Necrotizing Enterocolitis (NEC) Case presentaion By Dr. ASHRAF HAMED prof. Pediatric Surgery Al Azhar university
Case presentationMALE saudi baby ,5days old ,preterm(35weeks gestational age)with history of PROM for >48 hour,referred from El zulfi general hospital as a case of sepsis with ?NEC.There was history of bleeding per rectum since second day of life and oliguria ,as the baby passed only 5 ml urine in last 24 hour.
O/E 19-4-24 at 3pm The baby looks severly ill,dehydrated,toxic facies & poor perfusion B W:2.4Kgm Unstable vital signs: low BP,pulse:170/min,temp:38.5 The abdomen was severly distended with erythema of abdominal wall. Generalized tenderness. Absent bowel sound (died silent)
CONT. The baby admitted in NICU and immediately started aggressive resuscitations with IV fluids and antibiotics(claforan,flagyl infusion and vancomycin),NGT decomression of the stomach
Case presentationWe inserted penrose sheet drain at right lower quadrant under local anaesthesia which brought air and faecal matter to give chance for improvement of general condition,meanwhile packed rbcs, platelets and FFP were given with continous IV fluids resuscitation.
20/4 at 10:00 a.m.Pt submitted for laparotomy and we found:Sever faecal peritonitis and amulgmation of intestine with pyogenic membrane covering it.we discovered oedema and hyperaemia of the descending colon and big necrotic segment in the sigmoid colon up to level of peritoneal reflection over the rectum.
Also there were 2 yellowish dirty, non viable patches, but not yet perforated 1st one 15 cm from DJF and the 2nd 15 cm distal to the 1st one which were Imbricated with interrupted lambert sutures with 5/0 viryl .Lt hemicolectomy done with hartman’s procedure closure of distal stump and the proximal end as a terminal colostomy .
at 7:30 pm urine output was 5 ml, with appearance of sclermatous skin changes & muscle stiffness .21/4 at 9:00 a.m.The whole body got oedematous and baby gained 300 gm post- operatively due to renal shut down as only 5 ml urine passed.- Signs of fulminant sepsis in the form of persistant hypotension, poor perfusion and scleroderma that carrying out bad prognosis. C.B.G. also showed mixed acidosis on high ventilation sitting.
case diagnosed as acute renal failure so, managed by:--Lasix I.V. 5 mg / kg state- D/C amikine- Adjust dose of vancomycin and cefotaxim serum urea 16.7 mmol/l serum.Creatinine 147 mmol.T .protein 23&albumin 12albumin 20% 1 gm 1kg over 1 hour given stat then lasix as before9:00 pm pt passed 31 cc clear urine after that ttt with progressive improvement of renal function and urine output.
2 4/4Development of purpura fulminans ,ulceration, oral,ETT bleeding with platelets 10,ooo but pt passed blackish stool from stoma.28/4Sub- cutaneous wound collection without burst abdomen.faccal matter coming from the wound with sluggish instestinal sounds but x-ray abdomen ----- no pneumoperitoneum so, 2nd laparatomy done
The finding of 2nd laparotomy (29-4-24)The previous two yellowish dirty non viablepatches in the jejuneum were perforated so,refreshment of the edges and repair with 5/0vicryl single layer interrupted for theproximal one and for the distal one we took itsproximal end as a jejunostomy and the distalend closed. (jejunostomy at Rt iliac fossa)
Gastrograffin follow through done and showed no leakage of the dye with healing of the proximal perforating.Then we started oral feeding through N.G.T. and every now and then correction of anaemia and thrombocytopenia.
The pt became deeply jaundiced with elevated liver enzymes, ⇑ total and direct bilirubin and his body weight decrease to 1.9 kg as stoma extrude the milk undigested after feeding through N.G.T., so, at 1st we changed to N.G.T. drip method.There is little increase of body weight to 2 kg.So, we started T.P.N. through Rt subclavian veiv(cut doun) when all other substitutes failed. Also, with little improvement.
CASE PRESENTION2/6/24So,we decided to close the jejunostomy.colostomy became functioning after 2 days postoperatively . also, we stopped T.P.N. because of increasing liver enzymes.Then patient tolerated gradually NGT feeding and passed post operative smoothly till discharged at 26/6/24 and 2 months later the patient came for closure of colostomy
Nectotizing EnterocolitisIntroductionit is the most common newbornsurgical emergency, with a mortalityrate that far exceeds that of any othergastrointestinal condition requiringoperation.It is a syndrome of acute intestinalnecrosis.NEC is a disease of paradoxes. Ittypically affect the preterm infant but
Nectotizing Enterocolitis It usually occurs on the tenth day of life, but it may develop on the first day, several weeks, or even months after birth. The disease frequently appears sporadically but can present in epidemic – like clusters. Most patients who develop the disease were fed enterally, but babies who have never been fed also are susceptible.
Nectotizing Enterocolitis N.E.C. is widely accepted as a complication of prematurity in which there is intestinal hypoxia, usually due to a low perfusion state which results in mucosal injury and allows translocation of bacteria into the intestinal wall and portal venous system. Two-Thirds of patients respond to medical management, only about one- third require surgical intervention.
EpidemiologyI – Incidence Overall incidence of NEC is 2-5% of all NICU admissions. The incidence of NEC is approximately 1-3 cases/1000 live births in the u.s. or about 25,000 new cases of NEC worldwide / year. The incidence is much higher in premature (90-93%)and extreme low birth weight infants.
Epidemiology Incidence varies between hospitals and also within the same institution in different periods reflecting periodic epidemics It occurs more commonly among infants fed formula( 9o to 95% enterely fed)compared to those who have been fed with breast milk.
Epidemiology Mean age of onset is 3-4 days for term infants and 3-4 weeks for infant born at less than 28 week’s gestation. Mortality rate varies(30-40%) with a higher rate in advanced NEC (stage III or with perforation) and in infants with birth weight 1000gm or less( >80%).
CIRCULATORY INSTABILITYPRIMARY INFECTIOUS AGENTS Hypoxic-ischemic eventBacteria, Bacterial toxin, Virus, Fungus Polycythemia MUCOSAL INJURYINFLAMMATORYMEDIATORS ENTERAL FEEDINGSInflammatory cells (macrophage) Hypertonic formula or medicationPlatelet activating factor (PAF) Malabsorption, gaseous distentionTumor necrosis factor (TNF) H2 gas production, EndotoxinLeukotriene C4, Interleukin 1; 6 production
EpidemiologyII Risk FactorsDespite several decades of research theetiology and pathogenesis remainedelusive.There is wide controversy about thistopic .
Epidemiology .Prematurity The most dominant risk factor for NEC is the degree of Immaturity. – 90% of cases are premature infants – immature gastrointestinal system mucosal barrier poor motility – immature immune response – impaired circulatory dynamics
Epidemiology 2 –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 >20kcal/kg/day 3 – Infant related conditions A. Perinatal asphyxia & hypoxic ischaemia It has more significant role in term and near term infants.
EpidemiologyB – Umbilical Catheterization-Embolization of catheters may result inembolization of mesenteric arteries.-Infusion of medications such as calciummay cause vasospasm and frankintestinal necrosis.C - Congenital heart disease EspeciallyPDA and cyanotic heart disease.
EpidemiologyD-– Extreme Low birth weightIt was proved that the incidence of NEC is higher in those babies with birth weight 1500 gm or less.E -DrugsI) Theophylline→ toxic oxygen radiclesII) Oral Vitamin E→interfere with killing of bacteriaIII) Indomethacin→vasoconstriction→↓mesenteric blood flow
EpidemiologyF. Infection- Although no specific single organism was implicated in the diseaseG. Hypotension & ShockH. PolycythemiaI Exchange TransfusionJ ThrombocytosisK AnaemiaL RDS
Pathogenesis Most investigators suggested that NEC resulted from intestinal mucosal injury from low - Flow states, or hypoxia, with secondary bacterial invasion. These factors may initiate the disease process by injuring the protective barriers of the intestine (The mucosa). The injury can be direct or indirect. Direct injuries are caused by bacteria or by exposure to hypertonic solution.
PathogenesisIndirect injuries are the result of mucosalcell hypoxia from low flow states: .Shock .Hyperviscosity .Vascular obstruction Or generalized hypoxia ⇒ birth asphyoxia →lung or heart disease
Pathogenesis* Once the mucosa has been injured,bacteria in the lumen can breach the gut barrierAnd initiate an inflammatory cascade, causingfurther damage and eventual intestinalnecrosis. The presence of substrate provided byFeedings, bowel stasis and reduction indefences of the local gut Mucosa ⇒ Allfacilitate bacterial proliferation.
PathologyNEC may beI. FOCAL(Isolated) diseaseWhen a single area of bowel is necrotic or perforatedII.MULTIFOCAL Multi – segmental disease (> 50% viable.).The most common site of NEC is the terminal ileum, the second most common site in the left colon..The disease can occur anywhere from the stomach to the rectum.
PathologyInvolvement of both the large and small intestine occurs in 44% of cases.III. Pan – Involvement (NEC totalis) Account for 19% of the cases Characterized by necrosis of at least 75% of the gut.
Macroscopic Features Grossly distended loops of the intestine with spotty intramural haemorrhagl and areas of necrosis with serosal gas collection. The diseased bowel wall may be thinned with fibrinous exudate covering the serosal surface. Mucosal ulceration with associated epithelial sloughing may be extensive.
Microscopic Features The essential feature are haemorrhage and necrosis (coagulation necrosis). Mucosal oedema followed with formation of a pseudo-membrane of fibrinous exudates and necrotic cellular debris. The necrosis may progressively involve all layers of the bowel to the serosa.
ComplicationsShort Term1 – Perforation.2 – Septicaema.3 – Metabolic and electrolyte distubance.4 – Prolonged need for artificial ventilation with its hazards.
II – Long Term1. Strictures - It is the most common long term GIT complication of NEC, occuring in (10-35%) of all survivors.2 – Short bowel syndrome The most important determinant of future GIT function is the absence or presence of ileocecal valve, regardless of the length of gut resected. The syndrome refers to malabsorption and under nutrition after extensive bowel resection.
3.FULMINANT SEPSIS4.TPN related complications:Cholestasis;Thrombosis;Infection5. Complications related to stoma:Retraction,Prolapse&Parastomal hernia6. adverse neurodevelopmental outcomesDelays in “locomotor,” “hearing and speech,” “intellectual performance” and “personal and social” skills.[
6. Wound complications Infection, Dehiscence or Enterocutaneous fistula7. RecurrenceOccurred in 10% (Rennie and Roberton 2002)8. Others:Chronic anaemia & malabsorptionEye complicationAbscess & Fistula formation
CLINICAL PRESENTATIONGastrointestinal: Systemic• Feeding intolerance • Lethargy• Abdominal distention • Apnea/respiratory• Abdominal tenderness distress• Emesis • Temperature instability• Occult/gross blood in • Hypotension stool • Acidosis• Abdominal mass • DIC• Erythema of abdominal • Positive blood cultures wall
CLINICAL PRESENTATIONSudden Onset: Insidious Onset: Full term or preterm Usually preterm infants Acute catastrophic Evolves during 1-2 deterioration days Respiratory Feeding intolerance decompensation Shock/acidosis Change in stool Marked abdominal pattern distension Intermittent Positive blood culture abdominal distention Occult blood in stools
BELL STAGING CRITERIASTAGE CLINICAL X-RAY TREATMENTI. Suspect Mild abdominal Mild ileus Medical distention Work up for NEC Poor feeding Sepsis EmesisII. Definite The above, plus Significant Medical Marked abdominal Ileus NEC distention Pneumatosis GI bleeding Intestinalis PVGIII. Advanced The above, plus Pneumo- Surgical Unstable vital signs Peritoneum NEC Septic Shock
Modified Bell StagesStage Stage Systemic Intestinal Radiolog Signs Signs ical Sign I IA Temperature −⇑Gastric .IntestinalSuspected instability aspirate .mild dilutation NEC Apnea abdominal .Mild ileus Bradycardic distension Lethergy .Emesis .Faecal occult Blood I IB Same as IA Bright red blood per Same as IASuspected rectum NEC
Modified Bell StagesStage Stage Systemic Intestinal Radiologic Signs Signs al Sign II II A Same as IA Same as IB + •Intestinal Definite (mildly ill) .absent bowel dilatation NEC sound •Ileus .Abd. Tenderness •Pneumatosis intestinalis II II B Same as II A plus: Same as II A plus Same as II A Definite (moderat *Metabolic Acidosis *Defenite Abd. plus NEC ely Ill) *Mild Distension +portal vein Thrombocytopenia +Abd. Celluitis gas + Lower Abd. + Ascites Quadrant mass *Absent bowel sound
Modified Bell StagesStage Stage Systemic Signs Intestinal Radiologi Signs cal Sign III III A Same as II B plus Same as II B Same as II BAdvanced (Severely Ill) * Hypotension plus plus NEC * Bradycardia *Generalized * Definite * Severe Apnea Peritonitis Ascites * Combined respiratory *Marked &metabolic acidosis Abd.Tenderness * DIC *Abd. * Neutropenia and Distension * Anuria *abd Wall Erythema III III B Same as III A plus Same as III A Same as III AAdvanced (Severely ill sudden perforation plus plus & bowel Increased pneumoperito perforation) Distension neum
IMAGING The cornerstone of the diagnosis of NEC is plain anteroposterior and left lateral decubitus radiography.1. Bowel distensionIt is the earliest and most common radiologic finding in patients with NEC (55% to 100% of cases)2. Pneumatosis intestinalis• It is intramural gas (mainly hydrogen)• The frequency ranges from19% to 98%.
Pneumatosis Intestinalis hydrogen gas within the bowel wall A by-product of bacterial metabolism a. linear streaking pattern represents subserosal air more diagnostic b. bubbly (cystic form)pattern o More common o . represent submucosal air o appears like retained meconium o less specific
3. Portal venous gas• It appears as linear branching arborizing pattern of air over the liver shadow.• It may be fleeting and accounting for 9% to 20%. Portal vein gas is associated with poor prognosis.4. Pneumoperitoneum• It is best noted in left lateral decubitus• Pneumoperitoneum may occur without intestinal perforetion (barotraume).
5. Intraperitoneal fluid• It is assoicated with high mortality rate.• Amenable to paracentesis6. Persistent dilated loops* When a single loop or sevseral loops of dilated bowel remained unchanged in position and configuration for 24 to 36 hours usually occurs in full-thickness necrosis.
Contrast Study It may be used in patients with equivocal radiologic signs Barrium should never be used Newer water – soluble agents as isotonic metrizamide which produce excellent specification of the gut.
Ultra- SonographyIt has been used to identifya. Necrotic bowelb. Intraperitoneal fluidc. Portal venous gas• It is the most applicable tool in patient with gasless abdomen and to localize intra-abdominal fluid for paracentesis.
MANAGEMENTNon-operativeThe mainstay of treatment for NEC is non-operative therapy.1. NPO & gastric suction ⇒ 10 – 14 days2. Cultures:- Blood culture is essential - CSF, Urine, Other Sites cultured as indicated.3. Antibiotics:- Vancomycin Aminoglycoside Flagyl Infusion for at least Cephalosposine 2 weeks
4. Intravenous fluids: 150 – 250 ml / kg5. TPN6. Blood and Blood products: as needed forcorrection of anaemia and coagulopathy.7. Close Clinical observation ⇒ consists of:a.Frequent physical examinationb.Abdominal radiography every 8 hoursc.C.B.C. Blood gas analysis, serum electrolyte & serum platelet
Indications for operation1. Pneumoperitoneum• This is the only absolute indication for surgery.• Relative indication:2. clinical deterioration despite adequate therapy: a. Erythema and oedema of abdominal wall b. Abdominal mass c. Signs of peritonitis on physical examination d. Increasing acidosis and e. Persistent and progressive thrombocytopenia.
3. Fixed dilated intestinal loop.4. Ascites• 43% of patient’s ascites will have bowel necrosis.• The demonstration of ascites mandates paracentesis .
Paracentesis5. Positive Paracentesis– Indicated for infants with extensive pneumatosis intestinalis or who have failed to improve on medical management– This is defined as free flowing aspiration of more than 0.5 ml of brown or yellow brown fluid that contains bacteria on gram stain.6.Portal Venous Gas
Operative Managementbed-side Peritoneal drainage* It is insertion of a penrose drain through a right lower quadrant incision under local anaesthesia for extremely ill infants with bowel perforation.
Laparoscopy�Clarckand Mackinaly reported the use of laparoscopy in the treatment of a VLBW infant (900 g) with perforated NEC.Tan et al.:4 babies (500-1000 g) Needlescopic diagnosis is feasible and appears to be safe, even in critically ill baby less than 1000 g. The technique can provide useful information for surgical decision-making and allows for precise placement of the incision over the site of perforation, thus minimizing the trauma from open surgery in this special group of patients. Clark C, MackinlayGA. Laparoscopy as an adjunct to peritoneal drainage in perforated necrotizing enterocolitis. J Laparoendosc Adv Surg Tech A. 2006 Aug;16(4):411-3.39 Tan HL et al. The role of diagnostic laparoscopy in micropremmieswith suspected necrotizing enterocolitis. Surg Endosc. 2007 Mar;21(3):485-7.
II. Principles of ResectionA. When a single area of bowel is necrotic or perforated only limited resection is necessary.• A proximal ostomy and distal mucus fistula are createdB. Resection with primary anastomosis in these conditions i) a sharply localized (proximal)segment of disease ii) undamaged appearance of the remaining intestine. iii) good general condition.
Segmental NECContrast study Segmenal necrosis Multiple segments necrotic of one segment but >50%viable bowel Resection of Resection of necrotic segment necrotic segments Primary stoma Proximal stoma anastomosis!? Muliple anastomoses of distal Contrast defunctionalized bowel study Stoma closure 4-6 weeks
III. Multi – segmental disease( > 50% viable)A. Excision of each diseased segment and creates multiple stomas.B. “Patch, drain, and wait” procedure; which include- Transverse single layer suture approximation of perforations (patch).- Insertion of two penrose drains (drain)- Long-term TPN (wait)
C. “Clip and drop-back” Technique• Obviously necrotic bowel is removed.• The cut ends are closed with titanium clips.• Re-exploration is done 48 to 72 hours later ⇒ All segments are re-anastomosed without any stoma.
Pan – Involvement = Nec Totalis Operation 1.high stoma in proximal viable bowel 2.irrigate distal bowel Perforated No segments perforations No resection Mucus fistula of distal bowel Limited resectionsMucs fistula Muliple Stent deteriorate stabledistal bowel stomas Muliple segmnts over silastic cather TPN for 6-8 weeks Contrast study Multiple resections of scarred segments ananastomosis. Proximal stoma remains Proximal stoma closure in 6 weeks
IV. Pan – Involvement = Nec TotalisThe Treatment options1. Simple closure of the abdomen2. Resection of all necrotic bowel3. Proximal diversion without bowel resection…..second-look operation after 6- 8weeks.