The survey included 85 infants born with meconium ileus (MI) in 13 Italian centers, in the years 2009-2014. They represent 70% (85/121) of the reported cases in Italy during the same time-frame as per CF national register. In 20 infants (24%) the diagnosis of intestinal obstruction was also made possible through fetal ultrasound at the third trimester of pregnancy. Most cases (84%) went through surgery, which involved an intestinal resection and the creation of a transient stoma. In 18% of the infants, further abdominal surgery within the first month of life was necessary due to surgical complications.
Clinical data during the first year of life were collected and analysed. They showed in 37% of the patients a poor clinical evolution, characterized by insufficient nutritional status or by chronic Pseudomonas infection (22 children underweight and 9 with colonization from Pseudomonas). Factors identified as related to negative evolution were prenatal diagnosis through fetal ultrasound (pointing out the severity of the intestinal obstruction), the need for intensive care hospitalization, the use of oxygen therapy (sign of postoperative adverse conditions and rapid pulmonary involvement). Since the diagnosis of cystic fibrosis was not available at an early stage (it occurred at the age of 31 days, on average) and the advice from the CF specialist occurred during the first hospitalization only in a small number of cases, this study indicates the need for an immediate and close cooperation between neonatologists, surgeons and CF specialists.