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Background: Meconium intestinal obstructions in newborns can be caused by various pathologies such as meconium ileus, meconium obstruction of prematurity, meconium disease, and meconium plug syndrome. These are often mistaken as the same clinical entities; however, meconium ileus stands out through its association with the genetic disorder known as cystic fibrosis. In case of delayed diagnosis, the complete obstruction can progress to a complex form of meconium ileus, with numerous complications. The primary focus of the search was to emphasize on the necessity of a prompt diagnosis using diagnostic tools, and to react accordingly with an adequate therapeutic approach. Methods: PubMed database were searched for relevant articles. Results: Meconium ileus is a multifactorial pathology combining genetical, physiological and clinical elements. This includes a mutation of the cystic fibrosis transmembrane receptor gene, immaturity of a synchronized neuromuscular coordination to reach functional peristalsis, and the influence of maternal and infant-related risk factors. Meconium ileus initially presents with abdominal distention and emesis but can however rapidly progress to a complex form. This is best prevented integrating the following diagnostic tools: 1) clinical evaluation, 2) abdominal radiographs and/or 3) diatrizoate (Gastrografin®) enema. Following diagnosis, different therapeutical approaches, such as saline and N-acetylcysteine irrigation, glycerin suppositories, Gastrografin® enema and surgery, can be established depending on the clinical condition. Conclusion: By reaching a prompt diagnosis of meconium ileus, therapeutic measures can be taken which helps infants reach a more stable hemodynamic condition quickly with less invasive methods. Furthermore, immediate diagnose also contributes to prevent infant morbidity and mortality.