Introduction
Polysplenia syndrome is a rare congenital subtype of heterotaxy syndrome associated with various visceral and vascular anomalies. It is characterized by the presence of 2 or more spleens and asymmetrical abnormalities of other organs [1]. Technological advances in medical imaging through ultrasound and computed tomography have improved their detections and radiological characterizations [2]. It is very often incidentally discovered in adults during an imaging assessment [3]. We report a case of polysplenia syndrome revealed by acute pancreatitis.
Observation
A 36-year-old female patient with a history of type 1 diabetes under treatment presented to the emergency department with non-specific abdominal pain evolving for 5 days before admission. The blood test revealed a lipase level of 1500 without disturbance of liver or kidney function, suggesting a diagnosis of acute pancreatitis. An abdominal-pelvic computed tomography was then performed for staging of pancreatitis. It revealed acute edematous-interstitial pancreatitis of Balthazar stage C on a short pancreas with absence of a part of its body and tail (Figure 1). It also revealed the presence of a spleen fusion defect and asymmetry in kidney size (Figure 2) and a pre-pancreatic arrangement of the mesenteric trunk and portal trunk (Figure 3). All of these malformations together led us to suspect polysplenia syndrome, with the particularity of being incidentally discovered during pancreatitis staging. Symptomatic treatment was initiated with good clinical evolution.
Figure 1: Abdominal CT scan in portal phase showing a short pancreas increased in size at the level of its head, with absence of its body and tail occupied by infiltration of fat surrounding the pancreas (white triangle) classified as Balthazar grade C with a CT severity score of 2.
Figure 2: Abdominal CT scan in venous phase after injection of iodinated contrast agent: showing a defect in fusion of the spleen numbering 3 (white arrows) associated with asymmetrical kidney size (blue triangles).
Figure 3: Abdominal CT scan in venous phase revealing a pre-pancreatic disposition of the portal venous and mesenteric trunk (white arrow).
Discussion
Polysplenia syndrome refers to the association of two or more spleens with multiple congenital abnormalities in the abdomen and thorax. However, some cases of polysplenia syndrome have been described with a single lobulated spleen or a single normal spleen [1-3]. In all cases, the spleens are always located on the same side of the stomach [3]. This is a rare congenital disease initially described by Helwig in 1929 [1,3]. Since then, few cases have been described in the literature with an incidence of 1/250,000 live births [3]. Although a short pancreas with multiple spleens is associated with most patients with polysplenia, some studies report involvement with a single lobulated spleen or a normal spleen [4,5]. Usually, it is a short pancreas with agenesis of the dorsal part, as in the case found in our patient, where only the pancreatic head with a small part of the body is present. A preduodenal portal vein has been reported as one of the anomalies associated with polysplenia [6]. In our case, in addition to the short pancreas, we have a pre-pancreatic disposition of the mesenteric trunk and a spleen fusion defect, which corroborates with the literature data. There is no characteristic clinical picture of polysplenia syndrome reported in the literature [2-6]. There are also no specific laboratory findings or hypersplenism related to the spleen fusion defect [6]. In asymptomatic patients, this congenital syndrome can be incidentally discovered during an abdominal surgical procedure or radiological examination [4-7]. The most frequently found clinical signs are generally related to associated biliary-pancreatic abnormalities (ulcers from biliary reflux, pancreatitis) [5]. Symptomatic patients present an increased risk of intestinal volvulus, diabetes mellitus, or pancreatitis, as found in the clinical case we report. In terms of management, there was no therapeutic indication for this malformative syndrome. Treatment depends on associated pathologies or complications [3,4]. In our patient, symptomatic treatment for acute pancreatitis with hygienic-dietary measures was instituted with good clinical and biological evolution.
Conclusion
Polysplenia syndrome is a complex syndrome with a wide range of anomalies, the most common of which are multiple spleens with a short pancreas. Cross-sectional imaging is the technique of choice for visualizing these anomalies. It is therefore important for radiologists to become familiar with these anomalies.