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Congenital bronchobiliary fistula: Diagnosis and postoperative surveillance with HIDA scan
Congenital
Bronchobiliary Fistula: Diagnosis and Postoperative Surveillance With HIDA Scan
By Sepehr
Egrari,
Mohan
Krishnamoorthy, Los
Angeles,
Corine
A. Yee, and Harry Applebaum
California
0 Congenital bronchobiliary fistula (CBBF) is an extremely rare anomaly with myriad presentations that often include common bile duct abnormalities. Traditionally, bronchoscopy and bronchography have been used to establish the diagnosis. A full-term neonate with pneumonia and bilious secretions was diagnosed as having CBBF by means of a HIDA scan. Successful repair consisted of a right thoracotomy, high ligation of the fistula via an extrapleural approach, and cholecystography to confirm biliary drainage into the duodenum. The HIDA scan is a safe and efficient means to diagnose CBBF. When used in combination with surgical contrast studies, it provides clear delineation of all variations of this abnormality, enabling one-stage correction. Copyright o 1996 by W.B. Saunders Company INDEX
WORDS:
Congenital
bronchobiliary
fistula,
HIDA
scan.
ONGENITAL bronchobiliary fistula (CBBF) is a rare condition. The first case was reported by C Neuhauser et al in 19.52.l Several patients have been diagnosed and have had successful repair. Although bronchography has been the mainstay of diagnosis, we present a case diagnosed by HIDA scan. CASE
REPORT
The patient, a 3,040-g girl, was the product of a full-term gestation. She was delivered vaginally and had Apgar scores of 8 and 9. Her clinical condition after birth was good. On the third day she was noted to have respiratory difficulty and was evaluated and eventually intubated, and was given the diagnosis of aspiration pneumonia. During the ensuing days, a greenish endotracheal aspirate was noted, and she continued to have difficulty being weaned from mechanical ventilatory support. Chemical analysis of the fluid showed that it was consistent with bile. A HIDA scan was obtained (Fig l), which showed sequential drainage of bile toward the chest. Bronchobiliary fistula was diagnosed. For confirmation, a bronchogram was obtained, which showed a fistulous tract arising from the carina and extending toward the esophageal hiatus, Surgical management included a right posterolateral thoracotomy, using an extrapleural approach through the fifth intercostal space. A bronchobiliary fistula was found to arise from the carina. The fistula was divided and repaired in a manner similar to that used to correct tracheoesophageal fistula. A minilaparotomy and cholecystogram were performed, which revealed no abnormalities of the biliary system. The postoperative course was uneventful. The patient was extubated on postoperative day 3 and had remarkable improvement of the right lung. On the 10th postoperative day, a HIDA scan was obtained (Fig 2) which showed some delay in drainage of the left side of the liver. She was discharged on postoperative day 12 and has maintained normal liver function parameters. DISCUSSION
The diagnosis of CBBF can be delayed2 and requires a high degree of suspicion, because it can be Journaloffedmfr~c
Surgery,
Vol31,
No 6 (June),
1996: pp 785-786
Fig 1. bile.
Preoperative
HIDA scan showed
cephalad
drainage
of the
mistaken for bilious emesis associated with gastrointestinal obstruction. Bronchoscopy with contrast studies has been the mainstay of diagnosis.3$4 We describe a case that was correctly diagnosed with the HIDA scan. Use of this mode obviates bronchoscopy and contrast studies, and decreases the insult to the lungs of these patients, all of whom present with chemical pneumonitis. The HIDA scan is useful not only in defining and diagnosing biliary fistula but also in effectively delineating the liver and distal biliary tree. Most of the From the Departments of Surgery and Nuclear Medicine, Katser Permanente Medical Center, LosAngeles, CA. Presented at the 27th Annual Meeting of the Canadtan Association of Paediatnc Surgeons, Montreal, Quebec, September 2-4, 1995. Address reprint requests to Hary Applebaum, MD, Department of Pediatric Surgery, Kaiser Pemanente Medical Center, 4747 Sunset Blvd, Los Angeles. CA 90027. Copyright o I996 by W B. Saunders Company 0022-346819613106-0013$03.00l0 785
EGRARI
786
Fig 2. Postoperative HIDA scan showed in the left side of the liver (*).
delayed
of passage
of bile
ET AL
previously reported cases have shown the fistula communicating with the left lobe of the liver. Furthermore, the left lobe has been observed to decrease in size after ligation of the fistula.5 This is attributable to the biliary cirrhosis and fibrosis that ensue from diminution of bile drainage after ligation of the fistula, which is believed to be a major passage for bile drainage. Chan et al6 reported a case of biliary fistula associated with biliary atresia. The HIDA scan is ideal to assessbile drainage distally and can evaluate the hepatic dysfunction that can occur. The HIDA scan also can play a role in the decision to perform cholangiography during the operation. In 1963, Enjoji et al7 reported a case of CBBF associated with stenosis of the common bile duct in a 7-monthold boy, detected by a cholecystocholangiogram. Others have diagnosed numerous anomalies of the distal biliary architecture by means of cholangiography. Hepatobiliary scintigraphy reportedly was useful as a diagnostic tool in the evaluation of CBBF.8,9 We believe that the HIDA scan is effective in determining the need for laparotomy and cholangiography. Herein we report on a rare congenital anomaly, CBBF, and demonstrate the unique role of the HIDA scan in diagnosing and clarifying its presence. The scan is effective and safe preoperatively, and can be a tool in postoperative monitoring of hepatic drainage. We believe that it is an alternative to the bronchogram in the diagnosis of this anomaly.
REFERENCES 1. Neuhauser EBD, Elkin M, Landig B: Congenital direct communication between biliary system and respiratory tract. Am J Dis Child 83:654-659,1952 2. Weitzman JJ, Cohen SR, Wood LO, et al: Congenital bronchobiliary fistula. J Pediatr Surg 73:329-334, 1968 3. Lindahl H, Nyman R: Congenital bronchobiliary fistula successfully treated at age of three days. J Pediatr Surg 21:734-735, 1986 4. Sane SM, Sieber KW, Girdany BR: Congenital bronchobiliary fistula. Surgery 69:599-608,197l 5. Wagget J, Stool S, Bishop HL, et al: Congenital bronchobiliary fistula. J Pediatr Surg 5:566-569,197O
6. Chan YT, Ng WD, Mule WP, et al: Congenital bronchobiliary fistula associated with biliary atresia. Br J Surg 71:240-241,1984 7. Enjoji M, Watanabe H, Nakamura Y: A case reportCongenital biliotracheal fistula with trifuration of the bronchi. Ann Pediatr 200:321, 1963 8. Mavunda K, Heiba S, Oiticica C, et al: Congenital bronchobiliary fistula-Diagnosis by hepatobiliary scintigraphy. Respir Care 34:731-733, 1989 9. Gauderer MW, Oiticica C, Bishop HC: Congenital bronchobiliary fistula: Management of the involved hepatic segment. J Pediatr Surg 28:452-455,1993
Bronchobiliary Fistula: Diagnosis and Postoperative Surveillance With HIDA Scan
By Sepehr
Egrari,
Mohan
Krishnamoorthy, Los
Angeles,
Corine
A. Yee, and Harry Applebaum
California
0 Congenital bronchobiliary fistula (CBBF) is an extremely rare anomaly with myriad presentations that often include common bile duct abnormalities. Traditionally, bronchoscopy and bronchography have been used to establish the diagnosis. A full-term neonate with pneumonia and bilious secretions was diagnosed as having CBBF by means of a HIDA scan. Successful repair consisted of a right thoracotomy, high ligation of the fistula via an extrapleural approach, and cholecystography to confirm biliary drainage into the duodenum. The HIDA scan is a safe and efficient means to diagnose CBBF. When used in combination with surgical contrast studies, it provides clear delineation of all variations of this abnormality, enabling one-stage correction. Copyright o 1996 by W.B. Saunders Company INDEX
WORDS:
Congenital
bronchobiliary
fistula,
HIDA
scan.
ONGENITAL bronchobiliary fistula (CBBF) is a rare condition. The first case was reported by C Neuhauser et al in 19.52.l Several patients have been diagnosed and have had successful repair. Although bronchography has been the mainstay of diagnosis, we present a case diagnosed by HIDA scan. CASE
REPORT
The patient, a 3,040-g girl, was the product of a full-term gestation. She was delivered vaginally and had Apgar scores of 8 and 9. Her clinical condition after birth was good. On the third day she was noted to have respiratory difficulty and was evaluated and eventually intubated, and was given the diagnosis of aspiration pneumonia. During the ensuing days, a greenish endotracheal aspirate was noted, and she continued to have difficulty being weaned from mechanical ventilatory support. Chemical analysis of the fluid showed that it was consistent with bile. A HIDA scan was obtained (Fig l), which showed sequential drainage of bile toward the chest. Bronchobiliary fistula was diagnosed. For confirmation, a bronchogram was obtained, which showed a fistulous tract arising from the carina and extending toward the esophageal hiatus, Surgical management included a right posterolateral thoracotomy, using an extrapleural approach through the fifth intercostal space. A bronchobiliary fistula was found to arise from the carina. The fistula was divided and repaired in a manner similar to that used to correct tracheoesophageal fistula. A minilaparotomy and cholecystogram were performed, which revealed no abnormalities of the biliary system. The postoperative course was uneventful. The patient was extubated on postoperative day 3 and had remarkable improvement of the right lung. On the 10th postoperative day, a HIDA scan was obtained (Fig 2) which showed some delay in drainage of the left side of the liver. She was discharged on postoperative day 12 and has maintained normal liver function parameters. DISCUSSION
The diagnosis of CBBF can be delayed2 and requires a high degree of suspicion, because it can be Journaloffedmfr~c
Surgery,
Vol31,
No 6 (June),
1996: pp 785-786
Fig 1. bile.
Preoperative
HIDA scan showed
cephalad
drainage
of the
mistaken for bilious emesis associated with gastrointestinal obstruction. Bronchoscopy with contrast studies has been the mainstay of diagnosis.3$4 We describe a case that was correctly diagnosed with the HIDA scan. Use of this mode obviates bronchoscopy and contrast studies, and decreases the insult to the lungs of these patients, all of whom present with chemical pneumonitis. The HIDA scan is useful not only in defining and diagnosing biliary fistula but also in effectively delineating the liver and distal biliary tree. Most of the From the Departments of Surgery and Nuclear Medicine, Katser Permanente Medical Center, LosAngeles, CA. Presented at the 27th Annual Meeting of the Canadtan Association of Paediatnc Surgeons, Montreal, Quebec, September 2-4, 1995. Address reprint requests to Hary Applebaum, MD, Department of Pediatric Surgery, Kaiser Pemanente Medical Center, 4747 Sunset Blvd, Los Angeles. CA 90027. Copyright o I996 by W B. Saunders Company 0022-346819613106-0013$03.00l0 785
EGRARI
786
Fig 2. Postoperative HIDA scan showed in the left side of the liver (*).
delayed
of passage
of bile
ET AL
previously reported cases have shown the fistula communicating with the left lobe of the liver. Furthermore, the left lobe has been observed to decrease in size after ligation of the fistula.5 This is attributable to the biliary cirrhosis and fibrosis that ensue from diminution of bile drainage after ligation of the fistula, which is believed to be a major passage for bile drainage. Chan et al6 reported a case of biliary fistula associated with biliary atresia. The HIDA scan is ideal to assessbile drainage distally and can evaluate the hepatic dysfunction that can occur. The HIDA scan also can play a role in the decision to perform cholangiography during the operation. In 1963, Enjoji et al7 reported a case of CBBF associated with stenosis of the common bile duct in a 7-monthold boy, detected by a cholecystocholangiogram. Others have diagnosed numerous anomalies of the distal biliary architecture by means of cholangiography. Hepatobiliary scintigraphy reportedly was useful as a diagnostic tool in the evaluation of CBBF.8,9 We believe that the HIDA scan is effective in determining the need for laparotomy and cholangiography. Herein we report on a rare congenital anomaly, CBBF, and demonstrate the unique role of the HIDA scan in diagnosing and clarifying its presence. The scan is effective and safe preoperatively, and can be a tool in postoperative monitoring of hepatic drainage. We believe that it is an alternative to the bronchogram in the diagnosis of this anomaly.
REFERENCES 1. Neuhauser EBD, Elkin M, Landig B: Congenital direct communication between biliary system and respiratory tract. Am J Dis Child 83:654-659,1952 2. Weitzman JJ, Cohen SR, Wood LO, et al: Congenital bronchobiliary fistula. J Pediatr Surg 73:329-334, 1968 3. Lindahl H, Nyman R: Congenital bronchobiliary fistula successfully treated at age of three days. J Pediatr Surg 21:734-735, 1986 4. Sane SM, Sieber KW, Girdany BR: Congenital bronchobiliary fistula. Surgery 69:599-608,197l 5. Wagget J, Stool S, Bishop HL, et al: Congenital bronchobiliary fistula. J Pediatr Surg 5:566-569,197O
6. Chan YT, Ng WD, Mule WP, et al: Congenital bronchobiliary fistula associated with biliary atresia. Br J Surg 71:240-241,1984 7. Enjoji M, Watanabe H, Nakamura Y: A case reportCongenital biliotracheal fistula with trifuration of the bronchi. Ann Pediatr 200:321, 1963 8. Mavunda K, Heiba S, Oiticica C, et al: Congenital bronchobiliary fistula-Diagnosis by hepatobiliary scintigraphy. Respir Care 34:731-733, 1989 9. Gauderer MW, Oiticica C, Bishop HC: Congenital bronchobiliary fistula: Management of the involved hepatic segment. J Pediatr Surg 28:452-455,1993