Cholecystocutaneous Fistula 

Updated: Jul 09, 2019
Author: Cherry Ee Peck Koh, MBBS, MS, FRACS; Chief Editor: John Geibel, MD, MSc, DSc, AGAF 

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.[1] 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.[2, 3]

Spontaneous cholecystocutaneous fistula was first described by Thilesus in 1670.[4] 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).[5, 6, 7, 8] 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.

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.

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.[9]  Carcinoma of the gallbladder can cause cystic duct obstruction, which leads to inflammation in a manner similar to that of gallstones.[10]

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.[11, 12] 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.[13] Rare cases have been noted after percutaneous cholecystostomy drain placement and removal.[14]

Salmonella typhi, which has a predilection for the gallbladder, can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula.[15]  Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition.[15]

Epidemiology

Spontaneous cholecystocutaneous fistula is rare. Since the advent of surgical treatment of gallbladder calculous disease, the incidence of cholecystocutaneous fistula has fallen dramatically. Between 1890 and 1949, only 37 cases were identified in the published literature.[8]  A literature review of cases published between 1961 and 2013 identified 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 one was due to spontaneous cholecystocutaneous fistula.[16]

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[17]

2012

1

India

Ozdemir et al[18]

2012

1

Turkey

Andersen et al[19]

2012

1

Denmark

Ioannidis et al[20]

2012

1

Italian

Baty et al[21]

2011

1

Australia

Cheng et al[22]

2011

1

Taiwan

Khan et al[23]

2011

1

Ireland

Gordon et al[24]

2011

1

United States of America

Sayed et al[25]

2010

1

United Kingdom

Pezzilli et al[26]

2010

1

Italy

Metsemakers et al[27]

2010

1

Belgium

Tallon Aquilar et al[28]

2010

1

Spain

Hawari et al[29]

2010

1

United Kingdom

Gandhi et al[30]

2009

1

New Zealand

Murphy et al[31]

2008

1

United Kingdom

Ijaz et al[32]

2008

1

United Kingdom

Chatterjee et al[33]

2007

1

India

Malik et al[34]

2007

1

United Kingdom

Nagral et al[35]

2007

1

India

Marwah et al[36]

2007

1

India

Shrestha et al[37]

2006

1

United Kingdom

Cruz et al[38]

2006

1

Brazil

Salvador-Izquierdo et al[39]

2006

1

Spain

Yuceyar et al[40]

2005

1

Turkey

Khan et al[41]

2005

1

Saudi Arabia

Dutriaux et al[42]

2005

1

France

Gossage et al[43]

2004

1

United Kingdom

Vasanth et al[44]

2004

1

United States of America

Mathonnet et al[45]

2002

1

France

Chang et al[46]

2002

1

Taiwan

Flora et al[47]

2001

1

United Kingdom

Ramos Rincon et al[48]

2001

1

Spain

Nicholson et al[49]

1999

1

United States of America

Avital et al[50]

1998

1

Israel

Kumar[51]

1998

1

United States of America

Andley et al[52]

1996

1

India

Birch et al[53]

1991

1

United Kingdom

Carragher et al[54]

1990

1

United Kingdom

Rosario et al[55]

1990

1

United States of America

Sevonius et al[56]

1988

1

Sweden

Gibson et al[57]

1987

1

United Kingdom

Bilanovic et al[58]

1987

1

Croatia

Tuna et al[59]

1986

1

United States of America

Hakaim et al[60]

1986

1

United States of America

Rye et al[61]

1985

1

Denmark

Kulicki et al[62]

1984

1

Poland

Davies et al[63]

1984

1

United Kingdom

Abril et al[64]

1984

1

United States of America

Nayman[65]

1983

1

Australia

Ulreich et al[66]

1983

1

United States of America

Hoffman et al[67]

1982

1

United States of America

Fitchett et al[68]

1970

1

United States of America

Callen[69]

1979

1

United States of America

Orr[70]

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 have neuropathy that causes altered sensation.[67]  Women are also affected more than men, reflecting the higher incidence of cholelithiasis and cholecystitis among women.

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.[13]

 

Presentation

History

Most patients with cholecystocutaneous fistulae have a history suggestive of biliary disease; however, these symptoms are unlikely to be severe, in that 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.[65]  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.

Physical Examination

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.

A 90-year-old man referred with abdominal wall abs A 90-year-old man referred with abdominal wall abscess in the right upper quadrant.

The external opening is usually in the right upper quadrant, though 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.

 

DDx

Diagnostic Considerations

Conditions to be considered in the differential diagnosis include the following:

 

Workup

Laboratory Studies

Blood studies to be considered include the following:

  • Full blood examination (FBE) - Leukocytosis supports an infective and inflammatory process
  • C-reactive protein (CRP) - An elevated CRP level indicates the presence of an infective process
  • Liver function tests (LFTs) and alkaline phosphatase (ALP) - Levels are typically elevated because of extrahepatic duct obstruction (cystic duct); however, jaundice is uncommon, even in the presence of choledocholithiasis [67]

Microbiologic analysis of fluid discharged from the fistula reveals the type of fluid present (eg, bilirubin in bile) and provides bacteriologic results (purulent fluid guides antibiotic therapy). Common offending bacteria include Escherichia coli and Proteus species.

Imaging Studies

The following imaging modalities can be used in the presence of a cholecystocutaneous fistula:

  • Ultrasonography (US)
  • Computed tomography (CT)
  • Fistulography
  • Cholangiography

US is useful for demonstrating gallstones, a thickened gallbladder adjacent to the anterior abdominal wall, and an overlying abdominal wall that is edematous as a consequence of inflammation. Occasionally, it can demonstrate the gallbladder herniating into the subcutaneous tissue.[54] Inflammation of the skin occasionally limits examination because of pain.

CT can demonstrate the unusual position of the gallbladder adhering to the anterior abdominal wall. It also demonstrates the presence of edema, as well as inflammation within the overlying tissue (see the image below). In the event of a malignancy, a heterogeneous mass may be visible.

Computed tomography (CT) scan demonstrating a gros 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.

Fistulography is useful in establishing the diagnosis.[71] The contrast demonstrates the tract and fills the gallbladder. In an unobstructed system, fistulography also demonstrates the common bile duct (CBD), allowing examination of biliary anatomy. Rarely, in the event of multiple fistulae, it demonstrates communication with other, neighboring viscera.

Cholangiography demonstrates biliary anatomy and excludes the concomitant presence of a CBD stone, which should be addressed during resection of the fistula and gallbladder. If the patient is to be treated conservatively, this is particularly important, in that an obstructed CBD can prevent spontaneous fistula closure.

 

Treatment

Medical Therapy

All patients should be treated with antibiotics because of associated sepsis, cholecystitis, or empyema. However, antibiotics are an adjunctive therapy and should not be the only treatment.

Surgical Therapy

Both the gallbladder and fistula must be resected to achieve a cure. However, given that this condition commonly occurs in elderly patients who may have multiple medical comorbidities, surgical treatment must be tailored according to the patient's fitness for surgery.

Choice of surgical approach

Several decisions must be made at the time of surgery, including whether to use a one-stage or a staged procedure (drainage of abscess with surgical excision of gallbladder and fistula vs drainage of abscess to control sepsis followed with definitive treatment of gallbladder disease and fistula). Other considerations include the incision site, whether to incorporate the external opening into the incision, and which method of closure to use (see Operative Details below).

Drainage of the cholecystocutaneous abscess prior to spontaneous discharge turns the abscess into a fistula and allows control of sepsis. Appropriate intravenous (IV) antibiotics should be initiated as well. A drainage tube may be inserted into the fistula to keep the tract patent. However, only an experienced physician should perform this maneuver; a nonfibrous tract can easily be perforated. The tract can also be gently dilated to allow the passage of forceps to remove stones within the gallbladder. Drainage of the abscess also offers temporizing care while transfer to a specialized institution is under way should further expertise be required.

For definitive treatment of the underlying gallbladder disease or fistula, either a laparoscopic or an open approach can be considered. A laparoscopic approach is less invasive; however, the conversion rate may be high, depending on the intra-abdominal findings (eg, adhesions to the surrounding tissue).[51] Port placement may also have to be altered, depending on adhesions to the surrounding tissue and the course of the fistula tract. An open approach should be used if an underlying malignancy is suspected.

Conservative treatment vs definitive excision

Definitive surgical excision of both the gallbladder and the tract is the treatment of choice. However, in the surgically prohibitive patient, conservative management can be considered.

Indications for conservative management include the patient's inability to tolerate anesthesia that permits definitive surgical treatment or a poor prognosis (eg, coexisting advanced malignancy) such that surgical intervention is not warranted. With progress in the safety of surgery and anesthesia, there are in fact very few absolute contraindications, and management options depend on local expertise and tailored treatment to suit the patient’s clinical status.

In conservative management, spontaneous healing of the fistula can occur, provided that there is no distal biliary tree obstruction.[8, 72] In the 1949 series from Henry et al, six of 37 patients (16%) experienced spontaneous healing.[8] Incision and drainage of the cholecystic abscess without definitive excision of the tract or gallbladder led to spontaneous healing in three more. Whereas this report demonstrates that simple drainage and conservative treatment can lead to healing in a substantial number of patients (24%) with spontaneous cholecystocutaneous fistula, it also shows that most require formal surgical intervention.

Preparation for surgery

Before surgical treatment, an infective or inflammatory process should be adequately treated with antibiotics. Bacteriologic studies are helpful in guiding antibiotic therapy.

Consider performing ultrasonography (US) and fistulography (see Imaging Studies).[71]

Patient consent should be obtained for an open excision of the gallbladder and fistula. For patients with choledocholithiasis, open common bile duct exploration should be discussed, although a separate, endoscopic procedure can also be performed.

Operative details

Considerations include the incision site, whether to incorporate the external opening into the incision, and method of closure. The procedure can be performed via a midline laparotomy or a subcostal incision. Many choose to close the muscle and fascia at the fistula site but leave the skin to heal by secondary intention.

In attempting this procedure laparoscopically, perform the gallbladder dissection in the usual fashion to reveal the cystic artery and duct. After ligation of both the cystic duct and the cystic artery, the gallbladder must be mobilized off the liver bed and abdominal wall fistula or abscess. Significant edema and thickening of the gallbladder hilum may complicate laparoscopic dissection.

In either technique, a drain should be inserted in the subhepatic space if a subhepatic collection is found.

Complications

Cholecystocutaneous fistula is a complication of neglected cholelithiasis.[1, 14]

Prior to discharge of the fistula, the patient may be septic. Necrotizing fasciitis of the anterior abdominal wall due to sepsis has been reported.[65]

Following discharge of the fistula, bilious discharge can cause skin irritation and dermatitis.

Chronic untreated fistulae can lead to dysplasia and subsequent malignant change similar to Marjolin ulcer. This is rare, and a review of the literature has revealed only one case of adenocarcinoma arising from a biliary fistula tract.[73]

Long-Term Monitoring

If the patient's wounds are allowed to heal through secondary intention, he or she should be observed until healing is complete.

Patients who are treated conservatively should be observed to ensure tract closure and to confirm that adequate skin care is provided to prevent skin irritation.