Trisomy 18, Ductal-Dependent Circulation, and a Family's Request for Surgery
2.9 kg term girl with trisomy 18, an atrioventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. She is ductal-dependent, on PGE1, and has had one episode of necrotizing enterocolitis treated medically. Her parents, who have limited English proficiency, want full surgical intervention. Two intensivists believe surgery is non-beneficial; the surgeon says a staged repair is possible but high risk. Hospital policy requires an ethics consult when clinicians disagree about non-beneficial treatment. One NICU bed and one ECMO circuit are available.
1. How would you prepare for and conduct the family meeting?
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Preparation:
- Arrange a qualified medical interpreter and a quiet room; include both parents, the primary intensivist, surgeon, palliative care, and ethics if appropriate.
- Review the records and ask open questions: what have you been told, and what are your hopes for her?
- Explain the diagnosis in plain language: trisomy 18, the heart lesion, the need for a source of pulmonary blood flow, and the difference between survival and meaningful survival. Avoid quoting a single prognosis number.
- Present options: staged surgical repair, a time-limited trial, or comfort-focused care. Be honest that surgery is high-risk and may not change her underlying neurodevelopmental prognosis.
- Explore the family's values, religious beliefs, and practical supports; use the interpreter to confirm understanding.
- Summarize, document, and schedule follow-up.
2. How should the team resolve the disagreement and address the resource constraints without using trisomy 18 alone as a categorical exclusion?
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Do not let trisomy 18 alone decide. Use a structured ethics framework:
- Clarify the clinical facts: what surgery would involve, expected survival, and burdens.
- Distinguish non-beneficial from high-risk but potentially beneficial; reasonable people can disagree.
- Ask ethics to facilitate, not to rubber-stamp. Include a second surgeon or cardiologist.
- Address resource constraints transparently: one NICU bed and one ECMO circuit are real, but rationing by disability alone is ethically and legally problematic. Use published triage criteria if scarcity is imminent.
- Seek consensus on a time-limited trial with explicit goals and withdrawal criteria.
- If consensus fails, follow hospital policy for dispute resolution and continue supportive care in the meantime.
3. If the parents insist on surgery and the surgeon refuses, what systems-based steps should be taken?
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If the surgeon refuses and the parents insist:
- Convene the ethics committee and document the discussion.
- Offer transfer to a center willing to operate, if the parents want that and transport is safe.
- Do not abandon the family; continue PGE1, feeding support, and palliative care.
- If disagreement persists, involve risk management and legal counsel according to policy; seek a court order only as a last resort.
- Ensure the family has interpreter services and written information.
- Review the case at M&M or ethics rounds to improve future processes. The goal is a transparent, fair process, not a unilateral decision.