Transcript Slide 1

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.