1. Spontaneous intestinal
perforation vs NEC:
What is the difference??
Dr Varsha Atul Shah

2. Spontaneous intestinal perforation
 Isolated perforation of newborn
 Typically at terminal ileum
 Separate clinical entity from NEC*
 Differentiation is important as there are
management considerations
*J Am Coll Surg. 2002 Dec;195(6):796-803.
Spontaneous localized intestinal perforation in very-low-birth weight infants: a distinct clinical entity different from necrotizing
enterocolitis

3. epidemiology
 Commonly found in VLBW, ELBW
 Risk~2-3 % in VLBW, 5% in ELBW
 Median gestational age 25-27 weeks
 Median BW 670-973g
 More frequent in male infants

4. Risk factors
 Prematurity
 Antenatal
3. Severe placental chorioamnionitis*
4. ? Glucocorticoids/NSAIDS
 Postnatal
6. Early postnatal glucocorticoids@
7. ? Indocid
* Maternal factors in extremely low birth weight infants who develop spontaneous intestinal perforation.
Ragouilliaux CJ; Keeney SE; Hawkins HK; Rowen JL Pediatrics 2007.
@Focal small bowel perforation: an adverse effect of early postnatal dexamethasone therapy in extremely low birthweight infants.
Gordon PV; Young ML; Marshall DD J Perinatol. 2001 Apr-May;21(3)
New insights into spontaneous intestinal perforation using a national data set
Attridge JT; Clark R; Gordon PV J Perinatol. 2006 Nov;26(11):667-70. Epub 2006 Oct 5.

5. Pathology and pathogenesis
 Single isolated perforation
 Typically in terminal iluem, but also reported
in jejunum, colon
 Focal hemorrhagic necrosis with well defined
margins seen(in contrast to ischemic,
coagulative necrosis in NEC)
 Bowel proximal and distal to perforation
normal

6. Clinical presentation
SIP NEC
First week of life, median age After first week,
7(0-15) Median age 15
Abdominal distension, bluish Abdominal distension
discoloration(groin, scrotum) Abdominal erythema
Hypotension Crepitus, induration
Pneumoperitoneum, gasless Pneumatosis intestinalis, portal
abdomen venous gas, transient thickening of
intestinal wall, fixed dilated SB
loops, pneumoperitoneum
Associated sepsis due to CONS,
fungemia
Leukocytosis, raised ALP,
bilirubin, decreased platelet, hct

7. Clinical diagnosis based upon:
 Clinical presentation
 Physical examination
 Abdominal radiographs support diagnosis
Definitive diagnosis :
Direct visualization of intestinal perforation in setting of
otherwise healthy appearing small bowel

9. Management
 NBM, drip and suck
 Fluid resuscitation
 Intravenous antibiotics
 Surgical treatment
5. Definitive treatment
6. Exploratory laparotomy with bowel
resection
7. Primary peritoneal drainage

10. Primary peritoneal drainage
 Avoids laparotomy, need for GA and transport
 Many do recover without any further surgical
intervention
 Laparotomy indicated if:
4. Reaccumulation of free air after drain removed,
indicating perforated bowel did not seal
5. Fistula with intestinal drainage that fails to close
6. Bowel obstruction secondary to adhesions or
stricture at site of perforation

11. Long term outcome
 Survival rates of 64-90%
 Neurodevelopmental outcome better than those with
NEC*
 Increased risk to develop ROP and PVL compared
to controls
*Intestinal perforation in very low birth weight infants: growth and neurodevelopment at 1 year of age.
Adesanya OA; O'Shea TM; Turner CS; Amoroso RM; Morgan TM; Aschner JL J Perinatol. 2005 Sep;25(9):583-9.

12. NEC
 Ischaemic necrosis of intestinal mucosa,
associated with inflammation, invasion of
enteric gas forming organisms and dissection
of gas into muscularis and portal venous
system.

13. NEC
 1-3 per 1000 live births
 Predominently in prems, up to 6-7% in VLBW
 Incidence decreases with increasing GA, BW
 Males and females equally affected
 Sometimes occurs in clusters, associated with
epidermics
 Reported mortality of 15-30%
 13% occur in term infants

14. Pathogenesis
 Terminal ileum and colon
 Entire GIT in severe cases
 Pathogenesis remains unknown
 Heterogeneous disease
 results from multiple factors that result in mucosal injury in
susceptible host
6. Prematurity
7. Microbial bowel overgrowth
8. Milk feeding
9. Impaired mucosal defense
10. Circulatory instability of intestinal tract
11. Medications
12. CHD, perinatal asphyxia, polycythemia, sepsis, respiratory
disease
13. Inflammation

16. Clinical presentation
 Timing of onset of symptoms varies, inversely
related to GA
 Systemic signs
3. Nonspecific (apnea, resp failure, lethargy, poor
feeding, temp instability)
4. Hypotension
 Abdominal signs
- distension, gastric retention, tenderness, vomiting,
diarrhoea, rectal bleeding, bilious aspirates

17. Bells staging
 Classifies severity of NEC based on severity
of systemic, intestinal, radiographic findings
 Treatment directed at clinical signs rather
than particular stage of NEC

18. Bells staging

19. Diagnosis
 Clinical
 Radiologic findings

20. Clinical
 Abdominal distension
 Rectal bleeding

21. Radiological
 AXR/lateral decubitus (left side down)
 Confirm diagnosis/follow progression of disease
 Abnormal gas patterns, dilated loops of bowel(ileus)
 Pneumatosis intestinalis(hallmark of NEC)
 Pneumoperitoneum (football sign)
 Sentinel loops(necrotic/perforation)
 Portal venous gas

25. Abdominal Ultrasound
 Bowel wall with central echogenic focus,
hypoechoic rim(pseudo-kidney)-necrotic
bowel and imminent perforation
 Intermittent gas bubbles in liver parenchyma,
portal venous system
 Free gas, focal fluid collection

26. Supportive investigations
 FBC(neutropenia, thrombocytopenia)
 Coagulation studies(evidence of DIC)
 Electrolytes (hyponatraemia, hyperglycaemia,
metabolic acidosis)
 CRP
 Septic workup
 Stool c/s, CD toxin
 Stool occult blood

27. Differential diagnosis
 Pneumatosis coli
 Infectious enterocolitis
 Intestinal obstruction secondary to hirschsprung,
ileal atresia, volvulus, meconium ileus,
intussusception
 SIP
 Anal fissures
 Neonatal appendicitis
 Cow’s milk protein allergy

28. Management
 Depends on severity of illness
 Medical management
 Surgical management

29. Medical management
 Supportive care-NBM, drip and suck, TPN,
fluid replacement, correction of hematological
and metabolic abnormalities,
cardiorespiratory support
 Antibiotic therapy
 Close monitering and radiologic
monitering(6-8 hrly)

30. Surgical intervention
 Perforation/severe peritonitis
 unremitting clinical deterioration despite
medical management suggesting extensive
necrosis
 presence of abdo mass, ascites or intestinal
obstruction
 Primary peritoneal drainage
 Laparotomy with bowel excision

31. Complications
 Acute: cardioresp, metabolic complications, DIC
 Late: GIT complications (short bowel syndrome,
intestinal strictures, increased frequency of bowel
movements)
 Rare: enterocele, enterocolic fistula, intra-abd
abscess
 Mortality
 Impaired Growth and neurodevelopmental outcome

32. Clinical presentation
SIP NEC
First week of life, median age 7(0-15) After first week,
Median age 15
Abdominal distension, bluish Abdominal distension
discoloration (groin, scrotum) Abdominal erythema
Hypotension Crepitus, induration
Pneumoperitoneum, gasless abdomen Pneumatosis intestinalis, portal venous
gas, transient thickening of intestinal
wall, fixed dilated SB loops,
pneumoperitoneum
Associated sepsis due to CONS, Concomitant bacteremia
fungemia
Leukocytosis, raised ALP, bilirubin, Thrombocytopenia,neutropenia, hypoNa,
decreased platelet, hct hyperglycaemia, metabolic acidosis