We experienced two cases of Gaucher's disease of adult type in brother aged four years and two years. The patients showed hepatosplenomegaly with anemia and thrombocytopenia. Typical Gaucher cells were found in bone marrow and biopsy specimens of liver and spleen through light and electron microscopic examination. Splenectomy was followed by improvement of anemia and thrombocytopenia. A brief review of literature was made.
Adult ; Anemia ; Biopsy ; Bone Marrow ; Gaucher Disease* ; Humans ; Liver ; Siblings* ; Spleen ; Splenectomy ; Thrombocytopenia

Harrington Instrumentation and Spinal Fusion to treat the unstable fracture and fracture-dislocation of the thoraco-Iumbar spine is a very effective method. This method not only decompresses the spinal cord and nerve roots by anatomical reduction and preserves spinal stability, but also makes possible early rehabilitation and prevention of complications. A clinical study was made of twelve patients who were hospitalized and treated at the Department of Orthopaedic Surgery, Chung-Nam National University Hospital from Oct. 1978 to J une 1980. The following results were obtained: 1. Of twelve patients, five(42%) had a flexion-compression fracture, four(33.3%) had a shear fracture-dislocation, two(16.7%) had a flexion-rotational fracture, and one(8.3%) had a bursting fracture. 2. Seven(58%) had a complete neural deficit and five (42%) had an Incomplete neural deficit. Two (28%) of the seven patients with complete neural deficit showed slight neurological recovery, but two patients(40%) with incomplete neural deficit gained complete recovery. 3. Correction of the kyphotic deformity was 16 degrees on the average, and the displacement was completely corrected in five of the six patients. 4. During the follow-up period, there was a final loss of about five degrees (range 3-16 degree) of kyphotic correction In our patients and there was no difference in loas of correction between laminectomy group and control group. 5. After an average of 18 postoperative days, ambulation was started, and acute hospital days were 77 days on the average. 6. There was no difference in spinal fusion rate between brace-wearing group (4 cases) and cast Immobilized group (8 cases). 7. Two cases (16.6%) of the twelve cases showed spontaneous interbody fusion.
Clinical Study ; Congenital Abnormalities ; Follow-Up Studies ; Humans ; Laminectomy ; Loa ; Methods ; Rehabilitation ; Spinal Cord ; Spinal Fusion ; Spine ; United Nations ; Walking

Rectovagianl fistula(RVF) is a congenital or acquired communication between the two epithelial-lined surface of the rectum and the vagina. We present our experience with 62 patients with RVF. There were various etiologies and repair methods of rectovaginal fistula. The purpose of this study was to retospectively review the clinical course of the patients we treated and to evaluate the efficacy of various treatment options. The mean age was 40.5 yr, The type of RVF was classified to one of two(simple and complex), according to their location, size and etiology. RVF was developed most commonly after radiotherapy due to cervical cancer(n=17), then after pelvic surgery due to malignancy(n=16), obstetric trauma after episiotomy at delivery(n=7), congenital malformation(n=4), inflammatory bowel disease(n=1), Bechet's disease(n=1), infections such as perianal fistula or abscess(n=2), direct invasion of carcinoma(n=3), after chemotherapy(n=1), and idiopathic(n=6). Three cases of them associated with rectovesicovaginal fistula. Surgical therapeutic option was divided to local repair, abdominal approach and tissue transposition by the type of RVF. Most simple RVFs were repaired with local approach through the vagina or rectum. Most complex RVFs were repaired through abdominal approach or tissue transposition. With an average follow up of 20 months, the treatment results were as follows: completely healed(n=36, 58.1%), persistent symptom(n=6, 9.7%), recurrence after repair(n=5, 8.1%), loss of search or death(n=15, 24.1%). Therefore we assist that the management of RVF depends on size, location, and cause. anal sphincter function and overall health status of the patient. Careful preoperative assessment of the fistula, surrounding tissues, and anal sphincter and exclusion of associated disease are essential. With through evaluation, thoughtful consideration of treatment options, and meticulous operative technique, patient can be assured of an optimal outcome.
Anal Canal ; Episiotomy ; Female ; Fistula ; Follow-Up Studies ; Humans ; Radiotherapy ; Rectovaginal Fistula* ; Rectum ; Recurrence ; Vagina

No abstract available.

Primary Cutaneous Neuroendocrine Carcinoma(CNEC : or Merkel Cell Tumor) usually occurs as a solitary tumor in middle aged to elderly individuals. This tumor may occur on any layer of the skin but the most frequent location is in the dermis. Electron microscopic studies reveal that the tumor cell contains round, dense core granules similar to epidermal Merkel cell and other cells of the neural crest derived APUD system. We describe the clinical, histological, electron microscopic, immunohistochemical observation of a case of primary CNEC in a 66-year-old female and discuss the theories regarding the histogenesis of this unusual tumor.
Aged ; Carcinoma, Neuroendocrine* ; Dermis ; Female ; Humans ; Middle Aged ; Neural Crest ; Skin

Brachycephaly is a kind of craniosynostosis. Because of premature closure of the coronal suture, the skull is shorter in the anteroposterior diameter but is widened with a high vault and the occiput and forehead are flattened. Diabetes insipidus had been reported in oxycephaly. We have experienced a case of central diabetes insipidus associated with brachycephaly. A brief review of related literatures is included in this report.
Craniosynostoses* ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Forehead ; Skull ; Sutures

To evaluate the radiographic characteristics of osteochondritis dissecans (OCD) and useful parameter for predicting mechanical stability, we retrospectively analysed 26 plain radiographic examinations and seven MR imagings in 28 cases of OCD in 24 patients. Typical radiologic findings were osteochondral defect with sclerotic rim of variable thickeness and osteochondral fragment. Sites of osteochondral defect were medial (35.9%) or lateral (32%) femoral chondyle and medial (7.1%) or lateral (25%) side of talar dome. Sclerotic rim was seen in 24 cases (85%) and osteochondral fragments including nine loose bodies were seen in 21 cases (75%). The size of osteochondral defect with unstable fragment (average 2.05cm) and loose body (2.04cm) in the knee joint were similar to, but statistically larger than that with stable fragment (1.35cm). All osteochondral defects were well visualized on MR images. Abnormalities of articular cartilage and effusion in the interface between the parent bone and fragment were seem in five cases of which there were confirmed three unstable cases arthroscopically. We conclude that size of defect may be a good parameter for predicting mechanical stability and MRI may be useful in the diagnosis of OCD and determining the methods of treatment.
Cartilage, Articular ; Diagnosis ; Humans ; Knee Joint ; Magnetic Resonance Imaging ; Osteochondritis Dissecans* ; Osteochondritis* ; Parents ; Retrospective Studies

PURPOSE: To assess the spinal canal and neural foraminal stenosis associated with spondylolisthesis on MR imaging. MATERIALS AND METHODS: We retrospectively analysed MR findings of 63 cases of spondylolisthesis(degenerative type:23 cases, isthmic type:40 cases) regarding the type and grade of spondylolisthesis, prensence or absence of associated spinal canal stenosis, and the severity of associated neural foraminal stenosis. RESULTS: Central canal stenosis were more frequent in degenerative type(91%) than isthmic type(33%), and more frequent in grade II spondylolisthesis of degenerative type(100%) and isthmic type(89%) than in grade spondylolisthesis of degenerative type(45%) and isthmic type(20%). There was positive correlation between the severity of neural foraminal stenosis and the grade of spondylolisthesis, whereas there was no significant difference between degenerative and isthmic types. CONCLUSION: Degenerative spondylolisthesis were frequently associated with central canal stenosis more than isthmic type. When the grade of spondylolisthesis was higher, it was more frequently associated with central canal stenosis and severe neural foraminal stenosis.
Constriction, Pathologic* ; Magnetic Resonance Imaging ; Retrospective Studies ; Spinal Canal ; Spondylolisthesis*

No abstract available.
Mucormycosis

The intraosseous ganglion is a rare cystic lesion of bone most frequently located in the subchondral epiphysis of long bones with or without direct communication with a joint cavity. This case is reported to call attention to this lesion which deserves consideration in the differential diagnosis of solitary cystic lesions of bone. We were reported a 59-aged woman without direct communication with the knee joint and osteoarthritis which was treated with the curettage and chip-bone graft.
Bone Cysts ; Curettage ; Diagnosis, Differential ; Epiphyses ; Female ; Humans ; Joints ; Knee Joint ; Osteoarthritis ; Tibia ; Transplants