Continuing Education Activity

Cholecystocutaneous fistulas are a rare surgical condition. This activity reviews their clinical presentation, underlying causes, pathophysiology, and potential complications and highlights the role of the interprofessional team in evaluating and managing patients with this condition.

Objectives:

• Review the epidemiology of cholecystocutaneous fistula.
• Describe the pathophysiology of cholecystocutaneous fistulas.
• Identify principles of evaluation and management of cholecystocutaneous fistulas.
• Outline recommendations to the health care interprofessional team to improve the management of cholecystocutaneous fistulas to enhance patient outcomes.

Introduction

A fistula is known in the medical field as an abnormal communication between two epithelial surfaces. This abnormal communication can result as a complication of a disease or surgical intervention.  The naming of the fistula follows the two surfaces or lumens it connects[1]. A cholecysto-cutaneous fistula is, therefore, abnormal communication between the gallbladder and the skin. Similarly, entero-cutaneous fistula is an abnormal communication between the small bowel and the skin.

Cholecysto-cutaneous fistula is a rare clinical entity in the surgical practice, nowadays. Reports of spontaneous cholecysto-cutaneous fistulae occurring as early as the 17th century were registered. The condition develops as a result of a complication from neglected calculous cholecystitis. However, it occurs extremely infrequently in the current surgical practice. With the advancement in diagnostic imaging, biliary tract diseases are diagnosed early nowadays. The availability of efficient and safe surgical treatment made the complication like fistula extremely uncommon.[2][3][4] 

Etiology

Cholecysto-cutaneous fistulas present as an uncommon complication of neglected calculous cholecystitis. This condition has also been associated with acalculous cholecystitis or carcinoma of the gall bladder. A significant proportion of patients with this condition present with nonspecific symptoms and rarely disclose a history of previous gallbladder disease.[5] Once the gallbladder wall is inflamed, it starts to necrosis if left untreated. Complete wall necrosis results in leaking of the bile into the adjacent tissue. One of the potential sites of leaking is the abdominal wall and eventually the covering skin resulting in a cholecysto-cutaneous fistula. 

Epidemiology

Due to the rarity of the condition, the available information is limited. The affected patients are usually older females. When questioned, patients may mention previous episodes of right upper quadrant pain. This pain hints at gallstone disease. The most common site of the fistulous opening is the right upper quadrant of the abdomen. Painless, draining fistulas associated with this condition have been reported in many locations on the abdominal wall such as right upper quadrant, right iliac fossa, umbilicus, right lumbar region, left lower quadrant, and even as far as the gluteal region. Gallbladder fistulas have been known to occur following percutaneous treatment for acute cholecystitis and reoccurrence of cholecystitis with the fistula forming along the previous track of the cholecystostomy drain.[6][7]

Pathophysiology

Obstruction of the biliary outflow by a gallstone is thought to play a significant role in the pathophysiology of the development of this condition. The obstruction of the cystic duct leads to increased intra-gallbladder pressure. Unrelieved obstruction to the bile outflow leads to compromise of gallbladder wall blood circulation as well as lymphatic drainage which eventually results in necrosis of the gallbladder wall. An open perforation of the gallbladder pericholecystic abscess follows. Once perforated, the gallbladder may drain into the peritoneal cavity, localized causing Perito-ni localized abscess. This abscess can then develop into an external fistula due to its adherence to the abdominal wall. It expels its contents through an area of least resistance or shortest route. Conditions such as polyarteritis nodosa, typhoid, trauma, or drug treatments such as steroids, may be predisposing factors. Carcinoma of the gallbladder is known to present with an external fistula. Cutaneous gallbladder fistula is usually a late sequela of chronic biliary tract disease. It has also been reported after inadequate treatment with acute cholecystitis. 

History and Physical

The clinical presentation may be variable depending on the associated pathology, the age of the patient, and the presence or absence of an associated abdominal wall abscess. The typical clinical presentation is that of a patient's sinus persistently discharging bile. There is usually a history of chronic biliary tract disease even though patients may not report it as a primary complaint. At times the patient may give a history of the expulsion of gallstones through the fistula. The site of the external opening of the fistula is variable and depends on the anatomical course. The commonest site of the outer opening is the right upper quadrant of the abdominal wall. However, the opening of the fistula tract may be present in the left costal margin, right iliac fossa, right groin, or right gluteal region. Patients may be toxic when there is an acute infection or associated abscess. The character of the discharge from the fistula may vary according to the underlying pathology. A lump may be palpable deep to the fistula due to the underlying inflamed or malignant gallbladder.

Evaluation

Ultrasonography is the first modality of imaging in the background of gallstone disease, but it has not been found to be of significant use in gallbladder fistula even though it demonstrates the presence of gallstones reliably. Computed tomography (CT) scan is the most useful imaging modalities for diagnosing this complication. CT imaging will reveal evidence of gallstones and communication to the gallbladder. A CT fistulogram can demonstrate the fistula connecting to the gallbladder and biliary tract which is contributory to the establishment of this diagnosis. A CT fistulogram can be achieved by injecting diluted contrast through the fistulous tract while performing the CT. A CT fistulogram helps to delineate the course of the track accurately and establish the anatomical relationship of this tract. This imaging modality also helps excludes any internal fistula, and other associated pathology. A clear delineation of the fistulous tract helps in planning the approach and extent of surgery. Routine blood tests may not be helpful unless there is an abscess collection. MRI with Cholangiopancreatography (MRCP) has also been found to be useful in establishing the diagnosis.[8][9]

Treatment / Management

The management of cholecyst-cutaneous fistula requires initial drainage of any associated abscess and administration of appropriate antibiotics. If there is an associated mass, it is expedient to obtain a cytologic diagnosis by fine-needle aspiration cytology (FNAC) to exclude an underlying malignancy. If the cytology is negative, or there is no suspicion of malignancy, the next step to follow is an elective cholecystectomy once the patient is stable and his or her general condition optimized. An open cholecystectomy with excision of the fistulous tract is the definitive treatment. Laparoscopic cholecystectomy can be considered to reduce the morbidity since many of these patients will be older with multiple associated comorbidities. Percutaneous treatment with removal of the gallstones only is reserved for the elderly with multiple comorbidities, or for those who are too debilitated to withstand surgery. Before the definitive surgery is performed, an effort must be taken to ensure that there is no associated bile duct obstruction or stones. An indication of an unresectable disease is if a gallbladder fistula occurs in the setting of cancer with an associate dated mass. In this case, the overall outcome is dismal because gallbladder cancer has a predilection for seeding and spreading to the adjoining anatomical structures.[10][11]

Differential Diagnosis

• Chronic osteomyelitis of ribs

• Discharging tuberculoma

• Infected epidermal inclusion cyst

• Metastatic carcinoma

• Pyogenic granuloma

Prognosis

The prognosis of cholecystocutaneous fistula is generally good. Complications may occur in the elderly with underlying comorbid conditions. Malignant change of the fistulous tract is rare. 

Complications

• Necrotizing fasciitis
• Skin irritation
• Malignant changes

Deterrence and Patient Education

Patient education on discharge should focus on adequate wound care and follow up to prevent secondary complications such as infection and septicemia. 

Pearls and Other Issues

In any elderly patient who has a fistula discharging bile on the right abdominal wall, practitioners should consider the possibility of a cholecysto-cutaneous fistula. It is possible a fistula formed in a patient who underwent previous percutaneous treatment of acute cholecystitis and has recurrent cholecystitis. Such patients should be investigated adequately for underlying pathology and associated biliary tract disease.

Enhancing Healthcare Team Outcomes

Cholecysto-cutaneous fistulas are rare today but like any cutaneous fistula need proper drainage and skin protection, while at the same time managing the primary cause. Specialized would care nurses usually provide day to day management of cholecysto-cutaneous fistulas in the same manner as any cutaneous fistula by recording the amount of drainage, characteristics of the drainage and at the same time providing skin protection. The results should be reported to the surgeon. If abnormal, such as slow drainage, pus, or signs of infection, the surgeon should be contacted immediately. The nurse should monitor the patient and provide family support including ongoing education of the patient and family. The prognosis for benign causes is good as long as the primary condition is managed. However, in patients with a malignant cause, the prognosis is guarded and the fistula may not be treatable. It is important for healthcare workers to know that any chronic wound that is left untreated can develop malignant changes after 10-20 years, hence close monitoring by the interprofessional team is necessary The best outcomes are achieved by interprofessional teamwork in treating these challenging cases.[9][8][12]