Hemosuccus pancreaticus
Encyclopedia
Hemosuccus pancreaticus, also known as pseudohematobilia or Wirsungorrhage is a rare cause of hemorrhage
Lower gastrointestinal bleeding
Lower gastrointestinal bleeding, commonly abbreviated LGIB, refers to any form of bleeding in the lower gastrointestinal tract. LGIB is a common ailment seen at emergency departments. It presents less commonly than upper gastrointestinal bleeding . It is estimated that UGIB accounts for 100-200 per...

 in the gastrointestinal tract
Gastrointestinal tract
The human gastrointestinal tract refers to the stomach and intestine, and sometimes to all the structures from the mouth to the anus. ....

. It is caused by a bleeding source in the pancreas
Pancreas
The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...

, pancreatic duct
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...

, or structures adjacent to the pancreas, such as the splenic artery
Splenic artery
In anatomy, the splenic artery is the blood vessel that supplies oxygenated blood to the spleen. It branches from the celiac artery, and follows a course superior to the pancreas.-Branches:...

, that bleed into the pancreatic duct, which is connected with the bowel at the duodenum
Duodenum
The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear and the terms anterior intestine or proximal intestine may be used instead of 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
Melena
In medicine, melena or melaena refers to the black, "tarry" feces that are associated with gastrointestinal hemorrhage. The black color is caused by oxidation of the iron in hemoglobin during its passage through the ileum and colon.-Melena vs...

, which is a dark, tarry stool caused by digestion of red blood cells. They may also develop abdominal pain. It is associated with pancreatitis
Pancreatitis
Pancreatitis is inflammation of the pancreas. It occurs when pancreatic enzymes that digest food are activated in the pancreas instead of the small intestine. It may be acute – beginning suddenly and lasting a few days, or chronic – occurring over many years...

, pancreatic cancer
Pancreatic cancer
Pancreatic cancer refers to a malignant neoplasm of the pancreas. The most common type of pancreatic cancer, accounting for 95% of these tumors is adenocarcinoma, which arises within the exocrine component of the pancreas. A minority arises from the islet cells and is classified as a...

 and aneurysms of the splenic artery. Hemosuccus may be identified with esophagogastroduodenoscopy
Esophagogastroduodenoscopy
For other expansions of the initialism "OGD", see the disambiguation page.In medicine , esophagogastroduodenoscopy is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract up to the duodenum...

, where fresh blood may be seen from the pancreatic duct
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...

. 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
Embolization
Embolization is a non-surgical, minimally-invasive procedure performed by an interventional radiologist and interventional neuroradiologists. It involves the selective occlusion of blood vessels by purposely introducing emboli.-Therapeutic applications:...

 of the end vessel may terminate the hemorrhage. However, a distal pancreatectomy -- surgery to removal of the tail of the pancreas—may be required to stop the hemorrhage.

Symptoms

Hemosuccus pancreaticus is a rare entity, and estimates of its rate are based on small case series. As a result, the diagnosis may easily be overlooked. The usual presentation of hemosuccus is the development of symptoms of upper
Upper gastrointestinal bleeding
Upper gastrointestinal bleeding refers to hemorrhage in the upper gastrointestinal tract. The anatomic cut-off for upper GI bleeding is the ligament of Treitz, which connects the fourth portion of the duodenum to the diaphragm near the splenic flexure of the colon.Upper GI bleeds are considered...

 or lower gastrointestinal bleeding
Lower gastrointestinal bleeding
Lower gastrointestinal bleeding, commonly abbreviated LGIB, refers to any form of bleeding in the lower gastrointestinal tract. LGIB is a common ailment seen at emergency departments. It presents less commonly than upper gastrointestinal bleeding . It is estimated that UGIB accounts for 100-200 per...

, such as melena
Melena
In medicine, melena or melaena refers to the black, "tarry" feces that are associated with gastrointestinal hemorrhage. The black color is caused by oxidation of the iron in hemoglobin during its passage through the ileum and colon.-Melena vs...

 (or dark, black tarry stools), maroon stools, or hematochezia
Hematochezia
Hematochezia is the passage of fresh blood per anus, usually in or with stools . Hematochezia is commonly associated with lower gastrointestinal bleeding, but may also occur from a brisk upper GI bleed...

, which is frank rectal bleeding. The source of hemorrhage is usually not determined by standard endoscopic
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...

 techniques, and the symptoms of the condition are usually grouped as a cause of obscure overt gastrointestinal hemorrhage
Gastrointestinal bleeding
Gastrointestinal bleeding or gastrointestinal hemorrhage describes every form of hemorrhage in the gastrointestinal tract, from the pharynx to the rectum. It has diverse causes, and a medical history, as well as physical examination, generally distinguishes between the main forms...

. Over one-half of patients with hemosuccus also develop abdominal pain
Abdominal pain
Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...

, usually located in the epigastrium
Epigastrium
The epigastrium is the upper central region of the abdomen. It is located between the costal margins and the subcostal plane....

, 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
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...

 from the source of bleeding, or from clots. If the source of the bleeding also involves obstruction of the common bile duct
Common bile duct
The common bile duct is a tube-like anatomic structure in the human gastrointestinal tract. It is formed by the union of the common hepatic duct and the cystic duct . It is later joined by the pancreatic duct to form the ampulla of Vater...

 (such as with some tumours
Pancreatic cancer
Pancreatic cancer refers to a malignant neoplasm of the pancreas. The most common type of pancreatic cancer, accounting for 95% of these tumors is adenocarcinoma, which arises within the exocrine component of the pancreas. A minority arises from the islet cells and is classified as a...

 of the head of the pancreas
Pancreas
The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...

), the patient may develop jaundice
Jaundice
Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...

, or "silver stools", an uncommon finding of acholic stools mixed with blood.

Testing and diagnosis

The diagnosis of hemosuccus pancreaticus can be difficult to make. Most patients who develop bleeding in the gastrointestinal tract have endoscopic procedures
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...

 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
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...

 which enters into the first part of the small intestine
Small intestine
The small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...

, termed the duodenum
Duodenum
The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear and the terms anterior intestine or proximal intestine may be used instead of duodenum...

. Typical gastroscopes
Esophagogastroduodenoscopy
For other expansions of the initialism "OGD", see the disambiguation page.In medicine , esophagogastroduodenoscopy is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract up to the duodenum...

 used to visualize the esophagus
Esophagus
The esophagus is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. During swallowing, food passes from the mouth through the pharynx into the esophagus and travels via peristalsis to the stomach...

, 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
Endoscopic retrograde cholangiopancreatography
Endoscopic retrograde cholangiopancreatography is a technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems...

 (ERCP), a procedure to visualize the bile duct
Bile duct
A bile duct is any of a number of long tube-like structures that carry bile.Bile, required for the digestion of food, is excreted by the liver into passages that carry bile toward the hepatic duct, which joins with the cystic duct to form the common bile duct, which opens into the intestine.The...

s and pancreatic duct
Pancreatic duct
The pancreatic duct, or duct of Wirsung , is a duct joining the pancreas to the common bile duct to supply pancreatic juices which aid in digestion provided by the "exocrine pancreas"...

 on fluoroscopy, can be used to localize the bleeding to the pancreatic duct. It can be confused with bleeding from the common bile duct on endoscopy, leading to the term pseudohematobilia.

Once the bleeding has been localized to the pancreatic duct, angiography, or the injection of dye into the celiac artery
Celiac artery
The celiac artery, also known as the celiac trunk, is the first major branch of the abdominal aorta. Branching from the aorta anterior to the upper border of L1 vertebra in humans, it is one of three anterior/ midline branches of the abdominal aorta The celiac (or coeliac) artery, also known as...

, which supplies the pancreas, can be performed to determine which vessel is the cause of the bleeding. This is additionally useful to treatment, as the bleeding vessel can be occluded (termed "embolization") at the time of angiography.

Causes

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
Acute pancreatitis
Acute pancreatitis or acute pancreatic necrosis is a sudden inflammation of the pancreas. It can have severe complications and high mortality despite treatment...

 and chronic pancreatitis
Chronic pancreatitis
Chronic pancreatitis is a long-standing inflammation of the pancreas that alters its normal structure and functions. It can present as episodes of acute inflammation in a previously injured pancreas, or as chronic damage with persistent pain or malabsorption....

, pancreatic cancer
Pancreatic cancer
Pancreatic cancer refers to a malignant neoplasm of the pancreas. The most common type of pancreatic cancer, accounting for 95% of these tumors is adenocarcinoma, which arises within the exocrine component of the pancreas. A minority arises from the islet cells and is classified as a...

, pancreatic duct stones, ruptured aneurysms of the splenic artery, and pseudoaneurysms of the splenic artery and hepatic artery
Hepatic artery
Hepatic artery can refer to:* Common hepatic artery * Hepatic artery proper...

. Pseudoaneurysms are complications of pancreatitis
Pancreatitis
Pancreatitis is inflammation of the pancreas. It occurs when pancreatic enzymes that digest food are activated in the pancreas instead of the small intestine. It may be acute – beginning suddenly and lasting a few days, or chronic – occurring over many years...

 where a pseudocyst
Pseudocyst
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. CT scans are used for initial imaging of cysts, and endoscopic ultrasounds are used in differentiating...

 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
Accessory pancreatic duct
Most people have just one pancreatic duct. However, some have an additional accessory pancreatic duct also called the Duct of Santorini, which connects straight to the duodenum at the minor duodenal papilla...

 (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
Pancreas divisum
Pancreas or Pancreatic divisum is a congenital anomaly in the anatomy of the ducts of the pancreas in which a single pancreatic duct is not formed, but rather remains as two distinct dorsal and ventral ducts.-Causes:...

, a possible congenital cause of pancreatitis.

Treatment

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. Both coils in the end-artery and stent
Stent
In the technical vocabulary of medicine, a stent is an artificial 'tube' inserted into a natural passage/conduit in the body to prevent, or counteract, a disease-induced, localized flow constriction. The term may also refer to a tube used to temporarily hold such a natural conduit open to allow...

s across the area of bleeding have been used to control the hemorrhage. 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
Pancreas
The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...

, is required.

History

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.
In 1969, Vankemmel proposed the term "wirsungorrhagia" (currently used in France). In 1970, Sandblom published 3 cases and coined the term "hemosuccus pancreaticus" to describe the similarity of the disorder to the clinical syndrome of hemobilia.
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