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Spontaneous intestinal perforation vs NEC:What is the difference??
Dr Varsha Atul Shah
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
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
Risk factors
Prematurity Antenatal1. Severe placental chorioamnionitis*2. ? Glucocorticoids/NSAIDS Postnatal1. Early postnatal glucocorticoids@2. ? 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 setAttridge JT; Clark R; Gordon PV J Perinatol. 2006 Nov;26(11):667-70. Epub 2006 Oct 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
Clinical presentation
SIP NEC
First week of life, median age 7(0-15)
After first week,
Median age 15
Abdominal distension, bluish discoloration(groin, scrotum)
Hypotension
Abdominal distension
Abdominal erythema
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, fungemia
Leukocytosis, raised ALP, bilirubin, decreased platelet, hct
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
Management
NBM, drip and suck Fluid resuscitation Intravenous antibiotics Surgical treatment1. Definitive treatment2. Exploratory laparotomy with bowel
resection3. Primary peritoneal drainage
Primary peritoneal drainage
Avoids laparotomy, need for GA and transport Many do recover without any further surgical
intervention Laparotomy indicated if:1. Reaccumulation of free air after drain removed,
indicating perforated bowel did not seal2. Fistula with intestinal drainage that fails to close3. Bowel obstruction secondary to adhesions or
stricture at site of perforation
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.
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.
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
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 host1. Prematurity2. Microbial bowel overgrowth3. Milk feeding4. Impaired mucosal defense5. Circulatory instability of intestinal tract6. Medications7. CHD, perinatal asphyxia, polycythemia, sepsis, respiratory
disease8. Inflammation
Clinical presentation
Timing of onset of symptoms varies, inversely related to GA
Systemic signs
1. Nonspecific (apnea, resp failure, lethargy, poor feeding, temp instability)
2. Hypotension Abdominal signs
- distension, gastric retention, tenderness, vomiting, diarrhoea, rectal bleeding, bilious aspirates
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
Bells staging
Diagnosis
Clinical Radiologic findings
Clinical
Abdominal distension Rectal bleeding
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
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
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
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
Management
Depends on severity of illness Medical management Surgical management
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)
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
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
Clinical presentation
SIP NEC
First week of life, median age 7(0-15) After first week,
Median age 15
Abdominal distension, bluish discoloration (groin, scrotum)
Hypotension
Abdominal distension
Abdominal erythema
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, fungemia
Concomitant bacteremia
Leukocytosis, raised ALP, bilirubin, decreased platelet, hct
Thrombocytopenia,neutropenia, hypoNa, hyperglycaemia, metabolic acidosis
Recommended