Predisposing Factors for Spontaneous Closure of Congenital Portosystemic Shunts

Paganelli, Massimiliano; Lipsich, José; Sciveres, Marco; Alvarez, Fernando

doi:10.1016/j.jpeds.2015.06.073

Cited by 35 publications

(39 citation statements)

References 40 publications

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“…Until 1 year of age, a wait-and-see policy is the initial recommended treatment of IPSS. Spontaneous closure of an IPSS is more often seen in girls, in the presence of multiple shunts, and in children with neonatal cholestasis [9]. Our patient showed no tendency toward spontaneous closure.…”

Section: Discussionmentioning

confidence: 42%

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report

et al. 2020

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Background: Congenital portosystemic shunt (CPSS) is a rare malformation that leads to hyperammonemia, hypermanganesemia, and various symptoms. CPSSs are divided into intrahepatic and extrahepatic shunts. In patients with persistent CPSS including an intrahepatic portosystemic shunt (IPSS), early intervention to occlude the shunt reverses the associated complications. Case presentation: The patient was a 1-year-and-7-month-old girl. She presented with hypergalactosemia and elevation of blood ammonia level (75 μg/dL) and total bile acid levels (68.2 μmol/L) during the neonatal period. Two IPSSs were detected using ultrasound and enhanced computerized tomography. Magnetic resonance imaging (MRI) at 1 year and 3 months of age showed abnormally high signal intensity in the pallidum of her brain. Spontaneous closure was not observed. We performed a right hepatectomy at 1 year and 7 months of age. The portal vein pressure was 16 mmHg after temporary occlusion of the right portal vein. Blood ammonia and serum manganese levels decreased immediately after the operation. The abnormal signal on brain MRI disappeared. She had a favorable course with no sign of recurrence of IPSS 5 years postoperatively. Conclusion: Liver resection for an IPSS to control the symptoms of a portosystemic shunt is reasonable in a child for whom interventional radiological treatment is not indicated.

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Section: Discussionmentioning

confidence: 42%

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report

et al. 2020

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“…Franchi-Abella et al 27 reported that shunt closure should always be performed when complications are present, with the exception of those resolving spontaneously. 36 They suggested that even when clinical manifestations are not meaningful, the procedure is still recommended when the patient is able to tolerate, because the treatment can be ineffective if performed after important complication occurs. Noteworthy, as previously reported, once patients begin to develop severe shunting, the shunt ratio can quickly increase.…”

Section: Discussionmentioning

confidence: 99%

Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults—case report and review of the relevant literature

et al. 2018

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The Abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Although congenital extrahepatic portosystemic shunts are increasingly suspected and diagnosed in specialized centres, much of their clinical presentation and natural history is not fully understood. Symptoms of portosystemic shunt are mainly caused by increased levels of ammonia, which lead to signs of encephalopathy. Therapeutic options depend on the type of shunt and its clinical course, so the classification of the congenital portosystemic shunt is a key finding in these patients.

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“…The type of CPSS was subclassified into four types:extrahepatic end to side shunt with no detectible flow into the intrahepatic portal system, (type 1);extrahepatic either side to side or H type shunt with some preserved intrahepatic portal flow, (type 2);intrahepatic shunt(s), any configuration except for persistent ductus venosus (intrahepatic type);persistent ductus venosus (PDV) [9–13]…”

Section: Methodsmentioning

confidence: 99%

Histopathology of livers in patients with congenital portosystemic shunts (Abernethy malformation): a case series of 22 patients

et al. 2018

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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.Electronic supplementary materialThe online version of this article (10.1007/s00428-018-2464-4) contains supplementary material, which is available to authorized users.

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Predisposing Factors for Spontaneous Closure of Congenital Portosystemic Shunts

Cited by 35 publications

References 40 publications

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report

Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults—case report and review of the relevant literature

Histopathology of livers in patients with congenital portosystemic shunts (Abernethy malformation): a case series of 22 patients

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