A 30 y.o. woman presents with DKA and R flank pain
what do you notice in the RUQ?
Our patient had multiple spleens in the RUQ. The stomach Is also in the RUQ. There is distention of the azygos vein within the chest which corresponds to the patient’s right paratracheal opacity on cxr. This is secondary to interrupted IVC with azygos continuation. There was also intestinal malrotation. The pt had been sent for CT to ro PE. She did have a PE in the RLL with elevation of the R hemidiaphragm.
Our pt had a R paratracheal mass which is a dilated azygous vein from vena caval interruption
Our pt has a condition called heterotaxy, where the organs are not arranged as would be expected in the chest and abdomen. This comes from the Greek (hetero, meaning “different” and taxy, meaning “arrangement”. Often this is associated with congenital heart defects. The true incidence of the syndrome is not known but is thought to occur in 1 in 8,000 births. About 20% are associated with immotile cilia syndrome(including Kartagener syndrome) More specifically, our patient has polysplenia syndrome or left isomerism. This can be associated with multiple splenules without a parent spleen, interrupted IVC, bilateral bilobed lungs , a midline liver and intestinal malrotation.
1 is the dilated azygous seen on cxr; a central line in this pt would cross the midline
In spite of the fact that there are multiple spleens present, splenic function may be impaired. In polysplenia syndrome the clinician should look for Howell-Jolly bodies to confirm the absence of splenic function. Howell-Jolly bodies are nuclear remnants that would have been removed by a functioning spleen.
look for Howell-Jolly bodies if you are concerned about functional asplenia
Our patient had multiple amputations in the past and presented with a PE and severe infection of her remaining foot with DKA. She was treated for sepsis and underwent an amputation of the fifth toe on the L foot.
the stomach is always on the side of the spleens as in this 70 y.o. man
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Peoples W, Moller J, Edwards J. Polysplenia: a review of 146 cases. 1983. Pediatr Cardiol. 4:129-137.
Arnold G, Bixler D, Girod D. Probable autosomal recessive inheritance of polysplenia,situs inversus and cardiac defects in an Amish family. Am J Med Genet. 1983;16:35-42.
Sears M, Udden M. Howell-Jolly bodies: A brief historical review. 2012 The Am Journal of Medical Science Vol 343(5):407-409.