ARTICLE

Abdominal Surgical Emergencies in

Neonates
Pablo Laje, MD, FACS*†
*Division of General, Thoracic, and Fetal Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA
†
Children’s Hospital of Philadelphia, Philadelphia, PA

EDUCATION GAPS

As medical knowledge expands and residency training becomes more and

more focused on each chosen field, it is increasingly difficult to remain up to
date on the areas of medicine beyond our own specialty. This is particularly
true for medical trainees, who rarely get a solid background on the manage-
ment of surgical issues. This review aims to narrow that gap by providing a
practical approach to the care of a newborn with an acute abdomen, avoid-
ing unnecessary delays in care.

OBJECTIVES After completing this article, readers should be able to:

1. Recognize the most common causes of surgical abdominal emergencies

in newborns.
2. Explain the sequence of imaging studies required for each of these
clinical scenarios.
3. Be able to act promptly and systematically when treating a newborn
with an acute abdomen.

ABSTRACT
Abdominal surgical emergencies are relatively common in neonates. Some
of them are related to congenital diseases such as intestinal atresia and
intestinal malrotation, whereas some are entirely postnatal conditions such
as necrotizing enterocolitis and gastric perforation. While there is a wide
range of clinical severity for these conditions, outcomes are most favorable
with prompt identification and expeditious treatment. In this review, we AUTHOR DISCLOSURES Dr Laje has
disclosed no financial relationships
describe the most common neonatal abdominal surgical emergencies, relevant to this article. This commentary
highlight the signs that can help with early detection, and explain the does not contain a discussion of an
approach to diagnosis and management. unapproved/investigative use of a
commercial product/device.

ABBREVIATIONS
INTRODUCTION DA duodenal atresia
Abdominal surgical emergencies are relatively common in neonates. Some of NEC necrotizing enterocolitis
SIP spontaneous intestinal
them are related to congenital diseases such as intestinal atresia and intestinal perforation
malrotation, whereas some are entirely postnatal conditions such as necrotizing UGI upper gastrointestinal study

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 enterocolitis (NEC) and gastric perforation. Although there
is a wide range of clinical severity for these conditions,
outcomes are most favorable with prompt identification
and expeditious treatment. Certain congenital abnormali-
ties such as duodenal atresia (DA) can be suspected prena-
tally, ensuring early family education and antepartum
planning, including delivery at a facility with surgical capa-
bilities. None of these congenital diseases requires prena-
tal intervention (except for the management of severe
polyhydramnios for obstetrical purposes, if present).
The clinical hallmark of nearly all surgical abdominal
emergencies in neonates is bilious emesis, which, despite
being highly nonspecific, indicates a surgical problem un-
til proven otherwise. The age of the newborn and a few
simple clinical signs such as abdominal distension, ab-
dominal wall erythema, or the absence of a well-formed
anus can narrow the differential diagnosis with great accu-
racy. Imaging studies are critical in the workup of all sus-
pected abdominal surgical emergencies, but complex
studies such as magnetic resonance imaging and com-
puted tomography scans are rarely required. In this re-
view, we describe the most common neonatal abdominal
surgical emergencies, highlight the signs that can help
with early detection, and explain the approach to diagnosis
and management.
GASTRIC PERFORATION
Gastric perforation is quite rare in neonates and accounts
for only 7% to 12% of all gastrointestinal perforations in
this population. (1) However, it must be suspected in any
newborn who develops a sudden massive pneumoperito-
neum and clinical deterioration. Approximately half of the
cases are idiopathic, and the remaining half are either iat-
rogenic or have a clear correlation with another condition.
In general, any episode of extreme gastric dilatation can
result in a tear of the gastric wall, such as infants with un-
recognized DA (more so if combined with type C esopha-
geal atresia) and accidental intubation of the esophagus
with delivery of positive pressure. (1) Some cases of gastric
perforation can be related to global intestinal ischemia,
such as NEC and hypoperfusion, due to congenital cardiac
anomalies; however, because the gastric wall is particularly
well vascularized, ischemia is an extremely rare etiology.
Lastly, some cases are clearly iatrogenic, secondary to the
instrumentation of the stomach (eg, esophageal gap cali-
bration, incorrect placement of a nasogastric tube).
Cases that are thought to be idiopathic typically occur
in premature newborns and in newborns with low birth-
weight within the first days after birth. (1) It is common
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for these newborns to have nasogastric tubes for decom-
pression; therefore, the possibility of perforation being the
result of trauma must always be considered (although the
management is the same, regardless of the cause). When
abdominal radiographs show the end of the nasogastric
tube to be located in the pelvis, it is highly likely that it
has gone through the gastric wall. It is particularly impor-
tant that practitioners caring for newborns with low birth-
weight take special precautions when choosing the size of
the nasogastric tube and determining the depth at which
the tube is placed. Cases that are truly idiopathic might
be related to histological anomalies in the structure of
the gastric wall, but no hypothesis has been conclusively
proven yet.
From a clinical perspective, gastric perforation should
be suspected in any newborn who develops acute abdomi-
nal distension, along with lethargy, apnea, and respiratory
distress. (2) Bloody output through the nasogastric tube is
also a common finding. A plain abdominal radiograph is
the only study needed, which typically shows an extensive
pneumoperitoneum that is larger than the pneumoperito-
neum, as commonly seen in cases of intestinal perforation
(Fig 1).
If a gastric perforation is suspected in the setting of a
pneumoperitoneum, an exploratory laparotomy is required.
During this surgery, the surgeon approaches the abdomen
through a transverse supraumbilical laparotomy. Most gas-
tric perforations occur along the greater curvature, although
the lesser curvature and the esophagogastric areas can also
be involved. Some perforations are small defects that re-
quire a simple closure, but others can be extensive and sur-
rounded by ischemic gastric wall tissue, requiring wide
resections that lead to markedly decreased gastric volume.
In addition to closing the defect and resecting all devitalized
tissue, a gastrostomy should be created, if at all feasible.
The gastrostomy tube ensures effective decompression of
Figure 1. A. Extensive pneumoperitoneum on an anteroposterior radio-
graph, with the so-called “football” sign (arrow). B. Extensive pneumoperi-
toneum on a lateral abdominal radiograph. The star marks the “Rigler”
sign, or the visualization of both sides of the bowel wall, as a result of the
peritoneum.
 the stomach after the operation without the need to keep
a nasogastric tube in place. Maintaining, manipulating,
and replacing a nasogastric tube after surgery carry a risk
of damaging the sutures of the gastric wall repair and,
thus, should be avoided postoperatively. Around 5 to 7
days after the operation, a contrast study is recom-
mended to confirm appropriate healing of the gastrorrha-
phy, and if that is the case, gastric feedings can be
initiated. The gastrostomy should remain until the new-
born has accomplished full-volume oral skills. It is, how-
ever, rare that the gastrostomy is closed before 6 months
of age.
In newborns with idiopathic gastric perforation, early
recognition with surgical exploration prior to the develop-
ment of overwhelming sepsis clearly improves outcomes, al-
though other factors such as prematurity and low birthweight
also play a significant role in the overall prognosis. (3)
In newborns with gastric perforations secondary to other con-
ditions such as congenital heart disease and NEC, the out-
comes are mostly determined by the underlying disease.

DUODENAL ATRESIA Figure 2. Anteroposterior radiograph showing the classic “double

DA is thought to be caused by a lack of recanalization
within the duodenum between 8 and 10 weeks’ gestation.
It occurs in one in 5,000 to 10,000 newborns. (4) DA
rarely constitutes a surgical emergency, but lack of proper
identification can lead to unnecessary complications. With
the ever-increasing widespread access to prenatal care, DA
is commonly detected prenatally in the developed world. (4)
The classic fetal ultrasonographic finding is the “double
bubble” sign, representing the distended stomach and prox-
imal duodenum along with polyhydramnios. The “double
bubble” sign can also be seen in postnatal radiographs
along with the absence of distal intestinal air (Fig 2). Most
cases of DA are isolated, but affected patients can have
other congenital malformations, such as esophageal atresia,
anorectal anomalies, and structural heart disease, or chro-
mosomal anomalies, such as trisomy 21. The atresia can be
located proximal to the ampulla of Vater, in which case the
output of the nasogastric tube will be clear, or distal to the
ampulla of Vater, in which case the output of the nasogas-
tric tube will be bilious.
If there is a high prenatal suspicion for DA, postnatal
management includes gastric decompression soon after
birth, initiation of parenteral nutrition, and echocardiog-
raphy to rule out congenital cardiac disease. (5) Other
evaluations should be guided by the postnatal physical ex-
amination findings. As noted earlier, the diagnosis of DA
can be confirmed with a plain radiograph. Some affected
bubble” sign of duodenal atresia with the distended stomach (black star)
and the distended proximal duodenum (white star). The tip of the gastric
tube is in the area of the pylorus.
neonates with appropriate gastric decompression may
not have a “double bubble” sign. In this case, a small
amount of air (10 mL/kg) can be injected through the na-
sogastric tube just before obtaining another radiograph
in an attempt to visualize the gastric bubble as well. An
upper gastrointestinal imaging (UGI) study is rarely
needed in typical cases of DA but might be useful in
cases where the radiographs show the “double bubble”
along with distal air in the small bowel. This scenario oc-
curs when the duodenal obstruction is caused by an incom-
plete membrane that allows air to pass. This incomplete
obstruction represents a diagnostic challenge and is usually
diagnosed beyond the neonatal period (the more incom-
plete the obstruction, the later the diagnosis).
In addition to DA, the differential diagnosis of duodenal
obstruction includes several entities that can be surgically
challenging for the treating surgeon (eg, annular pancreas,
preduodenal portal vein), but the preoperative management
does not differ from that for classic DA. Midgut volvulus
should always be considered in the differential diagnosis.
This is a complication of intestinal malrotation that gener-
ates an acute obstruction of the duodenum and can show ra-
diological findings that resemble those of DA. However, it is
extremely rare for this event to happen during the first day

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 after birth. Rather, most affected patients have tolerated feed-
ings for some time without radiographic abnormalities and
become acutely ill when the volvulus develops.
The repair of DA is never an emergency and should be
performed only after the newborn is clinically stable and
all potential associated conditions have been ruled out.
Premature newborns with low birthweight should not un-
dergo repair until a reasonable weight has been achieved.
The outcomes of DA are dictated by the concomitant con-
ditions, if present. Isolated cases of DA have a survival
rate of more than 95%. (6)
MIDGUT VOLVULUS
Midgut volvulus is the most severe complication of intesti-
nal malrotation. While it can occur prenatally, most cases
of midgut volvulus occur after birth. The incidence of in-
testinal malrotation in the general population is unknown;
therefore, the percentage of patients with intestinal malro-
tation who develop midgut volvulus is also unknown.
However, it is clear that the incidence of midgut volvulus
is the highest within the first 2 months after birth and de-
creases sharply thereafter, with approximately 80% of
cases occurring within the first year of age. (7) Cases that
occur after 5 years of age are anecdotal.
Because the rotation of the intestine is tightly related to
the formation of other abdominal and thoracic organs, ro-
tation anomalies are quite common in patients with ab-
dominal wall defects, congenital diaphragmatic hernia,
prune belly syndrome, and heterotaxy. Approximately
40% of cases of intestinal malrotation are isolated.
From an anatomical perspective, intestinal malrotation
encompasses a wide range of anomalies. (8) In normal
conditions, the duodenojejunal junction is in the left up-
per quadrant and the ileocecal valve in the right lower
quadrant. These sites are far from each other, creating a
wide mesenteric base that is unlikely to twist. The classic,
complete intestinal malrotation includes the following 2
features that make the midgut prone to a volvulus: the
midabdominal location of the duodenojejunal junction
and the location of the ileocecal valve in the right upper
quadrant/midabdomen. These 2 features combined create
a narrow pedicle at the base of the mesentery of the small
bowel, making it likely to rotate. Other forms of intestinal
malrotation include “nonrotation” (observed in cases of
gastroschisis) and “reverse rotation.” In these conditions,
the duodenojejunal junction is not located in the right up-
per quadrant, but the ileocecal region is free of attach-
ments and/or far enough from the duodenum that the
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mesentery of the small bowel does not have a narrow
pedicle. (9)
Intestinal malrotation can be an incidental finding in
patients who undergo imaging studies for other reasons.
In the newborn period, the most common presentation of
intestinal malrotation is an acute midgut volvulus. With
the rare exception of the volvulus occurring in the perina-
tal period, a newborn with an acute midgut volvulus typi-
cally presents acutely with feeding intolerance and bilious
emesis. Practitioners must have a high index of suspicion
for midgut volvulus in any newborn with new-onset bil-
ious emesis. The volvulus of the mesentery not only re-
sults in a proximal intestinal obstruction but also leads to
vascular compromise. When intestinal ischemia occurs,
the patient rapidly develops abdominal pain, acidosis, and
septic shock. Bloody stools are commonly seen. Abdomi-
nal distension is a later sign, often occurring 3 to 6 hours
later.
When patients with a midgut volvulus present with ad-
vanced clinical deterioration, a decision must be made be-
tween obtaining imaging studies and urgent surgery.
While this must be decided on a case-by-case basis, in the
presence of pain, sepsis, and abdominal distension, unless
there is another clear explanation for which an operation
is not needed, the patient should undergo surgery without
the delay imposed by imaging studies. If the patient is
deemed stable enough to have imaging studies, several op-
tions are available. Plain abdominal radiographs are not
specific because the obstruction is quite proximal. Sugges-
tive radiographic signs include a distended gastric lumen
with a paucity of distal air. A radiograph is important to
rule out a pneumoperitoneum; if it is found, no other
studies are required, and the patient needs an exploratory
laparotomy. A UGI is the gold standard for the diagnosis of
both malrotation and volvulus. The confirmatory sign of a
midgut volvulus is a proximal intestinal obstruction with a
“corkscrew” sign (Fig 3). In many centers around the
world, ultrasonography is used as a first-line study to di-
agnose midgut volvulus. As is the case for many other
ultrasonographic studies, its sensitivity is highly opera-
tor-dependent. The most indicative ultrasonographic
finding is the “whirlpool” sign, given by the axial rota-
tion of the superior mesenteric vessels. (10) Ultrasonog-
raphy can also diagnose malrotation in the absence of a
volvulus, but the specificity and sensitivity are much
lower.
At the time of exploratory laparotomy in patients sus-
pected to have a malrotation with midgut volvulus, there
are three possible surgical approaches depending on the

Figure 3. Upper gastrointestinal contrast study confirming the diagnosis
of intestinal malrotation with midgut volvulus as a result of the
“corkscrew” (white arrow).
infant’s intestinal compromise. In the first scenario, the
volvulus has not resulted in significant ischemia, in which
case the patient undergoes a Ladd procedure. The Ladd
procedure involves the following 5 steps:
1. Devolvulizing the midgut
2. Widening the mesentery and taking down all adhesions
to separate the duodenojejunal junction as far as possi-
ble from the ileocecal region
3. Verticalizing the duodenum
4. Performing appendectomy
5. Replacement of the small bowel on the right hemi-ab-
domen and the colon on the left hemi-abdomen
In the second scenario, the midgut has sustained a sig-
nificant ischemic hit, but it is not frankly necrotic. In this
case, the abdomen is closed loosely or left open and con-
tained in a silo, and the patient requires another operation
24 hours later. By this time, after aggressive resuscitation,
the intestine has recovered. In the third scenario, the mid-
gut is frankly necrotic and nonrecoverable. In this severe
case, there are 2 possible approaches: the infant’s entire
midgut is removed, leading to prolonged hospitalizations,
a high incidence of liver failure, and high morbidity and
mortality, or the surgery is halted so that the infant can re-
turn to the neonatal intensive care with the possibility of
transitioning to comfort care. Multidisciplinary discus-
sions with the family are needed to help determine the
preferred approach.
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SMALL BOWEL ATRESIA
Small bowel atresias are commonly suspected before birth.
Suggestive prenatal signs are polyhydramnios and dis-
tended bowel loops. Proximal atresias are easier to diag-
nose than distal atresias because the imaging features are
more sensitive and specific. The true incidence of small
bowel atresias is unknown, but most reports suggest 1 to 3
cases per 10,000 live births. (11) The etiology of small
bowel atresias is also unknown. Most clinical observations
and experimental models suggest that small bowel atresias
(except for DA) are the result of segmental vascular occlu-
sions and, depending on the extent of the ischemia, can
involve the mesenteric tissue and result in a mesenteric
gap. (12)
From a clinical standpoint, proximal (jejunal) atresias
present early after birth with bilious emesis and mild ab-
dominal distension. In distal (ileal) atresias, the proximal
bowel can accommodate a remarkable amount of fluid,
and, thus, emesis is a later finding. In fact, it is common
that patients with unknown distal atresias are fed for 1 or
more days before developing bilious emesis. Failure to
pass meconium is commonly seen in patients with small
bowel atresia, but passing meconium does not rule out
small bowel atresia.
Plain abdominal radiographs of patients with small
bowel atresia typically show distended bowel loops. The
more distal the atresia, the greater the number of distended
loops on the radiograph (Fig 4). Plain radiographs are gen-
erally the only images needed to determine whether an in-
fant should undergo an exploratory laparotomy. A UGI is
useful in ruling out a midgut volvulus, but if the suspicion
is low, it is not required. In cases of distal atresias with
multiple distended bowel loops, a contrast enema can be
helpful in ruling out some conditions that may not need an
operation (eg, meconium ileus) or may need a different
Figure 4. A. Abdominal radiograph consistent with jejunal atresia, show-
ing a handful of widely distended bowel loops. B. Abdominal radiograph
consistent with ileal atresia, showing multiple distended bowel loops.
Vol. 24 No. 2 F E B R U A R Y 2 0 2 3 e101
 surgical approach (eg, total colonic Hirschsprung’s disease,
colonic atresia).
There are several anatomical types of small bowel atresias
(Fig 5). Surgical repair can be challenging, particularly in
cases of multiple atresias or atresias that have resulted in
short bowel, which is common in type IIIb atresias. The re-
pair of a small bowel atresia is hardly ever an emergency,
but failure to recognize the diagnosis can potentially lead to
severe complications such as bowel perforation and extreme
abdominal distension with respiratory compromise. (13)(14)
The overall prognosis of healthy newborns with isolated
small bowel atresias is remarkably good, with a survival rate
of more than 90%. (15) Prematurity, concomitant malforma-
tions, and short bowel syndrome, on the other hand, are fac-
tors associated with increased morbidity and mortality. (15)
SPONTANEOUS INTESTINAL PERFORATION
Spontaneous intestinal perforation (SIP) consists of a single,
discrete perforation of the distal small bowel, with no appar-
ent explanation and no vascular compromise of the rest of
the intestine. Even though the perforation can occur any-
where, most cases occur in the terminal ileum. SIP has
some clinical features that overlap with those of NEC, but
these diagnoses are 2 completely different conditions that
have different pathophysiologies and management strategies.
Figure 5. Different anatomical types of small bowel atresias. Type IIIb is
also known as “apple peel,” has a different pathophysiology than the other
4 types, and is commonly associated with other malformations. (From
Huebner R, Azarow KS, Cusick RA. Intestinal atresia due to intrauterine
intussusception of a Meckel's diverticulum. J Pediatr Surg Case Rep.
2013;1(8):232–234, reproduced with permission from Creative Commons.)
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SIP occurs mainly in premature newborns, with an inci-
dence that increases as the gestational age at birth decreases.
(16) SIP tends to occur within the first few days after birth
in otherwise stable newborns, whereas NEC tends to occur
beyond the first week of age. Lastly, SIP is more common in
males than females. (16)(17)
The pathophysiology of SIP is not well understood.
Some well-known risk factors such as the perinatal use of
maternal indomethacin as a tocolytic agent, the postnatal
use of indomethacin, and maternal preeclampsia suggest
that an ischemic vascular event might be involved. (18)
Other risk factors for which a mechanistic explanation has
not been found are the use of postnatal steroids, fungal in-
fections, and chorioamnionitis. Lastly, SIP has been de-
scribed several times in sets of twins. (19)
The typical clinical presentation of SIP is that of a sta-
ble premature newborn who develops acute abdominal
distension within the first few days after birth, followed by
progressing signs of sepsis. Plain abdominal radiographs
typically show a large pneumoperitoneum, without the
classic findings of NEC (eg, pneumatosis intestinalis, por-
tal venous gas) (Fig 6). No further imaging studies are
needed after an abnormal plain abdominal radiograph has
been obtained.
The management of SIP is surgical, supported by wide-
spectrum antibiotics. Newborns with weight less than
1,000 g typically undergo placement of a percutaneous
drain at the bedside. The goals of this maneuver are to 1)
release the high-pressure pneumoperitoneum to eliminate
any abdominal competition and potential splanchnic/renal
hypoperfusion and 2) provide an outlet for the spilled in-
testinal contents. Approximately 50% of patients who un-
dergo a drain placement go on to recover and regain
Figure 6. Large-volume pneumoperitoneum shown in a lateral radio-
graph (left) and anteroposterior radiograph (right) in a premature new-
born with spontaneous intestinal perforation. No portal venous gas and
no pneumatosis intestinalis are seen.
 bowel function without the need for any further surgical
intervention. (20) The other 50% either do not improve
with the drain and need a laparotomy shortly after the
drain placement or recover from the acute event but re-
quire surgical exploration later to repair a stricture. (20)
Another frequent outcome is that the site of the drain
gradually turns into an enterocutaneous fistula, which
eventually requires an elective surgical repair. Newborns
with a SIP who weigh more than 1,000 g generally un-
dergo a laparotomy instead of drain placement. Most com-
monly, these affected infants have a single perforation in
the distal ileum, with an otherwise healthy bowel. This
perforation can be managed in 2 ways: 1) simple primary
closure (usually suitable for patients who are hemodynam-
ically stable), or 2) a temporary ostomy (a safer option for
unstable patients).
The outcomes of newborns with SIP are mostly related
to their degree of prematurity and associated complica-
tions rather than the perforation itself. No differences in
outcomes have been found between patients who under-
went a drain placement alone and patients who underwent
a laparotomy. (20)
NECROTIZING ENTEROCOLITIS
NEC is one of the most severe gastrointestinal diseases of
the neonatal period and has a wide range of clinical pre-
sentations. It is characterized by global intestinal ische-
mia that leads to damage to the intestinal mucosa,
bacterial invasion of the intestinal wall, and sepsis. NEC
is a disease of prematurity with a global incidence of ap-
proximately 5% in newborns born less than or equal to
30 weeks’ gestation. (21) While NEC is mostly observed
in premature newborns, approximately 10% of the cases
occur in term newborns. The affected term patients tend
to have preexisting conditions that lead to global hypoper-
fusion such as congenital heart disease or overwhelming
sepsis.
The pathophysiology of NEC is unknown, but the high
incidence observed in premature newborns suggests that
the immaturity of the intestinal wall and the immune sys-
tem play a key role through multiple mechanisms such as
poor mucin production and over permeability of the inter-
cellular junctions. (22) Changes in the bacterial composi-
tion of the intestine are clearly associated with NEC,
which is evidenced by a higher incidence in newborns who
have received antibiotics for several days. (23) Other well-
known factors associated with NEC are treatment with H2
blockers, the use of nonhuman milk, and the enteral admin-
istration of hyperosmolar medications and formulas. (24)
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Advances in the care of premature newborns and meas-
ures to prevent NEC have resulted in a decreasing inci-
dence in the United States in the last decade. Among
those advances are the wider availability of pasteurized do-
nor human milk, the decreased use of H2 blockers, and
the more conscientious use of blood transfusions and anti-
biotics in premature newborns. (25)
Most cases of NEC occur after the second week of age in
newborns who have been receiving enteral feedings. NEC is
a progressive disease that starts when a certain threshold is
reached. Some cases of NEC advance quickly regardless of
any intervention, and in some cases the progression is halted
by medical measures. There is no single test that can be
used to confirm the diagnosis of NEC. Instead, the diag-
nosis is suspected or confirmed based on several clinical,
laboratory, and radiological findings. This approach al-
lows us to establish the following 3 main stages of NEC:
• Stage I: Suspected NEC
• Stage II: Definite mild or moderate NEC
• Stage III: Definite severe NEC
The Table summarizes the specific findings associated
with each stage. While this classification originally de-
scribed by Bell is arbitrary and has been modified several
times, it allows for a practical approach to each case.
Imaging studies are key in the diagnosis and monitor-
ing of NEC. Two-view plain abdominal radiographs have
been, and remain, the diagnostic gold standard, but there
is a growing body of literature supporting the use of ab-
dominal ultrasonography. Bowel dilation is the earliest
sign that suggests NEC in the appropriate clinical context.
Findings that are confirmatory of NEC, again in the appro-
priate clinical context, are pneumatosis intestinalis, portal
venous gas, and pneumoperitoneum. Ascites can be sus-
pected on plain radiography but only when it is of large
volume. Ultrasonographic studies can show some of the
radiological findings such as ascites and pneumatosis but
can also show additional features of injured bowel such as
hypoperistalsis, hypoperfusion, bowel wall thickening, and
bowel wall thinning. (26) At this point, however, there are
no standardized ultrasonographic findings that are supe-
rior to plain radiographs to diagnose NEC or help deter-
mine the optimal timing of surgical intervention.
From a surgical standpoint, NEC presents several chal-
lenges. Patients who have a pneumoperitoneum (stage IIIb)
need surgical intervention. As is the case for SIP, if the pa-
tient’s weight is less than 1,000 g, the most common initial
approach is the placement of a peritoneal drain to release
the tension pneumoperitoneum, eliminate the competition
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 Table. NEC staging and management based on clinical, laboratory, and radiologic findings
Stage Systemic Signs Abdominal Signs Radiologic Signs Management
Ia: suspected NEC Temperature instability, Abdominal distension, Normal or mildly NPO and antibiotics for
apnea, bradycardia, vomiting, gastric distended loops 3 days
lethargy residuals, heme-positive
stool
Ib: suspected NEC Same as above Grossly bloody stool Same as above Same as above
IIa: definite NEC (mild) Same as above Same as above 1 no Ileus and/or pneumatosis NPO and antibiotics for
bowel sounds ± 7 days
abdominal pain
IIb: definite NEC Same as above 1 mild Same as above 1 definite Same as above 1 ascites NPO and antibiotics for
(moderate) acidosis and tenderness ± 14 days
thrombocytopenia abdominal wall redness
IIIa: severe NEC (intact Same as above 1 Same as above 1 frank Same as above Same as above 1
bowel) hypotension, severe peritonitis cardiorespiratory
acidosis, and coagulopathy support
IIIb: severe NEC Same as above Same as above Same as above 1 Same as above 1
(perforated bowel) pneumoperitoneum surgery

NEC5necrotizing enterocolitis, NPO5nothing by mouth.

between the abdominal pressure and the diaphragm, and
evacuate the intestinal contents. Some patients will recover
gradually without the need for further surgical intervention,
and some patients will either remain ill and require a surgi-
cal exploration or will develop post-NEC complications that
will require surgical repair (eg, enterocutaneous fistula, stric-
ture). Patients who do not improve within 24 to 48 hours af-
ter the drain placement require surgical exploration, but it is
important to inform the family that the mortality associated
with this intervention is significant.
The clinical parameters associated with NEC that can be
used to determine the need for an exploration (regardless
of the patient’s weight) are greater degree of acidosis, in-
creased cardiorespiratory support, worsening coagulop-
athy, and greater erythema or bluish discoloration of the
abdominal wall, among others. The decision to operate
should not be made on the basis of a single parameter.
The rationale behind the need for surgical exploration
and bowel resection is that the ischemic (not necrotic) in-
testinal tissue keeps the patient in a vicious circle of pro-
found acidosis ! sepsis ! cardiovascular compromise
! further intestinal hypoperfusion ! further acidosis.
This vicious circle can be interrupted by removing the is-
chemic bowel.
Patients who have a pneumoperitoneum and a weight of
more than 1,000 g typically undergo an exploratory laparot-
omy instead of a drain placement. There are several potential
clinical scenarios at the time of the laparotomy, ranging
from a minimal amount of compromised bowel to NEC to-
talis, characterized by visible necrosis of most of the small
bowel and colon. Cases with only a minimal amount of com-
promised bowel are usually managed with resection of the
necrotic bowel and a temporary ostomy. The ostomy is taken
down several weeks later once the patient has recovered
from the acute event and is back on full enteral feedings. It
is critical to obtain contrast studies of the distal bowel prior
to closing the ostomy to rule out strictures.
Patients with NEC totalis represent an ethical dilemma.
Resecting the entire bowel can, in fact, lead to rapid clini-
cal improvement, but the morbidity and mortality associ-
ated with short bowel syndrome are remarkably high.
Those affected patients who survive have an exceedingly
long hospital stay and are prone to multiple subsequent
hospitalizations, recurrent central line infections, and liver
failure. The alternative to a resection of virtually the entire
bowel is to close the abdomen and provide end-of-life
comfort care. Cases of NEC totalis require an open con-
versation between the family, the surgeon, and the neo-
natology team prior to making any irreversible surgical
decisions.
Some challenging cases of NEC have, at the time of the
laparotomy, evidence of ischemia (without frank necrosis)
of an extensive amount of bowel that does not qualify as
NEC totalis but would result in significant bowel loss if a
resection were to be performed. Those cases benefit from
temporary abdominal wall coverage and a second-look lap-
arotomy 24 hours later. During this time period, the bowel
can either reperfuse and improve, or become frankly ne-
crotic. By delaying the operation, the surgeon can gain a
better understanding of how much bowel needs to be
resected.
Another challenge in the management of NEC is if and
when to do an exploratory laparotomy in the absence of
pneumoperitoneum. These cases are, by definition, stage

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 IIIa. As mentioned earlier, there is no single test that can
tell the surgical team when to operate and when to con-
tinue expectant management. The most appropriate way
to approach these cases is to be involved in the care of the
patient from the early stages of the disease. As a rule,
when the clinical parameters worsen despite maximal
medical therapy, an operation is indicated. Lastly, there
are cases that at the time of the initial evaluation are al-
ready in extremis and have diffuse pneumatosis suggest-
ing NEC totalis. In this scenario, if there is a reasonable
level of suspicion, avoidance of an unnecessary laparotomy
and end-of-life comfort care should be considered, particu-
larly if the affected infants are extremely premature.
CONCLUSION
Surgical abdominal emergencies are common in the hos-
pital setting. Neonatal clinicians should be able to detect
early signs of an abdominal emergency and initiate a rapid
and systematic diagnostic plan. Involving the surgical
team early is also of utmost importance. Improvements in
the quality of imaging studies will always result in better
outcomes. However, nothing will replace the astute clini-
cal eye of the clinician who can put in place a prompt and
proper diagnostic plan.
American Board of Pediatrics
Neonatal-Perinatal Content
Specification
• Know the clinical and diagnostic features, evalu-
ation, management, and complications of NEC.
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