Cholecystocutaneous Fistula

Updated: Sep 13, 2017
  • Author: Cherry Ee Peck Koh, MBBS, MS, FRACS; Chief Editor: John Geibel, MD, DSc, MSc, AGAF  more...
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Overview

Background

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. 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. [1, 2]

Spontaneous cholecystocutaneous fistula was first described by Thilesus in 1670. [3] 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). [4, 5, 6, 7] 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.

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Pathophysiology

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. As with cholecystocutaneous fistula, the incidence of cholecystoduodenal fistula has also declined because of expeditious surgical intervention.

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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. [8] Carcinoma of the gallbladder can cause cystic duct obstruction, which leads to inflammation in a manner similar to that of gallstones. [9]

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. [10, 11] 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. [12]

Salmonella typhi, which has a predilection for the gallbladder, can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula. [13] Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition. [13]

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Epidemiology

Spontaneous cholecystocutaneous fistula is rare. Since the advent of surgical treatment of gallbladder calculous disease, the incidence of cholecystocutaneous fistula has reduced dramatically. Between 1890 and 1949, only 37 cases were identified in the published literature. [7]  A literature review of cases published between 1961 and 2013 identifies just over 50 cases (see Table 1 below). 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. [14]

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 [15] 2012 1 India
Ozdemir et al [16] 2012 1 Turkey
Andersen and Friis-Andersen [17] 2012 1 Denmark
Ioannidis et al [18] 2012 1 Italian
Baty et al [19] 2011 1 Australia
Cheng et al [20] 2011 1 Taiwan
Khan et al [21] 2011 1 Ireland
Gordon et al [22] 2011 1 United States of America
Sayed et al [23] 2010 1 United Kingdom
Pezzilli et al [24] 2010 1 Italy
Metsemakers et al [25] 2010 1 Belgium
Tallon Aquilar et al [26] 2010 1 Spain
Hawari et al [27] 2010 1 United Kingdom
Gandhi et al [28] 2009 1 New Zealand
Murphy et al [29] 2008 1 United Kingdom
Ijaz et al [30] 2008 1 United Kingdom
Chatterjee et al [31] 2007 1 India
Malik et al [32] 2007 1 United Kingdom
Nagral et al [33] 2007 1 India
Marwah et al [34] 2007 1 India
Shrestha et al [35] 2006 1 United Kingdom
Cruz et al [36] 2006 1 Brazil
Salvador-Izquierdo et al [37] 2006 1 Spain
Yuceyar et al [38] 2005 1 Turkey
Khan et al [39] 2005 1 Saudi Arabia
Dutriaux et al [40] 2005 1 France
Gossage et al [41] 2004 1 United Kingdom
Vasanth et al [42] 2004 1 United States of America
Mathonnet et al [43] 2002 1 France
Chang et al [44] 2002 1 Taiwan
Flora et al [45] 2001 1 United Kingdom
Ramos Rincon et al [46] 2001 1 Spain
Nicholson et al [47] 1999 1 United States of America
Avital et al [48] 1998 1 Israel
Kumar [49] 1998 1 United States of America
Andley et al [50] 1996 1 India
Birch et al [51] 1991 1 United Kingdom
Carragher et al [52] 1990 1 United Kingdom
Rosario et al [53] 1990 1 United States of America
Sevonius et al [54] 1988 1 Sweden
Gibson et al [55] 1987 1 United Kingdom
Bilanovic et al [56] 1987 1 Croatia
Tuna et al [57] 1986 1 United States of America
Hakaim et al [58] 1986 1 United States of America
Rye et al [59] 1985 1 Denmark
Kulicki et al [60] 1984 1 Poland
Davies et al [61] 1984 1 United Kingdom
Abril et al [62] 1984 1 United States of America
Nayman [63] 1983 1 Australia
Ulreich et al [64] 1983 1 United States of America
Hoffman et al [65] 1982 1 United States of America
Fitchett et al [66] 1970 1 United States of America
Callen [67] 1979 1 United States of America
Orr [68] 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 more recent 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 have neuropathy that causes altered sensation. [65]  Women are also affected more than men, reflecting the higher incidence of cholelithiasis and cholecystitis among women.

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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. [12]

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