Portal vein absence and nodular regenerative hyperplasiaof the liver with giant inferior mesenteric vein

Arana, Estanislao; Martí‐Bonmatí, Luis; Martínez, Vicente; Hoyos, Marcos López; Montes, Hortensia

doi:10.1007/s002619900249

Cited by 46 publications

(29 citation statements)

References 16 publications

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“…Either type 1 or type 2 portocaval shunts can be associated with neonatal jaundice (Arana et al, 1997;Howard and Davenport, 1997), as in Case 5 in this series. Liver function tests show a conjugated hyperbilirubinemia which may be due to biliary atresia or to a nonspecific neonatal hepatitis.…”

Section: Extrahepaticmentioning

confidence: 89%

“…The recipient systemic vein may be the left renal vein (Kim et al, 1998;Shinkai et al, 2001;Murray et al, 2003), right renal vein (Murray et al, 2003), or azygos vein (Shinkai et al, 2001;Bonington et al, 2002). More rarely, and somewhat bizarrely, there have been a few reports of a direct communication between the inferior mesenteric vein (or its superior rectal tributaries) and the common, left or right internal iliac vein (Arana et al, 1997;Grazioli et al, 2000;Charre et al, 2004;Goo, 2007).…”

Section: Extrahepaticmentioning

confidence: 94%

“…Both complications are much more likely with type 1 shunts. Benign hepatic tumors (nodular regenera- tive hyperplasia, adenoma, focal nodular hyperplasia) (Nakasaki et al, 1989;Arana et al, 1997;Grazioli et al, 2000;Kinjo et al, 2001;Kanamori et al, 2003;Murray et al, 2003) and malignant liver tumors (hepatoblastoma and hepatocellular carcinoma) (Joyce and Howard, 1988;Barton and Keller, 1989) have been reported. Benign tumors may regress if the type 2 shunt is closed as in Case 5 in this series.…”

Section: Extrahepaticmentioning

confidence: 95%

“…Morgan and Superina (1994) reported one child where an abnormal tortuous retrohepatic PV drained directly into the right atrium. In some patients with type 1 shunts, portal venules within the liver appear small or absent (Arana et al, 1997;Altavilla and Cusatelli, 1998;Grazioli et al, 2000) while in others liver biopsy appearances are normal (Akahoshi et al, 2000). The hepatic artery and its intrahepatic branches are frequently noted to be enlarged (Howard and Davenport, 1997;Grazioli et al, 2000;Kinjo et al, 2001).…”

Section: Extrahepaticmentioning

confidence: 96%

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The clinical anatomy of congenital portosystemic venous shunts

2007

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Congenital portosystemic venous shunts are rare. Their gross anatomy has not been well defined. Four different varieties of congenital portosystemic venous shunts are described in six children seen during a 10-year period, focusing on the anatomy of the shunt as determined by imaging studies and surgery. A detailed review of the literature indicates that congenital portosystemic venous shunts are best classified as: extrahepatic or intrahepatic. Extrahepatic shunts may be further subdivided into portocaval shunts (type 1 end-to-side and type 2 side-to-side) and others. Intrahepatic shunts are due to an abnormal intrahepatic connection between the portal vein and hepatic vein/inferior vena cava or a persistent patent ductus venosus. Additional congenital anomalies, particularly cardiac malformations, may be associated with any type. Some congenital intrahepatic portosystemic venous shunts close spontaneously in infancy; all other congenital portosystemic venous shunts tend to remain patent. To a variable extent, depending largely on the volume and duration of the shunt, affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor. The key to understanding the pathogenesis of these shunts lies in the normal developmental mechanisms underlying the formation of the portal vein and inferior vena cava in the embryo.

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Section: Extrahepaticmentioning

confidence: 89%

Section: Extrahepaticmentioning

confidence: 94%

Section: Extrahepaticmentioning

confidence: 95%

Section: Extrahepaticmentioning

confidence: 96%

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The clinical anatomy of congenital portosystemic venous shunts

2007

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“…Other than CAPV, many cases are presented with liver abnormalities, including FNH [7,15,[18][19][20][21][22][23][24][25][26][27][28][29][30] or nodular regenerative hyperplasia (NRH) [8,[31][32][33][34][35] . Several patients have been found to have combined hepatocellular adenoma [31,36,37] .…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Insight into congenital absence of the portal vein: Is it rare?

Shen

Zhu²

2008

WJG

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Congenital absence of the portal vein—Case report and a review of literature

Mištinová

Valacsai²,

Varga

2010

Clinical Anatomy

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Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and the splenic venous drainage bypass the liver and drain into systemic veins through various venous shunts. To our knowledge, we have reviewed all 83 cases of CAPV, since first described in 1793. This equates to a rate of almost 2.5 cases per year over the last 30 years. Morgan and Superina (1994, J. Pediatr. Surg. 29:1239-1241) proposed the following classification of portosystemic anomalies; either the liver is not perfused with portal blood because of a complete shunt (Type I) or the liver is perfused with portal blood due to the presence of a partial shunt (Type II). In our case, abdominal venous blood drained into the suprarenal inferior vena cava via the left renal vein and dilated left gastric veins. After analyzing all reported cases, we recognize that more than 65% of patients are females and more than 30% of all published cases had been diagnosed by the age of 5 years. Additional anomalies are common in CAPV. In the reported cases, more then 22% of patients had congenital heart disease. Other commonly found anomalies include abnormalities of the spleen, urinary and male genital tract, brain as well as skeletal anomalies. Hepatic changes such as focal nodular hyperplasia, hepatocellular carcinoma, and hepatoblastoma are diagnosed in more then 40% of patients. This article also illustrates the radiological findings of CAPV. Radiological evaluation by ultrasound, CT, and MRI is helpful to detect coexisting abnormalities.

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Portal vein absence and nodular regenerative hyperplasiaof the liver with giant inferior mesenteric vein

Cited by 46 publications

References 16 publications

The clinical anatomy of congenital portosystemic venous shunts

The clinical anatomy of congenital portosystemic venous shunts

Insight into congenital absence of the portal vein: Is it rare?

Congenital absence of the portal vein—Case report and a review of literature

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