Chordoma is a rare distinctively malignant tumor thought to be histogenetically related to the primitive notochord, It is locally infiltrative but does not metastasize until late. The usual initial symptom is pain or some symptom caused by involvement of the nervous system. Involed in the order of frequency are the sacro-coccygeal area, the spheno-occipital area, and the other vertebral area. This case is a sacro-coccygeal chordoma that is confirmed by histologic examination. The lesion was treated by complete excision.
Chordoma ; Nervous System ; Notochord

Trichorhinophalangeal syndrome (TRPS) was first described in 1966 by Giedion. It is a rare genetic disease and divided into two groups : TRPS type I and TRPS type II. TRPS type I is an autosomal dominant inherited disorder, defined by three characteristic findings : first, sparsely distributed brittle and slow-growing scalp hair, second, a bulbous pear-shaped nose with an elongated philtrum, third brachydactyla with angular deformities. The characteristic radiological features of the hands include cone shaped epiphysis of the proximal interphalangeal joints. Most TRPS type II is sporadic and shows multiple exosteosis, mental retardation and microcephaly in addition to the three chracteristic findings of TRPS type I . A 20-year-old female visited our department for the evaluation of her sparse, slow-growing scalp hair. She also had a bulbous pearshaped nose, ulnar deviated fingers and shortened both hallux. Some relatives of patients show similar symptoms of scalp hair. A Radiologic aly, histopathologic aly and chromosomal study were done, and we established the diagnosis, TRPS type I.
Congenital Abnormalities ; Diagnosis ; Epiphyses ; Female ; Fingers ; Hair ; Hallux ; Hand ; Humans ; Intellectual Disability ; Joints ; Lip ; Microcephaly ; Nose ; Scalp ; Young Adult

We made a randomized prospective comparison of the compression hip screw and the Gamma nail for the internal fixation of 60 intertrochanteric fractures to compare perioperative details and to analyze radiographic and clinical results in elderly patients. The operation time for Gamma nail group was shorter than for CHS group and also the intraoperative blood loss was lower in the Gamma nail group. And the Gamma nail enabled earlier ambulation. We found no significant difference of the union time and the sliding length of lag screw between two groups. The decrease of neck shaft angle in the Gamma nail group was significantly smaller than the CHS group. There was no significant mechanical complication in the Gamma nail group such as a fracture of the femoral shaft or fixation failure. We obtained satisfactory results on treatment of elderly patients with intertrochanteric fracture of femur using Gamma nail. So we consider that in the treatment of intertrochanteric fractures of geriatric patients, the Gamma nail would be more efficient than the CHS.
Aged ; Femur ; Hip Fractures ; Hip ; Humans ; Neck ; Prospective Studies ; Walking

No abstract available.
Animals ; Atrophy* ; Electric Stimulation* ; Muscle, Skeletal* ; Rats* ; Sciatic Nerve*

Syndromal acanthosis nigricans is associated with cellular resistance to the action of insulin and other endocrinopathies. In the insulin resistant state, increased insulin binds to the receptors of insulin-like growth factors (IGFs). IGFs are growth prompting peptides, which act as direct mitogens on cells containing the receptors. Through these actions, increased cellular growth and metabolism cause the skin lesions of acanthosis nigricans. Hyperandrogenism is also found in syndromal acanthosis nigricans patients. When associated with insulin resistance, these states are proportionally related. Increased insulin prompts the synthesis of androgen and conversely, increased androgen elevates the insulin resistance. We present three cases of syndromal acanthosis nigricans with hyperpigmented skin lesions and obesity. Laboratory findings demonstrated normal fasting blood suga levels with increased insulin and C-peptide levels and decreased insulin receptors. The oral glucose tolerance test was normal. We suggest that these patients were in an insulin resistant state. Skin biopsies from the axilla in all three cases showed hyperkeratosis, irregular acanthosis and upward projection of dermal papillae as finger like fashion. Thus all three cases represent syndromal acanthosis nigricans associated with an insulin resistant state based on clinical, histopathological, and laboratory findings. The three patients were treated with dietary control.
Acanthosis Nigricans* ; Axilla ; Biopsy ; C-Peptide ; Fasting ; Fingers ; Glucose Tolerance Test ; Humans ; Hyperandrogenism ; Insulin Resistance* ; Insulin* ; Metabolism ; Mitogens ; Obesity ; Peptides ; Receptor, Insulin ; Skin ; Somatomedins

No abstract available.
Humans ; Infant, Newborn* ; Pneumonia*

Neoplastic disease of the spine can be a most difficuIt and perflexing problem. Nineteen cases of tumors in the the spine were studied in the Department of Orthopedic Surgery, Seoul National University Hospital for 5 years and 6 months from January 1972 to June 1977. The results were as follows; 1. In 19 cases of tumors of the spine, primary tumor was 6 cases and metastatic tumor was 13 cases. 2. Primary tumors of the spine show hemangioma, osteochondroma, chondrosarcoma, giant cell tumor, chordoma, and chondromyxoid fibroma with each of one case 3. Primary sites of the metastatic tumors were lung (2 cases), genito-urinary system (2 cases), gastro-in testinal tract, skin, bone (each l case), and unknown (5 cases). 4. Thoracic and lumbar spines were most fregeuntly involved (75%). 5. Primary tumors were treated by radical excision and metastatic tumors by consevstive 6. Tumors of the spine must be differenciated from any diseases of the spine and the clinician must be alert that any spine lesion could be tumorous condition.
Chondrosarcoma ; Chordoma ; Fibroma ; Giant Cell Tumors ; Hemangioma ; Lung ; Orthopedics ; Osteochondroma ; Seoul ; Skin ; Spine

Treatment of infected nonunions of long bone is confronted with various difficult problem, e.g. eradication of infection and osseous union even in the presence of devastating infection. To solve this problem various method were proved including exerternal fixation, electrical stimulation and functional casting method with some success. Intreating these difficult infected nonunions, we adopted another program which includes through debridment of the infected and necrotic soft tissue and bone, autogenous bone graft, rigid internal fixation of fragments and ope.n drainage of the wound until union of the fracture. We treated 8 cases of infected nonunions with this method from march, 1980 to september 1983 and results are as follows: 1. Satisfactory union was obtained within average 5 months. 2. In some cases pus drainage continued even after osseous union but it was easily controlled after removal of fixation devices. 3. After bone union and removal of internal fixation devices, some kind of protective measures, e.g. crutches and braces were used for about 6 montes to prevent refracture. 4. In conclusion our method is considered to be an effctive means is treating infected nonunion of fractures.
Braces ; Crutches ; Drainage ; Electric Stimulation ; Internal Fixators ; Methods ; Suppuration ; Transplants ; Wounds and Injuries

No abstract available.
Fracture Fixation, Intramedullary*

The purpose of this study is to evaiuate the results of the reconstruction of acetabular bone defect with morselized bone graft and screw fixed hemispherical cup in revision of failed acetabular cup. Thirty four revisions of an acetabular component that had been performed in 34 patients between 1988 and 1993 at SNUH, and had followed more than four years were included in this study. The revised acetabulum was evaluated clinically by Harris hip score and radiologically by evaluating the osseous union, incorporation and resorption of graft, the amount of migration and cup angle change of revised components and periacetabular radiolucency. The mean Harris hip score was improved 52 to 84. All of the bone grafts united by 6.4 months and incorporated by 13.1 months. The graft bone resorption less than onefourth of the initial graft thickness was 91.2% (31 cases). There was no significant difference between pure allograft and allograft mixed with autograft in clinical and radiological results. Of 34 cases, 2 cases (5.9%) required rerevision of acetabulum for aseptic loosening. The two complications, postoperative infection and trochanteric bursitis were managed successfully with conservative methods. The results of the present study confirm the success of revision of the acetabulum with use of a hemispherical component stabilized with multiple small screws and morselized bone grafts filling bone defects.
Acetabulum* ; Allografts ; Autografts ; Bone Resorption ; Bursitis ; Femur ; Hip ; Humans ; Postoperative Complications ; Transplants*