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

consent. The operation notes lacked detail and did not record that the infant was under NICU care or indications for surgery (sac rupture), they only listed anatomical details of the intra-abdominal organs and the technical details of silo formation. The condition of the sac (thickness, integrity, rupture) and details of the rupture repair should have been included in the findings. The inability to enter detailed preoperative diagnoses into the integrated electronic Medical Record (ieMR) operative notes for paediatric surgical pathologies is a well-known flaw of that electronic record system. The consent form was of insufficient standard. It failed to detail the condition and indications for the procedure and consideration of the infant’s severe comorbidities, nor did it detail discussions with the family of the mortality risk and multiple issues associated with silo placement in an exomphalos major, including silo replacement, tissue necrosis, ongoing fluid loss and failure to close the abdominal defect. This was misleading for the FLA or any others scrutinising this document.

The literature documents that a giant omphalocele associated with sac rupture, pulmonary hypertension, pulmonary hypoplasia, cardiac anomalies and low birth weight is associated with a poor outcome and high expected mortality rates and morbidity compared with those infants with a giant omphalocele and no sac rupture or pulmonary hypertension.1,2 This infant was managed with maximal appropriate NICU interventions and with full family involvement in determining and reassessing limits of care.

CLINICAL LESSONS This case highlights a senior consultant-led multidisciplinary approach to decision-making and clinical management for infants with multiple neonatal and congenital anomalies. Consultants should supervise the obtaining of consent by junior team members and entry of documentation into the electronic record for surgical ward rounds to ensure that the complexity of decision-making and anticipation of complications is accurately recorded. The iEMR does not support complex congenital diagnoses within the operative notes. This should serve as a lesson for all health services or jurisdictions to implement new electronic health records that will provide some flexibility to allow this important surgical information to be included by the surgical team.

REFERENCES 1. Nolan HR, Wagner ML, Jenkins T, Lim FY. Outcomes in the giant omphalocele population: A single center comprehensive experience. J Pediatr Surg. 2020. 55(9):1866-1871. doi: 10.1016/j.jpedsurg.2020.04.019. Epub 2020 May 3.

PMID: 32475506.

2. Partridge EA, Hanna BD, Panitch HB, Rintoul NE, Peranteau WH, Flake AW, et.al.,.

Pulmonary hypertension in giant omphalocele infants. J Pediatr Surg. 2014. 49(12):1767-70. doi: 10.1016/j.jpedsurg.2014.09.016. Epub 2014 Nov 14.

PMID: 25487480.