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PubMed1980

[Essential deformans osteolysis. Report of a case with progressive and generalized evolution (author's transl)].

The authors report a clinical and radiographic course of a 16 years old caucasian girl with a progressive deformation of her skeleton. The primary lesions occur in knees and wrists. This disease was at first considered as a rheumatoid arthritis, then as a dysplasia epiphysialis multiplex, and now as essential deformans osteolysis (with carpal lysis, shortening of the forearm bones, dislocation of the elbows, disparition of the humeral and femoral heads, contracture of hips and knees, posterior tarsal lysis, and kypho-scoliosis). The clinical (particularly ophtalmologic), biological (including inflammatory, phospho-calcic and nephrologic evaluation with mucopolysaccharidosis urinary excretion) and histological (skin, muscle and bone) check-up were normal. They review the different classification established on lesion topography, on association or not with a nephropathy, finally on an dominant or recessive autosomal inheritance. Then the authors think that their case is similar to the ones of Winchester and Hollister. They discuss the etiopathogenic factors, and do not consider their case as a new mucopolysaccharidosis, but rather as a generalized disease of the collagen of bones.

Adolescent↗
PubMed1994

[Is surgery of the hip adductor muscles justified in children with cerebral palsy?].

INTRODUCTION: Restricted passive hip abduction in children with cerebral palsy (CP) may be caused by uninhibited resting contractions and/or retractions, i.e., shortened muscle body or tendons. Pathological short tendons require surgical intervention, but lack of muscle body elasticity responds to physiotherapy or a postural splinting. Clinical examination can distinguish between short tendons and short muscle body. The thigh is slowly and passively extended while palpating the tendon. Tension is detected in the tendon when the leg is at angle Ao. The elastic tension of the muscle body then increases until no further movement is possible, at angle Amax. The difference Amax-Ao is an index of the structural length of the muscle body. If this difference is reduced during passive straightening there is shortening of the muscle body; if it is displaced it indicates shortening of the tendon. The value Ao indicates the muscular or tendon origin of the retraction for a given passive limitation (Amax). This study defines the physiological values of Ao and the relative precisions of chemical and instrumental measurements. MATERIALS AND METHODS: A total of 30 children aged 9-11 years, 10 CP patients (7 girls and 3 boys, mean age 10.3 years) and 20 controls (11 girls and 9 boys, mean age 10.5 years) were studied. All the CP children had lower limb spasticity and adopted an adduction posture. None had undergone hip muscle surgery. Ao and Amax were measured clinically with a goniometer and EMG to monitor muscle silence, and experimentally using a deformable parallelogram and force transducers. RESULTS: The minimum physiological value of Ao was 8 degrees with the knee flexed and 0 degree with the knee extended. Smaller values of this angle indicated tendon retraction. The difference between Ao and Amax in the controls and CP children was < or = 10 degrees; the reproducibilities of the clinical measurement of Ao and Amax were very similar. DISCUSSION: Clinical examination provides an acceptably accurate method of distinguishing between tendon and muscle body retraction of adductor muscles in CP children. The conditions required for successful measurement are: careful examination with strict positional reference and sufficiently relaxed pelvic muscles. A hip extension angle Ao of less than 8 degrees with the knee flexed or 0 degree with the knee straight indicates tendon retraction requiring tendon surgery, otherwise, the retraction involves only the muscle body. This reduced elasticity can be overcome by prolonged extension (at least 6 hours/24). Effective muscle extension may be hindered by non-suppressed adductor contractions. This must be overcome prior to physiotherapy by surgery of the ramus ant. n. obturatorii. CONCLUSION: Clinical measurement of Ao of adductor muscles is a reliable way of distinguishing between tendon retractions requiring surgery and muscle body retractions resulting from staying too long in a position with the muscle shortened. This muscle body shortening can be due to lack of physiotherapy or a stretching apparatus treatment, pathological contractions, or compensation for disorders of the controlateral limb.

Adolescent↗
PubMed1977

Fibrous contracture of muscles following intramuscular injections in adults.

Periarticular fibrous muscle contractures in adults from repeated injections in the same site is predictable. The causes of joint contracture in children are many and complex, but in adults it seems certain that this phenomenon is the result of repeated injections of analgesics or other agents into 1 muscle area. Any drug if repeatedly injected locally may cause fibrosis of the muscle and subsequent joint contracture. Five cases of bilateral abduction contracture of the shoulder in adults including the first case of bilateral abduction contractures of shoulder and hip plus bilateral flexion contracture of elbow and extension contracture of a knee are reported. No underlying disease which might predispose to this fibrosis of muscles was noted. The frequency and period of injections were variable over several years. In all patients the interference in activtities of daily living were serious, but the deformities were corrected by release of the fibrous band with relief of discomfort and restoration of joint motion without recurrence. Noting the potential complication of repeated intramuscular injections in one area, this practice should be avoided whenever possible in adults, as well as in children.

Adult↗
PubMed1995

Clinical results of early orthopaedic management in Duchenne muscular dystrophy.

Although the primary defect of Duchenne muscular dystrophy has been found, there is no causal treatment to alter the natural course of this disease. Based on the recommendations by Glorion and Rideau with early treatment of contractures of the hips and the lower limbs we performed a modified release of the spina muscles, resection of tensor fasciae latae muscle and a lengthening of the tendo calcaneus in 32 patients. The mean age of DMD patients at time of operation was 6.1 years. The mean follow-up was 3.4 years. All children underwent mobilisation the day after surgery. Complete correction of all contractures was immediately achieved after operation and kept in all but two cases up to the follow-up examination after 3.4 years. No loss of ambulation was observed. Our results demonstrate that early selective surgery in DMD patients just at or better before the onset of contractures without performing an additional aponeurectomy of the iliotibial band and percutaneous tenotomy of the hamstrings according to the original Glorion-Rideau technique safely prevents severe contractures and thereby delays the progression of scoliosis.

Child↗
PubMed1980

Joint motion limitation in newborns.

Fifty neonates were evaluated and followed for 2 years with attention to the passive range of joint motion. Passive range of motion was symmetrical throughout this period. Only the hips were noted to have persistent contractures in flexion and external rotation during these 2 years. Such deformities of the hip make judgements about anteversion in infancy difficult. They also help explain the peculiarity of the lordotic, externally rotated gait seen initially in many toddlers.

Ankle Joint↗
PubMed2004

Infantile skeletal skew: the use of ultrasound in management.

The term 'infantile skeletal skew' recognizes the prenatal or postnatal moulding of a small proportion of babies who develop characteristic changes including plagiocephaly, wry neck, scoliosis, pelvic obliquity and postural foot deformities. Pelvic obliquity may cause an abduction contracture of the hip on one side and an adduction contracture on the other, and radiographs may suggest a 'dysplasia' of the acetabulum on the adducted side, giving rise to concern that the hip is unstable. In these cases we have found that early ultrasound assessment of the adducted side will reassure the clinician that the hip is 'in joint' and the condition should be allowed to resolve itself over several months without any treatment. Eighteen infants with features of moulding in whom there was concern over the development of the hip on the adducted side, have been prospectively studied with the use of ultrasound until the age of 18 months when a final radiograph confirmed normal development of the hips.

Hip Joint↗
PubMedDate not supplied

Gluteus maximus contracture.

Abduction contracture of the hip (frog-leg deformity) is caused by contracture of the gluteus maximus muscle. A simple operation consisting of complete detachment of involved fibrous portions of the gluteus maximus from the greater trochanter to the ischial tuberosity is sufficient to release the contracture and enable the patient to squat without abducting the hips. Possible sciatic nerve injury is avoided by a periosteal elevator inserted between the muscle and the greater trochanter before division of the muscle. All patients showed marked improvement, and complications were virtually nonexistent. The etiology is probably associated with injections, but keloidal collagen disease may be important. A small incision is desirable because skin keloid formation invariably occurred in 200 cases followed for one to eight years.

Adolescent↗
PubMed2002

Congenital contractures, short stature, abnormal face, microcephaly, scoliosis, hip dislocation, and severe psychomotor retardation in two unrelated girls. a new MCA/MR syndrome?

Severe mental retardation, congenital contractures, short stature, microcephaly, ptosis, myopia, beaked nose, abnormal teeth, hip dislocation, and severe scoliosis, are described in a 16-year-old and an unrelated 24-year-old females. Results of all laboratory investigations were normal. Review of the literature, of the London Dysmorphology Data Base and POSSUM did not yield to any diagnosis. Whether these patients present a new MCA/MR syndrome is discussed.

Abnormalities, Multiple↗
PubMed2006

Recent observations in the biomechanical etiology of so-called idiopathic scoliosis. New classification of spinal deformity--I-st, II-nd and III-rd etiopathological groups.

The article examines the biomechanical etiology of so-called idiopathic scoliosis (AIS). It describes I-st, II-nd and III-rd etiopathological groups (epg) of spine deformity which were developed during the years 2001/2004/2005. All children with so-called idiopathic scoliosis had an abduction contracture of the right hip, often connected with a flexion and external rotation contracture. In other cases we found only limited range of adduction of the right hip in comparison to the left hip. We maintain that children with this real abduction contracture of the right hip constitute the first etiopathological group of the development of scoliosis (I-st epg). This group has an "S" double shaped scoliosis with the rib hump on the right. Other patients, with only limited adduction of right hip in comparison to the left hip, constitute the second etiopathological group of development of scoliosis (II-nd epg). This group has a "C" shaped lumbar, sacro-lumbar or lumbo-thoracic left convex scoliosis. The third etiopathological group (III-rd epg) shows either no or a minimal curve on X-ray with either no rib hump or a very minor one but have a "stiffness of spine". Such patients have problems with sporting activities and, as adults, the spinal stiffness leads to considerable "back pain". The right hip structural abduction contracture, or the differences in adduction, is connected with the "syndrome of contractures" in neonates and babies described by many authors and in depth by Mau. How does scoliosis develop? Our explanation is as follows. Asymmetry of movement of the hips during gait provokes asymmetry of loading and asymmetry of growth of both sides - left and right - and the gradual development of scoliosis. In I-st epg, the scoliosis is a secondary compensation for deformities in the pelvis and spine. The II-nd epg is linked to a permanent standing posture maintained on a free right leg during the first years of life. The III-rd epg comprises of patients from the boarder groups of I-st and II-nd epg. This classification establishes a clear therapeutic approach to every etiopathological group of scoliosis and allows for the possibility of introduction of causative prophylaxis.

Adolescent↗