Annular pancreas

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Annular pancreas
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Duodenum and pancreas (normal anatomy)
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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. [1] The ambiguity arises from the fact that not all cases are symptomatic. [2]

Contents

Signs and symptoms

Early signs of abnormality include polyhydramnios (an excess of amniotic fluid), low birth weight, and feeding intolerance immediately after birth, in particular a tendency to develop epigastric distention associated with non-biliary vomiting (the obstruction is generally above the papilla of Vater, therefore superior to the junction with the bile ducts). Different chromosomal diseases (for example trisomy 21 and, with a minor frequency, trisomy 18 and trisomy 13) are present in about 33% of subjects affected by annular pancreas. [3] [4] In adults, the clinical picture is often dominated by the sensation of postprandial distension, abdominal pain in the epigastric region, nausea and vomiting that may be present for a long time (sometimes for years) before reaching a precise diagnosis.[ citation needed ]

Causes

It is typically associated with abnormal embryological development, however adult cases can develop. It can result from growth of a bifid ventral pancreatic bud around the duodenum, where the parts of the bifid ventral bud fuse with the dorsal bud, forming a pancreatic ring. It can also result if the ventral pancreatic bud fails to fully rotate, so it remains on the right or if the dorsal bud rotates in the wrong direction, such that the duodenum is surrounded by pancreatic tissue. Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas.[ citation needed ]

Diagnosis

Postnatal diagnostic procedures include abdominal x-ray and ultrasound, CT scan, and upper GI and small bowel series. Abdominal radiography can show the classic sign of the "double bubble": the presence of air in the stomach and duodenum. [5] [6] Unfortunately, this double-bubble sign is not pathognomonic for annular pancreas, as it can also be observed in other conditions, such as duodenal atresia [7] and intestinal malrotation. [8] Upper GI series may be suggestive of annular pancreas, especially if they show a duodenal narrowing of the second portion of the duodenum and the concomitant dilatation of the proximal duodenum. In some cases it is possible to have signs of inverse peristalsis of the duodenal tract which is proximal to the narrowing caused by the annular pancreas, and the dilatation of the duodenal portion distal to the anomaly. An abdominal CT scan or an MRI allows to highlight the narrowing of the descending duodenal tract and the ring of pancreatic tissue surrounding the duodenum: this ring can be complete or, in patients with an incomplete annular pancreas, extended in a postero-lateral or anterolateral direction with respect to the second part of the duodenum. ERCP or MRCP with secretin allow precise delineation of the anatomical structure and in particular a good visualization of pancreatic ducts, as well as a careful analysis of pancreatic secretion into the duodenum lumen. [9]

Treatment

In neonates, treatment for relief of obstruction usually is bypassing the obstructed segment of duodenum by duodeno-jejunostomy. [10] In adults, due to the minor duodenal mobility, the approach is laparoscopic gastrojejunostomy or duodenojejunostomy. [11] [12]

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">Small intestine</span> Organ in the gastrointestinal tract

The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the pancreatic duct to aid in digestion. The small intestine is about 5.5 metres long and folds many times to fit in the abdomen. Although it is longer than the large intestine, it is called the small intestine because it is narrower in diameter.

<span class="mw-page-title-main">Meckel's diverticulum</span> Medical condition

A Meckel's diverticulum, a true congenital diverticulum, is a slight bulge in the small intestine present at birth and a vestigial remnant of the vitelline duct. It is the most common malformation of the gastrointestinal tract and is present in approximately 2% of the population, with males more frequently experiencing symptoms.

<span class="mw-page-title-main">Omphalocele</span> Rare abdominal wall defect in which internal organs remain outside of the abdomen in a sac

Omphalocele or omphalocoele also called exomphalos, is a rare abdominal wall defect. Beginning at the 6th week of development, rapid elongation of the gut and increased liver size reduces intra abdominal space, which pushes intestinal loops out of the abdominal cavity. Around 10th week, the intestine returns to the abdominal cavity and the process is completed by the 12th week. Persistence of intestine or the presence of other abdominal viscera in the umbilical cord results in an omphalocele.

<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 minors, 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">Pancreaticoduodenectomy</span> Major surgical procedure involving the pancreas, duodenum, and other organs

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.

<span class="mw-page-title-main">Suspensory muscle of duodenum</span> Muscle between the duodenum and jejunum

The suspensory muscle of duodenum is a thin muscle connecting the junction between the duodenum and jejunum, as well as the duodenojejunal flexure to connective tissue surrounding the superior mesenteric and coeliac arteries. The suspensory muscle most often connects to both the third and fourth parts of the duodenum, as well as the duodenojejunal flexure, although the attachment is quite variable.

Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus. Malrotation can refer to a spectrum of abnormal intestinal positioning, often including:

<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">Pancreas divisum</span> Congenital disorder of digestive system

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. 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. 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.

<span class="mw-page-title-main">Foregut</span> Anterior part of the gastrointestinal tract

The foregut in humans is the anterior part of the alimentary canal, from the distal esophagus to the first half of the duodenum, at the entrance of the bile duct. Beyond the stomach, the foregut is attached to the abdominal walls by mesentery. The foregut arises from the endoderm, developing from the folding primitive gut, and is developmentally distinct from the midgut and hindgut. Although the term “foregut” is typically used in reference to the anterior section of the primitive gut, components of the adult gut can also be described with this designation. Pain in the epigastric region, just below the intersection of the ribs, typically refers to structures in the adult foregut.

<span class="mw-page-title-main">Uncinate process of pancreas</span>

The uncinate process is a small part of the pancreas. The uncinate process is the formed prolongation of the angle of junction of the lower and left lateral borders in the head of the pancreas. The word "uncinate" comes from the Latin "uncinatus", meaning "hooked".

<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">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.

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

Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum. It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies. Newborns present with bilious or non-bilous vomiting within the first 24 to 48 hours after birth, typically after their first oral feeding. Radiography shows a distended stomach and distended duodenum, which are separated by the pyloric valve, a finding described as the double-bubble sign.

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

Choledochal cysts are congenital conditions involving cystic dilatation of bile ducts. They are uncommon in western countries but not as rare in East Asian nations like Japan and China.

Megaduodenum is a congenital or acquired dilation and elongation of the duodenum with hypertrophy of all layers that presents as a feeling of gastric fullness, abdominal pain, belching, heartburn, and nausea with vomiting sometimes of food eaten 24 hours prior.

The development of the digestive system in the human embryo concerns the epithelium of the digestive system and the parenchyma of its derivatives, which originate from the endoderm. Connective tissue, muscular components, and peritoneal components originate in the mesoderm. Different regions of the gut tube such as the esophagus, stomach, duodenum, etc. are specified by a retinoic acid gradient that causes transcription factors unique to each region to be expressed. Differentiation of the gut and its derivatives depends upon reciprocal interactions between the gut endoderm and its surrounding mesoderm. Hox genes in the mesoderm are induced by a Hedgehog signaling pathway secreted by gut endoderm and regulate the craniocaudal organization of the gut and its derivatives. The gut system extends from the oropharyngeal membrane to the cloacal membrane and is divided into the foregut, midgut, and hindgut.

<span class="mw-page-title-main">Pancreatic progenitor cell</span>

Pancreatic progenitor cells are multipotent stem cells originating from the developing fore-gut endoderm which have the ability to differentiate into the lineage specific progenitors responsible for the developing pancreas.

References

  1. ↑ Lainakis N; Antypas S; Panagidis A; et al. (2005), "Annular pancreas in two consecutive siblings: an extremely rare case", European Journal of Pediatric Surgery, 15 (5): 364–8, doi:10.1055/s-2005-865838, PMID   16254852, S2CID   260138452
  2. ↑ Ravitch, MM. (1975). "The pancreas in infants and children". Surg Clin North Am. 55 (2): 377–85. doi:10.1016/S0039-6109(16)40587-6. PMID   165579.
  3. ↑ Sencan A, Mir E, Günsar C, Akcora B (June 2002). "Symptomatic annular pancreas in newborns". Med. Sci. Monit. 8 (6): CR434–7. PMID   12070435 . Retrieved 2018-03-12.
  4. ↑ Yigiter M, Yildiz A, Firinci B, Yalcin O, Oral A, Salman AB (December 2010). "Annular pancreas in children: a decade of experience". Eurasian J Med. 42 (3): 116–9. doi:10.5152/eajm.2010.33. PMC   4261261 . PMID   25610139.
  5. ↑ Dankovcik R, Jirasek JE, Kucera E, Feyereisl J, Radonak J, Dudas M (2008). "Prenatal diagnosis of annular pancreas: reliability of the double bubble sign with periduodenal hyperechogenic band". Fetal Diagn. Ther. 24 (4): 483–90. doi:10.1159/000178759. PMC   2814148 . PMID   19047797.
  6. ↑ Raman VS, Arora M, Khanna SK (2015). "Annular pancreas, type I choledochal cyst and malrotation in a low-birth weight newborn: A case report". J Indian Assoc Pediatr Surg. 20 (3): 155–6. doi: 10.4103/0971-9261.154656 . PMC   4481632 . PMID   26166991.
  7. ↑ Poki HO, Holland AJ, Pitkin J (June 2005). "Double bubble, double trouble". Pediatr. Surg. Int. 21 (6): 428–31. doi:10.1007/s00383-005-1448-z. PMID   15912365. S2CID   21589667.
  8. ↑ Imamoglu M, Cay A, Sarihan H, Sen Y (April 2004). "Rare clinical presentation mode of intestinal malrotation after neonatal period: Malabsorption-like symptoms due to chronic midgut volvulus". Pediatr Int. 46 (2): 167–70. doi:10.1046/j.1442-200x.2004.01859.x. PMID   15056243. S2CID   8583290.
  9. ↑ Cholet F, Bideau K, Nonent M, Nousbaum JB, Gouérou H, Robaszkiewicz M (2004). "Coexistence of annular pancreas with carcinoma in the dorsal part of pancreas divisum: diagnostic value of magnetic resonance cholangiopancreatography". Abdom Imaging. 29 (6): 703–6. doi:10.1007/s00261-004-0178-3. PMID   15185031. S2CID   19701907.
  10. ↑ Pansini M, Magerkurth O, Haecker FM, Sesia SB (September 2012). "Annular pancreas associated with duodenal obstruction". BMJ Case Rep. 2012: bcr2012006855. doi:10.1136/bcr-2012-006855. PMC   4544741 . PMID   22987909.
  11. ↑ De Ugarte DA, Dutson EP, Hiyama DT (2006). "Annular pancreas in the adult: management with laparoscopic gastrojejunostomy". The American Surgeon. 72 (1): 71–3. doi:10.1177/000313480607200117. PMID   16494188. S2CID   535703.
  12. ↑ Cheng L, Tian F, Zhao T, Pang Y, Luo Z, Ren J (October 2013). "Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases". BMC Gastroenterol. 13: 153. doi: 10.1186/1471-230X-13-153 . PMC   4015270 . PMID   24156788.
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