Polysplenia Syndrome

Author(s)
Eranga Perera, Shweta Bhatt,MD, Vikram S Dogra,MD.
Research assistant
University of Rochester.
Images
  • Figure 1 polysplenia.jpg
  • Figure 2 polysplenia.jpg
  • Figure 3A polysplenia.jpg
  • Figure 3B polysplenia.jpg
  • Figure 4 polysplenia.jpg

Figure Legends : 

Figure 1: Coronal reformatted image of chest shows bilateral hyparterial brochi (arrow) and left side aortic arch with Right aberrant subclavian artery (arrow head).

Figure 2: Axial section of chest shows the origin of right aberrant subclavian artery (arrow head).

Figure 3A and 3B: Axial (figure 3A) and coronal reformatted image (figure 3B) demonstrate the continuation of the azygos vein (arrow) and absence of IVC.

Figure 4: Axial section of a CT scan at the level of liver and stomach shows the normal spleen replaced by multiple spleens of varying sizes (arrows).

Case Description

Clinical History: 

A 19 year old girl presents with abdominal pain.

Diagnosis: 

Polysplenia Syndrome with azygous continuation of Inferior Vena Cava.

Discussion: 

Heterotaxy or cardio splenic syndromes refer to spectrum of various truncal abnormalities with frequent cardiac malformations from asplenia to polysplenia [1]. Heterotaxy syndrome with polysplenia or the bilateral left sidedness is characterize by the presence of bilateral bilobed lungs, bilateral pulmonary atria, a centrally located liver, stomach in indeterminate position and multiple spleens [2]. Interruption of inferior vena cava with azygos or hemiazygos continuation is the consistent features of heterotaxy with polysplenia.

Polysplenia syndrome is more common in females and the patients usually present in infancy or adulthood with a variable clinical manifestation [1,2]. Cardiac anomalies are less common and less complex than asplenia. Fewer polysplenic patients present with cyanosis compared to asplenic patients and They(polysplenia syndrome) present more commonly with congestive heart failure from left to right shunt [2].

Most common cardiac anomalies in this group are anomalous pulmonary venous return, atrial septal defect and artrioventricular canal defects [1,2]. There is also bilateral pulmonary/left atria, bilateral superior vena cava (50%). Interruption of hepatic segment of inferior vena cava with continuation of azygos and hemiazygos is seen in 65% to 70% and considered the most consistent finding [1].

The lungs commonly have bilateral morphologic left lung (55% to 68%), absence of middle lobe fissure and increase or normal pulmonary vascularity. They also demonstrate bilateral hyparterial bronchi (pulmonary arteries projecting superior to bronchi) [1,2].

Abdominal heterotaxy is reported in 56% of the time with polysplenia, centrally located liver, absence of gall bladder, malrotation of bowel and preduodenal portal vein [1,3]. The spleen or the spleens lie always on the same side as the stomach, typically along the greater curvature.

Imaging is helpful in patients who present with heterotaxy syndrome to locate atypical positioning of organs. Ultrasound scan can be used to evaluate the aorta, IVC and the spleen [2]. Multidetector CT is capable demonstrating cardiac defects in adults and in pediatric patients [3].

References / Suggested Reading: 

1: Grainger RG, Allison DJ. Diagnostic Radiology. A text book of radiology. 5th edition. Volume 2. Chapter 48.

2: Applegate KE, Goske MJ, Pierce G. et al. Situs revisited: Imaging of Heterotoxy syndrome. RadiGraphics 1999; 19: 837-852.

3: Leschka S, Oechslin E, Musmann L. et al. Pre and Post Operative Evaluation of Congenital Heart Disease in adults and Children with 64-section CT.

Citation:

Polysplenia Syndrome, Eranga Perera, Shweta Bhatt,MD, Vikram S Dogra,MD., Imaging Science Today, 2011, 2599.