I. Katsuki

Transtrochanteric rotational osteotomy of the femoral head for the treatment of osteonecrosis. Follow-up statistics.

Transtrochanteric rotational osteotomy of the femoral head was technically established as a surgical procedure in 1972. This procedure has been successfully performed on 250 hips in 204 patients. In the treatment of idiopathic and steroid-induced osteonecrosis of the femoral head, the results of 128 hips in 90 patients followed two to nine years were reviewed. In 98 of 128 hips, excellent results were obtained clinically and roentgenographically. Progressive collapse in the newly created weight-bearing area occurred in 25 hips where the lesions had been extensive. In 35 of 39 Grade I or II hips, excellent results were obtained in every respect (success rate: 90%). Following the analysis of results on the preoperative lateral roentgenograms of the femoral head, 76 of the 80 hips in the group in which the intact area was greater than one third of the total articular surface showed no collapse of the newly created weight-bearing area, thus accounting for a 95% success rate. In cases when a further collapse was prevented, the necrotic lesions were smaller at follow-up. Excellent results were obtained when the osteotomy was performed during the early stage of necrosis and in the absence of an advanced collapse, even in extensive lesions. In advanced cases with an extensive lesion, adequate anterior rotation is essential and intentional varus position should be designed. These results suggest that this procedure will inhibit progressive collapse of the femoral head and restore the joint more effectively than other surgical procedures.

Intermediate form of osteopetrosis with recessive inheritance

The clinical and radiographic features of the intermediate form of osteopetrosis in two sibs are presented in which the disorder appears to have been inherited as a recessive trait. Although this form of osteopetrosis has been poorly delineated, its recognition is practically important in order to give an accurate prognosis. This paper also presents an unusual complication of bilateral avascular necrosis of the femoral head in the younger sib. Radiographic changes of the femoral heads suggest those of Legg-Calvé-Perthes disease, though the possibility of avascular necrosis following unrecognized femoral neck fracture is not completely excluded.

Spondyloepiphyseal dysplasia tarda with progressive arthropathy

The clinical and roentgenographic manifestations of six cases suffering from spondyloepiphyscal dysplasia tarda with progressive arthropathy are presented. The roentgenographic features consist of generalized platyspondyly and varying degrees of epiphyseal involvement with conspicuous enlargement of both ends of the short tubular bones of the hands. This disorder appears at an earlier age, and is more crippling than the usual form of spondyloepiphyseal dysplasia tarda. It is suggested that orthopaedic measures are essential for all patients with this disorder, as profound disability may be expected by progressive involvement of the major joints.

Phalangeal microgeodic syndrome in childhood: Report of seven cases and review of the literature

Seven cases of phalangeal microgeodic syndrome in childhood are reported. Reviewing the literature, we have found a total of thirty Japanese cases, including the cases of our own. On the other hand, only ten cases have been reported outside Japan. The mean age of onset of the Japanese cases was six and a half years, which is older than that in the initial description. Of further interest is the fact that all the cases in Japan occurred sporadically and exclusively in winter, and regressed spontaneously and fairly rapidly within six months. Furthermore, most of the cases reported in Europe also occurred in the winter months. These facts imply an etiological relationship between the lowered temperature and the bone lesions.

Generalized enchondromatosis with unusual complications of soft tissue calcifications and hemangiomas

Generalized enchondromatosis is a newly delineated type of enchodromatosis. Radiographically there are multiple enchondromata in almost all metaphyses of the long and short tubular bones and the lesions are in almost the same stage of development with mild platyspondyly and skull deformity. The pelvic changes are characteristic and, together with the metaphyseal changes of the long and short tubular bones, are probably diagnostic of this disorder. The present case demonstrates advanced radiographic features of this disorder associated with unusual soft tissue calcifications and hemangiomas. The presence of hemangiomas in our case as well as three cases in the literature suggests this feature is more than a coincidence.

A Case of Pelvic Ring Fracture in a Child

今回，我々は非常に稀な小児の骨盤輪骨折を経験したので報告する．症例は9才男児，停車中の軽自動車の後ろで遊んでいたところ，車の下敷きとなり当院へ救急搬送された．骨盤部CTを施行したところ左仙腸関節脱臼骨折及び後腹膜内出血を認めた．緊急血管造影を施行したが，明らかな動脈性出血は認めなかった．仙腸関節脱臼の離開は軽度であり，患側からのスポンジ牽引と骨盤部固定装具を装着し保存的治療を行った．受傷2週目より仙腸関節近傍に仮骨を認めた．受傷10週目より全荷重で歩行を開始したが骨盤部に疼痛の残存なく，自宅退院した．仙腸関節脱臼は一般的に骨折ではないため仮骨による骨癒合は起こりにくいと言われている．しかし，今回の症例は小児であり受傷早期より仮骨を認め，疼痛の残存無く治癒した．

Hurler-Scheie phenotype with parental consanguinity. Report of an additional case supporting the concept of genetic heterogeneity

The Hurler-Scheie phenotype in a 27-year-old woman of first-cousin parentage is possibly the first reported in the orthopedic literature. The patient exhibited short stature, coarse facies, corneal clouding, multiple stiff joints, normal intelligence, and a long history of bilateral carpal tunnel syndrome, which has not been relieved after operation. The irreversible nerve damage was apparently produced by the marked thickening of the transverse carpal ligament. Surgical findings in this case and data from published reports emphasize the need for early surgical treatment of the associated carpal tunnel syndrome in patients with the Hurler-Scheie phenotype. Parental consanguinity present in this patient is further evidence supporting the concept of a third mutant allele different from both the Hurler gene and the Scheie gene.