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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.


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].

Hypoxemia
  • The neonate presented an overwhelming course, with hypoxemia refractory to treatment. At autopsy lung histology showed the characteristic features of ACD/MPV.[ncbi.nlm.nih.gov]
Painless Jaundice
  • Eight weeks later she presented with painless jaundice. Duodenopancreatectomy revealed a duodenal adenocarcinoma, surrounded by an incomplete AP.[ncbi.nlm.nih.gov]
Chronic Abdominal Pain
  • Although annular pancreas in the adult is rare, it may be recognized with increased frequency as a result of more liberal use of pancreatic imaging studies in patients with chronic abdominal pain and suspected chronic pancreatitis.[ncbi.nlm.nih.gov]
  • In older children and adults, it can cause similar symptoms and chronic abdominal pain. Most people with annular pancreas don’t have any symptoms. Annular pancreas is linked with Down syndrome.[assets.cooperhealth.org]
Recurrent Abdominal Pain
  • All children subsequently complained of recurrent abdominal pain.[ncbi.nlm.nih.gov]
  • A 40-years-male patient presented with recurrent abdominal pain for the 8 years. He presented several episodes of postprandial epigastric pain,radiating to back with improvements in position intermittent and vomiting.[ijri.org]
Large Fontanel
  • Less frequent defects seen with VACTERL association are prenatal and postnatal growth deficiency, laryngeal stenosis, ear anomaly, large fontanels, defect of lower limb, rib anomaly, tethered cord, and defects of external genitalia.[ncbi.nlm.nih.gov]

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].

Treatment

  • 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]
  • English language literature about annular pancreas etiology, diagnosis and treatment was reviewed. Both of the patients had uneventfully recovered.[ncbi.nlm.nih.gov]
  • We reviewed overall management, imaging, and surgical treatment in these children. All children subsequently complained of recurrent abdominal pain.[ncbi.nlm.nih.gov]
  • The cases were evaluated according to birth weight, sex, symptoms, methods of diagnosis, associated anomalies, and surgical treatment modalities.[ncbi.nlm.nih.gov]
  • Treatment strategies for annular pancreas with obstructive jaundice remain controversial.[ncbi.nlm.nih.gov]

Prognosis

  • 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]

Etiology

  • 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]
  • English language literature about annular pancreas etiology, diagnosis and treatment was reviewed. Both of the patients had uneventfully recovered.[ncbi.nlm.nih.gov]
  • Several etiologies have been suggested for intestinal perforations. This report hypothesizes the possible involvement of breath stacking in conjunction with partial obstruction by an annular pancreas.[ncbi.nlm.nih.gov]
  • Glazer, GM, Margulis, AR 1979 Annular pancreas: etiology and diagnosis using endoscopic retrograde cholangiopancreatography Radiology 133 303 306 PubMed Google Scholar 5.[dx.doi.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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. Updated by: Michael M.[medlineplus.gov]
  • 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]
  • 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]

Prevention

  • 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]
  • To explain the development of annular pancreas, following theories have been put forward; 1 (i) persistence of left ventral anlage; (ii) adherence of the ventral anlage to the anterior duodenal wall preventing its rotation with the gut; (iii) fusion of[jpgmonline.com]

References

Article

  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.

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Last updated: 2019-06-28 11:56