Short summary

Large spontaneous portosystemic shunts (SPSSs) have been described in cirrhotic patients with chronic or recurrent hepatic encephalopathy (HE). In most cases, their presence is due to portal hypertension. The diagnosis is obtained by imaging techniques (mainly CT angiography), and a high clinical suspicion is required.

In patients with refractory HE despite treatment it has been shown that the occlusion of SPSS is a therapeutic target.

The aim of the study is to carry out a large international multicenter retrospective study of SPSSs in cirrhotics detected by angio-CT.  

Rationale

Large spontaneous portosystemic shunts (SPSSs) have been described in cirrhotic patients with chronic or recurrent hepatic encephalopathy (HE). In most cases, their presence is due to portal hypertension. The diagnosis is obtained by imaging techniques (mainly CT angiography), and a high clinical suspicion is required.

In patients with refractory HE despite treatment it has been shown that the occlusion of SPSS is a therapeutic target. However, the prevalence of SPSSs in cirrhotic patients with or without hepatic encephalopathy is not well established.

Riggio, et al. carried out a case-control study in 2005 including 28 patients: 14 with cirrhosis and chronic or recurrent HE (cases) and 14 cirrhotic patients without previous history of HE (controls), matched by age and liver function, using MELD score. Patients were submitted to a multidetector-row spiral abdominal CT. Large SPSSs were diagnosed in 10 patients with HE and only in 2 patients without history of HE, with statistical differences (71% vs 14%, X² 9.16, p 0.0002). This study showed how patients with large SPSSs had more risk of developing an episode of HE. A low percentage of large SPSSs in patients without previous history of HE was also described.  However, the sample size of this study was small.

Another study published in 1986 that included 28 cirrhotic patients with large SPSSs, showed hepatic encephalopathy in almost half of the sample (13 patients), while 15 patients remained free of HE⁵, despite the presence of a large collateral.

Many questions regarding SPSSs in cirrhotic patients still remain; the prevalence is unknown, the risk factors for developing HE, the role of small size shunts, etc. We propose to carry out a large international multicenter retrospective study of SPSSs in cirrhotics detected by angio-CT. 

Description of the cohort

Multicenter retrospective study. Patients will be detected by systematically reviewing all angio-CTs performed for any reason to patients with liver cirrhosis during the study period. Cirrhotic patients with an evaluable CT will be classified in 3 groups: 1) With a large SPSS, 2) With small SPPSs, and 3) Without detectable SPPSs. For each patient with a large SPSS another patient without SPSS will be selected, matched for age and sex. Clinical and radiological data from all patients from groups 1 and 2, and selected patients from group 3 will be collected. Data will be anonymous for all the duration of the study.

Study population

Inclusion criteria

• Aged 18 years or older
• Liver cirrhosis
• An evaluable angio-CT performed by any reason in the study period (from January 2010 to December 2014)

• Presence of previous surgical shunts or TIPS
• Prior liver transplant
• Neurological or psychiatric disorder that do not permit to establish the diagnosis of HE
• Presence of hepatocellular carcinoma beyond Milan criteria
• Terminal disease

Data and biological material

Basal data

• Date of birth
• Date of angio-CT
• Gender
• Etiology of cirrhosis: Alcohol/Hepatitis C/Hepatitis B/NASH/Cryptogenic/Combined/Other
• Date of diagnosis of Cirrhosis:
• Comorbidities: Diabetes mellitus/Hypertension/Cardiovascular disturbances: cardiac failure/Cerebrovascular disease: previous stroke/Other major comorbidity.

Prior history

• Hepatic encephalopathy/Type (episodic, chronic, recurrent)/Number of episodes/HE maximal grade (I to IV)/
• Portal hypertension (evaluation of the closest endoscopy to CT): Presence of varices/Type/Grade/Portal gastropathy/Bleeding/Cause/Treatment
• Ascites/Grade/Refractory-Paracentesis
• SBP
• HRenal syndrome
• HCC/date/size/stage/therapy

Basal labs (at time of CT)

• Hemoglobin/Platelets/Creatinine/Sodium/Bilirubin/Albumin/INR

Basal clinical status

• Hepatic encephalopathy/HE grade (I to IV)/
• Bleeding/Cause
• Ascites/Grade/Refractory-Paracentesis

• HRenal syndrome
• Current therapy: Diuretics/Betablockers/Lactulose or lactitol/Rifaximin or neomycin
• Clinical status (according to modified Rankin scale (mRS):
• completely autonomous
• limited autonomy (need of help by caregivers)
• institutionalized in a nursing home (date of admission at nursing home)

Imaging data (CT)

• Reason for CT:
• Type of large portal-systemic shunt (8 mm or more): Splenorenal/Mesocaval/Mesorenal/Inferior mesenteric-caval/Gastro-renal/Umbilical/Others:
• Number
• Diameter (mm):
• Additional small portosystemic shunts/Type/Size (mm)
• Presence of varices at the CT/Type
• Portal diameter/Permeability/Cavernomatosis/Splenomegaly/Ascites

Imaging data (sonography closest to CT)

• Portal flow direction/flow velocity/ portal permeability/splenomegaly

Imaging data (cerebral MR closest to CT)

• Pallidal T1 hyperintensity

Follow-up data

• Date of last follow-up
• Death/Liver Tx/Alive
• Hepatic encephalopathy/Type (episodic, chronic, recurrent)/Number of episodes/HE maximal grade (I to IV)/
• Bleeding/Cause/Treatment
• Ascites/Grade/Refractory-Paracentesis
• SBP
• HRenal syndrome
• HCC/date/size/stage/therapy

Collaborating researchers and departments

Department of Medical Gastroenterology, Odense University Hospital

• Staff specialist Annette Dam Fialla

Department of Radiology, Vejle Hospital

• Specialist registrar Claus Dam

Unit of Internal Medicine, Hepatology, University Hospital Vall D'Hebron, Barcelona

• Joan Genesca Ferrer