Abernethy syndrome is a rare congenital anomaly characterized by an intrahepatic or extrahepatic portosystemic shunt. Most patients are asymptomatic; however, due to the alteration in, or lack of, a portovenous flow, patients with Abernethy syndrome are at high risk of developing sequelae of liver failure. Once these complications develop, the only definitive treatment is transplantation. Patients with Abernethy syndrome are also at a higher risk of developing benign and malignant liver lesions, including hepatic adenomas. Here, we describe the first case of deceased donor liver transplantation as a treatment for a patient with type 1 Abernethy syndrome complicated by large, unresectable hepatic adenoma, found to have focal hepatocellular carcinoma on pathologic examination. Our male patient was found to have elevated liver enzymes at age 33, during a routine outpatient medical appointment. Despite being asymptomatic, his history of prior liver resection prompted CT imaging, which revealed two large liver lesions concerning for hepatic adenomas. When surveillance imaging showed a significant growth of the liver lesions, biopsy was pursued, which confirmed a diagnosis of hepatic adenomas. However, given the size of these lesions, resection was not a viable option for the patient. Instead, the patient underwent liver transplantation at age 41, which he tolerated well. Our case demonstrates the utility of deceased donor liver transplantation as a treatment for patients with Abernethy syndrome complicated by unresectable adenomas.

Abernethy syndrome is a rare congenital malformation stemming from a portosystemic shunt. Diagnosis proves challenging due to nonspecific clinical symptoms, with presentation varying based on age and disease severity. Consequences include hepatic, cardiovascular, renal, gastrointestinal, and neurological complications, and growth retardation. We report the case of a child presenting with perioral and digital cyanosis, observed in early childhood. Clinical examination revealed low saturation, telangiectasias, digital clubbing, and collateral venous circulation in the thorax. Imaging confirmed the diagnosis of Abernethy syndrome.

BACKGROUND: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes.
METHODS: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging.
RESULTS: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011).
CONCLUSIONS: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic.
METHODS: Level III.

Neurological symptoms that occur after treatment of portosystemic shunts, in cats, known as post-attenuation neurological signs (PANS) can be quite severe. This study seeks to analyze a better understanding of the neurological outcomes that result from reducing portosystemic shunts in felines and provide insights that could guide future clinical approaches and treatment strategies for congenital portosystemic shunts (CPSS).The research utilized the MOOSE Checklist as a guide. PubMed/MEDLINE, Web of Science ScienceDirect, Embase, Scopus, ProQuest, and Google Scholar were used. The study investigated diversity using variance, Cochran Q tests with Applied fixed effects, and random effects models. A meta-regression model identified contributors. Eggers test funnel plot and Beggs test for asymmetry addressed publication bias. 12 high-quality studies were discovered from 664 research papers. This research covered years, shunt morphology, and surgery. PANS occurred 38.9 % of the time in cats, while PAS occurred 20.2 %. The overall PANS mortality rate was 17 %, while PAS was 37.2 %. The complete ligation technique was most common in subgroup analysis. PANS occurrence ranged from 26.8 % to 56.5 % in cats with congenital extrahepatic portosystemic shunts The cause of PANS in cats is still unknown, and there is only limited evidence to justify the use of preventive antiepileptic medications such as levetiracetam. The treatment primarily aims to control neurologic symptoms, and the long-term outlook varies, with the potential for the reappearance of symptoms.

Hepatic encephalopathy is uncommon in the absence of cirrhosis. We report a 71-year-old woman who presented with altered mental status in the setting of hyperammonemia for the second time in 6 months. Magnetic resonance imaging of the abdomen revealed an uncommon portosystemic shunt involving an enlarged posterior branch of the right portal vein and an accessory right hepatic vein, with no features of cirrhosis. Appropriate management of these patients with ammonia-lowering therapy can reduce repeat episodes and improve quality of life. This case demonstrates the importance of diagnosing non-cirrhotic hepatic encephalopathy in patients with altered mental status.

Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterise paediatric HCAs and determine their course, complications, and management. Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive review of the published literature. A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) and at a mean age of 11.5 (range 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and MODY3 (maturity-onset diabetes of the young type 3). Each of these diseases leads to a well-defined HCA molecular pattern. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in children with portosystemic shunts and in β-catenin-mutated HCAs, while haemorrhages were more frequent in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat adenomatosis, as well as any underlying diseases. Progress in understanding genetic and/or malformative contributions, which appear to be significant in paediatric HCAs, have provided insights into tumour pathogenesis and will further guide patient surveillance and management.

Portal vein thrombosis is a rare complication after laparoscopic sleeve gastrectomy, a widely performed bariatric surgery procedure. Occasionally, the development of portal vein thrombosis can progress to more severe conditions, including portal hypertension and cavernomatosis, thereby presenting a complex and challenging clinical scenario. The management of such complications often requires careful consideration; however, surgical intervention in the form of a splenorenal shunt is an exceptional indication. We present the case of a 33-year-old female patient who had previously undergone laparoscopic sleeve gastrectomy in 2014 and subsequently developed portal thrombosis, followed by cavernomatosis and associated complications of portal hypertension. A proximal splenorenal shunt procedure and splenectomy were successfully performed to manage portal hypertension. The presentation of this clinical case aims to contribute to the available evidence and knowledge surrounding this rare and challenging pathology.

We describe the endovascular embolization of a 65-year-old man with chronic hepatic encephalopathy. A contrast-enhanced computed tomography demonstrated a splenorenal shunt and a recanalized paraumbilical vein as a continuous portal shunt connecting the left branch of the portal vein and the right common femoral vein. A 2-session embolization was performed for the splenorenal shunt. First, the transvenous approach was used for coil embolization of the splenorenal shunt. It was difficult to advance the catheter system to the embolization site, and it was unstable during coil placement. Second, the paraumbilical venous approach was used to place additional coils. The catheter system had good maneuverability and easily reached the embolization site. Additionally, the stable system allowed for densely packed additional coil implantations. This report demonstrated the paraumbilical venous approach\'s effectiveness in catheter maneuverability and system stability during coil embolization.

Hepatic myelopathy (HM) is a rare neurological complication in the end stage of many liver diseases and is characterized by bilateral spastic paraparesis without sensory and sphincter dysfunction. It occurs owing to metabolic disorders and central nervous system dysfunction associated with cirrhosis. Without timely and effective clinical intervention, the prognosis of these patients is devastating. Although liver transplantation (LT) is an effective treatment for HM, the prognosis of these patients remains unsatisfactory. Early recognition and diagnosis of this disease are essential for improving patient prognosis. Here, we report a case of hepatitis B virus-associated decompensated cirrhosis with HM. The patient recovered well after LT. We also summarize the clinical characteristics and post-transplant outcomes of 25 patients with HM treated by LT through 2023, including this case.

The prevalence of anatomical-based subtypes of feline congenital extrahepatic portosystemic shunts (EHPSS) has not been completely elucidated. The goal of this study was to use CT angiography to create an anatomical-based nomenclature system for feline congenital EHPSS. Additionally, subjective portal perfusion scores were generated to determine if intrinsic portal vein development was associated with different shunt conformations or patient age at the time of CT. The SVSTS and VIRIES list services were used to recruit cases. Data collected included patient DOB, gender, breed, weight, CT date, and reported diagnosis. Shunts were classified based upon (1) the shunt portal vessel(s) of origin, (2) the shunt systemic vessel(s) of insertion, and (3) any substantial portal vessels contributing to the shunt. Additionally, hepatic portal perfusion was subjectively scored between 1 (poor/none) and 5 (good/normal) based on the caliber of the intrahepatic PVs. A total of 264 CT scans were submitted from 29 institutions. Due to exclusion criteria, 33 (13%) were removed, leaving 231 CT scans to be included. Twenty-five different EHPSS anatomies were identified with five classifications accounting for 78% of all shunts (LGP [53%], LGC-post [11%], LCG [7%], LGC-pre [4%], and PC [4%]). Shunt origin involved the left gastric vein in 75% of the described classifications. Significant differences were identified among the five most common shunt types with respect to age at the time of CT scan (P = .002), breed (P < .001), and subjective portal perfusion score (P < .001). This refined anatomical classification system for feline EHPSS may enable improved understanding, treatment comparisons, and outcome prediction for cats with these anomalies.