Faculty Bibliography

INTRODUCTION: Cytomegalovirus (CMV) primarily affects immunocompromised patients, and has multiple manifestations in the gastrointestinal (GI) tract. The incidence of CMV-related GI disease has decreased since the advent of antiretroviral therapy (ART) for HIV. This, along with varied and nonspecific symptoms, makes it difficult to diagnose. CASE DESCRIPTION/METHODS: We report a 34-year-old man with AIDS (CD4 count 114 cells/muL) on ART presenting with five days of fevers. He was admitted to the ICU for sepsis and stabilized. His course was complicated by pulmonary embolism, recurrent fevers, and development of biopsy proven pyoderma gangrenosum. He also reported odynophagia, and was empirically treated for candida esophagitis with fluconazole. On hospital day 37, he developed massive hematochezia. An urgent esophagogastroduodenoscopy (EGD) did not reveal the source of bleeding, but showed a 5-cm linear esophageal scar, which appeared to be a healing tear. Sigmoidoscopy revealed a solitary rectal ulcer with a visible vessel, which was clipped for hemostasis. His bleeding resolved. The patient's odynophagia persisted, significantly limiting his ability to tolerate oral intake. Repeat EGD revealed a large, cratered, non-bleeding esophageal ulcer which was biopsied. The same day, the patient developed hematochezia; repeat sigmoidoscopy showed a deeply cratered rectal ulcer with a visible vessel successfully treated with hemostatic clips. Esophageal biopsies returned positive for CMV; valganciclovir was initiated. His odynophagia improved after starting treatment and he had no further GI bleeding. Given the clinical response, the rectal ulcers were presumed to be due to CMV, making this a case of extensive CMV-related GI disease. DISCUSSION: This is a case of extensive CMV-related GI disease which eluded diagnosis during a prolonged and complicated hospitalization. CMV-related GI disease has varied presentations, most commonly affecting the esophagus, but has also been noted to manifest with gastritis, duodenitis, pancreatitis, and colitis. The most common endoscopic findings for esophageal involvement are discrete, shallow, punched-out ulcers surrounded by normal appearing mucosa. Clinical manifestations of colonic CMV include abdominal pain and diarrhea, while discrete ulceration causing bleeding is less commonly reported. This case highlights the importance of a high index of suspicion for CMV in immunocompromised patients with multiple, unexplained GI symptoms to avoid a delay in diagnosis and management. (Figure Presented)

Double aortic arch is a rare congenital anomaly usually recognized in infancy or childhood. We present an unusual case of massive gastrointestinal hemorrhage resulting from aortoesophageal fistula complicating previously unrecognized double aortic arch in an adult. A 52 year-old man with a prior history of mild intermittent asthma was admitted to the hospital with a severe asthma exacerbation requiring endotracheal intubation. His course was complicated by prolonged respiratory failure and lower extremity deep venous thrombosis. He received enteral feeding via nasogastric tube throughout the period of mechanical ventilation. Several days after initiation of anticoagulation with enoxaparin, he developed melena, hypotension, and acute blood loss anemia. Urgent upper endoscopy revealed an actively bleeding ulcer in the mid esophagus, thought to have been caused by nasogastric tube trauma. Endoscopic hemostasis was achieved with epinephrine injection and clip placement. The patient recovered from this episode and anticoagulation was resumed after 72 hours. Four days later, the patient developed large volume hematemesis and hemodynamic shock requiring massive transfusion. Upper endoscopy revealed active hemorrhage at the same site in the mid esophagus, and hemostasis was again achieved with clip placement. Due to repeat massive esophageal bleeding of uncertain etiology, the patient underwent computed tomography of the chest, which revealed a right-dominant double aortic arch and aortoesophageal fistula. He underwent emergent surgery with ligation of the left-sided arch and repair of the fistula. He recovered from surgery and bleeding has not recurred. Double aortic arch (DAA), or vascular ring, is a rare developmental anomaly which usually presents in infancy or childhood. Aortoesophageal fistula due to a tight vascular ring is exceedingly rare and usually fatal. It has been reported in a handful of cases to have been precipitated by the use of a nasogastric tube. To our knowledge, this is the first case of aortoesophageal complicating DAA in a patient over 40 years old. Due to the high risk of massive hemorrhage and circulatory collapse, this case illustrates that vascular anomalies should be considered in adults with presenting with significant nonvariceal esophageal hemorrhage, prompting early consideration of cross sectional imaging. (Figure Presented)

PURPOSE: Patient education is critical in ensuring patient compliance and good health outcomes. Fellows must be able to effectively communicate with their patients, delivering enough information for the patient to understand their medical problem and maximize patient compliance. We created an objective structured clinical examination (OSCE) with four liver disease cases to assess fellows' knowledge and ability to inform standardized patients about their clinical condition. METHODS: We developed four cases highlighting different aspects of liver disease and created a four station OSCE: hepatitis B, acute hepatitis C, new diagnosis of cirrhosis, and an end-stage cirrhotic non transplant candidate. The standardized patient (SP) with hepatitis B was minimizing the fact that she could not read English. The acute hepatitis C SP was a nursing student who is afraid that having hepatitis C might jeopardize her career. The SP with the new diagnosis of alcoholic cirrhosis needed to stop drinking, and the end-stage liver disease patient had to grapple with his advanced directives. Twelve fellows from four GI training programs participated. Our focus was to assess the fellows' knowledge about liver diseases and the ACGME competencies of health literacy, shared decision making, advanced directives and goals of care. The goal for the fellows was to communicate effectively with the SPs, and acknowledge that each patient had an emotionally charged issue to overcome. The SPs used a checklist to rate fellow's performance. Faculty and the SPs observed the cases and provided feedback. The fellows were surveyed on their performance regarding the case. RESULTS: The majority of fellows were able to successfully summarize findings and discuss a plan with the patient in the new diagnosis of cirrhosis (76.92%) and hepatitis C case (100%), but were less successful in the hepatitis B (30.77%) and end-of-life case (41.67%). Overall, a small percentage of fellows reflected that they did a good job (22-33%), except at the end-of-life case (67%). The fellows' greatest challenge was trying to cover a lot of information in a single outpatient visit. CONCLUSION: Caring for patients with liver diseases can be complex and time consuming. The patients and fellows' observations were discordant in several areas: for example. the fellows believed they excelled in the end-of-life case, but the SP thought only a small percentage of fellows were able to successfully summarize and discuss the plan. This discrepancy and others highlight important areas of focus in training programs. OSCEs are important to help the fellows facilitate striking the right balance of information delivery and empathy, and this will lead to better patient education, compliance, rapport, and satisfaction.

BACKGROUND:To determine clinical-pathologic variables in patients with a new diagnosis of hepatocellular carcinoma (HCC) and underlying hepatitis B vs. C infection. METHODS:Patients presenting to a single urban hospital with a new diagnosis of HCC were entered into a clinical database. Variables including number and size of tumors, presence of metastases, serum alpha-Fetoprotein, hepatitis serologies, severity of hepatic dysfunction, and presence of cirrhosis were evaluated in 127 patients. RESULTS:Patients with hepatitis B (HBV) were more likely to develop HCC at a younger age than patients with hepatitis C (HCV) (HBV-26% under age 40, HCV-0% under age 40; p < 0.001), with greater serum alpha-Fetoprotein production (median level: HBV-1000 ng/ml vs. HCV-37 ng/ml; p = 0.002), with larger tumors (HBV-78% >5 cm, HCV-28% >5 cm; p < 0.001), in the absence of cirrhosis (HBV-40%, HCV-0%; p < 0.001), and a decreased eligibility for curative treatment (HBV-14%, HCV-34%; p < 0.05). Conversely, patients with HCV were more likely to develop HCC in association with multiple co-morbidities, cirrhosis, and older age. CONCLUSIONS:Significant clinical-pathologic differences exist among HCC patients with underlying HBV vs. HCV. These differences impact eligibility for potentially-curative therapy and prognosis.

OBJECTIVE: The aim of this study was to determine the impact of hepatocelluar carcinoma (HCC) screening in chronic hepatitis B patients who did not meet the current screening recommendations. METHODS: Patients who were admitted to Bellevue Hospital Center with HCC were assessed for risk factors, cirrhosis and tumor-specific factors. Eligibility for liver transplantation or resection with favorable outcome was determined by applying Milan criteria. RESULTS: In all 93 patients were diagnosed with hepatitis B virus (HBV)-associated HCC, 18 of whom were under 40 years. Cirrhosis was infrequently associated with HCC in this group, with most cancers occurring in non-cirrhotic patients (12/18, 66.7%). No patient developed HCC outside the American Association for the Study of Liver Diseases (AASLD) cancer screening recommendations (young age, non-cirrhotic) were eligible for liver transplantation or resection with favorable outcomes (within Milan criteria). However, HCC patients who were diagnosed within AASLD screening recommendations did meet Milan criteria in 17.3% (14/81) patients. CONCLUSIONS: Current guidelines for HCC screening in patients with HBV may lead to a delay in diagnosis in non-cirrhotic patients under 40 years. Consideration should be given to modifying current recommendations to advocate entering HBV patients into a cancer-screening program at young age.

Background. The hepatitis C virus is a major cause of hepatocellular carcinoma. Extrahepatic manifestations of hepatitis C include mixed cryoglobulinemia which can result in ischemic damage to multiple organs. The management of these sequelae in posthepatectomy patients is unclear. Case Report. A 49-year-old male with hepatitis C was found to have a 4 cm hepatocellular carcinoma on surveillance imaging. He underwent portal vein embolization followed by hepatectomy. His postoperative course was complicated by the development of splenic infarcts, small bowel ischemia, skin lesions, and liver damage. Findings of elevated cryocrit and elevated rheumatoid factor suggested the diagnosis of cryoglobulin-related vasculitis. The patient improved on supportive care. Conclusion. Cryoglobulinemia is associated with hepatitis C and may complicate the care of this patient population. The treatment of cryoglobulinemia posthepatectomy patients is complicated by concerns over how medications may affect the regenerating liver. Steroids should be used with caution in this setting. Summary. Brief report of hepatectomy complicated by vasculitis in the context of hepatocellular carcinoma secondary to hepatitis C addresses the management of mixed cryoglobulinemia in post-hepatectomy patients