Pancreas divisum

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Pancreas divisum
Other namesPancreatic divisum
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Specialty Medical genetics   OOjs UI icon edit-ltr-progressive.svg

Pancreas 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. Most individuals with pancreas divisum remain without symptoms or complications. A minority of people with pancreatic divisum may develop episodes of abdominal pain, nausea or vomiting due to acute or chronic pancreatitis. The presence of pancreas divisum is usually identified with cross sectional diagnostic imaging, such as endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP). In some cases, it may be detected intraoperatively. [1] If no symptoms or complications are present, then treatment is not necessary. However, if there is recurrent pancreatitis, then a sphincterotomy of the minor papilla may be indicated.

Contents

Causes

The human embryo begins life with two ducts in the pancreas, the ventral duct and the dorsal duct. Normally, the two ducts will fuse together to form one main pancreatic duct; this occurs in more than 90% of embryos. In approximately 10% of embryos the ventral and dorsal ducts fail to fuse together, resulting in pancreas divisum. In utero, the majority of the pancreas is drained by the dorsal duct which opens up into the minor duodenal papilla. The ventral duct drains the minority of the pancreas and opens into the major duodenal papilla. In adults however, this situation is reversed whereby 70% of the pancreas is drained by the ventral duct. Therefore in pancreas divisum, where fusion of the ducts does not occur, the major drainage of the pancreas is done by the dorsal duct which opens up into the minor papilla.[ citation needed ]

Diagnosis

MRCP image of pancreas divisum. MRCP Pankreas divisum.jpg
MRCP image of pancreas divisum.

The most common and accurate way of diagnosing an individual with this anomaly is by ERCP and MRCP. This test can demonstrate the presence of two separately draining ducts within the pancreas

Pancreas divisum has been suggested as a potential contributor of chronic pancreatitis in certain cases. [2] The separate ducts could lead to poor drainage of pancreatic secretions and increased pressure in the dorsal duct, [3] increasing the risk of inflammation and the development of complications. As a result, pancreas divisum is found more commonly in patients with pancreatitis.

Treatment

Pancreas divisum in individuals with no symptoms does not require treatment. For cases with mild and infrequent attacks, management may involve a low-fat diet, medications to reduce pain and gastrointestinal reactions, and pancreatic enzyme supplementation. [4]

A surgeon may attempt a sphincterotomy by cutting the minor papilla to enlarge the opening and allow pancreatic enzymes to flow normally. During surgery, a stent may be inserted into the duct to ensure that the duct will not close causing a blockage. This surgery can cause pancreatitis in patients, or in rare cases, kidney failure and death. ERCP are sometimes used for symptomatic pancreas divisum, which offers the benefit of a less invasive approach compared with surgery. [5] No large-scale clinical studies comparing surgical and endoscopic approaches are available. [6]

Occurrence

Studies involving autopsy and imaging series indicate that between 6% and 10% of the population have pancreas divisum, but it is asymptomatic in the majority (>95%) of cases. In those who develop symptoms, the symptoms seen in pancreas divisum and pancreatitis with typical anatomy are the same: [4] abdominal pain is common, typically of sudden onset and located in the left upper quadrant of the abdomen and often accompanied by nausea and vomiting. [7] Pancreatic pain is characteristically described as a constant, severe, dull, epigastric pain that often radiates to the back and typically worsens after high-fat meals. However, many different pain patterns have been described, ranging from no pain to recurrent episodes of pain and pain free intervals, to constant pain with clusters of severe exacerbations. [8]

Related Research Articles

<span class="mw-page-title-main">Pancreas</span> Organ of the digestive system and endocrine system of vertebrates

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.

<span class="mw-page-title-main">Cholecystectomy</span> Surgical removal of the gallbladder

Cholecystectomy is the surgical removal of the gallbladder. Cholecystectomy is a common treatment of symptomatic gallstones and other gallbladder conditions. In 2011, cholecystectomy was the eighth most common operating room procedure performed in hospitals in the United States. Cholecystectomy can be performed either laparoscopically, or via an open surgical technique.

<span class="mw-page-title-main">Endoscopic retrograde cholangiopancreatography</span> Use of endoscopy and fluoroscopy to treat and diagnose digestive issues.

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.

<span class="mw-page-title-main">Acute pancreatitis</span> Medical condition

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.

<span class="mw-page-title-main">Chronic pancreatitis</span> Medical condition

Chronic pancreatitis is a long-standing inflammation of the pancreas that alters the organ's 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. It is a disease process characterized by irreversible damage to the pancreas as distinct from reversible changes in acute pancreatitis. Tobacco smoke and alcohol misuse are two of the most frequently implicated causes, and the two risk factors are thought to have a synergistic effect with regards to the development of chronic pancreatitis. Chronic pancreatitis is a risk factor for the development of pancreatic cancer.

<span class="mw-page-title-main">Common bile duct stone</span> Medical condition

Common bile duct stone, also known as choledocholithiasis, is the presence of gallstones in the common bile duct (CBD). This condition can cause jaundice and liver cell damage. Treatments include choledocholithotomy and endoscopic retrograde cholangiopancreatography (ERCP).

<span class="mw-page-title-main">Pancreatic duct</span> Duct associated with the human pancreas

The pancreatic duct or duct of Wirsung is a duct joining the pancreas to the common bile duct. This supplies it with pancreatic juice from the exocrine pancreas, which aids in digestion.

<span class="mw-page-title-main">Pancreatic pseudocyst</span> Medical condition

A pancreatic pseudocyst is a circumscribed collection of fluid rich in pancreatic enzymes, blood, and non-necrotic tissue, typically located in the lesser sac of the abdomen. Pancreatic pseudocysts are usually complications of pancreatitis, although in children they frequently occur following abdominal trauma. Pancreatic pseudocysts account for approximately 75% of all pancreatic masses.

<span class="mw-page-title-main">Pseudocyst</span> Medical condition

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.

<span class="mw-page-title-main">Pancreatic fistula</span> Medical condition

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.

<span class="mw-page-title-main">Annular pancreas</span> Medical condition

Annular pancreas is a rare condition in which the second part of the duodenum is surrounded by a ring of pancreatic tissue continuous with the head of the pancreas. This portion of the pancreas can constrict the duodenum and block or impair the flow of food to the rest of the intestines. It is estimated to occur in 1 out of 12,000 to 15,000 newborns. The ambiguity arises from the fact that not all cases are symptomatic.

<span class="mw-page-title-main">Ascending cholangitis</span> Medical condition

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.

<span class="mw-page-title-main">Hemosuccus pancreaticus</span> Medical condition

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.

<span class="mw-page-title-main">Major duodenal papilla</span> Rounded projection in the duodenum into which the common bile duct and pancreatic duct drain

The major duodenal papilla is a rounded projection in the duodenum into which the common bile duct and pancreatic duct drain. The major duodenal papilla is, in most people, the primary mechanism for the secretion of bile and other enzymes that facilitate digestion.

<span class="mw-page-title-main">Pancreatic bud</span> Anatomical feature of human embryo

The ventral and dorsal pancreatic buds are outgrowths of the duodenum during human embryogenesis. They join to form the adult pancreas.

The minor duodenal papilla is the opening of the accessory pancreatic duct into the descending second section of the duodenum.

<span class="mw-page-title-main">Sphincter of Oddi dysfunction</span> Medical condition

Sphincter of Oddi dysfunction refers to a group of functional disorders leading to abdominal pain due to dysfunction of the Sphincter of Oddi: functional biliary sphincter of Oddi and functional pancreatic sphincter of Oddi disorder. The sphincter of Oddi is a sphincter muscle, a circular band of muscle at the bottom of the biliary tree which controls the flow of pancreatic juices and bile into the second part of the duodenum. The pathogenesis of this condition is recognized to encompass stenosis or dyskinesia of the sphincter of Oddi ; consequently the terms biliary dyskinesia, papillary stenosis, and postcholecystectomy syndrome have all been used to describe this condition. Both stenosis and dyskinesia can obstruct flow through the sphincter of Oddi and can therefore cause retention of bile in the biliary tree and pancreatic juice in the pancreatic duct.

Pancreaticobiliary maljunction(PBM) is a congenital malformation where the pancreatic and bile ducts meet outside of the duodenum. There are two varieties of PBM: one with biliary dilatation and the other without. When an abnormally long common channel is visible on direct cholangiography, such as endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography, PBM is diagnosed.

<span class="mw-page-title-main">Choledochoduodenostomy</span>

Choledochoduodenostomy (CDD) is a surgical procedure to create an anastomosis, a surgical connection, between the common bile duct (CBD) and an alternative portion of the duodenum. In healthy individuals, the CBD meets the pancreatic duct at the ampulla of Vater, which drains via the major duodenal papilla to the second part of duodenum. In cases of benign conditions such as narrowing of the distal CBD or recurrent CBD stones, performing a CDD provides the diseased patient with CBD drainage and decompression. A side-to-side anastomosis is usually performed.

<span class="mw-page-title-main">Biliary endoscopic sphincterotomy</span> Use of endoscopy and fluoroscopy to treat and diagnose digestive issues.

Biliary endoscopic sphincterotomy is a procedure where the sphincter of Oddi and the segment of the common bile duct where it enters the duodenum are cannulated and then cut with a sphincterotome, a device that includes a wire which cuts with an electric current (electrocautery).

References

  1. Moran, Robert A.; Klapheke, Robert; John, George K.; Devlin, Sarah; Warren, Daniel; Desai, Niraj; Sun, Zhaoli; Walsh, Christi; Kalyani, Rita R.; Hall, Erica; Stein, Ellen M.; Kalloo, Anthony N.; Zaheer, Atif; Hirose, Kenzo; Makary, Martin A. (2017-09-01). "Prevalence and predictors of pain and opioid analgesic use following total pancreatectomy with islet autotransplantation for pancreatitis". Pancreatology. 17 (5): 732–737. doi:10.1016/j.pan.2017.07.005. ISSN   1424-3903. PMID   28733148.
  2. Gregg, James A. (1977-11-01). "Pancreas divisum: Its association with pancreatitis". The American Journal of Surgery. 134 (5): 539–543. doi:10.1016/0002-9610(77)90429-9. ISSN   0002-9610. PMID   920876.
  3. Wood, Cecil G.; Lopes Vendrami, Camila; Craig, Elizabeth; Mittal, Pardeep K.; Miller, Frank H. (2020-05-01). "Pancreatitis in the developmentally anomalous pancreas". Abdominal Radiology. 45 (5): 1316–1323. doi:10.1007/s00261-019-02197-8. ISSN   2366-0058. PMID   31468154. S2CID   201655246.
  4. 1 2 Gutta, Aditya; Fogel, Evan; Sherman, Stuart (2 November 2019). "Identification and management of pancreas divisum". Expert Review of Gastroenterology & Hepatology. 13 (11): 1089–1105. doi:10.1080/17474124.2019.1685871. PMC   6872911 . PMID   31663403.
  5. Zippi, M; Familiari, P; Traversa, G; De Felici, I; Febbraro, I; Occhigrossi, G; Severi, C (2014). "Role of endoscopic sphincterotomy of the minor papilla in pancreas divisum". La Clinica Terapeutica. 165 (4): e312-6. doi:10.7417/CT.2014.1748. PMID   25203348.
  6. Hafezi, Mohammadreza; Mayschak, Bartosch; Probst, Pascal; Büchler, Markus W.; Hackert, Thilo; Mehrabi, Arianeb (September 2017). "A systematic review and quantitative analysis of different therapies for pancreas divisum". The American Journal of Surgery. 214 (3): 525–537. doi:10.1016/j.amjsurg.2016.12.025. PMID   28110914.
  7. Quinlan, Jeffrey D. (1 November 2014). "Acute Pancreatitis". American Family Physician. 90 (9): 632–639. PMID   25368923.
  8. Poulsen, Jakob Lykke; Olesen, Søren Schou; Malver, Lasse Paludan; Frøkjær, Jens Brøndum; Drewes, Asbjørn Mohr (14 November 2013). "Pain and chronic pancreatitis: A complex interplay of multiple mechanisms". World Journal of Gastroenterology. 19 (42): 7282–7291. doi: 10.3748/wjg.v19.i42.7282 . PMC   3831210 . PMID   24259959.