During the period of chronic neglect, these patients acquire extreme deformities of various joints either due to active disease (ankylosing spondylitis, rheumatoid arthritis) or irreversible changes in the joint configuration like ankylosis and soft-tissue contracture. Associated spine and thoracic cage affection create problems for anaesthesia and peri-operative positioning. We report 2 cases of multiple joint replacements for young polyarthritic patients who were bedridden for 6 to 11 years. Surgeries were performed in a phased manner and after extensive rehabilitation both patients were able to walk unaided. Various problems and difficulties encountered have been addressed so as to serve as a guide to surgeons who may have to deal with such unusual situations of chronic neglect. We also report a modified exposure technique without trochanteric osteotomy for total hip replacement, which is valuable in extreme external rotation ankylosis.

Journal Article↗

Subtrochanteric missile fractures of the femur.

The treatment results of subtrochanteric missile fractures of the femur in 41 casualties are analyzed. The wounds were inflicted by infantry weapon missiles in 29 (70.7%) and by fragments of mine or explosive devices in 12 (29.3%) casualties. The associated neurovascular bundle injuries were present in 11 (26.8%) patients. Fracture stabilization was done by external fixation in 25 (62.5%): group A; and by plaster of Paris in 15 (37.5%) patients: group B. The postoperative period was uneventful in 14 (35.0%) and early or late complications occurred in 26 (65.0%) patients. Bone infection developed in six (15.0%) and fractures did not heal in four (10.0%) patients. Complications were present in 13 (52.0%) patients from group A and in 13 (86.7%) patients from group B. Delayed fracture healing and fracture nonunion were present in group A and contractures of large joints in group B. Subtrochanteric missile fractures of the femur present very severe injuries where the treatment is followed by a very high percentage of complications. External fracture fixation in this region facilitates the care, stability, early covering of soft tissue defects and early physical therapy.

Adult↗

[Pathological fractures of the knee].

The authors present the methods and results of treatment of 20 epiphyseal and low-metaphysis pathological knee fractures treated at the Department of Orthopedics of the Lublin University of Medical Sciences between 1962-1999. The average age was 39 years. Malignant tumours (2 lymphomas, 1 plasmocytoma, 1 hemangiopericitoma, 2 adenocarcinoma metastases) and benign lesions (10 giant cell tumours, 3 solitary bone cysts, 1 intraosseous extension of pigmented villonodular synovitis) causes 18 femur fractures and 2 tibia fractures. The fractures caused by lymphomas in 2 patients and plasmocytoma in 1 patients were treated with either a plaster cast or skeletal traction and healed within 16 weeks. Tumour resection and reconstruction with Rush rods, Kuntscher nail, plate and methylmetacrylate were performed in 2 cases. In 1 case with hemangiopericytoma hip exarticulation was performed. In 5 cases pain relief and weight bearing capacity of the limb was not achieved because of neoplasm recurrences; 5 deaths occurred within 6 months to 7 years after femur fracture because of pulmonary metastases. Benign bone lesions were treated by curettage of pathological tissues, burring bony walls of the lesions and filling the cavities with bone grafts or methylmetacrylate (1 case). Displaced fragments were reduced whenever possible. Limbs were immobilised in plaster casts. The follow-up period for patients with benign lesions was 8 years. All fractures healed. Arthritis with persistent pain and knee contracture was noted in 1 patient who underwent surgery 3 times for giant cell tumour recurrences, complicated by infection and varus deformity.

Adolescent↗

Arthrogryposis. Pediatric review and habilitative aspects.

A precise diagnosis among the types of arthrogryposis must be made. For amyoplasia, the recurrence risk is nonexistent, the natural history favorable. During the first weeks, frequent stretching supported by maintenance casting and splinting of severe or generalized contractures is indicated in a residential multidisciplinary center. The key to deformity is the rigidity of the periarticular structures. With limb growth, contractures progress or recur. The growth years must therefore be dominated by physical management, which consists of stretching, casting, and bracing interspersed by staged soft-tissue procedures. At completion of limb growth, final corrections should be attained by osteotomies. Adolescence should be a time to follow social and prevocational pursuits in preparation for a place in society.

Adolescent↗