Documentos de Académico
Documentos de Profesional
Documentos de Cultura
Gallstones
Minh B. Luu, MD*, Daniel J. Deziel, MD
KEYWORDS
Gallstones Complications Lost stones Fistula Obstruction
KEY POINTS
Extrinsic compression of the bile duct from gallstone disease is associated with bilio-
biliary fistulization requiring biliary-enteric reconstruction.
Biliary-enteric fistulas are associated with intestinal obstruction at various levels. The pri-
mary goal of therapy is relief of intestinal obstruction; definitive repair is performed for
selected patients.
Hemobilia from gallstone-related pseudoaneurysms is preferentially controlled by selec-
tive arterial embolization.
Rapidly increasing jaundice with relatively normal liver enzymes is a diagnostic hallmark of
bilhemia.
Acquired thoraco-biliary fistulas are primarily treated by percutaneous and endoscopic in-
terventions.
INTRODUCTION
Gallstones are a routine commodity for general surgeons. Symptomatic disease pre-
sentations in their common forms of biliary colic, acute cholecystitis, choledocholithia-
sis, and gallstone pancreatitis are well known. This does not imply that these daily
conditions are without difficult challenges, even for the most experienced biliary sur-
geons. However, when stones migrate to involve adjacent viscera or vascular struc-
tures, the clinical challenge is far less familiar. Biliary eruption into the gut, into the
chest, or into the pelvis is a confounding situation; and, in circumstances that violate
major blood vessels, a rapidly catastrophic one. Most surgeons will encounter few, if
any, of these baffling patients during the course of their career. Yet, such occurrences
have been recognized for centuries and will continue. This article is a simple primer to
aid in the recognition and management of some of these gallstone oddities.
Department of General Surgery, Rush University Medical Center, Rush Medical College, 1633
West Congress Parkway, Chicago, IL 60612, USA
* Corresponding author.
E-mail address: minh_luu@rush.edu
BILIARY FISTULA
Internal Fistula
Bilio-biliary fistula
Mirizzi syndrome
Background Although Hans Kehr1 in 1905 first reported on patients with partial bile
duct obstruction due to an impacted stone in the gallbladder, it was Pablo Luis Mirizzis
article in 1948 entitled Sindrome del conducto hepatico2 that called attention to this
condition.
Pathophysiology The eponym Mirizzi syndrome (MS) is used to describe mechan-
ical obstruction of the common hepatic duct due to extrinsic compression (with or
without an associated fistula) from stones in the gallbladder or cystic duct. The actual
obstruction is usually from a large impacted stone in the gallbladder neck or infundib-
ulum adjacent to the extrahepatic ducts (Fig. 1). Sometimes the extrinsic obstruction
is caused by a small stone impacted in the cystic duct that has a long parallel course to
the common hepatic duct or even by a tensely distended gallbladder itself. In his
famous article, Mirizzi2 incorrectly postulated a functional spasm of the common he-
patic duct as the cause of the bile duct obstruction. MS occurs in approximately 0.2%
to 1.5% of patients with gallstones. Beltran3 theorized that the external compression
of the bile duct and the later development of cholecystobiliary and cholcystoenteric
fistulas are different stages of the same disease process.
Classification Classification schemes are founded on the presence or absence of
fistulous erosion between the gallbladder and common bile duct and the extent of
Fig. 1. An intraoperative picture of a large stone removed from the infundibulum of a pa-
tient with Mirizzi syndrome.
Unusual Complications of Gallstones 379
Fig. 2. Mirizzi syndrome type I to V. (A) Type I is the extrinsic compression of the common
bile duct by an impacted gallstone. (B) Type II involves one-third the circumference of the
bile duct. (C) Type III involves two-thirds the circumference of the bile duct. (D) Type IV
involves the whole circumference of the bile duct. (E) Type V involves types I to IV
with the addition of a bilioenteric fistula. (From Beltran MA, Csendes A, Cruces KS. The rela-
tionship of Mirizzi syndrome and cholecystoenteric fistula: validation of a modified classifi-
cation. World J Surg 2008;32:223743; with permission.)
380 Luu & Deziel
been used to allow time for surgical planning and medical optimization. Surgical treat-
ment is required and the extent based on the type of MS. Type I can be managed with
cholecystectomy or subtotal cholecystectomy alone, whereas types II, III, and IV often
require biliary reconstruction. Suggested surgical treatment based on Beltrans clas-
sification of MS is outlined in Table 1.9 Although the laparoscopic management of MS
has been described,10 surgeons should be wary because of the high risk of bile duct
injury. When diagnosed preoperatively, serious consideration should be given to
transferring the patient to a facility capable of major biliary reconstruction. Because
gallbladder cancer can be found in up to 25% of MS cases, an intraoperative frozen
section of the gallbladder wall should be performed and capability for an appropriate
oncologic resection available.11
Biliary-enteric fistula
Spontaneous internal biliary fistula: Most internal biliary fistulas are due to chronic or
neglected gallstone disease. Cholecystoduodenal fistulas are the most frequent, fol-
lowed by cholecystocolonic fistulas. Less common fistula sites are cholecystogastric,
cholecystocholedochal, choledochoduodenal, cholecystojejunal, and hepaticobron-
chial fistulas.1216 Choledochoduodenal fistulas are most commonly due to dorsal
erosion of a duodenal ulcer. A systematic review by Petrowsky and Clavien17 summa-
rized the distribution of biliary-enteric fistulas shown in Table 2.
Cholecystoduodenal fistulas account for 75% to 80% of cholecystoenteric fistulas.
Most are clinically silent or have vague digestive symptoms. Passage of large stones
can result in gallstone ileus. Cholecystocolic fistulas are the second most common
type.18 Costi and colleagues19 reviewed literature between 1950 and 2006 and found
231 cases. Only 7.9% of these cases were diagnosed preoperatively. In contrast to the
Unusual Complications of Gallstones 381
Table 1
New classification and suggested surgical treatment of Mirizzi syndrome
Type of
Mirizzi Description Treatment
I External compression of Open cholecystectomy
the bile duct Open subtotal cholecystectomy
Laparoscopic cholecystectomy
Laparoscopic subtotal cholecystectomy
IIa Cholecystobiliary fistula Open cholecystectomy
<50% the diameter of Open subtotal cholecystectomy
the bile duct
IIb Cholecystobiliary fistula Open subtotal cholecystectomy
>50% the diameter of Biliary-enteric derivation:
the bile duct Side-to-side to the duodenum
En-Y-de-Roux to the jejunum
IIIa Cholecystobiliary fistula and Simple closure of the fistula and treatment of
cholecystoenteric fistula the gallbladder according to the presence of
without gallstone ileus Mirizzi I, IIa, or IIb
IIIb Cholecystobiliary fistula and Treatment of the gallstone ileus and deferred
cholecystoenteric fistula treatment of the gallbladder according to the
with gallstone ileus presence of Mirizzi I, IIa or IIb
From Beltran MA. Mirizzi syndrome: history, current knowledge and proposal of a simplified clas-
sification. World J Gastroenterol 2012;18(34):463950.
Gallstone ileus
Background Gallstone ileus is classically described as the impaction of gallstone(s)
in the distal ileum and accounts for 1% to 3% of all mechanical bowel obstruction.21
The anatomist Thomas Bartholin first described gallstone ileus in 1654.22 In a series of
131 cases, Courvoisier,23 in 1890, reported a surgical mortality rate of 44%. More
contemporary mortality rates of 15% to 18% are still considerable and reflect the
associated afflictions of many patients with this condition.17,24,25
Clinical presentation Most cholecystoenteric fistulas are clinically silent, but if larger
(2 cm) stones enter the intestine, a mechanical obstruction results. Half of the pa-
tients with gallstone ileus will have a history of prior biliary symptoms and 20% to
30% of the patients have associated acute cholecystitis. Approximately 85% of
affected people are women and the median age is 70 years. Obstructive symptoms
may be partial and intermittent as the gallstone progresses aborally. This has been
referred to as a tumbling obstruction and may be responsible for the diagnostic
delay for several days that is characteristic in these patients. The Rigler triad, consist-
ing of small bowel obstruction (SBO), pneumobilia, and an aberrant stone in the GI
382
Luu & Deziel
Table 2
Incidence and frequency of biliary-enteric fistulas
From Petrowsky H, Clavien P. Biliary fistula, gallstone ileus, and Mirizzis syndrome. In: Clavien P, Baillie J, editors. Diseases of the gallbladder and bile ducts: diag-
nosis and treatment. 2nd edition. Malden (MA): Blackwell Publishing; 2006. p. 23951.
Unusual Complications of Gallstones 383
Fig. 4. Plain abdominal film demonstrating central pneumobilia and dilated small intestine
in gallstone ileus.
384 Luu & Deziel
Fig. 7. An endoscopic view of a gallstone obstructing the gastric outlet in a patient with
Bouveret syndrome.
Biliary-vascular fistula
Hemobilia Hemorrhage into the biliary system was recognized by Francis Glisson in
1654 as a potential consequence of blunt or penetrating injury to the liver.34 Antoine
Portal, Professor of Anatomy and Surgery at College Royale de France, is credited
with the first ante mortem diagnosis of the condition in 1777 while he was treating a
servant of the Marquise de Cambis who suffered from hepatic abscesses.35 In the
modern era, Swedish surgeon Philip Sandblom made comprehensive study of this
problem in his 1972 monograph.36 It was he who coined the term hemobilia to
denote bleeding into or through the biliary tract.
Hemobilia may originate from the liver, the gallbladder, the intrahepatic or extrahe-
patic bile ducts, or from the pancreas (hemosuccus pancreaticus). Bleeding may
range from occult to massive and has a tendency to occur periodically. Clinically sig-
nificant hemobilia is usually from an arterial source, although venous bleeding can be
significant in cases of portal hypertension. Trauma is the most frequent antecedent of
hemobilia; either accidental trauma or, more commonly, iatrogenic trauma from any of
a litany of transhepatic or endoscopic interventions, including cholangiography,
biopsy, drainage, stent placement, balloon dilatation, and lithotripsy. Other causes
include hepatobiliary malignancy, inflammatory conditions, vascular disorders, and,
of course, gallstones.
The symptoms of hemobilia depend on the rate of bleeding and the presence of bile
duct obstruction from clots. Flowing bile has natural fibrinolytic activity that dissolves
clots unless the bleeding is too rapid. Conversely, minor hemobilia may persist with
fibrinolysis and remain asymptomatic except for chronic anemia. The classic clinical
triad of biliary tract hemorrhage was highlighted by Quincke37 in 1871: gastrointestinal
hemorrhage, biliary colic, and jaundice. Gastrointestinal hemorrhage is most
commonly manifested as melena (90%), sometimes as hemetemesis (60%), with
biliary colic present in 70% of patients and jaundice in 60% of patients with clinically
apparent symptoms.36 Rarely, clot obstruction has caused acute cholecystitis or
pancreatitis.3844 There is a tendency to overlook the biliary tract as a source of signif-
icant gastrointestinal hemorrhage. Indeed, Professor Sandblom lamented that Blind
resection of a healthy stomach has been performed many times because of misinter-
preted hemorrhage into the biliary tract.36
Hemobilia from gallstones is usually microscopic and not uncommon. Fecal occult
blood has been demonstrated in one-quarter of patients with cholelithiasis.45 The
mechanism is direct trauma to the biliary mucosa from the stones. Gallstone disease
accounts for up to 10% of cases of major hemobilia.36,46 This occurs when gallstones
erode through the gallbladder wall or bile duct into an adjacent portion of the gut with
fistula formation and/or into an adjacent blood vessel with creation of a pseudoaneur-
ysm. Rupture of a pseudoaneurysm results in massive hemobilia, gastrointestinal
bleeding, and occasionally intraperitoneal bleeding as well.47 Not surprisingly, this
complication has frequently proven fatal.
Spontaneous gallstone erosion can involve various arteries, including the cystic ar-
tery, hepatic artery, replaced right hepatic artery (originating from the superior mesen-
teric artery), gastroduodenal artery, or even the aorta.36,4753 Pseudoaneurysms
resulting from operative trauma may cause life-threatening hemobilia as a late or early
complication following either open or laparoscopic cholecystectomy.5463 Post chole-
cystectomy pseudoaneurysms have been associated with bile leaks and formation of
late bile duct strictures.64 Three gallstones were discovered in the portal vein of Igna-
tius of Loyola at the time of his autopsy.65 Gallstones have been found in portal venous
collaterals along the common bile duct in a patient with portal vein thrombosis.36
When hemobilia is suspected, upper gastrointestinal endoscopy is the initial exam-
ination to exclude other sources and to directly visualize blood issuing from the
ampulla of Vater. Imaging by transabdominal ultrasonography, CT, magnetic reso-
nance imaging, or direct cholangiography via various routes can demonstrate blood
in the gallbladder or bile ducts and identify vascular anomalies. Selective hepatic arte-
rial angiography is the most specific method for identifying the source of an active
bleed and affords the opportunity for therapeutic embolization.
Treatment of gallstone-related hemobilia is by cholecystectomy and removal of bile
duct stones. When major arterial hemorrhage is identified, selective arterial emboliza-
tion is the mainstay of therapy today. Arterial injury discovered intraoperatively is
managed by vessel ligation or aneurysm repair. Bile duct obstruction caused by
hemobilia is successfully treated with endoscopic biliary drainage in most cases.66
Bilhemia Bilhemia is direct flow of bile into the blood through a biliary-vascular fistula.
This occurs when there is an abnormal communication between the higher-pressure,
typically obstructed, biliary system and the lower-pressure portal vein, hepatic veins,
or inferior vena cava. Clinically significant bilhemia is rare but has often resulted in fatal
Unusual Complications of Gallstones 387
pulmonary embolization of bile or sepsis.67 The cause is most often blunt trauma with
central liver rupture or iatrogenic penetrating injury associated with percutaneous
transhepatic interventions, such as biopsy, drainage, and placement of biliary stents
or transjugular intrahepatic porto-systemic shunts.6870 In exceptionally rare in-
stances, bilhemia may be caused by venous erosion of gallstones.36,65
The cardinal diagnostic feature of bilhemia is rapidly increasing jaundice and pro-
nounced direct hyperbilirubinemia without a substantial elevation of liver enzymes.68
Cholangiography by endoscopic retrograde or transhepatic approaches is the best
method for imaging the fistula. Unlike hemobilia, arteriography is not diagnostic. Hep-
atobiliary scintigraphy may also indicate the diagnosis.71
Treatment of bilhemia is founded on relief of bile duct obstruction by endoscopic,
transhepatic, or surgical means, which may result in spontaneous closure of the fistula.
Interruption of the fistula by placement of covered stents or by balloon tamponade or
angiographic coil occlusion has been successful.72,73 Although not applicable to gall-
stone bilhemia, operative solutions have included liver resection, liver transplantation,
and external drainage of the bile duct or disrupted liver.68
Biliary-thoracic fistula
Biliary-thoracic fistulas are abnormal communications between the biliary tract and
the thoracic cavity. The end point within the thoracic cavity can be either the pleural
space or the bronchial tree. The causes of biliary-thoracic fistulas are many and can
be categorized into thoracoabdominal trauma, parasitic liver disease, suppurative
biliary tract obstruction, or iatrogenic due to external drainage procedures. In Western
countries, gallbladder perforation, leading to subhepatic abscesses or postoperative
bile duct stenosis, is the primary cause of biliary-thoracic fistulas, whereas in devel-
oping countries, liver abscesses due to echinococcal or amebic infections are more
common.17 Patients often present with right upper quadrant and pleuritic pain, but
sometimes only vague symptoms, such as shortness of breath, are present.74 In pa-
tients with bronchobiliary fistulas, bitter taste and productive yellow sputum may be
present, suggesting bile pigments in sputum, known as biliptysis.75 Signs of systemic
infection, such as fever, chills, or leukocytosis, may be present in only half of the pa-
tients. Imaging will often show a right pleural effusion.76 Percutaneous and endo-
scopic interventions should first be attempted to treat acquired fistulas and surgery
should be reserved as second-line treatment.77 Singh and colleagues78 suggested a
treatment algorithm for thoracobiliary fistulas, as shown in Fig. 9.
Biliary-genitourinary fistula
Fistulous connections between the biliary tract and GU organs may occur spontane-
ously or consequent to accidental or operative trauma but are extremely unusual.
Connections have been described between the gallbladder or common bile duct
and the right renal pelvis, the urinary bladder, and the embryonic remnant ura-
chus.7982 Rabinowitz and colleagues82 reported on an elderly man with multiple ep-
isodes of urinary tract infections who was found to have gallstones in his urinary
bladder. Retrograde injection of a sinus at the dome of the bladder confirmed a
connection between the urachal remnant and the gallbladder. Definitive repair
involved resection of the gallbladder, bladder dome, and the urachal remnant. Fistu-
lization between the common bile duct and a neobladder ileal conduit has been suc-
cessfully managed endoscopically.83 Gallstone migration into GU structures has been
observed as a complication of gallstones lost at the time of cholecystectomy.8486
Symptoms include recurrent urinary tract infections and passage of gallstones
through the urethra. Gallstones in the urinary bladder may be amenable to cystoscopic
388 Luu & Deziel
extraction with spontaneous closure of the fistula tract. If the stones are multiple or
embedded in the bladder wall, operative removal may be necessary.
Although not specifically due to gallstone disease, a host of other rare urinary fis-
tulas have been described involving upper abdominal organs, including nephrohepatic
fistulas,87 pyelo-duodenal fistulas from pyonephrosis,88,89 and unusual fistulas from
migration of biliary stents.90 Posttraumatic nephro-bilary fistula has been treated by
percutaneous embolization.91
External
Spontaneous cholecystocutaneous fistula was once a common complication of
gallstone disease but now is a rare condition because of early diagnosis and treat-
ment. A total of 226 cases have been reported since the early twentieth century and
fewer than 25 cases have been reported in the past 50 years.92,93 Most patients are
women older than 50 years and most fistulas drain externally to the right upper quad-
rant. Other locations, such as chest wall, umbilicus, right groin, or gluteus, have been
reported.92,94,95
LOST GALLSTONES
Fig. 10. Diagram of possible complications of lost gallstones. (From Zehetner J, Shamiyeh A,
Wayand W. Lost gallstones in laparoscopic cholecystectomy: all possible complications. Am J
Surg 2007;193(1):738.)
Suggestions to minimize the spillage of stones and the resulting complications are
early controlled decompression of dilated gallbladders, early recognition and control
of the operation by the attending physician during a difficult case or with an inexperi-
enced trainee, and meticulous survey of the abdomen for spilled stones.
390 Luu & Deziel
SUMMARY
The management of gallstone disease is commonplace for general surgeons. Yet, rar-
ities exist and, although not unique, their appearances may be seldom and so sepa-
rated in time as to elude recognition. The wary clinician who is cognizant of
anomalous biliary passages and stone migrations will be better prepared to handle
these unusual conditions that are benign, but potentially fatal and properly curable.
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