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GI Grand Rounds October 21st, 2005 Yoshi Makino, M.D. USC Department of Internal Medicine Division of Gastrointestinal and Liver Disease Laurie DeLeve, M.D. Faculty Moderator Case Presentation • Patient M.G. is a 74 year old Hispanic female, p/w dull R-sided abdominal pain x 3 days, accompanied by subjective fevers and chills x 3 days. Constant pain, without radiation. Not worsened by PO intake. (+)N/V • PSH: – Cholecystectomy: 20 years ago – Unknown RUQ surgery: 10 years ago – ERCP (2/2005): multiple stones extracted with CBD stent placement (all procedures done in Guadalajara, Mexico) Case Presentation • PMH: – HTN – cholelithiasis • SH: – Denies EtOH/tobacco/illicit drug use – Recently moved from Mexico • FH: – Non-contributory Case Presentation • Allergies: NKDA • Medications – Vitamin D – Folate – CaCO3 – Benazepril 10 mg • ROS: – Non-contributory Physical Exam • Vital: – Tmax: 100.9 / T 98.4 / P 60 / R 16 / BP 124/78 • Gen: drowsy but A+O x 4 in NAD • HEENT: PERRLA, EOMi, MMM • Cardiac: RRR • Lungs: CTA(B) • Abdomen: – soft, obese, mild RUQ tenderness on deep palpation – RUQ and midline abdominal scars both well healed • Ext: without c/c/e • Rectal: normal tone, OB(-) Laboratories (10/6/05) 12.0 13.1 38.3 225 137 102 17 3.4 23 0.7 137 89 / 6 / 5 / 0 / 0 MCV RDW PT INR PTT PTT rat 86.5 13.5 14.0 1.07 35.6 1.20 Alk P TProt Alb TBili DBili 130 7.4 4.4 0.8 0.2 AST ALT Amy Lip 62 44 78 14 CT Scan (10/6/05) CT Scan (10/6/05) • Stent seen in the common bile duct associated with intrahepatic duct dilation and gas in the left intrahepatic duct. The stent is probably patent • Chronic liver disease • Low density lesion along the anterior surface of the left hepatic lobe Hospital Course • 10/6/05: EGD with side-view scope performed for stent removal – Old biliary stent with evidence of obstruction, removed without complication – Small, white stone also passed following stent removal – A diverticulum-like lesion was noted along the anterior duodenal wall EGD (Images) ERCP • Upon entering the duodenum, a small 4-5 mm stone seen in duodenal bulb ERCP ERCP • Dilated CBD to 2 cm with IHDD bilaterally, s/p sphincterotomy with multiple stones removed with balloon sweeps ERCP ERCP Hospital Course • Pt was started on Zosyn 3.375 mg IV q6h at time of admission, with rapid resolution of fevers • Pt offered surgical correction of the choledochofistula by HBS, but both the patient and daughter declined surgery at this time Choledochoduodenal Fistulas Introduction • Spontaneous internal biliary fistulas are not uncommon, seen in up to 5% of patients with biliary disease • Most fistulas form between the gallbladder and duodenum • However, advances in ERCP have lead to an increased detection of choledochoduodenal fistulas (CDFs) Types of Bilioenteric Fistulas • • • • • Cholecysto-duodenal (68%), Cholecysto-colonic (13.6%) Choledochoduodenal (8.6%) Cholecysto-gastric (4.9 %) Duodeno-left hepatic (4.9%) Stagnitti F. G Chir 2000 Yamada et al. Textbook of Gastroenterology 2003. Subclassification of CDFs • Proximal CDFs – Primarily located along the posterior wall of the duodenal bulb • Distal CDFs – Periampullary – Typically connects to the distal 2 cm of the CBD Incidence of CDFs • In a review of 2012 ERCPs in Argentina observed 14 cases (0.7%) Jorge et al. Endoscopy 1991. • A similar review of 1929 ERCPs in Japan found 33 cases (1.9%) Yamashita et al. HPB Surg 1997. • Another review of 1066 ERCPs in Taiwan found 27 cases (2.5%) Sheu et al. Am J Gastro 1996. Epidemiology of CDFs • Historically, CDFs have been reported more frequently in females – Proximal CDFs: 2:1 – Distal CDFs: 3:1 Yamada et al. Textbook of Gastroenterology 2003. • More recently, it has been suggested that Proximal CDFs are more common in men Naga et al. Endoscopy 1991. Epidemiology of CDFs • 75-90% of bilioenteric fistulas are associated with cholelithiasis • 5-6% are associated with duodenal PUD Iso Y et al. Hepato-Gastro 1996. • In the past, 75-80% of CDFs reported in Western countries were due to PUD, while only 15% in Japan Fukunaga H et al. Jpn J Clin Surg 1982. • With improved treatment options for PUD, these number appear to be changing Overview of Proximal CDFs • 80% of Proximal CDFs are caused by a penetrating duodenal ulcer, in a patient with a long history of PUD • Overall incidence of CDFs due to duodenal ulcers is low – Jaballah et al in 2001 found only 2 cases (0.6%) out of 200 cases – This may be due to the fact that duodenal ulcers typically occur within 4 cm distal to the pylorus while the CBD is about 7 cm distal to the pylorus Shah P. J Postgrad Med 1990. Presentation of Proxial CDFs • Symptoms mirror that of PUD • However, there have been case reports of relief of abdominal pain with the formation of CDFs • It has been postulated that bile flowing through the fistula bathes and alkalinizes the ulcer site Kyle J. Brit J Surg 1958. Diagnosis of Proximal CDFs • Demonstration of an ostium in the duodenal bulb discharging bile during endoscopy is the most common means of diagnosis • Pneumobilia is an inconsistent finding, present in only 14-58% of patients • Barium reflux into the biliary tree is highly suggestive of the disease Medical Management of Prox CDFs • Treatment of Proximal CDFs remains controversial • The natural history of CDFs due to ulcer disease are determined by the ulcer itself – Healing of ulcers leads to frequently leads to the healing of the fistula – With recent advances in acid-suppression therapy, many authors advocate medical therapy Jaballah et al. Dig Dis Sci 2001. • In the absence of primary biliary disease, there is minimal risk of cholangitis of biliary stricture Naga et al. Endoscopy 1991. Surgical Management of Prox CDFs • The loss of positive pressure due to CDFs leads to inability of the gallbladder to fill and contract adequately – As stagnant bile in the GB may become a nidus for infection, cholecystectomy is advocated – Laparoscopic suturing or stapling can be performed concurrently as well Lee JH. Surg Endosc 2004. Exclusion by Billroth II • Vagatomy with distal gastrectomy (antrectomy) and gastrojejunostomy by Billroth II reconstuction has also been suggested Walker and Large. Ann Surg 1954. Iso Y et al. Hepatogastro 1996. Duois F. Presse Med 1985. Overview of Distal CDFs • Greater than 90% of cases are believed to be due to cholelithiasis • Data is further supported by greater prevalence of Distal CDFs in cholelithiasis-endemic areas Karincaoglu et al. ANZ Surg 2003. • The presentation of Distal CDFs also mimics cholelithiasis, with RUQ pain, fever and jaundice Sheu et al. Am J Gastro 1996. Mirizzi Syndrome • Biliary obstruction caused by a gallstone impacted in the cystic duct or GB neck, first described by P. L. Mirizzi in 1948 – Type I: simple external compression of the CHD – Type II: cholecysto-choledochal fistula due to direct pressure necrosis of the adjacent duct walls • While technically a distinct entitiy, distal CDFs can be considered a variant of Mirizzi Syndrome Type II Ikeda Classification of Distal CDFs • Type I – Fistula present on longitudinal fold, just orad to the papilla • Type II – Fistula present on duodenal mucosa, proximal and adjacent to the duodenal fold Ikeda et al. Gastro 1975. Formation of Type I+II Distal CDFs • Type I – Form when small stone enters intramural portion of CBD – Fistulas and stones tend to be smaller • Type II – Form when a larger stone impacts in the extramural portion of CBD – Fistulas and stones are larger, with a 1.5 cm fistula and 4.2 x 2.6 x 2.5 cm stone reported Ikeda et al. Gastro 1975. Other Comparisons of Distal CDFs Type I Type II (n = 7) (n = 17) 3.18 1.35 0 17 Stone size (mm) 0.79 1.51 Fistulas Single / Multiple 7/0 14 / 3 Bilirubin (mg/dl) Pneumobilia P <0.01 <0.05 Sheu et al. Am J Gastro 1996. Bouveret’s Syndrome • Obstruction of the stomach or the duodenum from a gallstone, first described by Bouveret in 1891 • Stone migrates through a cholecysto or choledochoduodenal fistula, lodging in the duodenal bulb and resulting in obstruction • Rare condition, with roughly 100 cases reported over the past century • Most commonly in women (65%) with a median age of 68.6 years Gajanan et al. Ind J Gastro 2004. Geron et al. Surg Today 2003. Bouveret’s Syndrome (Diagnosis) • May be diagnosed by plain X-ray showing the classical triad of – distended stomach – pneumobilia – ectopic radio-opaque gallstone Rigler L et al. JAMA 1941. • Today, diagnosis is almost always made endoscopically, • Treatment via stone extraction or mechanical lithotripsy has limited success – Large impacted stones are difficult to remove – One case report of large stone becoming stuck in esophagus Moscho J et al. Rom J Gastroenterol 2005. Bouveret’s Syndrome (Surgery) • Surgery is required in over 90% of cases, with mortality rates as high as 19% to 24% • Typically, stone is removed via enterolithotomy, followed by possible cholecystectomy with closure of the fistula Lowe AS. Endoscopy 2005. • The addition of laser of shockwave lithotripsy have reduced mortality rate to 12% Cholangitis and Distal CDFs • Karincaoglu et al. retrospectively reviewed 841 patients who underwent ERCPs in Turkey, with – 311 patients with CBD stones – 16 patients with CBD stones + Distal CDFs • 7 without prior surgeries/ERCPs • 9 with history of cholecystectomy – 6 with intraoperative bile duct exploration – 3 without Karincaoglu et al. AZN J Surg. 2003. Results: CBDS+CDF vs CBDS only CBDS and CDF # of cases Age Sex (F/M) WBC Alk-P GGT TBili CBD size (mm) CBDS only 16 62 ± 14 6 : 10 311 56 ± 16 186 : 125 11.7 k 416 407 9.7 k 366 323 5.2 12.5 3.6 9.9 Iatrogenic Distal CDFs • While Karincaoglu et al argue that of their patients with distal CDFs – …only 37.5% (6 out of their 16) had prior instrumentation of the CBD – …however, 56% (9 out of 16) had a prior cholecystectomy Karincaoglu et al. AZN J Surg. 2003. • In a series by Rimer in Scandanavia, the incidence of iatrogenic CDFs during CBD exploration was 9.3%, rising to 23% when a rigid probe was used Rimer et al. Acta Chir Scan 1986. Iatrogenic Distal CDFs (con’t) • Hunt and Blumgart in 1980 reviewed 90 patients referred for severe post-cholecystectomy problems, finding 8 cases of distal CDFs – 3 cases occurred during sphincteroplasty or immediately following instrumentation – 5 cases involved the use of rigid probes with high resistance at the sphincted • In 7 out of the 8 cases, a Type II fistula was seen 1.0-1.5 cm proximal to the papilla Hunt and Blumgart. Br J Surg 1980. Iatrogenic Distal CDFs (con’t) • However, in Sheu’s review, of the 516 patients with cholelithiasis • 492 patients without CDFs • 24 patients with CDFs – Both groups had similar rates of previous surigal intervention to the biliary tract Sheu et al. Am J Gastro 1996. • Distal CDFs may also be created deliberately using a needle knife when routine cannulation methods are unsuccessful Espinel et al. Gastroenterol Hepatol 2005. Distal CDFs due to Malignancy • Case reports of ampullary carcinoma and cholangiocarcinoma have also been reported • Fistulas may be a due to weakening of the bile duct due to malignancy • However, reflux of duodenal contents via a preexisting fistula may play a role in biliary carcinogenesis as well Hakamada et al. Surgery 1997. Tanaka M et al. Gastointest Endosc 1998. Treatment of Type I Distal CDFs • Mainstay of treatment is via an extended sphincterotomy to avoid “sump syndrome” • Sump Syndrome – A recognized complication of a choledochoenterostomy, a sump (a pit or well) develops in the distal, nonfunctioning limb of the common bile duct – Lithogenic bile, gastrointestinal contents, and debris accumulate Rational for Treatment Distal Type Proximal Type Recurrence of BTI Conservative tx 12 0 12/12 (100%) Endoscopic tx 8 0 1/8 (12.5%) ENBD 5 1 Papillotomy 3 0 Surgery 4 Incidence of recurrent biliary tract infections after 1 year follow-up 3 1/7 (14.3%) Sheu et al. Am J Gastro 1996. Fistulotomy and Sphincterotomy Distal CDF and Papillotomy • LEFT: The ampulla with cannula in place. Contrast injected through the cannula flows back into the duodenum through the fistula tract • RIGHT: After papillotomy, two large CBD stones being extracted into the duodenum using the papillotomy. Endoscopic images from http://www.endoatlas.com/du_am_07.html Copyright © Atlanta South Gastroenterology, P.C Surgical Therapy for Distal CDFs • Little literature exists regarding the surgical management of distal CDFs • Hunt et al. recommended hepaticodochojejunostomy Hunt and Blumgart. Br J Surg 1980. • More recently, fibrin sealants have been used to endoscopically close the fistula Adrian P. JVIR 1993. Summary • Choledochoduodenal fistulas should be considered in patients with long history of cholelithiasis, especially with history of prior bile duct exploration • Proximal CDFs are typically due to peptic ulcer disease • Distal CDFs are typically associated with cholelithiasis • Mainstay of treatment involves cholecystectomy and extended sphincterotomy Questions / Comments Major References • Ikeda S, Okada Y. Classification of choledochoduodenal fistula diagnosed by duodenal fiberscopy and its etiological significance. • Gastroenterology. 1975 Jul;69(1):130-7. • Sheu BS. Shin JS. Lin XZ. Lin CY. Chen CY. Chang TT. Chen CY. Cheng PN. Clinical analysis of choledochoduodenal fistula with cholelithiasis in Taiwan: assessment by endoscopic retrograde cholangiopancreatography. American Journal of Gastroenterology. 91(1):122-6, 1996 Jan. • Hunt DR, Blumgart LH. Iatrogenic choledochoduodenal fistula: an unsuspected cause of post-cholecystectomy symptoms. Br J Surg. 1980 Jan;67(1):10-3. • Karincaoglu M, Yildirim B, Kantarceken B, Aladag M, Hilmioglu F. Association of peripapillary fistula with common bile duct stones and cholangitis. ANZ J Surg. 2003 Nov;73(11):884-6. Other References • • • • • • • • • Cooper SG, Sherman SB, Steinhardt JE, Wilson JM Jr, Richman AH. Bouveret's syndrome. Diagnostic considerations. JAMA. 1987 Jul 10;258(2):226-8. Feller ER. Warshaw AL. Schapiro RH. Observations on management of choledochoduodenal fistula due to penetrating peptic ulcer. Gastroenterology. 78(1):126-31, 1980 Jan. Fowler CL. Sternquist JC. Choledochoduodenal fistula: a rare complication of peptic ulcer disease. American Journal of Gastroenterology. 82(3):269-71, 1987 Mar. H'ng MW, Yim HB. Spontaneous choledochoduodenal fistula secondary to longstanding ulcer disease. Singapore Med J. 2003 Apr;44(4):205-7. Jaballah S, Sabri Y, Karim S.. Choledochoduodenal fistula due to duodenal peptic ulcer. Dig Dis Sci. 2001 Nov;46(11):2475-9. Martin DF. Tweedle DE. The aetiology and significance of distal choledochoduodenal fistula. British Journal of Surgery. 71(8):632-4, 1984 Aug. Ohtsuka T. Tanaka M. Inoue K. Nabae T. Takahata S. Yokohata K. Yamaguchi K. Chijiiwa K. Ikeda S. Is peripapillary choledochoduodenal fistula an indication for endoscopic sphincterotomy?. Gastrointestinal Endoscopy. 53(3):313-7, 2001 Mar. Shimao K. Yamaue H. Nishimoto N. Terasawa H. Saigan S. Onishi H. Tanimura H. Hashimoto T. Choledochoduodenal fistula at the anterior wall of the duodenal bulb: a rare complication of duodenal ulcer. Yamashita H, Chijiiwa K, Ogawa Y, Kuroki S, Tanaka M. The internal biliary fistula-reappraisal of incidence, type, diagnosis and management of 33 consecutive cases. HPB Surg. 1997;10(3):143-7.