Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Small-Bowel Metastasis from Bile Duct Carcinoma with Coincidental Congenital Absence of Gallbladder Nelson Gurll To cite this article: Nelson Gurll (1976) Small-Bowel Metastasis from Bile Duct Carcinoma with Coincidental Congenital Absence of Gallbladder, Postgraduate Medicine, 59:3, 229-231, DOI: 10.1080/00325481.1976.11714313 To link to this article: http://dx.doi.org/10.1080/00325481.1976.11714313
Published online: 07 Jul 2016.
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Date: 21 August 2017, At: 07:40
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• A 78-year-old woman was admitted to Beth Israel Hospital, Boston, with a six-week history of crampy, nonradiating, infraumbilical pain, which was occasionally accompanied by abdominal fullness, gurgling, and borborygmi. She had had three episodes of nausea and vomiting unassociated with the pain. Bowel movements had become semiformed, watery, and light-colored, and her weight had dropped from 114 to 90 lb. She had not had abdominal surgery. An abdominal x-ray film showed severa! loops of dilated small bowel, and cholecystography failed to visualize the gallbladder on single and repeat oral administration of contrast medium. The patient was referred for biliary tract surgery, with a diagnosis of chronic cholecystitis and cholelithiasis. On physical examination, the abdomen was distended and peristaltic waves moved visibly from the left to the right hypochondrium. Rushes of laud, high-pitched bowel sounds were heard. Palpation elicited tenderness in the right lower quadrant, but no masses were felt. Rectal examination showed no masses or tenderness, but a guaiac test of a stool specimen gave a 4+ reaction for occult blood. The hematocrit reading, urinalysis results, prothrombin time, and levels of electrolytes, creatinine, blood urea nitrogen, blood sugar, bilirubin, serum glutamic oxalacetic transaminase, serum glutamic pyruvic transaminase, and lactic dehydrogenase were ali within normal limits. The alkaline phosphatase leve! was elevated to 28.3 KingArmstrong units (normal3 to Il units) and the albumin-globulin ratio was 3.0: 1. 9. The clinical diagnosis was small-bowel obstruction. A barium follow-through upper gastrointestinal examination showed dilated small intestine from the jejunum to the midileum, at which point were two lesions-an "apple-
Vol. 59 • No. 3 • March 1976 • POSTGRADUATE MEDICINE
case report SMALL-BOWEL METASTASIS FROM BILE DUCT CARCINOMA WITH COINCIDENTAL CONGENITAL ABSENCE OF GALLBLADDER Nelson Gurli, MD Beth Israel Hospital Boston
core" constnct10n (figure) and a nearby short, eccentric narrowing. After receiving an elemental diet for severa! days, the patient underwent laparotomy, and tumor nodules were found in the omentum and posterior surface of the liver. At sites defined by the radiologist, tumor nodules not involving mucosa were found in the smallbowel wall. No gallbladder or cys tic duct was found. The porta hepatis contained large lymph nades that were thought ta correspond ta calcified lymph nades seen on the abdominal films. Biopsies of the omental nodules and microscopie sections of the small-bowel lesions showed adenocarcinoma. The involved section of sm ali bowel was resected; construction of a closed end-to-end ileoileostomy was complicated by a spill of intestinal contents from the obstructed proximal small bowel. Five days after the operation, the patient became drowsy, confused, and diaphorectic. By the eight postoperative day, her temperature had reached 101.4 F. Two days later, despite treatment with penicillin and
229
Downloaded by [Australian Catholic University] at 07:41 21 August 2017
Follow-through small-bowel barium study showing "apple-core" constriction in ileum.
chloramphenicol, an intra-abdominal abscess developed. After sorne initial improvement following drainage of the abscess, the patient became febrile and obtunded. Her condition gradually worsened, and she died on the 17th postoperative day. Autopsy confirmed the absence of the gallbladder, cystic duct, and gallbladder fossa. An adenocarcinoma involving the right hepatic and common bile ducts had metastasized to lymph nades in the porta hepatis. The bile ducts were not dilated and did not contain stones. A disruption of the ileoileostomy was surrounded by an abscess cavity. Discussion
Anatomie variations in the biliary tract are fairly common, but true congenital absence of the gallbladder and cystic duct is quite rare. It has been reported in only 0.03% to 0.07% of autopsies 1 - 3 and 0.04% ofbiliary tract operations. 4 ' 5 Biliary tract cancer, however, is found in 0.1% to 0.46% of autopsies and in 0.3% to 1.8% of biliary tract operations. 6 - 8
230
The coexistence of congenital absence of the gallbladder and cancer of the biliary tract, as was found in this patient, has been reported in only two others. Y.lo This very low incidence of coexistence of these two conditions, although possibly due to the rarity of agenesis of the gallbladder, tends to ne gate a causeand-effect relationship. Because gallstones have been found in 20% to 57% of ali cases of bile duct cancer, they have been implicated in its etiola gy. 7 • 8 Common duct abnormalities, such as stones, dilatation, or bath, exist in about half of ali patients with agenesis of the gallbladder. 11 - 14 Yet the common duct was normal in this patient and in the two reported elsewhere. Thus, if the presence of gallstones is a criterion, it would seem that an etiologie link between the cancer and the agenesis is nonexistent. The failure of cholecystography to visualize the gallbladder was obviously misleading. The diagnosis of congenitally absent gallbladder is difficult and is seldom made preoperatively. Documentation of the disorder at operation requires cholangiography, and often the absence cannat be substantiated until autopsy. Symptoms, when present, are those ofbiliary tract disease, with episodes of epigastric pain, nausea, vomiting, flatulence, and jaundice. More than half of symptomatic patients have common duct calculi, dilatation, or bath to explain symptoms. 11 - 14 Yet many patients have no abnormality except the absence of the gallbladder and cystic duct. In the three patients with this abnormality and bile duct cancer, the symptoms may have been due to the cancer. Certainly in the case described here, the symptoms were due to mechanical sm ali- bowel obstruction-the preoperati ve diagnosis. Metastasis to intra-abdominal viscera from bile duct cancer has been reported. 6 Thorbjarnarson found small-bowel metastasis from bile duct carcinoma in 1 of 31 cases. 15 Extensive reviews indicate the relative infrequency of small-bowel metastasis as an isolated event. 16 - 18 Moreover, the association of small-bowel metastasis with the pathologie
POSTGRADUATE MEDIC.. E o March 1976 o Vol. 59 o No. 3
conditions discussed here makes this case quite singular. • Dr. Gurli was formerly in the department of surgery, Beth Israel Hospital, Boston. He is now chief, department of surgical gastroenterology, division of surgery, Walter Reed Army Institute of Research, Washington, DC 20012.
Downloaded by [Australian Catholic University] at 07:41 21 August 2017
References 1. Talmadge GK: Congenital absence of the gallbladder. Arch Pathol 26:1060-1062, 1938 2. Monzas G, Wilson AK: Congenital absence of gallbladder with stone in common bile duct. Lance! 1:628-629, 1953 3. Mclllrath DC, Remine WH, Baggenstoss AH: Congenital absence of the gallbladder and cystic duct: Report of ten cases found at necropsy. JAMA 180:782-783, 1962 4. Malmstrom L: Congenital absence of gallbladder: Report of a case diagnosed at operative cholangiography. Acta Chir Scand 105:440-447, 1953 5. Lindskog BI: Congenital absence of the gallbladder. Acta Chir Scand 139:208-209, 1973 6. Sako K, Seitzinger GL, Garside E: Carcinoma of the extrahepatic bile ducts: Review of the literature and report of six cases. Surgery 41:416-437, 1957 7. Neibling HA, Dockerty MB, Waugh JM: Carcinoma of extrahepatic bile duels. Surg Gynecol Obstet 89:429-438, 1949 8. Stewart HL, Lie ber MM, Morgan DR: Carcinoma of the extrahepatic bile duels. Arch Surg 41:662-713, 1940 9. Robertson HF, Robertson WE, Bower JO: Congenital absence of the gallbladder with primary carcinoma of the common duct and carcinoma of the liver. JAMA 114:1514-1517, 1940 10. Richards RN: Congenital absence of the gallbladder and cystic duc.t associated with primary carcinoma of the common bile duct. Can Med Assac J 94:859-860, 1966 Il. Dixon CF, Lichtman AL: Congenital absence of the gallbladder. Surgery 17:11-21, 1945 12. Carnevali JF, Kunath CA: Congenital absence of gallbladder. Arch Surg 78:440-445, 1959 13. Gerwig WH Jr, Countryman LK, Gomez AC: Congenital absence of the gallbladder and cystic duct: Report of six cases. Ann Surg 153:113-125, 1961 14. Rogers Al, Crews RD, Kaiser MH: Congenital absence of the gallbladder with choledocholithiasis. Gastroenterology 48:524-529, 1965 15. Thorbjarnarson B: Carcinoma of the bile ducts. Cancer 12:708-713, 1959 16. deCastro CA, Dockerty MB, Maya CW: Metastatic tumors of the small intestines-. Surg Gynecol Obstet 105:159-165, 1957 17. Farmer RG, Hawk WA: Metastatic tumors of the small bowel. Gastroenterology 47:496-504, 1964 18. Marshak RH, Khilnani MT, Eliasoph J, et al: Metastatic carcinoma of the small bowel. Am J Roentgenol Radium Ther Nucl Med 94:385-394, 1965
POSTGRADUATE MEDICINE invites submission of brief case reports for earl y publication. Illustrations and references should be included only when essential.
Vol. 59 • No. 3 • March 1976 • POSTQAADUATE MEDICINE
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Small-Bowel Metastasis from Bile Duct Carcinoma with Coincidental Congenital Absence of Gallbladder Nelson Gurll To cite this article: Nelson Gurll (1976) Small-Bowel Metastasis from Bile Duct Carcinoma with Coincidental Congenital Absence of Gallbladder, Postgraduate Medicine, 59:3, 229-231, DOI: 10.1080/00325481.1976.11714313 To link to this article: http://dx.doi.org/10.1080/00325481.1976.11714313
Published online: 07 Jul 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [Australian Catholic University]
Date: 21 August 2017, At: 07:40
Downloaded by [Australian Catholic University] at 07:41 21 August 2017
• A 78-year-old woman was admitted to Beth Israel Hospital, Boston, with a six-week history of crampy, nonradiating, infraumbilical pain, which was occasionally accompanied by abdominal fullness, gurgling, and borborygmi. She had had three episodes of nausea and vomiting unassociated with the pain. Bowel movements had become semiformed, watery, and light-colored, and her weight had dropped from 114 to 90 lb. She had not had abdominal surgery. An abdominal x-ray film showed severa! loops of dilated small bowel, and cholecystography failed to visualize the gallbladder on single and repeat oral administration of contrast medium. The patient was referred for biliary tract surgery, with a diagnosis of chronic cholecystitis and cholelithiasis. On physical examination, the abdomen was distended and peristaltic waves moved visibly from the left to the right hypochondrium. Rushes of laud, high-pitched bowel sounds were heard. Palpation elicited tenderness in the right lower quadrant, but no masses were felt. Rectal examination showed no masses or tenderness, but a guaiac test of a stool specimen gave a 4+ reaction for occult blood. The hematocrit reading, urinalysis results, prothrombin time, and levels of electrolytes, creatinine, blood urea nitrogen, blood sugar, bilirubin, serum glutamic oxalacetic transaminase, serum glutamic pyruvic transaminase, and lactic dehydrogenase were ali within normal limits. The alkaline phosphatase leve! was elevated to 28.3 KingArmstrong units (normal3 to Il units) and the albumin-globulin ratio was 3.0: 1. 9. The clinical diagnosis was small-bowel obstruction. A barium follow-through upper gastrointestinal examination showed dilated small intestine from the jejunum to the midileum, at which point were two lesions-an "apple-
Vol. 59 • No. 3 • March 1976 • POSTGRADUATE MEDICINE
case report SMALL-BOWEL METASTASIS FROM BILE DUCT CARCINOMA WITH COINCIDENTAL CONGENITAL ABSENCE OF GALLBLADDER Nelson Gurli, MD Beth Israel Hospital Boston
core" constnct10n (figure) and a nearby short, eccentric narrowing. After receiving an elemental diet for severa! days, the patient underwent laparotomy, and tumor nodules were found in the omentum and posterior surface of the liver. At sites defined by the radiologist, tumor nodules not involving mucosa were found in the smallbowel wall. No gallbladder or cys tic duct was found. The porta hepatis contained large lymph nades that were thought ta correspond ta calcified lymph nades seen on the abdominal films. Biopsies of the omental nodules and microscopie sections of the small-bowel lesions showed adenocarcinoma. The involved section of sm ali bowel was resected; construction of a closed end-to-end ileoileostomy was complicated by a spill of intestinal contents from the obstructed proximal small bowel. Five days after the operation, the patient became drowsy, confused, and diaphorectic. By the eight postoperative day, her temperature had reached 101.4 F. Two days later, despite treatment with penicillin and
229
Downloaded by [Australian Catholic University] at 07:41 21 August 2017
Follow-through small-bowel barium study showing "apple-core" constriction in ileum.
chloramphenicol, an intra-abdominal abscess developed. After sorne initial improvement following drainage of the abscess, the patient became febrile and obtunded. Her condition gradually worsened, and she died on the 17th postoperative day. Autopsy confirmed the absence of the gallbladder, cystic duct, and gallbladder fossa. An adenocarcinoma involving the right hepatic and common bile ducts had metastasized to lymph nades in the porta hepatis. The bile ducts were not dilated and did not contain stones. A disruption of the ileoileostomy was surrounded by an abscess cavity. Discussion
Anatomie variations in the biliary tract are fairly common, but true congenital absence of the gallbladder and cystic duct is quite rare. It has been reported in only 0.03% to 0.07% of autopsies 1 - 3 and 0.04% ofbiliary tract operations. 4 ' 5 Biliary tract cancer, however, is found in 0.1% to 0.46% of autopsies and in 0.3% to 1.8% of biliary tract operations. 6 - 8
230
The coexistence of congenital absence of the gallbladder and cancer of the biliary tract, as was found in this patient, has been reported in only two others. Y.lo This very low incidence of coexistence of these two conditions, although possibly due to the rarity of agenesis of the gallbladder, tends to ne gate a causeand-effect relationship. Because gallstones have been found in 20% to 57% of ali cases of bile duct cancer, they have been implicated in its etiola gy. 7 • 8 Common duct abnormalities, such as stones, dilatation, or bath, exist in about half of ali patients with agenesis of the gallbladder. 11 - 14 Yet the common duct was normal in this patient and in the two reported elsewhere. Thus, if the presence of gallstones is a criterion, it would seem that an etiologie link between the cancer and the agenesis is nonexistent. The failure of cholecystography to visualize the gallbladder was obviously misleading. The diagnosis of congenitally absent gallbladder is difficult and is seldom made preoperatively. Documentation of the disorder at operation requires cholangiography, and often the absence cannat be substantiated until autopsy. Symptoms, when present, are those ofbiliary tract disease, with episodes of epigastric pain, nausea, vomiting, flatulence, and jaundice. More than half of symptomatic patients have common duct calculi, dilatation, or bath to explain symptoms. 11 - 14 Yet many patients have no abnormality except the absence of the gallbladder and cystic duct. In the three patients with this abnormality and bile duct cancer, the symptoms may have been due to the cancer. Certainly in the case described here, the symptoms were due to mechanical sm ali- bowel obstruction-the preoperati ve diagnosis. Metastasis to intra-abdominal viscera from bile duct cancer has been reported. 6 Thorbjarnarson found small-bowel metastasis from bile duct carcinoma in 1 of 31 cases. 15 Extensive reviews indicate the relative infrequency of small-bowel metastasis as an isolated event. 16 - 18 Moreover, the association of small-bowel metastasis with the pathologie
POSTGRADUATE MEDIC.. E o March 1976 o Vol. 59 o No. 3
conditions discussed here makes this case quite singular. • Dr. Gurli was formerly in the department of surgery, Beth Israel Hospital, Boston. He is now chief, department of surgical gastroenterology, division of surgery, Walter Reed Army Institute of Research, Washington, DC 20012.
Downloaded by [Australian Catholic University] at 07:41 21 August 2017
References 1. Talmadge GK: Congenital absence of the gallbladder. Arch Pathol 26:1060-1062, 1938 2. Monzas G, Wilson AK: Congenital absence of gallbladder with stone in common bile duct. Lance! 1:628-629, 1953 3. Mclllrath DC, Remine WH, Baggenstoss AH: Congenital absence of the gallbladder and cystic duct: Report of ten cases found at necropsy. JAMA 180:782-783, 1962 4. Malmstrom L: Congenital absence of gallbladder: Report of a case diagnosed at operative cholangiography. Acta Chir Scand 105:440-447, 1953 5. Lindskog BI: Congenital absence of the gallbladder. Acta Chir Scand 139:208-209, 1973 6. Sako K, Seitzinger GL, Garside E: Carcinoma of the extrahepatic bile ducts: Review of the literature and report of six cases. Surgery 41:416-437, 1957 7. Neibling HA, Dockerty MB, Waugh JM: Carcinoma of extrahepatic bile duels. Surg Gynecol Obstet 89:429-438, 1949 8. Stewart HL, Lie ber MM, Morgan DR: Carcinoma of the extrahepatic bile duels. Arch Surg 41:662-713, 1940 9. Robertson HF, Robertson WE, Bower JO: Congenital absence of the gallbladder with primary carcinoma of the common duct and carcinoma of the liver. JAMA 114:1514-1517, 1940 10. Richards RN: Congenital absence of the gallbladder and cystic duc.t associated with primary carcinoma of the common bile duct. Can Med Assac J 94:859-860, 1966 Il. Dixon CF, Lichtman AL: Congenital absence of the gallbladder. Surgery 17:11-21, 1945 12. Carnevali JF, Kunath CA: Congenital absence of gallbladder. Arch Surg 78:440-445, 1959 13. Gerwig WH Jr, Countryman LK, Gomez AC: Congenital absence of the gallbladder and cystic duct: Report of six cases. Ann Surg 153:113-125, 1961 14. Rogers Al, Crews RD, Kaiser MH: Congenital absence of the gallbladder with choledocholithiasis. Gastroenterology 48:524-529, 1965 15. Thorbjarnarson B: Carcinoma of the bile ducts. Cancer 12:708-713, 1959 16. deCastro CA, Dockerty MB, Maya CW: Metastatic tumors of the small intestines-. Surg Gynecol Obstet 105:159-165, 1957 17. Farmer RG, Hawk WA: Metastatic tumors of the small bowel. Gastroenterology 47:496-504, 1964 18. Marshak RH, Khilnani MT, Eliasoph J, et al: Metastatic carcinoma of the small bowel. Am J Roentgenol Radium Ther Nucl Med 94:385-394, 1965
POSTGRADUATE MEDICINE invites submission of brief case reports for earl y publication. Illustrations and references should be included only when essential.
Vol. 59 • No. 3 • March 1976 • POSTQAADUATE MEDICINE
