A case of two shunts in the endovascular treatment of type II Abernethy syndrome

Background Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population. Case presentation We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT. Conclusions This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature

Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously.Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He was admitted for complaint of syncope. He underwent a series of examinations and cardiac ultrasound showed that his pulmonary artery pressure was elevated. Further imaging revealed the absence of intrahepatic portal veins. His blood ammonia was significantly increased. All signs and symptoms pointed to an Abernethy diagnosis. He was finally diagnosed as having Abernethy type II. He was discharged after 17 days of in-hospital treatment with sildenafil (50 mg/day) and ornithine aspartate (20 g/day).Conclusion: We now report this rare case of syncope that is caused by Abernethy malformation. As a typically pediatric disease, it was not identified in this patient until adulthood due to long-term treatment for jaundice and liver cirrhosis. Furthermore, we present a review of portosystemic shunts previously reported in the literature.

Normal liver-to-heart transit time and shunt fraction after transplenic injection of 99MTC-pertechnetate in healthy cats

Portosystemic shunts (PSS) are rare vascular anomalies in cats. Transsplenic portal scintigraphy (TSPS) can aid in diagnosing PSS in cats. Although the actual performance of the scan remains the same between species, it is questionable whether the generally accepted transit time of seven seconds for small dogs can be applied to cats, thereby influencing shunt fraction (SF) calculation. In this study, normal mean transit time and SF were determined in a population of cats without PSS following two methods established in canine medicine. For both, the mean ± SD transit time was calculated as 6.75 ± 1.58 seconds and 7.40 ± 1.64 seconds respectively, without significant difference between both methods. The results confirmed the validity of the generally used transit time of seven seconds for SF calculation in cats. The average normal SF (± SD) for the cats in this study was 0.73 % (±0.74; range 0.11-2.48%).

Clinical characteristics of, prognostic factors for, and long-term outcome of dogs with multiple acquired portosystemic shunts: 72 cases (2000–2018)

OBJECTIVE To identify clinical characteristics of, prognostic factors for, and long-term outcome of dogs with multiple acquired portosystemic shunts (MAPSSs) and determine whether survival time was associated with previous portosystemic shunt attenuation. ANIMALS 72 client-owned dogs with MAPSSs. PROCEDURES Medical records of dogs in which MAPSSs had been diagnosed between January 2000 and August 2018 were reviewed for signalment, historic and diagnostic findings, management methods, and outcome. RESULTS Median survival time of dogs (n = 23) that died of causes related to MAPSSs was 580 days (range, 156 to 1,363 days). Factors significantly associated with dying of MAPSS-related versus unrelated causes included body weight, albumin concentration at the first and last recheck examinations, and cholesterol, total solids, and glucose concentrations at the last recheck examination. Dogs not receiving medical management or without signs of depressed mentation at the time of initial presentation were less likely to die of causes related to MAPSSs. Patient status (alive vs dead of causes related to MAPSSs vs dead of causes unrelated to MAPSSs vs dead of unknown causes) was not significantly associated with survival time. CONCLUSIONS AND CLINICAL RELEVANCE Survival time for dogs with MAPSSs was not shortened by previous portosystemic shunt attenuation surgery and was not different when death was versus was not related to MAPSSs. Dogs with MAPSSs that had progression of biochemical changes consistent with liver dysfunction were more likely to die of causes related to MAPSSs and were unlikely to live a normal lifespan.

Whether Gastrorenal Shunt Embolization Would Benefit the Outcomes of Post Transjugular Intrahepatic Portosystemic Shunt

Background & Aim: Whether the spontaneous portosystemic shunts in cirrhosis who require embolization during transjugular intrahepatic portosystemic shunt (TIPS) remains a therapeutic controversial. This study was retrospectively conducted to evaluate the effectiveness of the gastrorenal shunts (GRS) embolization in cirrhosis post-TIPS。Methods: 70 cirrhotic patients who underwent TIPS in a tertiary-care center were included, of which 43 patients had no GRS and 27 had GRS with embolization during TIPS placement. Then, to assess the outcomes of embolization of GRS on post-TIPS hepatic encephalopathy (HE), clinical relapse, mortality and shunt dysfunction.Results: During a median follow-up period of 497.01 days, 25 patients (35.7%) experienced HE, of 14 patients in GRS group and 11 in another (p = 0.026). Within 50 days after TIPS, 12 patients performed initial HE in GRS group while 6 in the reference group (p < 0.001). After TIPS of 150th to 200th, one in the former group and five in another experienced HE (p < 0.001). However, there was no significant difference in the 1-year cumulative risk of HE (p = 0.287). Meanwhile, during the 2-year follow-up, the patients performed lower incidence of ascites after GRS embolization with TIPS (p < 0.002). And there was no difference in rebleeding, mortality and shunt dysfunction. Conclusions: TIPS with GRS embolization appeared to be a safe and efficacious procedure in the treatment of portal hypertension with concomitant GRS. Furthermore, the procedure seemed to reduce the recurrences of ascites for a long term observation.

Microbiota transplants from feces or gut content attenuated portal hypertension and portosystemic collaterals in cirrhotic rats

Liver cirrhosis and portal hypertension is the end of chronic liver injury with hepatic, splanchnic and portosystemic collateral systems dysregulation. Liver injury is accompanied by gut dysbiosis whereas dysbiosis induces liver fibrosis, splanchnic angiogenesis and dysregulated vascular tones vice versa, making portal hypertension aggravated. It has been proved that intestinal microbiota transplantation alleviates dysbiosis. Nevertheless, the influences of microbiota transplantation on cirrhosis related portal hypertension are not so clear. Liver cirrhosis with portal hypertension was induced by bile duct ligation in rats. Sham rats were surgical controls. Rats randomly received vehicle, fecal or gut (terminal ileum) material transplantation. The results showed that microbiota transplantation from feces or gut material significantly reduced portal pressure in cirrhotic rats (P = .010, .044). Hepatic resistance, vascular contractility, fibrosis and relevant protein expressions were not significantly different among cirrhotic rats. However, microbiota transplantation ameliorated splanchnic hyperdynamic flow and vasodilatation. Mesenteric angiogenesis, defined by whole mesenteric window vascular density, decreased in both transplantation groups and phosphorylated eNOS was downregulated. Portosystemic shunts determined by splenorenal shunt flow decreased in both transplantation groups (P = .037, .032). Shunting severity assessed by microsphere distribution method showed consistent results. Compared to sham rats, cirrhotic rats lacked Lachnospiraceae. Both microbiota transplants increased Bifidobacterium. In conclusion, microbiota transplantation in cirrhotic rats reduced portal pressure, alleviated splanchnic hyperdynamic circulation and portosystemic shunts. The main beneficial effects may be focused on portosystemic collaterals-related events, such as hepatic encephalopathy and gastroesophageal variceal hemorrhage. Further clinical investigations are mandatory.

Case Report: Clinical Features of Congenital Portosystemic Shunts in the Neonatal Period

Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.

Multistage closure of a congenital extrahepatic portosystemic shunt

Background Congenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS. Case presentation A 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed. Conclusion This paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment. Level of Evidence: Level 4, Case report.