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
| Diagnosis | Key Features | Investigation |
|---|---|---|
| NEC | Preterm, distension, bloody stool, pneumatosis | AXR, blood gas, FBC |
| Spontaneous intestinal perforation | Very preterm, isolated perforation without NEC features | AXR (free air but no pneumatosis) |
| Sepsis with ileus | Systemically unwell, distension without pneumatosis | Blood culture, AXR |
| Malrotation with volvulus | Bilious vomiting, acute obstruction | Upper GI contrast study |
| Hirschsprung's enterocolitis | Delayed meconium, chronic constipation, explosive diarrhoea | Rectal biopsy |
| Cow's milk protein intolerance | Blood in stool, usually term infant, well | Dietary 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
| Stage | Clinical | Radiological | Management |
|---|---|---|---|
| IA (Suspected) | Non-specific signs, mild distension | Normal or mild distension | NPO, antibiotics 48-72h |
| IB | Bright red blood PR | Normal or mild distension | NPO, antibiotics 48-72h |
| IIA (Definite, mild) | Moderate distension, absent bowel sounds | Pneumatosis intestinalis | NPO, antibiotics 7-14d |
| IIB (Definite, moderate) | Abdominal tenderness, acidosis, thrombocytopenia | Pneumatosis + portal venous gas | NPO, antibiotics, surgical consult |
| IIIA (Advanced, no perforation) | Shock, DIC, peritonitis | As above, ascites | Medical + surgical standby |
| IIIB (Advanced, perforation) | As IIIA | Pneumoperitoneum | Emergency surgery |
AXR Findings in NEC
| Finding | Significance |
|---|---|
| Dilated bowel loops | Early/non-specific |
| Pneumatosis intestinalis | Pathognomonic — intramural gas |
| Portal venous gas | Severe NEC |
| Pneumoperitoneum | Perforation — surgical emergency |
| Fixed dilated loop | Full-thickness necrosis |
| 'Football sign' | Free air outlining falciform ligament |