Annular Pancreas (Anular Pancreas)

An annular pancreas is a congenital anomaly in which the pancreas either partially or completely encircles the duodenum, resulting in the obstruction of the gastrointestinal tract or the biliary tree. Epigastric discomfort, and nausea, followed by relief after vomiting are the main symptoms. The diagnosis is made through the use of imaging studies and invasive upper gastrointestinal endoscopy.

Annular Pancreas is related to the following process: congenital.

Presentation

Annular pancreas, defined as encircling of the duodenum by the pancreas due to improper embryonal development, was initially considered to be a rare finding in clinical practice, with an incidence rate of approximately 1 in 20,000 newborn babies [1] [2]. However, it is recognized as the second most common congenital anomaly of the pancreas (after pancreas divisum), and studies show that up to 1 in 250 individuals have this congenital abnormality, but not all patients develop symptoms [3]. It is diagnosed in two patient populations - in newborns and adults [2] [4] [5]. In newborns, severe obstruction of the duodenum with vomiting is the main presenting symptom [2] [6]. Down syndrome (trisomy 21) has been strongly associated with annular pancreas [3]. On the other hand, the condition may be asymptomatic until adulthood, and patients between 20-60 years of age (more specifically within the 30-50 year range period) can develop a sudden onset of epigastric pain, nausea, relief after vomiting and post-prandial fullness, while hematemesis is seen in about 10% of cases [1] [5]. In addition, an increased incidence of peptic ulcer disease (PUD), acute pancreatitis, biliary obstruction, and jaundice, as well as pancreatic carcinoma is noted in this patient population [1] [6]. It is not uncommon for annular pancreas to be detected incidentally [1] [5].

gastrointestinal
Vomiting
  • […] not through vomiting tho coz both will have bilious vomiting![forums.studentdoctor.net]
  • The main form of presentation is vomiting, which in 90% of cases is the vomiting of food, and not bilious vomiting, because the most frequent location is preampullary. 2 Other types of presentations are pancreatitis, peptic ulcer and obstructive jaundice[elsevier.es]
  • […] annular portion may drain into the main pancreatic duct or directly into the duodenum * Clinical Findings Frequently asymptomatic May present either in infancy or, more commonly, 4 th -5 th decade of life In newborn, duodenal obstruction with bilious vomiting[learningradiology.com]
  • Nausea and vomiting are the usual symptoms and reflect the intestinal obstruction.[medicinenet.com]
  • Clinical features: • Abdominal pain and swelling, nausea or vomiting. • Note: symptoms absent without pancreatitis Exams and Tests: • Abdominal ultrasound • Abdominal CT scan • Amylase and lipase blood test Treatment: • Surgery.[slideshare.net]
Bilious Vomiting
  • […] not through vomiting tho coz both will have bilious vomiting![forums.studentdoctor.net]
  • The usual presentation is with bilious vomiting and intolerance to feed, however, the patient may have associated prolonged jaundice.[jpss.eu]
  • […] from the annular portion may drain into the main pancreatic duct or directly into the duodenum * Clinical Findings Frequently asymptomatic May present either in infancy or, more commonly, 4 th -5 th decade of life In newborn, duodenal obstruction with bilious[learningradiology.com]
  • The main form of presentation is vomiting, which in 90% of cases is the vomiting of food, and not bilious vomiting, because the most frequent location is preampullary. 2 Other types of presentations are pancreatitis, peptic ulcer and obstructive jaundice[elsevier.es]
Chronic Abdominal Pain
  • As explained above, acute recurrent pancreatitis had a higher response rate than chronic pancreatitis or chronic abdominal pain associated with pancreas divisum.[emedicine.medscape.com]
  • In older children and adults, it can cause similar symptoms and chronic abdominal pain.[saintlukeshealthsystem.org]
Recurrent Abdominal Pain
  • A 40-years-male patient presented with recurrent abdominal pain for the 8 years.[ijri.org]
Abdominal Bloating
  • Side effects of pancreatic enzymes are rare but can include abdominal bloating, gas, hyperglycemia (14), and fibrosing colonopathy, which was primarily seen in children with cystic fibrosis receiving high dose pancreatic enzymes (4).[pancreapedia.org]
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  • Entire body system
    Down Syndrome
    Inflammation
    • Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas.[en.wikipedia.org]
    • : inflammation of the tissue that lines the inner wall of the abdomen In addition, adults who have annular pancreas have a higher risk for developing certain types of cancer, including biliary tract and pancreatic cancer.[healthline.com]
    • Disease Peritonitis Peritonitis is an inflammation (irritation) of the peritoneum.[scripps.org]
    • - there is more inflammation that requires hospitalization, careful replacement of body fluid that may be lost and there is usually no permanent damage Severe - the inflammation progresses to tissue death, shock, infection, failure of many organs and[yoursurgery.com]
    • Complications may include: Obstructive jaundice Pancreatic cancer Pancreatitis (inflammation of the pancreas) Peptic ulcer Perforation (tearing a hole) of the intestine due to obstruction Peritonitis Call your health care provider if you or your child[nlm.nih.gov]
    Surgical Procedure
    • Some studies have found that patients with chronic pancreatitis have better response to surgical procedure than endotherapy. [ 20 ] Author Rajan Kanth, MD Hospitalist, Ministry Saint Joseph’s Hospital Rajan Kanth, MD is a member of the following medical[emedicine.medscape.com]
    • Both these surgical procedures are used in bypassing the blocked duodenum Current medical literature informs that a surgical removal of the Annular Pancreas should not be performed.[dovemed.com]
    • The most common surgical procedure to treat an annular pancreas is called a duodenoduodenostomy.[wisegeek.com]
    • Pancreatic cancers occur most often in older people and big operations are required to remove the cancers Surgical Procedures Symptomatic obstructing annular pancreas is treated with a bypass operation.[yoursurgery.com]
    Falling
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  • Workup

    Because certain reports have identified its surprisingly common occurrence, annular pancreas must be included in the differential diagnosis of unexplained gastrointestinal complaints. In neonates and in adults, the role of a detailed patient history revealing the course and progression of symptoms is of pivotal importance, whereas a carefully obtained physical examination is equally important in the assessment of such complaints. Once valid clinical suspicion is raised about a gastrointestinal pathology, imaging studies should be employed. Plain radiography, although being of little benefit in the evaluation of the GI tract, can detect air in the dilated stomach and the duodenal bulb (known as the double-bubble sign) [1] [2]. Abdominal ultrasonography is of limited use in evaluating hollow organs, and so radiological techniques like computed tomography (CT) and magnetic resonance imaging (MRI) are favored [1] [7]. The presence of pancreatic tissue completely or partially surrounding the duodenum is a cardinal feature of annular pancreas [7]. The anomaly may not be large enough to be confirmed through non-invasive imaging studies, however, which is why more direct methods are often necessary to determine the cause of GI symptoms. Magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), and endoscopic ultrasonography (EUS) are recommended procedures definitive diagnosis [1] [2] [3] [6] [7].

    Pathology

    Other Pathologies
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  • Laboratory

    Serum
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  • Imaging

    X-ray
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  • Test Results

    Amniocentesis
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  • Treatment

    Prognosis

    Complications

    Duodenal Obstruction
    • Jaundice neonatorum is usually prolonged in case of any congenital duodenal obstruction.[jpss.eu]
    • The main goal of treatment is the relief of the duodenal obstruction.[joplink.net]
    • If the encirclement is complete, it may be associated with complete or incomplete duodenal obstruction.[usmleforum.com]
    • However, it can cause duodenal obstruction in adults.[radiopaedia.org]
    Small Bowel Obstruction
    • Radiology Plain films may demonstrate proximal small bowel obstruction.[med-ed.virginia.edu]
    • Evidence Based Medicine Research for Annular pancreas Medical research articles related to Annular pancreas include: Duodenal Atresia Tracheoesophageal Fistula (Overview) Small-Bowel Obstruction (Diagnosis) Pancreatitis, Chronic (Overview) Duodenal Atresia[rightdiagnosis.com]
    Duodenal Atresia
    • But I look at it like this, Duodenal atresia will most likely be complete blockage while annular pancreas will be partial blockage - so in real life, Duodenal atresia will most likely show up on the 1st day of life, while annular pancreas may not show[forums.studentdoctor.net]
    • As in other forms of duodenal atresia, malformations of the cranial intestine are also frequently present in patients with annular pancreas.[orpha.net]
    • Petrikovsky BM: First-trimester diagnosis of duodenal atresia.[karger.com]
    • Google Scholar Shih HS, Ko SF, Chaung JH: Is there an association between duodenal atresia and choledochal cyst?.[bmcgastroenterol.biomedcentral.com]
    • Duodenal atresia and stenosis.[jpss.eu]
    Polyhydramnios
    • 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] Presentation [ edit ] Early signs of abnormality include polyhydramnios (an excess of amniotic fluid ), low[en.wikipedia.org]
    • Early signs of abnormality include polyhydramnios, low birth weight and feeding intolerance immediately after birth.[imedpub.com]
    • Conditions that may be associated with annular pancreas include: Down syndrome Excess amniotic fluid during pregnancy (polyhydramnios) Other congenital gastrointestinal problems Pancreatitis Newborns may not feed well.[nlm.nih.gov]
    • Annular pancreas has been associated with maternal polyhydramnios and congenital abnormalities such as Down syndrome, esophageal and duodenal atresia, imperforate anus, and Meckel's diverticulum [ 4-6 ].[uptodate.com]
    Annular Pancreas
    • "Annular pancreas in the adult: management with laparoscopic gastrojejunostomy".[en.wikipedia.org]
    • Causes of Annular pancreas Read more about causes of Annular pancreas .[rightdiagnosis.com]
    Intestinal Obstruction
    • Nausea and vomiting are the usual symptoms and reflect the intestinal obstruction.[medicinenet.com]
    • ., Chebbi, F. and Sassi, S.A. (2011) A rare cause of proximal intestinal obstruction in adults—Annular pancreas: A case report.[scirp.org]
    • Chebbi A rare cause of proximal intestinal obstruction in adults: annular pancreas: a case report Pan Afr Med J, 10 (2011), pp. 56 2 J.[elsevier.es]
    • The most frequently found symptoms are: abdominal pain (70%), vomiting and nausea (47%), and they are generally reflecting a proximal intestinal obstruction [8].[dx.doi.org]
    • Lynn : Intestinal obstruction in the newborn infant.[link.springer.com]
    Down Syndrome
    Congenital Abnormality
    • INTRODUCTION Annular pancreas is a rare congenital abnormality characterized by a ring of pancreatic tissue surrounding the descending portion of the duodenum.[uptodate.com]
    • Clinical Annular pancreas is a rare congenital abnormality in which a ring of pancreatic tissue encircles the duodenum at or above the major papilla.[med-ed.virginia.edu]
    • It has a prevalence of one in 2,000 persons and occurs either as an isolated finding or with other congenital abnormalities.[manju-imagingxpert.blogspot.com]
    • Typically, in such cases, other congenital abnormalities are present in the infant, together with Annular Pancreas, thus increasing its mortality If the symptoms occur during adulthood, then with prompt and appropriate treatment, the prognosis is usually[dovemed.com]
    • abnormality in which the duodenum is surrounded by a ring of pancreatic tissue.[icd10data.com]
    Pancreatitis
    • 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.[en.wikipedia.org]
    • Pancreatitis and the risk of pancreatic cancer.[pancreapedia.org]
    • RVP, right ventral pancreatic anlage; LVP, left ventral pancreatic anlage; DP, dorsal pancreatic anlage.[hindawi.com]
    • Annular pancreas associated with diffuse chronic pancreatitis.[joplink.net]
    Dystocia
    • The baby was born to a 20-year old primigravida, in that hospital, through a Caesarian section due to cervical dystocia.[jpss.eu]

    Etiology

    Causes

    Epidemiology

    Sex distribution
    Age distribution

    Pathophysiology

    Prevention

    Summary

    Patient Information

    Other symptoms

    Congenital
    • Q45 ICD-10-CM Diagnosis Code Q45 Other congenital malformations of digestive system 2016 2017 2018 Non-Billable/Non-Specific Code Type 2 Excludes congenital diaphragmatic hernia ( Q79.0 ) congenital hiatus hernia ( Q40.1 ) Other congenital malformations[icd10data.com]
    • There are various types of congenital anomalies of the pancreas in humans.[hindawi.com]
    • Abstract Annular pancreas is a congenital malformation of pancreas often associated with congenital duodenal stenosis.[jpss.eu]
    • No other congenital anomaly of the intra abdominal organs was noted.[dx.doi.org]
    No Development
    • Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas.[en.wikipedia.org]
    • Figure 1: (a) Normal development of the pancreas.[hindawi.com]
    • Develop Your Own Curriculum Choose courses based on your needs.[medscape.com]
    • (Etiology) Annular Pancreas is caused by an abnormal development of the pancreas.[dovemed.com]
    • Annular pancreas develops due to failure of ventral bud to rotate with duodenum, causing encasement of duodenum.[scradiology.com.au]
    No Abnormalities
    • Causes [ edit ] It is typically associated with abnormal embryological development, however adult cases can develop.[en.wikipedia.org]
    • Annular Pancreas is a congenital developmental abnormality.[dovemed.com]
    • ICD-10-CM Codes › Q00-Q99 Congenital malformations, deformations and chromosomal abnormalities › Q38-Q45 Other congenital malformations of the digestive system › Q45- Other congenital malformations of digestive system › Annular pancreas 2016 2017 2018[icd10data.com]
    • INTRODUCTION Annular pancreas is a rare congenital abnormality characterized by a ring of pancreatic tissue surrounding the descending portion of the duodenum.[uptodate.com]
    • It is usually occurs due to abnormal or extra embryological development.[imedpub.com]
    Birth
    • […] weight , and feeding intolerance immediately after birth.[en.wikipedia.org]
    • Summary Epidemiology The annual incidence is estimated at around 1/50,000 births, representing 10% of all cases of duodenal stenosis.[orpha.net]
    • Early signs of abnormality include polyhydramnios, low birth weight and feeding intolerance immediately after birth.[imedpub.com]
    • Annular pancreas is a birth defect.[saintlukeshealthsystem.org]
    Duodenal Stenosis
    • These findings might be attributed to the absence of intrinsic duodenal stenosis.[jpss.eu]
    • The factors initiating symptoms are recurrent pancreatitis, duodenal stenosis, and duodenal or gastric ulceration [3].[dx.doi.org]
    • Differential diagnosis The differential diagnosis should include other forms of duodenal stenosis.[orpha.net]
    • Gas charging demonstrated duodenal stenosis with dilatation of the stomach and duodenal bulb (triangle) (B) .[bmcgastroenterol.biomedcentral.com]
    • Stanley P, Law BS, Young LW: Down’s syndrome, duodenal stenosis/annular pancreas, and a stack of coins.[karger.com]
    Endoscopic Retrograde Cholangiopancreatography
    • Endoscopic retrograde cholangiopancreatography (ERCP) is the diagnostic modality of choice, and in combination with computed tomography the diagnosis can be obtained preoperatively in many patients.[ncbi.nlm.nih.gov]
    • ., Shibue, T. and Hashimoto, S. (1987) Annular pancreas detected in adults, diagnosed by endoscopic retrograde cholangiopancreatography: Report of four cases.[scirp.org]
    • retrograde cholangiopancreatography) UGI series May show extrinsic compression on both lateral and medial walls of the 2 nd portion of duodenum CT May be mistaken for thickening of the duodenal wall On MRCP or ERCP, the duct of the annular pancreas usually[learningradiology.com]
    • Yogi, Y., Shibue, T. and Hashimoto, S. (1987) Annular pancreas detected in adults, diagnosed by endoscopic retrograde cholangiopancreatography: Report of four cases.[dx.doi.org]
    • A diagnosis of Annular Pancreas would involve: X-ray study of the abdomen: This may show the classical “double-bubble” sign Ultrasound, CT scan of abdomen Contrast studies of small intestines, using barium An ERCP (endoscopic retrograde cholangiopancreatography[dovemed.com]
    Low Birth Weight
    • birth weight , and feeding intolerance immediately after birth.[en.wikipedia.org]
    • Early signs of abnormality include polyhydramnios, low birth weight and feeding intolerance immediately after birth.[imedpub.com]
    • They include the following, observed during or after childbirth: Low birth weight Difficulty in feeding, right from birth, resulting in nausea and vomiting Polyhydramnios (increased amount of amniotic fluid) during pregnancy, seen in the mother Not all[dovemed.com]
    Laparoscopy
    • Laparoscopy confirmed the preoperative findings of dilatation of the first duodenal portion and pancreatic tissue surrounding the second portion.[elsevier.es]
    • Gluer S, Petersen C, Ure BM: Simultaneous correction of duodenal atresia due to annular pancreas and malrotation by laparoscopy.[karger.com]
    • Functioning endocrine tumors will have elevated hormone levels that are specific to the tumor type Laparoscopy - A lighted tube with lenses at one end and a TV camera at the other is inserted into the abdomen.[yoursurgery.com]
    Pancreatic Disease
    • Epidemiology of alcohol-related liver and pancreatic disease in the United States.[pancreapedia.org]
    • Understanding how the pancreas develops is essential to understand the pathogenesis of congenital pancreatic diseases.[hindawi.com]
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    References

    1. Rondelli F, Bugiantella W, Stella P, et al. Symptomatic annular pancreas in adult: Report of two different presentations and treatments and review of the literature. Int J Surg Case Rep. 2016;20(Suppl):21-24.
    2. Kandpal H, Bhatia V, Garg P, Sharma R. Annular pancreas in an adult patient: diagnosis with endoscopic ultrasonography and magnetic resonance cholangiopancreatography. Singapore Med J. 2009;50(1):e29-31.
    3. Mittal S, Jindal G, Mittal A, Singal R, Singal S. Partial annular pancreas. Proc (Bayl Univ Med Cent). 2016;29(4):402-403.
    4. Patra DP, Basu A, Chanduka A, Roy A. Annular Pancreas: A Rare Cause of Duodenal Obstruction in Adults. Indian J Surg. 2011;73(2):163-165.
    5. Kasper DL, Fauci AS, Hauser SL, Longo DL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 19e. New York, NY: McGraw-Hill; 2016.
    6. Sandrasegaran K, Patel A, Fogel EL, Zyromski NJ, Pitt HA. Annular pancreas in adults. AJR Am J Roentgenol. 2009 Aug;193(2):455-60.
    7. Alahmadi R, Almuhammadi S. Annular Pancreas: A Cause of Gastric Outlet Obstruction in a 20-Year-Old Patient. Am J Case Rep. 2014;15:437-440.

    • Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn - J Alameh, A Bachiri, L Devisme, P Truffert - European journal of , 2002 - Springer
    • Annular pancreas divisum—a report of two cases and review of the literature - RE England, MK Newcomer, JWC Leung - British journal of , 1995 - Br Inst Radiology
    • Annular pancreas causing localized recurrent pancreatitis in a child: report of a case - Y Ohno, T Kanematsu - Surgery today, 2008 - Springer
    • Annular pancreas - MM Ravitch, AC Woods Jr - Annals of Surgery, 1950 - ncbi.nlm.nih.gov


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