Abdominal Emergencies in Children NEC, Haemorrhagic Disease of Newborn, Perforations Sharon Cox Red Cross War Memorial Children’s Hospital and University of Cape Town

NEC Incidence At risk neonates Premature Stressed Diagnosis Clinical: Distension, vomiting Sepsis Radiology

NEC Medical management Full ICU support IVI antibiotics NPO, NGT TPN Vit K Refer surgery

Why Operate? Improve patient condition Remove ischemic/ necrotic bowel and spilled intraperitoneal fluid, stool 30 – 50% Keep complications low How to achieve this is controversial and lacking good level of evidence

What is the evidence?  Incidence Mortality Higher: selection bias Multiple studies over 3 decades, majority small case series Conflicting results Recent breakdown of institutional and geographic barriers, investigators working together Multi-center RCT commenced and results starting to be evaluated

Indications Absolute Perforation: free air on AXR Intestinal Stricture Relative Fixed intestinal loops on AXR Palpabable mass Abdominal wall erythema Failure of medical treatment* Portal venous gas* Thrombocytopaenia Necrotizing enterocolitis — bench to bedside: novel and emerging strategiesYigit S. Guner, MD, Nikunj et al Seminars in Pediatric Surgery (2008) 17, 255-265 .

Perforation - Free Air No dispute since early 70’s Only universally accepted absolute indication Indication in 30 to 40% of cases Will never be a trial/evidence But: Pneumoperitoneum is not always demonstrable on AXR 50% to 75% of patients with perforation overall specificity for pneumoperitoneum was 92% and positive predictive value, 88% newer techniques are being evaluated - ultrasound

Positive Paracentesis Kosloske 1982: Fluid for bile, bacteria – Good indicator of gangrenous bowel Ricketts et al reported positive Paracentesis (>0.5 ml of flee-flowing brownish fluid and/or positive for bacteria on Gram stain) as an indication for operation Paracentesis can be used to aid decision-making in neonates who don’t meet absolute criteria for surgery False negatives described: sealed perforation

Fixed loop Persistent loop (>24hrs) on serial X Rays Specificity and PPV for gangrenous bowel close to 100% Low prevalence O’Neill, Grossfelt 1998; Caty, Azizkhan 2000 50% recover without undergoing surgery Pneumatosis intestinalis. Loren G Yamamoto, Kapiolani Medical Center for Women & Children, University of Hawaii

Palpable Mass Is cited as evidence of localized perforation with abscess formation Specificity and PPV for gangrenous bowel close to 100% Prevalence less than 10% Limited use Indication for surgery if associated with continued sepsis or obstruction (Stringer, Spitz 1993)

Abdominal Wall Erythema Indication for surgery if associated with continued sepsis or obstruction Specificity and PPV for gangrenous bowel almost 100% Prevalence less than 10% Subjective Limited use © www.medscape.com

Portal venous Gas Assoc with poor prognosis in many studies Conclusion: “Should be considered operative indication” Not universally accepted Rowe et al 1998, 90% with PVG will develop necrosis, 50% will have pan intestinal involvement Sharma and Tepas et al* 30/64 (47%) survived without surgery * Sharma R, Tepas JJ et al; Portal Venous Gas and Surgical Outcome of Neonatal NEC. J Pediatr Surg 40: 371 – 376 2005

Clinical Deterioration Deterioration despite adequate medical therapy has often been used as a broad term to indicate likely intestinal gangrene and has been cited as the commonest indication (59%) for surgery Broad term, no statistics to confirm

Thrombocytopaenia Sudden platelet drop is considered indication of gangrenous bowel Ververidids et al. 2001 – greater extent of disease, lower platelets Severe thrombocytopaenia: sens: 69%, spec: 60% PPV: 89% - predict gangrene Rapid Platelet fall sens: 32%, spec: 89% PPV 92% Therefore, thrombocytopaenia cannot predict extent of disease or predict need for lap

Stricture >30% NEC patients and >50% of surgically managed patients will develop stricture Post-NEC strictures are believed to occur as a consequence of scarring after ischemic intestinal injury No specific criteria can be identified to predict at risk patients Evidenced by feed intolerance, abdo distension, vomiting, bradycardia and apneas Most in colon, sigmoid Routine imaging of distal bowel prior to closure of stoma is suggested Source: Appl Radiol Online © 2003 Anderson Publishing. Ltd.

Optimal timing Optimal timing After severe ischaemia and demarcation Before perforation Avoid unnecessary surgery: increased loss of bowel due to lack of demarcation Prevent complications of advanced intraperitoneal sepsis Identifying this window is a challenge (imaging? Biological markers?)

New Techniques Colour Doppler and standard US imaging of bowel offer a useful non invasive method to evaluate intestinal blood flow Abdominal US is can delineate free air, intramural gas, portal venous gas, fluid collections, bowel wall thinning/thickening, increased bowel wall echogenicity, and perfusion One factor limiting it’s expansion may be the timely availability of a radiologist

Summary Absolute: free air Relative: Evidence scanty – need critical evaluation 3 decades after initial description NEC remains a challenge to the Paediatric Surgeon, optimal management continues to generate debate Clear and deliberate educated judgment on individual patient basis

Management Percutaneous drainage Surgical exploration Resection Glove drain Urine bag Local and adrenaline Temporary measure Surgical exploration Resection Primary anastomosis Stoma formation Laparoscopic assessment – Fluorescin and UV light Continued ICU support TPN

Surgical Management Resect and anastomosis Conservative Diverting stoma Clip and drop Early Closure Diverting stoma Continued Medical Management Late Closure Resect and anastomosis Medical Management Laparotomy NEC Stenting Surgical Intervention Laparotomy Lapscope Conservative Planned Laparotomy Drain Laparotomy for deterioration Indications for surgery Drain as definative management Delayed lap for strictures

Perforations NEC >50% Meconium ileus Spontaneous Gastric Ileal Rectal Traumatic Instrumentation Nasal Ventilation Co-existent pathology CMV Colitis

Rectal Perforation Very rare Characteristic features Recent series of 9 cases Treatment is stoma, drainage, image rectum, close stoma

Associations Steroids, NSAID Systemic candidiasis Conditions that diminish bowel perfusion – cardiac lesions, umbilical catheters Nasal ventilation Instrumentation Cystic Fibrosis

Presentation First few days of life Sepsis: relatively well Feed intolerance and vomiting Abdominal distension Respiratory distress ‘Blue’ discolouration Free Air Football sign Saddle bag sign Arcade sign No pneumatosis, portal gas Water soluble contrast study

Management Resus NPO, NGT IVI antibiotics Percutaneous drainage before transfer Relieve respiratory distress Slow progression to severe sepsis Surgery Primary closure Stoma

Prognosis Cause: NEC vs Other Prematurity UWFA Delay in transfer Immune status

Haemorrhagic Disease Rare Vitamin K deficiency Bleeding sites: Umbilicus Mucous membranes GIT Venepunctures Intracranial Intra abdominal

Neonatal haemoperitoneum Haemorrhagic disease of the newborn Haemophillia Not for surgical intervention Congenital angioma of the liver