Hyperplastic Nodular Hepatic Lesions Following End-to-side Portacaval Shunting in Childhood

Fukushima, Nobuyoshi; Kuromatsu, Ryoko; Uchiyama, Daiji; Itano, Satoshi; Takata, Akio; Ando, Eiji; Sumie, Shuji; Torimura, Takuji; Uchida, Masafumi; Nakashima, Osamu; Kojiro, Masamichi; Sata, Michio

doi:10.2169/internalmedicine.46.6419

Cited by 7 publications

(4 citation statements)

References 23 publications

(5 reference statements)

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“…While CHF belongs to a group of fibropolycystic diseases of the liver that affects the biliary system, portal hypertension dominates the clinical presentation of this disease, as the number of portal vein branches is often reduced [9]. Treatment of CHF with portosystemic shunt surgery may also add to the abnormal heterogeneous vascularity in the parenchyme [10]. As a result, the uneven hepatic perfusion may give rise to the formation of hepatocellular nodules.…”

Section: Discussionmentioning

confidence: 99%

Multiple hepatocyte nuclear factor 1A (HNF1A)-inactivated hepatocellular adenomas arising in a background of congenital hepatic fibrosis

Lee

Park

Lee

et al. 2021

J Pathol Transl Med

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Section: Discussionmentioning

confidence: 99%

Multiple hepatocyte nuclear factor 1A (HNF1A)-inactivated hepatocellular adenomas arising in a background of congenital hepatic fibrosis

Lee

Park

Lee

et al. 2021

J Pathol Transl Med

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“…The absence of portal hepatotrophic factors (such as insulin and glucagon [ 27 , 28 ] and epidermal growth factor [ 29 ]) due to CAPV is probably a risk factor for the development of nodular liver lesions: benign tumors such as hepatocellular adenomas, focal nodular hyperplasia (FNH) [ 30 ], and nodular regenerative hyperplasia (NRH) [ 31 ], as well as malignant ones, such as hepatoblastoma [ 32 ], and hepatocellular carcinoma (HCC) [ 8 ]. Another possible explanation is that initially benign tumors reactively arise in the areas of the liver with poor perfusion, due to the lack of portal venous blood flow [ 33 ].…”

Section: Discussionmentioning

confidence: 99%

Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review

et al. 2015

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Patient: Female, 21Final Diagnosis: Unresectable liver adenomatosis associated with congenital absence of portal veinSymptoms: —Medication: —Clinical Procedure: Living donor liver transplantationSpecialty: TransplantologyObjective:Rare diseaseBackground:Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults.Case Report:We report the case of a 21-year-old woman diagnosed with type Ib AM (portal vein draining directly into the inferior vena cava) and unresectable liver adenomatosis. The patient presented mild liver dysfunction and was largely asymptomatic. Living donor liver transplantation was performed using a left hemiliver graft from her mother. Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life.Conclusions:We consider that living donor liver transplantation is the best therapeutic solution for AM associated with unresectable liver adenomatosis, especially because compared to receiving a whole liver graft, the waiting time on the liver transplantation list is much shorter.

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“…Experiments in rat models shows that hyperplastic lesions develop in liver following vascular disruption such as surgical porto systemic shunts 2 . Hyperplastic hepatic lesions following portacaval shunting surgery in childhood are reported in case reports 6 . In the largest case series in pediatric patient population, post porto‐systemic shunt surgery for extra hepatic portal vein obstruction, seven out of 45 children (15%) developed liver nodules after a median of 80 months 7 …”

Section: Introductionmentioning

confidence: 99%

Epidemiology, pathogenesis, diagnosis, surveillance, and management of hepatocellular carcinoma associated with vascular liver disease

Prasad

Nguyen

2021

The Kaohsiung J of Med Scie

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Vascular liver disease (VLD) presents special challenges in the diagnosis, surveillance, and treatment of hepatocellular carcinoma (HCC). HCC arising in the setting of vascular liver disease is often thought to be due to elevated hepatic arterial blood flow, rather than progressive fibrosis from chronic inflammation as with other chronic liver conditions such as viral hepatitis, autoimmune, and metabolic liver diseases. Vascular alteration inherent in VLD often impedes HCC non‐invasive diagnosis and loco‐regional treatment that depend on vascular properties found in typical liver environment. Benign and pre‐malignant liver nodules such as focal nodular hyperplasia and hepatocellular adenoma are also more common in certain VLDs, further adding to surveillance and diagnostic challenges. In this synopsis, we aimed to review available literature on the epidemiology, surveillance, diagnosis, and management of HCC in patients with VLD and specifically Budd–Chiari syndrome, congenital porto‐systemic shunts, Fontan‐associated liver disease, hereditary hemorrhagic telangiectasia.

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Hyperplastic Nodular Hepatic Lesions Following End-to-side Portacaval Shunting in Childhood

Abstract: We describe a 48-year-old

Cited by 7 publications

References 23 publications

Multiple hepatocyte nuclear factor 1A (HNF1A)-inactivated hepatocellular adenomas arising in a background of congenital hepatic fibrosis

Multiple hepatocyte nuclear factor 1A (HNF1A)-inactivated hepatocellular adenomas arising in a background of congenital hepatic fibrosis

Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review

Epidemiology, pathogenesis, diagnosis, surveillance, and management of hepatocellular carcinoma associated with vascular liver disease

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