Melkersson-Rosenthal syndrome(MRS) is a rare disorder, having a symptom triad of recurrent facial palsy, orofacial swelling and fissured tongue(lingua plicata). This disorder is usually recurrent or progressive, and monosymptomatic or oligosymptomatic forms have been reported to be more common than classic forms. Generally, MRS occurs in young adults at the end of the second decade of life and incidence of the disease in childhood is known to be very low. Although the clinical manifestation of MRS in children is similar to that in adults, early diagnosis and management is essential to avoid long-lasting functional disorders and psychological problems. We experienced MRS in a 13 year old boy with a history of recurrent facial palsy. We report this case with review of related literature.
Adolescent ; Adult ; Child ; Early Diagnosis ; Facial Paralysis ; Humans ; Incidence ; Male ; Melkersson-Rosenthal Syndrome* ; Young Adult

Allergic angitis and granulomatosis is a kind of rare systemic vasculitis, with various manifestations of disease of lung, heart, skin, musculoskeletal system, nervous system and hepatobiliary tract. There was no report of a case with manifestation of pleuritis and pericarditis, while several cases had been reported in Korea. So we here report a case of allergic angitis and granulomatosis with manifestations of pleuritis and pericarditis. The case also showed clinical manifestations of hypereosinophilia, asthma, rhinitis, pulmonary infilterates with eosinophilia and nephritis. Open lung biopsy showed arteritis with heavy infilteration of activated eosinophil in lung, pleura and pericardium. The involvement of heart might cause critical complication leading death. The patients who are supposed as allergic angitis and granulomatosis should be examined for the involvement of heart.
Arteritis ; Asthma ; Biopsy ; Eosinophilia ; Eosinophils ; Heart ; Humans ; Korea ; Lung ; Musculoskeletal System ; Nephritis ; Nervous System ; Pericarditis* ; Pericardium ; Pleura ; Pleurisy* ; Rhinitis ; Skin ; Systemic Vasculitis

OBJECTIVE: Recently some reports suggested substance P and CGRP might be important factors for inflammation and hyperalgesia. This study was performed to see whether substance P or CGRP containing nerve fibers might be changed by mustard oil-induced inflammation. METHODS: After injection of mustard oil(5%) into uterine lumen, the uteri were removed and examined with immunohistochemical methods for substance P and CGRP. RESULTS: In the normal uterus, most of the substance P- or CGRP-immunoreactive nerve fibers were observed along the vascular structure and some in the myometrium, only few in the endometrium. Mustard oil did not changed this pattern of nerve fiber distribution but after 48 hrs, the amount of substance P or CGRP immunoreactive nerve fibers were greatly reduced compared with the normal uterus. It is not clear whether the decrease of substance P and CGRP immunoreactive fibers in the uterus was resulted from the depletion of the neuropeptides in the nerve fibers or the retraction of nerve fibers. CONCLUSIONS: These results suggest that the inflammation should cause the change of nerve fibers included in the nociception. This change may attribute the generation of inflammation and inflammatory hyperalgesia.
Animals ; Endometrium ; Female ; Hyperalgesia ; Inflammation* ; Mice ; Mustard Plant ; Myometrium ; Nerve Fibers ; Neuropeptides ; Nociception ; Rats* ; Substance P* ; Uterus*

Granular cell tumors (GCTs) have been reported in various tissues, especially the skin and subcutaneous soft tissue of the head and neck. We report a 60-year-old man who presented with intermittent headache and dizziness for 3 months, but no other neurological symptoms. Magnetic resonance imaging (MRI) showed the presence of a mass in the pituitary stalk, and contrast-enhanced MRI showed nodular enhancement in this region. The lesion was completely excised microscopically via a frontotemporal (pterional) approach. On pathological examination, a final diagnosis of a typical GCT was made.
Diagnosis ; Dizziness ; Granular Cell Tumor* ; Head ; Headache ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neck ; Pituitary Gland* ; Pituitary Neoplasms ; Skin

Natural killer (NK) cell neoplasms are a group of rare but highly malignant tumors. We report here one case of NK cell leukemia. A 54-yr-old woman presented with a 2-month history of progressive left neck mass. Based on the positive result of tissue PCR for Mycobacterium tuberculosis, she was at first diagnosed with tuberculous lymphadenopathy. After two weeks, she developed generalized lymphadenopathy, hepatosplenomegaly, fever and anemia. Subsequent evaluation was performed including bone marrow aspiration and biopsy. Peripheral blood smear showed leukoerythroblastic features with 31% blasts. Bone marrow was packed with agranular blastoid cells, which were periodic acid-Schiff (PAS) positive and myeloperoxidase (MPO) negative. Immunophenotyping showed that these cells were positive for CD45 and HLA-DR, whereas negative for CD3, CD5, CD7, CD10, CD13, CD14, CD19, CD20, CD22, CD33, CD34, and CD61. Because of the absence of the markers of T-cell, B-cell, and myeloid lineage-specific antigens, we added CD16/56 for the immunophenotyping and the blasts were positive (94%). The tumor cells of biopsied lymph node were only positive for CD56, consistent with NK cell lymphoma. Epstein-Barr virus (EBV) was not detected by RNA in situ hybridization. Culture for M. tuberculosis was negative. Thus this patient was diagnosed with blastic NK cell lymphoma/leukemia involving bone marrow and lymph node.
Antigens, CD45/metabolism ; Bone Marrow/pathology ; Female ; HLA-DR Antigens/metabolism ; Humans ; Killer Cells, Natural/immunology/*pathology ; Leukemia/*diagnosis/immunology/pathology ; Middle Aged ; Tuberculosis, Lymph Node/diagnosis

BACKGROUND/AIMS: Determining the cause of suspected biliary stricture is often challenging in clinical practice. We aimed to compare the diagnostic yields of endoscopic ultrasound-guided tissue sampling (EUS-TS) and endoscopic retrograde cholangiopancreatography-guided tissue sampling (ERCP-TS) in patients with suspected biliary stricture at different primary lesions. METHODS: We enrolled patients who underwent same-session EUS- and ERCP-TS for the evaluation of suspected biliary stricture. Forceps biopsy and/or brush cytology of intraductal lesions and fine-needle aspiration for solid mass lesions were performed during ERCP and EUS, respectively. RESULTS: One hundred and twenty-five patients treated at our institution between January 2011 and September 2016, were initially considered for the study. However, 32 patients were excluded due to loss of follow-up (n=8) and ERCP-TS on the pancreatic duct (n=20) or periampullary lesions (n=4). Of the 93 patients included, 86 had a malignant tumor including cholangiocarcinoma (n=39), pancreatic cancer (n=37), and other malignancies (n=10). Seven patients had benign lesions. EUS-TS had higher rate of overall diagnostic accuracy than ERCP-TS (82.8% vs. 60.2%, p=0.001), and this was especially true for patients with a pancreatic lesion (84.4% vs. 51.1%, p=0.003). CONCLUSIONS: EUS-TS was found to be superior to ERCP-TS for evaluating suspected biliary strictures, especially those caused by pancreatic lesions.
Biopsy ; Biopsy, Fine-Needle ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Constriction, Pathologic ; Diagnosis ; Endosonography ; Follow-Up Studies ; Humans ; Pancreatic Ducts ; Pancreatic Neoplasms ; Surgical Instruments

Background/Aims: Endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) is integral to the diagnosis of gastrointestinal (GI) subepithelial tumors (SETs). The impact of different EUS-FNB tissue sampling techniques on specimen adequacy and diagnostic accuracy in SETs has not been fully evaluated. This study aimed to compare the diagnostic outcomes of slow-pull (SP) and standard suction (SS) in patients with GI SETs. Methods: In this retrospective comparative study, 54 patients were enrolled. Medical records were reviewed for location and size of the target lesion, FNB needle type/size, technical order, specimen adequacy, diagnostic yield, and adverse events. The acquisition rate of adequate specimens and diagnostic accuracy were compared according to EUS-FNB techniques. Results: The mean lesion size was 42.6±36.4 mm, and most patients were diagnosed with GI stromal tumor (75.9%). The overall diagnostic accuracies of the SP and SS techniques were 83.3% and 81.5%, respectively (p=0.800). The rates of obtaining adequate core tissue were 79.6% and 75.9%, respectively (p=0.799). No significant clinical factors affected the rate of obtaining adequate core tissue, including lesion location and size, FNB needle size, and final diagnosis. Conclusions SP and SS had comparable diagnostic accuracies and adequate core tissue acquisition for GI SETs via EUS-FNB.

Xanthogranulomatous inflammation is a rare benign condition involving various organs. However, its pancreas involvement is very rare. To the best of our knowledge, only 17 cases have been described in the literature. Interestingly, all reported 17 cases due to various causes underwent surgical resection. Here, we present a case of xanthogranulomatous pancreatitis in a 63-year-old man. He presented with epigastric pain and solid mass mimicking ductal adenocarcinoma in the body and tail of pancreas on magnetic resonance imaging. The patient was diagnosed as xanthogranulomatous pancreatitis via endoscopic ultrasound-guided fine needle aspiration. After that, he was followed up and monitored without any surgical treatment. Here, we show imaging findings and serial image changes of xanthogranulomatous pancreatitis for this case.
Adenocarcinoma ; Endoscopic Ultrasound-Guided Fine Needle Aspiration ; Humans ; Inflammation ; Magnetic Resonance Imaging* ; Middle Aged ; Pancreas ; Pancreatic Neoplasms* ; Pancreatitis* ; Tail

BACKGROUNG: The aim of this study was to investigate the mid-term outcomes of our modifications to the maze procedure using cryoablation for treating atrial fibrillation associated with rheumatic mitral valve disease. MATERIAL AND METHOD: Between March 2000 and February 2004, 177 consecutive patients underwent the modified maze procedure with the use of cryoablation concomitant with mitral valve surgery for atrial fibrillation associated with rheumatic mitral valve disease, and were divided into three groups: (1) modified Cox-maze III (CM group, n=88); (2) modified Kosakai-maze (KM group, n=63); and (3) left atrial maze procedure (LA group, n=26). The postoperative and follow- up results were analyzed and compared between the groups. RESULT: There were three hospital deaths (1.7%) and no significant differences in the incidence of postoperative complications between the three groups. The operative time, such as the cardiopulmonary bypass and aortic crossclamp time, were significantly longer in the CM group than in the KM and LA groups, respectively (p<0.0001). The mean follow-up was 22.4+/-15.1 months (1~52.6 months) for all patients. One late death developed in the CM group (0.6%). At last follow-up, 139 patients exhibited sinus rhythm (79.9%), which was also regained in 67 patients (77.9%) in the CM group, 50 (80.7%) in the KM group and 22 (84.6%) in the LA group (p=0.743). The actuarial freedom from stroke at 4 years was 84.6+/-9.4% in the CM group, 95.0+/-4.9% in the KM group, and 92.9+/-6.9% in the LA group (p=0.916). CONCLUSION: The modified maze procedure using cryoablation is safe and effective in treating chronic atrial fibrillation associated with rheumatic mitral valve disease.
Atrial Fibrillation* ; Cardiopulmonary Bypass ; Cryosurgery ; Follow-Up Studies ; Freedom ; Humans ; Incidence ; Mitral Valve* ; Operative Time ; Postoperative Complications ; Rheumatic Diseases ; Stroke

Multidirectional coronary artery fistulas (CAFs) are rare in patients with tetralogy of Fallot (TOF). We report an adult patient who underwent open-heart surgery for TOF 24 years before the discussed presentation. Coronary angiogram and cardiac computed tomography revealed multidirectional CAFs originating from the left main coronary artery and draining to the left atrium, pulmonary artery and subphrenic artery. The patient also showed additional congenital anomalies, such as persistent left superior vena cava and right-sided aortic arch.
Adult* ; Aorta, Thoracic ; Arteries ; Arteriovenous Fistula ; Coronary Vessels* ; Fistula* ; Heart Atria ; Humans ; Pulmonary Artery ; Tetralogy of Fallot* ; Vena Cava, Superior