Pediatric SurgeryA. Tubbs

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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

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TY 7263849

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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

Congenital Diaphragmatic Hernia

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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

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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

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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

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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)

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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