Meconium ileus in cystic fibrosis

Recent reviews of meconium ileus (MI) in cystic fibrosis (CF) have documented reduced mortality when compared to earlier studies but suggest significant morbidity is still present, with feeding difficulties and prolonged hospital admission. A chart review was made of 98 infants with MI and CF born in Victoria in the 20-year period 1973–1992. Results were analysed in two consecutive 10-year cohorts. A questionnaire about infant feeding was sent to all current patients from the most recent cohort. MI was the mode of presentation in 21% of all cases of CF. Results of the 1983–1992 cohort (n = 48) showed that 27 of 48 cases (56%) were uncomplicated MI without associated pathology or complications. The remaining 44% had complicated MI. Sixteen of the 27 infants (62%) with uncomplicated MI had a gastrografin enema (GE) performed with a success rate of 50%, with no complications. Surgery was performed in 36 of 48 infants, with the majority (89%) having a stoma fashioned, most commonly an ileostomy (84%), with a mean stoma duration of 17 days (range 5–83). Breast-feeding was established in 25 of 35 cases (71%). The mean duration of hospital stay was 27 days (range 5–90). There was an early mortality of 8%, with a 6-month survival of 90%. Early and late surgical complications were present in 17% of cases. The mean length of hospital stay for cases of uncomplicated MI was 12 days for patients managed non-operatively and 33 days for those treated surgically. Comparison of the two cohorts shows that there has been no further reduction in early mortality (8% in the recent cohort vs 10% in the earlier cohort) and 6-month survival remains unchanged at 90% in both 10-year cohorts. There has been a reduction in early and late surgical morbidity (17% versus 29%). A greater proportion of cases of uncomplicated MI managed non-operatively would result in a reduction in both early and late surgical complications and a shorter hospital stay. The majority of infants with MI do not require long-term parenteral nutrition or pre-digested formula, but can be successfully breast-fed.