A 38-year-old woman was admitted to our university hospital with loss of muscle strength. She was diagnosed with dermatomyositis and underwent contrast-enhanced computed tomography of the entire body to check for malignant tumors. Computed tomography revealed multiple enhanced hepatic nodules and an extrahepatic portosystemic shunt. Although a needle biopsy of the nodule could not diagnose definitive hepatocellular carcinoma, some nodules increased in size after three months. Because of the inconclusive results of the second biopsy, we performed shunt embolization using a vascular plug. After another three months, the hepatic nodules shrank markedly, as expected.

Spontaneous portosystemic shunts (SPSS) are a common cause of recurrent hepatic encephalopathy (HE). Shunt occlusion is an effective and safe procedure when performed in patients with cirrhosis and preserved liver function. We aimed to describe our experience with SPSS embolization after liver transplantation (LT).
We identified five patients who underwent SPSS embolization after LT. Clinical, biochemical and technical procedure data were collected.
At presentation, all patients had developed graft cirrhosis and HE after LT. Median Model for End-stage Liver Disease (MELD) at embolization was 9 (range 7-12), median Child-Pugh was 8 (range 7-9). Splenorenal and mesocaval shunt were the most frequent types of SPSS found. Three patients have been completely free of HE. Of the two patients who had HE recurrence after embolization, one patient had two episodes of HE which was controlled well with medications. The other patient required three embolizations because of recurrent HE. Median follow-up was 4.4 years (range 1.0-5.0) and MELD score at last follow up was 13 (range 10-18) and median Child-Pugh score B, 7 points (range 5-12).
SPSS can be considered as a cause of HE after LT. SPSS embolization is feasible and safe in LT recipients.

Shunt obstruction is a common cause of shunt failure in the treatment of hydrocephalus. Valve occlusion is traditionally believed to originate from elevated CSF protein or cellular components, although detailed evidence is scarce and contradictory. Therefore, this study aimed to examine CSF protein and cell count as risk factors for valve obstruction.
We retrospectively examined 274 patients who underwent shunt placement for hydrocephalus between 2009 and 2018 and had at least 1 year follow-up. Age, aetiology of hydrocephalus, valve type, occurrence of revision, reason for revision and CSF protein and cell count at the time of shunt insertion and revision surgery were analysed.
Thirty-two of 274 patients (11.7%) required revision surgery due to valve occlusion. Mean time to revision was 143 days. CSF white blood cell (WBC) count but not protein was associated with valve occlusion overall. Of all obstructed valve patients, 25% showed CSF protein level within the normal range, whereas 13.6% of the patients overall showed greatly elevated CSF protein level without evidence of valve obstruction. Persistently elevated CSF protein level at the time of shunt revision was significantly associated with valve obstruction within 90 days of initial insertion (early occlusion). Children with congenital malformations and post-haemorrhagic patients were significantly overrepresented in the occlusion group, particularly in the early occlusion group.
Pathological CSF values such as WBC count and persistently elevated protein level serves as a risk factor for early valve obstruction. Late obstruction occurs independent of normal CSF values. Infants are particularly prone to early and late valve obstructions. CSF protein level at shunt insertion is not predictive of valve occlusion.

Portosystemic shunts (PSS) are associated with recurrent or persistent hepatic encephalopathy (HE), severe portal hypertensive (PHT) complications, and poor survival in cirrhosis patients. Shunt embolization improves HE in patients with recurrent or persistent HE. The role of early shunt embolization (ESE) in comparison with no and late SE (LSE) in cirrhosis patients with PSS and associated clinical outcomes are not studied.
ESE was defined as occlusion of PSS in patients with the first episode of spontaneous HE, while LSE was that when performed in patients with recurrent/persistent PSS-related HE. We retrospectively analyzed (November 2016 to March 2019) clinical outcomes, liver disease severity, and survival between patients undergoing ESE (n = 22) vs. LSE (n = 23) and compared ESE with matched historical controls (n = 22) not undergoing shunt embolization, followed-up for 18 months.
Males predominated, and the lienorenal type of shunt was the most frequent. Significantly larger and multiple shunts were noted in the LSE group. Arterial ammonia, total bilirubin, and Child-Pugh scores were significantly higher at baseline in the LSE group. Post-procedure length of stay in the intensive unit (mean 0.6 vs. 2.1 days; p = 0.04), infections (31.8% vs. 66.7% beyond 100 days; p = 0.02), recurrence of HE in first 9 months (4.5% vs. 28.6%; p = 0.03), and liver- and PHT-related clinical events beyond 10 months were significantly higher in LSE compared with those in the ESE group respectively. HE beyond 10 months was comparable between both the groups. 18.2% died in ESE while 60.87% died in the LSE group (p = 0.002). Compared with patients on only standard medical care, the occurrence of ascites, variceal bleeding, recurrence of HE, and portal vein thrombosis were significantly lower in those undergoing ESE, even though differences in survival were not significant.
Our study demonstrates the benefits of ESE of large PSS in patients with cirrhosis, probably by improving survival through a reduction in liver and PHT events that warrant validation through prospective randomized controlled multicenter trials.

Portosystemic shunt (PS) syndrome encompasses a spectrum of disease manifestations ranging from asymptomatic portal hypertension to recurrent and refractory hepatic encephalopathy, ultimately culminating in progressive hepatic failure in patients of cirrhosis and associated large PSs. PSs commonly seen in cirrhosis include splenorenal, gastrorenal, and dilated paraumbilical veins, all of which can present with recurrent or refractory hepatic encephalopathy. In this exhaustive review, we describe the anatomy of PSs, elucidate new theories on their pathophysiology, discuss the clinical implications of PSs in cirrhosis, provide details on different techniques (classical and novel) of shunt embolization, and explore all the pertinent current literature on shunt embolization for refractory and recurrent hepatic encephalopathy, all of which are enumerated with extensive images and illustrations.

We report a 74-year-old male patient with compensated cirrhosis after hepatic C virus eradication. After the patient underwent hepatectomy for hepatocellular carcinoma, multiple lung and lymph node metastases were detected by computed tomography. Computed tomography also revealed a portosystemic shunt from the superior mesenteric vein to the right testicular vein. He was administered lenvatinib (12 mg). Five days after the initiation of lenvatinib, he developed grade 3 hepatic encephalopathy, and his ammonia level increased. Lenvatinib was stopped, with improvement of the encephalopathy and decrease in ammonia level. When lenvatinib was restarted, grade 2 encephalopathy recurred which then improved upon stopping the drug. We thought that the encephalopathy was due to the portosystemic shunt, and occlusion of the shunt was performed. The day after shunt occlusion, lenvatinib (8 mg) was restarted, and the lenvatinib dose was increased to 12 mg at 2 days after shunt occlusion. Subsequently, the ammonia level remained stable and the patient remained alert and conscious. Lenvatinib was continued until the time of this report (40 days after shunt occlusion), and after 1 month of lenvatinib therapy, the computed tomography verified absence of the portosystemic shunt and stable disease of hepatocellular carcinoma.