Figure - available from: Virchows Archiv
This content is subject to copyright. Terms and conditions apply.
Patient 10. Portal tract including an interlobular bile duct and arterial branch of matching size but no portal vein. Narrow slit-like spaces could be of portal derivation. Please note also increase number of arterial profiles and the absence of vacuolate nuclei in periportal hepatocytes. H&E. Magnification × 400
Source publication
Congenital portosystemic shunt (CPSS) is a congenital anomaly resulting in partial or complete diversion of the portal blood into the systemic circulation. The literature on the histological changes in livers of patients with CPSS is limited. Liver histology of 22 consecutive patients managed in our institution between 2001 and 2016 was reviewed. T...
Citations
... Non-cirrhotic portal hypertension has been reported in a broad spectrum of genetic and congenital disorders including patients with Turner syndrome and Abernathy syndrome [52]. ...
Purpose of Review
The development of portal hypertension is typically a consequence of liver cirrhosis due mainly to primary liver disorders, whereas non-cirrhotic portal hypertension (NCPH) can be a complication of systemic, primarily extrahepatic diseases. Our purpose was to review the various systemic disorders leading to portal hypertension and provide a pathway for diagnosis and management.
Recent Findings
Non-cirrhotic portal hypertension is a heterogeneous group of liver disorders primarily of vascular origin that may manifest as portal hypertension. The diagnosis of NCPH in the setting of systemic diseases is challenging and a liver biopsy may be required to confirm the diagnosis. Etiologies include those of vascular origin, autoimmune disorders, drug exposures, and infections.
Summary
Complications of portal hypertension in the setting of systemic diseases are similar to patients having cirrhosis and should be addressed similarly while addressing the underlying systemic disorder if possible
... They are believed to occur because of embryogenetic vascular alterations during fetal life. 1 Patients can be asymptomatic or have variable clinical presentation. 2 On the basis of anatomy, Abernethy malformation can be divided into two types: type I where superior mesenteric vein (SMV) and splenic vein (SV) drain separately into the inferior vena cava (IVC), and type II where the SMV and SV form a common channel which drains into IVC. ...
... OPV has been reported to occur in patients having Turner syndrome and Abernathy syndrome [16,17]. Familial cases of OPV have been identified and certain genetic mutations isolated [18]. ...
Purposeof Review
To review the clinical and histopathological features of obliterative portal venopathy (OPV).
Recent Findings
OPV is one cause of non-cirrhotic portal hypertension and is part of the general group of vascular liver diseases termed porto-sinusoidal vascular disease (PSVD), along with nodular regenerative hyperplasia and incomplete septal fibrosis/cirrhosis. It is increasingly being found on liver biopsies in patients having abnormal liver tests and is reliably diagnosable only by liver biopsy. Radiologically, patients may appear to have a cirrhotic liver but typically their liver tests are normal or mildly abnormal, and the liver function is normal.
Summary
Most cases of OPV are idiopathic but it can be seen in collagen vascular diseases, in the setting of portal vein thrombosis, in hematologic disorders, and immunodeficiency syndromes, and as a result of certain medications. Liver biopsy may show subtle histological findings so there must be a keener awareness amongst both clinicians and pathologists of this entity, as well as a discourse alerting the pathologist to the presence of portal hypertension.
... Other histological findings may be associated with the type of CPSS and the degree of arterial buffer response. 54 Consequently, in addition to comprehensive nodule sampling if present (see above), we recommend a biopsy of the nonnodular liver according to current standards. 55 The rationale is threefold. ...
... First, to evaluate vascular alterations, fibrosis and architectural changes which may impact closure strategy and outcome. 54,56,57 Second, to rule out other causes of liver disease and to inform management decisions. Specifically, in CHD cases, a liver biopsy can help determine whether to close the shunt, given the additional post-hepatic vascular insult. ...
Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.
... [3,4] In addition, most patients were children or adolescents. [5,6] Currently, only 13 elderly patients with CEPS (aged >65 years) have been reported in the literature (s upplementary Table 1). In addition, clinical presentation and treatment vary according to age. ...
... [18] The clinical significance of spontaneous portosystemic shunt formation is that on the one hand, the shunt can share part of the portal vein pressure, whereas on the other hand, gastric variceal bleeding after the formation of the shunt channel increases the risk of ectopic embolization during endoscopic embolization treatment, and the formation of spontaneous portosystemic shunts channel makes the treatment of gastric variceal bleeding very difficult. [19] A study of 700 patients with cirrhosis combined with portal hypertension demonstrated spontaneous portosystemic shunts in 16.86% of patients (gastrorenal shunt in 7.14% and splenorenal shunt in 8.43%). [20] Of the six patients in the present study, three had GOV1, two had GOV2, and one had IGV1, and all six had a significant spontaneous portosystemic shunt. ...
Background and objective:
Ectopic embolism caused by cyanoacrylate glue for the treatment of gastric varices with obvious spontaneous portosystemic shunts is a serious complication of endoscopic therapy. This study was performed to investigate the safety and therapeutic effect of EUS-guided coil placement and cyanoacrylate glue injection for gastric varices with obvious spontaneous portosystemic shunts.
Materials and methods:
Six patients with gastric variceal bleeding and obvious spontaneous portosystemic shunts were included in this study. We evaluated the success rate of variceal occlusion after intraoperative embolization, the postoperative rebleeding rate at 48 h and 2 weeks posttreatment, and the incidence of ectopic embolism and other adverse events. Gastroscopy and computed tomography portal venography (CTPV) were performed 7 months later.
Results:
All patients underwent successful coil placement and cyanoacrylate glue injection under EUS guidance. The blood flow was confirmed by Doppler examination, the target vessels were successfully blocked, and no rebleeding had occurred at 48 h or 2 weeks after endoscopic treatment. Gastroscopy was repeated 7 months after endoscopic treatment, revealing local ulcer formation. CTPV was also repeated 7 months after endoscopic treatment, showing that the coils were present in the target vessels with no displacement, the portosystemic shunt vessels were occluded, and no ectopic embolization had occurred.
Conclusion:
The coil placement combined with cyanoacrylate glue embolism is a safe and effective method for patients with gastric variceal bleeding and obvious spontaneous portosystemic shunts.
... Currently, CPSSs can be divided into four anatomic types [13,29]: ...
... Type IV: Persistent ductus venosus, characterized as an intrahepatic shunt from the proximal part of the left portal branch to the terminal part of the left hepatic vein and located in the depth of the Arantius sulcus between the left and caudate lobes of the liver [13,29]. ...
In this overview of vascular changes of the liver, variations in the liver vessels are discussed, in addition to congenital malformations such as Abernethy malformation, patent ductus venosus Arantii and hereditary hemorrhagic telangiectasia (OslerWeber-Rendu disease). Particular attention is paid to focal liver lesions, especially focal nodular hyperplasia (FNH), but also other solid tumours that develop as a result of altered liver vascularisation. The article focuses on the ultrasonic appearances and changes of the liver, depicted in B-mode sonography, Doppler studies and in contrast-enhanced ultrasonography (CEUS). The clinical manifestations of these conditions associated with other organ systems are also highlighted.
... The clinical manifestation of Abernethy syndrome ranges from asymptomatic to presentations related to systemic or hepatic sequelae like pulmonary hypertension, hepatopulmonary syndrome, hepatic encephalopathy, liver nodules, or tumors (focal nodular hyperplasia, nodular regenerative hyperplasia, hepatocellular adenomas, and hepatocellular carcinomas) [2,[7][8][9]. It is recognized that congenital portosystemic shunts are correlated with nephrotic syndrome [10]. ...
Type II Abernethy malformation is an extremely reported congenital extrahepatic portosystemic shunt in complication with nephrotic syndrome. We present the case of an 8-year-old boy who presented with symptoms of type II Abernethy malformation and nephrotic syndrome. This diagnosis of this type II Abernethy malformation was based on physical examination, blood tests, urinalysis, nephrotic and hepatic function tests, routine clinical lipid measurements, abdominal ultrasonography, and computed tomographic angiography. A kidney biopsy revealed the pathological features of nephrotic syndrome. This is the second reported patient diagnosed with type II Abernethy malformation and nephrotic syndrome. Captopril treatment was effective in improving the symptoms of this case. A patient with type II Abernethy malformation related to immune complex-mediated glomerular injury was effectively improved with medication. Type II Abernethy malformation is a causative factor of immune complex-mediated glomerular injury in nephrotic syndrome. Captopril treatment significantly improved the symptoms in this case.
... 22 One of the studies in dogs showed a significant decrease in steatosis based on histological examination of the liver 2-13 months after surgical attenuation of PSS. 20 Interestingly, in humans with congenital PSS, capillarisation of the sinusoidal endothelial cells has been described; 23,24 however, no documentation about this is available in dogs with PSS. Capillarisation or the loss of fenestrations between the sinusoidal endothelial cells in the liver promotes steatosis in humans, and capillarisation in turn promotes hepatic fibrosis. ...
Background
Skin and coat quality can reflect nutritional deficiencies in humans and dogs with liver diseases.
Hypothesis/Objectives
Determine skin and coat quality based on a scoring protocol and skin biopsies in dogs with an extrahepatic portosystemic shunt (EHPSS), and determine total lipid concentrations in hairs of dogs at time of surgery and 3 months after successful shunt closure.
Animals
Ten client‐owned dogs that underwent successful gradual attenuation of EHPSS, as defined by transsplenic portal scintigraphy, were included.
Materials and methods
A prospective cohort study was performed. All dogs underwent gradual attenuation of the EHPSS. Skin and coat scoring was performed at diagnosis, surgery, and 1 and 3 months postoperatively. Hair was plucked from the lumbar region for total lipid analysis and an 8 mm punch skin biopsy was taken at time of surgery and 3 months postoperatively, when the dogs underwent transsplenic portal scintigraphy to determine EHPSS closure.
Results
No significant differences were observed in skin and coat scoring over time. Total lipid concentrations of hairs increased significantly from surgery to 3 months postoperatively [30 μg/mg hair (13–56 μg/mg hair) to 47 μg/mg hair (25–63 μg/mg hair); p = 0.005]. Skin biopsies showed the presence of significantly more scales 3 months postoperatively (p = 0.018).
Conclusions and clinical relevance
A significant increase in total lipid concentrations in hairs suggests that successful surgical attenuation of EHPSS improves either intestinal absorption of lipids, fat metabolism in the liver, or a combination of both.
... CPSS causes nodular transformation in the pediatric liver. Of all types of CPSS, patent ductus venosus, portal vein to IVC, or left portal venous system to IVC are the most common (17)(18)(19)(20)(21)(22)(23). Liver tumors have been observed in both extra-(35%) and intra-hepatic (13%) types at a median age of eight years; however, the malignant tumors (HBL, HCC, and sarcoma) are exclusively seen alongside the extrahepatic variety. ...
ABSTRACT Liver cancer, predominantly hepatocellular carcinoma, is the second most common cause of cancer-related death in adults. Although infrequent in children, hepatocellular carcinoma is a terrifying diagnosis. Rising levels of obesity and obesity-associated lipid metabolic reprogramming of hepatocytes are increasing the prevalence of lipid-rich hepatocellular carcinoma in young adults. Most pediatric liver cancers occur in otherwise healthy liver, with some exceptions such as progressive familial intrahepatic cholestasis, hereditary tyrosinemia, alpha-1-antitrypsin deficiency, and genetic hemochromatosis. In the last decade, although aggressive multidisciplinary treatments including surgical resection and chemotherapy have remarkably improved patient outcomes in terms of decreased recurrence rate and increased overall survival rate, in children with unresectable liver cancer, the 5-year survival rate is still less than 20%. This chapter provides an overview of malignant epithelial tumors of the liver in children and adolescents. Hepatocellular carcinoma, lipid-rich hepatocellular carcinoma, fibrolamellar carcinoma, and cholangiocellular carcinoma are discussed.
