title: A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon.
abstract: OBJECTIVE
  Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF), occurring in up to 20% of patients with CF. Our aim was to review and integrate current knowledge about the diagnosis and management of fetuses and neonates with MI that may aid the pediatric surgeon in caring for these patients.
  
  
  METHODS
  We identified areas of interest including pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis. We performed a Medline search using the search term meconium ileus for English language articles published in the last 20 years. We reviewed reference lists to identify other articles of historical significance.
  
  
  RESULTS
  Meconium ileus is primarily associated with CF transmembrane (conductance) regulator mutations F508del, G542X, W1282X, R553X, and G551D, and modifier genes have been found to explain approximately 17% of the phenotypic variability. Mouse, pig, and ferret models for CF demonstrate neonatal bowel obstruction mimicking MI. Sonographic findings of hyperechoic masses and dilated bowel in a high-risk fetus are suggestive of MI. Less than 7% of low-risk fetuses with hyperechoic bowel will have MI. Contemporary series of noninvasive management with Gastrografin enema report success rates of 36% to 39%, significantly lower than historical values. The optimal surgical technique remains controversial, although primary anastomosis results in surgical complication rates between 21% and 31%, higher than those noted with delayed anastomosis. Pulmonary function for patients with CF and MI at 15 and 25 years old is similar to those without MI, although height and weight percentiles may be lower.
  
  
  CONCLUSIONS
  This review for pediatric surgeons presents an examination of the literature and synthesizes current information about the pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis of the patient with CF and MI.
authors.affiliation: SUNY at Buffalo, Buffalo, NY 14222, USA.
authors.forename: Brent E
authors.initials: BE
authors.lastname: Carlyle
authors.forename: Drucy S
authors.initials: DS
authors.lastname: Borowitz
authors.forename: Philip L
authors.initials: PL
authors.lastname: Glick
mesh_terms: D000649:Amniocentesis; D000714:Anastomosis, Surgical; D003106:Colon / Q000601:surgery; D003108:Colonic Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D003550:Cystic Fibrosis / Q000150:complications*; D004733:Enema; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D045823:Ileus / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D007231:Infant, Newborn; D008470:Meconium; D011247:Pregnancy; D011379:Prognosis; D016216:Ultrasonography, Prenatal