Embolization of porto-systemic shunt as treatment for recurrent hepatic encephalopathy.

A 72-year-old female with a history of autoimmune hepatitis-related cirrhosis, osteoporosis, and hypertension was admitted with recurrent episodes of confusion and drowsiness over the course of two years. On each occasion, the patient demonstrated fluctuating confusion with drowsiness and was disorientated to time and place. A marked liver flap was present on examination with no other features of hepatic decompensation. These episodes were associated with raised serum ammonia levels, normal neuroimaging findings and no features to suggest a precipitating illness. They became more frequent, severe and longer in duration in early 2014, prompting three hospital admissions in 2 months despite being on optimal medical therapy of rifaximin, twicedaily enemas and maximally tolerated lactulose. Most recently she was admitted with a two-day history of increasing confusion, wandering around the house, and drowsiness. There were no features of jaundice, ascites, gastro-intestinal bleeding or sepsis. She was opening her bowels two to three times per day. Examination demonstrated marked confusion (abbreviated mental test score 4/10), drowsiness with a Glasgow Coma Score of 13/15 and a marked liver flap. There was no focal neurology, and the remainder of the examination was unremarkable except for 2cm splenomegaly. Initial blood tests demonstrated bilirubin: 19 μmol/L, ALT 54 IU/L, AST 50 IU/L, ALP 90 IU/L, albumin 33g/L, creatinine 63 umol/L, sodium 139 mmol/L, PT 12.8, CRP 2 mg/L and serum ammonia of 93 μmol/L. Oesophagoduodenoscopy (OGD) showed one small varix (grade 1) with no evidence of red signs or recent bleeding, while an electroencephalogram demonstrated slow waves suggestive of cerebral dysfunction, but no epileptiform activity. Despite an increase in laxatives, the patient’s confusion persisted with no appreciable change in her liver function tests, or her ammonia level. Given this patient’s otherwise good liver function, with a MELD score of 8 and unexplained, recurrent encephalopathy, a triple phase computed tomography (CT) of her liver liver was conducted to investigate for a large spontaneous porto-systemic shunt (SPSS)1 which could account for her symptoms.