Wilson's disease is a familial disease characterized by coarsely nodular cirrhosis of the liver, sometimes associated with progressive damage to the nervous system, and the appearance of a coloured ring in the cornea. Wilson's disease is an inborn error of copper metabolism in which the synthesis of ceruloplasmin, with which copper forms a table compound in the blood, is diminished and there is an increased absorption of copper from the gastrointestinal tract together with an increased output in the urine. The Kayser-Fleischer ring is originally described by Kayser(1902) and later by Fleischer(1903). The colours of Kayser-Fleischer ring is seen, varing from rub by red to bright green or an 1lltramarine blue. sometimes interspersed with yellow or smoky shade of brown. The ring starts dose to the limbus as a sharp line just where the endothelial pattern begins to be seen distinctly, extends over a breadth of I to 3mm.. and gradually fades away towards the center of the cornea. Harry et al(1970) described that electron microscopy showed the presence of electron dense deposits of varing size lying mainly in Descmet's membrane and confirmed that the deposits were copper. A 14 year old korean boy with Wilson's disease was found to have Kayser-Fleischer ring in the cornea(ou) and greenish brown pigments on the anterior surface of the lens (ou). In about 16 months after the administration of penicillamine(chelating agent), greenish brown pigments on the anterior surface of the lens (ou) were disappeared remaining Kayser Fleischer ring unchanged.
Absorption ; Adolescent ; Ceruloplasmin ; Copper ; Cornea ; Deception ; Fibrosis ; Gastrointestinal Tract ; Hepatolenticular Degeneration* ; Humans ; Liver ; Male ; Membranes ; Metabolism ; Microscopy, Electron ; Nervous System

Carcinoma of the lids has the highest incidence of any malignant ocular tumor. It is most frequent in men over 50 years of age. The majority of lid carcinoma are of the basal cell type, the remaining consist of squamous cell carcinomas and meibomian gland carcinomas. Basal cell carcinoma is much more common in the lower lid; squamous cell carcinoma in the upper lid. Squamous cell carcinoma grows slowly and painlessly, and may be present for months before it is noted. It may occlude meibomian ducts and produce an inflammatory element which may confuses the clinical picture. A 57 years old man with bronchogenic carcinoma was referred for eye consultation and found to have a small painless nodule on right upper lid that was thought to be chalazion. Palpation of preauricular, submaxillary or cervical node was negative. This small nodule was excised radically for microscopic examination to rule out a probable metastatic lesion from bronchogenic carcinma. Histopathologically, this tumor was reported not to be a secondary but to be a primary squamous cell carcinoma on right upper lid.
Carcinoma, Basal Cell ; Carcinoma, Bronchogenic ; Carcinoma, Squamous Cell* ; Chalazion* ; Eyelids* ; Humans ; Incidence ; Male ; Meibomian Glands ; Middle Aged ; Palpation

Extracorporeal shockwave therapy (ESWT) has been reported to be a safe and effective method for decreasing pain and relieving range of motion (ROM) limitations caused by neurogenic heterotopic ossification (NHO), though there has been no report that it might cause hematoma if applied to NHO. We hereby report a case of massive hematoma after ESWT, specifically the radial shockwave therapy (RSWT) device at both hips in a 49-year-old female patient with NHO. She had developed NHO after extensive subarachnoid hemorrhage. We had applied RSWT according to the previous report. The pain and the ROM limitations were gradually improved. Six weeks later, she reported pain and ROM limitations on the right hip. From a medial aspect, swelling and bruising of the right thigh could be seen. Magnetic resonance imaging and ultrasonography suggested a large hematoma between right hip adductor muscles. The symptoms disappeared after conservative treatment for one month, and subsequent follow-up imaging studies demonstrated resolution of the hematoma.
Female ; Follow-Up Studies ; Hematoma* ; Hip ; Humans ; Magnetic Resonance Imaging ; Methods ; Middle Aged ; Muscles ; Ossification, Heterotopic* ; Range of Motion, Articular ; Subarachnoid Hemorrhage ; Thigh ; Ultrasonography

Pulmonary embolism is a common clinical problem in patients with immobilization, cancer, indwelling central venous catheter and surgery. However, although rare, it may occur in patients with inherited thrombophilia. Protein S deficiency is known to increase the risk of venous thrombosis and pulmonary embolism. There are many reports of venous thrombosis with protein S deficiency, but there are few reports of arterial thrombosis, especially recurrent acute pulmonary embolism. Here, we report a case of recurrent pulmonary embolism associated with type II protein S deficiency.
Blood Coagulation Disorders, Inherited ; Central Venous Catheters ; Humans ; Immobilization ; Protein S ; Protein S Deficiency ; Pulmonary Embolism ; Thrombophilia ; Thrombosis ; Venous Thrombosis

BACKGROUND: The injection of rocuronium causes pain and withdrawal responses.This study was designed to determine an optimal dose of alfentanil to prevent the withdrawal responses associated with injection of rocuronium in children. METHODS: One hundred and ten ASA physical status I and II pediatric patients were randomly allocated into four groups; Group C (control; normal saline 3 ml, n = 28), Group A5 (alfentanil 5microgram/kg, n = 28), Group A10 (alfentanil 10microgram/kg, n = 27) and Group A15 (alfentanil 15microgram/kg, n = 27). After the induction of anesthesia with 5 mg/kg of thiopental sodium, the test drug was injected over 20 seconds, respectively. After one minute, rocuronium 0.6 mg/kg was injected over 5 seconds. The patient's response after injection was graded using a four-point scale. Additionally, the mean arterial pressure and heart rate were recorded upon arrival in the operating room, as well as 1 min before and 1 min after tracheal intubation. RESULTS: The incidence of withdrawal responses was 96%, 61%, 19% and 19% in groups C, A5, A10 and A15, respectively. The incidence and severity of withdrawal responses in group A10 and A15 were lower than group C and A5. CONCLUSIONS: After thiopental injection, alfentanil 10microgram/kg prevent the withdrawal responses on injecting rocuronium in pediatric patients.
Alfentanil ; Androstanols ; Anesthesia ; Arterial Pressure ; Benzeneacetamides ; Child ; Heart Rate ; Humans ; Incidence ; Intubation ; Operating Rooms ; Piperidones ; Thiopental

Congenital macrostomia is a result of defective union between the mandibular and maxillary processes and it is a rare deformity seen in every 100 to 300 facial clefts. Ohnizuka1`classified macrostomia into two groups as congenital and posttraumatic. We experienced two cases of acquired macrostomia due to NOMA sequelae(58/F:Lt & 51/F:Rt) and one case of congenital macrostomia (3 months/M:Rt). Many plastic surgeons have developed surgical procedures for repair of this congenital macrostomia. Among them, McCarthy6,11 described the classic commissuroplasty. We could repaired 1 case of congenital macrostomia and two cases of acquired macrostomia due to NOMA sequelae using modified technique of McCarthy,s classic commissuroplasty. McCarthy described new oral commissure 2-3mm laterally for prevention of postoperative contraction, orbicularis oris muscle transposition to restore labial function and a z- plasty cutaneous closure. But some author raise an objection to new oral commissure 2-3mm laterally, and they made new oral commissure at same distance of opposite side normal commissure. And so, we designed the new oral commissure moved 1mm laterally comparing to original commissuroplasty in a congenital case for the prevention of displacement. In cases of acquired macrostomia due to NOMA sequelae, we reconstructed new oral commissure like congenital case, moved 1mm laterally. Orbicularis oris muscle transposition could not be possible because of destruction of muscle, adhesion and atrophy. And so we dissected muscle and just sutured side by side. Acquired macrostomia following NOMA sequelae manifsted facial deformity variably, and reconstruction of the facial deformity is difficult by using simple approach. Other variable reconstructive procedures were needed with commissuroplasty as like Washio flap, rotation advancement flap, bone graft and free radial forarm flap, etc. Postoperative results were relatively good. We propose that macrostomia due to NOMA sequelae must add to Ohnizuka classification of acquired macrostomia.
Atrophy ; Classification ; Congenital Abnormalities ; Macrostomia* ; Noma ; Transplants

The rigid metal fixation devices are currently the most widely used in maxillofacial surgery. The use of metal plate and screw fixation, however, is not free of some postoperative complication. Once the fractures have healed, metal devices no longer serve any tissue purpose other than the potential for adverse reaction, including loosening, palpability, corrosion, and artifacts in CT and MRI, and they restrict growth of the neurocranium. To overcome these drawbacks, there had been a continuous research on the development of a bioabsorbable skeletal fixation system using polymers of polylactic and polyglycolic acid. There is an increasing acceptance of there use as an alternative fixation device in craniomaxillofacial surgery. We have used the BiosorbFX(R) system(Bionix Implants Ltd) in 48 patients of facial bone fractures. Age varied from 3 to 70(mean 34) and follow up period varied form 1 months to 10 months(mean 5 months). A total of 860 bioabsorbable devices(151 plates and 709 screws) was used. We encountered no significant intraoperative difficulties in obtaining placement of the devices. No patients has experienced any implant-related complications including infection, fracture instability or relapse, or radiographic evidence of osteolysis. The use of nonmetallic materials as a fixation devices that will be resorbed after facial bone fracture healing, therefore, would have advantages. The good results indicate that the use of bioabsorbable implants can be considered for the fixation of facial bone fractures.
Absorbable Implants ; Artifacts ; Corrosion ; Facial Bones* ; Follow-Up Studies ; Fracture Fixation* ; Fracture Healing ; Humans ; Magnetic Resonance Imaging ; Osteolysis ; Polyglycolic Acid ; Polymers ; Postoperative Complications ; Recurrence ; Surgery, Oral

Tuberculosis involving the central airway occasionally results in diffuse stenosis in the distal trachea and main bronchus. When the stenosis is more limited to the main bronchus, sleeve resection can be performed with high likelihood of a good result. Bronchial stenosis limited to 2 cm is considered favorable for bronchial sleeve resection. However, a longsegment stenosis may make sleeve resection difficult or impossible, and pneumonectomy or therapeutic bronchoscopy may be performed. An extended sleeve lobectomy is a procedure to remove more than one lobe using a bronchoplasty technique and its applications to the patients with locally advanced lung cancer were reported. We performed an extended sleeve lobectomy in a patient with tuberculous bronchial stenosis involving the right main bronchus, bronchus intermedius, right middle lobar bronchus and right lower lobar bronchus, and report this case with review of literatures.
Bronchi ; Bronchoscopy ; Constriction, Pathologic ; Humans ; Lung Neoplasms ; Pneumonectomy ; Trachea ; Tuberculosis

PURPOSE: To evaluate the usefulness of locking compression plate (LCP) and volar plating through anterior approach for distal radius fracture. MATERIALS AND METHODS: We retrospectively analysed that 15 distal radius fracture, which would not be reduced by closed reduction or too comminuated to maintain reduction or articular surface inconguency, were treated by open reduction through anterior approach and volar plating using LCP. The results were evaluated by preoperative and postoperative radiographs. Functional results were analysed using the Modified Mayo Wrist Scoring System. RESULTS: All cases achieved anatomical articular surface reduction postoperatively. In terms of radiologic analysis, mean radial length (9.0 mm vs. 11.8 mm), radial inclination (14.7degrees vs. 20.9degrees ), volar tilt (-6.3degrees vs. 8.3degrees ) and articular step-off (1.4 mm vs. 0.3 mm) were improved. The average Modified Wrist Score was 89. Nonunion or malunion was not occurred. CONCLUSION: Open reduction through anterior approach and volar plating using LCP is a useful method that provides excellent results with few complications in the treatment of fracture of the distal radius.
Radius Fractures ; Radius* ; Retrospective Studies ; Wrist

When soft tissue circular or elliptical pathologic lesions are located around the nasolabial fold, the most appropriate method is to make the excision parallel with the scar or along a natural wrinkle-crease. For this purpose, simple elliptical excision following primary closure is recommended. But when its long axis of elliptical defects is located vertically to the nasolabial fold, these will bring a bad aesthetic result after elliptical excision following primary closure due to long vertical straight scar to nasolabial fold. If soft tissue defect is larger, we should depend on the wide dissection for the closure of elliptical excised area. As a result, it is inevitable to make postoperative deformity due to tension around the eyelids, oral commissures, canthal fold, and alar nose. V-Y-S plasty was introduced by Algamaso in 1974 for closure of a round defect. It adopted some aspects of the double rotation flaps(or S-plasty) and some of the V-Y advancements. The authors applied from March 1998 to December 2000 to use single rotation flaps(or half-S plasty) and V-Y advancement for closure of a round defect, around nasolabial folds in 12 patients, named it half V-Y-S plasty, by modifying of Argamaso's V-Y-S plasty. We could obtain sufficient coverage of round defects and placement postoperative scar on the nasolabial fold and alar crease area using single V-Y-S plasty. Even in case of hypertrophic scars, we could obtain the same result and symmetric postoperative supralabium contour. The average soft tissue defect diameter was 1.9 cm (biggest one: 3.2 cm), and advanced gain of V-S advancement was 1.34 cm. As a result, we could obtain the final result more aesthetic and functional than that of straight line closure or other type of local flap. We described the experience of half V-Y-S plasty with a review of literature.
Axis, Cervical Vertebra ; Cicatrix ; Cicatrix, Hypertrophic ; Congenital Abnormalities ; Eyelids ; Humans ; Nasolabial Fold* ; Nose