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].

Skin
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  • gastrointestinal
    Intestinal Atresia
    • Ozturk H, Ozturk H, Gedik S, Duran H, Onen A: A comprehensive analysis of 51 neonates with congenital intestinal atresia.[karger.com]
    • Intestinal atresia, stenosis, and malrotation.[nlm.nih.gov]
    Chronic Abdominal Pain
    • In older children and adults, it can cause similar symptoms and chronic abdominal pain.[saintlukeshealthsystem.org]
    • 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]
    Recurrent Abdominal Pain
    • A 40-years-male patient presented with recurrent abdominal pain for the 8 years.[ijri.org]
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  • Face, Head & Neck
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  • Fetus
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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].

    Imaging

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    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]
    • Annular pancreas is one such unusual cause of peptic ulcer disease and duodenal obstruction.[shmabstracts.com]
    • However, it can cause duodenal obstruction in adults.[radiopaedia.org]
    Volvulus
    • Annular Pancreas was found at the surgery Answer Diagnosis: Annular Pancreas Differential Diagnosis for Double Bubble: Duodenal atresia Annular Pancreas Malrotation with midgut volvulus with Ladd bands Preduodenal portal vein Choledochal cyst Duplication[pedsradiology.com]
    • "Rare clinical presentation mode of intestinal malrotation after neonatal period: Malabsorption-like symptoms due to chronic midgut volvulus".[en.wikipedia.org]
    Intestinal Malrotation
    • "Rare clinical presentation mode of intestinal malrotation after neonatal period: Malabsorption-like symptoms due to chronic midgut volvulus".[en.wikipedia.org]
    • Associated anomalies Duodenal bands, intestinal malrotation, Meckel’s diverticulum, cryptorchidism, cardiac and spinal cord defects; up to 20% have Down syndrome.[medical-dictionary.thefreedictionary.com]
    • malrotation, esophageal atresia and cardiovascular anomalies. 2 Its diagnosis in adults is uncommon (3 out of 20 000 autopsies) 3 and is made during the study of a duodenal obstruction.[elsevier.es]
    • In the pediatric population, other congenital abnormalities such as cardiac defects, intestinal atresia, biliary anomalies, tracheoesophageal fistulae, intestinal malrotation and trisomy-21 are also often present with annular pancreas. 5 In the adult[digitalcommons.hsc.unt.edu]
    • Hsu CY, Chiba Y, Fukui O, Sasaki Y, Miyashita S: Counterclockwise barber-pole sign on prenatal three-dimensional power Doppler sonography in a case of duodenal obstruction without intestinal malrotation.[karger.com]
    Dystocia
    • The baby was born to a 20-year old primigravida, in that hospital, through a Caesarian section due to cervical dystocia.[jpss.eu]
    Appendicitis
    • […] in children : Because appendicitis is one of the more feared conditions for a child with abdominal pain, it can be over-diagnosed (it can, of course, also fail to be diagnosed with... read more » Poorly healing leg rashes a classic sign of chronic disease[rightdiagnosis.com]
    Crohn's Disease
    • In patients hospitalized for persistent symptoms of peptic ulcer disease in the absence of common causes like H. pylori , NSAID use etc, suspicion for unusual etiologies like gastrinoma, crohn’s disease, carcinoid or congenital malformations should be[shmabstracts.com]
    • Annular pancreas: Undiagnosed Conditions Commonly undiagnosed diseases in related medical categories: Chronic Digestive Disorders that can remain Undiagnosed: Crohn's Disease -- Undiagnosed Ulcerative Colitis -- Undiagnosed Celiac Disease -- Undiagnosed[rightdiagnosis.com]

    Etiology

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    Summary

    Patient Information

    Other symptoms

    Low Birth Weight
    • Early signs of abnormality include polyhydramnios, low birth weight and feeding intolerance immediately after birth.[imedpub.com]
    • "Annular pancreas, type I choledochal cyst and malrotation in a low-birth weight newborn: A case report" .[en.wikipedia.org]
    • 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]
    No Obesity
    • […] undiagnosed in children : A variety of conditions are associated with obesity (see obesity ), but these tend to be undiagnosed more often in child patients than in adults.[rightdiagnosis.com]
    Lung Scarring
    • Aspergilloma is a growth (fungus ball) that develops in an area of past lung disease or lung scarring such as tuberculosis or lung abscess .[aspirus.org]
    Clostridium Difficile
    • Stool studies for ova, parasites and clostridium difficile toxin were negative.[shmabstracts.com]
    Amphotericin B
    • Possible Complications Health problems from the disease or treatment include: Amphotericin B can cause kidney damage and unpleasant side effects such as fever and chills Bronchiectasis (permanent scarring and enlargement of the small sacs in the lungs[aspirus.org]

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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.

    1. 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
    2. 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
    3. Annular pancreas causing localized recurrent pancreatitis in a child: report of a case - Y Ohno, T Kanematsu - Surgery today, 2008 - Springer
    4. Annular pancreas - MM Ravitch, AC Woods Jr - Annals of Surgery, 1950 - ncbi.nlm.nih.gov


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