Faculty Bibliography

Liposarcomas of the spermatic cord, a rare cause of an inguinal mass, may closely mimic inguinal hernias on clinical examination. However, these tumors require a different surgical approach and treatment plan; therefore, intraoperative diagnosis might complicate patient management. We report the case of a 63-year-old man who presented with a mobile mass in the inguinal canal consistent with an inguinal hernia. The patient was subsequently diagnosed with a liposarcoma of the spermatic cord and successfully treated with extensive local resection, including radical orchiectomy and en bloc resection of the mass and associated cord structures. No adjuvant therapy was deemed necessary, and the patient remained asymptomatic and disease-free 10 years after surgery. The details of this case are presented, along with a review and discussion of the currently available data regarding the diagnosis and management of this challenging condition.

OBJECTIVE: To examine our pilot to transplant selected patients with acute alcoholic hepatitis, initiated in October 2012. BACKGROUND: Six months of alcohol abstinence is typically required before liver transplant. A Franco-Belgian protocol showed that early transplant in severe alcoholic hepatitis could improve survival with low incidence of alcohol relapse. Application of this controversial indication is growing despite unclear generalizability. METHODS: Data was collected on all patients with alcohol-related liver disease since initiation of the pilot through June 2015. Patients were stratified into two groups: severe alcoholic hepatitis as first liver decompensation (Group 1), alcoholic cirrhosis with >/=6 months abstinence (Group 2). Alcohol relapse was defined as any evidence of alcohol consumption after transplant, which was assessed for harmful patterns of binge or frequent drinking. RESULTS: Forty-three patients underwent liver transplant, including 17 patients in Group 1. Six-month survival was 100% versus 89% for Groups 1 and 2, respectively (P = 0.27). Alcohol relapse was similar in Group 1 versus Group 2: 23.5% versus 29.2% (P > 0.99). Harmful drinking was higher in Group 1 versus Group 2, despite lack of statistical significance: 23.5% versus 11.5% (P = 0.42). CONCLUSIONS: In this pilot with carefully selected patients, early liver transplant provided excellent short-term survival, and similar rates of alcohol relapse compared with patients with 6 months of abstinence. Harmful patterns of relapse remain challenging in this population, highlighting the need for validated models to predict alcohol relapse, and need for extreme caution in selecting patients for this exceptional indication. Larger prospective studies and longer follow up are necessary.

BACKGROUND/PURPOSE: Cardiac morbidities can occur during the peri- and post-liver transplant (LT) period, affecting the long-term survival. The purpose of this study was to identify the potential factors that predict a coronary event post-transplantation. METHODS: Medical records of patients who underwent liver transplantation at Johns Hopkins Hospital between 2009 and 2013 were retrospectively reviewed. We looked at pre-liver transplant cardiac risk factors and the diagnostic tests utilized for coronary artery disease screening. Patients with and without post-liver transplant coronary events were compared. RESULTS: There were a total of 146 patients with a mean age at LT of 55.3 years. The prevalence of hypertension, tobacco use and diabetes within the patient population was 61.6 % (n = 90), 39 % (n = 57) and 37.6 % (n = 55), respectively. There were 29 deaths and 30 coronary events over a median follow-up period of 1.75 years. Age at the time of liver transplant was predictive of coronary event (OR 1.11, CI 1.01-1.20). The 1-year survival in patients with a coronary event was 47 versus 94 % in patients without a coronary event. The combined use of a dobutamine stress echocardiogram and coronary artery calcium score predicted a coronary event with a sensitivity of 62.5 % and specificity of 66.7 %. CONCLUSION: In conclusion, LT recipients with cardiac events had limited survival as compared to the cohort without coronary events. Identification of such patients with noninvasive screening may provide a practical alternative to an invasive cardiac workup. Further improvement in screening strategies may minimize the liver transplant cardiac morbidity.

BACKGROUND: Recent evidence suggests that living kidney donors are at an increased risk of end-stage renal disease. However, predicting which donors will have renal dysfunction remains challenging, particularly among those with no clinical evidence of disease at the time of donation. Although renal biopsies are not routinely performed as part of the donor evaluation process, they may yield valuable information that improves the ability to predict renal function in donors. METHODS: We used implantation protocol biopsies to evaluate the association between histological abnormalities in the donated kidney and postdonation renal function (estimated glomerular filtration rate, eGFR) of the remaining kidney in living kidney donors. Longitudinal analysis using mixed-effects linear regression was used to account for multiple eGFR measures per donor. RESULTS: Among 310 donors between 1997 and 2012, median (IQR) follow-up was 6.2 (2.5-8.7; maximum 14.0) years. In this cohort, the overall prevalence of histological abnormalities was 65.8% (19.7% abnormal glomerulosclerosis, 23.9% abnormal interstitial fibrosis and tubular atrophy (IFTA), 4.8% abnormal mesangial matrix increase, 32.0% abnormal arteriolar hyalinosis, and 32.9% abnormal vascular intimal thickening). IFTA was associated with a 5-mL/min/1.73 m decrease of postdonation eGFR after adjusting for donor age at donation, sex, race, preoperative systolic blood pressure, preoperative eGFR, and time since donation (P < 0.01). CONCLUSIONS: In this single-center study, among healthy individuals cleared for living donation, IFTA was associated with decreased postdonation eGFR, whereas no other subclinical histological abnormalities provided additional information.

Portopulmonary hypertension is one of the main pulmonary conditions affecting patients with liver disease and/or portal hypertension. Other conditions include hepatopulmonary syndrome and hepatic hydrothorax. Portopulmonary hypertension is caused by pulmonary vasoconstriction and increased pulmonary vascular resistance. It develops as a result of portal hypertension with or without liver disease and is associated with a higher morbidity and mortality. However, portopulmonary hypertension is usually asymptomatic; the most common symptoms are dyspnea, fatigue, and peripheral edema. All liver transplant candidates should be screened for potential portopulmonary hypertension because its coexistence can affect survival rates after transplant. All patients with cirrhosis who present with dyspnea should also be screened. Transthoracic echocardiography is a noni nvasive, useful method for screening, but right heart-sided catheterization remains the criterion standard for diagnosis. Portopulmonary hypertension carries a poor prognosis without liver transplant, and its severe form is considered to be a contraindication for liver transplant. Treating patients with pulmonary arterial hypertension-specific therapies before liver transplant for moderate and severe portopulmonary hypertension appears to be beneficial.

A severe and common pulmonary vascular complication of liver disease is hepatopulmonary syndrome (HPS). It is a triad of liver dysfunction and/or portal hypertension, intrapulmonary vascular dilatations, and increased alveolar-arterial oxygen gradient. Prevalence varies according to various study groups from 4%-47%. While the most common presenting symptom of HPS is dyspnea, it is usually asymptomatic, and thus all liver transplant candidates should be screened for its presence. Pulse oximetry is a useful screening method, but arterial blood gas examination is the gold standard. If there is an abnormal P (A-a)O2 gradient, microbubble transthoracic echocardiography should be done for diagnosis. Outcome is unpredictable, and there is currently no effective medical therapy. The only effective therapy is considered to be liver transplantation. Complete resolution of HPS after liver transplantation is seen within a year in most HPS patients.

Hepatocellular carcinoma (HCC) is the most common primary liver cancer. One of the most important risk factors of HCC is cirrhosis. The optimal treatment of HCC is liver transplantation, since it treats both the underlying cirrhosis and the cancer. Patients that have risk factors should be included in surveillance programs since HCC can be cured only during the early stages. Surveillance can be performed by ultrasonography (US), which is an inexpensive, non-invasive, and widely available technique, but it is considered to have a low sensitivity. If a suspicious lesion is detected on US exam, computerized tomography (CT) or magnetic resonance imaging (MRI) can be used to further evaluate this lesion. MRI is considered to be superior to CT because it has greater contrast resolution and tissue characterization. In this article, we present a review of MRI for HCC in liver transplantation (LT) with a focus on characteristic MR features of this tumor and current guidelines.