Gastrointest Radiol 9: 223-226 (1984) Gastrointestinal Radiology �9 Springer-Verlag 1984

Difficulty in Diagnosing Hemobilia from a Hepatic Artery Aneurysm: Value of Endoscopic Retrograde Cholangiography

M a r k Wi lk inson , Michae l Michel l , G r a e m e Alexander , J o h n Ratcl iffe , Wi l l i am L a r k w o r t h y , and R o g e r Wi l l iams The Liver Unit and Department of Radiology, King's College Hospital, London, England; Liver Unit, Cromwell Hospital, London, England; and King Faisal Specialist Hospital, Riyadh, Saudi Arabia

Abstract. A case o f hemob i l i a due to a hepa t i c a r t e ry a n e u r y s m is descr ibed. Desp i te 2 a r te r io- g r ams a n d 2 l a p a r o t o m i e s , the cause o f the bleed- ing r e m a i n e d unde t ec t ed unti l a fu r the r selective c a n n u l a t i o n o f the celiac axis a r t e ry was per- fo rmed . E n d o s c o p i c r e t r o g r a d e c h o l a n g i o g r a p h y d e m o n s t r a t e d tha t p o s t o p e r a t i v e j a u n d i c e was n o t due to o b s t r u c t i o n a nd ou t l ined the a n e u r y s m wi th in a hepa t i c duct .

Key words: H e m o b i l i a - H e p a t i c a r t e ry a n e u r y s m - A r t e r i o g r a p h y - E n d o s c o p i c c h o l a n g i o g r a p h y .

H e m o b i l i a is ra re ly due to i n t r ahepa t i c a n e u r y s m , wh ich was ident i f ied as the cause in on ly 14 o f 355 cases in the w o r l d l i tera ture rev iewed by S a n d b l o m [1]. H e p a t i c a r t e ry aneu rysms , w h i c h m a y be myco t i c , syphili t ic, o r congeni ta l , a n d are also f o u n d in a s soc ia t ion wi th h y p e r t e n s i o n or po - lyar ter i t is n o d o s a , have m o s t f r equen t ly been de- scr ibed fo l lowing t r a u m a , wh ich was the m o s t like- ly cause in o u r pa t ien t . W h e n hemob i l i a has been sugges ted by the classic clinical t r iad o f a b d o m i n a l pain , j aund ice , a n d gas t ro in tes t ina l h e m o r r h a g e , the site a n d n a t u r e o f the unde r ly ing lesion can usua l ly be def ined by hepa t i c a r t e r i og raphy . I n the p resen t case, an a n a t o m i c v a r i a t i o n in the pa t i en t ' s ar ter ial supp ly was respons ib le for fai lure to iden- t ify the a n e u r y s m in the initial a r t e r i og ra ph i c se- ries. E n d o s c o p i c r e t r o g r a d e c h o l a n g i o g r a p h y was o f pa r t i cu l a r va lue in the final successful m a n a g e - m e n t o f this pa t ien t .

Address reprint requests to: Dr. Roger Williams, Liver Unit, King's College Hospital, London SE5, England

Case Report

A 36-year-old male Saudi ex-professional football player, now employed as a coach, who was previously well and had no recollection of any blow to the abdomen, was admitted to hos- pital in Saudi Arabia with a 5-day history of colicky right hypo- chondrial pain and melena. He was icteric, and upper gastroin- testinal endoscopy demonstrated a blood clot obscuring, and adherent to, the ampulla of Vater. Shortly afterwards he col- lapsed with hypovolemic shock. He underwent emergency lapa- rotomy with perioperative aortography and selective cannula- tion of the common hepatic artery, which arose from the superi- or mesenteric artery.

The films were interpreted, incorrectly, as demonstrating free blood flow from the common hepatic artery into the biliary tree. At operation the gallbladder and biliary tree were dis- tended with blood but the site of bleeding could not be identi- fied. The common hepatic artery was ligated distal to the origin of the cystic artery. A cholecystostomy and wedge biopsy of the liver were performed. The biopsy specimen showed red blood cells within the smallest biliary radicles (Fig. 1).

Postoperative recovery was initially uneventful with slow resolution of jaundice. After 3 weeks the patient had a further massive gastrointestinal hemorrhage. Further angiography was interpreted as demonstrating hypertrophy of the gastroduo- denal and cystic arteries. It was assumed that these arteries were supplying a vascular lesion within the liver. At a subse- quent laparotomy the 2 "hypertrophied" vessels were ligated and cholecystectomy was performed. Subsequently there was a further resolution of jaundice, but 3 weeks later a third mas- sive hemorrhage occurred.

The patient was then transferred to the Liver Unit at the Cromwell Hospital, London. On examination he was deeply jaundiced with multiple scratch marks. The liver edge was pal- pable 3 cm below the costal margin, and was firm, nontender, and without a bruit.

Results of laboratory tests included bilirubin 72 lamol/1, albumin 31 g/l, alkaline phosphatase (AP) < 100 IU/1, aspartate aminotransferase (AST) 124 IU/1 (normal < 45), gamma gluta- myl transpeptidase 229 IU/1 (normal < 50). Results of serologic test for syphilis were negative.

Arteriography with selective cannulation of both superior mesenteric and celiac axis arteries was carried out (Fig. 2) and demonstrated an anomalous left hepatic artery arising from the celiac axis. This artery supplied a small part of the left lobe of the liver and also filled a midline aneurysm 6 mm in

224 M. Wilkinson et al. : ERCP in Diagnosis of Hemobilia

Fig. 1. Liver biopsy specimen: Arrow shows a portal area with erythrocytes in a bile ductule (H&E x 320)

Fig. 2. Selective arteriography showing the left hepatic artery arising from the celiac axis. Arrow points to the midline aneurysm. Clips are from previous surgery

Fig. 3. Late phase of arteriogram (32 s). The aneurysm remains filled with contrast (arrow)

M. Wilkinson et al. : ERCP in Diagnosis of Hemobilia 225

Fig. 4. Endoscopic retrograde cholangiogram showing a smooth filling defect in the main left hepatic duct (arrow) corre- sponding in position to the left hepatic artery aneurysm

diameter. The aneurysm remained filled with contrast through- out the angiographic run of 32 s (Fig. 3). Selective catheteriza- tion of the celiac axis had not previously been performed and the anomalous left hepatic artery had, therefore, never been demonstrated. Selective cannulation of the anomalous artery was attempted with a view to embolization, but this was unsuc- cessful.

Shortly after this procedure the patient developed further colicky right hypochondrial pain with deepening jaundice. The serum bilirubin level rose to 242 gmol/1, serum AP to 1318 IU/1, and serum AST to 520 IU/1. Ultrasound appearances were in- terpreted as consistent with blood clot within a dilated common bile duct.

At laparotomy, performed at King's College Hospital, the biliary tree was again found to be distended with blood clot and the pancreas swollen. The anomalous left hepatic artery was identified, ligated, and transected. The serum bilirubin lev- el, however, did not fall postoperatively. It had remained at approximately 90 gmol/1 for 3 weeks, and ERCP was per- formed. This demonstrated a dilated common bile duct without clot or stricture, but with a smooth filling defect 9 mm in diame- ter. This partially occluded the main left hepatic duct, and cor- responded in position to the aneurysm demonstrated by arteri- ography (Fig. 4). It became clear from the structure of the bili- ary tree demonstrated during ERCP that the appearances sug- gestive of free blood flow from the hepatic artery to the biliary tree during the first hepatic angiogram were in fact due to extravasation of contrast around the hepatic artery.

Over the next few weeks the patient's jaundice gradually resolved and he remains weI1 and completely asymptomatic 12 months later.

Discussion

Although the possibility remains that the lesion in this particular patient was congenital, the mid- line position of the aneurysm in a professional ath- elete subject to injury suggests that it was probably traumatic and a consequence of a forgotten injury. Other recognized causes of hepatic aneurysm have been excluded in this patient. Despite a rapid clini- cal diagnosis, confirmed endoscopically, error re- sulted from misinterpretation of the initial angiog- raphy and failure to perform selective celiac axis angiography.

Woodburne [2] showed that the left hepatic ar- tery arises solely from the celiac axis in only 15% of cases, but the incidence of the complete hepatic arterial supply arising from the superior mesenteric artery, as was assumed in this patient, is even lower, 2.5%. The hypertrophy of the gastroduo- denal vessels observed at the second laparotomy was probably compensatory.

This is the first report of the outlining of an intrabiliary hepatic artery aneurysm by ERCP. However, in i previous instance reported by Carr- Locke and Westwood [3], ERCP identified a post- traumatic biliary stricture in association with an aneurysm that was separately identified by angiog- raphy.

In the present patient the aneurysm identified by ERCP and angiography was in the same posi- tion. The discrepancy between internal and exter- nal diameters can be explained by retained clot and/or fibrous tissue in the wall of the aneurysm rather than a true increase in size between the two examinations. The presence of fibrous tissue would explain persistence of the aneurysmal structure after successful surgery, although the remote possi- bility remains that a further anastomotic channel exists.

The prolonged period of jaundice following the third operation and definitive surgery is likely to have been due to retained clot within the smallest biliary radicles. This could have been combined with delayed hepatocyte recovery following pro- longed biliary obstruction and the cumulative stress of 3 laparotomies and anaesthetics within 2 months. Hepatic arterial blood flow was also likely to have been significantly reduced following extensive surgical ligation. Obstruction due to the aneurysm itself is unlikely to have been important, because of the aneurysm's relatively distal position in the left lobe.

226 M. Wilkinson et al. : ERCP in Diagnosis of Hemobilia

Acknowledgments. We are grateful to Dr. B. Portmann and Dr. U. Raeth for the histologic and ultrasound studies, respec- tively, to Mr. J.L. Dawson for performing the laparotomy, and to Dr. J.W. Laws for invaluable advice. Dr Wilkinson is the Smith, Kline and French Research Fellow of the British Society of Gastroenterology.

References

1. Sandblom PH: Hemobilia (Biliary Tract Haemorrhage): History, Pathology, Diagnosis, Treatment. Springfield: Charles C. Thomas, 1972

2. Woodburne RT: Segmental anatomy of the liver: blood sup- ply and collateral circulation. U Mich Med Bull 28:189-199, 1962

3. Carr-Locke DL, Westwood CA: Endoscopy and endoscopic retrograde cholangiopancreatography findings in traumatic liver injury and hemobilia. Am J Gastroenterol 73:162-164, 1980

Received: September 1, 1983; accepted." October 4, 1983