Osteoarticular manifestations of congenital insensitivity to pain with anhydrosis.

We report the case history of a boy who suffered from congenital insensitivity to pain with anhydrosis. We discuss the orthopaedic disorders occurring in 21 cases reported in the literature.

Idiopathic carpotarsal osteolysis with Bartter's syndrome. A case report and review of the literature.

Idiopathic carpotarsal osteolysis is 1 of the rare types of disappearing bone diseases characterized by painful limitation of movement and deformity of the wrist and tarsal joints, and associated with frequent episodes of joint inflammation and a radiographic appearance of osteolysis. Reported here is an 8-year-old girl with idiopathic carpotarsal osteolysis and Bartter's syndrome with significantly decreased re-absorption of chloride at distal renal tubules, low level of serum potassium, a metabolic alkalosis, and high levels of aldosterone and renin. Bartter's syndrome associated with idiopathic carpotarsal osteolysis has not been published previously. The typical plantar nodule in this patient confirmed the presence of fibromatosis with aggressive histologic signs in light and electron microscopic examination.

[Behavior of adamantinoma of the long bones based on long-term follow up studies]. / Csöves csontok adamantinomáinak viselkedése hosszútávú utánkövetés alapján.

Authors report on the long time (in average 12 years, range: 5-36 years) follow-up results of 5 cases of adamantinoma, localized on the tibia. In one case recurrence was found very late, 20 and 36 years after the primary wide resection, and resection was repeatedly performed. Because of problems of differential diagnosis in one case the tumor was excised intralesionally (curettage + plasty with cancellous bone). 7 years later the persistence of the process was found only. 1 patient died in consequence of pulmonary metastasis 9 years after the primary operation. Wide resection is suggested both for the removal of the primary tumor and the recurrences, appearing very late. For the reconstruction of the bone autologous fibula is proposed. Adamantinoma is thought to be a low malignity tumor, the outcome of which cannot be predicted from the clinical and histological findings. Considering the late recurrences and metastasis a long range, minimally 10 years, following of the patient is thought to be necessary.

[Experience with surgical treatment of osteosarcoma]. / Osteosarcomák sebészi kezelésével szerzett tapasztalataink.

The authors treated 79 patients with osteosarcoma by surgery and chemotherapy 1975 through 1991. The appearance, localization and differential diagnosis are discussed. Attention is called to the doctor's delay. The effective pre- and postoperative chemotherapy decreased the incidence of the lung metastases and increased the survival rate (71% 3 years overall survival and 41% 5 years survival) and made the limb-sparing surgery possible. The development of the reconstructive surgical technique and new designs of tumor endoprostheses enabled us to perform limb-sparing surgery in more than one third of the cases at the same survival chance. The limb-sparing means a decisive change in the quality of the rehabilitation of the osteosarcoma patients.

Aneurysmal bone cyst. A review of 52 primary and 16 secondary cases.

Authors report on the results of treatment of 52 primary and 16 secondary aneurysmal bone cysts (ABC). ABC grow rapidly; 84% of them have already destroyed more than the half of the bone width at recognition. En bloc resection is preferred when the ABC is growing superficially and eccentrically and more than half of the bone width is intact. Careful curettage and bone grafting still remains the surgical method of choice in the majority of cases, when the ABC is more destructive and affects the subchondral bone of the joints. Segmental resection is only indicated when removal of the affected bone does not influence the function of the extremity. Superselective embolization of the cyst was performed in seven cases with excellent results. This method is suggested for ABC in certain locations inaccessible to surgical intervention, e.g., the pelvis, or to avoid excessive bleeding in hypervascularized tumors. In one case, however, an incomplete rebuilding of the ABC could only be achieved by the administration of calcitonin. The 16 cases of secondary ABC were observed mostly in association with osteoblastomas, giant-cell tumors, and osteosarcomas. The incidence of the secondary ABC was 23% in the whole ABC group but not more than 2-4% among the osteosarcomas and giant-cell tumors. Secondary ABC may confuse the histological and clinical diagnoses and that, especially in cases of osteosarcoma, may have fatal consequences.

Surgical treatment of the hip in cerebral palsy.

Hips are affected generally in CP but in favourable cases we can prevent dislocation by adductor tenotomy performed in time (530 cases). Posterior transposition of the origins of the adductor muscles is performed if the patient walks with inwood rotated inferior extremities (105 cases). In the treatment of real subluxation and valgus deformity the open tenotomy of adductor muscles and femur osteotomy with derotation and varisation were used (180 cases). We accomplish open reduction in frank dislocation (35 cases). In such cases it is often necessary to shorten the femur. Also normal acetabulum is found in elder children.

[Experience with methylprednisolone-acetate therapy of juvenile bone cysts]. / A juvenilis csontcysta methylprednisolon-acetáttal történö kezelésével szerzett tapasztalataink.

Authors injected until 1989 in 92 patients with juvenile bone cyst Methylprednisolone-acetate locally after punction of the cyst. They report on the results and give a short literary review.

[Development in the treatment of congenital dislocation of hip (author's transl)]. / A veleszületett csípöficam kezelésének fejlödése.

Authors review the development in the treatment of congenital dislocation and dysplasy of hip and demonstrate the results with various methods. Results with treatment according to Lorenz (1040 cases) were unsatisfactory because of frequent occurance (66.3 percent) of osteochondritis. The treatment with Frejka cushion (360 cases) did not improve basically the results. Pavlik's strapping, especially when started under 1 month of age gave excellent results (3156 cases). In operative treated the results of 332 Colonna operations were unsatisfactory because of consecutive deformation of the hip and restriction of movements. Open reduction with varisation derotation osteotomy (474 cases) provided good acetabular development especially in cases where articular cartilage and upper limbus could be saved. Salter's operation is successful when proper indications are considered, but should be performed only after 1--2 years of observation of the acetabular development in non improving cases. The Chiari procedure can improve the weight bearing surfaces in cases of steep acetabular roof flat acetabulum and consecutive subluxation. In 557 cases of varisation derotation osteotomy subcapital coxa valga occured frequently. Basic principle of treatment remains prevention, early recognition and treatment after birth.