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Necrotizing enterocolitis (NEC) continues to be associated with extreme prematurity, resulting in increased morbidity and mortality. Various studies have reported an incidence of NEC of 14% in infants with a birth weight of 501–750 g and an odds ratio for death of 14 for nonsurgical NEC and 25 for surgical NEC. There remains some controversy regarding the diagnosis of perforated NEC vs spontaneous intestinal perforation, and an ongoing multicenter, randomized, controlled trial is attempting to better define the 2 conditions. Regardless of the etiology, severe inflammation and subsequent necrosis, ischemia, perforation, and peritonitis may result in not only death but also severe neurodevelopmental impairment and intestinal failure (IF).

The 2 common surgical interventions for intestinal perforation are exploratory laparotomy and peritoneal drainage. Several case series and observational studies examined the utility and outcome of both operations. To date, only 2 randomized studies compared the 2 operations, with conflicting results concerning mortality. There is a paucity of data regarding the long-term outcomes of either operation.

This chapter focuses on the dilemma of surgical intervention for intestinal perforation/NEC. This discussion includes preoperative, operative, and postoperative management. In addition, the chapter reviews novel therapies designed to prevent the complications associated with surgical NEC.


It is essential first to determine if a patient actually has perforated NEC. Whenever possible, it is strongly recommended to obtain a pediatric surgical consultation when concerned about perforation. Several clinical scenarios of perforation can present to the caregivers. The first is the presence of free abdominal gas within the peritoneal cavity. A patient may demonstrate a large pneumoperitoneum that is easily appreciated on supine abdominal imaging. Radiographic findings include a large gas bubble or gas surrounding the liver, as well as the so-called football sign by which free abdominal gas highlights the falciform ligament. It can be more difficult to detect when there is less free abdominal gas. In these cases, it is imperative to obtain both supine and left lateral decubitus radiographs. The left lateral decubitus image is preferred to a “cross-table” supine study as the former allows free abdominal gas to migrate between the body wall and the liver and not be confused with a gas-filled loop of intestine. In the absence of free abdominal gas, the determination of intestinal perforation is more challenging.

To date, there are no studies designed to confirm perforation by either radiographic or biomarker methods. There are case reports of clinicians utilizing abdominal paracentesis to detect perforation. This technique is associated with significant complications and should be reserved for patients who require therapeutic paracentesis for severe abdominal distension that is having an impact on ventilation. Often, patients with a “gasless” abdomen or with presumed NEC totalis (extensive NEC involving the entire intestinal tract) may require surgical intervention even with no clinical evidence of perforation.

Once the diagnosis of proven or presumed ...

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