Necrotizing enterocolitis (NEC), which typically occurs in the second to third week of life in premature,

formula-fed infants, is characterized by variable damage to the intestinal tract, ranging from mucosal
injury to full-thickness necrosis and perforation (see the image below). NEC affects close to 10% of
infants who weigh less than 1500 g, with mortality rates of 50% or more depending on severity, but may
also occur in term and near-term babies.

Signs and symptoms

In premature infants, onset of NEC is typically during the first several weeks after birth, with the age of
onset inversely related to gestational age at birth. In term infants, the reported median age of onset is 1-
3 days, but onset may occur as late as age 1 month.

Obtain radiographic studies if any concern about NEC is present. Pursue laboratory studies, especially if
the abdominal study findings are worrisome or the baby is manifesting any systemic signs. A CBC with
manual differential is usually repeated at least every 6 hours if the patient's clinical status continues to
deteriorate. Relevant findings may include the following:

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WBC Moderate to profound neutropenia (absolute neutrophil count [ANC] < 1500/L) strongly
suggests established sepsis

Hematocrit and hemoglobin Blood loss from hematochezia and/or a developing consumptive
coagulopathy can manifest as an acute decrease in hematocrit; an elevated hemoglobin level and
hematocrit may mark hemoconcentration due to notable accumulation of extravascular fluid

Platelet count Thrombocytopenia may be present

Other laboratory findings

Blood culture is usually negative

Hyponatremia An acute decrease in serum sodium (< 130 mEq/dL) is alarming

Low serum bicarbonate (< 20) may be seen in babies with poor tissue perfusion, sepsis, and bowel
necrosis

Reducing substances may be identified in the stool of formula-fed infants

 A breath hydrogen test may be positive

Arterial blood gas levels may indicate the infant's need for respiratory support and can provide
information on the acid-base status

Abdominal radiography

The mainstay of diagnostic imaging

An AP and a left lateral decubitus view are essential for initial evaluation

Should be performed serially at 6-hour or greater intervals, depending on presentation acuity and
clinical course, to assess disease progression

Characteristic findings on AP views include an abnormal gas pattern, dilated loops, and thickened
bowel walls

A fixed and dilated loop that persists over several examinations is especially worrisome

Scarce or absent intestinal gas is more worrisome than diffuse distention that changes over time

Other radiographic findings include the following:

Pneumatosis intestinalis Pathognomic of NEC

Abdominal free air Ominous; patients usually require emergency surgical intervention

Portal gas A poor prognostic sign

Distended loops of small bowel Common but nonspecific

Intraperitoneal free fluid

Abdominal ultrasonography

Available at bedside

Noninvasive
 Can identify areas of loculation and/or abscess consistent with a walled-off perforation

Excellent for identifying and quantifying ascites

Limited availability at some medical centers

Requires extensive training to discern subtle ultrasonographic appearance of some pathologies

Abdominal air can interfere with assessing intra-abdominal structures

See Workup for more detail.

Management

The initial course of treatment consists of the following:

Stop enteral feedings

Perform nasogastric decompression

Initiate broad-spectrum antibiotics (eg, ampicillin, gentamicin, and clindamycin or metronidazole)

Bell stages IA and IB suspected disease

NPO diet and antibiotics for 3 days

IV fluids, including total parenteral nutrition (TPN)

Bell stages IIA and IIB definite disease

Support for respiratory and cardiovascular failure, including fluid resuscitation

NPO diet and antibiotics for 14 days

 Consider surgical consultation

After stabilization, provide TPN while the infant is NPO

Bell stage IIIA advanced disease

NPO for 14 days

Fluid resuscitation

Inotropic support

Ventilator support

Obtain surgical consultation

Provide TPN during the period of NPO

Surgical intervention

Surgery

The principal indication for operative intervention in NEC is perforated or necrotic intestine, which is
most compellingly predicted by pneumoperitoneum. Other indications include the following:

Erythema in the abdominal wall

Gas in the portal vein

Positive paracentesis

Clinical deterioration

Background
Necrotizing enterocolitis (NEC) is the most common gastrointestinal (GI) medical/surgical emergency
occurring in neonates. An acute inflammatory disease with a multifactorial and controversial etiology,
the condition is characterized by variable damage to the intestinal tract ranging from mucosal injury to
full-thickness necrosis and perforation (see the image below). (See Etiology.)

Normal (top) versus necrotic section of bowel. Pho

Normal (top) versus necrotic section of bowel. Photo courtesy of the Department of Pathology, Cornell
University Medical College.

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Necrotizing enterocolitis represents a significant clinical problem and affects close to 10% of infants who
weigh less than 1500 g, with mortality rates of 50% or more depending on severity. Although it is more
common in premature infants, it can also be observed in term and near-term babies. (See Epidemiology
and Prognosis.)

NEC most commonly affects the terminal ileum and the proximal ascending colon. However, varying
degrees of NEC can affect any segment of the small intestine or colon. The entire bowel may be involved
and may be irreversibly damaged.

Numerous, vague reports in 19th-century literature report described infants who died from peritonitis in
the first few weeks of life. The first half of the 20th century brought more reports of peritonitis with ileal
perforation due to what was called infectious enteritis. In 1953, Scmid and Quaiser called this condition
newborn NEC. [1] The first clear report of NEC did not appear until 1964, when Berdon from the New
York Babies Hospital described the clinical and radiographic findings of 21 infants with the disease. [2]

As neonatal intensive care has progressed an d as premature newborns have come to survive long
enough for the disease to develop, the incidence of NEC in neonatal intensive care units (NICUs) has
increased. NEC remains one of the most challenging diseases confronted by pediatric surgeons. It likely
represents a spectrum of diseases with variable causes and manifestations, and surgical care must
therefore be individualized. (See Etiology, Epidemiology, and Prognosis.)

NEC typically occurs in the second to third week of life in the infant who is premature and has been
formula fed. Although various clinical and radiographic signs and symptoms are used to make the
diagnosis, the classic clinical triad consists of abdominal distension, bloody stools, and pneumatosis
intestinalis. Occasionally, signs and symptoms include temperature instability, lethargy, or other
nonspecific findings of sepsis. (See Clinical and Workup.)

Disease characteristics

Necrotizing enterocolitis affects the GI tract and, in severe cases, can cause profound impairment of
multiple organ systems. Initial symptoms may be subtle and can include 1 or more of the following (See
Clinical.):

Feeding intolerance

Delayed gastric emptying

Abdominal distention, abdominal tenderness, or both

Ileus/decreased bowel sounds

Abdominal wall erythema (advanced stages)

Hematochezia

Systemic signs are nonspecific and can include any combination of the following:

Apnea

Lethargy

Decreased peripheral perfusion

Shock (in advanced stages)

Cardiovascular collapse

Bleeding diathesis (consumption coagulopathy)

Nonspecific laboratory abnormalities can include the following (See Workup.):

Hyponatremia
 Metabolic acidosis

Thrombocytopenia

Leukopenia or leukocytosis with left shift

Neutropenia

Prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT), decreasing
fibrinogen, rising fibrin split products (in cases of consumption coagulopathy)

Etiology

Although the exact etiology of necrotizing enterocolitis (NEC) remains unknown, research suggests that
it is multifactorial; ischemia and/or reperfusion injury, exacerbated by activation of proinflammatory
intracellular cascades, may play a significant role. Cases that cluster in epidemics suggest an infectious
etiology. Gram-positive and gram-negative bacteria, fungi, and viruses have all been isolated from
affected infants; however, many infants have negative culture findings.

Furthermore, the same organisms isolated in stool cultures from affected babies have also been isolated
from healthy babies. Extensive experimental work in animal models suggests that translocation of
intestinal flora across an intestinal mucosal barrier rendered vulnerable by the interplay of intestinal
ischemia, immunologic immaturity, and immunological dysfunction may play a role in the etiology of the
disease, spreading it and triggering systemic involvement. Such a mechanism could account for the
apparent protection breast-fed infants have against fulminant NEC.

Animal model research studies have shed light on the pathogenesis of this disease. Regardless of the
triggering mechanisms, the resultant outcome is significant inflammation of the intestinal tissues, the
release of inflammatory mediators (eg, leukotrienes, tumor necrosis factor [TNF], platelet-activating
factor [PAF]) and intraluminal bile acids, and down-regulation of cellular growth factors, all of which lead
to variable degrees of intestinal damage.

Overview

Necrotizing enterocolitis (NEC) is a serious gastrointestinal disease of neonates. Its etiology is unknown.
NEC is characterized by mucosal or transmucosal necrosis of part of the intestine. Infants born before
term who are undersized and ill are most susceptible to NEC; the incidence of NEC is increasing because
of the improved survival rate in the high-risk group of premature infants. [1, 2, 3, 4, 5, 6, 7] (See the
images below.)

Radiography

Infants suspected of having NEC should undergo periodic radiography of the abdomen. In some centers,
infants in whom NEC is highly suspected undergo routine frontal abdominal radiography every 4-6
hours.

Cross-table lateral examinations with a horizontal beam are useful for detecting subtle, early collections
of free air, although some clinicians prefer to use lateral decubitus radiographs to detect free air (see
the images below). In the presence of peritoneal adhesions, keeping the patient in the decubitus
position for a prolonged period ensures that the air moves to the highest point.

This radiograph shows free air secondary to bowel

This radiograph shows free air secondary to bowel wall necrosis.

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Left lateral decubitus radiograph shows free air.

Left lateral decubitus radiograph shows free air.

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In this radiograph, free air is observed over the

In this radiograph, free air is observed over the liver that outlines the falciform ligament. This finding
indicates perforation of the bowel, which necessitates surgical exploration and resection of necrotic
bowel.

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Imaging findings

Radiography is sufficient for an accurate diagnosis of NEC; the presence of air on a horizontal-beam
radiograph is sufficient for diagnosing a bowel perforation.
Abdominal radiographs may demonstrate multiple dilated bowel loops that display little or no change in
location and appearance with sequential studies. Pneumatosis intestinalisgas in the bowel wall that
displays a linear or bubbly patternis present in 50-75% of patients. (See the images below.)

The radiograph demonstrates multiple dilated loops

The radiograph demonstrates multiple dilated loops in the large bowel and small bowel. Note the
pneumatosis intestinalis with bubbly and linear gas collections in the bowel wall.

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Increasing pneumatosis intestinalis is seen in thi

Increasing pneumatosis intestinalis is seen in this radiograph.

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Anteroposterior image shows necrotizing enterocoli

Anteroposterior image shows necrotizing enterocolitis with pneumatosis intestinalis.

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Lateral abdominal image shows pneumatosis intestin

Lateral abdominal image shows pneumatosis intestinalis.

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Portal venous gas and gallbladder gas are indicative of serious disease. Pneumoperitoneum indicates a
bowel perforation. (See the image below.)

Portal venous air is present in a patient with pne

Portal venous air is present in a patient with pneumatosis intestinalis.

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Computed tomography (CT) scanning or a water-soluble enema examination may be used to

demonstrate pneumatosis or a site of perforation. (See the image below.)

Image obtained during examination with a water-sol

Image obtained during examination with a water-soluble enema shows the pneumatosis well. This
technique is not recommended.

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A high index of suspicion is essential for the diagnosis of NEC.

Small amounts of free air may not be easily visible on supine abdominal radiographs. Thickening of the
bowel wall may not be easily observed in the presence of a dilated bowel.

Computed Tomography

The use of CT is not advocated for the diagnosis of NEC or for identifying the presence of free air. CT
scanning or an examination with a water-soluble enema may be used to demonstrate pneumatosis or a
site of perforation.

Ultrasonography

Ultrasonography of the abdomen characteristically shows thick-walled loops of bowel with hypomotility.
Intraperitoneal fluid is often present.

In the presence of pneumatosis intestinalis, gas is seen in the portal venous circulation within the liver.
[8]