Spontaneous cholecystocutaneous fistula.

Abstract: Spontaneous cholecystocutaneous fistula is rarely observed today because of the early diagnosis and management made possible by ultrasonography, broad-spectrum antibiotics, and effective surgical management of biliary tract disease. We present a case of spontaneous cholecystocutaneous fistula due to cholecystitis.

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Spontaneous cholecystocutaneous fistula is rarely observed today because of the early diagnosis and management made possible by ultrasonography, broad-spectrum antibiotics, and effective surgical management of biliary tract disease. We present a case of spontaneous cholecystocutaneous fistula due to cholecystitis.

Discussion

Cholecystocutaneous fistula was first reported by Thilesus (1) in 1670. Courvoisier (1) documented 499 cases of gallbladder perforation in the late 19th century; 169 of these cases formed cutaneous tracts. Over the past century, there have been fewer accounts of this problem because of prompt and safe management of biliary tract disease. In their review in 1949, Henry and Orr (1) found 36 cases of external biliary fistulas reported after 1890. During the past 50 years, fewer than 20 cases of spontaneous cholecystocutaneous fistulas have been reported.

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Cholecystocutaneous fistulas are almost always a result of neglected biliary tract disease. Patients with this complication usually do not report a distinct episode of acute cholecystitis in their history, since this would have brought such a patient to medical attention sooner. Patients are usually women over the age of 60. (2) The female predilection is most likely due to the higher incidence of cholecystitis in women than in men. (1) Also, cases have been documented in patients as young as 24 years. (3) Symptoms of underlying disease may be neglected by the patient or may be masked by some underlying neuropathy. (4) The fistula usually presents itself as an enlarging mass before spontaneous rupture, and it may be associated with symptoms of upper abdominal colic, dyspepsia, jaundice, or weight loss. (4)

These fistulas are painless and commonly appear in the right upper quadrant. However, cholecystocutaneous fistulas have also been observed at the umbilicus, left costal margin, right iliac fossa, right groin, and back. (4) Nicholson et al (5) even reported a case of spontaneous cholecystocutaneous fistula in the right gluteal region. The external opening of such fistulas has also been observed to occur at the site of an abdominal scar from previous surgical drainage. (6) The gallbladder may even herniate beneath subcutaneous tissue before fistula formation. (7) The external opening of the fistula can be confused with a pyogenic granuloma, infected epidermal inclusion cyst, or metastatic carcinoma. (4) The discharge from the fistula may be purulent and mucoid if the cystic duct is obstructed by a stone. In cases in which the cystic duct may be patent, bile may drain via the external opening. (1)

The pathophysiology of this condition involves obstruction of the cystic duct, most commonly due to calculi and rarely due to gallbladder carcinoma. (5) Obstruction of the cystic duct causes increased pressure in the gallbladder, leading to impaired blood flow and lymph supply to the gallbladder; this can lead to mural necrosis and perforation. Perforation can occur as 1) acute-free perforation causing peritonitis, 2) subacute perforation resulting in an abscess around the gallbladder, or 3) chronic perforation with the formation of an internal or external biliary fistula. (5) These fistulas usually arise from the fundus of the gallbladder. (6) Chronic inflammation of the gallbladder can cause the gallbladder fundus to adhere to the abdominal parietes, triggering the formation of a fistula tract. The state preceding spontaneous rupture has been termed "empyema necessitatis" by Nayman. (8) This term essentially describes a "burrowing abscess" of the abdominal wall as a result of gallbladder inflammation.

Imaging plays an important role in the diagnosis of this complication. Before fistula formation, the abscess can be diagnosed via ultrasonography, with findings that include a sonolucent mass with echogenic material adjacent to the anterior abdominal wall. (9) The diagnosis of a cutaneous fistula is confirmed with a fistulogram, which allows visualization of its origin and course. (5) Ultrasonography and CT imaging can also help in the diagnosis of this complication. A fistulogram was not done in our patient because her clinical presentation, analysis of the fistula drainage, and CT findings yielded a high index of suspicion for a cholecystocutaneous fistula. Ultimately, our patient had prompt surgical management.

Management of cholecystocutaneous fistula should initially include control of any acute inflammatory process. This can be done by incision and drainage of the sinus abscess, followed by wound cultures and appropriate antibiotic therapy. Our case was unique, since the wound cultures grew methicillin-resistant S. aureus without any coliforms. Surgically, the fistula tract can be laid open, with removal of any gallstones present. (10) Intraoperatively the gallbladder usually appears small, contracted, chronically inflamed, and adherent to the parietes. (2) A cholecystectomy should also be done. Some evidence indicates that the excision of the fistula tract decreases the risk for cancer developing in this tissue. Although adenocarcinoma in association with old wounds, scars, draining sinuses, and chronic inflammatory tracts has been well documented, there has been only one reported case of adenocarcinoma in association with a cholecystocutaneous fistula in particular. (11) In this case, the fistula formation preceded the adenocarcinoma by approximately 20 years. (11) Of the 36 cases of external biliary fistulas recorded between 1890 and 1948 and reviewed by Henry and Orr, (1) spontaneous healing was noted in eight cases. It is plausible that spontaneous healing may be due to the absence or elimination of factors such as persistent gallstones, infection, neoplasia, or epithelialization of the fistula tract, which are known to maintain the patency of a fistula tract. Spontaneous healing may be an option for patients with prohibitive surgical risks. (4)

The possibility of cholecystocutaneous fistula should be considered in any patient who has a discharging sinus in the right upper abdominal or chest wall. A fistulogram can make a definitive diagnosis of this complication and thus reduce morbidity via prompt cholecystectomy and excision of the fistula tract. Clinical presentation, analysis of sinus drainage, and radiologic or ultrasound imaging may also provide valuable information in making this diagnosis.

Key Points

* Cholecystocutaneous fistula is almost always a result of neglected binary tract disease and presents most often in female patients over the age of 60.

* A cholecystocutaneous fistula usually presents as an enlarging mass before rupture, most commonly in the right upper quadrant of the abdomen.

* Ultrasonography or computed tomography and fistulography play an important role in the diagnosis of cholecystocutaneous fistula.

Children are a great comfort in your old age--and they help you to reach it faster, too.

--Lionel M. Kauffman

Accepted March 25, 2002.

Copyright [c] 2004 by The Southern Medical Association

0038-4348/04/9702-0183

References

(1.) Henry CL, Orr TG Jr. Spontaneous external biliary fistulas. Surgery 1949;26:641-646.

(2.) Rosario P, Gerst P, Prakash K, et al. Cholecystocutaneous fistula: An unusual presentation. Am J Gastroenterol 1990;85:214-215.

(3.) Andley M, Biswas RS, Ashok S, et al. Spontaneous cholecystocutaneous fistula secondary to calculous cholecystitis. Am J Gastroenterol 1996;91:1656-1657.

(4.) Hoffman L, Beaton H, Wantz G. Spontaneous cholecystocutaneous fistula: A complication of neglected biliary tract disease. J Am Geriatr Soc 1982;30:632-634.

(5.) Nicholson T, Born M, Garber E. Spontaneous cholecystocutaneous fistula presenting in the gluteal region. J Clin Gastroenterol 1999;28:276-277.

(6.) Abril A, Ulfohn A. Spontaneous cholecystocutaneous fistula. South Med J 1984;77:1192-1193.

(7.) Carragher AM, Jackson PR, Panesar KJ. Subcutaneous herniation of gallbladder with spontaneous cholecystocutaneous fistula. Clin Radiol 1990;42:283-284.

(8.) Nayman J. Empyema necessitatis of the gallbladder. Med J Aust 1963;1:429-430.

(9.) Ulreich S, Henken EM, Levinson ED. Imaging in the diagnosis of cholecystocutaneous fistulae. J Can Assoc Radiol 1983;34:39-41.

(10.) Gibson TC, Howat JM. Cholecystocutaneous fistula. Br J Clin Pract 1987;41:980-982.

(11.) Gifford J, Saltzstein SL, Bavone RM. Adenocarcinoma occurring in association with a chronic sinus tract and biliary fistula. Cancer 1981;47:2093-2097.

RELATED ARTICLE: Case Report

An 80-year-old female nursing home resident had a history of multi-infarction dementia, multiple sclerosis, deep vein thrombosis, and pulmonary embolism. Her primary care physician saw her because of an anterior chest wall mass just below the right breast. A course of oral antibiotics produced no improvement in symptoms. Nineteen days later, when she was brought to St. Elizabeth' Medical Center, a thick, yellow, purulent discharge with some evidence of blood was draining from this mass. On presentation, the patient was afebrile withstable vital signs. She was obtunded. Physical examination showed an erythematous mass 4 cm below the right breast. The white blood cell (WBC) count was 12,500/m[m.sup.3], and results of liver function studies and other tests were within normal limits. Chest x-ray films showed no empyema. Incision and drainage yielded 300 ml of frank pus from the site. She was treated with local wound care and intravenous antibiotics. Wound cultures grew methicillin-resistant Staphylococcus aureus, and antibiotics were adjusted appropriately to the sensitivities. The patient was afebrile over the next 48 hours, and the WBC count decreased to 9,200/m[m.sup.3] in response to treatment. She was discharged to her nursing home in stable condition.

The patient returned 5 days later with increased yellow drainage from what had now become a right anterior chest wall sinus. She was afebrile with stable vital signs. Physical examination showed a 2 X 2 cm sinus below the right breast covered by an ostomy bag that had collected bilious material. At admission, the WBC count was 10,800/m[m.sup.3]; all other laboratory values were within normal limits. The sinus drainage material revealed a bilirubin value of 25.0, lipase value of 119, and amylase value less than 30. Computed tomography (CT) of the chest and abdomen with contrast medium showed inflammatory changes involving the gallbladder, particularly the fundus, and also involving adjacent fat, fascial planes, and anterior chest wall (Fig. 1). The patient was taken to the operating room. Intraoperatively, an inflamed fistula tract was seen emanating from the rightlower anterior chest wall to above the costal margin, entering the peritoneum over the liver edge and into the gallbladder. A cholecystectomy with cauterization of the fistula tract was done without complications. The patient' postoperative course was relatively unremarkable, and she was discharged with a 2-week course of antibiotics.

Adarsh Vasanth, MD, Aqeel Siddiqui, MD, and Kevin O'Donnell, MD

From the Department of Surgery, Caritas St. Elizabeth's Medical Center of Boston.

Reprint requests to Kevin O'Donnell, MD, Department of Surgery, St. Elizabeth's Medical Center of Boston, 736 Cambridge Street, Boston, MA 02135.