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 Table of Contents  
Year : 2017  |  Volume : 3  |  Issue : 3  |  Page : 154-157

Acute pancreatitis in a patient with Situs inversus/polysplenia syndrome: A case report and review of literature

1 Department of Surgery, Bronx Lebanon Medical Center, Bronx, NY; OPUS 12 Foundation, Columbus, OH, USA
2 Department of Emergency Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
3 OPUS 12 Foundation, Columbus, OH, USA

Date of Web Publication21-Apr-2017

Correspondence Address:
Srinivas Kavuturu
Department of Surgery, Bronx Lebanon Medical Center, 1650 Grand Concourse, Bronx, New York 10456
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJAM.IJAM_7_17

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A 45-year-old man was admitted and evaluated for epigastric pain. Computerized tomography scan revealed acute pancreatitis as well as incidental Situs inversus and polysplenia. The patient did not have any congenital anomalies that are usually associated with polysplenia syndrome. No anatomical anomalies were found in the duodenum, the pancreas, or biliary tree to account for pancreatitis. Based on history of alcohol abuse, this case was treated as alcohol-induced pancreatitis. Complete resolution of pancreatitis was seen. This case is unique in that it describes acute pancreatitis in the setting of heterotaxy (Situs inversus)/polysplenia syndrome.
The following core competencies are addressed in this article: Medical knowledge, Patient care.
Republished with permission from: Kavuturu S, Roper W, Stawicki SP. Acute pancreatitis in a patient with Situs inversus/polysplenia syndrome: A case report and review of literature. OPUS 12 Scientist 2008;2(4):10-12.

Keywords: Acute pancreatitis, computed tomography, heterotaxy, polysplenia, Situs inversus

How to cite this article:
Kavuturu S, Roper W, Stawicki SP. Acute pancreatitis in a patient with Situs inversus/polysplenia syndrome: A case report and review of literature. Int J Acad Med 2017;3, Suppl S1:154-7

How to cite this URL:
Kavuturu S, Roper W, Stawicki SP. Acute pancreatitis in a patient with Situs inversus/polysplenia syndrome: A case report and review of literature. Int J Acad Med [serial online] 2017 [cited 2022 Jun 27];3, Suppl S1:154-7. Available from: https://www.ijam-web.org/text.asp?2017/3/3/154/204962

  Introduction Top

Situs inversus is a rare congenital anomaly with an approximate incidence of 1 in 10,000 live births.[1],[2],[3],[4] The normal position of the organs is known as Situs solitus. Situs inversus is a condition, in which the usual positions of the organs are reversed from left to right as a mirror image of the normal anatomy. Situs ambiguous covers everything in between these two anatomic configurations on a continuum of asymmetric thoracic and abdominal organ formation. The term heterotaxyhas been used to encompass these syndromes which involve abnormal symmetry and malposition of the thoracic and abdominal organs and vasculature, including complex congenital heart and extracardiac abnormalities.[5],[6],[7],[8] Perhaps, the best-known example of heterotaxyis the Kartagener syndrome, a combination of Situs inversus, bronchiectasis, and male infertility attributed to abnormal ciliary motility.[9]

Polysplenia is seen in almost 50% of patients with heterotaxysyndrome. Various gastrointestinal tract malformations have been reported to occur in the syndrome as well, including failure of the head of pancreas to form, duodenal duplications or atresia, intestinal malrotation, and biliary atresia.

We present a case of an adult patient with Situs inversus, polysplenia, and pancreatitis without any duodenal or biliary malformations directly contributory to pancreatitis. Only one case of recurrent pancreatitis in polysplenic patient has been previously reported. The authors of that report attributed pancreatitis to a duodenal diverticulum that was found to be obstructing the pancreatic duct.[10]

  Case Report Top

A 45-year-old, previously healthy male presented to the emergency department with acute upper abdominal pain, nausea and vomiting. Laboratory tests were consistent with pancreatitis. Computed tomography (CT) scan revealed dextrocardia, Situs inversus totalis, polysplenia, and acute pancreatitis [Figure 1] and [Figure 2]. Malrotation of the bowel was noted, with the cecum in the midline. The duodenum did not cross the midline. The inferior vena cava (IVC) was on the right side, with azygos continuation of the IVC.
Figure 1: Computed tomographic scan of the abdomen showing pancreatitis with Situs inversus

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Figure 2: Computed tomography scan of abdomen of the patient showing polysplenia in the right upper quadrant of the abdomen

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An ultrasound of the upper abdomen failed to reveal any gallstones or common bile duct abnormalities. A contrast study of the stomach and duodenum revealed right-sided stomach and free emptying of the barium into the duodenum. There was no evidence of atresia or diverticulae of the duodenum or proximal jejunum. The small bowel was situated to the left of the midline. The contrast material was seen reaching the colon. Magnetic resonance imaging (MRI) of the biliary tract (magnetic resonance cholangiopancreatography [MRCP]) and pancreas did not reveal any other anatomical abnormalities. Electrocardiography and echocardiography did not demonstrate any cardiovascular anomalies. Due to the lack of any anatomic abnormalities, and given the patient's history of alcohol abuse, the pancreatitis was attributed to alcohol consumption. Patient was treated conservatively, with complete resolution of the disease.

  Discussion Top

Heterotaxyresults from failure of the developing embryo to establish normal left-right asymmetry. This results in malposition of thoraco-abdominal organs and vasculature, complex congenital heart, and extracardiac abnormalities. The term situsis applied to specify the left-right anatomic orientation of various asymmetric body structures. There are two types of unambiguous situs: (a) Situs solitus - the normal, and (b) Situs inversus- a mirror image of the normal.[1]

In patients with heterotaxysyndrome, the spleen is almost always affected although the reason is not clearly understood. Three types of splenic anomalies have been described: (a) The spleen may be absent; (b) the spleen may be composed of a cluster of smaller splenules, a large spleen may be accompanied by several smaller splenules or it may be multilobed; and (c) the spleen may be of normal size but located in the right upper quadrant of the abdomen. In a study of 109 autopsies of visceral heterotaxywith congenital heart disease, 58 patients (53%) had asplenia, 46 (42%) had polysplenia, and 5 (5%) had a single right-sided spleen. The polysplenia and asplenia syndromes, each have their own associated characteristic anomalies.[2],[3],[4]

In heterotaxywith polysplenia, the liver if frequently characterized by abnormal symmetry (50%–67%).[5],[6] Portal vein often runs ventral to the duodenum, and biliary atresia has been found to be associated with hypoplasia of the portal vein.[7] The preduodenal position of the portal vein also occurs in patients with duodenal atresia and among patients with annular pancreas. It is very important to be aware of this anomalous pattern during laparotomy to avoid undue bleeding during surgery. An abnormally “short” pancreas can be found in up to 50% of the cases.[7] Interruption of the IVC with continuation through a dilated azygos or hemiazygos vein into the superior vena cava is seen in 58%–100% of cases. There also may be a partial or total anomalous pulmonary venous drainage and dextrocardia are seen in 33%–42% of cases. Of interest, complete heart block is found in over 20% of patients.[8]

Patients with polysplenia and asplenia may present with intestinal obstruction resulting from mesenteric abnormalities, which include common mesentery, abnormal attachments, and intestinal malrotation. Rarely, an intraluminal duodenal diverticulum has been associated with duodenal atresia or stenosis as a part of the polysplenia syndrome.[10] Duodenal diverticulum was previously described to be contributory to recurrent attacks of pancreatitis in this setting, with resection of the diverticulum leading to complete remission of pancreatisis.[10]

Acute pancreatitis in heterotaxysyndrome with polysplenia seems not to have been reported in the past although it may seem that patients with similar anomalies of the biliary tree, duodenum, and/or the pancreas would be at increased risk for pancreatic-related complications. It is important to investigate patients with heterotaxy/polysplenia syndrome who present with pancreatitis for correctable structural anomalies as possible causes of their pancreatitis.

The combination of ultrasound and CT imaging can diagnose most of the intra-abdominal anomalies contributory to pancreatitis. MRI with MRCP may be useful as adjunctive diagnostic tools. Endoscopic retrograde cholangiopancreatography may be valuable as both diagnostic and therapeutic procedure in selected cases. An upper gastrointestinal series can help diagnose duodenal diverticulum, bowel atresia, and malrotation. An electrocardiogram should be performed to rule out cardiac conduction disturbances and other cardiac rhythm disorders. Echocardiography is the main diagnostic tool for diagnosing complex cardiovascular anomalies. Cardiac catheterization may be necessary in some patients.

  Conclusions Top

Any patient with heterotaxy/polysplenia syndrome with pancreatitis should be aggressively investigated for structural and biochemical abnormalities that could potentially contribute to pancreatitis. The finding of a surgically correctable cause of pancreatitis can lead to curative surgery. Alcohol and gallstones should still be regarded as more common causes of pancreatitis in heterotaxy/polysplenia syndrome.


Justifications for republishing this scholarly content include: (a) The phasing out of the original publication after a formal merger of OPUS 12 Scientist with the International Journal of Academic Medicine and (b) Wider dissemination of the research outcome(s) and the associated scientific knowledge.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Bartram U, Wirbelauer J, Speer CP. Heterotaxy syndrome -- Asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate 2005;88:278-90.  Back to cited text no. 1
Van Praagh S, Santini F, Sanders SP. Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndrome). In: Flyer D, editor. Nadas Pediatric Cardiology. Philadelphia: Hanley & Belfus Inc., Mosby; 1992. p. 589-608.  Back to cited text no. 2
Van Praagh S, Kakou-Guikahue M, Kim HS, Becker J, Alday L, Van Praagh R. Atrial situs in patients with heterotaxy and congenital heart disease: Conclusions based on findings in 104 postmortem cases. Coeur 1988;19:484-502.  Back to cited text no. 3
Van Praagh S, Kreutzer J, Alday L, Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy, with emphasis on the diagnosis of atrial situs: A study of 109 cases postmortem cases. In: Clark E, Takao A, editors. Developmental Cardiology: Morphogenesis and Function. Mt. Kisco, NY: Futura; 1990. p. 671-721.  Back to cited text no. 4
Moller JH, Nakib A, Anderson RC, Edwards JE. Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral “left-sidedness”. Circulation 1967;36:789-99.  Back to cited text no. 5
Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: Imaging of the heterotaxy syndrome. Radiographics 1999;19:837-52.  Back to cited text no. 6
Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al. Polysplenia syndrome detected in adulthood: Report of eight cases and review of the literature. Abdom Imaging 1999;24:178-84.  Back to cited text no. 7
Walsh EP. Electrocardiography and introduction to electrophysiological techniques. In: Flyer D, editor. Nadas' Pediatric Cardiology. Philadelphia: Hanley and Belfus Inc., Mosby; 1992. p. 117-58.  Back to cited text no. 8
Ihsan-ullah N, Shahab-ud D. Kartagener's syndrome. Gomal J Med Sci 2006;4:79-81.  Back to cited text no. 9
Lundstedt C, Lyttkens K, Andrén-Sandberg A. Intraluminal duodenal diverticulum causing pancreatitis in a patient with a polysplenia syndrome. Eur Radiol 1998;8:454-7.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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