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Saturday, December 1st 2007

Situs Inversus

What Isn’t Right In This CXR?

Medicine is not House, MD. Unusual diseases are…unusual. So, I’m happy to report that I just saw my first real ‘Zebra’ the other day. I ran into a patient with situs inversus totalis. Actually with Kartagener’s Syndrome.

Situs inversus refers to a mirroring of the position of the organs in the body. In Situs inversus totalis with dexocardia literally all thoracic and abdominal internal organs are on their opposite sides. You find the heart of the right, the liver on the left and so forth.

Granted, the patient wasn’t in the clinic for this condition but it was still pretty cool, especially since I walked in without a glance at the PMH and just discovered the condition on my own during the chest exam.

Situs inversus occurs in less than 1 in 10,000 people. Kartagener’s Syndrome is situs inversus with dexocardia + primary ciliary dyskinesia, which probably represents only somewhere between 20-25% of patients with situs inversus.

The underlying cause of Kartagener’s syndrome is a ciliary defect. [1] Cilia are motile processes that are present on the surfaces of many different types of cells. Cilia measure 0.2 to 0.5 [micrometer] X 2 to10 [micrometer] and are powered by an arrangement of nine microtubules surrounding two central tubules. Ciliated cells are found in the airways, the paranasal sinuses, the eustachian tubes, the ventricles of the brain, the oviducts and the vasa efferentia of the testes. Defective cilia are unable to adequately clear bacteria from the sinuses and the bronchial tree, thus predisposing patients with this condition to recurrent bacterial infections. [2] Sperm cells are powered by flagella that also contain microtubules, [2] and the immotile sperm in patients with Kartagener’s syndrome result in infertility.

The syndome is named after Austian/Polish physiologist Manes Karagener.

In anycase, there wasn’t a whole lot more to the patient encounter but it was still cool to run into.

[eMedicine: Situs Inversus]
[Wikipedia: Primary Ciliary Dyskinesia]
[Radiopedia: Kartagener Syndrome]