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Necrotising Enterocolitis

Summary 

  • NEC (necrotising enterocolitis) is the most common GI emergency in neonates
  • NEC can present late in tiny babies
  • early or ‘possible’ NEC is difficult to diagnose – if in doubt treat early and conservatively (nil by mouth and broad spectrum antibiotics)
  • babies with ‘definite’ NEC should be referred to a NICU
  • babies with ileostomies
    • need supplemental sodium
    • can become rapidly dehydrated with gastroenteritis
  • strictures can present many weeks after NEC

Introduction

Necrotising enterocolitis (NEC) is the most common gastrointestinal (GI) emergency in neonates. Ninety percent of babies with NEC are preterm. It is predominantly a disease of the very low birthweight infant and is most common in babies < 1000 g or those that are both preterm and growth restricted. The incidence of NEC is inversely proportional to birthweight. In general, the age of onset is inversely proportional to gestation; therefore smaller babies present later.

Approximately 50% of babies developing NEC require surgery. The mortality rate of NEC is 20-40%. Of those who survive, approximately 25% develop long term sequelae.

Early or suspected NEC is often difficult to diagnose as the clinical signs and symptoms are often non-specific, as are the radiological and laboratory findings. As babies with definite NEC should be transferred to a level III unit, this topic will concentrate mainly on the presentation and diagnosis of NEC as well the level II management of a baby who has had previous NEC.

Risk factors

  • prematurity
  • enteral feeding (although approx. 10% of cases occur in infants never fed)
  • formula feeding (6 times more common than if only breast milk fed)
  • often occurs in clusters (although organisms vary)
  • bowel ischaemia
  • in term infants
    • polycythaemia
    • cardiac surgery
    • abdominal surgery (esp. gastroschisis, intestinal atresia)
    • endocrine abnormalities

Clinical Presentation

  • clinical signs and symptoms are highly variable but include
  • GI dysfunction
    • abdominal distention
    • vomiting
    • bilious drainage from enteral feeding tubes
    • blood in stool
  • systemic
    • temperature instability
    • apnoea and/or bradycardia
    • lethargy
    • hypotension
  • the severity, radiology and management of NEC is best exemplified by the ‘Modified Bell’s Staging Criteria’

Modified Bell’s Staging Criteria for Necrotizing Enterocolitis

 STAGE

SYSTEMIC SIGNS

 INTESTINAL SIGNS  RADIOLOGIC SIGNS  TREATMENT
I. Suspected             

A

Temperature instability, apnoea, bradycardia Elevated pregavage residuals, mild abdominal distension, occult blood in stool Normal or mild ileus NPO, antibiotics x 3 days

B

Same as IA Same as IA, plus gross blood in stool Same as IA Same as IA
II. Definite        

A: Mildly ill

Same as IA Same as I, plus absent bowel sounds, abdominal tenderness

Ileus, pneumatosis intestinalis

NPO, antibiotics x 7 to 10 days

B: Moderately ill

Same as I, plus mild metabolic acidosis, mild thrombocytopenia

Same as I, plus absent bowel sounds, definite abdominal tenderness, abdominal cellulitis, right lower quadrant mass

Same as IIA, plus portal vein gas, with or without ascites

 

NPO, antibiotics x 14 days

III Advanced        

A: Severely ill, bowel intact

Same as IIB, plus hypotension, bradycardia, respiratory acidosis, metabolic acidosis, disseminated intravascular coagulation, neutropenia

Same as I and II, plus signs of generalised peritonitis, marked tenderness and distension of abdomen.

Same as IIB, plus definite ascites

NPO, antibiotics x 14 days, fluid resuscitation, inotropic support, ventilator therapy, paracentesis

B: Severely ill: bowel perforated 

Same as IIIA

Same as IIIA

Same as IIB, plus pneumoperitoneum

Same as IIA, plus surgery

Get Acrobat ReaderNB: Permission to us the Modified Bell's Staging Criteria has been given by Paediatrics in Review.  The Modified Bell’s Staging Criteria  chart is available as a .pdf file for printing. If you do not have a copy of Acrobat Reader to view this file, the latest version is available for free download from www.adobe.com 

 Differential Diagnosis of suspected NEC

  • dysmotility of prematurity
  • septic ileus
  • bowel obstruction
  • gastroenteritis
  • anal fissure
  • cow’s milk protein sensitive enterocolitis

 Radiographic findings

  •  nonspecific
    • diffuse gaseous distension
    • asymmetric, disorganised bowel pattern
    • ‘featureless’ loops
    • dilated bowel loops
    • bowel wall thickening
    • increased peritoneal fluid
  • diagnostic signs
    • persistent loop
    • pneumatosis intestinalis (virtually pathognomonic)
      • submucosal – bubbly or cystic appearance (may be confused with stool, although stool usually moves on serial x-rays)
      • subserosal – linear or curvilinear appearance
    • portal venous gas
    • pneumoperitoneum (although may not be due to NEC)

 Management

  • see ‘Modified Bell’s Staging Criteria’ for duration
  • nil by mouth
  • gastric tube on free drainage
  • blood culture
  • antibiotics
    • vancomycin
    • gentamicin
    • metronidazole ( only for definite NEC)
  • cases of definite NEC should be referred to a level III NICU for management, as the following (may be) required
    • gut rest for 10-14 days
    • total parenteral nutrition
    • fluid management
    • inotropes
    • ventilation
    • analgesia
    • frequent radiographs
    • surgery (25% to 50% of cases)

 Complications 

  • surgery requiring ileostomy
    • require supplemental sodium even when well
    • high risk of rapid dehydration with gastroenteritis
  • stricture
    • 20-30%
    • most commonly in large bowel
    • 80% on left side
    • may not develop for weeks to months post-NEC
    • presents with recurrent abdominal distension
    • surgical consultation and contrast enema required

Prevention

  • antenatal corticosteroids
  • early intervention (nil orally) for suspected NEC
  • breast milk
  • infection control practices may limit the size of disease clusters

 Areas of uncertainty 

  • effect of rate of feed upgrade in the prevention of NEC
  • prophylactic antibiotics (proven to reduce NEC risk but concern re development of antibiotic-resistant organisms)
  • enteral IgA (enteral IgG refuted)

References 

Taeusch HW, Ballard RA. Avery’s Diseases of the Newborn 7th Ed. W.B. Saunders Company, Philadelphia. 1998

Chandler JC, Hebra A. Necrotizing enterocolitis in infants with very low birth weight. Sem Pediatr Surg 2000;9:63-72

Buonomo C. The radiology of necrotizing enterocolitis. Radiol Clinics North Amer 1999;37:1187-98

Other Reading

Neu J, Weiss MD. Necrotizing enterocolitis: Pathophysiology and prevention. J Parenteral Enteral Nutrition 1999;23:S13-7

Caplan MS, Jilling T. New concepts in necrotizing enterocolitis. Current opinion in Pediatrics 2001;13:111-5

 

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