Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus—a case report of its rare clinical presentation in a young girl

Kanamori, Yutaka; Hashizume, Kohei; Kitano, Yoshihiro; Shimada, Masahiko; Motoi, Toru; Tange, Tsuyoshi

doi:10.1053/jpsu.2003.50153

Cited by 45 publications

(53 citation statements)

References 14 publications

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“…Typically, the shunt is wholly extrahepatic between the PV or its right branch and the retrohepatic IVC (Mboyo et al, 1995;Howard and Davenport, 1997;Kanamori et al, 2003) but in some cases, as in Case 5 in the present series, the shunt traverses the caudate lobe and is partly intrahepatic (Takayama et al, 2001).…”

Section: Extrahepaticmentioning

confidence: 66%

“…In type 2 shunts, the intrahepatic PV may be normal or hypoplastic (Ikeda et al, 1999;Kanamori et al, 2003;Tercier et al, 2006). Associated congenital anomalies are less common than with type 1 shunts although the spectrum of disorders is similar.…”

Section: Extrahepaticmentioning

confidence: 99%

“…Associated congenital anomalies are less common than with type 1 shunts although the spectrum of disorders is similar. The following associations have been recorded: pulmonary valve atresia (Howard and Davenport, 1997), patent ductus arteriosus (Kanamori et al, 2003), Goldenhar syndrome (Kalifa et al, 1978), polysplenia and IVC anomalies (Murray et al, 2003), and Cornelia de Lange syndrome (Ikeda et al, 1999).…”

Section: Extrahepaticmentioning

confidence: 99%

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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: 66%

Section: Extrahepaticmentioning

confidence: 99%

Section: Extrahepaticmentioning

confidence: 99%

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The clinical anatomy of congenital portosystemic venous shunts

2007

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“…Uchino, et al identified 51 cases of congenital portosystemic shunt in Japan ; 34 of the intrahepatic type and 17 of the extrahepatic type, of which spontaneous closure was occurred only in the intrahepatic type, but not in the extrahepatic type (17). If patients with CEPS are left untreated, they will sustain various consequences such as repeated encephalopathy (7,18), neurocognitive dysfunction (14), pulmonary hypertension (19), hepatopulmonary syndrome (9), cataract (3), liver atrophy (16), liver tumors (10,14) and so on. Eroglu, et al reported damage incurred during development in childhood could reduce the functional capacity of the adult (14).…”

Section: Discussionmentioning

confidence: 99%

Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children

et al. 2009

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“…Cells reactive to anti-CD68 along a nodule sinusoid were fewer than in surrounding liver (Fig. 6D) (11,12), and 3 cases with hyperplastic nodular hepatic lesions (13)(14)(15) …”

Section: F I G U R E 4 T H R E E -D I Me N S I O N a L C O Mp U T Ementioning

confidence: 99%

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

et al. 2007

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Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus—a case report of its rare clinical presentation in a young girl

Cited by 45 publications

References 14 publications

The clinical anatomy of congenital portosystemic venous shunts

The clinical anatomy of congenital portosystemic venous shunts

Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children

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

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