Abstract
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. Twenty-one patients were children at the time of diagnosis. Thirty-two specimens were available and consisted of three explant livers and 29 biopsy samples from 19 patients. Sixteen samples were from wedge biopsies taken at the time of shunt closure. Thirteen were from core needle biopsies taken during clinical work-up. A variable proportion of portal tracts contained prominent thin-walled channels (PTWCs) and arterio-biliary dyads. The proportion of portal tracts containing triads, arterio-biliary dyads and biliary monads varied considerably in the different samples. Dilated inlet venules, increase in the number of portal arteries or the presence of portal arteries of increased size, deposition of copper-associated protein, sinusoidal dilatation, capillarization and intralobular individual arteries were present. Physiological nuclear vacuolation of periportal hepatocytes was absent in most samples from our paediatric patients. Presence of PTWCs, arterial-biliary dyads, increased arterial profiles in portal tracts and lobule and lack of the physiological periportal vacuolated hepatocytes in children are the most characteristic histological changes of CPSS in the liver periphery.
Highlights
Congenital portosystemic shunt (CPSS) are congenital anomalies resulting in partial or complete diversion of the portalElectronic supplementary material The online version of this article contains supplementary material, which is available to authorized users.Geneva, Switzerland 3 Department of Paediatric Surgery, King’s College Hospital, London, UK 4 Paediatric Liver, GI & Nutrition Centre, King’s College HospitalLondon, London, UK 5 Department of Cellular Pathology Royal Free Hospital, UniversityCollege, London, UK blood into the systemic circulation
Our findings in a larger series of 22 patients with CPSS who were managed clinically in our institution over a period of 15 years, and from whom background liver tissue was available for histological review
We defined persistent ductus venosus (PDV) according to the criteria by Blanc et al [12]: ‘intrahepatic shunt running 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’
Summary
Switzerland 3 Department of Paediatric Surgery, King’s College Hospital, London, UK 4 Paediatric Liver, GI & Nutrition Centre, King’s College Hospital. First described by John Abernethy [1], they are thought to be related to defective portal vein embryogenesis, as discussed by Wanless et al [2]. CPSS are increasingly being diagnosed in the neonatal period when an ultrasound is being performed for neonatal jaundice. Another large proportion is picked up incidentally when having an ultrasound for unrelated reasons. If gone unnoticed until adult life, presentation may be due to symptoms related to a hepatic nodule, signs of low-grade encephalopathy, pulmonary hypertension, hypoglycaemia, chronic abdominal pain and/or elevated ammonia levels
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