Actu Pzdiatr Scand 68: 625-626, 1979 CASE REPORT
S P O N T A N E O U S PERFORATION OF THE COMMON BILE DUCT H . ENELL, B . CAVELL and G . MALMFORS From th P Departmen t., r?f P Nedia trics rr ntl Pu L
~ ~ tric Z I
Surgery, University Hospitml , Lun d, SMV don
ABSTRACT. Eneli, H., Cavell, B. and Malmfors, G . (Departments of Paediatrics and Paediatric Surgery, University Hospital, Lund, Sweden). Spontaneous perforation of the common bile duct. Acta Paediatr Scand, 68: 625, 1979.-An 18-week-old baby boy suffered an episode of acute abdominal symptoms followed by a silent period with mild obstructive jaundice, abdominal distension and failure to thrive. During the clinical workup he deteriorated suddenly, with progressive abdominal distension and ascites. At laparotomy a perforation of the common bile duct with bile peritonitis was found. Spontaneous perforation of the common bile duct is seldom listed as a cause of obstructive jaundice. The clinical picture is characteristic, Awareness of this diagnosis may help to avoid timeconsuming and unnecessary investigations and to lead to surgical treatment in good time. KEW WORDS: Obstructive jaundice, ascites, bile peritonitis, infancy
Spontaneous perforation of the common bile duct is seldom discussed as a cause of obstructive jaundice in the paediatric literature (6, 7). Lilly et al. ( 5 ) , however, state that this disease is second only to biliary atresia as a cause of surgical jaundice in the newborn. Until 1974 about 50 cases had been reported, most of them in the surgical literature (5). The aim of this paper is to draw attention to this disease and to report a case with a protracted course.
CASE HISTORY An 18-week-old boy was referred to a local hospital because of abdominal pains, irritability and anorexia. Pregnancy, delivery and neonatal period had been uneventful. On admission, the abdomen was distended and tender. The stools were pale and the urine dark. The emergency examination excluded an intestinal obstruction. The initial symptoms subsided gradually and the boy was referred 2 weeks later to the Paediatric Department, University Hospital. His general condition was then Fairly good. Physical examination revealed a normally developed, slightly jaundiced baby with moderate abdominal distension. During the following 2 weeks his general clinical condition further improved and his appetite returned. His weight,however, was stationary and the stools were still light-coloured, Laboratory investigation showed a conjugated hyper-
bilirubinemia around SO fimol/l. Alkaline phosphatases 18 ykatalll; glutamyltransferase 4.8 pkatal/l; aminotransferases normal: prothrombin time 32 s; urobilinogenl urine negative. Serum proteins including immunoglobulins were normal. Investigations for metabolic, endocrine or infectious causes of the illness turned out normal. Abdominal plain films taken early in the course showed slightly dilated bowels. A barium enema of the colon and an intravenous pyelography gave normal results. A choledochal cyst was suspected as a cause of the boy's stationary and mild cholestasis. A t intravenous cholegraphy the gallbladder appeared and emptied normally but the bile ducts were not visualized. In order to exclude an expansivity close to the bile duct. causing intermittent obstruction, ultrasonic echogram and X-ray of the stomach and upper small intestine were performed. The patient deteriorated quickly, however, with marked abdominal distension and signs of ascites. The X-ray showed wide intestinal loops. slow passage and ascites, but no signs of tumour dislocating the viscera. The ultrasonic echogrdm was negative. The boy was referred for emergency exploratory laparotomy in the department of paediatric surgery. On admission to this department the patient was in a rather poor general condition. Preoperatively he was given proper amounts of fluid, electrolytes and plasma. At laparotomy nearly one litre of bile-stained fluid was emptied from the abdominal cavity, before the bile ducts were exposed. An attempt to make a cholangiogram through the gallbladder was not successful. The choledochal duct was dissected and choledochotomy was performed. The lumen of the choledochal duct was normal whereas the external diameter was more than I cm due to a peritonitic swelling. A catheter was passed to the
626
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duodenum. proving passage. A leak was found a few millimetres distal of the cystic duct on the right lateral surface of the choledochal duct. The leak was oversewn and a choledochal drain was left in place. The area was thoroughly drained. The postoperative course was quite uneventful, A cholangiogram on the seventh day was normal. A few months post-operatively the child was healthy with normal liver function tests.
DISCUSS ION The clinical features presented by our patient agree well with those reported as characteristic in the literature (2,4,5). The infants usually present with symptoms at the age of one week to 3 months. Initial signs include mild fluctuating jaundice, pale or acholic stools, dark urine, irritability, vomiting and failure to thrive. There is a progressive abdominal distension which is sometimes accompanied by bile-staining of hydroceles, inguinal herniae or of the abdominal wall. The mild hyperbilirubinemia together with acholic stools indicate the correct diagnosis. The normal aminotransferases are helpful in differentiating from hepatitis. The i:311-Rose-Bengaltest may establish the preseence of a biliary leak ( I , 2). A protracted course of the disease, as observed in the case here reported, seems to be the most usual one, but an acute mode of presentation is sometimes seen (4). The symptoms and the time of onset are helpful in distinguishing the disease from other causes of obstructive jaundice in infancy, such as biliary atresia and choledochal cyst (3). It has been suggested that spontaneous perforation of the common bile duct and choledochal cyst are different manifestations of a common developmental aberration in ductal embryogenesis ( 5 ) . In many cases of spontaneous perforation of the common bile duct the operative finding is
an inflammatory sac, which has been misinterpreted as a 'ruptured choledochal cyst'. This may lead to an inadequate operative management with lethal outcome (5). We think that such a sac was initially formed in our patient, after which his condition improved. The sac then ruptured and gave rise to the alarming signs of bile peritonitis. Different operative procedures have been suggested, from simple drainage to choledochal resections with hepatico-enteric anastomosis. It seems as the simple drainage procedure is the method of choice (4, 5). Spontaneous perforation of the common bile duct is a rare disease. On the other hand an awareness of the condition is important as immediate surgical treatment is indicated.
REFERENCES I . Hansen, R., Wasnich. R., De Vries, P. & Sunshine, P.: Bile ascites in infancy: Diagnosis with '"'l-RoseBengal. J . Pediatr, 84: 719, 1974. 2. Howard, E., Johnston, D. & Mowat, A,: Spontaneous perforation of common bile duct in infants. Arch Dis Child, 51: 883, 1976. 3. Kobayashi, A. & Ohbe, Y . : Choledochal cyst in infancy and childhood. A w h Dls Child, 52: 121, 1977. 4. Lees, W. & Mitchell, J.: Bile peritonitis in infancy. Arch Dis Child, 41: 188, 1966. 5 . Lilly, J . , Weintraub. W . & Altman. P.: Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery, 75: 664. 1974. 6. Mowat, A . : Persistent neonatal jaundice. 111 J. T. Harris (ed .): Esseiztiiils o f Pediiitric Grist,.oPntrrologv. Churchill Livingstone, Edinburgh, London and New York 1977, p, 266. 7. Sass-Kortsak, A.: Management of young infants presenting with direct-reacting hyperbilirubinemia. Pedirrtr Clin N A t t i , 2/:777, 1974.
Submitted N o v . 13, 1978 Accepted Jan. 17, 1979 ( H . E.) Department of Paediatrics University Hospital S-221 XS Lund Sweden
S P O N T A N E O U S PERFORATION OF THE COMMON BILE DUCT H . ENELL, B . CAVELL and G . MALMFORS From th P Departmen t., r?f P Nedia trics rr ntl Pu L
~ ~ tric Z I
Surgery, University Hospitml , Lun d, SMV don
ABSTRACT. Eneli, H., Cavell, B. and Malmfors, G . (Departments of Paediatrics and Paediatric Surgery, University Hospital, Lund, Sweden). Spontaneous perforation of the common bile duct. Acta Paediatr Scand, 68: 625, 1979.-An 18-week-old baby boy suffered an episode of acute abdominal symptoms followed by a silent period with mild obstructive jaundice, abdominal distension and failure to thrive. During the clinical workup he deteriorated suddenly, with progressive abdominal distension and ascites. At laparotomy a perforation of the common bile duct with bile peritonitis was found. Spontaneous perforation of the common bile duct is seldom listed as a cause of obstructive jaundice. The clinical picture is characteristic, Awareness of this diagnosis may help to avoid timeconsuming and unnecessary investigations and to lead to surgical treatment in good time. KEW WORDS: Obstructive jaundice, ascites, bile peritonitis, infancy
Spontaneous perforation of the common bile duct is seldom discussed as a cause of obstructive jaundice in the paediatric literature (6, 7). Lilly et al. ( 5 ) , however, state that this disease is second only to biliary atresia as a cause of surgical jaundice in the newborn. Until 1974 about 50 cases had been reported, most of them in the surgical literature (5). The aim of this paper is to draw attention to this disease and to report a case with a protracted course.
CASE HISTORY An 18-week-old boy was referred to a local hospital because of abdominal pains, irritability and anorexia. Pregnancy, delivery and neonatal period had been uneventful. On admission, the abdomen was distended and tender. The stools were pale and the urine dark. The emergency examination excluded an intestinal obstruction. The initial symptoms subsided gradually and the boy was referred 2 weeks later to the Paediatric Department, University Hospital. His general condition was then Fairly good. Physical examination revealed a normally developed, slightly jaundiced baby with moderate abdominal distension. During the following 2 weeks his general clinical condition further improved and his appetite returned. His weight,however, was stationary and the stools were still light-coloured, Laboratory investigation showed a conjugated hyper-
bilirubinemia around SO fimol/l. Alkaline phosphatases 18 ykatalll; glutamyltransferase 4.8 pkatal/l; aminotransferases normal: prothrombin time 32 s; urobilinogenl urine negative. Serum proteins including immunoglobulins were normal. Investigations for metabolic, endocrine or infectious causes of the illness turned out normal. Abdominal plain films taken early in the course showed slightly dilated bowels. A barium enema of the colon and an intravenous pyelography gave normal results. A choledochal cyst was suspected as a cause of the boy's stationary and mild cholestasis. A t intravenous cholegraphy the gallbladder appeared and emptied normally but the bile ducts were not visualized. In order to exclude an expansivity close to the bile duct. causing intermittent obstruction, ultrasonic echogram and X-ray of the stomach and upper small intestine were performed. The patient deteriorated quickly, however, with marked abdominal distension and signs of ascites. The X-ray showed wide intestinal loops. slow passage and ascites, but no signs of tumour dislocating the viscera. The ultrasonic echogrdm was negative. The boy was referred for emergency exploratory laparotomy in the department of paediatric surgery. On admission to this department the patient was in a rather poor general condition. Preoperatively he was given proper amounts of fluid, electrolytes and plasma. At laparotomy nearly one litre of bile-stained fluid was emptied from the abdominal cavity, before the bile ducts were exposed. An attempt to make a cholangiogram through the gallbladder was not successful. The choledochal duct was dissected and choledochotomy was performed. The lumen of the choledochal duct was normal whereas the external diameter was more than I cm due to a peritonitic swelling. A catheter was passed to the
626
H . Enell et al.
duodenum. proving passage. A leak was found a few millimetres distal of the cystic duct on the right lateral surface of the choledochal duct. The leak was oversewn and a choledochal drain was left in place. The area was thoroughly drained. The postoperative course was quite uneventful, A cholangiogram on the seventh day was normal. A few months post-operatively the child was healthy with normal liver function tests.
DISCUSS ION The clinical features presented by our patient agree well with those reported as characteristic in the literature (2,4,5). The infants usually present with symptoms at the age of one week to 3 months. Initial signs include mild fluctuating jaundice, pale or acholic stools, dark urine, irritability, vomiting and failure to thrive. There is a progressive abdominal distension which is sometimes accompanied by bile-staining of hydroceles, inguinal herniae or of the abdominal wall. The mild hyperbilirubinemia together with acholic stools indicate the correct diagnosis. The normal aminotransferases are helpful in differentiating from hepatitis. The i:311-Rose-Bengaltest may establish the preseence of a biliary leak ( I , 2). A protracted course of the disease, as observed in the case here reported, seems to be the most usual one, but an acute mode of presentation is sometimes seen (4). The symptoms and the time of onset are helpful in distinguishing the disease from other causes of obstructive jaundice in infancy, such as biliary atresia and choledochal cyst (3). It has been suggested that spontaneous perforation of the common bile duct and choledochal cyst are different manifestations of a common developmental aberration in ductal embryogenesis ( 5 ) . In many cases of spontaneous perforation of the common bile duct the operative finding is
an inflammatory sac, which has been misinterpreted as a 'ruptured choledochal cyst'. This may lead to an inadequate operative management with lethal outcome (5). We think that such a sac was initially formed in our patient, after which his condition improved. The sac then ruptured and gave rise to the alarming signs of bile peritonitis. Different operative procedures have been suggested, from simple drainage to choledochal resections with hepatico-enteric anastomosis. It seems as the simple drainage procedure is the method of choice (4, 5). Spontaneous perforation of the common bile duct is a rare disease. On the other hand an awareness of the condition is important as immediate surgical treatment is indicated.
REFERENCES I . Hansen, R., Wasnich. R., De Vries, P. & Sunshine, P.: Bile ascites in infancy: Diagnosis with '"'l-RoseBengal. J . Pediatr, 84: 719, 1974. 2. Howard, E., Johnston, D. & Mowat, A,: Spontaneous perforation of common bile duct in infants. Arch Dis Child, 51: 883, 1976. 3. Kobayashi, A. & Ohbe, Y . : Choledochal cyst in infancy and childhood. A w h Dls Child, 52: 121, 1977. 4. Lees, W. & Mitchell, J.: Bile peritonitis in infancy. Arch Dis Child, 41: 188, 1966. 5 . Lilly, J . , Weintraub. W . & Altman. P.: Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery, 75: 664. 1974. 6. Mowat, A . : Persistent neonatal jaundice. 111 J. T. Harris (ed .): Esseiztiiils o f Pediiitric Grist,.oPntrrologv. Churchill Livingstone, Edinburgh, London and New York 1977, p, 266. 7. Sass-Kortsak, A.: Management of young infants presenting with direct-reacting hyperbilirubinemia. Pedirrtr Clin N A t t i , 2/:777, 1974.
Submitted N o v . 13, 1978 Accepted Jan. 17, 1979 ( H . E.) Department of Paediatrics University Hospital S-221 XS Lund Sweden
