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BILIARY OBSTRUCTION in the REGION of the PORTA HEPATIS Hunterian Lecture Delivered at the Royal College of Surgeons of England on 13Th February 1958* by Anthony J

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BILIARY OBSTRUCTION in the REGION of the PORTA HEPATIS Hunterian Lecture Delivered at the Royal College of Surgeons of England on 13Th February 1958* by Anthony J BILIARY OBSTRUCTION IN THE REGION OF THE PORTA HEPATIS Hunterian Lecture delivered at the Royal College of Surgeons of England on 13th February 1958* by Anthony J. H. Rains, M.S., F.R.C.S. Senior Lecturer in Surgery, University of Birmingham, Surgeon, United Birmingham Hospitals IN CLINICAL PRACTICE, cases of obstruction of the bile ducts in this particular region of anatomy are comparatively rare, though they are of acclaimed notoriety. In a series of 2,033 cases of disease of the gall- bladder and bile ducts treated in the United Birmingham Hospitals, there were ninety-three cases of primary obstruction in the region of the porta hepatis (4.5 per cent.). When first encountering a case, one is assailed by personal lack of experience of such lesions and the knowledge which can be applied to provide a remedy or relief for the patients who are often afflicted at the dawn or in the prime of their lives, by lesions which are not always intrinsically fatal but which have fatal consequences in so far as they lead to the impairment of the drainage of bile with increasing liver damage. Unlike obstruction to blood vessels, no help is to be expected from the natural development of collaterals, and the surgeon is faced with relieving the obstruction or the eventual death of the patient. His task is made difficult, not only by the anatomical inaccessibility and the complexity and variations of bile ducts, hepatic artery and portal vein, but also by a characteristic inflammatory response to local extravasations of bile, varieties of cholangitis, and what is often misguidedly called " previous operative interference." Types of lesion Table I shows the different types of lesion that may be encountered and the incidence in this series. Cases of obstruction due to secondary deposits in the liver or in the hilar lymph nodes have not been included. One case of liver fluke, encountered on surgical travels, has been added; also four cases of cholangitis from Mr. F. S. A. Doran. Problems and experience in pathology Besides the characteristics of each cause, there is also a general pattern or picture of the surgical pathology of the effects of obstruction on the bile ducts, the liver parenchyma and on liver function and bile formation. * While the parish register of Long Calderwood, near Glasgow, says that John Hunter was born on the 13th February 1728, he himself observed the 14th as his birthday. Probably he was born during the night of 13th to 14th. 69 ANTHONY J. H. RAINS TABLE I BILIARY OBSTRUCTION IN THE REGION OF ThE PORTA HEPATIS Types of Lesion INTRAMURA1L LESIONS Neonatal.. 13 Traumatic 17 Inflammation 20+4 Neoplastic 35 INTRALUMIN'AR OBSTRUCTIONS Stone .. 2 Foreign body Parasites .. 1 EXTRAMURAL LESIONS Choledochal cysts Liver abscess .. Hydatid cyst I Hepatoma and primary liver carcinoma Neoplastic hilar glands I Secondary deposits in liver Renal swellings. Cysts.. 93 Total with additions= 98 * The number of cases of obstruction which is secondary to metastatic deposits in the liver or to infiltration of portal lymph nodes have not been included in the numbers of types of lesion. With some special exceptions (fistulae) the clinical picture is one of jaundice, and the differential diagnosis between obstruction and primary liver disease must be considered, though the solution cannot always be found without exploratory laparotomy. The following discussion of special points in pathology is given in a manner to portray the problems as they are encountered by the surgeon at operation, at autopsy and in the surgical laboratory. Neonatal cases and congenital causes Different lesions and anomalies occur (Keith, 1933; Kirk, 1948; Gross, 1953 ; Bowden and Donohue, 1955). The commonest cause appears to be one of failure of canalisation of the endodermal bud, the " anlage " of the biliary tract, which grows out in the fourth to fifth week of life from the duodenum at the lower end of the foregut. The liver, which develops within the septum transversum from this bud, lies in close relationship to the duodenum, and it is only as the development of the foetus progresses that separation occurs, with elongation of the common and hepatic bile ducts and, incidentally, the formation of the gall-bladder. As a result of such elongation, stenosis and atresia may occur. It is also said that after primary canalisation, a period of occlusion from rapid endodermal proliferation takes place at about the fifth or sixth week (also occluding the duodenum). Normally restoration of the lumen follows, but, if not, partial or complete stenosis or atresia remains. Another variety of obliteration is fibrocystic disease in which there is an incomplete or atretic lumen (Fig. 1). When congenital obliteration occurs, it may be generalised in the extrahepatic bile ducts or it may pick out the gall-bladder or any section 70 BILIARY OBSTRUCTION IN THE REGION OF THE PORTA HEPATIS of the bile ducts in a bizarre fashion. If the gail-bladder is in continuity with the duodenum it will be found to be flaccid and empty save for some inspissated mucus. If the obliteration does not extend into the intrahepatic ducts, examination of the porta hepatis will reveal the left or right hepatic ducts bulging with pale or white bile, a relatively fortunate state of affairs if relief is to be obtained by a surgical by-pass. In some cases the bile Fig. 1. Section of the bile ducts in a case of neonatal jaundice showing fibrocystic disease. The lumen (which contains inspissated mucus) was not in continuity with the duodenum. x 30. ducts may be so small and empty as to make it extremely difficult at operation to distinguish between a failure of development and some of the hopeless forms of neonatal hepatitis and cirrhosis. In this respect the practical value of liver biopsy and frozen section is established, for where the liver structure is completely disorganised, fat and glycogen deposited, bile pigment granules and thrombi present with acute and chronic inflammatory cells, or bile ducts and canaliculi absent, the surgeon is quite unable to be of any assistance. There is some evidence to challenge the view that obliteration of the bile ducts is due to a fault in development, and to suggest that some cases are due to a stenosing cholangitis similar in nature to that affecting adults. Whether this disease spreads upwards from the duodenum or reaches the porta hepatis through the umbilicus and ligamentum teres or comes downwards from the liver is not known. Case report A girl aged thirteen days became jaundiced. Laparotomy revealed a flaccid gall-bladder. The bile ducts were tiny but patent and a fine probe passed into 71 ANTHONY J. H. RAINS : "'''§ < '7~~s~si _ M l l Fig. 2(a). Cross section ofpart ofthe ligamentum teres showing a heavy infiltration of inflammatory cells. x 60 011' I... r. a 1- :. -Y." .1-Aft. M. Fig. 2(b). A high power view of the liver parenchyma in the same case as Figure 2(a), showing bile pigment granules and thrombi consequent upon the compression of bile ducts by inflammatory cells. x 275 72 BILIARY OBSTRUCTION IN THE REGION OF THE PORTA HEPATIS the hepatic ducts was " gripped " as by a stricture, and a diagnosis of intra- hepatic obstruction was made, to be confirmed at autopsy. Histology of the liver showed compression of the bile ducts and a conspicuous infiltration of periportal spaces with lymphocytes and large mononuclear cells. Histology of the ligamentum teres revealed peri-arterial focal infiltration with lymphocytes and large mononuclear cells, and a similar heavy infiltration was found close to the remnant of the urachus (Fig. 2(a) and (b).) In some cases of neonatal obstruction the jaundice is fluctuating and comparatively late in appearing (three weeks) which, with the appearances of intrahepatic periportal cirrhosis supports the idea of an acquired disease. Of special interest is the report by Pappenheimer and others (1955) of the puzzling occurrence of neonatal jaundice due to the obliteration of the bile ducts in their stock of mice. Other cases of obstruction are due to blockage of the smaller bile ducts with inspissated plugs of bile, while others are due to large bile thrombi caused by hyperbilirubinaemia in haemolytic disease of the newborn. Strictures of traumatic origin Almost all of these strictures develop in relation to a previous operation on the gall-bladder or the bile ducts, but very occasionally one will follow rupture of the liver. There is one case in this series in which the rupture extended into the porta hepatis and repair over a T-tube was successful in relieving the jaundice which had developed. Damage to the bile ducts at operation, with resultant stricture formation is a subject concerning which a legion of contributions have been made (Cattell, 1947; Cole, 1948; Lahey and Pyrtek, 1950; Walters, 1953; Maingot, 1951; Ogilvie, 1957). Due in no small part to the stress laid in teaching and at examinations on the prophylaxis of such an accident, the incidence is very low, and in any collection of cases of obstruction at the porta hepatis, such lesions should not be expected to make up the bulk of the material. Of 1,730 cases having operations on the gall-bladder or common duct, eleven cases supposedly due to operative trauma occurred in the United Birmingham Hospitals (0.6 per cent.). An additional nine cases referred from other hospitals brings the total up to eighteen. Of these, sixteen (eight plus eight) were obstructions in the region of the porta hepatis. There is also a small group of cases, outside the scope of this paper, having low biliary strictures consequent upon disease or operations affecting the common bile duct in the region of the first and second parts of the duodenum (i.e., for duodenal ulcer).
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