Lance Dorsey (University of Missouri/Columbia/USA), Adam Patterson (University of Missouri/Columbia/USA)  

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15 Years  

Female  

Syndromes  

congenital  

15 year old female presents to the ER with nausea and mid-epigastric pain with some radiation to the back which began this morning. Patient also reports headache. No other complaints and complete ROS is otherwise negative. There is an uncertain reported past medical history of "colonic malrotation." Physical exam demonstrates some mild generalized tenderness in the mid upper abdomen, but otherwise unremarkable. Labs were sent and were within normal limits, including wbc count and amylase/lipase. An abdominal CT was then performed with oral and IV contrast.  

Polysplenia syndrome is a rare congenital anomaly which which may be sporadic or familial. Polysplenia syndrome is often associated with multiple cardiopulmonary and abdominal anomalies. This disorder is commonly seen/categoried in association with the spectrum of heterotaxy or cardiospenic syndromes.

The most common cardiac malformations seen with polysplenia are acyanotic L-R shunt lesions including ASD, PAPVC, and AV canal defects. The cardiac defects associated with asplenia are usually more complex with greater morbidity. Abscence of the suprarenal/intrahepatic IVC is also typical with azygous or hemiazygous continuation. Common abdominal anomalies include intestinal malrotation, abdominal heterotaxy, central location of the liver, pre-duodenal portal vein, short pancreas and agenesis of the gallbladder.  

<- view CT 1

CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



<- view CT 2

CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



<- view CT 3

CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





<- view CT 4

CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




<- view CT 5

CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



<- view CT 6

CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.

 

Laboratory diagnostics  

N/A  

Polysplenia syndrome is more common in females compared with males. Patients without severe cardiac defects often don't present until adolescence or early adulthood and sometimes are only incidentally discovered. These individuals have a better prognosis. However, polysplenia patients that do have significant cardiopulmonary anomalies can present early and often do not survive. Typically patients with asplenia present as neonates/infants due to very severe cardiac malformations. They have a poor prognosis with very high mortality rate in the first few years of life.  

This was an incidental finding discovered on the abdominal CT scan and no findings to explain the patient's acute symptoms were found. The patient's pain subsided and she was discharged from the emergency room in good condition. Follow-up evaluation for possible cardiac anomalies was recommended, however the patient has not returned to our institution for any further workup/evaluation.  

N/A  

1. Medline:
Joseph C Turbyville, MD
Pediatric Asplenia
Emedicine.com

2. Medline:
Michael P. Federle, MD, FACR
Asplenia and Polysplenia
statdx.com  

polysplenia, asplenia, malrotation, azygous continuation, heterotaxy, child, childhood, pediatric radiology  

Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
URL: www.PedRad.info/?search=20110407230957