NEC- What Lies Under the Big Umbrella?

Transcription

1 NEC- What Lies Under the Big Umbrella? Instructor in Surgery Associate Surgical Director, Center for Advanced Intestinal Rehabilitation Department of Surgery, Boston Children's Hospital Harvard Medical School Boston, MA Biren Modi MD is an Instructor in Surgery and Associate Surgical Director at Boston Children s Hospital, Center for Advanced Intestinal Rehabilitation. He is intermittently involved in weekly Department of Surgery teaching conferences and has been a speaker for continuing education lectures on pediatric surgery and retinopathy. Dr. Modi is a member of the American Academy of Pediatrics, Section on Surgery, Publications Committee and is an ad-hoc reviewer for the Journal of Parenteral and Enteral Nutrition, the Journal of Pediatric Surgery, and the Journal of Surgical Education and holds several department and hospital administrative roles at Boston Children s Hospital. Annual Quality Congress Breakout Session, Saturday, October 3, 2015 NEC- What Lies Under the Big Umbrella? Objective: Identify multiple pathways to the common diagnosis of Necrotizing Enterocolitis".

2 Disclosures Necrotizing Enterocolitis An Umbrella Diagnosis No relevant financial conflicts Center for Advanced Intestinal Rehabilitation (CAIR) Boston Children s Hospital Harvard Medical School 2 Focus Discuss types of NEC Discuss outcomes in these entities and how these data can inform discussions with parents and future investigation Will not discuss: Etiology and prevention Treatment strategies, peritoneal drainage Premise NEC is an umbrella diagnosis Micropremature infants with and without physiologic instability Infants and children with congenital heart defects and poor perfusion Infants with gastroschisis 3 4 Impetus Uncertainty is worse than all. ~Alexander Dumas Case Discussions What do we know about what happens to babies with NEC? The most fundamental question: survival? 5 6 October 3,

3 Case Discussion #1 Case Discussion #1 27 weeker, BW 1000g, DOL 12 Chorioamnionitis, neonatal sepsis, UAC/UVC Goal feeds at DOL 10 CPAP Sudden abdominal distention, hypotension, apneic spells, Replogle placed with full previous feed aspirated NPO, Antibiotics WBC 4K, Plts 120K Case Discussion #2 Case Discussion #2 27 weeker, BW 1000g, DOL 12 Chorioamnionitis, neonatal sepsis, UAC/UVC Goal feeds at DOL 10 CPAP Same child, started on Abx, bowel rest Followup Plts 30K, on dopamine Case Discussion #2 Case Discussion #2 October 3,

4 Case Discussion #2b Questions Raised Each of these babies has prematurity and its comorbidities Each of them has a slight variation on a common intestinal pathology What data do we have to be able to individually counsel these families on expected outcomes? Survival? Available Data Guthrie (US, J Perinatology 2003) , retrospective administrative dataset 15,000 neonates 390 cases NEC (245 medical, 145 surgical) NEC mortality 12% (23 Surg vs 5% Med) Rees (UK, J Pediatric Surgery 2010) , prospective cross-sectional survey 11,000 neonates 211 with NEC (145 medical, 66 surgical) NEC mortality estimate 13% Vermont Oxford Collaboration Network of 655 NICUs of varying type Prospective cohort of VLBW neonates Inborn or transferred in within 28 DOL Surgical collaboration since 2004 Goals: Provide a larger study set with a larger disease population Provide more granularity to the outcomes 15 Preliminary Work Mortality in NEC What is the risk of NEC in VLBW? Risk of NEC (%) Risk of NEC (%) Journal of the American College of Surgeons, g g g g Fitzgibbons, JPS October 3,

5 Mortality in NEC Specific Aim: Prospectively quantify the mortality of NEC in VLBW neonates Express mortality with respect to birth weight Compare mortality of medical and surgical NEC Hypothesis: Surgical NEC has a higher mortality than medical NEC Overarching goal: Provide new, essentially population-based, benchmark data for prognosis and eventually QI Methods 5 yr data collection Jan 2006-Dec 2010 Included VLBW neonates BW 401g-1500g Excluded major birth defects (major CHD) or LOS < 3 days Methods Survival defined as survival to discharge from hospital or in-hospital at one year of age Codes for the surgical treatment of NEC delineated prior to study period Uniform definition for NEC Operation or necropsy Clinical Criteria Methods Clinical and Radiologic Criteria Clinical findings ( 1): Radiologic findings ( 1): Bilious gastric Pneumatosis aspirate AND intestinalis Emesis Abdominal Hepatobiliary gas distention Pneumoperitoneum Occult/gross fecal blood (no anal fissures) Limitations Results Data are an amalgam of hospitals with variant resources and decision-making was not controlled Nonetheless, encompassed 80% of VLBW neonates born in the US during the study period and therefore represents a true picture of NEC October 3,

6 Results Incidence of NEC in VLBW neonates 9% Of NEC neonates, 52% required surgery Each 100g = 5% less surgery } P < Mortality All NEC: 28% Medical NEC: 21% Surgical NEC: 35% Mortality in NEC Percent mortality Mortality in Surgical vs. Medical NEC by Birth Weight OR 0.99 ( ) OR 1.12 ( ) * OR 1.56 ( ) * * OR 2.81 ( ) OR 6.10 ( ) Surgical NEC Medical NEC Birth weight (grams) 27 Study Conclusions If you are VLBW in the US, you have a 9% chance of developing NEC If you develop NEC, you have a 28% mortality rate If you get surgery, your mortality is 35% If you don t get surgery, your mortality is 21% Based on your birth weight alone, these mortality rates can be further specified If you get surgery, increased birth weight is less protective and survival plateaus at ~70% But what about Case #3 27 weeker, BW 1000g, DOL 12 Chorioamnionitis, neonatal sepsis, UAC/UVC Treated for PDA DOL 10 CPAP Sudden abdominal distention October 3,

7 Case Discussion #3 Case Discussion #3 Case Discussion #3 Spontaneous Intestinal Perforation Big data set lets us do smaller analyses Benchmark outcomes in this specific subset of NEC are not well documented Definitions based exclusively on operative findings to eliminate misclassification Reduced sample size Does not account for patients treated with peritoneal drainage only who were excluded 34 Spontaneous Intestinal Perforation Journal of Pediatric Surgery, 2014 Mortality in SIP VON dataset, Establish mortality rates in VLBW neonates for laparotomy-confirmed SIP Odds ratio of death in patients with laparotomy-confirmed SIP compared to: No NEC, No SIP Laparotomy-confirmed NEC 35 October 3,

8 Mortality in SIP Mortality of SIP vs. Baseline Baseline No NEC No SIP 171,459 neonates 5% mortality Surgical NEC 4072 neonates (2.3%) 38% mortality Laparotomy-confirmed SIP 2035 neonates (1.1%) 19% mortality OR vs. baseline: 1.24 ( ), P=0.003 OR vs. NEC: 0.33 ( ), P< Mortality of SIP vs. Surgical NEC Conclusions While mortality from laparotomy-confirmed SIP is half that from surgical NEC, it is nonetheless significant and much higher when compared to non-nec non-sip neonates Unlike surgical NEC, SIP mortality continues to decrease with increasing birth weight categories But what about case #4 Term infant POD4 from stage 1 repair of hypoplastic left heart syndrome Epinephrine and milrinone Baseline O2 sat 80-85% Abdominal distention Lactic acidosis Pneumatosis on KUB, no free air Mortality of NEC in Congenital Heart Disease Journal of the American College of Surgeons, October 3,

9 Mortality of NEC in CHD Mortality of NEC in CHD VON dataset, Establish mortality rates for necrotizing enterocolitis occurring in VLBW neonates with congenital heart disease 44 Mortality of NEC in CHD Conclusions **AOR 2.58 ( ) *AOR 4.14 ( ) CHD increases risk of NEC compared to baseline (13% vs. 9%) in VLBW neonates Mortality of NEC and CHD (55%) is significant higher than mortality of either disease alone This mortality does not significantly improve with increasing birth weight categories (not shown) Case Discussion (Bonus) Case #5: 35 weeker with gastroschisis and volvulus, now in continuity, at full feeds, preparing for discharge on HD90, with blood in stool and pneumatosis Similar presentations to others, similar findings on exam and imaging, how are they different? This is the topic of ongoing work What have we learned? Incidence of NEC Overall 9% in VLBW neonates 52% managed surgically with 5% decrease in need for surgery with each 100g increase in birth weight Laparotomy-confirmed SIP 1:2 surgical NEC 13% in VLBW neonates with CHD October 3,

10 What have we learned? Thank you! Mortality in NEC Overall 28% Surgical 35% no improvement with increased BW Medical 21% linearly decreases as BW increases Laparotomy-confirmed SIP 19% NEC combined with CHD 55% Surgical NEC Treatment Strategy October 3,