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402 Meconium Ileus
Complicated Form spread being the Bishop-Koop type anastomosis (Figure 67.3). The
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About half of meconium ileus patients have a complicated form, most distended part of the ileum is resected. An end–to-side anastomo-
associated with volvulus, perforation, or atresia. Massive distention, sis is constructed about 3 cm distal to the resection margin. The open
tenderness, or erythema indicates the presence of complications. There end is brought out as a stoma and sutured to the skin. In critical cases,
are no bowel sounds; vomiting is usually bilious. The child is in a a double-barrel stoma may be the best option.
critical condition.
Investigations
Plain abdominal radiographs show a distended intestine. Air-fluid
levels may or may not be present. A “soap bubble” appearance in the
right lower quadrant may be the result of air mixed with the meconium.
The initial diagnostic test is a contrast enema. In the case of a
meconium ileus, it shows a microcolon. Meconium pellets in the
distal ileum can also be determined. This diagnostic investigation also
enables one to exclude colonic atresia and rotation anomalies. The main
differential diagnosis is Hirschsprung´s disease. This will need to be
excluded by suction rectal biopsies.
The same diagnostic methods are used in premature babies, although
due to the prematurity, recognition of the problem may be delayed.
Treatment
Nonoperative Treatment Figure 67.3: Bishop-Koop anastomosis for irrigation.
Uncomplicated meconium ileus may be successfully treated nonopera-
tively. All patients require standard supportive care:
• oral gastric tube decompression; Postoperative Complications
General supportive care is provided as after any major laparotomy.
• intravenous fluids to replace deficits and counteract ongoing losses; The oral gastric tube is left in place until bowel function returns. Use
and of N-acetylcysteine via the oral tube, or a stoma, or by enema may
• meticulous attention to the acid-base balance. further aid passage. Oral feeding is started with pancreatic enzyme
supplementation.
In uncomplicated cases, a gastrografin enema (dilution 3:1) is the CF is confirmed or ruled out by determining the sweat chloride
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treatment of choice. It can be accompanied by use of N-acetylcysteine/ level. Close attention has to be given to pulmonary care in CF children.
saline (1:5) in several enemas and in addition to the oral tube. The Weaning is often not a problem. Extended physiotherapy and
effect of this enema is to draw large volumes of fluid into the lumen. specific attention to pulmonary infections and general growth of the
Therefore, the child must be well-rehydrated prior to this procedure. child are decisive for the quality of the child’s further life.
The child’s pulse rate and urine output have to be carefully monitored. In some cases, the Bishop-Koop anastomosis closes by itself.
This procedure is successful in more than 50% of affected children. The In other cases, special attention has to be paid to clearance of the
patient should evacuate spontaneously over the next 6–8 hours. If the obstructed segment. After this, closure of the enterostomy can be
patient fails to evacuate or if a complicated meconium ileus is present, carried out. In CF, distal ileum obstruction tends to recur, so special
a surgical procedure must be carried out. 7
care has to be taken to ensure normal bowel movements.
Operative Treatment
Operative treatment is indicated when the nonoperative treatment fails Prognosis
or is associated with complications such as perforation or in the compli- The prognosis for children suffering from CF has improved in the
cated type of meconium ileus. In all cases, a supraumbilical transverse developed countries due to neonatal care, general nutrition, treatment
incision is used. of pulmonary infections, and specific antibiotics.
Three procedures can be used: Neonates with very low birth weight require a specific type of
treatment. Their survival depends on intensive neonatal care with
1. enterostomy and decompression;
ventilation, broad spectrum antibiotics, and a specific enteral nutrition.
2. resection and stoma formation; or
Ethical Issues
3. resection and anastomosis.
In developed countries, CF is a genetic-based diagnosis. Specific care
Enterostomy has to be given to children with CF. This has enabled the life-span of
Enterostomy is performed by opening the bowel on the antimesen- CF sufferers to be extended to 30 to 40 years, whereas historically
teric border proximally where the dilated bowel tapers down. A size 10 in Europe these children died at about age 20. One of the main pro-
catheter is pulled upwards, and the sticky meconium is washed out by cedures that has made this improvement possible is the gastrografin
using gastrografin or a 1:5 solution of acetylcysteine/saline. Patience is enema developed by Helen Noblett. In combination with this, use of
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required during this procedure; it takes a considerable amount of time N–acetylcysteine often makes it possible to avoid having to perform a
because one has to work carefully. Further, the distal plugs have to be laparotomy. If the combination fails, surgery and enterostomy have to
washed out. When the small bowel is empty, the enterostomy can be be carried out. 2
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closed in the usual way.
Evidence-Based Research
Resection The condition is uncommon, and relevant studies of surgical treatment
Resection and formation of a stoma is the most common form of with a significant number of patients are not available.
management. Several forms of stoma are possible, with the most wide-