Which of the followings statements regarding an infant with meconium ileus are true?

a. The probability is 100% that he will have cystic fibrosis

b. Nonoperative therapy resolves this problem in approximately two-thirds of patients

c. The average life expectancy is approximately 26 to 28 years for this infant at present

d. The finding illustrated below on plain film is an absolute operative indication (Figure 103-23A)

Meconium ileus refers to the characteristic obstruction of the small intestine in neonates with cystic fibrosis (CF). Some 10% to 20% of infants with CF present initially with meconium ileus. All infants with meconium ileus have cystic fibrosis. CF is characterized by a transport defect of epithelium that results in impermeability of the chloride ion and therefore water. Inspissated secretions in the pancreas and gut lead to obturator obstruction of the terminal ileum from meconium in the neonate. Approximately two-thirds of these infants have simple meconium ileus, the remainder have complications such as proximal volvulus, perforation or atresia. These latter problems may be associated with the development of a meconium cyst. In this instance, speckled calcifications on plain radiograph or ultrasound are diagnostic. The film above is that of a meconium pseudocyst consistent with intraperitoneal spillage of meconium from intestinal perforation. This finding requires surgical exploration.

Sixty to 70% of infants with simple meconium ileus can be treated by enema installation of one several irrigation solutions into the obstructed terminal ileum. Saline, hyperosmolar contrast agents, dilute N-acetylcysteine and a variety of other solutions have been used successfully. Following resolution of the obstruction, most institutions now report survival rates as high as 70% to 100%.

The average life expectancy for CF patients is now well into the third decade of life. It is primarily determined by the course of the pulmonary disease rather than GI problems. A number of important medical advances, including the realistic prospect of gene therapy are foreseeable for these infants.