An Adult Moyamoya Disease Confirmed by Ring Finger Protein 213 Gene Test and Radiological Studies

A 63-year-old female complained of transient dysarthria. MRA was conducted to evaluate this symptom, revealing distal internal carotid artery occlusion with collateral vessel development, suggesting Moyamoya disease, which had not been detected in MRA performed 5 years previously. Vascular risk factors and laboratory findings suggested no cardiac or autoimmune diseases. The diameter of stenosis of the middle cerebral artery on high-resolution MRI was 2.11 mm, and genetic evaluation revealed mutation of the gene encoding ring finger protein 213 (RNF213). High-resolution MRI and gene studies are useful for distinguishing between Moyamoya disease and atherosclerosis.

The Significance of Teardrop Changes in Developmental Dislocation of the Hip / 대한정형외과학회잡지

Prediction of acetabular development after reduction in treatment of developmental dysplasia of thc hip (DDH) is earlier, the hetter results because it would help ensure optimal timing of additional procedure if necessary. In this respect, authors reviewed retrospectively the radiographs of the hips of 35 children with DDH who had unilateral involvement and treated hy senior author (S.H.Lee) from the heginning with single successful attempt of reduction. The radiographs which were made at the time of initial diagnosis, one, two year nfter reduction und final follow up were assessed of teardrop figures. The results of treatment were classified as satisfactory group(CE > 10degrees ) and unsatisfactory group(CE < 10degrees) judged hy center-edge angle(CE degrees) at final follow-ups. 1. The teardrop figures were classifiable into 4 distinct groups as i)absent. ii)V-shaped, iii)Ushaped, iv) inverted D-shaped. 2. The teardrop figures in normal sides of hip were all U-shaped. 3. In dislocated but with satisfactory result group(24 cases), absent at 2 cases(8%), U-shaped teardrop was seen at 13 cases(54%), V-shaped in 9 cases(38%), and inverted 2-shaped in 0 case at I year after reduction. 4. In dislocated but with unsatisfactory result group( 11 cases), they were mostly of V-shaped(7 cases, 64%). The rest were of ahsent in 4 cases(36%) . hut none of U-shaped and inverted 2-shaped. In conclusions, teardrop figures appeared as significant predictor of future development of hip joint. Teardrop figure which stay as V-shaped at one year after reduction seems suggestive of insufficient reduction of DDH, therehy calls for early additional procedure.

Physeal Injuries in Association with Severe Osteoporosis / 대한정형외과학회잡지

The physeal injuries in children occur result of trauma, infection, metabolic or ischemic disease, and these conditions cause significant problems, such as growth disturbance, deformity and premature closure of physis. The authors experienced 7 cases of children who had angular deformity of knee and limb shortenings, but no history of obvious causes of physeal injury. All of these cases had severe osteoporosis and underlying disease, which were osteogenesis imperfecta, fibrous dyplasia and long term steroid therapy. The purpose of this study are evaluation of pathomechanism of physeal injuries in severe osteoporotic children. Patients were range of age from 4 to 9 years and had common clinical features, which were angular deformities of knee, limb shortening. Also they had common radiographic findings, that were severe porosis, metaphyseal sclerosis and in some cases, pop corn like calcification around physis. At this, we supposed that chronic mechanical stress in osteoporotic bone make physis be damaged due to loss of capillary circulation and this event appear to result from collapse of bone plate, through which epiphyseal vessel penetrate. In conclusion, the children with severe osteoporosis should be observed carefully about physeal injuries, which pathomechanism are perhaps deprivation of capillary circulation through bone plate. Therefore, We should try to protect bone plate by being conscious of its existence.