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Author: Cherry Ee Peck Koh, MBBS, Surgical Registrar, Department of General Surgery, Alfred Hospital, Bayside Health

Cherry Ee Peck Koh is a member of the following medical societies: Royal Australasian College of Surgeons

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

Editors: Brian James Daley, MD, MBA, FACS, Associate Program Director, Professor, Department of Surgery, Division of Trauma and Critical Care, University of Tennessee School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; David L Morris, MD, PhD, Professor, Department of Surgery, St George Hospital, University of New South Wales, Australia; Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy; John Geibel, MD, DSc, MA, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital

Author and Editor Disclosure

Synonyms and related keywords: cholecystocutaneous fistula, spontaneous external biliary fistula, internal biliary fistula, therapeutic biliary fistula, traumatic biliary fistula, iatrogenic biliary fistula, deliberate cholecystocutaneous fistula, cholecystostomy, neglected calculous disease, cholecystoduodenal, cholecystocolonic, sepsis, cholecystitis, empyema, gallbladder carcinoma, acalculous cholecystitis, cystic duct obstruction, gallbladder, gallstones, cystic duct, cholecystic abscess

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.

Clinical

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.

History

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



See Pathophysiology.



See Surgical therapy.



Lab Studies

  • Hematology
    • 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 test (LFT) 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.13
  • Microbiology: 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

  • Ultrasonography: A useful test, this can demonstrate gallstones, a thickened gallbladder adjacent to the anterior abdominal wall, and an overlying abdominal wall that is edematous due to inflammation. Occasionally, ultrasonography can demonstrate the gallbladder herniating into the subcutaneous tissue.15 Inflammation of the skin occasionally limits examination because of pain.  
  • CT scanning: CT scanning can demonstrate the unusual position of the gallbladder adhering to the anterior abdominal wall. CT scanning also demonstrates the presence of edema and inflammation within the overlying tissue (see Media file 2). In the event of a malignancy, a heterogeneous mass may be visible.
  • Fistulography: This is useful in establishing the diagnosis. The contrast demonstrates the tract and fills the gallbladder. In an unobstructed system, fistulography also demonstrates the common bile duct, allowing examination of biliary anatomy. Rarely, in the event of multiple fistulae, it demonstrates communication with other, neighboring viscera.
  • Cholangiography: This demonstrates biliary anatomy and excludes the concomitant presence of a common bile duct stone, which should be addressed during resection of the fistula and gallbladder. If the patient is to be treated conservatively, this is particularly important, as an obstructed common bile duct can prevent spontaneous fistula closure.



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 need to be resected to achieve a cure. However, as this condition commonly occurs in elderly patients who may have multiple medical comorbidities, surgical treatment must be tailored depending on the patient's fitness for surgery.

Surgery

Several decisions must be made at the time of surgery, including whether to use a one-stage versus staged procedure (drainage of abscess with surgical excision of gallbladder and fistula or 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 Intraoperative details).

Drainage of the cholecystocutaneous abscess prior to spontaneous discharge turns the abscess into a fistula and allows control of sepsis. Appropriate intravenous antibiotics should be initiated as well. A drainage tube may be inserted into the fistula to keep the track patent. However, only an experienced physician should perform this maneuver, as 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 underway should further expertise be required.

In the definitive treatment of the underlying gallbladder disease or fistula, either a laparoscopic approach or an open approach can be considered. A laparoscopic approach is less invasive and has been described; however, the conversion rate may be high depending on the intra-abdominal findings, such as adhesions to the surrounding tissue.16 Port placement may also need 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

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 clinical judgment that benefits the patient.

In conservative management, spontaneous healing of the fistula can occur provided there is no distal biliary tree obstruction.5, 17 In the review by Henry and Orr in 1949, of 37 patients within their series, spontaneous healing occurred in 6 patients.5 Incision and drainage of the cholecystic abscess without definitive excision of the tract or gallbladder led to spontaneous healing in 3 more patients. While this report demonstrates that simple drainage and conservative treatment can lead to spontaneous healing in a substantial number of patients with fistulae, it also shows that most require formal surgical intervention.

Preoperative details

Prior to surgery, an infective or inflammatory process should be adequately treated with antibiotics. Bacteriologic studies are helpful in guiding antibiotic therapy.

Consider performing ultrasonography and fistulography (see Imaging Studies).  

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.

Intraoperative details

Considerations include the incision site, whether to incorporate the external opening into the incision, and which method of closure to use. 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 through secondary intention.

In attempting this procedure laparoscopically, perform the gallbladder dissection in the usual fashion to reveal the cystic artery and duct. Following ligation of both cystic duct and 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.

Follow-up

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 adequate skin care to prevent skin irritation.



  • Cholecystocutaneous fistula is a complication of neglected cholelithiasis.
  • Prior to discharge of the fistula, the patient may be septic. Necrotizing fasciitis of the anterior abdominal wall due to sepsis has been reported.7
  • 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.18



Prognosis is generally good. However, as 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.18



Media file 1:  A 90-year-old man referred with abdominal wall abscess in the right upper quadrant.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo

Media file 2:  CT scan demonstrating a grossly inflamed gallbladder with a stone within the gallbladder, with partial gallbladder herniation into overlying parietes, marked overlying tissue inflammation, and fistulous tract.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT



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Cholecystocutaneous Fistula excerpt

Article Last Updated: Mar 13, 2008