Biliary fistulae can be internal or external. External biliary fistulae, in turn, can be further subdivided based on etiology into spontaneous, therapeutic, traumatic, and iatrogenic fistulae. Spontaneous cholecystocutaneous fistula is a rare complication of neglected calculous biliary disease that has become even more increasingly rare because of prompt diagnosis and expedient surgical intervention for gallstones. Although most spontaneous cholecystocutaneous fistulae are related to underlying gallstones, even more rarely, it may be related to an underlying adenocarcinoma of the gall bladder. 
Spontaneous cholecystocutaneous fistula was first described by Thilesus in 1670.  Prior to 1900, 3 large series were published in quick succession, including a report by Courvoisier in 1890 (169 of 499 cases of gallbladder perforation), 1 by Naunyn in 1896 (184 cases), and another by Bonnet in 1897 (122 cases). [3, 4, 5, 6] These large case series prior to the 20th century reflect the state of surgical care at the time. However, with improved surgical care, the incidence of spontaneous cholecystocutaneous fistula has declined dramatically, with most cases now originating from developing countries or from elderly, institutionalized patients in developed countries.
History of the Procedure
Since the advent of surgical treatment of gallbladder calculous disease, the incidence of cholecystocutaneous fistula has reduced dramatically; from 1890-1949, only 37 cases were identified in the published literature.  A literature review of cases published in the last 52 years reveals just over 50 cases (see Table). The declining incidence has been attributed to prompt diagnosis, availability of antibiotics, and early surgical intervention for cholecystitis and empyema (see Pathophysiology). The decreasing incidence is further confirmed by the availability of large series published prior to the 20th century, in contrast to more recent literature, which consists mainly of individual case reports.
Table. Case Reports of Cholecystocutaneous Fistula in the Past 52 Years from 1961 to 2013 (Open Table in a new window)
|Author(s)||Year Published||Number of Cases||Country of Origin|
|Sodhi et al ||2012||1||India|
|Ozdemir et al ||2012||1||Turkey|
|Andersen and Friis-Andersen ||2012||1||Denmark|
|Ioannidis et al ||2012||1||Italian|
|Baty et al ||2011||1||Australia|
|Cheng et al ||2011||1||Taiwan|
|Khan et al ||2011||1||Ireland|
|Gordon et al ||2011||1||United States of America|
|Sayed et al ||2010||1||United Kingdom|
|Pezzilli et al ||2010||1||Italy|
|Metsemakers et al ||2010||1||Belgium|
|Tallon Aquilar et al ||2010||1||Spain|
|Hawari et al ||2010||1||United Kingdom|
|Gandhi et al ||2009||1||New Zealand|
|Murphy et al ||2008||1||United Kingdom|
|Ijaz et al ||2008||1||United Kingdom|
|Chatterjee et al ||2007||1||India|
|Malik et al ||2007||1||United Kingdom|
|Nagral et al ||2007||1||India|
|Marwah et al ||2007||1||India|
|Shrestha et al ||2006||1||United Kingdom|
|Cruz et al ||2006||1||Brazil|
|Salvador-Izquierdo et al ||2006||1||Spain|
|Yuceyar et al ||2005||1||Turkey|
|Khan et al ||2005||1||Saudi Arabia|
|Dutriaux et al ||2005||1||France|
|Gossage et al ||2004||1||United Kingdom|
|Vasanth et al ||2004||1||United States of America|
|Mathonnet et al ||2002||1||France|
|Chang et al ||2002||1||Taiwan|
|Flora et al ||2001||1||United Kingdom|
|Ramos Rincon et al ||2001||1||Spain|
|Nicholson et al ||1999||1||United States of America|
|Avital et al ||1998||1||Israel|
|Kumar ||1998||1||United States of America|
|Andley et al ||1996||1||India|
|Birch et al ||1991||1||United Kingdom|
|Carragher et al ||1990||1||United Kingdom|
|Rosario et al ||1990||1||United States of America|
|Sevonius et al ||1988||1||Sweden|
|Gibson et al ||1987||1||United Kingdom|
|Bilanovic et al ||1987||1||Croatia|
|Tuna et al ||1986||1||United States of America|
|Hakaim et al ||1986||1||United States of America|
|Rye et al ||1985||1||Denmark|
|Kulicki et al ||1984||1||Poland|
|Davies et al ||1984||1||United Kingdom|
|Abril et al ||1984||1||United States of America|
|Ulreich et al ||1983||1||United States of America|
|Hoffman et al ||1982||1||United States of America|
|Fitchett et al ||1970||1||United States of America|
|Callen ||1979||1||United States of America|
A fistula is an epithelium-lined tract between 2 epithelium-lined surfaces. Thus, a cholecystocutaneous fistula is an abnormal epithelial tract that allows communication between the gallbladder and the skin. This communication can be either spontaneous or deliberate as in the case of a therapeutic percutaneous cholecystostomy used to treat cholecystitis or empyema of the gallbladder, which is generally reserved for patients unfit for surgical intervention, while the former is often a complication of neglected gallstone disease.
Spontaneous cholecystocutaneous fistula is rare. No data exist for the incidence of this condition in the United States. In a retrospective review in Greece, of 210 cases of external biliary fistulae over a 22-year period, only 1 was due to spontaneous cholecystocutaneous fistula. 
To emphasize the rarity of this condition, from 1890-1949, only 37 published cases were found worldwide. After a careful search of indexed and nonindexed journals, just over 50 cases have been reported worldwide (see Table above). A closer look at the Table also reveals that just over half the cases have been reported in the past 10 years, which reflects the increased number of avenues for publication of case reports and recognition that the condition is rare.
This condition is invariably a complication of neglected gallstone disease, although isolated case reports have described spontaneous cholecystocutaneous fistula due to carcinoma of the gallbladder and acalculous cholecystitis.  Carcinoma of the gallbladder can cause cystic duct obstruction, which leads to inflammation in a manner similar to that of gallstones. 
In addition, retained gallstones following laparoscopic cholecystectomy may cause biliary fistula or abdominal wall sinuses. This occurs because gallstones can harbor bacteria, which may form a localized abscess with fistula or sinus in an attempt to discharge the foreign body. [63, 64] However, this complication of cholecystectomy is relatively uncommon despite the relatively common occurrence of stone spillage. Some authors recommend the liberal use of retrieval bags during surgery to avoid stone spillage and subsequent complications of retained stones. 
Salmonella typhi, which has a predilection for the gallbladder, can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula.  Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition. 
The cystic duct or gallbladder is almost always obstructed in patients with spontaneous cholecystocutaneous fistula. In the presence of obstruction, the gallbladder distends and the pressure within rises, impairing the vascular supply. The obstruction and impaired blood supply result in inflammation and may cause focal areas of necrosis. This inflammatory process is typically insidious and recurrent. Surrounding structures wall off the focal area of necrosis. Perforation of the gallbladder may occur, causing a localized cholecystic abscess. In an attempt to discharge this abscess, a fistula may therefore form between the gallbladder and the duodenum, colon, or abdominal wall.
In spontaneous cholecystocutaneous fistula, the abscess is walled off by the abdominal wall and progressively penetrates it. The fistula usually occurs via the fundus of the gallbladder, as this is the farthest from the cystic artery and most likely to be affected in inflammation-caused ischemia. The cholecystic abscess may initially cause a tender area in the abdominal wall and spontaneously rupture, forming a fistula with drainage onto the skin.
Because of the anatomy and position of the gallbladder, the gallbladder is more likely to adhere to neighboring viscera, such as the duodenum and colon, forming cholecystoduodenal fistula that predisposes to gallstone ileus or cholecystocolonic fistula. Similar to cholecystocutaneous fistula, the incidence of cholecystoduodenal fistula has also declined because of expedient surgical intervention.
Although patients with cholecystocutaneous fistula tend to be elderly, the condition has been reported in patients in their third decade of life. Similarly, young patients are likely to have neglected their symptoms for a period or have neuropathy that causes altered sensation.  Women are also affected more than men, reflecting the higher incidence of cholelithiasis and cholecystitis among women.
Most patients have a history suggestive of biliary disease; however, these symptoms are unlikely to be severe, as an acute attack would have precipitated early presentation for surgical intervention. Depending on the stage of progression, patients may present with empyema necessitatis or a discharging sinus.  Empyema necessitatis simply refers to a cholecystic abscess prior to rupture. The patient may report systemic symptoms, such as fevers, sweats, and anorexia associated with the infection, or a tender lump at the site of impending perforation. Patients in whom fistulae have discharged may report loss of bilious fluid or small stones via the external opening. The fistula itself is usually painless.
The patient may be febrile and diaphoretic because of the infection. Prior to rupture, a raised, erythematous, tender, hot area of affected skin may be observed (see the image below). The surrounding skin is often cellulitic, frequently leading to an initial diagnosis of abscess. An associated lump under the skin may be observed if the gallbladder has herniated through the overlying tissue or if an associated malignancy is present.
The external opening is usually in the right upper quadrant, although external openings in the periumbilical area, the lumbar area, and even the gluteal area have been described. The discharge varies depending on whether an obstruction is present. Discharge may be purulent in the presence of empyema, mucoid in the presence of a mucocele because of obstruction, or bilious in the absence of obstruction. Small stones within the discharge often confirm the diagnosis.
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