eMedicine Specialties > General Surgery > Abdomen

Cholecystocutaneous Fistula

Author: Cherry Ee Peck Koh, MBBS, Surgical Registrar, Department of General Surgery, Alfred Hospital, Bayside Health
Coauthor(s): David Merenstein, MBBS, FRACS, Consulting Staff, General and Endocrine Surgery, Department of Surgery, Monash Medical Centre, Faculty of Medicine, Nursing and Health Services; Consulting Staff, Sandringham and District Hospital, William Angliss Hospital and West Gippsland Hospital; Simon Roger Berry, MBBS, General, UGI, and HPB Surgeon, Surgical Consulting Group, Cabrini Hospital, Australia
Contributor Information and Disclosures

Updated: Mar 13, 2008

Introduction

Biliary fistulae can be divided into internal and external biliary fistulae. External biliary fistulae, in turn, can be further subdivided based on etiology into spontaneous, therapeutic, traumatic, and iatrogenic fistulae. Currently, iatrogenic fistulae complicating biliary tract surgery is the most common cause of external biliary fistula. Spontaneous cholecystocutaneous fistula, a rare surgical condition, is becoming even less common because of prompt diagnosis and expedient surgical intervention for biliary calculous conditions.

Thilesus first described cholecystocutaneous fistula in 1670.¹ Prior to 1900, several large reports were published in quick succession, including a report by Courvoisier in 1890 (169 of 499 cases of gallbladder perforation), one by Naunyn in 1896 (184 cases), and another by Bonnet in 1897 (122 cases).^2,3,4,5 The only one of the 3 series in which all cases of cholecystocutaneous fistulae were autopsy proven, Courvoisier’s series is quoted more widely than the other 2 papers.

History of the Procedure

Since the advent of surgical treatment of gallbladder calculous diseases, the incidence of cholecystocutaneous fistula has reduced dramatically; from 1890-1949, only 37 cases were mentioned in the literature.⁵ A literature review from the past 50 years reveals only a handful of cases. The declining incidence is likely related to prompt diagnosis, availability of antibiotics, and early surgical intervention for cholecystitis and empyema (see Pathophysiology). The decreasing incidence is further confirmed by the large series published prior to the 20th century, in contrast to more recent literature, which consists mainly of individual case reports.

Problem

A fistula is an epithelium-lined tract that connects 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. Spontaneous cholecystocutaneous fistula is often a complication of neglected gallstones, while deliberate cholecystocutaneous fistula (better known as a cholecystostomy) is used to treat cholecystitis or empyema of the gallbladder in patients who are medically unfit for surgical procedures.

Frequency

Spontaneous cholecystocutaneous fistula is a rare condition. 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.⁶

To emphasize the rarity of this condition, from 1890-1949, only 37 published cases were found worldwide. Reviewing the available literature over the past 50 years, fewer than 20 cases have been reported worldwide.

Etiology

This condition is invariably a complication of neglected calculous disease, although isolated case reports have described spontaneous cholecystocutaneous fistula due to carcinoma of the gallbladder and acalculous cholecystitis.⁷  Carcinoma of the gallbladder can cause cystic duct obstruction, which leads to inflammation in a manner similar to that of gallstones.⁸

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.^9,10 Although this is relatively uncommon despite a 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.¹¹

Salmonella typhi, which has a predilection for the gallbladder, can cause chronic cholecystitis and may predispose the patient to spontaneous cholecystocutaneous fistula.¹² Polyarteritis nodosa with gallbladder vasculitis and steroid use causing immunosuppression also may be associated with the condition.¹²

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 thus 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 much more commonly adheres 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 is also declining because of expedient surgical intervention.

Presentation

Epidemiology

Patients tend to be elderly, although cholecystocutaneous fistulae have been reported as early as the third decade of life. Younger patients are likely to have neglected their symptoms for a period of time or have neuropathy that causes altered sensation.¹³ Women are 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.¹⁴ 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.

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. 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.

Differential diagnosis

• Infected epidermal inclusion cyst
• Discharging tuberculoma
• Pyogenic granuloma
• Chronic osteomyelitis of ribs with sequestrum
• Metastatic carcinoma

Relevant Anatomy

See Pathophysiology.

Contraindications

See Surgical therapy.

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