1. Pediatric Surgery A. Tubbs

2. 1 TY 7263849  35 week 2.2kg infant with known L CDH to a 30 year old G6 P4 AA female via SVD  Intubated at 7 minutes of birth when she became apneic. Initially on minimal vent settings in NICU without need for ECMO.  DOL 2 hypotension and bradycardia requiring pressor support and continued to worsen over the next two days  DOL 5 ECMO, stabilized  Day 8 ECMO dramatically worsened with white out on the CXR and never recovered  Day 14 R chest tube placed for effusion, 50ml serous drainage, minimal improvement  Day 15 overnight flows gradually decreased, O2 sat in 20’s for several hours, coded as changing the circuit  Stabilized over the weekend  DOL 22/ ECMO Day 18 proceeded with L CDH repair on ECMO with gortex patch  Agenesis of the entire left hemidiaphragm except small anterior rim  Entire bowel in the chest with minimal lung tissue  Heparin bleeding  Chest tube and skin only closure  Actively resuscitated all night and POD 1 with ~700ml from chest tube  POD 2 hypotension requiring max doses of dopamine and dobutamine, anuria  Withdrawal of care

3. 3 TY 7263849

4. 14 Analysis of Complication Was the complication potentially avoidable? – No Would avoiding the complication change the outcome for the patient? – Yes What factors contributed the complication? – Patient disease – Agenesis of the diaphragm – Minimal good lung tissue – Prematurity – ECMO/Heparin

5. Congenital Diaphragmatic Hernia 15  Malformation of the diaphragm allowing bowel to herniate into the thoracic cavity before birth resulting in pulmonary hypoplasia and pulmonary hypertension  Most are left sided and are associated with malrotation  ~50% of survivors are treated with ECMO  Overall survival rate is 60%, less with prematurity  Delayed repair

6. 6 Survival in early- and late-term infants with congenital diaphragmatic hernia treated with ECMO. Stevens TP, Chess PR, et al. Pediatrics. 2002 Sep;110(3):590-6.  Retrospective cohort study of all infants in the ELSO registry placed on ECMO over past 25 yrs  Early term 38-39w, Late term 40-41w  53% v 63% survival rate, shorter ECMO duration, shorter hospital stay and fewer complications

7. 7 Cardiac arrest before repair or ECMO cannulation does not increase the mortality rate associated with CDH. Courcoulas AP, Reblock KK, Rowe MI, Ford HR. J Pediatric Surg. 1997 Jul;32(7):952-6.  Retrospective review 119 infants  21 suffered arrest before repair or cannulation  No sign difference in birth wts, GA, race/gender, preg/delivery complications  Significant number of those that arrested required ECMO for prolonged time  No sign difference in overall survival

8. 8 Factors associated with survival in infants with CDH requiring ECMO: a report from the CDH study group. Seetharamaiah R, et al. J Pediatric Surg. 2009 Jul;44(7):1315-21.  3100 children  Survivors:  Greater gestational age  Greater birth weights  Less often prenatally diagnosed  Required ECMO for shorter period of time (9 +/- 5 v. 12 +/- 5)

9. 18 Take Home Points  Delayed repair of CDH  50% CDH infants require ECMO  Survival rate ~60%, decreased with decreased gestational age and birth weight  Shorter duration of ECMO associated with improved survival  Not optimal to repair on ECMO