Meconium ileus in Cystic Fibrosis

Abstract

Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology of MI and its clinical presentation. It focuses on the medical and surgical management emphasizing the importance of nutrition and a multidisciplinary approach to improve both short-term and long-term outcomes for CF patients with MI.

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“…Their presence speaks against (but does not rule out) cystic fibrosis, which is present in 15 to 40% of children with meconium peritonitis in Caucasians. 6 Resection of the pseudocyst as well as the segment of small bowel followed by an enteral anastomosis is the treatment of choice. 3,4 Conclusion Meconium pseudocysts are easy to recognize and an important radiologic spot diagnosis that every pediatrician and pediatric surgeon should be aware of.…”

Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

“…51,52 Because the finding often subsequently resolves, antenatal ultrasounds should be repeated at least every 6 weeks, with referral to perinatologist recommended for coordination of multidisciplinary care should findings persist. 43,52,53 Other sonographic findings include peritoneal calcifications, dilated bowel proximal to the obstruction, and polyhydramnios. 51,53,54 Clinical presentation is often within 48 hours of life.…”

“…43,52,53 Other sonographic findings include peritoneal calcifications, dilated bowel proximal to the obstruction, and polyhydramnios. 51,53,54 Clinical presentation is often within 48 hours of life. In simple, or uncomplicated MI, luminal obstruction by inspissated meconium concretions anywhere from the distal ileum to proximal colon leads to proximal bowel distension, while the distal "microcolon" is small in caliber.…”

“…A partial septic work-up followed by antibiotic therapy should be considered; suggested investigations include electrolytes, complete blood count, lactate, and abdominal X-rays. 53,56 Nonspecific X-ray findings may include dilated bowel loops, air-fluid levels, and an absence of air in the rectum. Abdominal calcifications may be present if a prior perforation is contained or has closed, and a "soap-bubble" appearance may be noted in the right lower quadrant due to mixing of meconium and swallowed air in the distal ileum, also referred to as Neuhauser's sign.…”

“…43 Warm saline enemas ($10 mL/kg) with or without 4% N-acetylcysteine, or repeat hyperosmolar enemas can be considered every 12 to 24 hours during this time to aid in evacuation of residual meconium. 43,53 Contemporary studies suggest that hyperosmolar enemas are successful at disimpaction of simple MI approximately 35 to 55% of the time. 55,56,59,60 Potential complications from nonoperative management must be considered in advance.…”