Dysplasia epiphysialis punctata is a rare congenital disorder of infancy affecting in particular cartilage, muscle, jointtcapsules and the eyes. A case of dysplasia epiphysialis punctata with involvement of all epiphyses of extremities, spine and pelvis in 2 days old male is to be reported with review of literature.
Cartilage ; Chondrodysplasia Punctata ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Epiphyses ; Extremities ; Humans ; Male ; Pelvis ; Spine

Anal incontinence following pelvic trauma, surgery, or neurologic disorders has significant medical and social implication. Both Chetwood in 1902 and Bistom in 1944 utilized coccygeal origin portion of the gluteus maximus muscle for the anal sphincter reconstruction. The gluteus maximus muscle is a broad, fan-shaped muscle with a wide origin from the ilium sacrum and coccyx and a narrow insertion along the iliotibial band of the lateral femur. Its blood supply is from the inferior gluteal artery and its innervation is from L-5, S-1 roots by means of the inferior gluteal nerve Incisions are placed at the inferior border of the ischial tuberosity. Subcutaneous tunnels are created about the rectum and gluteal and perirectal incisions. Two overacting slings are being created Their opposing pull creates sphincter or valve effect about the distal rectum. We have experienced 2 cases of irregular, deep soft tissue defects of the perianal region requiring muscle coverage with the gluteus maximus muscle overlapping slings. We think the use of the gluteus maximus muscle is one of the most useful method for reconstruction of the anal sphincter mechanism.
Anal Canal* ; Arteries ; Coccyx ; Femur ; Ilium ; Nervous System Diseases ; Rectum ; Sacrum

Most of the malignant tumors of the pancreas are adenocarcinomas arising from the ductal epithelium. Primary leiomyosarcoma of the pancreas, even though it is the most common sarcoma of the pancreas, is very rare. We present a case of leiomyosarcoma of the pancreas, probably primary, with metastases to the stomach, lymph nodes, and abdominal wall. A 52-year-old woman visited the hospital with vague right upper abdominal pain and weigh loss of 6 kg for 2 months. The radiological and endoscopic examination revealed that she had a large heterogeneous mass, 11 cm in size, in the pancreatic body and tail, a 4 cm-sized mass in the paraaortic area, and a 3 cm-sized polypoid mass in the stomach. Histologically, they were all similar to one another and composed of markedly pleomorphic cells. Immunohistochemical and electron microscopic studies showed definite smooth muscle differentiation of the tumor cells. Two months later, the patient underwent an excision of a new 3 cm mass in the right lower abdominal wall, showing features of well differentiated leiomyosarcoma.
Abdominal Pain ; Abdominal Wall ; Adenocarcinoma ; Epithelium ; Female ; Humans ; Leiomyosarcoma* ; Lymph Nodes ; Middle Aged ; Muscle, Smooth ; Neoplasm Metastasis ; Pancreas* ; Sarcoma ; Stomach

We studied two hundread cases of replantations-61 cases of major limb replantation and 139 cases of finger replantation. 53 of 179 patients had multiple amputation. The most common cause of amputation was cutting-machine injury and the next one was belt and roller injury. The patients were followed than 6 months, ranging 6 months to 5 years and 10 months, averaging 36 months. The success rate of replantation is 86.9%(53/61) in the amputated limbs and 80.6%(112/139) in the amputated digits. Main causes of failure were tissue crushing, vessel thrombosis, long ischemic time, and amputation wound infection. Common complications were infection (25 cases), delayed union(7 cases), nonunion(2 cases), bleeding tendency and hematoma formation due to systemic heparinization(5 cases). We used Seddon's classification to assess the recovery of the nerve function of the replanted limbs and digits. 84.6 % of success cases of limb replantation presented the excellent result in the motor fuction recovery and 89.1% also presented the excellent sensory recovery. 89.8% of the success cases of the digit replantation showed the excellent result in the sensory recovery. The factors influencing the functional recovery of the replantation surgery were mechanism of injury, ischemic time, surgical technique, numbers of anastomosed vessels, ratio between anastomosed artery and vein, and wound infection. This analysis demonstrated that strict selection of the indication or the replantation is the first step in the limb and digit replantation surgery.
Amputation ; Arteries ; Classification ; Extremities ; Fingers ; Hematoma ; Hemorrhage ; Humans ; Operative Time ; Replantation ; Thrombosis ; Veins ; Wound Infection

The authors made an attempt to classify the supracondylar extension-valgus fracture according to the direction and location of the fracture line based on the radiographic manifestation. This new classification helps to dictate the model of treatment, possible sequence of reduction and the causes of deformity of the elbow after treatment. During the last 12 years of this study, 200 supracondylar fractures of the humerus were treated in the Kyung Hee University Hospital. Of the cases 82 were extension-valgus fractures. The results were as follows: 1. The supracondylar extension-valgus fracture were classified into 5 types: Type I: fish-tail fracture (14 cases) Type II : obtuse fish-tail fracture (27 cases) Type III: oblique fracture (16 cases) Type IV: oblique fracture with comminution of lateral column (14 cases) Type V: Transverse fracture (11 cases) 2. The displacement of the distal fragment in the fish-tail fracture is severe but it can be reduced easily by manipulation. Once reduction has been obtained, the fracture is stable and maintained by a long arm cast with acute flexion of the elbow alone. There is no need of percutaneous pinning. Among 14 cases of type I fracture there was no cubitus varus deformity. 3. The line of the obtuse fish-tail fracture lies distal to fish-tail fracture. This is unstable so it is necessary to percutaneous pinning. 4. The oblique fracture is produced by the hyperextension of the elbow and degrees of the obliquity of the fracture line were 12 to 20 degrees (Av. 14 degrees). The valgus angles in opposit elbows were 9 to 25 degrees (Av. 16 degrees). The forearm must be pulled into valgus position during the reduction of the fracture. 5. Type IV fracture occures under 4 years old, and more commonly in girls. This is unstable, so it is necessary to percutaneous pinning.
Arm ; Child ; Classification ; Congenital Abnormalities ; Elbow ; Female ; Forearm ; Humans ; Humerus

Rotation-plasty procedure was first done by Borggreve, in 1930 for the congenital short femur. Recently this procedure was used for the malignant bone tumor at the distal femur by Kotz and Salzer in 1982. In spite of its cosmetic problem of the distal stump, this procedure has great functional advantage of converting the above-knee amputation to the below-knee amputation. The authors applied this procedure in case of severe soft tissue crushing and large bone defect from the distal femur to the proximal tibia by the rail-road accident. Conventional below-knee prosthesis with thigh corset gave him normal ambulation. The inverted foot was good to control the prosthesis as a below-knee stump and heel functioned as a patella to support the body weight.
Amputation ; Body Weight ; Femur ; Foot ; Fractures, Comminuted ; Heel ; Knee ; Patella ; Prostheses and Implants ; Thigh ; Tibia ; Walking

To describe two cases of thoracic paraplegia due to a thoracic spinal cord tumor (meningioma) that was not detected during lumbar spinal decompressive surgery for lumbar canal stenosis and a complaint of claudication. The follow-up period ranged from 1 year and 6 months to 1 year and 8 months. The neurological deficit due to thoracic meningioma after surgery for lumbar canal stensois was decreased after mass excision. So, careful physical examination and magnetic resonance imaging can reveal another thoracic spine compressive lesion such as meningioma. Additional thoracic decompressive surgery can provide partial amelioration of each patient's neurological condition. Surgeons should know that a silent meningioma can aggrevate neurological symptoms after lower lumbar spine surgery and should inform their patient before surgery.
Constriction, Pathologic ; Follow-Up Studies ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Meningioma ; Paraplegia ; Physical Examination ; Spinal Cord Neoplasms ; Spine

Various methods of treatment, other than antibiotic therapy, have been proposed for the treatment of female urethral syndrome; however, the results of these treatment methods are disappointing, due perhaps to the use of the wrong treatment approach. The aim of this study was to evaluate the effectiveness of external sphincter relaxant and biofeedback (BFB) with electrical stimulation therapy (EST) in patients who do not respond well to antibiotics. One hundred and five patients with a diagnosis of female urethral syndrome were entered into this study. Antibiotics were given as a first-line therapy for about 3 months. In cases of recurrent or incurable urethral syndrome, antibiotic therapy combined with external sphincter relaxant or BFB with EST were performed. External sphincter relaxant group was composed of 31 patients (29.5%) who showed functional urethral obstruction. Biofeedback group was composed of 41 patients (39.0%) who had severe pain or discomfort with irritative voiding symptoms. Subjective symptom was measured before and after therapy using the Bristol Female Lower Urinary Tract Symptoms questionnaire. Thirty-three patients (31.4%) were treated with antibiotic therapy alone and 7 (21.2%) of these patients recurred. The symptom score of this group changed from 10.51 to 2.85. In the antibiotics plus external sphincter relaxant group (N=31), the symptom score changed from 12.39 to 3.96. Five (16.1%) of these patients recurred and 3 of these 5 underwent urethral dilatation. In the antibiotics plus biofeedback group (N=41), the average urinary frequency changed from 12.2 to 7.7 times a day and nocturia changed from 2.4 to 0.6 times a night. The symptom score improved from 15.22 to 4.69 and the overall satisfaction rate was 87.8% (41.5%: very satisfied, 46.3%: satisfied, 12.2%: no response). Female urethral syndrome is not due to a single factor but is a complex disease due to various combined symptoms and mechanisms. This condition needs to be treated with an appropriate treatment protocol. We believe that satisfactory results could be obtained in female urethral syndrome, which has shown poor prognosis until now, by appropriately combining treatment methods, which include the use of external sphincter relaxants, biofeedback therapy and bladder training, according to indication, and depending on whether symptoms continue after initial antibiotic therapy.
Adult ; Aged ; Antibiotics/*therapeutic use ; Biofeedback (Psychology) ; Electric Stimulation Therapy ; Female ; Human ; Middle Age ; Parasympatholytics/therapeutic use ; Urethral Diseases/etiology/*therapy

VATER association of vertebral defects, imperforate anus, tracheoesophageal (T-E) fistula with esiphageal atresia, and radial and renal dysplasia are utilized to identify this complex. Other anomalies are vascular anomalies (cardiac defects and single umbilical artery), and prenatal and postnatal growth deficiency, defects of external genital and urinary tract as well as bone defects (preaxial lower extremity anomalies, supernumerary digits, rib anomaly). This pattern of malformation generally occurs sporadically in an otherwise normal family, and may occur as a part of a broader pattern, such as the trisomy 18, del (4p, 6q, and 13q) syndromes, and sirenomelia. As inversion of chromosome does not lose of genetic materials, phenotypic abnormalities does not occur. But rare cases have been reported. We report a neonate who had multiple congenital anomalies, including the VATER association defects associated with an unusual chromosomal abnormality, a paracentric inversion of chromosome 6 : 46, XY, .inv (6)(q11q16).
Anus, Imperforate ; Chromosome Aberrations ; Chromosomes, Human, Pair 6 ; Ectromelia ; Fistula ; Humans ; Infant, Newborn ; Lower Extremity ; Ribs ; Trisomy ; Urinary Tract

Drowning is the second most common cause of accidental death of children in Korea. But the study of near-drowning is very rare in Korea. The purpose of our study is to determine the factors that may influence survival on the basis of submersion time, consciousness state upon admission to hospital, the kinds of water, laboratory findings and neurological outcomes. The survey was performed by a retrospective cohort study on 28 near drowning victims of less than 15 years of age who were admitted to the emergency room in the Chosun University Hospital between May 1988 & May 1997. The results were as follows 1) Sex distribution was 22 males and 6 females. 2) Submersion time was (5 minutes in 9 cases, 5-9 minutes in 6 cases,10-14 minutes in 5 cases,15-19 minutes in 3 cases, > or =20 minutes in 5 cases. 3) Comatose patients upon arrival were 6/7 cases in the death group(86%) and 2/21 cases in the improved group(10%). they had unfavorable outcomes(P<0.05). 4) The first pH value was mean 7.02)0.12 in the death group and mean 7.31 +/-0.13 in the improved group. there was a statistically significant difference between the death and the improved group(P<0.05). 5) The patients who had increased blood glucose concentration were all 7 cases in the death group and 12/22 cases in the improved group(54%). The mean blood glucose concentration was mean 424.7235.6mg/dl in the death group and mean 140.182.7mg/dl in the improved group. There was a statistically significant difference between the death and the improved group(P<0.05). 6) The patients who had pulmonary edema upon arrival were all 7 cases in the death group and 1/21 cases in the improved group(18%). They had unfavorable outcomes(P<0.05). 7) The patients who had been submerged more than 15 minutes were all 7 cases in the death group and 6/21 cases in the improved group(5%). They had unfavorable outcomes(P<0.05). We conclude that pediatric victims of near-drowning can be assigned to high or low likelihoods of unfavorable outcomes with the use of five variables, comatose mentation upon arrival, decreased initial blood pH, increased initial blood glucose concentration, pulmonary edema, and maximum submersion time estimated longer than fifteen minutes. This prediction rule may be useful if it can be validated in another cohort.
Blood Glucose ; Child* ; Cohort Studies ; Coma ; Consciousness ; Drowning ; Emergency Service, Hospital ; Female ; Humans ; Hydrogen-Ion Concentration ; Immersion ; Korea ; Male ; Near Drowning ; Pulmonary Edema ; Retrospective Studies ; Sex Distribution