Spontaneous Celiac and Splenic Artery Dissection.

Dissection of the splanchnic artery unrelated to an aortic lesion is extremely rare. We describe a patient with dissection of the celiac and splenic arteries causing splenic circulatory impairment. A 55-year-old Japanese man was referred to our hospital for left back pain that suddenly occurred 3 days previously and spread to the left flank. He had complicated sleep apnea syndrome well controlled with continuous positive airway pressure, and had been prophylactically taking aspirin (100 mg/day) because of asymptomatic cerebral lacunar infarcts. Contrast-enhanced computed tomography (CT) in the arterial phase revealed dissection from the celiac root extending to the entire splenic artery, the caliber of which was irregularly narrowed, causing malperfusion in the spleen. Because of hemodynamic stability and lack of impending sequelae, the patient was carefully observed with rest, strict blood pressure control, and aspirin administration. One month later, CT revealed restoration of the caliber of the dissected arteries and regression of the organizing false lumen, which confirmed the patient's recovery. Despite the extreme rarity or nonspecific symptoms, splanchnic artery dissection should be considered a potentially life-threatening emergency. This case supports the possible benefit of starting antithrombotic treatment early to prevent thrombotic sequelae such as organ infarction and aneurysmal formation.

Possible Contribution of a Diverticulum to the Development and Rupture of Colonic Lymphangioma.

A 55-year-old Japanese man with a history of diverticulitis underwent colonoscopy for careful evaluation of progressive anemia. A 5-mm depressed lesion oozing spontaneously was observed at the hepatic flexure. On suspicion of depressed-type of cancer, right-sided hemicolectomy was performed. Histopathological examination indicated a collapsed lymphangioma exactly over a diverticulum, which had previously been complicated diverticulitis. The colonic mucosa and lymphangioma prolapsed beyond the subserosal layer via the muscularis propria defect, resulting in a depressed lesion and mucosal laceration with hemorrhage. This case suggests the contribution of a colonic diverticulum to the development and rupture of lymphangioma, which needed to be distinguished from depressed-type colon cancer.

Endoscopic assessment of reflux esophagitis concurrent with hiatal hernia in male Japanese patients with obstructive sleep apnea.

OBJECTIVE: The pathogenetic relationship underlying the high prevalence of gastroesophageal reflux disease (GERD) in patients with obstructive sleep apnea (OSA) remains unclear. In addition, GERD has not been adequately assessed by endoscopy in patients with OSA. The purpose of this study was to use endoscopy to investigate potential interactions among reflux esophagitis, hiatal hernia (HH) and OSA. MATERIAL AND METHODS: A total of 243 consecutive male Japanese participants who underwent both overnight ambulatory polygraphic monitoring and esophagogastroduodenoscopy were retrospectively evaluated in a cross-sectional study. The prevalence and severity of HH and reflux esophagitis were assessed according to the Los Angeles classification and the Makuuchi classification, respectively. Associations among reflux esophagitis, HH and OSA were examined by univariate and multivariate analyses. RESULTS: OSA was diagnosed in 98 individuals (40.3%). Endoscopy-confirmed esophagitis (p = 0.027) and HH (p < 0.001) were significantly more prevalent among patients with OSA. Multivariate regression model analysis adjusted for age, body mass index, visceral obesity represented by waist circumference, presence of OSA, concurrence of OSA and HH, smoking, and alcohol consumption yielded OSA as the only variable significantly associated with HH (odds ratio [OR], 2.60; 95% confidence interval [CI], 1.35-4.99; p = 0.004), while concurrence of OSA and HH was related to reflux esophagitis (OR, 3.59; CI, 1.87-6.92; p < 0.001). CONCLUSIONS: OSA was associated with HH and concurrent OSA and HH with reflux esophagitis in male Japanese patients with OSA. Our results support the hypothesis that complicating HH may link reflux esophagitis to OSA.

Norovirus gastroenteritis accompanied by marked elevation of transaminases.

A 56-year-old woman was admitted because of frequent watery diarrhea, vomiting, and abdominal cramps. An examination of the stool for norovirus antigen was positive, and a blood examination revealed a marked elevation of liver enzymes. Liver dysfunction, as well as symptoms related to gastroenteritis, was ameliorated solely by supportive treatment. Although liver injury concurrent with norovirus gastroenteritis is rarely documented and its pathogenesis remains unknown, clinicians should consider liver injury as one of the possible extra-intestinal manifestations of norovirus gastroenteritis.

Norovirus gastroenteritis accompanied by ischemic colitis: a case report.

A 57-year-old woman was admitted to our hospital because of frequent bloody diarrhea, vomiting, and abdominal cramps. While the patient was diagnosed with norovirus gastroenteritis based on the presence by chance of positive norovirus antigen in the stool samples, endoscopic as well as pathological examinations demonstrated left-sided ischemic colitis. The patient soon recovered solely by supportive treatment. Although it is believed that patients with viral gastroenteritis do not develop bloody diarrhea, the present case suggested that conditions of viral gastroenteritis, such as intensified peristalsis, elevation of the intraluminal gut pressure, hypovolemia, and hemoconcentration might trigger ischemic colitis resulting in bloody diarrhea. Physicians should not exclude the diagnosis of viral gastroenteritis simply by depending on the presence of bloody diarrhea. Further studies to clarify the incidence of ischemic colitis in patients with viral gastroenteritis are indispensable.

Asymptomatic giant cell hepatitis: a subtype of post-infantile giant cell hepatitis?

Giant cell hepatitis in adults is considered a rapidly progressive and life-threatening disease, but there are few descriptions of a prolonged disease course. A 36-year-old Japanese man was referred to our hospital for further evaluation of abnormal liver function test results. Although asymptomatic, he had undergone follow-up for 9 years with these abnormalities. Because the cause of liver injury was not identified despite extensive noninvasive examinations, the patient underwent needle biopsy. He was finally diagnosed with post-infantile giant cell hepatitis (PIGCH) based on the presence of small numbers of giant multinucleated hepatocytes scattered primarily around the portal area. Necroinflammatory changes were very mild in the portal tracts and hepatic parenchyma. According to the histological findings as well as the accepted international diagnostic scoring system for autoimmune hepatitis (AIH), which is closely related to PIGCH, AIH was unlikely, although antinuclear antibody was positive at a titer of 1:160. The present case may describe an unknown subtype of PIGCH, characterized by insidious disease onset and progression with concurrent, mildly active underlying hepatitis, which is in contrast with the well-documented aggressive nature of PIGCH.

Progressive atrophy in a deformed liver as a contributor to sigmoid volvulus.

The effect of a prior defect on secondary liver atrophy is unknown. We describe a case of sigmoid volvulus that was facilitated by progressive atrophy in a deformed liver. A 75-year-old man with abdominal pain and fullness was referred to our hospital. Computed tomography (CT) revealed reduced left hepatic lobe volume and a whirl sign, characteristic of sigmoid volvulus. The sigmoid volvulus was successfully detorted with endoscopy. Retrospective evaluation of liver morphology on CT and magnetic resonance imaging showed that the portal vein at the liver hilum was denuded due to a parenchymal defect of the medial segment, with compression by the crossing artery. As pulse Doppler ultrasonography demonstrated reduced portal blood flow in the region where liver atrophy developed, compression of the denuded portal vein presumably facilitated secondary atrophy and contributed to sigmoid volvulus. The present case shows that a deformed liver itself can be a cause of secondary atrophy. Therefore, continued monitoring of liver morphology and evaluation of portal blood flow to predict liver atrophy may be required, when an individual with a partial liver defect is encountered.

Acute peri-myocarditis with an unusual initial manifestation of gallbladder edema and a profound eosinophilic surge during convalescence.

A 29-year-old man with fever and right upper quadrant pain was referred to our hospital. Ultrasonography revealed intense gallbladder edema and pericardial effusion. Despite no symptoms and signs related to heart failure, the patient was clinically suspected of cardiac dysfunction related to acute peri-myocarditis based on his symptoms of preceding fever and headache, pericardial effusion, positive troponin T value, elevated B-type natriuretic peptide level, and sequential changes on electrocardiography. With a profound eosinophilic surge (8,022/µL) during convalescence, acute peri-myocarditis and gallbladder edema resolved spontaneously. This case instructively shows that acute peri-myocarditis initially manifests with abdominal symptoms, mimicking acute acalculous cholecystitis. In addition, an extensive review of acute myocarditis cases with peripheral eosinophilia suggests that there is a subgroup characterized by a predilection for young and middle-aged men, concurrence of pericardial effusion, transient eosinophilic surge during convalescence, and favorable outcome.

A variant of thyrotoxicosis associated with chronic thyroiditis characterized by prolonged fever, absence of anti-thyroidal antibodies, and favorable response to naproxen.

An unusual form of thyrotoxicosis due to chronic thyroiditis is described. A 78-year-old debilitated woman was admitted because of fever to 38 degrees C persisting for the previous 16 months, for which the antipyretic effect of diclofenac sodium and pranoprofen had been insufficient or transient. Intense accumulation of gallium-67 citrate in the thyroid gland provided an initial clue to the diagnosis of masked thyrotoxicosis as a cause of the fever, and naproxen (300 mg/d) eliminated the fever and flaring of thyrotoxicosis. Despite the absence of autoantibodies related to chronic thyroiditis, needle biopsy revealed destructive thyroiditis due to chronic lymphocytic thyroiditis. This case suggests a previously unrecognized variant of thyrotoxicosis due to chronic thyroiditis, that is, neither painless thyroiditis nor acute exacerbation of Hashimoto thyroiditis, and instead characterized by prolonged fever, nontender thyroid, absence of antibodies associated with autoimmune thyroiditis, and excellent response to naproxen.

[Diffuse large B-cell lymphoma initially manifested by massive ascites and a small gastric lesion, clinically mimicking primary effusion lymphoma (PEL) in the abdominal cavity: a case report and review of the literature on Japanese PEL patients].

A 50-year-old man was admitted because of increasing massive ascites. While lymphoma cells (diffuse large B-cell lymphoma: DLBCL) were detected in the ascites, pleural effusion, cerebrospinal fluid and bone marrow, no tumor masses other than a submucosal lymphoma lesion in the stomach only 1 cm in diameter were identified. The patient was treated with chemotherapy including rituximab (R-CHOP-ESHAP) and injection of methotrexate and dexamethasone into the medullary cavity as well as radiation to the whole brain, and achieved complete remission 4 months later. The present case suggests that DLBCL can initially manifest as a form of effusion lymphoma with minimum solid tumor component. The distinctive clinical features of Japanese patients with primary effusion lymphoma are also reviewed.

Follicular lymphoma-related colitis resembling ulcerative colitis.

Among the various manifestations of colonic involvement in malignant lymphomas, an ulcerative colitis-like appearance is rare. Herein, we describe a case in which extranodal colonic involvement resembled ulcerative colitis in a patient with advanced follicular lymphoma. A 59-year-old Japanese man with diarrhea and body weight loss was referred to our hospital. Computed tomography (CT) revealed systemic lymphadenopathy, splenomegaly, and thickening of the sigmoid colon and rectum walls. 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET/CT) revealed intense tubular 18F-FDG accumulation extending from the rectum to the colon at the hepatic flexure and much weaker accumulation in the systemic lymph nodes, bone marrow, and spleen. The isotope-enriched areas had an ulcerative colitis-like appearance as shown via colonoscopy. The patient was ultimately diagnosed with follicular lymphoma (stage IV A, grade 1) based on a pathological examination of the neck lymph nodes, iliac bone marrow, and colon. After six courses of chemotherapy (R-CHOP), 18F-FDG-PET/CT confirmed complete remission of the lymphoma including the colonic lesion. This is presumably the first case of ulcerative-like colitis caused by a follicular lymphoma. As a novel approach, the lymphoma-related colitis was detected by comparing the pathology results and the 18F-FDG-PET/CT results.

Acute malignancy-related hydrocephalus uncovered after endoscopic manipulation: possible influence of gastrointestinal endoscopy on concurrent intracranial lesion.

A 78-year-old man underwent biliary stenting by means of endoscopy for obstructive jaundice due to common bile duct cancer. Despite uneventful operation, the patient remained comatose and began to hiccup and vomit with marked gastrointestinal gas retention. Neuroimaging studies disclosed obstructive hydrocephalus caused by a midbrain metastasis, probably from coexisting lung cancer. Ventricular peritoneal shunting restored consciousness and eliminated other gastrointestinal symptoms. This case suggests that gastrointestinal endoscopy may affect concurrent intracranial lesions, resulting in acute distress, presumably by transmission of pressure from the abdomen to a distant body compartment such as the intracranial space, or by endoscopy-related hypoventilation.

Achilles tendinitis as a rare extraintestinal manifestation of ulcerative colitis.

Patients with inflammatory bowel disease often have extraintestinal manifestations (EIMs) involving almost all organ systems, but little has been reported on Achilles tendinitis. Herein, we present a unique case of Achilles tendinitis, which manifested shortly after initiation of mesalazine therapy for ulcerative colitis. A 26-year-old Japanese woman with bloody diarrhea and abdominal cramps lasting for 7 days was referred to our hospital. The Lichtiger clinical activity index (CAI) score was 9 at the first visit. Based on the clinical symptoms and examination results, she was diagnosed with ulcerative pancolitis in the active phase, and treatment with mesalazine (2.4 g/day) and probiotics was initiated. Her symptoms resolved within 7 days of treatment (CAI 3). However, she then developed bilateral Achilles tendinitis without any apparent cause. The Achilles tendinitis subsided with conservative management within 2 weeks, despite continuation of mesalazine therapy. This case instructively suggests that Achilles tendinitis should be noted as an EIM of ulcerative colitis.

Small follicular lymphoma arising near the ampulla of vater: a distinct subtype of duodenal lymphoma?

A 49-yr-old Japanese woman underwent upper gastrointestinal endoscopy because of nonspecific dyspepsia. Endoscopy revealed a flat elevated lesion about 15 mm in diameter adjacent to the duodenal papilla, the surface of which was uneven and covered with whitish granules. Based on the results of histological examination with immunohistochemistry (positive for CD10, CD20, CD79a, and bcl-2 protein, negative for CD5 and cyclin D1), a diagnosis of grade 1/3 follicular lymphoma was established. Systemic staging examinations suggested the lymphoma was restricted to the mucosa and superficial portion of the submucosa in the duodenal wall. The patient was treated with a combination of CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone) and monoclonal anti-CD20 antibody (rituximab), in addition to radiotherapy. After six courses of this combination chemotherapy, complete regression of the lymphoma was observed. Although reports of small duodenal lymphoma (<20 mm or localized to the mucosa or submucosa) are extremely rare, the features of this case are characteristic of small duodenal lymphoma in terms of evolution around the ampulla of Vater, low-grade follicular type, occurrence in a women, occurrence in the fourth decade of life, and favorable outcome, and this type of tumor may need to be distinguished by pathogenesis and clinical behavior from various other gastrointestinal lymphomas.

Primary minute invasive de novo colonic adenocarcinoma appearing as submucosal tumor.

A 4 mm white-yellow submucosal tumor-like lesion was detected in the sigmoid colon of an asymptomatic 52-yr-old Japanese man. Because the lesion was unexpectedly suspicious for adenocarcinoma by pathological examination of the biopsy specimen, it was treated by endoscopic mucosal resection. The specimen obtained demonstrated well-differentiated adenocarcinoma without any adenomatous element, and was located principally in the submucosal layer with a maximum depth of 1600 mum from the muscularis mucosae. The cancer exposed to the luminal surface was pathologically concluded to be diminutive. Intriguingly, aggregation of lymphocytes was found beneath the mucosal layer, which might have compromised the integrity of the muscularis mucosae. Because of deep submucosal infiltration and the latent aggressive nature of de novo cancer, the patient underwent an additional partial sigmoidcolectomy, which demonstrated no residual cancer and no regional lymph node metastasis. The lesion in this patient exhibited a previously undescribed appearance of de novo colon cancer as submucosal tumor in an early phase of growth.

Severe alcoholic hepatitis accompanied by Fournier's gangrene.

Fulminant necrotizing soft-tissue infection of the external genitalia and perineum (Fournier's gangrene) occurred in a patient with severe alcoholic hepatitis. By means of radical débridement and disinfection of the necrotizing tissue, use of broad-spectrum antibiotics and prednisolone, and other supportive measures, Fournier's gangrene and severe alcoholic hepatitis eventually subsided with broad skin defects in the waist and external genitalia. Later, the skin defects were successfully reconstructed with skin grafts. Although the route of bacterial intrusion could not be defined, Fournier's gangrene presumably developed in a background of impaired immunological defences, principally associated with habitual massive alcohol consumption and profound liver dysfunction. This present case highlights not only the underlying immunocompromised condition but also the need for concern regarding the urogenital and anorectal areas as potential foci of life-threatening infection in patients with liver dysfunction, especially when related to alcohol intake.

Does the use of insulin in a patient with liver dysfunction increase water retention in the body, i.e. cause insulin oedema?

A 68-year-old female with mild diabetes mellitus was admitted because of acute liver dysfunction due to autoimmune hepatitis. While 40 mg/day of prednisolone improved hepatic dysfunction dramatically, her diabetic milieu deteriorated seriously. The induced hyperglycaemia could not be controlled sufficiently, despite a high dose of insulin (> 110 units/day), suggesting the existence of insulin insensitivity and hyperinsulinaemia. Soon after introduction of insulin therapy, she developed severe anasarca, including marked peripheral oedema, ascites and pleural effusion. Anasarca eventually subsided within 4 weeks with the use of a diuretic agent. We conjectured that the side effects of insulin, such as anti-natriuresis and increased vascular permeability, might be pronounced in the presence of the hepatic dysfunction that accompanies insulin insensitivity, hyperinsulinaemia and hypoalbuminaemia.

Significant liver injury with dual positive IgM antibody to Epstein-Barr virus and cytomegalovirus as a puzzling initial manifestation of infectious mononucleosis.

A 35-year-old man was admitted because of significant hepatic dysfunction with mild splenomegaly and intra-abdominal lymphadenopathy of unknown cause. Infectious mononucleosis was suggested by subsequently detected high fever, pharyngotonsillitis and cervical lymphadenopathy, but IgM to Epstein-Barr virus (EBV) and cytomegalovirus (CMV) showed dual positivity. A definite diagnosis of EBV-induced infectious mononucleosis was established 3 months later on the basis of seroconversion to Epstein-Barr nuclear antigen (EBNA)-IgG positivity and reduced CMV-IgM titer with persistently negative CMV-IgG. This case highlights the initial diagnostic difficulties of EBV-induced infectious mononucleosis particularly in older patients, due to concomitant abnormal humoral immunity and unusual initial manifestations such as significant liver injury and extensive intra-abdominal lymphadenopathy.