Freeman-Sheldon Syndrome is one of the very rare genetic diseases which primarily affects face, hands and feet. At first, Freeman and Sheldon described this syndrome as cranio-carpo-tarsal dystrophy in 1938 and later Burian, as "whistling face" syndrome in 1963. There were 60 cases of reports in the world up to now, and only one paper with 5 cases in a family was reported in Korea. The authors report 2 cases of Freeman-Sheldon Syndrome associated with bilateral inguinal hernias and undescended tests in a family, briefly review the literature and alert orthopaedic surgeons to this condition.
Cryptorchidism ; Foot ; Hand ; Hernia, Inguinal ; Humans ; Korea ; Male ; Surgeons

BACKGROUNDS: Various methods have been devised for choosing the correct internal diameter (ID) of endotracheal tubes and the proper lengths for oral endotracheal tubes at different ages in children. This study was performed to evaluate the appropriate sizes of uncuffed endotracheal tubes and distance from upper incisor to carina in Korean children under 8 years old. METHODS: Five hundred thirty five children under 8 years in ASA class 1 and 2 were evaluated for the study by age groups: 0~3 months, 4~11 months and every each year until 8 years. Appropriate sizes were to permit a gas leak at 15~25 cmH2O with positive pressure ventilation. Distances from incisor to carina were at the point which bilateral lung sounds were noticed during slow extubation from endobroncheal intubation. RESULTS: Appropriate tube sizes were 3.0 mm to 3.5 mm for infants under 3 months, 3.5 mm to 4.0 mm for 4 to 11 months and ""4.15+0.28xage (yr) (mm)"" (R2=0.77, p<0.05) for children between 1 and 8 years old. Distances from upper incisor to carina were 12.0 cm for infants under 3 months, 13.7 cm for 4 to 11 months and ""14.5+0.6xage (yr) (cm)"" (R2=0.62, p<0.05) for children between 1 and 8 years old. CONCLUSIONS: We conclude that the endotracheal tube ID for the Korean children are a little greater than those of previous reports in foreign countries and airway lengths for the Korean children are similar to those of foreigners.
Child* ; Emigrants and Immigrants ; Humans ; Incisor* ; Infant ; Intubation ; Positive-Pressure Respiration ; Respiratory Sounds

Currently, many techniques for the diagnosis of carpal disorders have been developed. Plain radiograph is the most simple and low-cost procedure that offers various information on the normal wrist. Nonetheless, a sufficient amount of morphometric database for Koreans has not been studied. In this study, the authors reviewed 100 cases of plain posteroanterior radiograph of the wrist in normal Korean adults for both sexes in order to establish normal databases. Various parameters were measured, and comparisons between the sexes, age groups, foreign databases and other domestic databases were made. The results were as follows. 1. In all measurements of the length and ratio except ulnar variance, there were significant differences between the sexes. But there was no significant difference between the sexes in angular measurements. No significant difference in measurements were found among the groups based on their age. 2. The average ulnar variance was 0.6 mm, and it was higher than Europeans and Americans. 3. The average carpal height ratio was 53.5% and significantly lower in females, but the revised carpal height ratio was higher. This means that the length of the capitate is shorter and the length of the third metacarpal bone is relatively longer in females, if the carpal height is constant. 4. The carpal-radial distance ratio, the carpal-ulnar distance ratio, and the carpal width-carpal height ratio were significantly lower in females. This means that the carpal width is relatively narrower in females. The authors hope that information concerning normal roentgenographic measurements of the wrist can help to diagnose the carpal disorders including the diseases appearing carpal collapse or carpal translation and to design wrist implants.
Adult* ; Diagnosis ; Female ; Hope ; Humans ; Wrist*

PURPOSE: To evaluate the effectiveness of magnetic resonance imaging (MRI) for the diagnosis and usefulness of vertebroplasty in osteoporotic occult vertebral fractures. MATERIALS AND METHODS: Of 472 osteoporotic vertebral fractures treated from May 2003 to July 2009, 45 patients were diagnosed with occult osteoporotic vertebral fracture. Their medical charts and radiographs were reviewed. The degree of vertebral body collapse was graded by a semiquantitative method. In order to increase the reliability, interpretation was based on radiographic diagnoses from 3 orthopedic surgeons. Vertebroplasty was performed at 31 of the 45 patients, for whom conservative treatment failed. Pre-operatively and post-operatively, pain was evaluated using a visual analog scale (VAS). RESULTS: We observed 55 occult fractures in 45 patients. Forty vertebrae (72.7%) among the 55 vertebrae were just adjacent to an old vertebral fracture with deformation of the vertebral body. The rediagnosis rate of occult fracture attempted without MRI was only 21.8%. The average pre-operative VAS score of 8.07 (6~9) was improved to 2.43 after surgery and showed no delayed vertebral body collapse. CONCLUSION: Gadolinium enhancement of MRI is critical to the diagnosis of occult vertebral fractures, which are 9% of whole osteoporotic vertebral fractures. Seventy-two point seven percent of occult vertebral fractures were just adjacent to an old vertebral fracture with collapse of the vertebral body. This means that if vertebroplasty is performed without thorough MRI examination, it may fail.
Fractures, Closed ; Gadolinium ; Humans ; Magnetic Resonance Imaging ; Orthopedics ; Osteoporosis ; Spine ; Vertebroplasty

PURPOSE: To compare the hematologic changes and the rates of transfusion of patients using rivaroxaban or aspirin for venous thromboembolism prophylaxis after a total knee arthroplasty. MATERIALS AND METHODS: Among patients with total knee arthroplasty from July 2010 to March 2011, two groups of 100 consecutive cases were enrolled in this study, 50 patients with Rivaroxaban group and 50 patients with Aspirin group for venous thromboembolism prophylaxis after a total knee arthoplasty. Hematologic changes and transfusion rates were calculated in each group. RESULTS: The mean of decreased hemoglobin was 4.7 (3.1-6.6) in the Rivaroxaban group and 3.6 (2.0-5.1) in the Aspirin group (p<0.05). The number of patients with decreased hemoglobin of less than 8 g/dl was observed in 23 cases (46%) in the Rivaroxaban group, and 9 cases (18%) in the Aspirin group. The numbers of patients who needed transfusion were 12 in the Rivaroxaban group, and 2 in the Aspirin group (p<0.05). CONCLUSION: Rivaroxaban group revealed more significant decrease of hemoglobin and needed more transfusion than the Aspirin group did. For the prevention of venous thromboembolism after total knee arthroplasty, we should be careful using Rivaroxaban for the standard risk patients of venous thromboembolism.
Arthroplasty ; Aspirin ; Hemoglobins ; Humans ; Knee ; Morpholines ; Thiophenes ; Venous Thromboembolism ; Rivaroxaban

A 55-year-old female presented to Pusan National University Yangsan Hospital with left neck and shoulder pain. An anterior mediastinal mass was detected on chest CT and there were no other specific lesions in the lung or pleural cavity. An infected pericardial cysts was suspected and excision was performed through a left-sided VATS approach. The patient was discharged on the second post-operative day with left diaphragm palsy and praziquantel was prescribed after paragonimaisis was confirmed on pathology. The patient has not shown any particular problems at my outpatient clinic.
Ambulatory Care Facilities ; Diaphragm ; Female ; Humans ; Lung ; Mediastinal Cyst ; Mediastinum ; Middle Aged ; Neck ; Paragonimiasis ; Paralysis ; Pleural Cavity ; Praziquantel ; Shoulder Pain ; Thoracic Surgery, Video-Assisted ; Thorax

There are more than 120 different theories on the possible cause of sudden infant death syndrome (SIDS). Recent studies indicate a possible role of infections in SIDS, one of which suggested that a maximum percentage of up to 43.5% of SIDS showed virus-induced myocardial affection. The enterovirus, mainly the cardiotropic coxsackievirus group B, serotypes B1-B5, which causes most of viral myocarditis in infancy, seems to be the most common virus associated with SIDS. The aim of our study was to investigate the enterovirus-induced myocardial affections in cases of SIDS. All 7 cases of postmortem heart samples from SIDS victims were studied. Postmortem heart samples of non-SIDS death in perinatal period, infancy and young childhood, pediatric cases of myocarditis and unnatural death in young adult were served as controls. An immunohistochemical study was performed on all samples using monoclonal mouse anti-enterovirus antibody and anti-LCA with quantification of positive cells. Our results demonstrated that 2 of 7 cases of SIDS showed strong immunoreactivity in the myocardium against antienterovirus antibody and there was more inflammatory cell infiltrate in SIDS cases than controls. Our study suggests that the enteroviral myocardial affection would be a possible role in pathogenesis of SIDS.
Animals ; Enterovirus Infections* ; Enterovirus* ; Heart ; Humans ; Immunohistochemistry ; Mice ; Myocarditis ; Myocardium ; Sudden Infant Death* ; Young Adult

Since patients with intracranial neoplasms usually represent their neurological symptoms rather early in the course of the disease, sudden unexpected death due to intracranial neoplasms is an uncommon event seen only exceptionally in medicolegal autopsy practice. Although meningiom is one of the most common primary intracranial neoplasms, sudden death due to the tumor is an uncommon event. We report a sudden, unexpected death of a 56-year-old female with an undiagnosed olfactory groove meningioma. It is assumed that peritumoral edema is related to the sudden death among the several mechanisms.
Female ; Humans

Cervical pregnancy is a rare form of ectopic gestation and it can cause serious bleeding, shock and death. Although hysterectomies are still performed due to massive hemorrhages, there are some conservative approaches to cervical pregnancies to conserve fertility. Methotrexate is a very useful agent in the management of trophoblastic disease, and has been utilized recently for the successful management of tubal, interstitial, and cervical ectopic gestations. We report a case of viable cervical pregnancy treated successfully with intraamniotic injection of methotrexate guided by transabdominal ultrasonography.
Fertility ; Hemorrhage ; Hysterectomy ; Methotrexate* ; Pregnancy* ; Shock ; Trophoblasts ; Ultrasonography*

BACKGROUND: When using a target controlled infusion (TCI) of propofol, combination with N2O or fentanyl as an analgesic adjuvant is common in clinical practice. In a previous study, a minimal steady state plasma concentration necessary to prevent a response in 50% of the patients following a skin incision (Cp50i) for propofol was reduced from 6ng/ml to 4.5ng/ml with 67% nitrous oxide/oxygen compared to air/oxygen. The goal of this study was to quantify the effect site concentration of fentanyl required to replace 67% N2O at a propofol effect site target concentration of 4.5ng/ml. METHODS: Forty six ASA class I or II adult patients scheduled for lower extremity surgery were randomly allocated to one of three groups according to assigned effect site concentration of fentanyl. Group 1, n = 15; 0.5 ng/ml, Group 2, n = 15; 1.0 ng/ml, Group 3, n = 15; 1.5 ng/ml. Patients received propofol with target concentration 4.5ng/ml and predetermined target concentration of fentanyl in three groups. A laryngeal mask airway was placed after anesthesia induction and all patients were controlled ventilation with 67% air/33% oxygen. The response to the skin incision was observed and the patients categorized as movers or non-movers according to Eger's criteria. Cp50i for fentanyl was evaluated using nonlinear regression analysis. RESULTS: Non-movers to skin incision was 20%, 43.7%, 73.7% in groups 1 3 respectively. Cp50i for fentanyl combined with propofol 4.5ng/ml was 1.08 ng/ml. CONCLUSIONS: We concluded that the MAC for 67% N2O is equivalent to an effect site target fentanyl concentration of 1.08 ng/ml in terms of no movement to skin incision.
Adult ; Anesthesia ; Anesthesia, General* ; Fentanyl* ; Humans ; Laryngeal Masks ; Lower Extremity ; Oxygen ; Plasma ; Propofol* ; Skin ; Ventilation