A 62 y.o. woman presents with chest pain and weakness

As part of a stroke evaluation she had a head CT

What do you notice on the head CT?

What do you notice on the head CT?

Our patient had an empty sella turcica.  She had thickening of the skull and was found to have a pituitary tumor with elevated insulin like growth factor.   Clinically,  she had acromegaly and was scheduled for surgery. 

The empty sella is often an incidental finding on imaging and pituitary function is normal.  In the case of our patient the “empty sella” actually filled with a pituitary tumor. Sella turcica is Latin for “Turkish seat” and is a depression in the sphenoid bone.

empty sella with skull thickening

empty sella with skull thickening

The empty sella may also develop from dural herniation causing a pituitary infarct . This can occur because of a brain tumor, trauma,  or idiopathic intracranial hypertension. Sheehan’s syndrome,  which is postpartum pituitary necrosis  because of blood loss and shock during delivery,  can also produce an empty sella.

trauma or increased ICP can cause dural herniation

trauma or increased ICP can cause dural herniation

 A third cause of the empty sella is genetic. Developmental hypofunction of the pituitary can occur with Kallmans syndrome which is a chromosomal abnormality resulting in colorblindness, deafness, micropenis, anosmia  and renal abnormalities.

Acromegaly results from hypersecretion of growth hormone which stimulates hapatic secretion of insulin-like growth factore IGF-1.  Growth hormone is not used to make the diagnosis because it varies from hour to hour according to food intacke, exercise and sleep.    In contrast IGF-1 does not vary and is a better indication of the diagnosis of acromegaly. It is usually diagnosed in individuals 30-50 years of age.  The presentation can be varied with  DM, carpal tunnel,  chf, visual changes and headache. The diagnosis does not depend on classic clinical features like increased height, large hands  or mandibular prognathism which were more common in the era before imaging.

A Russiian wrestler with acromegaly.

A Russiian wrestler with acromegaly.

Perhaps the most famous local person with acromegaly was Robert Wadlow. He lived in Alton Illinois and was 8 feet 11  inches when he died of sepsis at age 22.

sella wadlow.JPG

Bengtsson B Eden S, Ernest I, et al. Epidemiology and long-term survival in acromegaly. A study of 166 cases diagnosed between 1955 and 1984. Acta med Scand 1988; 223-327.

Ribeiro-Oliveira A, Barkan A. The changing face of acromegaly—advances in diagnosis and treatment.  Nat Rev Endocrinol 2012;8:605.

Melmed S. Medical progress: Acromegaly; state of the art. Expert Opin Med Diagn 2013;7:443.