Necrotizing enterocolitis (NEC) is the most common gastrointestinal condition in premature neonates. It is characterized by inflammation, ischemia, and permeability of the neonatal bowel wall to bacteria. It is potentially life-threatening with significant associated morbidity 1.
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Epidemiology
Necrotizing enterocolitis usually develops within the first two weeks of life, but may occur at several weeks of age 4. The incidence is inversely proportional to gestational age, with 90% occurring in premature infants. Its overall incidence is ~1 in 1000 births but is as high as 20% in low birth weight infants (<1500 grams) 5.
Risk factors
prematurity (50-80%)
perinatal asphyxia
decreased umbilical flow in utero
intrauterine growth restriction
Clinical presentation
The presentation is often non-specific and includes:
feed intolerance
bile-stained vomit/aspirates
abdominal distension
blood-stained stools +/- explosive diarrhea
respiratory distress with acidosis
Pathology
Necrotizing enterocolitis is usually idiopathic and multi-factorial. A combination of ischemic and infective etiology with added contributive factors such as immature immunity have been proposed 1. Although a partially infective etiology has been hypothesized, no causative organism has been isolated. Translocation of intestinal flora through immature mucosa has been postulated 5.
Inflammation starts from the mucosal surface and progresses to hemorrhagic and coagulative necrosis. There is ensuing loss of mucosal integrity, transmural necrosis, and perforation.
Although necrotizing enterocolitis can affect any part of the large or small bowel, the most common location is the terminal ileum. The right colon (including cecum) is occasionally involved. Involvement of the stomach is less common.
Radiographic features
Plain radiograph
Supine abdominal x-rays are the mainstay of diagnosis. If perforation is suspected clinically, or there is a concern on supine films, an additional cross-table lateral or left-lateral decubitus view provides increased sensitivity 5. Findings include:
dilated bowel loops (often asymmetrical in distribution)
loss of the normal polygonal gas shape
bowel wall edema with thumbprinting
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pneumoperitoneum indicates severe disease
only 50-75% of patients with proven perforation will have visible free gas 5
air on both sides of the bowel (Rigler sign)
air outlining the falciform ligament (football sign)
For follow-up, supine and lateral projections have been recommended for the first 48 hours as this is when most perforation occurs. After 48 hours, vertical beam supine projections may be sufficient if there no concern for perforation 1.
Ultrasound
bowel wall thickening
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abnormal bowel wall vascularity
hypervascular (viable but engorged in early stage)
hypovascular (infarcted in a later stage)
intramural gas manifesting as hyperechoic foci within the bowel wall 9
free fluid, especially with echogenic debris, suggests perforation 5
pneumoperitoneum (stacked echogenic lines outside the bowel lumen) 9
portal venous gas (shadowing hyperechoic foci within the portal venous system) 9
Treatment and prognosis
Necrotizing enterocolitis can be managed both medically and surgically and appropriate patient selection is essential in optimizing outcome. A clinical staging system has been developed (see necrotizing enterocolitis staging), with stage I and II receiving medical therapy and stage III undergoing surgery 8.
Medical management consists of supportive measures and cessation of oral feeding, along with broad-spectrum antibiotics and gastric aspiration 7.
Surgery is usually reserved for patients with evidence of perforation and entails resection of clearly necrotic bowel and the creation of a proximal enterostomy 5,6. Other relative indications for surgery include portal venous gas, a fixed dilated loop on serial x-rays and abdominal wall erythema 7. Re-anastomosis is usually delayed until the infant has completely recovered.
Despite therapy, mortality remains significant, ranging between 9 and 28% 5. Furthermore ~20% of surviving patients will go on to develop stricture. These are more common in the large bowel (80%) and may result in bowel obstruction weeks to months later 5,8.
Differential diagnosis
In the correct clinical scenario, the presence of gas within bowel wall has a little differential. Conditions worth keeping in mind include 8: