The Eck fistula in animals and humans

Abstract

In all species so far studied, including man, portacaval shunt causes the same changes in liver morphology, including hepatocyte atrophy, fatty infiltration, deglycogenation, depletion and disorganization of the rough endoplasmic reticulum (RER) and its lining polyribosomes, and variable but less specific damage to other organelles. Many, perhaps all, biosynthetic processes are quickly depressed, largely secondary to the selective damage to the RER, which is the "factory" of the cell. These structural and metabolic changes in the liver after portal diversion are caused by the diversion around the liver of the hepatotrophic substances in portal venous blood, of which endogenous insulin is the most important. In experimental animals, the injury of Eck's fistula can be prevented by infusing insulin into the tied-off hilar portal vein. The subtle but far-reaching changes in hepatic function after portal diversion have made it possible to use this procedure in palliating three inborn errors of metabolism: glycogen storage disease, familial hypercholesterolemia, and alpha 1-antitrypsin deficiency. In these three diseases, the abnormalities caused by portal diversion have counteracted abnormalities in the patients that were caused by the inborn errors. In these diseases, amelioration of the inborn errors depends on the completeness of the portal diversion. In contrast, total portal diversion to treat complications of portal hypertension is undesirable and always will degrade hepatic function if a significant amount of hepatopetal portal venous blood is taken from the liver. When total portal diversion is achieved (and this is to be expected after all conventional shunts), the incidence of hepatic failure and hepatic encephalopathy is increased. If portal diversion must be done for the control of variceal hemorrhage, a selective procedure such as the Warren procedure is theoretically superior to the completely diverting shunt. In practice, better patient survival has not been achieved after selective shunts than after conventional shunts, but the incidence of hepatic encephalopathy has been less.

Fig 1

The primitive technique of side-to-side portacaval anastomosis used by Eck and by Pavlov’s group. Note that the connection between the two vessels was made by cutting or avulsing the adjacent walls inside a row of approximating simple sutures. The illustration is a photograph of the original drawing by Hahn et al.

Fig 2

Completely diverting portacaval shunt (Eck’s fistula). A side-to-side anastomosis is constructed between the portal (or superior mesenteric) vein (P.v.) and the anterolateral surface of the inferior vena cava (l.v.c.). The shunt is made completely diverting by tying off the portal vein in the hilum of the liver. (From Starzl et al. Reproduced by permission.)

Fig 3

Human liver biopsy appearances before (A) and after (B and C) portacaval shunt for familial hypercholesterolemia. Rough endoplasmic reticulum (r) and glycogen are abundant in the preoperative biopsy specimen. The hepatocyte is normal. Six months after portacaval anastomosis (B, C), there are major changes. Note that only isolated profiles of rough endoplasmic reticulum remain. Glycogen is absent, and there are numerous fat droplets (electron micrographs; A, ×3015; B, ×5575; C, ×25,100). (From Starzl et al. Reproduced by permission.)

Fig 4

The operation of portacaval transposition. The procedure is a standard laboratory experiment but it has been used clinically to treat two patients with glycogen storage disease. Note that the central portal vein is revascularized with vena caval blood. (From Starzl et al. Reproduced by permission.)

Fig 5

Auxiliary liver transplantation in dogs by a modification of Welch’s original technique. Note that the reconstituted portal blood supply is from the distal inferior vena cava. (From Starzl et al. Reproduced by permission.)

Fig 6

An auxiliary homograft (right) and the recipient dog’s own liver (left) 45 days after transplantation. Note the well-preserved but dimensionally reduced general structure of the homograft. At the time of transplantation, both the host organ and the transplant had been about the same size. (From Starzl et al. Reproduced by permission.)

Fig 7

The operation of partial (split) transposition in dogs. Note that one of the main portal veins (left in A, right in B) retains the natural splanchnic flow and that the other one receives the total input of the suprarenal inferior vena cava. (From Marchioro et al. Reproduced by permission.)

Fig 8

Splanchnic division experiments. In these dogs, the right liver lobes received venous return from the pancreaticogastroduodenosplenic region, and the left liver lobes received venous blood from the intestines. A, nondiabetic dogs; B, alloxan-induced diabetic dogs; C, dogs with total pancreatectomy. (From Starzl et al. Reproduced by permission.)

Fig 9

Hepatocyte shadows traced during histopathologic examination. These were later cut out on standard paper and weighed as an index of hepatocyte size. The right lobes with the large hepatic cells received venous blood from the pancreas, stomach, duodenum, and spleen. The relatively shrunken left lobes with the small hepatocytes received intestinal blood. (From Starzl et al. Reproduced by permission.)

Fig 10

Experiments in which Eck’s fistula is constructed and postoperative infusions of hormones are made into the left portal vein. (From Starzl et al. Reproduced by permission.)

Fig 11

Experimental models used in dogs and pigs to test the hypothesis that the liver can remove transplantation antigens. A, orthotopic renal transplantation in which the venous return was into the inferior vena cava. B, orthotopic transplantation, but venous return is into the portal vein. (From Mazzoni et al. Reproduced by permission.)

Fig 12

Plasma insulin and glucose concentrations before and after portacaval shunt in a child with type I glycogen storage disease. (From Starzl et al. Reproduced by permission.)

Fig 13

Effect of parenteral hyperalimentation and end-to-side portacaval shunt on the plasma lipids of a patient with the diagnosis of type I glycogen storage disease. Note the rapid and relatively complete reversal of all abnormalities. (From Starzl et al. Reproduced by permission.)

Fig 14

The dramatic wrist and hand bone growth and mineralization in a patient with type I glycogen storage disease by 11½ months postoperatively. The bracket on the left index finger is 5 cm in length. (From Starzl et al. Reproduced by permission.)

Fig 15

The hands of a patient with hyperlipidemia 2 weeks before (left) and 16 months after (right) portacaval shunt. (From Starzl et al. Reproduced by permission.)

Fig 16

Kinds of portal-systemic shunts. A, end-to-side portacaval shunt. B, variety of side-to-side shunts. C, selective portal-systemic shunts.

Fig 17

End-to-side portacaval shunt, combined with arterialization of the tied-off central portal vein.