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Meconium ileus

The problem

Fifteen percent of children with CF have neonatal intestinal obstruction due to inspissated meconium requiring urgent treatment. In the majority the diagnosis is made with a contrast enema (which shows a microcolon and meconium pellets in the ascending colon and terminal ileum). In 30 or 40% of infants the obstruction can be relieved with a gastrograffin enema (care must be taken to ensure sufficient iv hydration). In the remainder surgery is required, which will involve either simple flushing out of the meconium via an enterostomy, or resection which is usually combined with a Bishop-Koop enterostomy. If an ileostomy is formed, it is usually reversed at around 3 months of age. It may be necessary to flush the distal limb of the ileostomy to ensure it is clear (this is a surgical decision)

Post enema or Post op management

The diagnosis of CF needs to be made. In young babies it is difficult to get enough sweat for a sweat test, so it is important that a blood sample is sent to the Churchill genetics lab for CF mutation screening. IRT is unreliable in children with meconium ileus. Post op or post enema the babies will initially be on iv fluids. If CF has been confirmed, as milk feeds increase pancreatic enzyme supplements need to start. Once babies are on half milk feeds they should be given ½ a Creon micro-scoop (5000iu/scoop) per feed. This is given with fruit puree on a teaspoon. If the child is fed via a nasogastric tube, Pancrex sachets can be used (Creon granules will not fit down an ng tube).

Attention should also be given to the chest. Physiotherapy needs to be started once the diagnosis has been confirmed. If there are any respiratory symptoms, a cough swab should be arranged. We have had babies with Pseudomonas aeruginosa chest infections even at this early stage. Any significant cough should be treated aggressively with iv antibiotics and physiotherapy in the usual way. Vitamin supplements and prophylactic flucloxacillin should be started once the infant is on full feeds. These babies should receive im rather than oral vitamin K.