Congenital absence of portal vein with nodular regenerative hyperplasia of the liver

Abstract

Congenital absence of the portal vein is a very rare anomaly. The intestinal and splenic venous drainage bypasses the liver and drain into the inferior vena cava (IVC). Two cases of such anomaly are described. Both cases were investigated by US coupled with echo-colour Doppler examination, CT and MR imaging, followed by digital subtraction angiography (DSA) and liver biopsy. In the first case the splenic and superior mesenteric vein formed a venous trunk which emptied directly into the IVC; in the second case, the splanchnic blood flowed into a dilated hepatofugal inferior mesenteric vein which connected to the left internal iliac vein. In both cases nodular regenerative hyperplasia of the liver was present, presumably due to an abnormal hepatic cell response to the absent portal flow. The particular contribution of MR imaging to the diagnosis of both vascular abnormalities and liver parenchyma derangement and its advantages over the other diagnostic techniques is emphasized. The clinical and radiological features of 17 previously reported cases are reviewed.

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

“…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) …”

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

“…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] .…”

“…A large number of non-heart abnormalities are found in all the cases reviewed [ malformations have been reported, such as double SV [1] , double inferior vena cava [40] , left sided IVC [2,17,26] , hepatic artery originating from superior mesenteric artery [8,50] , intrapulmonary shunting [7] , azygos and hemiazygos continuation [1,17,43,48] , and skin hemangioma [14,43] . Obvious compensatory changes may be found in blood vessels, especially in veins returning visceral blood or ramus anastomoticus, such as the SV [51] , inferior mesenteric vein [33,52] , left renal vein [44,53] , azygos system [30,51] , IVC [14,44,53] , and right atrium [14] . Hepatic artery is enlarged and hypertrophied [1,19] due to the same reason.…”

“…The urinogenital system including cystic dysplasia of the kidneys [22,24] , bilateral ureteropelvic obstruction of the kidneys [45] , vesicoureteral reflux [33] , crossed fuses renal ectopia [33,40] , and hypospadias [56] , is also involved. Nonfunctioning pancreatic tumor [25] , ulcerative colitis(UC) [33] , juvenile polyposis [21] , inguinal hernia [23,37] , and even situs inversus viscerum [2,4] , are sporadically observed in CAPV patients.…”

“…The skeletal systematic abnormalities, such as radial hypoplasia [33] and congenital absence of the first metacarpophalangeal complex of the right hand [33] , are noted in Grazioli's patients. Vertebral abnormalities or hemivertebra scoliolosis [15,40,57] , and oculoauriculovertebral dysplasia or Goldenhar's syndrome [16,26,28,58] exhibit thoracic hemivertebrae, right maxillary hypoplasia, mild micrognathia, and short fifth fingers, are not uncommon in CAPV patients.…”