Case 10: Giant omphalocele complicated by sac rupture, congenital pulmonary hypertension and cardiac anomalies Neonatal Paediatrics CASE SUMMARY A 34-week-gestation baby with known giant omphalocele was born by emergency caesarean for premature labour and foetal distress. The birth was attended by senior neonatology staff in the delivery suite. During delivery, a 5 cm rupture of the sac occurred. The baby was ventilated and admitted to a quaternary neonatal ICU (NICU) in the same facility. The sac rupture was sutured by paediatric surgeons in the NICU. In addition to the sac rupture, the baby had multiple comorbidities including severe pulmonary hypertension (suprasystemic pulmonary artery pressures on echo), pulmonary hypoplasia, a double outlet right ventricle with subaortic ventricular septal defect with right-to-left shunting, chronic renal impairment and low birth weight. On day 3, a silo pouch was placed during daytime hours in the paediatric emergency theatre. A reduction in the silo pouch occurred on day 14. Despite maximal intervention for both the pulmonary hypertension and the hypoplasia the baby was unable to be oxygenated. In consultation with neonatologists, the parents decided to provide comfort care on the last day of the baby’s life.
DISCUSSION The chances of survivorship for this infant were low. The paediatric surgery team attended the NICU on arrival of baby after birth and the senior consultant paediatric surgeon performed all surgical procedures. Neonatology staff notes were comprehensive, allowing for clinical continuity of care. Support for and involvement of the parents in the decision-making were excellent. There were multiple family meetings with senior clinicians to outline events, explain decisions and define limits of intervention. These multiple disciplinary team discussions were well documented in the NICU chart. The children’s operating theatres are physically linked to the NICU facility but use a different record system. It was noted by the second-line assessor that not all information for analysis was available to the first-line assessor (FLA). None of the copious NICU notes, including records of surgeon ward rounds, were provided to the FLA. The only available information was the hospital operation record and
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NATIONAL CASE NOTE REVIEW BOOKLET
