In 1941, Madame Louis-Bar described a clinical syndrome characterized by a progressive cerebllar ataxia and telangiectasia of the skin and conjunctiva. In this syndrome ataxia is usually the first symptom, appearing at the time when the child starts walking. The ataxia is cerebellar and affects station, gait, and intention. The telangiectasiae have a later onset at about four to six years. We also have studied a case of eight-year old Japanese child with the ataxia telangiectasia.
Diastrophic Dwarfism was described in 1960 by Lamy and Maroteaux. This syndrome is characterized by short limbed dwarfism, club feet, distinctive deformity of the hands and external ears, cleft palate, progressive scoliosis sometimes combined with kyphosis, limitation of mobility and tendency to subluxation and dislocation of many joints. Case 1, T. I., a 17-days-old male, was admitted to the Adachi Gakuen, Crippled Children's Hospital. He was the product of 9-months pregnancy. He weighed 2800gr, at birth. The multiple deformities of the typical Diastrophic Dwarfism was found. He was treated with serial casts applied to feet and additional posterior release for equinus deformity.
Y. I. A boy 12yrs old had polyostotic fibrous dysplasia and cafe-au-lait pigmentation on the neck, back, inguinal area, buttocks. and only Alkaliphosphatase markedly increased in examination of blood, serum and urine. There were also no partiular abnormal findings in each pituitary, thyroid, adrenocortical function except a slight decreass of cortisol in blood. Precocious puberty was not found in this case as in the girl with Albright Syndrome.
1) One case of dysplasia epiphysialis punctata was described. 2) A boy, two months old, shows micromegalia, shortening of the upper extremities, stippled epiphyses, short neck, flat nose and varus deformities of the lower limbs. 3) Laboratory findings are almost within normal limits, except for decrease in 17 KS and 17 OHCS. Both parents are not syphilitic. 4) Histological examination of epiphysial cartilage showe no significant abnormality. 5) The etiology is not yet fully determined. 6) Valgus Osteotomy was performed for right tibia and fibula.
Roentogenographic observation has been made on bony changes in twenty-seven patients with renal failure during dialysis, and histopathological examination of bone specimens was carried out in six cases. Subperiosteal resorption of bone and radiolucent cyst-like lesion as seen in hyperparathyroidism were present in seventeen patients, focal and extensive demineralisation in seven, ricket-like lesion in five, periosteal thickening in two and ostesclerosis in one. Demineralisation of bone was predominantly found in the specimen from spine and femur in 5 cases. Bone biopsy of a rib with Villanueva tetrachrome staining (Frost) showed the evidence that the activity of bone formation and bone resorption were raised to a greater degree and osteoid seam markedly increased in as well as width.
A female, who died at 48 year-old, had been suffering from pain in back, joints and hypochondrium since around 32 years of age. A diagnosis of the tuberculosis of the both kidney was made, and she was admitted to another hospital. An x-ray examination revealed the gradual increase of radiolucency of the whole bone and the nephrocalcinosis. Laboratory findings were as follows: urine…average volume: 2500ml/day, pH: 6 to 7 specific gravity: 1.010 glucose: negative protein: 1+reaction serum…alkaline phosphatase: 30 to 60 King-Armstrong units inorganic phosphor: 2.1 to 3.4mg/dl etc. The bone was able to be cut with knife at autopsy. A case of osteomalacia without specific teratment, presumably caused by renal tubular acidosis, was reported.
Cases of spontaneous subarachnoid hemorrhage are rare and only a few cases have been reported in Japan. Our case is 38 year old male, worker. He suddenly complained of severe back pain radiating lateral chest during his work, on January 6th, 1973. Several hours later fluccid paralysis of both lower limbs developed, he was taken to our hospital. At hospitalization, absence of rectal sphincter tone, loss of sensory and motor faculty below the 5th thoracic spinal segment were noted. Spinal tap revealed bloody cerebrospinal fluid and myelogram showed complete block from the lebel of the 7th cervical to the 11th thoracic vertebra. Two weeks after accident operation for decompression was done and severe adheision of the arachnoid membrane due to subarachnoid bleeding was observed. Five months after operation he was able to walk by himself.
It is common knowledge that attempts to correct spastic hip-flexion deformity in patients with cerebral palsy often fail when established procedures, such as the Ober-Yount fasciotomy, the rectus femoris release, adductors myotomy and obturator neurectomy, the Campbell or Soutter muscle-slide operation, and iliopsoas tenotomy, are used. Because of the previous failures of correction of the spastic hip-flexion deformity after the usual operations, Bleck concluded that a method of controlled lengthening of the iliopsoas, termed iliopsoas recession, might give better results than simple tenotomy. Recently we also treated sixteen patients, twenty hips with spastic hip-flexion deformity by iliopsoas recession. It is the purpose of this article to report on the indication, method, and benefit of this procedure.
In a boy, aged 11 months, a marked hypoplasia of the iliac bone was found occasionally by X-ray when congenital club foot and flexion contracture of the knee in the same side were examined. On physical examination, no abnormalities of the pelvis could be noticed and the hypoplasia was thought to be caused by disturbed ossification of the iliac bone. The abnormalities were reported in detail and discussed briefly.
An ADL analyzing system built in the Beppu Rehabilitation Center is introduced. Sinepulse-camera system, steal-camera system, slit-camera system, foot print system, myograph system, their control system and data processing system were built in this system. The results on the gate analysis by the sinepulse-camera system were reported.
Seven cases of Polysyndactyly in the 5 patients are presented in this report. Author's technique for polysyndactyly of toe and its postoperative evaluation was discribed. The skin graft is not also essential for the web reconstruction between the 4-th and 5-th toes which united further to the PIP joint crease. A plantar rectangular pantaloon flop produces better results for the web space reconstruction.
Case 1. Forty-year-old housewife complained of moving pain in her left shoulder. She had no history of frequent injections into the deltoid muscle. Case 2. Sixteen-year-old high school girl came to our clinic with anterior dislocation of the left humeral head and restriction of the range of the shoulder motions. On examinations of both cases, moderate to marked contractures of horizontal extension and abduction of the shoulder joints were detected. The tight bands were palpable in the middle and posterior part of the deltoid muscles, which were released by excision of the fibrous bands. After surgery the shoulder movements were markedly improved.
55 year-old repairman had feeling of numbness and weakness of flexion in his right fingers. Muscle atrophy on his right thenar eminence and no sensory changes were noticed. EMG findings and subjective symptomes which were made worse with supination of the forearm suspected the pronator syndrome. Surgical exploration was taken because of no relief of 2 months' rest. The median nerve was compressed by tendinous thickening at the lower portion of humeral head of pronation muscle. But we couldn't find macroscopically the abnormal findings of the median nerve. Fortunately feeling of numbness disappered after operation. It seemed difficult case to diagnnsis from other differential diseases.
Changes of the elbow and the wrist joint were studied by analysing the indirect roentgenograms of 100 fellers using chain saws. The incidence of changes was significantly higher (p<0.01) in the wrist joint (16.3%) than in the elbow (10.9%). The occurrence rate of changes in the elbow was not correlated with the period of chain-saw-use, but rather with age of the individual, while, in the wrist joint no correlation with the both. The following changes were seen with comparatively high frequency: In the elbow, osteophyte formation (25.0%), spur formation at the olecranon (21.0%) and bone atrophy (13.5%). In the wrist joint, displacement of radial and ulnar articular surfaces (46.0%), bone atrophy with bone cysts (11.5%) and osteophyte formation (9.0%). The right elbow showed significantly higher incidence of subchondral sclerosis and bone cysts (p<0.05), and joint space narrowing and joint mice (p<0.01) than the left side. As to the wrist joint, no predominant difference of the changes between left and right was found.
Nine patients with osteochondritis dissecans of the elbow joint were seen at the Beppu National Hospital during past four years. Particular emphasis was given to a discussion of the history of the sports and results. In baseball there were seen most commonly, but two cases were seen in vollyball. Eight cases were treated by operative measure and one conservative measure. According the result, if the fragment remains in situ, then conservative measures are to be adopted particulary in children.
We have experienced five cases of children's meniscopathy. These are all congenital lateral discoid meniscus. It's most characteristic feature is decrease of movement; the more movement, the more pain and resistence. It is most important for diagnosis that existence of meniscopathy in children is known.
Since Lannelongue was probably the first to report “snapping knee” in 1879, many authors have described “snapping” of the knee caused by intra- or extra-articular lesions. In any orthopaedist's practice, it is usually caused by meniscus lesions. Some authors have tried to make a classification of “snapping knee”, but there are some cases which do not fall within any type in these classification. Therefore, we have classified “snapping knee” into five groups; 1) meniscal, 2) synovial, 3) osteocartilaginous, 4) tendinous, and 5) patello-femoral lesions. Eight patients have been operated on for intra- or para-articular masses which had produced “snapping” of the knee joint, but four cases had been preoperatively made a diagnosis of meniscus lesions. A summary of data in these cases is as follows: synovial fringe-two, hypertrophied alar fold-one, intra-articular lipoma-three, snapping tendon over the para-articular ganglion-one, and snapping tendon over the tibial exostosis-one.
In Japan, recently, acrylic cement is frequently used for fixing the prosthesis. But there are several death case-reports at implantation of bone cement in foreign countries. Experiments werecarriedout to identify sm the mechanicus of the cardiovascular effect of bone cement on dogs. We have found a falling blood pressure in the central circulation immediately after venous injection of methylmethacrylate monomer. And there are no drug preventing this change. We should pay attention to the falling blood pressure and the cardiac arrest, when the cement is implantated.
We report 18 cases of traumatic dislocation of the hip joints. According to Bray's classification, Type I 10 cases, Type II 3 cases, Type III 5 cases and except 2 cases of central dislocation, rest 16 cases were all posterior dislocations. Age varies from 18 to 72 (mean 39), Male 17 and female 1, Time interval from injury to reduction, 14 were within 24hr., the others were than 24hr., 17 cases were treated with closed reduction, only one with open reduction. Immobilization duration varies from 7-188 days, non-weight bearing from 13-218 days and follow-up duration 3-88 months. According to Japan Orthopedic Association ‘Estimation of osteo-arthritis of the hip joint’, results were almost good in Type I but in Type II and III more careful follow-up were needed.
Ten femoral heads (diver's disease: 1, caisson disease: 1, the idiopathic: 2, the steroid therapeutic: 2, the post-traumatic: 4) were obtained from the patients. To observe the changes of the osteocyte, the undecalcified sections were prepared. In the cases of idiopathic necrosis, the necrotic change of the osteocyte and the bone marrow was severe and it prevailed to almost all parts of the head. In the cases of steroid therapy, there could be found a wide spreading dystrophy of the osteocyte, but no remarkable changes in the bone marrow. The circumscribed osteocyte death resulting from the repairing of the pre-existing lesion were found in the case of the diver's, the caisson and the post-traumatic bone necrosis.
The results of seventeen dislocated hips, which have been followed up from eight to sixteen years after open reduction are as follows; 7 of them (41%) seemed normal condition in the joint, except very mild deformity of the femoral head, neck or acetabulum, roentgenographically, and they had no complaints symptomatically, and had normal R. O. M. Another 10 of them showed unsatisfactory results both roentgenographically and functionally. From these results, it is found that the important factors for satisfactory development of the hip is to make every effort to get concentric complete reduction of the femoral head in the operation and to maintain this condition by the use of additional proper procedures after the course of the operation.
Between 1955 and April 1970, 26 unilateral and 10 bilateral dislocations of the hip joint were treated by open reduction. This paper presents an interim result of these 36 cases with congenital dislocation of the hip. The first hip was operated twelve years and mine months ago. the most recent one in this seriese, 3 year's ago. Time and technique of the operation, age at the time of operation and methods of fixation after the operation are discussed. The results of 36 hip operative reduction were analysed. Six hip (16.7%) were good, 25 hips (69.4%) were fair and 5 hips (13.9%) were reluxations.
1. Three cases of dysplasia epiphysealis hemimelica are reported and the literature is briefly reviewed. 2. The course of these cases is described and discussed. 3. It is stressed that the treatment of this disease must be individualized depending on the amount of deformity and pain.
A case of eosinophilic granuloma in the left clavicula is reported. A boy aged 4. was admitted to our clinic on August 1972, complaining of pain and swelling in the left clavicular region. The rentogenographic examination revealed a round lytic lesion of the cortex in the proximal end of clavicula with excessive periosteal reaction. At operation the cortex showed partial destruction although pathological fracture was not seen. The lesion was completely removed and its appearance was a raddish-tan solid granulation. Smears and culuture of curettage material were negative for pathogens. Pathological diagnosis was eosinophilic granuloma. Five month after surgery a radiograph showed the lesion to have healed almost completely.
For induction of experimental bone tumor, newborn Wistar-King-Aptekman or wistar-Lewis rats were received the intraperitoneal injection of the virus suspension. The samples from the induced bone tumors were examined by histochemical procedures on mucopolysaccharides, nucleic acid, lipid, non-specific alkaline phosphatase and acid phosphatase. The tumor cytoplasm showed the intensive activity of alkaline phosphatase. Lipid contents and glycogen were scanty. The histochemical findings of the induced tumor in rats were almost similar to that of osteoblastic type of human osteosarcoma.
“Multiple Intraosseous Lipoma” is a rarest benign tumor of bone. There were five reported cases in Japan. A twenty-five-year-old female visited our clinic because of the right ankle joint pain. Roentgenographic findings were irregular translucent areas in the right talus, the cuboid bone and the distal part of the fibula. Following roentgenographic survey revealed same findings in the left same bones, the left navicular bone, the bilateral carpal bones, metacarpal bones, phalanxes, metatarsar bones and the left patella. Curettage and auto-iliac-bone graft was performed to the right talus, cuboid bone and the distal part of the fibula. A mass of fatty tissue was encountered within the medullary cavity. Microscopically, the majority of the tissue consisted of mature adipose elements with delicate fibrous stroma.
Tumors arising specifically from the peripheral nerve trunks and their branches are relatively infrequent. Recently we have had a case of neurinoma arising from thoracic nerve. The tumor was removed from the back of a 26-year-old man. Microscopically the field is composed mixture of Antoni type A and Antoni type B. By electron-microscopy, the tumor cells have spindle-shaped nuclei and irregular cytoplasmic processes, and are surrounded by basement membrane. Small number of collagen fibrills are present between cells and “long spacing collagen” with approximate 2000 Angstroms periodicity is occasionally observed in the matrix.
Two cases of fibroxanthosarcoma are reviewed. Case I. A 54-year-old woman had a painless elastic mass removed from her left thigh. Tumor cells were composed of fibroblasts, histocytes and giant cells. Fibrous elements were arranged in a storiform pattern. She died of cachexia sixteen months after initial surgery. The autopsy showed metastases and infiltrations in lungs, diaphragma, pleura, rectum and pelvic cavity. Case 2. A 49-year-old man had a painful soft mass removed from his right thigh. Histological findings were same as case I. He was treated by wide local excision. Metastasis and recurrence have been not observed three months after operation.
1. Our case had typical clinical features and physical sings, and so we had a preoperative diagnosis of the haemangioma of the knee joint after the pneumo-arthrography. 2. Present case was a localized, intermediary and caveranous type of the synovial haemangioma of the knee joint, and it was characteristic that the adjacent synovium had villonodular projection in a variety size. We think, the transeformation was caused by frequent bleeding in a joint cavity. 3. Our method of a pneumatic tourniquet is useful in the excision of haemangioma, because it is possible to find the entity of the tumor without miss of a mass and without bleeding.
The clinical courses and the histological findings of Juxtacortical bone tumor in 4 cases were presented. Two of them, 27-year-old and 23-year-old female, were confirmed to be parosteal osteosarcoma by biopsy. They refused amputation, and the former is still alive 7 years and the latter 3 years after the irradiation therapy. Although the other two cases, 22-year-old male and 31-year-old female, were clinically suspected to be parosteal osteosarcoma, the biopsy revealed that the former was parosteal osteoma and the latter well-differentiated chondrosarcoma, and en block resections were carried out.
The benign chondroblastoma was given its present name by Jaffe and Lichtenstein in 1942. We have experienced a case of this tumor involved in the patella. The patient, 14-year-old boy, was admitted to our clinic, complaining of pain and swelling of the patella. Roentgenograms revealed a multiloculated radiolucent area with cortical destruction in the patella. Curettage and packing of the cavity with bone chips was performed. Microscopically, there were rounded or polyhedral stroml cells and benign multinucleated cells. Areas of calcification were seen in the chondroid matrix. The patient was asymptomatic six months after operation.