Hemosuccus pancreaticus | |
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Other names | Pseudohematobilia, Wirsungorrhage |
The pancreatic duct orifice is seen on the side of the duodenum, at the ampulla of Vater, which may necessitate the use of side-viewing endoscopes to diagnose hemosuccus pancreaticus. | |
Specialty | Gastroenterology |
Hemosuccus pancreaticus is a rare cause of hemorrhage in the gastrointestinal tract. It is caused by a bleeding source in the pancreas, pancreatic duct, or structures adjacent to the pancreas, such as the splenic artery, that bleed into the pancreatic duct, which is connected with the bowel at the duodenum, the first part of the small intestine. Patients with hemosuccus may develop symptoms of gastrointestinal hemorrhage, such as blood in the stools, maroon stools, or melena, which is a dark, tarry stool caused by digestion of red blood cells. They may also develop abdominal pain. It is associated with pancreatitis, pancreatic cancer and aneurysms of the splenic artery. Hemosuccus may be identified with endoscopy (esophagogastroduodenoscopy), where fresh blood may be seen from the pancreatic duct. Alternatively, angiography may be used to inject the celiac axis to determine the blood vessel that is bleeding. This may also be used to treat hemosuccus, as embolization of the end vessel may terminate the hemorrhage. However, a distal pancreatectomy—surgery to remove of the tail of the pancreas—may be required to stop the hemorrhage.
Hemosuccus pancreaticus is a rare entity, and estimates of its rate are based on small case series. [1] [2] It is the least frequent cause of upper gastrointestinal bleeding (1/1500) and is most often caused by chronic pancreatitis, pancreatic pseudocysts, or pancreatic tumors. [3] As a result, the diagnosis may easily be overlooked. [4] The usual presentation of hemosuccus is the development of symptoms of upper or lower gastrointestinal bleeding, such as melena (or dark, black tarry stools), maroon stools, or hematochezia, which is frank rectal bleeding. The source of hemorrhage is usually not determined by standard endoscopic techniques, and the symptoms of the condition are usually grouped as a cause of obscure overt gastrointestinal hemorrhage. Over one-half of patients with hemosuccus also develop abdominal pain, usually located in the epigastrium, or uppermost part of the abdomen. The pain is described as being "crescendo-decrescendo" in nature, meaning that it increases and decreases in intensity slowly with time. This is thought to be due to transient blockage of the pancreatic duct from the source of bleeding, or from clots. [1] If the source of the bleeding also involves obstruction of the common bile duct (such as with some tumours of the head of the pancreas), the patient may develop jaundice, or "silver stools", an uncommon finding of acholic stools mixed with blood. [5]
The causes of hemosuccus pancreaticus can be grouped into diseases of the pancreas and diseases of the vascular structures around the pancreas. Diseases of the pancreas include acute and chronic pancreatitis, [6] pancreatic cancer, [2] pancreatic duct stones, [7] ruptured aneurysms of the splenic artery, [4] and pseudoaneurysms of the splenic artery [8] and hepatic artery. [9] Pseudoaneurysms are complications of pancreatitis where a pseudocyst is formed, with one wall abutting an artery, usually the splenic artery. Should the arterial wall rupture, the pseudoaneurysm will hemorrhage into the pancreatic duct.
Rarely the bleeding is not channeled into the bowel from the main pancreatic duct (or duct of Wirsung), but rather comes from the accessory pancreatic duct (or duct of Santorini). The former is termed Wirsungorrhage and the latter is termed Santorinirrhage. Bleeding from the duct of Santorini can be caused by pancreas divisum, a possible congenital cause of pancreatitis. [10]
The diagnosis of hemosuccus pancreaticus can be difficult to make. Most patients who develop bleeding in the gastrointestinal tract have endoscopic procedures done to visualize the bowel in order to find and treat the source of the bleeding. With hemosuccus, the bleeding is coming from the pancreatic duct which enters into the first part of the small intestine, termed the duodenum. Typical gastroscopes used to visualize the esophagus, stomach and duodenum are designed with fiber-optic illumination that is directed in the same direction as the endoscope, meaning that visualization is in the forward direction. However, the pancreatic duct orifice is located on the side of the duodenum, meaning that it can be missed on forward-viewing endoscopy. A side-viewing endoscope (known as a duodenoscope, or side-viewer) used for endoscopic retrograde cholangiopancreatography (ERCP), a procedure to visualize the bile ducts and pancreatic duct on fluoroscopy, can be used to localize the bleeding to the pancreatic duct. [11] [12] It can be confused with bleeding from the common bile duct on endoscopy, leading to the term pseudohematobilia.
Liver function test is normal apart from an increased serum bilirubin in the event of pancreaticobiliary reflux. Serum amylase is normal outside episodes of acute pancreatitis. It is difficult to diagnose HP because the bleeding is usually intermittent. Endoscopy is essential in ruling out other causes of upper gastrointestinal bleeding and in rare cases; active bleeding can be seen from the duodenal ampulla. Even though endoscopy may be normal, it helps to rule out other causes of upper digestive bleeding (erosive gastritis, peptic ulcers, and oesophageal and gastric fundus varices, etc.). Ultrasonography can be used to visualize pancreatic pseudocysts or aneurysm of the peripancreatic arteries. Doppler ultrasound or dynamic ultrasound has been reported to be diagnostic. Contrast-enhanced CT is an excellent modality for demonstrating the pancreatic pathology and can also demonstrate features of chronic pancreatitis, pseudocysts, and pseudoaneurysms. On precontrast CT, the characteristic finding of clotted blood in the pancreatic duct, known as the sentinel clot, is seldom seen. Computed tomography may show simultaneous opacification of an aneurysmal artery and pseudocyst or persistence of contrast within a pseudocyst after the arterial phase. Again, these findings are only suggestive of the diagnosis. Ultimately, angiography is the diagnostic reference standard. Angiography identifies the causative artery and allows for delineation of the arterial anatomy and therapeutic intervention. [3] [13]
Treatment of hemosuccus pancreaticus depends on the source of the hemorrhage. If the bleeding is identified on angiography to be coming from a vessel that is small enough to occlude, embolization through angiography may stop the bleeding. [3] Both coils in the end-artery and stents across the area of bleeding have been used to control the hemorrhage. [14] However, the bleeding may be refractory to the embolization, which would necessitate surgery to remove the pancreas at the source of hemorrhage. Also, the cause of bleeding may be too diffuse to be treated with embolization (such as with pancreatitis or with pancreatic cancer). This may also require surgical therapy, and usually a distal pancreatectomy, or removal of the part of the pancreas from the area of bleeding to the tail, is required. [1] [3] [4]
Hemosuccus pancreaticus was first described as a cause of hemorrhage in 1931 by Lower and Farrell, who described an aneurysm of the splenic artery causing bleeding through the pancreatic duct. [4] [15] In 1969, Vankemmel proposed the term "wirsungorrhagia" (currently used in France). [16] In 1970, Sandblom published three cases and coined the term "hemosuccus pancreaticus" to describe the similarity of the disorder to the clinical syndrome of hemobilia. [17]
The pancreas is an organ of the digestive system and endocrine system of vertebrates. In humans, it is located in the abdomen behind the stomach and functions as a gland. The pancreas is a mixed or heterocrine gland, i.e., it has both an endocrine and a digestive exocrine function. 99% of the pancreas is exocrine and 1% is endocrine. As an endocrine gland, it functions mostly to regulate blood sugar levels, secreting the hormones insulin, glucagon, somatostatin and pancreatic polypeptide. As a part of the digestive system, it functions as an exocrine gland secreting pancreatic juice into the duodenum through the pancreatic duct. This juice contains bicarbonate, which neutralizes acid entering the duodenum from the stomach; and digestive enzymes, which break down carbohydrates, proteins and fats in food entering the duodenum from the stomach.
Upper gastrointestinal bleeding is gastrointestinal bleeding (hemorrhage) in the upper gastrointestinal tract, commonly defined as bleeding arising from the esophagus, stomach, or duodenum. Blood may be observed in vomit or in altered form as black stool. Depending on the amount of the blood loss, symptoms may include shock.
In medicine (gastroenterology), angiodysplasia is a small vascular malformation of the gut. It is a common cause of otherwise unexplained gastrointestinal bleeding and anemia. Lesions are often multiple, and frequently involve the cecum or ascending colon, although they can occur at other places. Treatment may be with colonoscopic interventions, angiography and embolization, medication, or occasionally surgery.
Dieulafoy's lesion is a medical condition characterized by a large tortuous artery most commonly in the stomach wall (submucosal) that erodes and bleeds. It can present in any part of the gastrointestinal tract. It can cause gastric hemorrhage but is relatively uncommon. It is thought to cause less than 5% of all gastrointestinal bleeds in adults. It was named after French surgeon Paul Georges Dieulafoy, who described this condition in his paper "Exulceratio simplex: Leçons 1-3" in 1898. It is also called "caliber-persistent artery" or "aneurysm" of gastric vessels. However, unlike most other aneurysms, these are thought to be developmental malformations rather than degenerative changes.
Endoscopic retrograde cholangiopancreatography (ERCP) is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems. It is primarily performed by highly skilled and specialty trained gastroenterologists. Through the endoscope, the physician can see the inside of the stomach and duodenum, and inject a contrast medium into the ducts in the biliary tree and pancreas so they can be seen on radiographs.
Gastric varices are dilated submucosal veins in the lining of the stomach, which can be a life-threatening cause of bleeding in the upper gastrointestinal tract. They are most commonly found in patients with portal hypertension, or elevated pressure in the portal vein system, which may be a complication of cirrhosis. Gastric varices may also be found in patients with thrombosis of the splenic vein, into which the short gastric veins that drain the fundus of the stomach flow. The latter may be a complication of acute pancreatitis, pancreatic cancer, or other abdominal tumours, as well as hepatitis C. Gastric varices and associated bleeding are a potential complication of schistosomiasis resulting from portal hypertension.
Acute pancreatitis (AP) is a sudden inflammation of the pancreas. Causes, in order of frequency, include: a gallstone impacted in the common bile duct beyond the point where the pancreatic duct joins it; heavy alcohol use; systemic disease; trauma; and, in children, mumps. Acute pancreatitis may be a single event; it may be recurrent; or it may progress to chronic pancreatitis and/or pancreatic failure.
Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum; and the accessory organs of digestion, the liver, gallbladder, and pancreas.
A pancreaticoduodenectomy, also known as a Whipple procedure, is a major surgical operation most often performed to remove cancerous tumours from the head of the pancreas. It is also used for the treatment of pancreatic or duodenal trauma, or chronic pancreatitis. Due to the shared blood supply of organs in the proximal gastrointestinal system, surgical removal of the head of the pancreas also necessitates removal of the duodenum, proximal jejunum, gallbladder, and, occasionally, part of the stomach.
Pseudocysts are like cysts, but lack epithelial or endothelial cells. Initial management consists of general supportive care. Symptoms and complications caused by pseudocysts require surgery. Computed tomography (CT) scans are used for initial imaging of cysts, and endoscopic ultrasounds are used in differentiating between cysts and pseudocysts. Endoscopic drainage is a popular and effective method of treating pseudocysts.
A pancreatic fistula is an abnormal communication between the pancreas and other organs due to leakage of pancreatic secretions from damaged pancreatic ducts. An external pancreatic fistula is one that communicates with the skin, and is also known as a pancreaticocutaneous fistula, whereas an internal pancreatic fistula communicates with other internal organs or spaces. Pancreatic fistulas can be caused by pancreatic disease, trauma, or surgery.
Endoscopic ultrasound (EUS) or echo-endoscopy is a medical procedure in which endoscopy is combined with ultrasound to obtain images of the internal organs in the chest, abdomen and colon. It can be used to visualize the walls of these organs, or to look at adjacent structures. Combined with Doppler imaging, nearby blood vessels can also be evaluated.
In human anatomy, the greater pancreatic artery, is the largest artery that supplies the pancreas. It arises from the splenic artery.
The inferior pancreaticoduodenal artery is a branch of the superior mesenteric artery. It supplies the head of the pancreas, and the ascending and inferior parts of the duodenum. Rarely, it may have an aneurysm.
Pancreatic diseases are diseases that affect the pancreas, an organ in most vertebrates and in humans and other mammals located in the abdomen. The pancreas plays a role in the digestive and endocrine system, producing enzymes which aid the digestion process and the hormone insulin, which regulates blood sugar levels. The most common pancreatic disease is pancreatitis, an inflammation of the pancreas which could come in acute or chronic form. Other pancreatic diseases include diabetes mellitus, exocrine pancreatic insufficiency, cystic fibrosis, pseudocysts, cysts, congenital malformations, tumors including pancreatic cancer, and hemosuccus pancreaticus.
Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is inflammation of the bile duct, usually caused by bacteria ascending from its junction with the duodenum. It tends to occur if the bile duct is already partially obstructed by gallstones.
A self-expandable metallic stent is a metallic tube, or stent that holds open a structure in the gastrointestinal tract to allow the passage of food, chyme, stool, or other secretions related to digestion. Surgeons insert SEMS by endoscopy, inserting a fibre optic camera—either through the mouth or colon—to reach an area of narrowing. As such, it is termed an endoprosthesis. SEMS can also be inserted using fluoroscopy where the surgeon uses an X-ray image to guide insertion, or as an adjunct to endoscopy.
An endoclip is a metallic mechanical device used in endoscopy in order to close two mucosal surfaces without the need for surgery and suturing. Its function is similar to a suture in gross surgical applications, as it is used to join together two disjointed surfaces, but, can be applied through the channel of an endoscope under direct visualization. Endoclips have found use in treating gastrointestinal bleeding, in preventing bleeding after therapeutic procedures such as polypectomy, and in closing gastrointestinal perforations. Many forms of endoclips exist of different shapes and sizes, including two and three prong devices, which can be administered using single use and reloadable systems, and may or may not open and close to facilitate placement.
Cystogastrostomy is a surgery to create an opening between a pancreatic pseudocyst and the stomach when the cyst is in a suitable position to be drained into the stomach. This conserves pancreatic juices that would otherwise be lost. This surgery is performed by a pancreatic surgeon to avoid a life-threatening rupture of the pancreatic pseudocyst.
Kenneth Frank Binmoeller is a medical doctor and author of multiple scientific contributions and over 300 publications, as well as the inventor of the lumen-apposing metal stent (LAMS) and AXIOS System. These are medical devices used to relieve blockages while creating a direct connection between two bodily structures. He practices in the field of Gastroenterology with a specialty of Advanced Endoscopic Intervention. Binmoeller has been published for his innovations in medical devices and training in the field of Endoscopy.