Introduction: Epiploic appendagitis (EA) is a self-limiting condition that typically presents with sudden onset of lower abdominal pain which can mimic common causes of an acute abdomen. Situs inversus totalis (SIT) is an extremely rare embryologic laterality defect that results in an internal organ arrangement that is a mirror image of normal anatomy. A high index of suspicion and special attention to anatomical variants is required to correctly diagnose less common gastrointestinal diseases in patients with disorders of laterality. Case description/methods: A 24-year-old obese male with a history of SIT presented with right upper quadrant (RUQ) abdominal pain. The pain was sharp, severe, and aggravated by inspiration. There was no bright red blood per rectum, melena, dysuria, association with eating, or radiation to the back. Physical exam was notable for RUQ tenderness and a negative Murphy’s sign. Labs were unremarkable. The patient was initially discharged but returned two days later prompting abdominal imaging. CT abdomen and pelvis with IV contrast confirmed SIT and revealed a 3.6 cm area of central fat density with surrounding inflammatory change along the antimesenteric border of the descending colon in the RUQ compatible with EA. The patient was discharged with plans for supportive care. Discussion: Epiploic appendages are small physiologic peritoneal fat pouches attached to the antimesenteric surface of the large intestine by vascular stalks. Torsion or venous thrombosis of epiploic appendages results in EA, a disease process that is diagnosed by the pathognomonic CT scan finding of a 2-4 cm oval shaped fat density lesion surrounded by inflammatory changes. The sigmoid colon is the primary site of epiploic appendages, and, as such, pain from EA is usually located in the lower abdominal quadrants where it can mimic diverticulitis and appendicitis. SIT is a rare embryonic defect with an estimated prevalence of 0.3 cases per 10,000 people. We present what is, to our knowledge, the first reported case of EA in a patient with SIT. Our patient was unique to have both an altered anatomy and an uncommon location of EA in the descending colon which led to a rare cause of RUQ abdominal pain. EA is difficult enough to diagnose due to a lack of classic clinical features. Physicians must be aware of this disease process and be particularly mindful of the anatomical variations in patients with SIT to avoid unnecessary surgical intervention for a self-limiting condition.Figure 1.: Colonic ulceration secondary to CMV colitis.
Background and Aim Infection is associated with substantial morbidity and mortality in cirrhosis, but presumably, not all infections carry the same risk of mortality. We compared outcomes of different sites of infection in a nationally representative sample of inpatients with cirrhosis. Methods We queried the Nationwide Readmissions Database for patients with cirrhosis from 2011 to 2014. Cirrhosis and infection diagnoses were identified by previously used algorithms of ICD-9 codes. The following infections were compared: urinary tract infection (UTI), pneumonia, cellulitis, spontaneous bacterial peritonitis (SBP), and Clostridium difficile infection (CDI). The primary outcome was inpatient mortality. Secondary outcomes included sepsis, any organ failure, multiple organ failures, and 30-day readmission. Outcomes were analyzed using logistic regression and included a priori covariates. Results A total of 1 798 830 weighted index admissions were identified. Infection was present in 29.2% overall-including UTI (13.7%), pneumonia (8.9%), cellulitis (5.2%), CDI (2.8%), and SBP (2.0%). Mortality was significantly higher in pneumonia (19.6%), SBP (18.6%), and CDI (17.4%) compared with cellulitis (7.6%) and UTI (11.8%). Sepsis, any, and multiple organ failures were most commonly seen in pneumonia, SBP, and CDI. Multivariable analysis demonstrated that pneumonia had the highest associated mortality (odds ratio [OR] 2.73, confidence interval [CI] 2.68-2.80) and multiple organ failures (OR 3.59, CI 3.50-3.68). Significantly increased 30-day readmission was seen only with SBP (24.9%). Conclusions Outcomes of inpatients with cirrhosis vary significantly depending on the type of infection. The severity and epidemiology of infection in cirrhosis appears to be shifting with pneumonia, not SBP, having the highest prevalence of multiple organ failures and inpatient mortality.
Introduction: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytic neoplasm. ECD can present with widely varied clinical, radiographic, and histopathologic features; however, liver involvement of disease is considered uncommon. Increased awareness of the rare hepatic manifestations of this disease is needed to improve diagnosis. Case Description/Methods: A 72-year-old man with a JAK-2 positive myeloproliferative neoplasm presented with large volume ascites. Analysis of peritoneal fluid revealed a serum albumin to ascites gradient of > 1.1 and total protein < 2.0 consistent with ascites from portal hypertension. CT with contrast revealed a large, ill-defined soft tissue infiltrate throughout the retroperitoneum encasing bilateral adrenals, kidneys, and aorta as well as questionable micronodular contour of the liver. PET CT showed no significant FDG avidity in the liver. Core needle biopsy of the perinephric mass revealed fibrosis and histiocytic infiltrate. Genetic profile confirmed the diagnosis of ECD with the presence of a BRAF mutation. Liver biopsy for abnormal liver enzymes, one year prior, showed portal infiltration by neutrophils and lymphocytes and epithelioid granulomas. There was minimal steatosis and no cirrhosis of the liver parenchyma. In retrospect, these changes are thought to be due to underlying ECD. Unfortunately, by the time of diagnosis the patient was not a candidate for advanced therapies because of poor performance status and was discharged to inpatient hospice. Discussion: ECD is extremely difficult to diagnosis due to its diverse presenting features and disease spectrum that ranges from incidentally imaged lesions to critical illness from multiple organ dysfunction. Classic histopathologic findings include foamy histiocytes and surrounding fibrosis but there are often atypical findings. Immunohistochemistry and molecular testing should therefore be performed to confirm diagnosis. Liver involvement of disease is rare and often cited as the organ least likely to be affected. We present, to our knowledge, only the second case of ECD leading to portal hypertensive ascites and the first to fail to demonstrate liver involvement on imaging. Delayed diagnosis of ECD is common and can lead to prolonged morbidity and, in this case, death. Improved awareness of ECD, its hepatic manifestations, and histopathological features is needed to raise the index of suspicion for this disease and lead to more prompt identification and treatment.
Introduction: To provide a risk-benefit analysis for donating Hepatitis C virus (HCV) positive kidneys to HCV-negative recipients in an attempt to increase the donor pool and reduce the time to transplant.Figure 1Methods: We reviewed current literature looking at (i) the efficacy of treatment for HCV post-transplant with direct-acting antiviral agents (DAAs) and (ii) post-transplant complications associated with HCVpositive donor kidneys. Waitlist times and donor pool were compared analyzed. We also weighed the reduction of time on the waitlist against post-transplant graft survival to create a model to predict the potential benefit of transplanting HCV-positive kidneys. Results: There were 317 deceased kidney donors in 2015 with 8,261 recipient candidates on the waitlist for kidney transplantation. The median waiting time was approximately 4.57 years based on the 2009 data. Our model concludes that HCV-negative patients on the waitlist for kidney transplantation can expect a 12.58% greater survival rate 5 years post-transplant if a HCV-positive donor kidney is accepted compared to an HCV-negative donor kidney. Also based on the prevalence data depicting 3.45% of HCV in potential organ donors and 317 deceased kidney donors in NY in 2015, the use of the additional 6 HCV(+) donor organs wasted each year will result in a modest decrease in time on waitlist of approximately 1 month for all HD patients awaiting deceased organ transplant Conclusion: Treatment of HCV with DAAs is so recent that there are currently no data demonstrating post-transplant survival rates of HCV-negative patients receiving HCV-positive kidneys. However, multiple studies have demonstrated tremendous efficacy and safety profiles associated with post-transplant treatment of HCV with DAAs. Also, the waitlist times for organ transplant are highly center specific and at certain centers the recipients could see a reduction in wait time of nearly one year. Further research is certainly needed to understand the interactions of DAAs with immunosuppressants and its effect on graft survival and glomerulonephritis.