Gyrate atrophy of choroid and retina is a rare hereditary disorder, which is characterized by progressive decrease of visual acuity, nyctalopia, visual field constriction and posterior subcapsular cataract. The authors experienced a case of gyrate atrophy of choroid and retina in a 22 year-old male.
Cataract ; Constriction ; Gyrate Atrophy* ; Humans ; Male ; Night Blindness ; Visual Acuity ; Visual Fields ; Young Adult

In order to observe the change of epicardial ECG(Eep), left ventricular pressure, left ventricular dp/dt and the development of arrhythmia during regional myocardial ischemia and reperfusion, proximal LAD was ligated for 30 minutes and reperfused suddenly for 30 minytes in eleven mongrel dogs which were grouped into control(n=6) and diltiazem(n=5) group. In diltiazem group, diltiazem infusion was started 10 minutes prior to reperfusion with the speed of 0.02mg/kg/min for 25 minutes. The amount of injury current was measured from TQ segment and ST segment changes of Eep, and its effect on the incidence of reperfusion arrhythmia was evaluated. Eep, LV pressure, LV dp/dt and ECG were simultaneously recoreded with the paper speed of 100mm/sec at predetermined time intervals, and 6 channel ECG(standard lead I, II, III, AVR, AVL, AVF) was recorded continuously with paper speed of 10mm/sec throughout the experiment. The results were as follows ; 1) After ligation of LAD, the polarity QRS of Eep changed to show monophasic shape from 3-4 minutes, TQ segment depressed to reach minumum level at 4-7 minutes and ST segment elevated to reach maximum level at 4-5 minutes. These changes recovered rapidly to pre-ligation state after reperfusion, and this tendency was not affected by diltiazem. 2) The absolute value of LV dp/dt max and LV dp/dt min decreased 10% at 2-4 minutes after LAD ligation, and began to recover from 7 minutes after reperfusion to reach peak recovery value at 20 minutes after reperfusion in control group. In diltiazem group, it decreased 15% after diltiazem infusion and began to recover from 1 minutes after reperfusion to reach peak recovery value at 7 minutes after reperefusion. 3) Ischemic ventricular fibrillation was observed at the time of maximum TQ depression and ST segment elevation and 4 out of 6 events were developed within 5 minutes after LAD ligation. The cases with Isch-Vf developed Rep-Vf without exception, which was observed in 8 out of 11 cases and was noted within 1 minutes after reperfusion except one. 4) Maximum ST elevation was significantly higher in group with Rep-Vf then in group without Rep-Vf(Rep-Vf(+);18.5+/-11.1, Rep-Vf(-);10.3e+/-6.9, p<0.05), and also maximum ST elevation was significantly higher in group with both Isch-Vf and Rep-Vf then in group with only Rep-Vf(Isch-Vf+Rep-Vf;28.5+/-7.8, Rep-Vf only;10,5+/-4.7, P<0.01). 5) The incidende of reperfusion ventricular fibrillation was 83% in control group(5 out of 6) and 60% in diltiazem group(3 out of 5), but the inhibitory effect of diltiazem on the reperfusion Vf could not be confirmed due to the difference of the incidence of ischemic Vf between the two groups(control group;67%(4 out of 6), ditiazem group;20%(1out of 5)). In conclusion, maximum injury current developed 4-7 minutes after coronary artery ligation, and maximum ST elevation value was significantly related with the development of ischemic Vf and reperfusion Vf, and the inhibitory effect of diltiazem on the reperfusion ventricular fibrillation could not be confirmed in this study.
Animals ; Arrhythmias, Cardiac* ; Coronary Vessels* ; Depression ; Diltiazem* ; Dogs ; Electrocardiography* ; Incidence ; Ligation* ; Myocardial Ischemia ; Reperfusion* ; Ventricular Fibrillation ; Ventricular Pressure

PURPOSE: Fractures of the mandibular condylar area are common injuries that account for 29% to 40% of fractures of the facial bones and represent 20% to 62% of all mandibular fractures. Currently 3 main methods are being used in the treatment of mandibular subcondylar fractures: closed reduction; open reduction and internal fixation; Endoscopic reduction and internal fixation. Each method has its proponents and opponent as well as advantages and disadvantages, and indications for each vary among surgeons. There are six approaches of open reduction: submandibular, retromandibular, preaurilcular, postauricular, intraoral, transparotid approach. Among them, transparotid approach has been described for subcondylar exposure with dissection in the direction of facial nerve fibers to expose the bone through the parotid gland. This approach carries the risk of a parotid glandular fistula as well as facial nerve injury but has the advantage of being directly over the fracture site. We report safety and efficacy of surgical treatment using a transparotid approach for direct plating. METHODS: A 43-year-old man sustained multiple facial bone fractures by driver traffic accident. Mandibular subcondyle was fractured and dislocated internally. We performed open reduction and internal fixation by transparotid approach. Fractured site was fixed by titanium mini plate & screw. We applicated arch bar for approximately 3 weeks. RESULTS: Follow-up length was about 5months. Scar of surgical incision was indistinct, there was no symptoms and signs of facial nerve and parotid gland injury, and maximal mouth opening was measured 49.5 mm. CONCLUSION: Transparotid approach has high risks of facial nerve and parotid gland injury, but paradoxically it is the most effective technique in saving facial nerve. Open reduction and internal fixation of mandibular subcondylar fracture by transparotid approach with precise and versed procedure, best outcome can be expected.
Accidents, Traffic ; Adult ; Cicatrix ; Facial Bones ; Facial Nerve ; Facial Nerve Injuries ; Fistula ; Follow-Up Studies ; Humans ; Imidazoles ; Mandibular Fractures ; Midazolam ; Mouth ; Nitro Compounds ; Parotid Gland ; Titanium

Objective: A simultaneous detection of germline and somatic mutations in ovarian cancer (OC) using tumor materials is considered to be cost-effective for BRCA1/2 testing. However, there are limited studies of the analytical performances according to various sample types. The aim of this study is to propose a strategy for routine BRCA1/2 next-generation sequencing (NGS) screening based on analytical performance according to different sample types. Methods: We compared BRCA1/2 NGS screening assay using buffy coat, fresh-frozen (FF) and formalin-fixed paraffin-embedded (FFPE) from 130 samples. Results: The rate of repeated tests in a total of buffy coat, FF and FFPE was 0%, 8%, and 34%, respectively. The accuracy of BRCA1/2 NGS testing was 100.0%, 99.9% and 99.9% in buffy coat, FFPE and FF, respectively. However, due to the presence of variant allele frequency (VAF) shifted heterozygous variants, tumor materials (FFPE and FF) showed lower sensitivity (95.5%–99.0%) than buffy coat (100%). Furthermore, FFPE showed 51.4% of the positive predictive value (PPV) on account of sequence artifacts. When performed in the post-filtration process, PPV was increased by approximately 20% in FFPE. Buffy coat showed 100% of sensitivity, specificity and accuracy in BRCA1/2 NGS test. Conclusions On the comparison of the analytical performance according to different sample types, the buffy coat was not affected by sequencing artifacts and VAF shifted variants. Therefore, the blood test should be given priority in detecting germline BRCA1/2 mutation, and tumor materials could be suitable to detect somatic mutations in OC patients without identifying germline BRCA1/2 mutation.

Interests in the microanatomy of the proximal segment of the internal carotid artery(ICA) have been increased due to the anatomical complexity of this region and the potential sites of the aneurysmal formation. In this study, microanatomical dissections were performed in 70 specimens obtained from 35 formalin-fixed human cadaveric heads to examine the superior hypophyseal arteries(SHAs), ophthalmic artery, and the carotid cave. In our study, 54(77%) carotid caves were presented in 70 specimens. Twenty-five(71%, 50 caves) cadavers harbored the carotid caves on both sides, 4(11%, 4 caves) cadavers only on the one side and the remaining 6(17%) didn't have the caval structure. Total of 162 SHAs which have showen to arise from the medial or posterior aspect of the ophthalmic(C6) segment of the ICA in all cases were identified: 108(67%) arose from the proximal half of the C6 segment and 54(33%) from the distal half. Interestingly, in 54 specimens which had the carotid caves, one to four SHAs arose from the part of the C6 segment within the cave in the 43(80%) specimens. Total number of the SHAs originated within the carotid cave were 51. Sixty-four(91%) of ophthalmic arteries have shown to arise just distal to the distal dural ring on C6 segment, whereas 4(6%) from the clinoid(C5) segment and in 2(3%) cases, the ophthalmic artery arose just at the insertion of the distal dural ring level. The authors describe the microanatomical relationships between the SHA and the carotid cave, and the origin of the SHA and the ophthalmic artery. We hope to get better knowledge of the vascular relationship and the possible mechanism of the SHA aneurysm.
Aneurysm ; Arteries* ; Cadaver ; Caves ; Head ; Hope ; Humans ; Ophthalmic Artery

The intracranial hematoma following cerebral aneurysmal rupture makes patient's clinical status and outcome worse by pressure effect on the brain structure. It's incidence has varied with authors between 5-30% of patients with ruptured cerebral aneurysms. Several authors emphasized the importance of early operation to obtain better results by early removal of hematoma and reducing intracranial pressure. The authors retrospectively evaluated seventy-six patients with intracranial hematomas in 370 patients with ruptured aneurysms from Jan. 1987 to Dec. 1992. The incidence of hematoma resulting from ruptured aneurysm was 20.5%. The most frequent site of aneurysm which was accompanied by intracranial hematoma was the middle cerebral artery, and the next was the anterior cerebral artery. The amount of hematoma and/or evidence of midline shift were the important factors in predicting the clinical outcome. The most favorable outcome was found in cases with intracerebral hematoma only, and the worst was in cases with intracerebral hematoma associated with intraventricular hemorrhage. Fifty out of 76 patients with intracranial hematomas were operated on. Their outcomes were more favorable than in the patients who were not operated on. Aggressive surgical intervention was especially effective in patients with poor Hunt & Hess grades(IV or V) .
Aneurysm ; Aneurysm, Ruptured* ; Anterior Cerebral Artery ; Brain ; Hematoma* ; Hemorrhage ; Humans ; Incidence ; Intracranial Aneurysm ; Intracranial Pressure ; Middle Cerebral Artery ; Retrospective Studies ; Rupture

Adenomyoma is a rare non-neoplastic lesion of the biliary tract. Here we report on three cases of adenomyoma; one located in the ampulla of Vater and two located in the common bile duct. Although preoperative endoscopic and radiological evaluations could not determine whether lesions were benign or malignant, intra-operative frozen section histologic examinations aided the differential diagnosis. Microscopic features of a lobular gland architecture with basally located nuclei and the absence of desmoplastic stromal reaction were found to be characteristic in frozen and paraffin sections.
Adenomyoma* ; Ampulla of Vater* ; Biliary Tract ; Common Bile Duct* ; Diagnosis, Differential ; Frozen Sections ; Paraffin

PURPOSE: Purpose: To compare the diagnostic accuracy of ferumoxides-enhanced MR with that of combined CT during arterial portography (CTAP) and CT hepatic arteriography (CTHA) in the preoperative detection of hepatocellular carcinoma (HCC). MATERIALS AND METHODS: For preoperative evaluation, 20 patients with HCC underwent ferumoxides-enhanced MR and combined CTAP and CTHA. The MR protocol included fat-suppressed respiratory-triggered fast spin echo, T2*-weighted fast multiplanar gradient-recalled acquisition in the steady state, proton density-weighted fast multiplanar spoiled gradient-recalled echo, and breath-hold in-phase T1-weighted fast multiplanar spoiled gradient-recalled echo. In all patients, laparotomy was performed. The presence or absence of HCC was confirmed by pathologic examination in the resected liver and by intraoperative ultrasonography of remaining liver, or by follow up. Images were reviewed by three radiologists working independently; regarding the presence or absence of HCC in each segment, each observer assigned one of five confidence levels. A receiver operating characteristic (ROC) curve was fitted to these confidence ratings, and the diagnostic accuracy of each modality was evaluated by calculating the Az value (area under the ROC curve) and compared with that of other modalities. The sensitivity and specificity of each modality in the detection of HCC were also calculated and compared, and using a κstatistic, inter-observer agreement for each modality was assessed. RESULTS: In 28 of 160 liver segments, 30 HCCs were present. For ferumoxide-enhanced MR the mean Az value was 0.958, and for combined CTAP and CTHA this value was 0.948. The difference was not statistically significant. The mean sensitivities of ferumoxide-enhanced MR and combined CTAP and CTHA were 92.9% and 90.9%, respectively, the difference being statistically insignificant. The mean specificities of these modalities were, respectively, 98.9% and 93.6%. The difference was statistically significant. For both ferumoxide-enhanced MR and combined CTAP and CTHA, interobserver agreement was excellent. CONCLUSION: In the preoperative detection of HCC, ferumoxide-enhanced MR imaging of the liver showed a diagnostic accuracy similar to that of combined CTAP and CTHA. Its specificity, however, was higher.
Angiography* ; Carcinoma, Hepatocellular* ; Follow-Up Studies ; Humans ; Iron ; Laparotomy ; Liver ; Liver Neoplasms ; Magnetic Resonance Imaging ; Portography* ; Protons ; ROC Curve ; Sensitivity and Specificity ; Ultrasonography

PURPOSE: To determine treatment protocol for inoperable esophageal cancer patients, we evaluated survival rate and prognostic factors. MATERIALS AND METHODS: We evaluated esophageal cancer treated by curative or palliative aim in KCCH from 1992 to 1996, retrospectively. Recurrent or underdose case below 40 Gy were excluded. The number of male and female were 35 and 5, respectively. Thirty-eight patients were squamous carcinoma and 2 patients were not biopsy proven. Ten patients were treated with radiation therapy and chemotherapy. Median dose of radiation therapy was 59.4 Gy and the range was 40-60 Gy. RESULTS:The median survival is 6.5 months and 1-year survival rate was 28.3%. Age, location, radiation dose and chemotherapy were not significant prognostic factors. Median survivals of patients with below stage III and over stage IVA were 7.6 and 6.2 months respectively, but it is not significant. CONCLUSION:The survival for esophageal cancer is very poor. For patients with curative aim, chemotherapy must be considered. For patients with palliative aim, short-term external beam radiation therapy and/or brachytherapy must be considered.
Biopsy ; Brachytherapy ; Carcinoma, Squamous Cell ; Clinical Protocols ; Drug Therapy ; Esophageal Neoplasms ; Female ; Humans ; Male ; Retrospective Studies ; Survival Rate

Epithelioid sarcoma is a rare tumor which is usually presented with a nontender nodule on a distal extremity. It is sometimes confused with granulomatous process or chronic inflammation. We report of a case of epithelioid sarcoma on a foot of an adult male, which progressed rapidly.
Adult ; Extremities ; Foot* ; Humans ; Inflammation ; Male ; Sarcoma*