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Congenital atresia of the common bile duct
CONGENITAL
ATRESIA
GEORGE P. ROSEMOND, M.D., W.
OF THE COMMON
BILE DUCT*
EMORY BURNETT, M.D. AND ROBERT M.
BUCHER, M.D.
Philadelphia, Pennsylvania
c
ONGENITAL atresia of the extrahepatic bile ducts has heId the particular interest of surgeons since Holmes in 1916 estimated that 16 per cent of the cases reported up to that time were theoreticaIly amenabIe to surgicaI therapy. He suggested the establishment of an anastomosis or, if this couId not be done, the estabIishment of an externa1 biIiary tistuIa and Iater the construction of continuity with the intestina1 tract. However, it was not until 1935 when Ladd’ reported a case in which the patient was successfuIIy treated that this condition was satisfactoriIy handled. Since that time there have been additional reports of successfu1 surgery*-” but not as many as one wouId expect. In October, 1948, Gray, DuShane and Henega? reported two cases from the Mayo CIinic and couId find onIy fifteen additional cases reported in the English Iiterature. These fifteen incIuded nine individual case reports and six cases reported previousIy by Ladd and Gross.” AIso in October, 1948, Leyva and Madiganll reported five successful cases from the Children’s HospitaI of Washington. One of these had apparentIy been previousIy reported by StiegIer in 1946’ and was included in the fifteen mentioned by Gray and his associates. Additiona search through the literature has reveaIed no other cases in which the patient survived for any length of time and these twenty-one cases remain as the onIy compIeteIy successfu1 ones reported. Chesterman12 reported a case of atresia of the common duct treated by choIecystoduodenostomy in which the patient died on the ninth postoperative day foIIowing a wound separation. Autopsy showed an intact anastomosis. Also, there have been * From the Temple University December,
1949
903
reported cases in which an obstruction due to inspissated biIe was reIieved by surgery,gv13s14 the thick biIe apparentIy being on the basis of Rh incompatibiIity. Analysis of the twenty-one reported cases reveals that the gaIIbIadder was anastomosed to either the stomach or smaI1 bowe1 in the majority of cases. The procedures used are as foIIows: cholecystogastrostomy eight, cholecystoduodenostomy six, choIecystojejunostomy one, choIedochoduodenostomy three and hepaticoduodenostomy three. CASE REPORT A. E., a Chinese-American boy, aged two weeks, was one of twins born in the Reading Hospital on June 18, 1948. It was the mother’s First pregnancy and both children seemed normal at birth following an uncomplicated Jabor. The baby’s birth weight was 5 pounds, 435 ounces. It was noted that the child had the usual amount of “physioIogic” jaundice which seemed to lessen about the eighth or ninth day. On the tenth day he was discharged from the hospital but was readmitted the following day because of the deveJopment of extreme jaundice and dehydration. The baby’s temperature was normal. The abdomen was distended and the Jiver edge paJpabJe two and a half lingers below the costal margin. There had been no vomiting. The stooIs were yeJJowish and the urine dark. The baby’s weight was 4 pounds, 15 ounces. BIood count showed hemoglobin 79 per cent, red blood ceJJs 3,800,000 and white blood cells 10,500. On the twelfth day the weight was 4 pounds, 9 ounces; icterus index was 150 and the serum bilirubin 6 mg. The van den Bergh test showed a delayed direct reaction; the child was Rh positive. He was maintained on 234 per cent glucose in norma saline, vitamin K and skimmed miIk formuJa, and was admitted to Temple University HospitaI on July and, the fourteenth day of Jife. Hospital,
PhiladeIphia,
Pa.
904
Rosemond
et al.-Congenital
On admission to this hospita1 the chiId was extremeIy jaundiced and dehydrated. There was moderate apathy and skin turgor was poor. The only other positive finding on physica examination was that the Iiver edge was paIpabIe 4 cm. beIow the costal margin. The spleen was not pa!pabIe and the chiId’s temperature was 98.4%. per rectum. A stoo1 on the day of admission was noted as being Iarge, mucoid and paIe yellow. Laboratory studies were as foIIows: hemogIobin 8.7 Gm., red bIood cells, 2,710,000, white blood ceIIs 9,200, polymorphonuclears 73 per cent, Iymphocytes 20 per cent and monocytes 7 per cent. CoaguIation time was four minutes and bleeding time thirty minutes. UrinaIysis showed yeIIow coIor, no sediment, pH 6.0, negative for aIbumin, sugar, biIe and occuIt blood. Microscopic study of the urinary sediment showed onIy 2 to 3 white bIood ceIIs per high power fieId. The bIood Wassermann test was negative and the icterus index was 171 units. The van den Bergh direct reaction was very strongIy positive and the indirect reaction showed 15.3 mg. per IOO cc. BIood cuItures were repeatedIy negative. The chiId was maintained on a high protein formula with vitamin suppIements and was given IO mg. of vitamin K parenteraIIy daiIy. On JuIy 3, 1948, he was given 50 cc. of bIood; bIeeding time was one minute on this date. On July 9th examination of the urine showed a sIightIy positive reaction for biIe pigments and a negative reaction for urobiIinogen. The feces were negative for urobiIinogen. Blood count showed hemoglobin I I .g Gm., red bIood ceIIs 3,17g,ooo and white blood cells 18,300, with poIymorphonucIears 40 per cent, lymphocytes 45 per cent, monocytes 8 per cent and eosinophiIes 6 per cent. A tentative diagnosis of congenitaI atresia of the bile ducts was made and the chitd was prepared for expIoration. During this time peniciIIin was given (10,000 units every three hours from JuIy 2nd to JuIy 8th and 40,000 units every tweIve hours from JuIy 9th to JuIy 2 I st, eight days postoperatively). AbdominaI expIoration was done under local anesthesia on July 13th. The liver was somewhat congested. A norma gaIIbIadder was observed; it was distended with dark, sticky biIe. The common duct was present and Iarge, and obviousIy extended down past the insertion of the cystic duct. The common duct
Atresia
was not expIored due to the poor genera1 condition of the chiId. ChoIecystojejunostomy was done and the abdomen cIosed as quickIy as possible. During and immediateIy foIlowing the operation IOO cc. of bIood were given intravenousIy. Postoperatively the chiId made a satisfactory recovery. Gastric suction was maintained for twenty-four hours and then oral and gavage feedings were begun. On JuIy 15th, the second postoperative day, the chiId’s temperature was 99.4%. per rectum and it was noted that a stoo1 showed mixed brownish and yeIIow coIor. A blood count showed hemogIobin 13.2 Gm., red bIood ceIIs 4,16g,ooo and white bIood ceIIs 9,200; the differentiar was normaI. On JuIy 16th the urine was negative for urobiIinogens and on the seventeenth the icterus index had faIIen to 86. During this period the chiId had a tendency to lose weight but subcutaneous amigens seemed to enabIe him to begin gaining. On JuIy 23rd the feces were found to be moderateIy positive for urobiIinogen and on this day the skin sutures were removed. By JuIy 26th the icterus index had faIIen to 20.5 units, the van den Bergh direct was slightly positive and the indirect 0.7 mg. per IOO cc. By the thirtieth the wound was we11 heaIed. On August 2nd hemoglobin was I 1.5 Gm., red bIood cells 4,480,ooo and white blood ceIIs 18,400, with a normal differentia1. By the fifth the icterus index was Ig units, the van den Bergh direct was stiI1 moderateIy positive and the indirect was 0.6 mg. per IOO cc. On August 12th the child was discharged from the hospital in good condition. Since that time he has been foIIowed up in our Pediatric CIinic. On his Iast visit to the cIinic in October, 1948, four months after operation, he was entireIy normal. COMMENT Our purpose in presenting this case report is to emphasize the fact that aIthough most children with this condition have proven to be inoperabre on expIoration, there is a definite salvage; and such patients must be carefuIIy expIored before we Iabel them with a hopeless prognosis. When such patients appear to be operabIe, the simplest possible method shouId be empIoyed due to the associated debility of the patient. American
Journal
of Surgery
Rosemond
et aI.-CongenitaI
REFERENCES I. LADD, WILLIAM E. CongenitaI
obstruction of the biIe ducts. Ann. Surg., 102: 742-751, 1935. 2. GRAY, HOWARD K., DUSHANE, JAMES W. and HENEGAR, GEORGE C. ChoIecystogastrostomy for congenita1 atresia of the common biIe duct: report of case. Proc. St& Meet., Mayo CL., 23: 473-476 1948. 3. LADD, WILLIAM E. and GROSS, ROBERT E. Sur-
gical anastomoses between the biliary and intestinal tracts of children; foIIow-up studies. Ann. Surg., I 12: 51-63, rg4o. 4. BEAVEN, T. E. D. and DUNCAN, GORDON W. Congenital atresia of the common bile-duct. Report of a successful case. &it. J. Surg., 33: 378-380, 1946. 5. STRAUSS, ABRAHAM, GROSS, JOSEPH and KYMAN, SEYMOUR. Congenital atresia of the common bile duct; case report. Ann. Surg., 117: 723-727, ‘943. 6. BILDERBACK,J. B., BUEERMANN,W. H. and GOODNIGHT, S. H. CongenitaI maIformation of biIe ducts. Report of case with severe hemorrhagic manifestations with recovery. Northwest Med., 36: 111-113, 1937. 7. STIEGLER, CHARLES. CongenitaI biliary atresia. Clin. Proc. Child. Hosp., 2: I 19-122, 1946. 8. DONOVAN, EDWARD J. CongenitaI atresia of the biIe ducts. Ann. Surg., 106: 737-744, 1937.
December,
1949
Atresia
904
g. PENBERTHY,GROVER C. and BENSON, CLIFFORD D. Surgery of the biliary tract in infants and children. Am. J. Surg., 40: 232-236, 1938. I o. LARGE, OCTAVUSP., THORP, FRANCISQ. and KANE, SIDNEY. Congenital atresia of the biIe ducts. Am. J. Surg.,-72: 91-96, 1946. I I. LEYVA. FERNANDO R. and MADICAN. IIOWARD S. Summary of the ChiIdren’s HospitaI exhibit at the meeting of the American Academy of Pediatrics, AtIantic City, November, 1948. Congenita1 biIiary atresia. Clin. Proc. C&/d. Hosp., 4: 309-3 I 3, ‘948. 12. CHESTERMAN,JUDSONT. CongenitaI atresia of the bile-ducts. Bit. J. Surg., 29: 52-55, ,941. 13. SKELTON, M. 0. and TOVEY, GEOFFREY II. The relation between congenita1 obliteration of the biIe ducts and icterus gravis neonatorum. Brit. M. J., 2: yr4-916, 1945. 14. DAVIDSOHN, ISRAEL. FetaI erythrobhlstosis. J. A. M. A., 127: 633-638, 1945. 15. STOLKIND, E. Congenital abnormalities of the gal1 bladder and extra-hepatic ducts. A review of 245 cases with reports of 31 unpublished cases. hit. J. Child. Dis., 36: I 15-131, 182-212 and 295-307, ‘939. 16. HOLMES, JAMES B. Congenital obliteration of the bile ducts; diagnosis and suggestions for trcatmcnt. Am. J. Dis. Cbild., I I: 405-43’. Igrh. 17. HOPKINS, N. K. CongenitaI absence of the common duct; three cases in one family. J. Lancet, 61: 90-91, ‘941.
ATRESIA
GEORGE P. ROSEMOND, M.D., W.
OF THE COMMON
BILE DUCT*
EMORY BURNETT, M.D. AND ROBERT M.
BUCHER, M.D.
Philadelphia, Pennsylvania
c
ONGENITAL atresia of the extrahepatic bile ducts has heId the particular interest of surgeons since Holmes in 1916 estimated that 16 per cent of the cases reported up to that time were theoreticaIly amenabIe to surgicaI therapy. He suggested the establishment of an anastomosis or, if this couId not be done, the estabIishment of an externa1 biIiary tistuIa and Iater the construction of continuity with the intestina1 tract. However, it was not until 1935 when Ladd’ reported a case in which the patient was successfuIIy treated that this condition was satisfactoriIy handled. Since that time there have been additional reports of successfu1 surgery*-” but not as many as one wouId expect. In October, 1948, Gray, DuShane and Henega? reported two cases from the Mayo CIinic and couId find onIy fifteen additional cases reported in the English Iiterature. These fifteen incIuded nine individual case reports and six cases reported previousIy by Ladd and Gross.” AIso in October, 1948, Leyva and Madiganll reported five successful cases from the Children’s HospitaI of Washington. One of these had apparentIy been previousIy reported by StiegIer in 1946’ and was included in the fifteen mentioned by Gray and his associates. Additiona search through the literature has reveaIed no other cases in which the patient survived for any length of time and these twenty-one cases remain as the onIy compIeteIy successfu1 ones reported. Chesterman12 reported a case of atresia of the common duct treated by choIecystoduodenostomy in which the patient died on the ninth postoperative day foIIowing a wound separation. Autopsy showed an intact anastomosis. Also, there have been * From the Temple University December,
1949
903
reported cases in which an obstruction due to inspissated biIe was reIieved by surgery,gv13s14 the thick biIe apparentIy being on the basis of Rh incompatibiIity. Analysis of the twenty-one reported cases reveals that the gaIIbIadder was anastomosed to either the stomach or smaI1 bowe1 in the majority of cases. The procedures used are as foIIows: cholecystogastrostomy eight, cholecystoduodenostomy six, choIecystojejunostomy one, choIedochoduodenostomy three and hepaticoduodenostomy three. CASE REPORT A. E., a Chinese-American boy, aged two weeks, was one of twins born in the Reading Hospital on June 18, 1948. It was the mother’s First pregnancy and both children seemed normal at birth following an uncomplicated Jabor. The baby’s birth weight was 5 pounds, 435 ounces. It was noted that the child had the usual amount of “physioIogic” jaundice which seemed to lessen about the eighth or ninth day. On the tenth day he was discharged from the hospital but was readmitted the following day because of the deveJopment of extreme jaundice and dehydration. The baby’s temperature was normal. The abdomen was distended and the Jiver edge paJpabJe two and a half lingers below the costal margin. There had been no vomiting. The stooIs were yeJJowish and the urine dark. The baby’s weight was 4 pounds, 15 ounces. BIood count showed hemoglobin 79 per cent, red blood ceJJs 3,800,000 and white blood cells 10,500. On the twelfth day the weight was 4 pounds, 9 ounces; icterus index was 150 and the serum bilirubin 6 mg. The van den Bergh test showed a delayed direct reaction; the child was Rh positive. He was maintained on 234 per cent glucose in norma saline, vitamin K and skimmed miIk formuJa, and was admitted to Temple University HospitaI on July and, the fourteenth day of Jife. Hospital,
PhiladeIphia,
Pa.
904
Rosemond
et al.-Congenital
On admission to this hospita1 the chiId was extremeIy jaundiced and dehydrated. There was moderate apathy and skin turgor was poor. The only other positive finding on physica examination was that the Iiver edge was paIpabIe 4 cm. beIow the costal margin. The spleen was not pa!pabIe and the chiId’s temperature was 98.4%. per rectum. A stoo1 on the day of admission was noted as being Iarge, mucoid and paIe yellow. Laboratory studies were as foIIows: hemogIobin 8.7 Gm., red bIood cells, 2,710,000, white blood ceIIs 9,200, polymorphonuclears 73 per cent, Iymphocytes 20 per cent and monocytes 7 per cent. CoaguIation time was four minutes and bleeding time thirty minutes. UrinaIysis showed yeIIow coIor, no sediment, pH 6.0, negative for aIbumin, sugar, biIe and occuIt blood. Microscopic study of the urinary sediment showed onIy 2 to 3 white bIood ceIIs per high power fieId. The bIood Wassermann test was negative and the icterus index was 171 units. The van den Bergh direct reaction was very strongIy positive and the indirect reaction showed 15.3 mg. per IOO cc. BIood cuItures were repeatedIy negative. The chiId was maintained on a high protein formula with vitamin suppIements and was given IO mg. of vitamin K parenteraIIy daiIy. On JuIy 3, 1948, he was given 50 cc. of bIood; bIeeding time was one minute on this date. On July 9th examination of the urine showed a sIightIy positive reaction for biIe pigments and a negative reaction for urobiIinogen. The feces were negative for urobiIinogen. Blood count showed hemoglobin I I .g Gm., red bIood ceIIs 3,17g,ooo and white blood cells 18,300, with poIymorphonucIears 40 per cent, lymphocytes 45 per cent, monocytes 8 per cent and eosinophiIes 6 per cent. A tentative diagnosis of congenitaI atresia of the bile ducts was made and the chitd was prepared for expIoration. During this time peniciIIin was given (10,000 units every three hours from JuIy 2nd to JuIy 8th and 40,000 units every tweIve hours from JuIy 9th to JuIy 2 I st, eight days postoperatively). AbdominaI expIoration was done under local anesthesia on July 13th. The liver was somewhat congested. A norma gaIIbIadder was observed; it was distended with dark, sticky biIe. The common duct was present and Iarge, and obviousIy extended down past the insertion of the cystic duct. The common duct
Atresia
was not expIored due to the poor genera1 condition of the chiId. ChoIecystojejunostomy was done and the abdomen cIosed as quickIy as possible. During and immediateIy foIlowing the operation IOO cc. of bIood were given intravenousIy. Postoperatively the chiId made a satisfactory recovery. Gastric suction was maintained for twenty-four hours and then oral and gavage feedings were begun. On JuIy 15th, the second postoperative day, the chiId’s temperature was 99.4%. per rectum and it was noted that a stoo1 showed mixed brownish and yeIIow coIor. A blood count showed hemogIobin 13.2 Gm., red bIood ceIIs 4,16g,ooo and white bIood ceIIs 9,200; the differentiar was normaI. On JuIy 16th the urine was negative for urobiIinogens and on the seventeenth the icterus index had faIIen to 86. During this period the chiId had a tendency to lose weight but subcutaneous amigens seemed to enabIe him to begin gaining. On JuIy 23rd the feces were found to be moderateIy positive for urobiIinogen and on this day the skin sutures were removed. By JuIy 26th the icterus index had faIIen to 20.5 units, the van den Bergh direct was slightly positive and the indirect 0.7 mg. per IOO cc. By the thirtieth the wound was we11 heaIed. On August 2nd hemoglobin was I 1.5 Gm., red bIood cells 4,480,ooo and white blood ceIIs 18,400, with a normal differentia1. By the fifth the icterus index was Ig units, the van den Bergh direct was stiI1 moderateIy positive and the indirect was 0.6 mg. per IOO cc. On August 12th the child was discharged from the hospital in good condition. Since that time he has been foIIowed up in our Pediatric CIinic. On his Iast visit to the cIinic in October, 1948, four months after operation, he was entireIy normal. COMMENT Our purpose in presenting this case report is to emphasize the fact that aIthough most children with this condition have proven to be inoperabre on expIoration, there is a definite salvage; and such patients must be carefuIIy expIored before we Iabel them with a hopeless prognosis. When such patients appear to be operabIe, the simplest possible method shouId be empIoyed due to the associated debility of the patient. American
Journal
of Surgery
Rosemond
et aI.-CongenitaI
REFERENCES I. LADD, WILLIAM E. CongenitaI
obstruction of the biIe ducts. Ann. Surg., 102: 742-751, 1935. 2. GRAY, HOWARD K., DUSHANE, JAMES W. and HENEGAR, GEORGE C. ChoIecystogastrostomy for congenita1 atresia of the common biIe duct: report of case. Proc. St& Meet., Mayo CL., 23: 473-476 1948. 3. LADD, WILLIAM E. and GROSS, ROBERT E. Sur-
gical anastomoses between the biliary and intestinal tracts of children; foIIow-up studies. Ann. Surg., I 12: 51-63, rg4o. 4. BEAVEN, T. E. D. and DUNCAN, GORDON W. Congenital atresia of the common bile-duct. Report of a successful case. &it. J. Surg., 33: 378-380, 1946. 5. STRAUSS, ABRAHAM, GROSS, JOSEPH and KYMAN, SEYMOUR. Congenital atresia of the common bile duct; case report. Ann. Surg., 117: 723-727, ‘943. 6. BILDERBACK,J. B., BUEERMANN,W. H. and GOODNIGHT, S. H. CongenitaI maIformation of biIe ducts. Report of case with severe hemorrhagic manifestations with recovery. Northwest Med., 36: 111-113, 1937. 7. STIEGLER, CHARLES. CongenitaI biliary atresia. Clin. Proc. Child. Hosp., 2: I 19-122, 1946. 8. DONOVAN, EDWARD J. CongenitaI atresia of the biIe ducts. Ann. Surg., 106: 737-744, 1937.
December,
1949
Atresia
904
g. PENBERTHY,GROVER C. and BENSON, CLIFFORD D. Surgery of the biliary tract in infants and children. Am. J. Surg., 40: 232-236, 1938. I o. LARGE, OCTAVUSP., THORP, FRANCISQ. and KANE, SIDNEY. Congenital atresia of the biIe ducts. Am. J. Surg.,-72: 91-96, 1946. I I. LEYVA. FERNANDO R. and MADICAN. IIOWARD S. Summary of the ChiIdren’s HospitaI exhibit at the meeting of the American Academy of Pediatrics, AtIantic City, November, 1948. Congenita1 biIiary atresia. Clin. Proc. C&/d. Hosp., 4: 309-3 I 3, ‘948. 12. CHESTERMAN,JUDSONT. CongenitaI atresia of the bile-ducts. Bit. J. Surg., 29: 52-55, ,941. 13. SKELTON, M. 0. and TOVEY, GEOFFREY II. The relation between congenita1 obliteration of the biIe ducts and icterus gravis neonatorum. Brit. M. J., 2: yr4-916, 1945. 14. DAVIDSOHN, ISRAEL. FetaI erythrobhlstosis. J. A. M. A., 127: 633-638, 1945. 15. STOLKIND, E. Congenital abnormalities of the gal1 bladder and extra-hepatic ducts. A review of 245 cases with reports of 31 unpublished cases. hit. J. Child. Dis., 36: I 15-131, 182-212 and 295-307, ‘939. 16. HOLMES, JAMES B. Congenital obliteration of the bile ducts; diagnosis and suggestions for trcatmcnt. Am. J. Dis. Cbild., I I: 405-43’. Igrh. 17. HOPKINS, N. K. CongenitaI absence of the common duct; three cases in one family. J. Lancet, 61: 90-91, ‘941.