Gastrointest Radiol 2, 71
Gastrointestinal
74 (1977)
Radiology 9 by Springer-Verlag 1977
Hemobilia: Calcified Hepatic Artery Aneurysm Presenting with Massive Gastrointestinal Bleeding Emil J. Balthazar Department of Radiology, New York Medical College, New York, New York, USA
Abstract. A case of a calcified and ruptured hepatic artery aneurysm presenting with hemobilia and massive gastrointestinal bleeding is reported and illustrated. The clinical diagnosis should be entertained whenever the triad of findings (abdominal colic, gastrointestinal bleeding, elevated bilirubin levels) is present. Angiography is the method of choice in confirming this diagnosis. A short review of the clinical and radiographic syndrome of hemobilia with emphasis on the occurence, significance, and prognosis of hepatic artery aneurysms is presented. Key words: Abdomen, calcification - Aneurysm Arteries, hepatic - Liver, angiography - Liver, diseases.
Hemobilia is the term introduced in 1948 by Sandblom to define the presence of blood in the biliary tract [1]. It is the result of a communication between a blood vessel (usually an artery) and the biliary tree and generally presents as a puzzling case of massive gastrointestinal hemorrhage. The syndrome was popularized b'y Sandblom with the publication of several communications but especially by the extensive review of the world literature analyzed in his excellent monograph [2]. The etiologic factors responsible for the development of hemobilia underwent continuous changes during the years. The earliest report is attributed to Glisson (1654) and liver abscesses were the most common entities incriminated [2]. In the 20th century, ruptured hepatic artery aneurysm became more common [2, 3] and finally in the last 15 years traumatic hemobilia surpassed all other etiologic agents in frequency. The change is due primarily to the increasing number of cases reported as complicaAddress reprint requests to." Emil J. Balthazar, M.D., Associate Professor, Department of Radiology, New York Medical College, 5th Avenue and 106th Street, New York, N Y 10029, USA
tions of c o m m o n diagnostic procedures such as liver biopsies performed in increasing numbers throughout the country [4 7]. From the total number of 355 documented cases described until 1972, 55% were produced by different types of trauma followed by cholelithiasis, inflammations (including parasites), vascular abnormalities, and tumors. Liver parenchyma was the site of origin of the majority of the cases ofhemobilia, with the biliary ducts, gallbladder, and occasionally the pancreas constituting the rest of the cases [2]. Many reported cases and review articles [8, 9] stress the fact that, consistently, the correct diagnosis of hemobilia in cases of massive gastrointestinal bleeding has been unusually difficult. Lack of awareness of this syndrome has led to misses or significant delays in diagnosis, to unnecessary and repeated laparotomies, to futile gastrectomies, and to high mortality rates [2, 8, 9]. To emphasize some of the clinical and radiographic aspects of spontaneous hemobilia, a proven case secondary to a ruptured hepatic artery aneurysm, is reported and illustrated.
Case Report A 74-year-old white female was admitted to the hospital because of severe dizziness, chest pain, and several tarry stools. She gave a history of increasing weakness and diarrheic black stools preceded by severe pain in the mid-epigastric area. Over the past year the patient had five similar episodes and four previous admissions to different hospitals. Repeated work-ups for upper gastrointestinal bleeding, including seven previous barium examinations, and several endoscopies were consistently negative. She was treated medically, receiving transfusions, and was discharged each time in good condition but without a definite diagnosis. There was in addition, a history of chronic lung disease and of chronic congestive heart failure for which she received intermittent treatment. The hemoglobin was 5.4 g% with previous values between 5 and 7 g%. Red blood cell count fluctuated at different admissions between 2 3,500,000/ram 3. The rest of the laboratory findings were unre-
72
E.J. Balthazar: Hemobilia: Hepatic Artery Aneurysm markable. Total bilirubin levels varied at different times between 0.6 mg% and 1.3 mg%. The patient never developed clinical jaundice, abdominal tenderness, or a palpable abdominal mass. Radiographic examination of the abdomen revealed a cystic calcified mass measuring about 4 x 4 cm in the region of the right lobe of the liver (Fig. 1). Liver scan confirmed the presence of a space occupying lesion in the liver. An oral gallbladder study showed an oval large filling defect in a poorly opacified gallbladder, thought to be a calculus (Fig. 2). A celiac angiogram demonstrated a large aneurysm of the right hepatic artery within the liver parenchyma (Fig. 3). The patient was not bleeding at the time of the examination and the communication with the biliary system was not demonstrated. At surgery, the aneurysm was found to be atheromatous in origin, ruptured, and communicating with a large cavity filled with blood and with the biliary system. Exploration of the main biliary ducts showed intraluminal blood and the removed gallbladder contained a large solitary blood clot. A segmental ligation of the hepatic artery was performed. Post-operatively the patient developed pulmonary and cardiac complications and expired a few days later.
Comment Fig. 1. Abdominal supine film, close-up view of the right upper quadrant. Cystic mass with calcified rim is visualized in the right lobe of the liver (arrows)
Hepatic artery aneurysms can be classified as traumatic and non-traumatic in origin. The traumatic variety (false aneurysm) may be secondary to blunt ab-
Fig. 2. Oral gallbladder examination. Faint opacification of the gallbladder with large oval central lucency. At surgery the gallbladder contained a large blood clot Fig. 3. Celiac angiogram. Arterial phase demonstrates a large aneurysm of the right hepatic artery situated in the right lobe of the liver
E.J. Balthazar: Hemobilia: Hepatic Artery A n e u r y s m
dominal injury, previous surgical procedures or liver biopsies and may present clinically with a dormant period lasting up to two years [2, 3, 10, 11]. The great majority of the non-traumatic aneurysms are either inflammatory (mycotic) or arteriosclerotic in origin [2, 10, 12, 13] and only this latter variety presents occasionally with discrete, cyst-like calcifications in the right upper quadrant [12, 14, 15]. About 75% of hepatic artery aneurysms are extrahepatic with the remainder in an intrahepatic location [10, 12, 16]. There is general agreement about the sudden life-threatening potential of otherwise silent hepatic artery aneurysms. The incidence of rupture has been reported to be around 5-10% [12]; however, if not treated, up to 80% of hepatic artery aneurysms will eventually rupture and bleed profusely [14]. Depending upon their location and site of rupture they may bleed into the peritoneal cavity, stomach, duodenum, or biliary tract. Among the 103 cases of ruptured hepatic artery aneurysms reviewed by Guida et al. [i7], 53 communicated with the biliary tract. Although calcification of a hepatic artery aneurysm has been considered a sign that it is probably less likely to rupture, aneurysms that are larger than 3 cm in diameter or enlarging on follow-up examinations constitute definite indications for elective surgery [12]. Rupture of a hepatic artery aneurysm leading to hemobilia and presenting as massive gastrointestinal hemorrhage is a known medical entity [16 18]. The clinical syndrome is characterized by the presence of upper abdominal pain associated with gastrointestinal bleeding and jaundice. The abdominal pain is colicky in nature, localized mainly to the right upper quadrant, and distinctively subsiding or disappearing at the beginning of the hemorrhage. The gastrointestinal bleeding may be serious, leading to shock and may present as massive melena as well as hematemesis [16-18[. Clinical jaundice is not common. Most of the cases have shown temporary elevation of the serum bilirubin levels although even this finding was not present in this case report. In addition what characterizes the syndrome is the transitory episodic nature of these symptoms with silent periods of days, weeks, or sometimes months between the attacks. The association of the above triad, particularly recurrent in nature, should be considered highly suspicious for hemobilia and proper studies should be ordered in an attempt to confirm this diagnosis. A variety of diagnostic procedures has been reported as helpful in securing this diagnosis [2, 10]. Isotopic scans and intravenous or oral cholangiography may document a liver mass as well as blood
73
clots in the biliary tract and gallbladder (Fig. 2). Duodenoscopy may actually visualize the bleeding site during an episode of active bleeding and E.R.C.P. examinations may help in further delineating the pathologic site. The method of choice however, in confirming the diagnosis of bleeding hepatic artery aneurysm is angiography [2, 12, 13, 19, 20, 21]. Celiac arteriography has been effectively used in demonstrating the nature and the exact location of the pathologic process as well as, sometimes, the actual communication with the biliary system. It represents the only means of arriving at a prompt and definite preoperative diagnosis and is invaluable in planning for the surgical treatment. Angiographic examinations should be performed without unnecessary delays in every case in which the clinical diagnosis of hemobilia is suspected.
References 1. Sandblom P: Hermorrhage into the biliary tract following trauma. Traumatic hemobilia. Surgery 24." 571 586, 1948 2. Sandblom P: Hemobilia. pp 5-117. Springfield: T h o m a s 1972 3. Schatzki SC: Hemobilia. Radiology 77:717 721, 1961 4. Cox EF: Hemobilia following percutaneous needle biopsy of the liver. Arch Surg 94." 198 201, 1967 5. Levinson JD, Olsen G, Terman JW, Cleaveland CR, G r a h a m CP, Breen K J: Hemobilia secondary to percutaneous liver biopsy. Arch Intern Med 130: 3 9 6 4 0 0 , 1972 6. Seltzer RA, Rossiter SB, C o o p e r m a n LR, Liebowitz D : Hemobilia following needle biopsy of the liver. Am J Roentgenol Radium Ther Nucl Med 127:1035 1036, 1976 7. Attiyeh FF, McSweeney J, Fortner JG: Hemobilia complicating needle liver biopsy; Treated with hepatic artery ligation: A case report with arteriographic demonstration. Radiology 118." 559 560, 1976 8. Sedgwick CE, Coburn RJ : Evaluation of the patient with hemobilia. Surg. Clin North Am 50:683 690, 1970 9. Bismuth H : Hemobilia. Editorial - N Engl J Med 288 : 617 619, 1973 10. Berenson M M , Freston JW: Intrahepatic artery aneurysm associated with hemobilia. Gastroenterology 66." 254-259, 1974 11. Wilkinson G M , Middelsen WP, Berne CJ: The treatment of posttraumatic hemobilia by ligature of the c o m m o n hepatic artery. Surg Clin North Am 48." 1337 1346, 1968 12. Koehler RP, Nelson JA, Berenson M M : Massive extra-enteric gastrointestinal bleeding: Angiographic diagnosis. Radiology 119." 4 1 4 4 , 1976 13. Hughes JH, G u z m a n JG, Roberts SS: Massive hemobilia from ruptured hepatic artery aneurysm. J A M A 214. 913 914, 1970 14. Jarvis L, Hodes PJ: A n e u r y s m of hepatic artery demonstrated roentgenographically. Am J Roentgenol Radium Ther Nucl Med 72." 1037 1040, 1954 15. Quinn JL, Martin JF: Hepatic artery aneurysm. Case report. Am J Roentgenol Radium Ther Nucl Med 87." 284 286, 1962 16. Tetreault AF, Bowen R J: Hemobilia secondary to intrahepatic aneurysm of the hepatic artery. J A M A 192: 154-156, 1965
74 17. Guida PM, Moore SW: A n e u r y s m of the hepatic artery. Report of 5 cases with a brief review of the previously reported cases. Surgery 60 : 299 310, 1966 18. Mackay A G , Page G H : Hematemesis associated with hemobilia. Report of a case due to an intrahepatic artery aneurysm, with survival. N Engl J M e d 260: 4 6 8 4 7 1 , 1959 19. D o p p m a n J, Shapiro R, Conte M: A n e u r y s m s of the hepatic artery. The importance of angiographic visualization. Am J Roentgenol Radium Ther Nucl M e d 90." 578-582, 1963
E.J. Balthazar: Hemobilia: Hepatic Artery Aneurysm 20. Boijsen E, Gothlin J, Hallbook T, Sandblom P: Preoperative angiographic diagnosis of bleeding aneurysms of abdominal visceral arteries. Radiology 93." 781 791, 1969 21. Steinberg 1: Diagnosis of aneurysm of hepatic and splenic arteries by intravenous abdominal aortography. N Engl J Med 263: 341-343, 1960
Receit'ed: M a y 3, 1977, accepted. May 16, 1977
Gastrointestinal
74 (1977)
Radiology 9 by Springer-Verlag 1977
Hemobilia: Calcified Hepatic Artery Aneurysm Presenting with Massive Gastrointestinal Bleeding Emil J. Balthazar Department of Radiology, New York Medical College, New York, New York, USA
Abstract. A case of a calcified and ruptured hepatic artery aneurysm presenting with hemobilia and massive gastrointestinal bleeding is reported and illustrated. The clinical diagnosis should be entertained whenever the triad of findings (abdominal colic, gastrointestinal bleeding, elevated bilirubin levels) is present. Angiography is the method of choice in confirming this diagnosis. A short review of the clinical and radiographic syndrome of hemobilia with emphasis on the occurence, significance, and prognosis of hepatic artery aneurysms is presented. Key words: Abdomen, calcification - Aneurysm Arteries, hepatic - Liver, angiography - Liver, diseases.
Hemobilia is the term introduced in 1948 by Sandblom to define the presence of blood in the biliary tract [1]. It is the result of a communication between a blood vessel (usually an artery) and the biliary tree and generally presents as a puzzling case of massive gastrointestinal hemorrhage. The syndrome was popularized b'y Sandblom with the publication of several communications but especially by the extensive review of the world literature analyzed in his excellent monograph [2]. The etiologic factors responsible for the development of hemobilia underwent continuous changes during the years. The earliest report is attributed to Glisson (1654) and liver abscesses were the most common entities incriminated [2]. In the 20th century, ruptured hepatic artery aneurysm became more common [2, 3] and finally in the last 15 years traumatic hemobilia surpassed all other etiologic agents in frequency. The change is due primarily to the increasing number of cases reported as complicaAddress reprint requests to." Emil J. Balthazar, M.D., Associate Professor, Department of Radiology, New York Medical College, 5th Avenue and 106th Street, New York, N Y 10029, USA
tions of c o m m o n diagnostic procedures such as liver biopsies performed in increasing numbers throughout the country [4 7]. From the total number of 355 documented cases described until 1972, 55% were produced by different types of trauma followed by cholelithiasis, inflammations (including parasites), vascular abnormalities, and tumors. Liver parenchyma was the site of origin of the majority of the cases ofhemobilia, with the biliary ducts, gallbladder, and occasionally the pancreas constituting the rest of the cases [2]. Many reported cases and review articles [8, 9] stress the fact that, consistently, the correct diagnosis of hemobilia in cases of massive gastrointestinal bleeding has been unusually difficult. Lack of awareness of this syndrome has led to misses or significant delays in diagnosis, to unnecessary and repeated laparotomies, to futile gastrectomies, and to high mortality rates [2, 8, 9]. To emphasize some of the clinical and radiographic aspects of spontaneous hemobilia, a proven case secondary to a ruptured hepatic artery aneurysm, is reported and illustrated.
Case Report A 74-year-old white female was admitted to the hospital because of severe dizziness, chest pain, and several tarry stools. She gave a history of increasing weakness and diarrheic black stools preceded by severe pain in the mid-epigastric area. Over the past year the patient had five similar episodes and four previous admissions to different hospitals. Repeated work-ups for upper gastrointestinal bleeding, including seven previous barium examinations, and several endoscopies were consistently negative. She was treated medically, receiving transfusions, and was discharged each time in good condition but without a definite diagnosis. There was in addition, a history of chronic lung disease and of chronic congestive heart failure for which she received intermittent treatment. The hemoglobin was 5.4 g% with previous values between 5 and 7 g%. Red blood cell count fluctuated at different admissions between 2 3,500,000/ram 3. The rest of the laboratory findings were unre-
72
E.J. Balthazar: Hemobilia: Hepatic Artery Aneurysm markable. Total bilirubin levels varied at different times between 0.6 mg% and 1.3 mg%. The patient never developed clinical jaundice, abdominal tenderness, or a palpable abdominal mass. Radiographic examination of the abdomen revealed a cystic calcified mass measuring about 4 x 4 cm in the region of the right lobe of the liver (Fig. 1). Liver scan confirmed the presence of a space occupying lesion in the liver. An oral gallbladder study showed an oval large filling defect in a poorly opacified gallbladder, thought to be a calculus (Fig. 2). A celiac angiogram demonstrated a large aneurysm of the right hepatic artery within the liver parenchyma (Fig. 3). The patient was not bleeding at the time of the examination and the communication with the biliary system was not demonstrated. At surgery, the aneurysm was found to be atheromatous in origin, ruptured, and communicating with a large cavity filled with blood and with the biliary system. Exploration of the main biliary ducts showed intraluminal blood and the removed gallbladder contained a large solitary blood clot. A segmental ligation of the hepatic artery was performed. Post-operatively the patient developed pulmonary and cardiac complications and expired a few days later.
Comment Fig. 1. Abdominal supine film, close-up view of the right upper quadrant. Cystic mass with calcified rim is visualized in the right lobe of the liver (arrows)
Hepatic artery aneurysms can be classified as traumatic and non-traumatic in origin. The traumatic variety (false aneurysm) may be secondary to blunt ab-
Fig. 2. Oral gallbladder examination. Faint opacification of the gallbladder with large oval central lucency. At surgery the gallbladder contained a large blood clot Fig. 3. Celiac angiogram. Arterial phase demonstrates a large aneurysm of the right hepatic artery situated in the right lobe of the liver
E.J. Balthazar: Hemobilia: Hepatic Artery A n e u r y s m
dominal injury, previous surgical procedures or liver biopsies and may present clinically with a dormant period lasting up to two years [2, 3, 10, 11]. The great majority of the non-traumatic aneurysms are either inflammatory (mycotic) or arteriosclerotic in origin [2, 10, 12, 13] and only this latter variety presents occasionally with discrete, cyst-like calcifications in the right upper quadrant [12, 14, 15]. About 75% of hepatic artery aneurysms are extrahepatic with the remainder in an intrahepatic location [10, 12, 16]. There is general agreement about the sudden life-threatening potential of otherwise silent hepatic artery aneurysms. The incidence of rupture has been reported to be around 5-10% [12]; however, if not treated, up to 80% of hepatic artery aneurysms will eventually rupture and bleed profusely [14]. Depending upon their location and site of rupture they may bleed into the peritoneal cavity, stomach, duodenum, or biliary tract. Among the 103 cases of ruptured hepatic artery aneurysms reviewed by Guida et al. [i7], 53 communicated with the biliary tract. Although calcification of a hepatic artery aneurysm has been considered a sign that it is probably less likely to rupture, aneurysms that are larger than 3 cm in diameter or enlarging on follow-up examinations constitute definite indications for elective surgery [12]. Rupture of a hepatic artery aneurysm leading to hemobilia and presenting as massive gastrointestinal hemorrhage is a known medical entity [16 18]. The clinical syndrome is characterized by the presence of upper abdominal pain associated with gastrointestinal bleeding and jaundice. The abdominal pain is colicky in nature, localized mainly to the right upper quadrant, and distinctively subsiding or disappearing at the beginning of the hemorrhage. The gastrointestinal bleeding may be serious, leading to shock and may present as massive melena as well as hematemesis [16-18[. Clinical jaundice is not common. Most of the cases have shown temporary elevation of the serum bilirubin levels although even this finding was not present in this case report. In addition what characterizes the syndrome is the transitory episodic nature of these symptoms with silent periods of days, weeks, or sometimes months between the attacks. The association of the above triad, particularly recurrent in nature, should be considered highly suspicious for hemobilia and proper studies should be ordered in an attempt to confirm this diagnosis. A variety of diagnostic procedures has been reported as helpful in securing this diagnosis [2, 10]. Isotopic scans and intravenous or oral cholangiography may document a liver mass as well as blood
73
clots in the biliary tract and gallbladder (Fig. 2). Duodenoscopy may actually visualize the bleeding site during an episode of active bleeding and E.R.C.P. examinations may help in further delineating the pathologic site. The method of choice however, in confirming the diagnosis of bleeding hepatic artery aneurysm is angiography [2, 12, 13, 19, 20, 21]. Celiac arteriography has been effectively used in demonstrating the nature and the exact location of the pathologic process as well as, sometimes, the actual communication with the biliary system. It represents the only means of arriving at a prompt and definite preoperative diagnosis and is invaluable in planning for the surgical treatment. Angiographic examinations should be performed without unnecessary delays in every case in which the clinical diagnosis of hemobilia is suspected.
References 1. Sandblom P: Hermorrhage into the biliary tract following trauma. Traumatic hemobilia. Surgery 24." 571 586, 1948 2. Sandblom P: Hemobilia. pp 5-117. Springfield: T h o m a s 1972 3. Schatzki SC: Hemobilia. Radiology 77:717 721, 1961 4. Cox EF: Hemobilia following percutaneous needle biopsy of the liver. Arch Surg 94." 198 201, 1967 5. Levinson JD, Olsen G, Terman JW, Cleaveland CR, G r a h a m CP, Breen K J: Hemobilia secondary to percutaneous liver biopsy. Arch Intern Med 130: 3 9 6 4 0 0 , 1972 6. Seltzer RA, Rossiter SB, C o o p e r m a n LR, Liebowitz D : Hemobilia following needle biopsy of the liver. Am J Roentgenol Radium Ther Nucl Med 127:1035 1036, 1976 7. Attiyeh FF, McSweeney J, Fortner JG: Hemobilia complicating needle liver biopsy; Treated with hepatic artery ligation: A case report with arteriographic demonstration. Radiology 118." 559 560, 1976 8. Sedgwick CE, Coburn RJ : Evaluation of the patient with hemobilia. Surg. Clin North Am 50:683 690, 1970 9. Bismuth H : Hemobilia. Editorial - N Engl J Med 288 : 617 619, 1973 10. Berenson M M , Freston JW: Intrahepatic artery aneurysm associated with hemobilia. Gastroenterology 66." 254-259, 1974 11. Wilkinson G M , Middelsen WP, Berne CJ: The treatment of posttraumatic hemobilia by ligature of the c o m m o n hepatic artery. Surg Clin North Am 48." 1337 1346, 1968 12. Koehler RP, Nelson JA, Berenson M M : Massive extra-enteric gastrointestinal bleeding: Angiographic diagnosis. Radiology 119." 4 1 4 4 , 1976 13. Hughes JH, G u z m a n JG, Roberts SS: Massive hemobilia from ruptured hepatic artery aneurysm. J A M A 214. 913 914, 1970 14. Jarvis L, Hodes PJ: A n e u r y s m of hepatic artery demonstrated roentgenographically. Am J Roentgenol Radium Ther Nucl Med 72." 1037 1040, 1954 15. Quinn JL, Martin JF: Hepatic artery aneurysm. Case report. Am J Roentgenol Radium Ther Nucl Med 87." 284 286, 1962 16. Tetreault AF, Bowen R J: Hemobilia secondary to intrahepatic aneurysm of the hepatic artery. J A M A 192: 154-156, 1965
74 17. Guida PM, Moore SW: A n e u r y s m of the hepatic artery. Report of 5 cases with a brief review of the previously reported cases. Surgery 60 : 299 310, 1966 18. Mackay A G , Page G H : Hematemesis associated with hemobilia. Report of a case due to an intrahepatic artery aneurysm, with survival. N Engl J M e d 260: 4 6 8 4 7 1 , 1959 19. D o p p m a n J, Shapiro R, Conte M: A n e u r y s m s of the hepatic artery. The importance of angiographic visualization. Am J Roentgenol Radium Ther Nucl M e d 90." 578-582, 1963
E.J. Balthazar: Hemobilia: Hepatic Artery Aneurysm 20. Boijsen E, Gothlin J, Hallbook T, Sandblom P: Preoperative angiographic diagnosis of bleeding aneurysms of abdominal visceral arteries. Radiology 93." 781 791, 1969 21. Steinberg 1: Diagnosis of aneurysm of hepatic and splenic arteries by intravenous abdominal aortography. N Engl J Med 263: 341-343, 1960
Receit'ed: M a y 3, 1977, accepted. May 16, 1977
