A biliary fistula is an abnormal connection that exists between the gall bladder and other parts of the biliary tree, the digestive tract (internal fistula) or the abdominal wall (external fistula).
Presentation
A biliary fistula is suspected in people who have had a history of cholelithiasis, peptic ulcer disease, Crohn's disease, biliary infections (e.g. hydatid disease), malignancies of the gastrointestinal (GI) tract or in those who have recently been surgically operated upon. Most patients are asymptomatic with overt clinical manifestations visible only in 20-30% of cases [1]. Symptoms are usually non-specific and are noticed in a variety of GI disorders. The diagnosis is thus, easily missed in the early stages of the disease.
Amongst individuals who do present with symptoms, abdominal pain may be a frequent finding if the fistula tracts are infected. Other complaints that may be seen include nausea, vomiting, diarrhea, fever, flatulence and weight loss. Cholangitis may lead to systemic manifestations like sepsis and peritonitis. Ascites may be observed in a few people.
Jaundice secondary to common bile duct obstruction or due to strictures of the bile duct (Mirizzi's syndrome) may be recorded [2]. Gallstones may pass into the gastrointestinal tract via these abnormal connections and may cause small bowel obstruction, especially in the terminal ileum (Bouveret's syndrome) [3]. Abdominal pain, vomiting, and constipation ensue in such patients.
In rare instances, fistulas may form between the biliary tree and the pleural/ bronchial spaces with pulmonary complaints occurring in such individuals.
Entire Body System
- Soft Tissue Mass
After antibiotic treatment and surgical opening of both lesions, abdominal computed tomography demonstrated a soft tissue mass cephalad to the umbilicus. We excised the mass, and found it to be associated with a fistula through the linea alba. [ncbi.nlm.nih.gov]
Gastrointestinal
- Chronic Diarrhea
A pathognomonic triad consisting of pneumobilia, chronic diarrhea, and vitamin K malabsorption was recently suggested by Savvidou and colleagues (2009) to aid in the diagnosis of cholecystocolonic fistula. [clinicalgate.com]
- Gastropathy
We report on a 70-year old woman with chronic active hepatitis and portal gastropathy who was treated with TIPS. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
- Biliary Colic
The hydatid cyst can rupture into the biliary tract, and cyst fluid escapes into the biliary tract with daughter cysts discharged into the common bile duct, causing biliary colic, obstructive jaundice and possibly liver abscess. [ncbi.nlm.nih.gov]
Interestingly, one third of the patients are absent of prior symptoms of biliary colic and up to 50% have no history of gallbladder disease. [scielo.org.pe]
[…] carcinoma 胆道癌 biliary colic 胆石仙痛 biliary liver cirrhosis胆汁性肝硬変 biliary tract 胆道 bilirubin ビリルビン board-like abdomen 板状硬 bougienageブジー拡張術 bowel movement便通 bowel preparation腸管洗浄 brushing cytology擦過細胞診 c cancer of stomach (stomach cancer)胃癌 carbohydrate [tokyo-med.ac.jp]
Workup
Patients presenting with clinical features suggestive of a biliary fistula are often diagnosed via surgery, with preoperative confirmation occurring only in 8-17% of all affected cases [4]. In such a scenario, most investigations are geared towards determining the type of surgery that needs to be conducted. This, in turn, is dependent on the likely etiology of the fistula, the presence of biliary tree obstruction/ erosion and the coexistence of a bilioenteric fistula.
Gallstones, the most common cause of a biliary fistula, are easily diagnosed by a transabdominal ultrasound (US) with a sensitivity of 96% [5]. Similarly, Mirizzi's syndrome can be detected via the transabdominal US with a sensitivity of 29% [4]. Magnetic resonance cholangiopancreatography (MRCP) may offer a higher accuracy in the evaluation of this disorder. It can be used to delineate other abnormalities seen in the biliary tract.
A computed tomography (CT) scan may help in locating the level of biliary tree obstruction. Aerobilia, a frequent sign of a bilioenteric fistula, may be easily observed via US or CT. A CT scan may help to rule out the presence of intestinal obstruction due to biliary stones and/or hepatic neoplasms.
The evaluation of these lesions may be determined by certain invasive studies [6]. Although the usage of endoscopic ultrasound (EUS) for recognizing biliary fistulas is still uncertain, some reports have shown that Mirizzi's syndrome can be reliably confirmed with EUS with 97% sensitivity and 100% specificity [7] [8] [9]. Endoscopic retrograde cholangiopancreatography (ERCP) shows up to 90% accuracy in diagnosing primary biliary fistulas and it may also have a therapeutic role in such cases, assisting in stone retrieval and biliary tract drainage via stents [10].
Laparoscopic cholecystectomy may be used to identify the site, size and potential cause of biliary leaks. A percutaneous transhepatic cholangiography is reserved for people with a completely stenosed biliary tree.
X-Ray
- Nonvisualization of the Gallbladder
However, a nonvisualized or shrunken gallbladder seen at US often coexisted in these cases. CT yielded the diagnosis in one case of gallstone ileus, and a Gastrografin meal yielded it in the case of Bouveret's syndrome. [ncbi.nlm.nih.gov]
Treatment
[…] duct, and persisting biliary fistulas after treatment with ERCP and ES. [ncbi.nlm.nih.gov]
So a conservative treatment was made immediately: it has been known that the treatment with somatostatin can reduce bile secretion, even if its benefits in promoting closure of fistula are unproved [ 7 ]. [wjes.biomedcentral.com]
Prognosis
This rare variant accounts for 3.5% of gallbladder cancers, and is associated with a worse prognosis than adenocarcinoma. The patient is in good condition without any signs of recurrence 42 months after the HPD. [ncbi.nlm.nih.gov]
Comorbidity was associated with a worse short-term and long-term prognosis. [scielo.isciii.es]
We have concluded, based on the literature that an early diagnosis associated with appropriate therapy can lead to a better prognosis. Keywords: Ileus; Gallbladder; Intestinal obstruction (source: MeSH NLM). [scielo.org.pe]
Prognosis Prognosis is generally good. However, given that most patients with this condition are elderly, potential coexisting medical problems may complicate surgical intervention. [emedicine.medscape.com]
Prognosis and postoperative period are good. Clinical case: 63-year-old patient with a clinical picture of 15 days of pain in the epigastrium and bilious vomiting. [revistas.unal.edu.co]
Etiology
The etiologic factors responsible for this complication are discussed. It is speculated that as more aggressive therapy for hepatic metastases is undertaken, this potential complication will be seen more frequently. [ncbi.nlm.nih.gov]
Pathology Etiology A wide variety of etiologies are implicated: congenital acquired surgical post inflammatory e.g. [radiopaedia.org]
Gallstones are the primary etiologic agent in most internal biliary-enteric fistulas. [surgerysearch.blogspot.com]
This, in turn, is dependent on the likely etiology of the fistula, the presence of biliary tree obstruction/ erosion and the coexistence of a bilioenteric fistula. [symptoma.com]
Epidemiology
It's divided into three sections on anatomy, pathophysiology, and epidemiology; diagnostic and therapeutic approaches including the latest therapeutic modalities; and specific conditions. Includes more than 250 illustrations for rapid reference. [books.google.com]
Epidemiology of Gallbladder Disease: Cholelithiasis and Cancer. Gut Liver. 2012;6(2):172-87. http://doi.org/cj3k. 11. Sánchez-García M, Tomoiu IG, Reina-Escobar D, Delgado-Casado JA. Causa infrecuente de obstrucción intestinal. [redalyc.org]
Epidemiology of Gallbladder Disease: Cholelithiasis and Cancer. Gut Liver. 2012;6(2):172-87. http://doi.org/cj3k. 11. S ánchez-García M, Tomoiu IG, Reina-Escobar D, Delgado-Casado JA. Causa infrecuente de obstrucción intestinal. [revistas.unal.edu.co]
[…] can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula. [13] Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition. [13] Epidemiology [emedicine.medscape.com]
Pathophysiology
This report describes the utility of this approach in the evaluation of the pathophysiology underlying apparent biliary obstruction in a patient with a large traumatic biliary fistula. [ncbi.nlm.nih.gov]
It's divided into three sections on anatomy, pathophysiology, and epidemiology; diagnostic and therapeutic approaches including the latest therapeutic modalities; and specific conditions. Includes more than 250 illustrations for rapid reference. [books.google.com]
Pathophysiology The cystic duct or gallbladder is almost always obstructed in patients with spontaneous cholecystocutaneous fistula. [emedicine.medscape.com]
Prevention
On the contrary, the indications for such intervention are always widening, as its risks become always smaller and the requirements for prevention higher. [books.google.com]
Suspicion of IBF should be kept in mind, especially in case of difficult dissection during cholecystectomy and attention should be paid in order to prevent iatrogenic injuries. [doi.org]
This catastrophy which is largely preventable, often necessitates repeated surgical intervention and accrues an estimated 5-year mortality rate approaching 30%. [ncbi.nlm.nih.gov]
References
- Abou-saif A, Al-kawas FH. Complications of gallstone disease: Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Am J Gastroenterol. 2002;97(2):249-54.
- Atli AO, Coşkun T, Ozenç A, Hersek E. Biliary enteric fistulas. Int Surg. 1997;82(3):280-3.
- Jones TA, Davis ME, Glantz AI. Bouveret's syndrome presenting as upper gastrointestinal hemorrhage without hematemesis. Am Surg. 2001;67(8):786-9.
- Beltrán MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol. 2012;18(34):4639-50.
- Costi R, Gnocchi A, Di mario F, Sarli L. Diagnosis and management of choledocholithiasis in the golden age of imaging, endoscopy and laparoscopy. World J Gastroenterol. 2014;20(37):13382-401.
- Yonetci N, Kutluana U, Yilmaz M, Sungurtekin U, Tekin K. The incidence of Mirizzi syndrome in patients undergoing endoscopic retrograde cholangiopancreatography. HBPD INT. 2008;7(5):520-4.
- Lakhtakia S, Gupta R, Tandan M, Rao GV, Reddy DN. Mirizzi's syndrome: EUS appearance. Gastrointest Endosc. 2006;63(2):322-3.
- Pelaez-luna M, Levy MJ, Arora AS, Baron TH, Rajan E. Mirizzi syndrome presenting as painless jaundice: a rare entity diagnosed by EUS. Gastrointest Endosc. 2008;67(6):974-5.
- Wehrmann T, Riphaus A, Martchenko K, et al. Intraductal ultrasonography in the diagnosis of Mirizzi syndrome. Endoscopy. 2006;38(7):717-22.
- Gomez D, Rahman SH, Toogood GJ, et al. Mirizzi's syndrome--results from a large western experience. HPB (Oxford). 2006;8(6):474-9.