Complications related to endoscopic retrograde cholangiopancreatography (ERCP) include pancreatitis, hemorrhage, cholangitis, and perforation. ERCP-related perforation is uncommon, but mortality rates are high. Diagnosis requires a high clinical suspicion for early detection to allow optimal management of the perforation and a better prognosis. Treatment depends on the location and mechanism and increasingly involves nonoperative management. We report a case of successful nonsurgical treatment of a patient with extensive air involving the peritoneum, retroperitoneum, thorax, mediastinum, and subcutaneous tissues following an ERCP perforation.
Endoscopic retrograde cholangiopancreatography (ERCP) has developed into an essential part of contemporary gastrointestinal practice since its introduction in 1968 [
Perforation is a potentially serious complication of ERCP, leading in some cases to peritonitis, sepsis, and even death. As a result, most perforations after ERCP historically were treated with surgical repair. However, nonsurgical management has been shown to be increasingly successful in the management of most perforations, except for those that occur distant from the ampulla or bile ducts. We report a case of perforation as a complication of ERCP which resulted in large quantity of extraluminal air and was managed successfully without surgical intervention.
A 73-year-old female was hospitalized for sudden onset of abdominal pain, jaundice, elevated aminotransferases, and hyperbilirubinemia. She had previous gastrointestinal surgeries with cholecystectomy and choledochoduodenostomy for congenital biliary obstruction at six months of age and gastroduodenostomy for bowel obstruction due to adhesions at five years. Computed tomography (CT) of the abdomen with contrast revealed dilated intrahepatic ducts and a
The ERCP of note was performed two months later. After extraction of the previously placed biliary stents, cholangiogram showed a persistent biliary anastomotic stricture. Balloon dilatation was performed and then a total of four stents, two 10 Fr. and two 8.5 Fr., were placed across the anastomosis to maintain patency. The patient remained stable throughout the procedure, with close monitoring by anesthesiology, and no complications were noted.
Postoperatively, the patient developed dyspnea and right-sided, nonradiating chest pain. Despite normal oxygen saturation on room air, supplemental oxygen via non-rebreather face mask at 100% provided no relief of her symptoms. She denied any hematemesis, hemoptysis, cough, dysphagia, or abdominal pain. She was afebrile and mildly hypertensive, without tachycardia or tachypnea. Physical exam revealed subcutaneous emphysema of the neck, thorax, abdomen, and proximal upper and lower extremities. Her heart and lung sounds were notably decreased. She had mild abdominal distention, but no peritoneal signs. The remainder of her examination was normal. Laboratory studies, including arterial blood gas, complete blood count with differential, basic metabolic panel, and cardiac enzymes, were unrevealing. Her electrocardiogram showed normal sinus rhythm without any acute changes. Portable chest and abdominal X-rays revealed subcutaneous emphysema, pneumomediastinum, small left pneumothorax, and retroperitoneal and intraperitoneal free air (Figures
Portable chest X-ray showing subcutaneous emphysema, pneumomediastinum, and a left pneumothorax (arrow).
Portable abdominal X-ray showing retroperitoneal and intraperitoneal-free air.
CT scan with intravenous and oral contrast of the thorax, abdomen and pelvis revealed diffuse subcutaneous emphysema (Figures
Coronal section of CT thorax showing subcutaneous emphysema, bilateral pneumothorax, and pneumomediastinum (arrow).
Transverse section of CT thorax showing subcutaneous emphysema, bilateral pneumothorax, and pneumomediastinum (arrow).
Transverse section of CT abdomen showing subcutaneous emphysema, pneumoperitoneum, and pneumoretroperitoneum (arrow).
Transverse section of CT abdomen showing subcutaneous emphysema and pneumoperitoneum.
The patient was admitted to the intensive care unit where she started on broad spectrum antibiotics and bowel rest. She underwent bilateral chest tube placement for decompression of the pneumothroaces. Her dyspnea and chest pain promptly resolved. Additional diagnostic studies, including esophagram, nasopharyngoscopy, and laryngoscopy, showed no gross site of perforation. Serial abdominal and radiographic examinations over the next three days showed partial resorption of peritoneal air. The patient remained hemodynamically stable throughout her treatment. Chest tubes were removed after 3 days, diet advanced without difficulty, and the patient was discharged uneventfully after one week. On follow-up visits, the patient was doing well with no residual symptoms. The biliary stents were removed by a final ERCP four months later. Cholangiogram at that time showed resolution of the anastomotic stricture with effective spontaneous biliary drainage.
Perforation is an uncommon complication of ERCP, with an incidence between 0.3% and 2.1% of procedures [
Although the incidence of ERCP-related perforations is low, mortality has been reported in up to 20% of patients [
There are two main classification systems for ERCP-related perforations. Howard et al. proposed a 3-group classification system based on the mechanism of ERCP-related perforation [ type I: lateral or medial duodenal wall perforations (Howard group III), type II: peri-Vaterian injury (Howard group II), type III: bile or pancreatic duct injury (comparable to Howard group I since majority of these perforations are caused by guidewire instrumentation), type IV: presence of retroperitoneal air alone.
Stapfer type I perforations are due to the endoscope itself, tend to be large, and are usually intraperitoneal. Manipulation of the ampulla during sphincterotomy or other therapeutic measures causes type II perforations, which is the most common type of injury [
Any perforation may present with retroperitoneal air that tracks to the thorax and subsequently into subcutaneous tissues, thus causing subcutaneous emphysema. It has been hypothesized that there are pores in the diaphragm, formed either congenitally or acquired, that allow air to move between the abdominal and thoracic cavity [
A high clinical suspicion is essential for diagnosing ERCP-related perforations to allow for early diagnosis and subsequent optimal management and better prognosis. With the use of sedation, older age of patients, and chronic multiple comorbid medical issues, symptoms may initially be masked, making the diagnosis more difficult [
Diagnosis can occur during the ERCP if extravasation of air or contrast is seen outside the bile ducts and duodenum into the retroperitoneal space. In addition, abnormal guidewire position on fluoroscopy may also indicate perforation [
The clinical presentation of patients with perforation in the postprocedure period is usually nonspecific. One study performed a prospective analysis of patients with perforation after ERCP found that 100% of patients with perforation had abdominal or flank discomfort, 74% had elevated heart rate, 64% had mild to moderate abdominal tenderness, 47% had low-grade fever, 37% had hyperamylasemia (amylase
Because of its ease of administration, the first imaging study performed is usually an abdominal X-ray. This may demonstrate retroperitoneal air as streaking opacities in the right upper quadrant and outlining the kidney margins and along the psoas muscle [
Treatment of ERCP-related perforations depends on the type of injury and the patient’s clinical symptoms. All type I perforations, since they are usually large, are immediately repaired with general surgery [
The approach to management of type II perforations is less clear. Most tend to seal spontaneously by 2-3 days, but 10–43% of patients may require surgical repair [
Our patient serves as an excellent example of nonsurgical management of a Stapfer type II or III perforation. The likely cause of perforation was dilation and stenting at the anastomosis of common hepatic duct and duodenal diverticulum or choledocele remnant, which can best be understood as a biliary perforation at the surgical anastomosis. The initial presentation might readily have led to unnecessary surgical intervention with massive subcutaneous air, symptoms of chest pain and dyspnea, and the radiologic findings of marked retroperitoneal, intraperitoneal, mediastinal air and bilateral pneumothoraces. However, with chest tube placement, all symptoms resolved and the patient’s clinical course was entirely uneventful. With close observation and patience, her hospital stay was shortened and she avoided the additional risks and morbidity of surgical intervention.
ERCP-related perforation is uncommon but has a high mortality rate, making it a feared complication. Because it usually presents with clinical findings similar to those of pancreatitis, a high clinical suspicion is needed to recognize perforation and initiate therapy promptly to achieve better outcomes. Stapfer type I perforations routinely require surgery and type IV perforations can be managed with observation alone. Treatment of Stapfer types II and III must be individualized based on the clinical and radiographic features of the patient. In this case of type II or III perforation, massive subcutaneous emphysema and extensive air throughout the abdomen and chest might have indicated surgery, but the outcome was very good with nonoperative management. As experience grows with the conservative management of perforation after ERCP, surgery may be required only for the most compelling indications of fluid extravasation, peritonitis, or sepsis.