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].

• Pain

  The patient reported history of multiple episodes of abdominal pain similar in nature over the last 2 years. [ncbi.nlm.nih.gov]

  Laying supine aggravated the pain while belching alleviated the pain. He had no fever, chills, shortness of breath, cough or chest pain. Nevertheless, his initial lab work indicated a white count of 12,600 with left shift. [digitalcommons.hsc.unt.edu]

  […] obstruction in adults. [2] Case Reports Case 1 A 42-year-old male patient presented with 7 days history of epigastric pain and vomiting after meals. [ijhas.in]

• Inflammation

  Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas. Postnatal diagnostic procedures include abdominal x-ray and ultrasound, CT scan, and upper GI and small bowel series. [en.wikipedia.org]

  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]

  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]

• Trisomy 21

  It does not correlate as strongly with trisomy 21 karyotype as do the duodenal atresias, and oral feeding tolerance time is nearly the same between the DD and DD+TE+TJT groups. [ncbi.nlm.nih.gov]

  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. [en.wikipedia.org]

  Chromosomal abnormalities are present in one-third of cases of annular pancreas, with trisomy 21 (followed by trisomy 18 and 13; see these terms) being the most frequently detected anomaly. [orpha.net]

• Falling

  Albrecht, E. (1901) Ein Fall von Pankreasbildung in einem Meckelschen Divertikel. Münchener Med. Wochenschrift 48:20612062. Albrecht, E. und L. [anatomyatlases.org]

  Cases usually fall in two age groups - infantile (from birth to 4 years) and adults (from 17 onwards). When it occurs in infants, the mothers may have polyhydramnios. [jpgmonline.com]

  Der Fall wird gemeinsam mit den in der Literatur bereits vorgestellten 6 weiteren Fällen diskutiert. In allen publizierten Familien erkrankten insgesamt 16 Personen an der beschriebenen Anomalie, 14 scheinen gesund zu sein. [doi.org]

  : Ein Fall von Pankreas anulare. Fortschr. Roentgenstr. 101, 669 (1964). PubMed Google Scholar 41. Fèvre, M. : Deux points du diagnostic radiologique dans les sténoses duodénales par pancréas annulaire. Ann. Chir. infant. 2, 59 (1961). [link.springer.com]

• Underweight

  She had not previously sought any medical advice despite her intermittent vomiting and underweight. [ncbi.nlm.nih.gov]

• Vomiting

  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 epigastric distention associated with non-biliary vomiting (the obstruction [en.wikipedia.org]

  In newborns, severe obstruction of the duodenum with vomiting is the main presenting symptom. Down syndrome (trisomy 21) has been strongly associated with annular pancreas. [symptoma.com]

  Moderate obstruction causes recurrent vomiting in adults usually between 20 and 50 years of age. Diagnosis may be made with gastroscopy, CT and MRI 2 or at laparotomy. [casereports.bmj.com]

  Those with severe blockage present as a baby with vomiting, inability to tolerate milk or formula. Sometimes the vomited fluid has bile (color green or yellow). [eapsa.org]

  She was transferred, directly after delivery, to our neonatal intensive care unit presenting with distended upper abdomen and vomiting. [ncbi.nlm.nih.gov]

• Nausea

  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. [symptoma.com]

  A case is reported of an 82-year-old patient who presented with a 3-month history of nausea and vomiting associated with weight loss who at laparotomy was found to have an annular pancreas. [ncbi.nlm.nih.gov]

• Intestinal Atresia

  Keywords: Annular pancreas, duodenal obstruction, familial intestinal atresia How to cite this article: Noor R, Mohamad Z, Farhan S, Nor T. Annular pancreas causing duodenal obstruction in two consecutive sisters. [saudisurgj.org]

  Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. East Afr Med J. 1993; 70 :558–564. [ PubMed ] [ Google Scholar ] 49. Falade AG, Shonubi AM. [ncbi.nlm.nih.gov]

  Learning points First clue of an intestinal atresia will often be finding hydramnios on prenatal ultrasound. Following delivery, vomiting is most common early symptom. [doi.org]

• Epigastric Pain

  […] obstruction in adults. [2] Case Reports Case 1 A 42-year-old male patient presented with 7 days history of epigastric pain and vomiting after meals. [ijhas.in]

  We report a new case involving a 24-year-old woman hospitalised for epigastric pain, nausea and vomiting. Radiological examination was consisted with an annular pancreas. [ncbi.nlm.nih.gov]

  Figure 1 A 79-year-old man had a five-month history of dull, aching epigastric pain radiating to the back and a 16-kg (35-lb) weight loss. The pain was made worse by meals and was associated with early satiety. [nejm.org]

• Bilious Vomiting

  Abstract We present a low-birth weight newborn who presented with bilious vomit and had a “double bubble” sign on plain abdominal X-ray. Intraoperatively, we found annular pancreas with malrotation and type I choledochal cyst. [ncbi.nlm.nih.gov]

  2015/20/3/155/154656 Bilious vomit in neonates is almost always pathological and malrotation of the gut is one of the often encountered entities on exploration of such a case. [dx.doi.org]

  The baby also had two episodes of bulky bilious vomiting, few days th back, but the baby was tolerating the feeds and passing stool. The color of stool was some times greenish and other times pale. [jpss.eu]

• Back Pain

  PATIENT CONCERNS: A 74-year-old man suffered from upper abdominal and back pain. DIAGNOSES AND INTERVENTIONS: Contrast enhanced computed tomography indicated a low-density mass in the body of the pancreas. [ncbi.nlm.nih.gov]

  Symptoms of chronic pancreatitis include abdominal pain, nausea, vomiting, weight loss, diarrhea, and diabetes. Abdominal pain is the most common presenting symptom. The abdominal pain is typically epigastric and radiates to the back. [pancreapedia.org]

• Ulcer

  Incomplete Annular Pancreas: An Unusual Presentation of Peptic Ulcer Disease. Abstract published at Hospital Medicine 2012, April 1-4, San Diego, Calif. [shmabstracts.com]

  presenting with duodenal obstruction and duodenal ulcer underwent Billroth II gastrectomy. [ncbi.nlm.nih.gov]

  Duodenal obstruction with consecutive ulcers was the clinical manifestation in a young woman requiring duodeno-jejunostomy. [hungary.pure.elsevier.com]

• Suggestibility

  In addition to stringent diagnostic criteria based on 2D ultrasound, this case suggests the possible contribution of 3D ultrasound in rare cases of suspected annular pancreas. [ncbi.nlm.nih.gov]

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].

Support treatment, inhaled nitric oxide, oral sildenafil and nebulized iloprost were used with no clinical improvement. The neonate presented an overwhelming course, with hypoxemia refractory to treatment. [ncbi.nlm.nih.gov]

Management and treatment Investigations using a gastric tube allow drainage of the digestive tract prior to the site of obstruction. Treatment is surgical and is performed in the neonatal period. [orpha.net]

Prognosis The prognosis for annular pancreas is very favorable, but the overall prognosis depends on the presence of associated malformations. The documents contained in this web site are presented for information purposes only. [orpha.net]

Prognosis is excellent, despite the frequent association with chromosomal anomalies and major congenital malformations. [ncbi.nlm.nih.gov]

Prognosis: • Good outcome with surgery. [slideshare.net]

We will describe a case of identical (monochorionic diamniotic) female twins with neonatal presentation of duodenal obstruction and annular pancreas, and discuss data supportive of a genetic etiology. [ncbi.nlm.nih.gov]

Pregnancy test was negative and gynecologic etiologies were ruled out. [shmabstracts.com]

Summary Epidemiology The annual incidence is estimated at around 1/50,000 births, representing 10% of all cases of duodenal stenosis. [orpha.net]

Epidemiology of alcohol-related liver and pancreatic disease in the United States. Arch Intern Med 168:649–656, 2008. PMID: 18362258 [pancreapedia.org]

Digestive disorders in the United States: epidemiology and impact. U.S. Department of Health and Human Services, National Institutes of Health. Washington, DC: U.S. Government Printing Office,1994 : 691–712 [Google Scholar] 34. [ajronline.org]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 48. Updated by: Michael M. [nlm.nih.gov]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 48. Version Info Last reviewed on: 10/26/2017 Reviewed by: Michael M. [mountsinai.org]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 48. Reviewed By: Michael M. [ufhealth.org]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 48. Review Date: 10/26/2017 Reviewed By: Michael M. [pennstatehershey.adam.com]

The patient was successfully treated by duodenoduodenostomy, which, by correcting the duodenopancreatic reflux, prevented the later development of pancreatitis. [ncbi.nlm.nih.gov]

These products are not intended to diagnose, treat, cure, or prevent any disease. [nutrivene.com]

This can result in severe complications from an injured pancreas, such as pancreatic fistula formation and acute pancreatitis How can Annular Pancreas be Prevented? Annular Pancreas is a congenital developmental abnormality. [dovemed.com]

A loss of function of CTRC prevents trypsin breakdown, therefore allowing more trypsin to cause tissue injury. [pancreapedia.org]

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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.
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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.