Background: It is unclear whether neonates with cystic fibrosis (CF) who
present with meconium ileus (MI) have a different long term outcome than those
presenting later in childhood. We compared long term outcome in neonates with
MI, children who had CF without MI, and neonates who had meconium plug syndrome
(MPS) without CF.
Methods: 35 CF patients presented with MI from 1966-92 (grp 1). Also studied
were 35 age and sex-matched CF patients without MI (grp 2), and 12 presenting
with MPS during the same period (grp 3). Pulmonary, GI, and functional status,
and surgical complications were recorded.
Results: Mean followup was 12.6 ± 6, 12.6 ± 6, and 9.3 ± 4
yrs in groups 1, 2, and 3. Patients without CF (grp 3) had better growth, GI,
pulmonary and functional status. Although grp 1 was diagnosed earlier, there was
no difference between grps 1 and 2 with respect to hepato-biliary, nutritional,
functional or respiratory status. MI was associated with a slightly higher risk
of meconium ileus equivalent (20% vs 6%). Late adhesive bowel obstruction or
blind loop syndrome were seen in 39% of children with MI; there were no late
surgical complications in grps 2 or 3. More late surgical complications were
seen after complicated than uncomplicated MI (57% vs 17%), and those treated by
resection or enterostomy had more complications than those undergoing enterotomy
and lavage (33% vs 0%).
Conclusions: Long term outcome is similar in CF patients with and without
MI, except for a slightly higher incidence of meconium ileus equivalent, and a
higher rate of surgical complications which is greatest in those with
complicated MI and those undergoing resection or enterostomy. Non-CF patients
with MPS have an excellent long term prognosis. These data will be useful in
counselling the families of infants who present with neonatal meconium
obstruction.
