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. Saudi Med J 2007;28:1050–1054.[karger.com]
    • Intestinal atresia, stenosis, and malrotation. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 330. Semrin MG, Russo MA.[nlm.nih.gov]
    • Intestinal atresia, stenosis, and malrotation. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 330. Semrin MG, Russo MA.[ufhealth.org]
    Bilious Vomiting
    • […] not through vomiting tho coz both will have bilious vomiting! Last edited: Apr 21, 2013 That is true. They both would have double bubble sign but duodenal atresia is mostly associated with Down Syndrome so they would mention some history of that.[forums.studentdoctor.net]
    • 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]
    • […] 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
    • 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.[novanthealth.org]
    • 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. He presented several episodes of postprandial epigastric pain,radiating to back with improvements in position intermittent and vomiting.[ijri.org]
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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

    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]
    • With medical treatment, the clinical course of the pancreatitis and the related duodenal obstruction is generally favorable. The main goal of treatment is the relief of the duodenal obstruction.[joplink.net]
    • In children, it may be associated with other congenital anomalies or cause duodenal obstruction, while in adults, pancreatitis is the usual presentation. However, it can cause duodenal obstruction in adults.[radiopaedia.org]
    • Annular pancreas a rare anomaly, can present as recurrent peptic ulcer disease and symptoms of duodenal obstruction. Image 1Arrows pointing incomplete pancreatic annulas. Image 2GI Series.[shmabstracts.com]
    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". Pediatr Int . 46 (2): 167–70. doi : 10.1046/j.1442-200x.2004.01859.x . PMID 15056243 .[en.wikipedia.org]
    Intestinal Malrotation
    • "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 .[en.wikipedia.org]
    • Associated anomalies Duodenal bands, intestinal malrotation, Meckel’s diverticulum, cryptorchidism, cardiac and spinal cord defects; up to 20% have Down syndrome. Aetiology Unknown. Diagnosis Upper GI series, abdominal CT, endoscopy.[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. J Clin Ultrasound 2004;32:86–90.[karger.com]
    Dystocia
    • The baby was born to a 20-year old primigravida, in that hospital, through a Caesarian section due to cervical dystocia. The patient developed jaundice on the second day of life.[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

    Epidemiology

    Sex distribution
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    Pathophysiology

    Prevention

    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" . J Indian Assoc Pediatr Surg . 20 (3): 155–6. doi : 10.4103/0971-9261.154656 . PMC 4481632 . PMID 26166991 .[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 . Invasive pulmonary aspergillosis is a serious infection with pneumonia.[aspirus.org]
    Clostridium Difficile
    • Stool studies for ova, parasites and clostridium difficile toxin were negative. Pregnancy test was negative and gynecologic etiologies were ruled out.[shmabstracts.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.

    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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