Ultrasound studies of the intestinal wall in patients with cystic fibrosis.

BACKGROUND

In 1994, first published reports described cystic fibrosis patients who experienced a then unknown complication-ileocecal and colonic stenoses with submucosal proliferation requiring surgical intervention. To investigate a suspected correlation between increased intestinal wall diameter and high doses of pancreatic enzymes, we carried out a prospective study in our CF-outpatient clinic.

METHODS

By ultrasound analysis we measured the intestinal wall diameter in 201 patients. One hundred ninety-three patients treated with pancreatic enzymes had pancreatic insufficiency. Eight patients showed normal pancreatic function, seven of them had never been treated with pancreatic enzymes. The control group included 12 healthy children. Measuring points were the distal ileum, cecum, ascending, and descending colon. Measurements were made by the longitudinal and cross sectional cut. The following aspects of the patients' history were recorded (a) current type of pancreatic enzyme medication; (b) total dosage per day (with reference to lipase units); (c) duration of therapy with standard-strength pancreatic enzyme (SSPE) preparations (< or = 10,000 lipase units per capsule) and HSPE preparations (> or = 20,000 lipase units per capsule); (d) gastrointestinal complication (distal intestinal obstruction syndrome, meconium ileus, abdominal surgery, intussusception), diabetes mellitus, and hepatobiliary complications.

RESULTS

The intestinal wall diameter in patients receiving HSPE therapy was greater (with prominent submucosal layer) than that in patients receiving SSPE therapy or in patients with pancreatic sufficiency. Healthy subjects had the smallest intestinal wall diameter. There was no correlation between patient history and increased intestinal wall thickness.

CONCLUSIONS

Ultrasound detects characteristic ileocecal wall lesions in the majority of cystic fibrosis patients on pancreatic enzymes. These lesions may lead to significantly increased ileocecal wall thickness, which is correlated but not restricted to HSPE.