Meconium Ileus–Is a Single Surgical Procedure Adequate?

Abstract

Meconium ileus is one of the gastrointestinal manifestations of cystic fibrosis (CF), and affects 15% of neonates. The condition results from the accumulation of sticky inspissated meconium. Both nonoperative and operative therapies may be effective in relieving obstruction. The treatment of choice for uncomplicated meconium ileus is the use of enteral N-acetylcysteine or Gastrografin enemata. Once such therapy fails, surgery is indicated. A number of operative procedures are in use, including Bishop-Koop enterostomy, T-tube irrigation, resection and primary anastomosis, and enterotomy with irrigation and primary closure. During the period 1991-2003, five newborns required surgical intervention for uncomplicated meconium ileus. None responded to conservative management. All were males, including one set of twins. All underwent laparotomy, enterotomy, appendectomy, irrigation and closure of enterotomy. None required a second surgical procedure. CF was confirmed in all, and in each case, both parents were found to be genetic carriers of a mutational form of CF. A single surgical intervention is preferable in these patients, in view of the high rate of pulmonary involvement in CF patients. Enterotomy, irrigation and primary closure are the treatment of choice for uncomplicated meconium ileus.