TextbookPaediatrics & Child HealthNecrotising Enterocolitis

Necrotising Enterocolitis

NEC is a devastating inflammatory bowel condition predominantly affecting preterm infants. It presents with abdominal distension, bloody stools, and feeding intolerance. Pneumatosis intestinalis on AXR is pathognomonic.

Key Facts

NEC affects approximately 5-10% of very low birth weight (<1500g) infants; mortality 20-30% (higher in surgical NEC) Pneumatosis intestinalis (intramural gas) on AXR is pathognomonic; portal venous gas indicates severe disease; pneumoperitoneum indicates perforation Bell staging: Stage I (suspected), Stage II (definite — pneumatosis), Stage III (advanced — perforation/shock) Risk factors: Prematurity (strongest), formula feeding, perinatal asphyxia, PDA, polycythaemia, umbilical catheterisation Breast milk is protective — reduces NEC incidence by ~50% (donor human milk if maternal not available) Management: Nil by mouth, NGT on free drainage, IV antibiotics (triple therapy), TPN, surgical consultation Surgery indicated for: Pneumoperitoneum (perforation), clinical deterioration despite medical treatment, fixed dilated bowel loop Probiotics reduce NEC incidence in preterm infants — increasingly used in NICUs (NICE IPG )

Overview

Key Facts

NEC is the most common surgical emergency in neonatal intensive care. It has high mortality and significant long-term morbidity. Prevention through breast milk feeding and probiotic supplementation is increasingly emphasised.

Epidemiology

NEC affects 5-10% of VLBW infants (<1500g). Overall incidence in NICU admissions is ~2-5%. Mortality is 20-30% overall and up to 50% in those requiring surgery. NEC is more common in formula-fed infants and those with PDA.

Aetiology

NEC is multifactorial:

  • Prematurity: Immature gut mucosal barrier, motility, and immune function
  • Enteral feeding: Formula feeding increases risk; breast milk is protective
  • Ischaemia: Perinatal asphyxia, low-flow states (PDA, cardiac defects)
  • Infection: Gut colonisation with pathogenic bacteria
  • Iatrogenic: Umbilical catheters, indometacin/ibuprofen for PDA

Pathophysiology

NEC results from a combination of gut mucosal immaturity, abnormal bacterial colonisation, and an exaggerated inflammatory response. The immature gut barrier allows bacterial translocation, triggering a pro-inflammatory cascade (TNF-α, IL-6, PAF) leading to mucosal necrosis. This progresses from superficial mucosal injury to full-thickness bowel wall necrosis, perforation, peritonitis, and septic shock.

Clinical Presentation

Clinical Features

  • Feeding intolerance: Increased gastric aspirates, vomiting (bile-stained)
  • Abdominal distension: Progressive; tender; erythema of abdominal wall (late)
  • Bloody stools: Frank blood or occult blood positive
  • Systemic signs: Apnoeas, bradycardias, temperature instability, lethargy, metabolic acidosis, hypotension, DIC

Bell Staging

  • Stage I (Suspected): Feeding intolerance, mild distension, occult blood in stool; non-specific systemic signs
  • Stage II (Definite): Gross blood in stool, marked distension, absent bowel sounds; pneumatosis on AXR
  • Stage III (Advanced): Perforation, peritonitis, shock, DIC; pneumoperitoneum on AXR

Red Flags

  • Fixed dilated bowel loop on serial AXR — suggests full-thickness necrosis
  • Portal venous gas — severe NEC
  • Pneumoperitoneum — perforation; needs urgent surgery
  • Rapidly worsening metabolic acidosis and thrombocytopenia — fulminant NEC
  • Abdominal wall erythema — transmural necrosis

Differential Diagnosis

DiagnosisKey FeaturesInvestigation
NECPreterm, distension, bloody stool, pneumatosisAXR, blood gas, FBC
Spontaneous intestinal perforationVery preterm, isolated perforation without NEC featuresAXR (free air but no pneumatosis)
Sepsis with ileusSystemically unwell, distension without pneumatosisBlood culture, AXR
Malrotation with volvulusBilious vomiting, acute obstructionUpper GI contrast study
Hirschsprung's enterocolitisDelayed meconium, chronic constipation, explosive diarrhoeaRectal biopsy
Cow's milk protein intoleranceBlood in stool, usually term infant, wellDietary exclusion trial

Diagnosis / Investigation

Bedside

  • Observations: HR, RR, SpO2, BP, temperature — sepsis screen
  • Abdominal examination: Distension, tenderness, erythema, absent bowel sounds

Bloods

  • FBC: Thrombocytopenia (poor prognosis indicator), neutropenia or neutrophilia
  • CRP: Elevated (serial monitoring)
  • Blood gas: Metabolic acidosis (raised lactate, low pH, low bicarbonate)
  • Blood culture: Before starting antibiotics
  • Coagulation: DIC screen (PT, APTT, fibrinogen, D-dimer)
  • U&Es, glucose: Metabolic derangement

Imaging

  • AXR (supine and lateral decubitus): Key investigation
    • Dilated bowel loops
    • Pneumatosis intestinalis (intramural gas — pathognomonic)
    • Portal venous gas (branching lucencies over liver)
    • Pneumoperitoneum (free air = perforation)
    • Fixed dilated loop on serial films (failed to change position)
  • USS abdomen: May show free fluid, thickened bowel wall, absent peristalsis, portal venous gas

Special Tests

  • Serial AXR: 6-12 hourly in acute phase — track progression
  • Stool culture: If infective cause suspected

Management

Medical (Stage I-II)

  • Nil by mouth: For 7-14 days depending on severity
  • NGT on free drainage: Decompress stomach
  • IV antibiotics: Triple therapy — per local protocol (e.g., amoxicillin/ampicillin + gentamicin + metronidazole) for 7-14 days
  • TPN: Nutritional support during bowel rest
  • IV fluids: Resuscitation with 0.9% NaCl boluses (10-20mL/kg)
  • Blood products: Platelets if <50, FFP if DIC, pRBC if anaemic
  • Inotropes: If cardiovascular compromise despite fluid resuscitation
  • Serial AXR: 6-12 hourly during acute phase

Surgical (Stage III or Failed Medical)

  • Indications: Pneumoperitoneum (perforation), clinical deterioration despite medical treatment, fixed dilated bowel loop, abdominal wall cellulitis
  • Procedures: Laparotomy with resection of necrotic bowel ± stoma formation; or primary peritoneal drainage (PD) in very small/unstable infants
  • Stoma reversal: Usually 6-12 weeks later when recovered

Prevention

  • Breast milk feeding: Reduces NEC by ~50% — maternal EBM or donor human milk
  • Probiotics: Growing evidence for NEC prevention in preterm infants (Lactobacillus, Bifidobacterium); used in many UK NICUs
  • Cautious enteral feeding advancement: Standardised feeding protocols reduce NEC incidence
  • Avoid unnecessary antibiotics: Prolonged empirical antibiotics increase NEC risk

Referral Criteria

  • All suspected NEC — neonatal surgical team consultation
  • Pneumoperitoneum — emergency surgery
  • Clinical deterioration despite medical management — surgical review

Prognosis

  • Overall mortality: 20-30%; higher in surgical NEC (~50%)
  • Short bowel syndrome: Affects ~10-25% of surgical survivors; may need long-term TPN
  • Strictures: ~20-30% post-NEC — may present weeks later with obstruction
  • Neurodevelopmental impairment: Increased risk compared with gestational age-matched controls without NEC
  • Recurrence: ~5-10% in medically managed NEC

Other Relevant Information

Bell Staging of NEC

StageClinicalRadiologicalManagement
IA (Suspected)Non-specific signs, mild distensionNormal or mild distensionNPO, antibiotics 48-72h
IBBright red blood PRNormal or mild distensionNPO, antibiotics 48-72h
IIA (Definite, mild)Moderate distension, absent bowel soundsPneumatosis intestinalisNPO, antibiotics 7-14d
IIB (Definite, moderate)Abdominal tenderness, acidosis, thrombocytopeniaPneumatosis + portal venous gasNPO, antibiotics, surgical consult
IIIA (Advanced, no perforation)Shock, DIC, peritonitisAs above, ascitesMedical + surgical standby
IIIB (Advanced, perforation)As IIIAPneumoperitoneumEmergency surgery

AXR Findings in NEC

FindingSignificance
Dilated bowel loopsEarly/non-specific
Pneumatosis intestinalisPathognomonic — intramural gas
Portal venous gasSevere NEC
PneumoperitoneumPerforation — surgical emergency
Fixed dilated loopFull-thickness necrosis
'Football sign'Free air outlining falciform ligament