What is the role of abdominal radiography in the workup of cystic fibrosis (CF)?

Updated: Oct 22, 2019
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Kenan Haver, MD  more...
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Answer

In about 71% of uncomplicated meconium ileus cases, abdominal radiography reveals a characteristic pattern of unevenly dilated loops of bowel with variable air-fluid levels. Air-fluid levels may be absent because of the viscid nonliquid nature of the inspissated meconium.

Bubbles of gas may become evident as air mixes with the tenacious meconium. While this soap bubble appearance (or Neuhauser sign) depends on the viscosity of the meconium and is not a constant feature, this radiographic feature is highly suggestive of meconium ileus. Although none of these features alone is diagnostic for meconium ileus, they strongly suggest the diagnosis when combined with a family history of CF.

Radiologic findings in complicated meconium ileus vary based on the associated complication. Speckled calcification visible on abdominal plain radiography strongly suggests intrauterine intestinal perforation and meconium peritonitis. Visible obstruction and a large dense mass with a rim of calcification suggest a pseudocyst. In 1970, however, Leonidas et al reported no radiologic findings that suggest a complication in a third of patients with complicated meconium ileus. [36]

In utero perforation can lead to meconium peritonitis or meconium pseudocyst formation; only postoperative evaluation may differentiate between CF-related and non–CF-related meconium peritonitis or meconium pseudocyst formation.


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