Case 296: Phlebosclerotic Colitis.

History A 55-year-old woman without systemic underlying disease, such as diabetes mellitus, inflammatory bowel disease, autoimmune disease, or chronic kidney disease, presented with generalized dull abdominal pain of 1-week duration. She had ingested herbal medicine for physical conditioning for several years. Laboratory findings, including biochemistry, electrolyte levels, and complete blood count, were all within normal limits, except for elevated serum C-reactive protein level (7.719 mg/dL; normal range, <1 mg/dL). The patient underwent initial evaluation with conventional abdominal radiography. She underwent subsequent evaluation with noncontrast CT of the abdomen and colonoscopy.

Case 296.

History A 55-year-old woman without systemic underlying disease, such as diabetes mellitus, inflammatory bowel disease, autoimmune disease, or chronic kidney disease, presented with generalized dull abdominal pain of 1-week duration. She had ingested herbal medicine for physical conditioning for several years. Laboratory findings, including biochemistry, electrolyte levels, and complete blood count, were all within normal limits, except for elevated serum C-reactive protein level (7.719 mg/dL; normal range,<1 mg/dL). The patient underwent initial evaluation with conventional abdominal radiography (Fig 1). She underwent subsequent evaluation with noncontrast CT of the abdomen (Figs 2, 3) and colonoscopy (Fig 4).

Reappraisal of the Roles of the Sonic Hedgehog Signaling Pathway in Hepatocellular Carcinoma.

HCC remains one of the leading causes of cancer-related death globally. The main challenges in treatments of hepatocellular carcinoma (HCC) primarily arise from high rates of postoperative recurrence and the limited efficacy in treating advanced-stage patients. Various signaling pathways involved in HCC have been reported. Among them, the Sonic hedgehog (SHH) signaling pathway is crucial. The presence of SHH ligands is identified in approximately 60% of HCC tumor tissues, including tumor nests. PTCH-1 and GLI-1 are detected in more than half of HCC tissues, while GLI-2 is found in over 84% of HCC tissues. The SHH signaling pathway (including canonical and non-canonical) is involved in different aspects of HCC, including hepatocarcinogenesis, tumor growth, tumor invasiveness, progression, and migration. The SHH signaling pathway also contributes to recurrence, metastasis, modulation of the cancer microenvironment, and sustaining cancer stem cells. It also affects the resistance of HCC cells to chemotherapy, target therapy, and radiotherapy. Reappraisal of the roles of the SHH signaling pathway in HCC may trigger some novel therapies for HCC.

N-butyl cyanoacrylate embolization as the primary treatment of acute hemodynamically unstable lower gastrointestinal hemorrhage.

PURPOSE: To evaluate N-butyl cyanoacrylate (NBCA) embolization as the primary treatment for patients with severe and acute hemodynamically unstable lower gastrointestinal bleeding. MATERIALS AND METHODS: Twenty-seven patients with acute, unstable hemodynamics caused by lower gastrointestinal bleeding underwent therapeutic NBCA microcatheter embolization over a period of 27 months. The inclusion criteria were hematochezia or melena and hypotension refractory to conservative treatment and requiring blood transfusion. Bleeding was localized to the rectum, colon, or small intestine in all nine such cases. Fifteen patients had severe underlying comorbidities, including sepsis, respiratory failure, malignancy, or renal failure. RESULTS: The procedure was technically successful in all patients. Twenty-six patients were treated solely with NBCA, and one required microcoil embolization. Embolization was performed at the level of the arteria recta or as close as possible to the point of bleeding. Immediate hemostasis occurred in all cases. Four patients experienced repeat hemorrhage, one of whom died. The other three were treated successfully with repeat NBCA embolization. None of the surviving patients had evidence of bowel ischemia. In addition, none of the patients with severe underlying disease died during the follow-up period (range, 3 mo to 2 y). CONCLUSIONS: The present findings suggest that NBCA embolization may be a safe alternative treatment for the management of lower gastrointestinal bleeding. Further studies are warranted to confirm the findings.

Liver resection improves the survival of patients with multiple hepatocellular carcinomas.

BACKGROUND: According to current guidelines of hepatocellular carcinoma (HCC) treatment, multiple HCCs are usually not suitable for surgical resection. However, surgical resection is still possible for patients with multiple HCCs. The role of hepatic resection vs transarterial chemoembolization (TACE) for multiple HCCs should be further clarified. METHODS: We retrospectively enrolled 1065 patients with multiple HCCs. Among them, 294 received surgical resection, 367 received transarterial chemoembolization (TACE), and 404 received chemotherapy or supportive care. Three staging systems (TNM, CLIP, and BCLC) were used for comparison of stage-specific survival between different treatment modalities. RESULTS: The median survival of multiple HCC patients who received surgical resection was 37.9 months, while it was 17.3 months in TACE group, and 2.8 months in supportive group (P < .001). The 1-year, 3-year, 5-year survival rates for surgical group were 77.4%, 51.9%, and 36.6%, respectively. Kaplan-Meier survival analysis demonstrated that patients who received surgical resections had the best survival, followed by TACE and supportive care. For patients of the same stage, surgical resection yields better results than TACE. Surgery could offer better survival than TACE for patients either within or beyond Milan's criteria. CONCLUSIONS: Our results indicate that if patients have preserved liver functions, hepatic resection is helpful, even for patients with multiple HCCs.

Clinical manifestations and survival of hepatocellular carcinoma patients with peritoneal metastasis.

BACKGROUND AND AIM: Peritoneal metastasis is an uncommon manifestation of hepatocellular carcinoma (HCC). The aim of the present paper was to investigate the characteristics and survival of HCC patients with peritoneal metastases. METHODS: From January 1985 to December 2004, we retrospectively reviewed the records of 53 Taiwanese HCC patients with peritoneal metastases. RESULTS: Peritoneal metastases were detected at the time of HCC diagnosis (synchronously) in 10 patients and after the initial therapy for the primary tumors (metachronously) in 43 patients. The mean time for development of the metachronous peritoneal metastases was similar whether the primary cancer was treated with surgery (24 months) or transarterial chemoembolization (22.2 months). The single patient whose primary cancer was treated with supportive care alone developed peritoneal metastasis only 7.5 months after detection of the primary cancer. Surgical resection of the peritoneal metastases was possible in two-thirds of the 43 metachronous patients. The median survival for those who received surgery for these metastases was 12.5 months vs. 2.1 months for those without surgery (P = 0.0013). However, there was no difference in survival if patients were stratified to Child-Pugh grade. CONCLUSIONS: Peritoneal metastases of HCC are rare and can occur synchronously or metachronously. Though increased long-term survival was found in patients who had surgical removal of peritoneal metastases, the main determinant of better survival is Child-Pugh grade.

Balloon occlusion and hypothermic perfusion of the renal artery in laparoscopic partial nephectomy.

OBJECTIVES: We describe our initial experience with renal arterial catheterization for temporary balloon occlusion of renal artery and hypothermic perfusion during laparoscopic partial nephectomy and compare the preoperative and postoperative nephron function. METHODS: Fifteen patients received laparoscopic partial nephrectomy from September 2005 to December 2006. During the operations, the balloons of the arterial catheters were filled with distilled water to achieve pedicle control. Chilled Ringers lactate was continuously infused into the catheters for renal hypothermia. Postoperative Tc-99m diethylenetriamine pentaacetic acid (DTPA) renal scintigraphies were carried out to estimate differential renal function. The volumes of the renal tumors, tumor-bearing and contralateral kidneys from CT scans were measured using commercial software. Estimated creatinine clearance was calculated with Cockroft Gault formula. RESULTS: All procedures were successfully completed. Mean tumor size was 18.4 mL (range 2.14 to 59.0). Estimated mean intraoperative blood loss was 287 mL (range minimal to 1200). Mean estimated creatinine clearance per unit volume of functional renal parenchyma did not change statistically after the operation. Multiple regression analysis revealed that ischemic time was a significant variable which correlated with the value of lost total estimated creatinine clearance of the tumor bearing kidney. CONCLUSIONS: The initial experience shows that renal arterial catheterization for temporary balloon occlusion and hypothermic perfusion of the renal artery in laparoscopic partial nephectomy is safe, feasible and effective. The postoperative kidney function measured by mean estimated creatinine clearance per unit of functional renal volume was similar to the preoperative measurement.

Usefulness of 3D reconstructed computed tomography imaging for double outlet right ventricle.

BACKGROUND/PURPOSE: To evaluate the value of 3D reconstructed computed tomography (CT) imaging for patients with double outlet right ventricle (DORV). METHODS: CT images were obtained preoperatively from 17 patients who ranged in age from 5 days to 5 years. Reconstructed 3D images were created using gradient-shading surface rendering, which allowed partial subtraction of the anterior sections of the virtual heart to view the interior. Interpretations of CT, echocardiography and cine-cardioangiography were compared and verified from surgical findings, autopsy, and consensus upon review of all imaging and diagnostic tests. RESULTS: Three subaortic, seven subpulmonary, six non-committed, and one double-committed subtypes of ventricular septal defect (VSD) were observed. The 3D electron beam CT images provided good delineation of the spatial relationship inside the heart. The range of diagnostic accuracy for all VSD types in DORV was 88-100% for 3D CT, 71-94% for echocardiography, and 60-100% for cine-cardioangiography. In comparison, 3D CT offered better diagnostic accuracy for all variants of DORV. CONCLUSION: 3D constructed CT imaging is a good modality for differentiating VSD type in DORV. It allowed us to directly evaluate the inside of cardiac chambers for the right ventricular outlet, great arterial root, and determine the VSD relationships.

Management of ulcerative colitis in Taiwan: consensus guideline of the Taiwan Society of Inflammatory Bowel Disease.

Ulcerative colitis (UC) is an inflammatory bowel disease characterized by chronic mucosal inflammation of the colon, and the prevalence and incidence of UC have been steadily increasing in Taiwan. A steering committee was established by the Taiwan Society of Inflammatory Bowel Disease to formulate statements on the diagnosis and management of UC taking into account currently available evidence and the expert opinion of the committee. Accurate diagnosis of UC requires thorough clinical, endoscopic, and histological assessment and careful exclusion of differential diagnoses, particularly infectious colitis. The goals of UC therapy are to induce and maintain remission, reduce the risk of complications, and improve quality of life. As outlined in the recommended treatment algorithm, choice of treatment is dictated by severity, extent, and course of disease. Patients should be evaluated for hepatitis B virus and tuberculosis infection prior to immunosuppressive treatment, especially with steroids and biologic agents, and should be regularly monitored for reactivation of latent infection. These consensus statements are also based on current local evidence with consideration of factors, and could be serve as concise and practical guidelines for supporting clinicians in the management of UC in Taiwan.

Management of Crohn's disease in Taiwan: consensus guideline of the Taiwan Society of Inflammatory Bowel Disease.

Crohn's disease (CD) is a chronic relapsing and remitting inflammatory disease of the gastrointestinal tract. CD is rare in Taiwan and other Asian countries, but its prevalence and incidence have been steadily increasing. A steering committee was established by the Taiwan Society of Inflammatory Bowel Disease to formulate statements on the diagnosis and management of CD taking into account currently available evidence and the expert opinion of the committee. Thorough clinical, endoscopic, and histological assessments are required for accurate diagnosis of CD. Computed tomography and magnetic resonance imaging are complementary to endoscopic evaluation for disease staging and detecting complications. The goals of CD management are to induce and maintain remission, reduce the risk of complications, and improve quality of life. Corticosteroids are the mainstay for inducing re-mission. Immunomodulating and biologic therapies should be used to maintain remission. Patients should be evaluated for hepatitis B virus and tuberculosis infection prior to treatment and receive regular surveillance for cancer. These consensus statements are based on current local evidence with consideration of factors, and could be serve as concise and practical guidelines for supporting clinicians in the management of patients with CD in Taiwan.

Diffuse intraperitoneal metastasis after spontaneous rupture of hepatocellular carcinoma.

Rupture of hepatocellular carcinoma (HCC) is a fatal complication. Intraperitoneal metastasis after rupture of HCC is rare. We report a case of diffuse intraperitoneal metastases after rupture of HCC. A previously asymptomatic 32-year-old man was admitted because of massive ascites due to ruptured HCC. Poor liver reserve limited the therapeutic options. Transarterial chemoembolization was performed to stop tumor bleeding. Abdominal computed tomography demonstrated multiple large peritoneal metastases 3 months after the rupture episode. Echo-guided fine needle aspiration from the suprapubic area was performed. Cytology was positive for HCC. It is rare for HCC to develop intraperitoneal metastases in as short as 3 months.

Primary liver lymphoma in a patient with chronic hepatitis C.

Primary liver lymphoma is a very rare disease and is frequently overlooked as a possible diagnosis. We report the case of an asymptomatic middle-aged man with chronic hepatitis C who developed primary liver lymphoma (PLL). A large solitary tumor in the left lobe of the liver was incidentally detected on routine ultrasound examination. Imaging studies showed mixed iso- and hypoechogenicity with hypoechoic rim, hypodense in the pre-contrast phase and thick wall enhancement in the post-contrast phase on computed tomographic study, hypointensity on T1WI, and hyperintensity of the central portion and slightly higher intensity in the peripheral wall on T2WI. These pictures were different from focal nodular hyperplasia, hepatocellular carcinoma, cholangiocarcinoma or metastases. Atypical hepatectomy was performed and the pathology of the hepatic tumor revealed non-Hodgkin's lymphoma. Systemic staging revealed no evidence of nodal or bone marrow involvement, so PLL was diagnosed. There was no tumor recurrence more than 4 years after operation and chemotherapy. PLL should be included in the differential diagnosis of solitary hepatic tumor in patients who are hepatitis C virus-positive, and who have atypical imaging and no known malignancy or elevated tumor marker levels.

Microstructural Changes in Absence Seizure Children: A Diffusion Tensor Magnetic Resonance Imaging Study.

BACKGROUND: Absence seizures are a subtype of epileptic seizures clinically characterized by transient alterations in states of consciousness and by electroencephalography indicating diffuse spike-wave discharges (SWD). Conventional brain magnetic resonance imaging (MRI) is not routinely used to establish the diagnosis, but rather to rule out other diseases. The present study investigated tissue integrity in children with SWD epilepsy using diffusion tensor imaging (DTI). METHODS: Magnetic resonance imaging (MRI)-DTI was conducted in 18 patients with absence seizures and 10 control participants. Brain areas were evaluated using diffusion maps, and fractional anisotropy (FA), mean diffusivity (MD), parallel diffusivity (λ||), and perpendicular diffusivity (λ⊥) values were extracted and analyzed. Tractography at the regions of abnormal diffusion indices was then reconstructed in each group, and tract symmetry was evaluated by an index of asymmetry (AI). Statistical analyses were performed using nonparametric Mann-Whitney U tests, with p values < 0.05 indicating statistical significance. RESULTS: Compared to the control group, patients with SWD epilepsy had lower FA values and higher MD values at the genu of the corpus callosum. There was also a stronger negative correlation between MD and FA values at the genu of the corpus callosum in patients than in control participants. The AI for the fiber tracts through the genu of the corpus callosum in the SWD group was significantly higher than that of the control group, indicating that tract distribution was more asymmetric in patients with epilepsy. There were no significant differences between groups in diffusion indices for other brain areas. CONCLUSION: We observed microstructural changes in the genu of the corpus callosum, as well as reduced FA values, increased λ⊥ values, increased MD values, and asymmetric distribution of fiber tracts, indicating that DTI is more sensitive than conventional MRI to detect brain abnormalities in children with absence seizures.

Fetus in fetu in an adult: diagnosis by computed tomography imaging.

The term "fetus-in-fetu" refers to a monozygotic, diamniotic twin that is incorporated into the body of another sibling early in embryonic development. A fetus in fetu is a differentiated mass that, in contrast to a teratoma, has a discrete spinal column and well developed internal organs. Most cases of fetus-in-fetu are discovered as a retroperitoneal mass in the first year of life, and the condition is unusual in adulthood. We describe a case of fetus-in-fetu incidentally discovered in a 39-year-old man who complained of mild epigastralgia. Abdominal ultrasound revealed a mass at the right upper quadrant of the abdomen. Fetus-in-fetu was diagnosed by computed tomography as an incidental finding, which was not clearly related to his abdominal discomfort. The fetus-in-fetu was treated conservatively. His radiologic as well as clinical condition remained unchanged during 4 years of follow-up.

Acute pancreatitis combined with acute Budd-Chiari syndrome as the initial manifestation of small cell lung cancer.

Tumor metastasis to the pancreas is a rare but recognized cause of acute pancreatitis. Autopsy series have reported a 24-40% of pancreatic involvement in small cell lung cancer. However, only a very few cases of tumor-induced acute pancreatitis have been described. Budd-Chiari syndrome complicating lung cancer is a rarely reported condition. We report a 68-year-old woman with extensive small cell lung cancer with the unusual initial presentation of both acute pancreatitis and acute Budd-Chiari syndrome. This patient suffered from progressive epigastralgia for 3 weeks. Severe epigastralgia with radiation to back and progressive jaundice developed 2 days prior to admission. After admission, the liver enlarged rapidly and the ascites increased markedly. Chest roentgenogram showed a mass lesion over the left lower lung field. Poorly differentiated carcinoma cells were found in ascites and bone marrow. The patient died on the ninth day of hospitalization before chemotherapy was initiated. Prompt diagnosis of extensive-stage small cell lung cancer may allow early chemotherapy treatment which favorably influences recovery when the pancreatitis is mild. Although prolonged survival might have been expected had this patient recovered from pancreatitis and received chemotherapy, diagnosis was delayed due to difficulty in immunohistochemical diagnosis of the tumor and the unusual clinical presentation. The use of stains employing antibodies against neurofilament and neuron-specific enolase cell antigens is important for early diagnosis of poorly differentiated metastatic tumor cells.

Gastric outlet obstruction due to giant hyperplastic gastric polyps.

Hyperplastic gastric polyps account for the majority of benign gastric polyps. The vast majority of these lesions are small, asymptomatic, and found incidentally on radiologic or endoscopic examination. Giant hyperplastic gastric polyps are uncommon and most of them are asymptomatic. A 73-year-old man presented with a 6 cm pedunculated hyperplastic polyp that had led to progressive gastric outlet obstruction. It had a distinctive appearance on double-contrast barium studies, appearing as a conglomerate mass with smooth and multiple lobulated components and trapping of barium in the interstices between lobules. Endoscopy and computed tomography revealed similar features. Although hyperplastic gastric polyps are typically benign, total removal of this giant polyp should be undertaken in cases of symptomatic gastric outlet obstruction, followed by pathologic confirmation of benign nature.

Influence of Chronic Kidney Disease on Physical Function and Quality of Life in Patients after Coronary Artery Bypass Grafting.

AIMS: The purposes of this study were (1) to compare body composition, physical function, and quality of life (QOL) between patients after coronary artery bypass grafting (CABG) with and without chronic kidney disease (CKD) and (2) to analyze the factors associated with physical function and QOL domains in these patients. METHODS: Thirty male post-CABG patients with CKD and 30 matched controls were recruited. All subjects underwent dual-energy X-ray absorptiometry for body composition evaluation. Physical function tests included the grip strength test, 30-second chair stand test (30CST), and 6-min walk test (6MWT). Physical activity and QOL were assessed using the long form of the International Physical Activity Questionnaire and the World Health Organization Quality of Life Instrument (WHOQOL)-BREF, respectively. RESULTS: Post-CABG patients with CKD exhibited a lower arm lean mass and higher percent leg fat mass than those without CKD (p < 0.05). The patients with CKD also had lower 30CST scores, 6MWT distances, and QOL domain of social relationships scores than those without CKD after adjusting for covariates (p < 0.05). If NYHA class was considered in the model, NYHA class became the most important factor associated with 6MWT distances (ß = -0.647, p < 0.001) and the QOL domains of psychological health (ß = -0.285, p = 0.027) and environment (ß = -0.406, p = 0.001). CONCLUSION: Post-CABG patients with CKD had worse body composition, physical function, and QOL than those without CKD, and this might be associated with a worse NYHA class.

Protein C deficiency related obscure gastrointestinal bleeding treated by enteroscopy and anticoagulant therapy.

Obscure gastrointestinal bleeding is an uncommonly encountered and difficult-to-treat clinical problem in gastroenterology, but advancements in endoscopic and radiologic imaging modalities allow for greater accuracy in diagnosing obscure gastrointestinal bleeding. Ectopic varices account for less than 5% of all variceal bleeding cases, and jejunal variceal bleeding due to extrahepatic portal hypertension is rare. We present a 47-year-old man suffering from obscure gastrointestinal bleeding. Computed tomography of the abdomen revealed multiple vascular tufts around the proximal jejunum but no evidence of cirrhosis, and a visible hypodense filling defect suggestive of thrombus was visible in the superior mesenteric vein. Enteroscopy revealed several serpiginous varices in the proximal jejunum. Serologic data disclosed protein C deficiency (33.6%). The patient was successfully treated by therapeutic balloon-assisted enteroscopy and long-term anticoagulant therapy, which is normally contraindicated in patients with gastrointestinal bleeding. Diagnostic modalities for obscure gastrointestinal bleeding, such as capsule endoscopy, computed tomography enterography, magnetic resonance enterography, and enteroscopy, were also reviewed in this article.

Gelsolin decreases actin toxicity and inflammation in murine multiple sclerosis.

Gelsolin is the fourth most abundant protein in the body and its depletion in the blood has been found in multiple sclerosis (MS) patients. How gelsolin affects the MS brain has not been studied. We found that while the secreted form of gelsolin (pGSN) decreased in the blood of experimental autoimmune encephalomyelitis (EAE) mice, pGSN concentration increased in the EAE brain. Recombinant human pGSN (rhp-GSN) decreased extracellular actin and myeloperoxidase activity in the brain, resulting in reduced disease activity and less severe clinical disease, suggesting that gelsolin could be a potential therapeutic target for MS.