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The steeple sign does not appear in half of cases.
The steeple sign is suggestive of the diagnosis, but is absent in half of cases.
The presence of the steeple sign supports a diagnosis of croup, usually caused by paramyxoviruses.
In radiology, the steeple sign is a radiologic sign found on a frontal neck radiograph where subglottic tracheal narrowing produces the shape of a church steeple within the trachea itself.
An X-ray of the neck is not routine, but if it is done, it can show a narrowing of the trachea, called the steeple sign, because the narrow shape looks like a church steeple.