Mazabraud syndrome (MS) is a rare and sporadic disorder. It is mainly characterized by fibrous dysplasia (FD) of single or multiple bones and intramuscular myxomas (IM). Data on the prevalence since it was first reported, clinical features, and prognosis are extremely scarce. We report a case of a 59-year-old woman with IM and polyostotic FD. She also had multiple cafe’-au-lait spots suggestive of McCune-Albright syndrome (MAS). On magnetic resonance imaging, there are masses with well-defined heterogeneous enhancement, accompanied by an inner cyst in the vastus lateralis muscle and femur. These radiological results are identical to those of FD. After surgical intervention with excision of intramuscular soft-tissue mass, a diagnosis of IM of MS was confirmed. Given that cafe’-au-lait spots also appeared, the patient was diagnosed with a variant of MS with some of the clinical characteristics of MAS.

Sebaceous neoplasms are rare adnexal tumors that can present a challenge to clinicians. Only four cases of sebaceous carcinoma with sebaceoma have been reported in the literature. Herein, we describe the case of a sebaceous carcinoma originating from a sebaceoma in a solitary nodule of the posterior neck. Immunohistochemically, the tumor cells were strongly positive for epithelial membrane antigen and p53. It is possible that adnexal carcinomas may arise from malignant transformation of their benign counterparts as well as de novo. Malignant transformation was likely in this case because the lesion was composed of distinct benign and malignant components, and the benign component showed the typical histopathological features of sebaceoma. This case underscores the fact that partial and superficial biopsies sometimes may not provide the correct diagnosis. If a surgeon suspects malignancy based on a clinical examination, then it is mandatory to perform a deep biopsy.

Sebaceous neoplasms are rare adnexal tumors that can present a challenge to clinicians. Only four cases of sebaceous carcinoma with sebaceoma have been reported in the literature. Herein, we describe the case of a sebaceous carcinoma originating from a sebaceoma in a solitary nodule of the posterior neck. Immunohistochemically, the tumor cells were strongly positive for epithelial membrane antigen and p53. It is possible that adnexal carcinomas may arise from malignant transformation of their benign counterparts as well as de novo. Malignant transformation was likely in this case because the lesion was composed of distinct benign and malignant components, and the benign component showed the typical histopathological features of sebaceoma. This case underscores the fact that partial and superficial biopsies sometimes may not provide the correct diagnosis. If a surgeon suspects malignancy based on a clinical examination, then it is mandatory to perform a deep biopsy.

We experienced a case, which showed the sudden drop of arterial oxygen saturation and capnographic score in a femur surgery patient under general anesthesia. We installed a transesophageal echocardiography probe in the patient and detected free-floating emboli in the right atrium, enlargement of the right atrium and the right ventricle, and global hypokinesia of the right ventricle. Because the patient's vital sign was so unstable, emergent cardiac and pulmonary embolectomy was performed with the use of cardiopulmonary bypass. The postoperative course was uneventful and the patient was discharged without any neurologic sequale. We concluded that transesophageal echocardiography was a useful device for the diagnosis of intraoperative massive pulmonary thromboembolism.
Anesthesia, General ; Cardiopulmonary Bypass ; Echocardiography, Transesophageal ; Embolectomy ; Femur ; Heart Atria ; Heart Ventricles ; Humans ; Hypokinesia ; Oxygen ; Pulmonary Embolism ; Vital Signs

Tracheal rupture is a rare complication of tracheal intubation, but may result in pneumothorax, mediastinal emphysema, subcutaneous emphysema and other serious outcomes. Risk factors associated with tracheobronchial rupture include multiple vigorous attempts at intubation, inexperienced operator, intubating stylets, tracheal abnormalities, overdistension of tracheal or bronchial cuff, repositioning of tube without deflating the cuff, chronic obstructive airway disease and vigorous coughing while being intubated. We report a case of tracheal rupture after using single lumen endotracheal tube. A 41-year-old, 53 kg, female was operated for a repair of anterior cruciate ligament under general anesthesia in other hospital. Two hours later after the end of operation, facial swelling, dyspnea and chest pain developed. Chest computed tomography showed mediastinal emphysema. She was transferred to our hospital and bronchoscopy showed a rupture at posterior part of membranous portion 2 cm proximal to carina. Trachea was repaired and she was discharged from the hospital without complication.
Adult ; Anesthesia, General ; Anterior Cruciate Ligament ; Bronchoscopy ; Chest Pain ; Cough ; Dyspnea ; Emphysema ; Female ; Humans ; Intubation ; Mediastinal Emphysema ; Pneumothorax ; Pulmonary Disease, Chronic Obstructive ; Risk Factors ; Rupture* ; Subcutaneous Emphysema ; Thorax ; Trachea

Background: Frontal sinus fractures are relatively rare. Their surgical management significantly differs depending on whether the posterior wall is invaded and the clinical features vary. A bicoronal incision or endoscopic approach can be used. However, the minimally invasive approach has been attracting attention, leading us to introduce a simple and effective surgical method using multiple-threaded Kirschner wires. Methods: All patients had isolated anterior wall fractures without nasofrontal duct impairment. The depth from the skin to the posterior wall was measured using computed tomography to prevent injury. The edge of the bone segment on the skin was marked, a threaded Kirschner wire was inserted into the center of the bone segment, and multiple Kirschner wires were gently reduced simultaneously. Results: Surgery was performed on 11 patients. Among them, seven patients required additional support for appropriate fracture reduction. Therefore, a periosteal elevator was used as an adjunct through a small sub-brow incision because the reduction was incomplete with the Kirschner wire alone. The reduction results were confirmed using facial bone computed tomography 1 to 3 days postoperatively. The follow-up period was 3 to 12 months. Conclusion The patients had no complications and were satisfied with the surgical results. Here we demonstrated an easy and successful procedure to reduce a pure anterior wall frontal sinus fracture via non-invasive threaded Kirschner wire reduction.

PURPOSE: The aim of this study was to analyze survival outcomes in 1,000 consecutive liver transplantations (LTs) performed at a single institution from 1993 to April 2017. METHODS: The study population was divided into 2 groups based on donor type: deceased donor LT (DDLT; n = 181, 18.1%) and living donor LT (LDLT; n = 819; 81.9%), and into 3 periods based on the number of cases (first 300 cases, middle 300 cases, last 400 cases). RESULTS: Infection was the most common cause of death, accounting for 34.8% (95 of 273). Mortality due to hepatocellular carcinoma recurrence occurred most frequently between 1 and 5 years after transplantation. Mortality rate by graft rejection was highest between 5 and 10 years after transplantation. And mortality by de novo malignancy occurred most frequently after 10 years after transplantation. The patient survival rates for the entire population at 5 and 10 years were 74.7%, and 68.6%, respectively. There was no difference in survival rate between the LDLT and DDLT groups (P = 0.188). Cause of disease, disease severity, case period, and retransplantation had a significant association with patient survival (P = 0.002, P = 0.031, P = 0.003, and P = 0.024, respectively). CONCLUSION: Surgical techniques and perioperative management for transplant patients have improved and undergone standardization. Controlling perioperative infection and managing patients with HCC as LT candidates will result in better outcomes.
Carcinoma, Hepatocellular ; Cause of Death ; Graft Rejection ; Humans ; Liver Transplantation* ; Liver* ; Living Donors ; Mortality ; Recurrence ; Risk Factors ; Survival Rate ; Tissue Donors

Pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease and usually presents as pulmonary masses, mass-like consolidation, or pulmonary nodules on chest images. We report a case of a 43-year-old man with symptoms of chronic cough for 1 year, showing bilateral diffuse bronchovascular bundle thickening and focal ground glass opacities on a chest computed tomography scan. Video-assisted thoracoscopic surgery was performed and the final pathologic diagnosis was pulmonary MALT lymphoma. Concurrent involvement of the pancreas was discovered during staging workup. After diagnosis, he was treated with cytotoxic chemotherapy and rituximab and showed improvements in his lung lesion and pancreas.
Adult ; Cough ; Diagnosis ; Drug Therapy ; Glass* ; Humans ; Lung ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Pancreas* ; Rare Diseases ; Rituximab ; Thoracic Surgery, Video-Assisted ; Thorax

BACKGROUND: Nuss et al introduced a less invasive method for inserting a stainless steel bar through the small incision on the lateral chest wall into the pectus excavatum. This study was undertaken to assess the effect of the Nuss operation on lung mechanics, CT-Index and hemodynamics. METHODS: Twenty patients (age 4 to 17 years) with severe pectus excavatum underwent the Nuss operation. CT-Index (the internal transverse distance of the thorax/the vertebral-sternal distance at greatest depression) was evaluated before operation. Lung mechanics (dynamic compliance [Cdyn], static compliance [Cstat] and airway resistance [Raw]), hemodynamic changes (heart rate [HR], systolic blood pressure [SBP], and diastolic blood pressure [DBP]), and gas exchange (arterial oxygen tension [PaO2], arterial carbon dioxide tension [PaCO2], pulse oximeter saturation [SPO2] and end-tidal carbon dioxide tension[PETCO2]) were measured before and after the operation. RESULTS: Cdyn and Cstat decreased significantly (P < 0.05), but Raw did not change. PaCO2 and PETCO2 decreased significantly (P < 0.05), and SBP and DBP increased significantly (P < 0.05) postoperatively. CONCLUSIONS: It is concluded that decreased compliance after the Nuss operation may result from reduced thoracic elastance, not to a change of lung parenchyma.
Airway Resistance ; Blood Pressure ; Carbon Dioxide ; Compliance ; Funnel Chest* ; Hemodynamics ; Humans ; Lung* ; Mechanics* ; Oxygen ; Stainless Steel ; Thoracic Wall

PURPOSE: To evaluate the treatment results in early stage non-small cell lung cancer patients who have undergone fiducial-less CyberKnife radiosurgery (CKRS). MATERIALS AND METHODS: From June 2011 to November 2013, 58 patients underwent CKRS at Asan Medical Center for stage I lung cancer. After excluding 14 patients, we retrospectively reviewed the records of the remaining 44 patients. All analyses were performed using SPSS ver. 21. RESULTS: The median age at diagnosis was 75 years. Most patients had inoperable primary lung cancer with a poor pulmonary function test with comorbidity or old age. The clinical stage was IA in 30 patients (68.2%), IB in 14 (31.8%). The mean tumor size was 2.6 cm (range, 1.2 to 4.8 cm), and the tumor was smaller than 2 cm in 12 patients (27.3%). The radiation dose given was 48-60 Gy in 3-4 fractions. In a median follow-up of 23.1 months, local recurrence occurred in three patients (2-year local recurrence-free survival rate, 90.4%) and distant metastasis occurred in 13 patients. All patients tolerated the radiosurgery well, only two patients developing grade 3 dyspnea. The most common complications were radiation-induced fibrosis and pneumonitis. Eight patients died due to cancer progression. CONCLUSION: The results showed that fiducial-less CKRS shows comparable local tumor control and survival rates to those of LINAC-based SABR or CKRS with a fiducial marker. Thus, fiducial-less CKRS using Xsight lung tracking system can be effectively and safely performed for patients with medically inoperable stage I non-small cell lung cancer without any risk of procedure-related complication.
Carcinoma, Non-Small-Cell Lung* ; Chungcheongnam-do ; Comorbidity ; Diagnosis ; Dyspnea ; Fibrosis ; Fiducial Markers ; Follow-Up Studies ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Pneumonia ; Radiosurgery* ; Recurrence ; Respiratory Function Tests ; Retrospective Studies ; Survival Rate