Practice Essentials
A fistula is an epithelium-lined tract between two epithelium-lined surfaces. 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.
A cholecystocutaneous fistula is an abnormal epithelial tract that allows communication between the gallbladder and the skin. This communication can be either spontaneous (often a complication of neglected gallstone disease) 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).
Spontaneous cholecystocutaneous fistula is a rare condition that has become even rarer because of prompt diagnosis and expedient surgical intervention for gallstones. [1, 2] Although most spontaneous cholecystocutaneous fistulae are related to underlying gallstones, they may also, in very rare cases, be related to underlying adenocarcinoma of the gallbladder. [3, 4]
Spontaneous cholecystocutaneous fistula was first described by Thilesus in 1670. Before 1900, three large series were published in quick succession by Courvoisier in 1890 (169 of 499 cases of gallbladder perforation), Naunyn in 1896 (184 cases), and Bonnet in 1897 (122 cases). These large case series reflected the state of surgical care at the time. However, with subsequent improvements in 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.
All patients should be treated with antibiotics; however, antibiotics should not be the only treatment. (See Treatment.) Both the gallbladder and the fistula must be resected to achieve a cure. Surgical treatment must be tailored according to the patient's fitness for surgery. Surgical decisions to be made include whether to use a one-stage or a staged procedure, where to place the incision, whether to incorporate the external opening into the incision, and which method of closure to use.
Pathophysiology
The cystic duct or the 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. As with cholecystocutaneous fistula, the incidence of cholecystoduodenal fistula has also declined because of expeditious surgical intervention.
Etiology
This condition is invariably a complication of neglected gallstone disease, though isolated case reports have described spontaneous cholecystocutaneous fistula due to carcinoma of the gallbladder and acalculous cholecystitis. [5] Carcinoma of the gallbladder can cause cystic duct obstruction, which leads to inflammation in a manner similar to that of gallstones. [6]
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. [7, 8] 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. [9] Rare cases have been noted after percutaneous cholecystostomy drain placement and removal. [10]
Salmonella typhi, which has a predilection for the gallbladder, can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula. [11] Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition. [11]
Epidemiology
Spontaneous cholecystocutaneous fistula is rare. Since the advent of surgical treatment of calculous gallbladder disease, the incidence of cholecystocutaneous fistula has fallen dramatically. Between 1890 and 1949, only 37 cases were identified in the published literature. [12] A literature review of cases published between 1961 and 2013 identified just over 50 cases (see Table 1 below). In a retrospective review from Greece that included 210 cases of external biliary fistulae over a 22-year period, only one case was due to spontaneous cholecystocutaneous fistula. [13]
Table 1. Case Reports of Cholecystocutaneous Fistula From 1961 to 2013 (Open Table in a new window)
Author(s) |
Year Published |
Number of Cases |
Country of Origin |
|||
Sodhi et al [14] |
2012 |
1 |
India |
|||
Ozdemir et al [15] |
2012 |
1 |
Turkey |
|||
Andersen et al [16] |
2012 |
1 |
Denmark |
|||
Ioannidis et al [17] |
2012 |
1 |
Italian |
|||
Baty et al [18] |
2011 |
1 |
Australia |
|||
Cheng et al [19] |
2011 |
1 |
Taiwan |
|||
Khan et al [20] |
2011 |
1 |
Ireland |
|||
Gordon et al [21] |
2011 |
1 |
United States of America |
|||
Sayed et al [22] |
2010 |
1 |
United Kingdom |
|||
Pezzilli et al [23] |
2010 |
1 |
Italy |
|||
Metsemakers et al [24] |
2010 |
1 |
Belgium |
|||
Tallon Aquilar et al [25] |
2010 |
1 |
Spain |
|||
Hawari et al [26] |
2010 |
1 |
United Kingdom |
|||
Gandhi et al [27] |
2009 |
1 |
New Zealand |
|||
Murphy et al [28] |
2008 |
1 |
United Kingdom |
|||
Ijaz et al [29] |
2008 |
1 |
United Kingdom |
|||
Chatterjee et al [30] |
2007 |
1 |
India |
|||
Malik et al [31] |
2007 |
1 |
United Kingdom |
|||
Nagral et al [32] |
2007 |
1 |
India |
|||
Marwah et al [33] |
2007 |
1 |
India |
|||
Shrestha et al [34] |
2006 |
1 |
United Kingdom |
|||
Cruz et al [35] |
2006 |
1 |
Brazil |
|||
Salvador-Izquierdo et al [36] |
2006 |
1 |
Spain |
|||
Yuceyar et al [37] |
2005 |
1 |
Turkey |
|||
Khan et al [38] |
2005 |
1 |
Saudi Arabia |
|||
Dutriaux et al [39] |
2005 |
1 |
France |
|||
Gossage et al [40] |
2004 |
1 |
United Kingdom |
|||
Vasanth et al [41] |
2004 |
1 |
United States of America |
|||
Mathonnet et al [42] |
2002 |
1 |
France |
|||
Chang et al [43] |
2002 |
1 |
Taiwan |
|||
Flora et al [44] |
2001 |
1 |
United Kingdom |
|||
Ramos Rincon et al [45] |
2001 |
1 |
Spain |
|||
Nicholson et al [46] |
1999 |
1 |
United States of America |
|||
Avital et al [47] |
1998 |
1 |
Israel |
|||
Kumar [48] |
1998 |
1 |
United States of America |
|||
Andley et al [49] |
1996 |
1 |
India |
|||
Birch et al [50] |
1991 |
1 |
United Kingdom |
|||
Carragher et al [51] |
1990 |
1 |
United Kingdom |
|||
Rosario et al [52] |
1990 |
1 |
United States of America |
|||
Sevonius et al [53] |
1988 |
1 |
Sweden |
|||
Gibson et al [54] |
1987 |
1 |
United Kingdom |
|||
Bilanovic et al [55] |
1987 |
1 |
Croatia |
|||
Tuna et al [56] |
1986 |
1 |
United States of America |
|||
Hakaim et al [57] |
1986 |
1 |
United States of America |
|||
Rye et al [58] |
1985 |
1 |
Denmark |
|||
Kulicki et al [59] |
1984 |
1 |
Poland |
|||
Davies et al [60] |
1984 |
1 |
United Kingdom |
|||
Abril et al [61] |
1984 |
1 |
United States of America |
|||
Nayman [62] |
1983 |
1 |
Australia |
|||
Ulreich et al [63] |
1983 |
1 |
United States of America |
|||
Hoffman et al [64] |
1982 |
1 |
United States of America |
|||
Fitchett et al [65] |
1970 |
1 |
United States of America |
|||
Callen [66] |
1979 |
1 |
United States of America |
|||
Orr [67] |
1979 |
1 |
Australia |
The declining incidence has been attributed to prompt diagnosis, availability of antibiotics, and early surgical intervention for cholecystitis and empyema (see Pathophysiology). The decline is further confirmed by the availability of large series published before the 20th century, in contrast to the subsequent literature, which consists mainly of individual case reports.
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 to have neuropathy that causes altered sensation. [64] Women are also affected more than men, as a reflection of the higher incidence of cholelithiasis and cholecystitis among women. [1]
Prognosis
Prognosis is generally good. However, given that most patients with this condition are elderly, potential coexisting medical problems may complicate surgical intervention.
Malignant change in the fistulous tract is rare and generally occurs only after 10-20 years. [9]
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A 90-year-old man referred with abdominal wall abscess in the right upper quadrant.
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Computed tomography (CT) scan demonstrating a grossly inflamed gallbladder with a stone within the gallbladder, with partial gallbladder herniation into overlying abdominal wall musculature, marked overlying tissue inflammation, and fistulous tract.