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Clinical Brief
Spontaneous Biliary Perforation in Infancy and Childhood : Clues to Diagnosis Ravi P Kanojia, Shandip K. Sinha, Jiledar Rawat, Ashish Wakhlu, Shivnarain Kureel and Rajkumar Tandon Department of Pediatric Surgery King George Medical and University (KGMU), Lucknow (UP), India. [Received May 25, 2006; Accepted November 9, 2006]
ABSTRACT Spontaneous perforation of extrahepatic bile duct is rare. The cause is idiopathic once trauma and choledochal cyst are ruled out. The condition presents a diagnostic dilemma. Preoperative recognition is necessary as early surgical intervention gives excellent prognosis. We report clinical observations made in three cases with acute presentations. Diagnosis is to be suspected by the presence of jaundice after an initial anicteric period of good health with biliary ascites. This is confirmed by bilious abdominal paracentesis, signs of peritonitis and absent free gas on x ray. The constellation of these three findings was constant in three patients. The presented paper highlights the same as reliable clues to diagnosis. [Indian J Pediatr 2007; 74 (5) : 509-510] E-mail :
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Key words : Spontaneous biliary perforation; Biliary peritonitis; Biliary ascites
Idiopathic perforation of the nondilated extrahepatic bile duct is rare. Since its first description by Caulfield in 19361 , more than 150 cases have been reported in the literature. The condition may present acutely without previous signs of biliary tract disease. The patient usually has a mild fluctuating jaundice, abdominal distention, localized or generalized peritonitis and septicemic shock. The preoperative diagnostic dilemma in the absence of hepatobiliary scans is a common cause of delay in surgical intervention. It is emphasized that the few described clinical signs and X-ray can predict the diagnosis. This helps the surgeon to intervene early and plan accordingly. We report three cases of common bile duct perforation seen over a period of one yr (2004-05). There was a spontaneous biliary perforation without any obvious cause. Preoperative diagnosis was missed in first two patients. Clinical clues were later appreciated in retrospect. Presence of choledochal cyst and abdominal trauma as a part of surgical differential diagnosis were ruled out. All of them were successfully managed by surgery and recovered well.
Correspondence and Reprint requests : Dr Ravi Kanojia, Senior Resident, Department of Pediatric Surgery, King George Medical University (KGMU), Lucknow (UP), India. Phone 91-9839019309, 91 522-2257825
Indian Journal of Pediatrics, Volume 74—May, 2007
CASE REPORT Three patients one male and two female (patient one-8 mth female, patient two- 10 yr. female and patient three 20 mth male) presented in emergency with acute abdomen. Common to all 3 patients was the presence of abdominal signs of peritonitis, septicemic shock and absence of a prodrome. There was no free gas on plain abdominal X-ray. Mild unconjugated jaundice was present in patient one with other liver parameters in normal range. In patient one and two, surgical interventions was delayed by 48 hr due to presence of shock and diagnostic dilemma raised by a normal abdominal X-ray, despite a bilious peritoneal tap. All 3 patients underwent surgical exploration. The findings were common with bilious ascites with perforation at the junction of cystic duct with the common bile duct. Patient one and two were managed by T-tube drainage. Patient three was managed by flushing of bile duct with drainage of the lesser sac. Severe postoperative cholangitis was seen in patient 2 which was managed by 3rd generation cephalosporins. Normal cholangiograms were obtained after 2nd wk post operatively before removing the T-tube. Eventual recovery was satisfactory and all three patients have completed 1 yr of uneventful follow up. DISCUSSION Spontaneous perforation of the common bile duct is 509
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Ravi P. Kanojia et al commonly seen in children less than four years of age2 with the peak incidence during first year of life. Patients present both acutely and sub acutely. Acute presentation is with abdominal distention, ascitis and shock. Presence of jaundice is variable. Previous history pertaining to biliary tract disease is usually absent. This absence of a prodrome is notable and helps in distinguishing from other causes of peritonitis. The abdominal examination findings are that of generalized or localized peritonitis with ascites. Various forms of clinical presentation have been reported in literature. In a series of 11 patients 2 the presentation was generalized/ localized biliary peritonitis and secondary biliary stenosis. In a major review of 53 reported cases3 the clinical presentation was peritonitis with or without ascites. The past literature does not highlight the presentation as described in the three patients. The diagnosis has been made by the use of hepatobiliary nuclear scans. The access to such a modality is restricted in 3 rd world countries. Hence, emphasis is laid on clinical recognition of the rare entity supported with basic investigations like plain X-ray and sonogram. Ultrasound demonstrates loculated collection of bile as complex echogenic masses around the common bile duct and within the lesser sac. The preoperative diagnosis of patients presenting in emergency is difficult. Of authors three emergency patients the diagnosis was overlooked in two of them because of lack of previous experience. In the third patient the preoperative diagnosis was billiary perforation, as the child showed signs that were noted in previous two patients. There was ascites, signs of peritonitis, bilious fluid on paracentesis, and no pneumoperitoneum on erect abdominal X-ray. The diagnosis of biliary perforation can be reliably suspected preoperatively if these signs are present. Surgical management is mandatory as soon as the diagnosis is confirmed.4 In situations where the surgeon encounters billiary perforation without a pre-operative diagnosis, the safest policy is to drain the area and place a T-tube through the perforation. A per operative T-tube cholangiogram should be obtained if the conditions permit. The most common site of perforation is at the junction of cystic duct with the common bile duct. The choice between simple peritoneal drainage and Ttube drainage is a matter of discussion.5 If a peroperative cholangiogram is possible and shows normal passage of dye into the duodenum simple peritoneal drainage will suffice as there is no obstruction in CBD and the leak is expected to close spontaneously. If there is any evidence of distal CBD obstruction or if one is not able to obtain a
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peroperative cholangiogram then the condition is best managed by a T-tube drainage and peritoneal lavage. The patient requires postoperative antibiotics to counteract cholangitis and septicemia. The presumed obstruction causing the perforation is due to billiary sludge or inspissated bile. This is expected to clear by itself as the patient recovers from the acute condition. In no case CBD exploration is required to clear the obstruction. For other conditions encountered as perforation in the cystic duct, a simple cholecystectomy will cure the patient.6 Other less favored option for a small perforation in CBD can be a primary closure with cholecystostomy but with this incidence of re-accumulation of bile in the peritoneal cavity is high with the removal of the cholecystostomy tube. 7 In circumstances where the bile duct is obstructed and passage of dye into the duodenum is not seen on cholangiogram, CBD lavage to remove the plug by flushing through a fine catheter can be done followed by T-tube drainage. The post-operative course may be marked with cholangitis which has not been emphasized in the recent literature. The patient requires prolonged antibiotic therapy (3 rd generation cephalosporin) with other supportive measures. A normal cholangiogram is obtained after 2nd wk before removal of T-tube. Presence of spontaneous rupture of bile duct should always be considered in a young patient who develops jaundice after an initial anicteric period of good health or who presents with ascitis and peritonitis without pneumoperitoneum. Surgery is mandatory after appropriate investigations. Prognosis remains excellent with prompt diagnosis and treatment. REFERENCES 1. Caulfield E. Bile Peritonitis in infancy. Amer J Diseases in Children 1936; 52 : 1348-1360. 2. Chardot C, Iskandarani F et al. Spontaneous perforation of the biliary tract in infancy: a series of 11 cases. Eur J Pediatr Surg 1996; Dec: 6(6) : 341-346. 3. Lilly JR, Weintraub WH et al. Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 1992; 111 : 237-239. 4. Edward R Howard, Spontaneous Biliary Perforation In surgery of liver bile duct and pancreas in children. 2nd. Arnold Publisher 2002; 169-174 Ch 11. 5. N Spigland, R Greco, D Rosenfeld Spontaneous biliary perforation: Does external drainage constitute adequate therapy? J Pediatr Surg 1996 Jun; 31(6) : 782-784. 6. Stringel G, Mercer S Idiopathic perforation of the biliary tract in infancy. J Pediatr Surg 1983; 18 : 546-550. 7. Hammoudi SM, Allauddin A Idiopathic perforation of the biliary tract in infancy and childhood. J Pediatr Surg 1988; 23(2): 185-187.
Indian Journal of Pediatrics, Volume 74—May, 2007
