An İncidental Findings of Polysplenia Syndrome in an Adult Patient with Multiple Anomalies

C l i n M e d International Library Citation: Durmaz MS, Cengiz A, Arslan S, Erdogan H, Tolu I, et al. (2016) An İncidental Findings of Polysplenia Syndrome in an Adult Patient with Multiple Anomalies. Clin Med Rev Case Rep 3:148 Received: October 14, 2016: Accepted: December 13, 2016: Published: December 16, 2016 Copyright: © 2016 Durmaz MS, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Volume 3 | Issue 12


Introduction
Polysplenia syndrome is a rare congenital subtype of heterotaxy syndrome associated with various visceral and vasculer anomalies.Polysplenia syndrome is characterized by presence of two or more spleens and anomalies of other asymetric organs.It is reported incidence of 1 per 250.000 and more common in females.Here, we report the case of a 53-year-old woman who presented with polysplenia syndrome.The patient had multiple accessory spleens in right sided, dextrocardia, interrupted inferior vena cava with azygos continuation, left-sided liver and congenital short pancreas.Our patient was asymptomatic until now and were diagnosed incidentally.

Case Report
53-year-old female patient was admitted to the our internal medicine clinic with mildly abdominal pain and debility.There was no sigficant evidence in the patient's medical history.Physical examination showed mild abdominal sensivity and dextrocardia, other findings were normal.Ultrasound examinations were performed simultaneously with physical examination.Ultrasound demonstrate the presence of liver in the upper left quadrant of abdomen and multiple nodular spleen structures in the right upper quadrant of abdomen.Abdominal and chest computed tomography (CT) examination were performed to confirm the diagnosis of polysplenia and detect the accompanied additional findings.Initial CT scan shows partial situs inversus and a right-sided heart which is concomitant to right aortic arch.Liver was the in upper left quadrant of abdomen and multiple nodular spleen structures in the right upper quadrant (Figure 1).There was enlarged azygos vein on the left side of descending aorta and vena cava superior was left side of vertebral column (Figure 2).The corpus and tail of pancreas could not be monitored, there was only head of the pancreas in left quadrant (congenital short pancreas) (Figure 3).Vena cava inferior could not be monitored also.Both renal veins drain into azygos vein.Hepatic vein emptying into right atrium (Figure 3).Accordingly, the right lung consists of two lobes and left lung consisted of three lobes.Patient is diagnosed with polysplenia syndrome.More than 40 percent of reported cases have cardiac anomalies and majority of such children can not survive very long in the first five years of life.The most common cardiac anomalies are atrial septal defect (%78), azygous continuation of the inferior vena cava (65%), ventricular septal defect (63%).Death can ocur at an early age especially in case of concomitant cardiovascular abnormalities [4].
In conclusion, polysplenia syndrome is a infrequently seen disease, which may be accompanied by multiple systemic abnormalities.It is important to be familiar with this syndrome especially in order not to overlook accompanying abnormalities.Computerized tomography examinations are very helpful in the evalation of polysplenia syndrome patients.

Discussion
Polysplenia syndrome is a rare congenital disorder, which is characterized by multiple spleens and accompanying cardiopulmonary and abdominal anomalies that is a type of situs ambiguous believed to be left isomerism in most cases.Patients usually do not have a complaint and this congenital anomaly is noticed incidentally during abdominal surgery or radiological examination.Symptomatic patients presented with atrioventricular septal defects and may have anomalous inferior vena cava or other gastrointestinal structural abnormalities including partial or complete agenesis of the dorsal pancreas [1].These findings may have clinical relevance with a possible increased risk for intestinal volvulus, diabetes mellitus or pancreatitis.The certain underlying etiology of polysplenia is unknown.Embryonic, genetic and teratogenic components have all been implicated as causative factors in polysplenia.
The splenic structures is generally divided into fairly same sized masses, varying in number from 2 to 6 and ranging from 1 to 6 cm in diameter, Total quantity is equal the mass of a normal spleen.The location of the spleens is in either the left or right upper quadrant.The most frequent positional abnormalities of liver and gallbladder can be found in the midline or mirrored.Usually the pancreas is forming part of the head and found in the left upper quadrant.Gastrointestinal malrotation and renal agenesis or hypoplasia may be seen [2].The pulmonary manifestations include bilateral bilobed lungs [3].

Figure 1 :
Figure 1: The coronal and axial enhanced CT show, heart is in the right hemitorax (dextrocardia), liver is in the left upper quadrant of abdomen (arrow), there are multiple nodular spleen structures in the right upper quadrant (thin arrow).There is enlarged azygos vein on the left side of descending aorta (arrow head) and azygos vein is on the left side of aorta in the abdominal imaging (curved arrow).

Figure 2 :
Figure 2: Axial and sagittal enhenced CT show right aortic arch (arrow).Vena cava süperior is in the left side and enlarged azygos vein drain into the vena cava süperior (arrow head).

Figure 3 :
Figure 3: Head of pancreas is in the left upper quadrant (arrow).Renal veins drain into the azygos vein (arrow heads).Vena cava inferior isn't detected and hepatic veins directly drain into the right atrium (thick arrow).