Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant.

BACKGROUND: The management of portal hypertension (PHT) in children with well compensated cirrhosis and cystic fibrosis (CF) is controversial. We present our experience with distal splenorenal shunting (DSRS) for the treatment of PHT as an alternative to liver transplantation (LT). METHODS: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9) was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). RESULTS: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ±â€¯6 vs 28 ±â€¯4, p < 0.001). All 5 DSRS patients had grade III-IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ±â€¯1.5 cm to 4.4 ±â€¯1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ±â€¯48 to 91.8 ±â€¯35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24-7.79). CONCLUSIONS: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: IV.

Prevalence and Clinical Characteristics of Spontaneous Splenorenal Shunt in Liver Cirrhosis: A Retrospective Observational Study Based on Contrast-Enhanced Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) Scans.

BACKGROUND This is a retrospective observational study evaluating the prevalence and clinical characteristics of spontaneous splenorenal shunt in liver cirrhosis. MATERIAL AND METHODS We included a total of 105 cirrhotic patients who were admitted to our hospital between June 2012 and December 2013 and underwent contrast-enhanced CT and/or MRI scans at admissions. Spontaneous splenorenal shunt was identified. Clinical and laboratory data were compared between cirrhotic patients with and without spontaneous splenorenal shunt. RESULTS The prevalence of spontaneous splenorenal shunt was 10.5% (11/105). The prevalence of hepatic encephalopathy was higher in patients with spontaneous splenorenal shunt than in those without spontaneous splenorenal shunt (18.2% vs. 4.3%, p=0.062), but the difference between them was not statistically significant. The prevalence of acute upper-gastrointestinal bleeding was lower in patients with spontaneous splenorenal shunt than in those without spontaneous splenorenal shunt (0% vs. 18.1%, p=0.205), but the difference between them was not statistically significant. Patients with spontaneous splenorenal shunt had significantly higher Child-Pugh scores (9.50±1.65 vs. 7.43±2.02, p=0.002) and MELD scores (11.26±7.29 vs. 5.67±6.83, p=0.017) than those without spontaneous splenorenal shunt. In-hospital mortality was similar between them (0% vs. 4.3%, p=1.000). CONCLUSIONS Spontaneous splenorenal shunt might be associated with worse liver function in liver cirrhosis, but not with in-hospital mortality.

High patency of proximal splenorenal shunt: A myth or reality ? - A prospective cohort study.

BACKGROUND: Proximal splenorenal shunt (PSRS) is a well-accepted surgical procedure for non-cirrhotic portal hypertension (NCPH). Though a patent shunt is important for good long term outcome, there are very few studies on patency of these shunts. We analysed shunt patency using dynamic computed tomographic (CT) portography and compared it with other modalities. METHODS: From 2004 to 2014, 50 patients with PSRS were evaluated prospectively for shunt patency using dynamic CT portography, clinical parameters and ultrasound Doppler. RESULTS: The causes of NCPH were extrahepatic portal vein obstruction (EHPVO) in 38 patients and non-cirrhotic portal fibrosis (NCPF) in 12 patients. The shunt patency rate using clinical parameters, ultrasound Doppler and dynamic CT portography were 70%, 40% and 60% respectively. Clinical parameters overestimated while ultrasound Doppler underestimated the shunt patency rate. Dynamic portography had 100% correlation with conventional angiography in the five patients when this was done. The site of shunt could be demonstrated convincingly by dynamic CT portography. The shunt patency rate decreased over time. It was 64%, 60% and 43% in <1 year, 1-5 years and >5 years respectively. Our NCPF patients had a greater shunt patency rate compared to EHPVO patients (9/12 vs. 21/38) though the difference was not significant. Only size of the splenic vein had a significant impact on the shunt patency rate on statistical analysis. CONCLUSIONS: Dynamic CT portography is useful for evaluation of shunt patency. Proximal splenorenal shunts have a high blockage rate which has hitherto not been reported.

507 Warren-Zeppa distal splenorenal shunts: a 34-year experience.

OBJECTIVE: To define the long-term characteristics, prognostic factors, and outcomes of patients undergoing selective splenorenal shunting procedures for portal hypertension-induced recurrent upper gastrointestinal bleeding. MATERIALS AND METHODS: A retrospective evaluation of a prospectively collected data set. RESULTS: From June 1971 through May 2005, 507 Warren-Zeppa shunts were performed at a single institution. Indications included: alcoholic cirrhosis, 52.6%; viral cirrhosis, 21.8%; cryptogenic cirrhosis, 8.4%; autoimmune cirrhosis, 5.8%; and other causes, 6.3%. Median survival was 81 months (5-year survival, 58.9%; 10-year survival, 34.4%; 20-year survival, 12.5%). patients with portal vein thrombosis and biliary cirrhosis demonstrated better survival than others (P = 0.03), while patients with alcoholic cirrhosis trended toward worse survival than those with nonalcoholic causes (P = 0.11). Multivariate analysis of preoperative risk factors found body hair loss (hazard ratio, 17.3; P > 0.005), preoperative encephalopathy (hazard ratio, 1.93; P > 0.003), diuretic use (hazard ratio, 1.43; P > 0.003), and age (hazard ratio, 1.02 per year of age; P > 0.051) were independent predictors of poor long-term survival. Multivariate analysis of operative factors demonstrated blood loss <500 mL was predictive of up to a 4-fold improved long-term survival (hazard ratio, 3.95; P < 0.013). Postoperative complications included: recurrent bleeding, 12%; ascites, 17.5%; and encephalopathy, 13.9%. Multivariate analysis of postoperative factors prospectively collected in 130 patients found that alcoholic recidivism (hazard ratio, 2.66; P > 0.001) was the only independent predictor of poor prognosis. CONCLUSIONS: The Warren-Zeppa shunt provides long-term survival and control of bleeding in most patients with portal hypertension. Excellent long-term survival can be obtained in properly selected patients with portal hypertension and relatively spared hepatic function.