Season 1 Case 42

body May 5, 2013

Case 42       History: abdominal pain (no way!  I’ll give you the very dark appearance of the left kidney, that’s windowing artifact).



Did you find the spleen?

The abnormality here is not too hidden. The liver is not only present on the right, but extends across the midline to the left (in fact a majority of the liver is present on the left!).

Thus this is a situs abnormality. Remember that it is a spectrum disorder, with situs solitus being normal and situs inversus being the mirror image of normal. Often a patient lies somewhere in the middle with situs ambiguous (aka heterotaxy), as here. From the reference:

The complexity of this syndrome is reflected in the various terms used to subclassify it, including asplenia syndrome, double right-sidedness, right isomerism, or Ivemark syndrome and polysplenia syndrome, double left-sidedness, or left isomerism. The incidence of congenital heart disease in patients with heterotaxy is very high, ranging from 50% to nearly 100% (1,3,6). In asplenia (ie, right isomerism or bilateral right-sidedness), both lungs have three lobes and eparterial bronchi (Fig 1). The main bronchus is located superior to the ipsilateral main pulmonary artery on each side. Inpolysplenia (ie, left isomerism or bilateral left-sidedness), both lungs have two lobes and hyparterial bronchi (Fig 2). In this situation, the reverse is seen: The main bronchus passes inferior to the ipsilateral main pulmonary artery on each side

I think calling this situs ambiguous would be correct (most general) but also would take asplenia syndrome (ie double right-sidedness).



Professor of the Darkroom Arts

Great! You've successfully subscribed.
Great! Next, complete checkout for full access.
Welcome back! You've successfully signed in.
Success! Your account is fully activated, you now have access to all content.