NECROTIZING ENTEROCOLITIS
Reproduction System 2010
OBJECTIVES
Ability to diagnose and treat the signs and symptoms of NEC
Ability to evaluate radiographs for the classic findings of NEC List several long-term complications associated with NEC
NECROTIZING ENTEROCOLITIS
Epidemiology:
most commonly occurring gastrointestinal emergency in preterm infants leading cause of emergency surgery in neonates overall incidence: 1-5% in most NICUs most common in VLBW preterm infants
10% of all cases occur in term infants
NECROTIZING ENTEROCOLITIS
Epidemiology:
10x more likely to occur in infants who have been fed males = females blacks > whites mortality rate: 25-30% 50% of survivors experience long-term sequelae
NECROTIZING ENTEROCOLITIS
Pathology:
most commonly involved areas: terminal ileum and proximal colon GROSS:
bowel appears irregularly dilated with hemorrhagic or ischemic areas of frank necrosis
focal or diffuse
MICROSCOPIC:
mucosal edema, hemorrhage and ulceration
NECROTIZING ENTEROCOLITIS
MICROSCOPIC:
minimal inflammation during the acute phase
increases during revascularization
granulation tissue and fibrosis develop
stricture formation
microthrombi in mesenteric arterioles and venules
NECROTIZING ENTEROCOLITIS
Pathophysiology:
UNKNOWN CAUSE.
From UpToDate online, Adapted from Kliegman, RM, Pediatr Res 1993; 34:701.
RISK FACTORS
Prematurity:
* primary risk factor 90% of cases are premature infants immature gastrointestinal system
mucosal barrier poor motility
immature immune response impaired circulatory dynamics
RISK FACTORS
Infectious Agents:
usually occurs in clustered epidemics normal intestinal flora
E. coli Klebsiella spp. Pseudomonas spp. Clostridium difficile Staph. Epi Viruses
RISK FACTORS
Inflammatory Mediators:
involved in the development of intestinal injury and systemic side effects
neutropenia, thrombocytopenia, acidosis, hypotension
primary factors
Tumor necrosis factor (TNF) Platelet activating factor (PAF) LTC4 Interleukin 1& 6
RISK FACTORS
Circulatory Instability:
Hypoxic-ischemic injury
poor blood flow to the mesenteric vessels local rebound hyperemia with re-perfusion production of O2 radicals
Polycythemia
increased viscosity causing decreased blood flow exchange transfusion
RISK FACTORS
Enteral Feedings:
> 90% of infants with NEC have been fed provides a source for H2 production hyperosmolar formula/medications aggressive feedings
too much volume rate of increase
>20cc/kg/day
RISK FACTORS
Enteral Feedings:
immature mucosal function
malabsorption
breast milk may have a protective effect
IGA macrophages, lymphocytes complement components lysozyme, lactoferrin acetylhydrolase
CLINICAL PRESENTATION
Gestational age:
< 30 wks 31-33 wks > 34 wks Full term
Age at diagnosis:
20 days 11 days 5.5 days 3 days
*Time of onset is inversely related to gestational age/birthweight
CLINICAL PRESENTATION
Gastrointestinal:
Feeding intolerance Abdominal distention Abdominal tenderness Emesis Occult/gross blood in stool Abdominal mass Erythema of abdominal wall
Systemic
Lethargy Apnea/respiratory distress Temperature instability Hypotension Acidosis Glucose instability DIC Positive blood cultures
CLINICAL PRESENTATION
Sudden Onset:
Full term or preterm infants Acute catastrophic deterioration Respiratory decompensation Shock/acidosis Marked abdominal distension Positive blood culture
Insidious Onset:
Usually preterm Evolves during 1-2 days Feeding intolerance Change in stool pattern Intermittent abdominal distention Occult blood in stools
Modified Bell Staging for NEC
Stage & Severity Stage Ia Suspected NEC Stage Ib Suspected NEC Stage IIa Definite Mild NEC Stage IIb Definite Moderate NEC Stage IIIa Advanced, Severe NEC Bowel Intact Stage IIIb Advanced Severe NEC Bowel Perforated Systemic Signs Temp changes, apnea, bradycardia, lethargy Same as Ia Abdominal Signs Distension, gastric retention, emesis, heme positive stool Ia + grossly bloody stool Ib + absent bowel sounds +/- abdominal tenderness IIa + definite tenderness, +/- abd cellulitis, RLQ mass IIb + peritonitis, marked distension and tenderness Same as IIIa Radiographic Signs Normal, or intestinal dilation Mild ileus Same as Ia
Same as Ia
Intestinal dilation, ileus, pneumatosis intestinalis IIa + ascites
Ia + mild metabolic acidosis, thrombocytopenia IIb, but more severe, + combined respiratory & metabolic acidosis, neutropenia, & DIC Same as IIb
Same as IIb
IIIa + pneumoperitoneum
Adapted from sources showing Bell Staging
BELL STAGING CRITERIA
STAGE I. Suspect NEC II. Definite NEC CLINICAL
Mild abdominal distention Poor feeding Emesis The above, plus Marked abdominal distention GI bleeding
X-RAY TREATMENT
Mild ileus Medical Work up for Sepsis
Significant Ileus Pneumatosis Intestinalis PVG PneumoPeritoneum
Medical
III. Advanced NEC
The above, plus Unstable vital signs Septic Shock
Surgical
RADIOLOGICAL FINDINGS
Pneumatosis Intestinalis
hydrogen gas within the bowel wall
product of bacterial metabolism
a. linear streaking pattern
more diagnostic
b. bubbly pattern
appears like retained meconium less specific
Abdominal Distension
Severe Abdominal Distension
Pneumatosis Intestinalis
Image from LearningRadiology.com
RADIOLOGICAL FINDINGS
Portal Venous Gas
extension of pneumatosis intestinalis into the portal venous circulation
linear branching lucencies overlying the liver and extending to the periphery associated with severe disease and high mortality
RADIOLOGICAL FINDINGS
Pneumoperitoneum
free air in the peritoneal cavity secondary to perforation
falciform ligament may be outlined
football sign
surgical emergency
Pneumatosis Intestinalis
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Pneumatosis & Dilated Loops
Pneumatosis
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Dilated loops & Portal Air
Portal Air
Dilated stomach & loops of bowel
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A Bad Case of NEC
Portal Air
Abdomin al free air
Pneumatosis
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LABORATORY FINDINGS
CBC
neutropenia/elevated WBC thrombocytopenia
Acidosis
metabolic
Hyperkalemia
increased secondary to release from necrotic tissue
LABORATORY FINDINGS
DIC Positive cultures
blood CSF urine stool
Neonatal NEC Pathology
Pneumatosis
Necrosis
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Prevention
Encourage breast feeding
Breast fed babies have lower incidence than formula fed
No evidence shows that late initiation of enteral feeding or slow rate of feeding makes any difference Maintain high level of suspicion
Feeding babies with NEC worsens the disease
TREATMENT
Stop enteral feeds
re-start or increase IVF
Nasogastric decompression
low intermittent suction
Antibiotics
Medical Treatment
Stage Ia
NPO x 3 days
Stage Ib - IIb/IIIa
NPO Broad spectrum antibiotics
Cover Gram +, Gram - & Anaerobes Ib = 3 days, IIa = 7-10 d, IIb & up = 14 d
Follow x-ray for resolution
Resume enteral feeds 10-14 days after radiographic resolution
May require paracentesis if IIIa
Surgical Treatment
Stage IIIa - IIIb
Laparotomy
Resection of necrotic bowel Ileostomy with mucous fistula
Subsequent re-anastamosis
May result in strictures requiring further surgery later
Peritoneal drain
Placement in NICU under local anesthetic Used when infant is too clinically unstable for surgery May help stabilize pt for subsequent surgery
TREATMENT
Labs: q6-8hrs
CBC, electrolytes, DIC panel, blood gases
X-rays: q6-8hrs
AP, left lateral decubitus or cross-table lateral
Supportive Therapy
fluids, blood products, pressors, mechanical ventilation
PROGNOSIS
Depends on the severity of the illness Associated with late complications
* strictures short-gut syndrome malabsorption fistulas abscess
* MOST COMMON
Outcomes
Mortality varies with birth weight:
<1000 g = 40-100% <1500 g = 10-44% >2500 g = 0-20%
Morbidity/Mortality vary with severity:
Resection -> Short gut -> FTT, malabsorbtion Strictures -> further surgery in medical and surgical NEC Prolonged NPO status on TPN -> cholestasis & metabolic abnormalities
