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Biomedical subjects

S Ryöppy

Publications and source records attributed to S Ryöppy.

18 recordsLinked to original sources

Foot deformities in diastrophic dysplasia. An analysis of 102 patients.

The exceptionally high prevalence of diastrophic dysplasia in Finland has enabled us to analyse the foot deformities of 102 patients at their first orthopaedic evaluation and classify 204 feet into five categories. The most common finding (43%) was a foot with tarsal valgus deformity and metatarsus adductus; 37% showed either equinovarus adductus (29%) or equinus (8%) deformities. At the first examination 13% showed metatarsus adductus deformity alone, and 7% were clinically normal. The expression 'club foot', generally used for the foot deformity in diastrophic dysplasia is a misnomer. There is a wide spectrum of deformities, some of them specific for the condition.

Adolescent

The spine in diastrophic dysplasia.

Diastrophic dysplasia is an autosomal recessive disorder of the skeleton, characterized by disproportionate short stature, generalized joint deformities, club feet, deformed ear pinnae, and, frequently, spinal deformity and cleft palate. Diastrophic dysplasia is more common in Finland than elsewhere. We studied 101 patients with an age range from newborns to 79 years to find out the frequency and type of spinal deformities, the early signs of progressive cases, and to follow the natural history of the disease. In the follow-up study, 17 patients were under 10 years, 21 under 21 years, and 63 over 21 years of age. One-third of the patients had cervical kyphosis; in the most severe case the kyphosis was 180 degrees and led to quadriplegia during anesthesia. In three patients, cervical kyphosis resolved spontaneously before the age of 5 years. The overall frequency of scoliosis was 37%; 49% in women and 22% in men. Only 13 patients had curves greater than 50 degrees; these curves constituted distinct rotation at the apex from the early evolution of the curve. The early signs of severe curves were detectable at the age of 2 to 4 years. Only two patients were operated on because of scoliosis; one with fusion in situ and the other instrumented with the pediatric Cotrel-Dubousset instrumentation. Three patients had a brace, which did not prevent the progression of the curve. Symptoms referring to a narrow spinal canal were registered in four patients, two of which were operated on; a lumbar posterior decompressive procedure was made at adult age.

Adolescent

Twenty-six early operations in brachial birth palsy.

26 babies with brachial birth palsy were operated on at the age of 12-275 days (mean 75 days) in paediatric surgical departments of Helsinki (12 cases) and Turku (14 cases) University Central Hospitals during years 1970-1985 using microsurgical techniques. Epineuroraphy was used in 9, interfascicular nerve grafting in 7, neurolysis in 7, and intercostal neurotisation in 3 cases. The functional status of the upper extremity was recorded 1 to 14 years after the operation using a modified Mallet's classification: 6 were good, 17 fair and 3 poor. The outcome after epineuroraphy was similar to interfascicular grafting. One intercostal neurotisation resulted in fair function and 2 in poor, but the result in target function, i.g. in the peripheral neurotised nerve, was good.

Birth Injuries

Ten years' experience in patients with osteogenic sarcoma in Finland.

Ten years' results of 56 patients with high grade osteogenic sarcoma are reported. Fifty-two patients had M0 disease. Immediately after open biopsy the patients were treated with chemotherapy using modified Rosen's protocols T4, T7 and T10. The primary tumor was adequately removed in most patients. Six children were treated with limb saving. The actuarial and disease-free survival was 80% after 1 year, and 73% to 8 years. Two patients died because of toxic side effects of chemotherapy, one of septicemia, the other of late cardiac failure secondary to doxorubicin.

Actuarial Analysis

RAPADILINO syndrome with radial and patellar aplasia/hypoplasia as main manifestations.

A new malformation syndrome is described in a pair of sibs and 3 sporadic patients. The characteristic manifestations are radial aplasia or hypoplasia, absence of thumbs, absent or hypoplastic patellae, dislocations of joints, unusual face, cleft or highly arched palate, diarrhea in infancy, small stature, and normal intelligence. Recessive inheritance seems the most plausible cause. The acronym RAPADILINO syndrome is proposed.

Adolescent

Chronic osteomyelitislike disease with negative bacterial cultures.

During a seven-year period we observed 14 children who had chronic osteomyelitislike disease. The bacterial cultures from the bone lesions were negative. In eight patients the findings were compatible with chronic recurrent multifocal osteomyelitis (CRMO), in four the findings were compatible with chronic sclerosing osteomyelitis of Garré, and two had osteomyelitis of the clavicle. In patients with CRMO, lymphocyte subpopulations, the responses to mitogens, and the chemotactic and chemokinetic responses showed no consistent abnormalities. After a mean follow-up of 4.5 years (range, one to ten years), all four patients with osteomyelitis of Garré were symptomatic, and two had complications. Only two of the eight patients with CRMO had active disease. The course had been complicated by growth disturbances in one patient and by thoracic outlet syndrome in another. Wegener's granulomatosis later developed in a patient with CRMO.

Adolescent

Transient synovitis and Perthes' disease. Is there an aetiological connection?

A prospective study was made of 119 children with transient synovitis or any other cause for synovial effusion and elevated intra-articular pressure. During a follow-up of one year not one case of Perthes' disease was diagnosed and the late clinical and radiographic changes were minimal with moderate overgrowth of the femoral head in 33% and widening of the joint space in 14.2%. Our results do not support the widely accepted concept that Perthes' disease develops as a result of the period of elevated intra-articular pressure found in transient synovitis. Further research into this and Perthes' disease should follow the premise that they are two different diseases without any aetiological connection.

Acute Disease

The management of primary acetabular dysplasia. Its association with habitual side-lying.

Fifty-one infants with limited abduction of the hip and acetabular dysplasia were, between 1969 and 1975, treated with abduction-adduction exercises, administered by the parents; no abduction devices were used. In 1983 a follow-up examination was carried out on 41 of these patients. Although at birth these children had characteristics similar to patients with congenital dislocation of the hip, none of their hips dislocated. At birth acetabular measurements showed that half the children had severe dysplasia and the other half slight dysplasia; the difference between the affected and the healthy hips was significant. At follow-up the gait was normal in all the patients. Movements at the hips were symmetrical and within normal limits in all but one patient. The acetabular angle, the centre-edge angle, the shaft-neck angle, the hip ratio, and the size of the femoral head were the same on the previously affected side as on the normal side. Nineteen of the children followed up had preferred lying on one side and dysplasia of the upper hip had developed. The significance of the sleeping position on the development of acetabular dysplasia is discussed.

Acetabulum

Ultrasonography in hip disease in children.

Intra-articular synovial effusion was visualized in different juvenile hip diseases by ultrasonography; 166 hips from 149 children were examined. Joint aspiration of 97 hips confirmed that ultrasonography was more sensitive than conventional radiography in diagnosing effusion. The magnitude of the ultrasonic joint space correlated well with the clinical severity of the disease, the volume of synovial fluid, and the intra-articular pressure. Considerable widening of the ultrasonic joint space was seen in transient synovitis, septic arthritis, reactive arthritis and arthritis with urticaria; moderate widening was seen in some patients with Perthes disease, and symmetrical joint space in patients with nonspecific arthralgia. We conclude that ultrasonography is valuable in the diagnosis and follow-up of synovial effusion of the hip in children.

Adolescent

Fractures of the proximal femur in Finland in 1975.

Hospital admissions due to fractures of the proximal femur in Finland increased 50% between 1968 and 1976. In this study the established differences and results of treatment in regional and district hospitals are reviewed. From the mortality incidence and the length of hospitalisation, the results were better in regional than in district hospitals, and better for operatively than for conservatively treated patients. Of the different operative methods, the best primary results were obtained with endoprosthetic replacement and osteosynthesis with a fixed-angle nail plate.

Adult

[Osteomyelitis].

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Adult

Oculodentodigital dysplasia syndrome. Report of four cases.

Four cases of oculodentodigital dysplasia are reported. Three cases are from the same family, father and two daughers. These three cases have the characteristics typical of this disorder: narrow nose, hypoplastic alae nasi, microphthalmia, defects of the teeth, syndactylyl of the IV and V fingers, and skeletal anomalies. The fourth case differs from the earlier reported cases; he has all the typical findings of oculodentodigital dysplasia but in addition he shows features not previously reported, namely exceptionally poor vision, mental retardation, monilethrix and pili annuli changes of the hair.

Abnormalities, Multiple

[Osteosynthesis].

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Bone Nails

Early semiclosed intramedullary stabilization in osteogenesis imperfecta.

Fourteen patients with severe osteogenesis imperfecta (OI) were treated operatively by closed or semiclosed intramedullary stabilization, with nonextensible rods and nails. Altogether, 55 bones (both upper and lower limbs) were stabilized primarily. In infants with a severe form of OI (Sillence classification), the operative technique was modified significantly from those presented earlier in the literature. Early intramedullary stabilization, even soon after birth, seems justified in selected patients with severe OI. The possibilities for motor development are improved and later insertion of telescoping nails becomes easier.

Bone Nails

The knee in arthrogryposis multiplex congenita.

The functional, radiological, and ambulatory status of 30 patients (58 knees) with arthrogryposis multiplex congenita (AMC) were analyzed after a follow-up of 1-36 years. The muscular imbalance presented in many of these patients corresponded well to the type of deformity. The primary treatment was nonoperative in most cases. In spite of the initial promising results, a significant number of patients underwent operation for residual symptoms. Treatment of flexion contractures seems to be more discouraging than the treatment of extension contractures. On the other hand, the risk for degenerative arthritis seemed to be elevated, especially in the extension-contracture group.

Adolescent