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PubMed This is a summary of 23 peer-reviewed journal articles Updated
Neonatology

Making Decisions: Medical and Surgical Options

At a Glance

For a child with Trisomy 18, surgery is not automatically ruled out: the care team should weigh the child's anatomy and stability, expected benefits, risks, family goals, and quality of life. Intensive, selective, time-limited, or comfort-directed care may all be considered.

Deciding whether to pursue surgery for a child with Trisomy 18 is one of the most complex choices a family can face. In the past, these options were rarely offered, but modern medicine has moved toward a shared decision-making model. This means your family’s goals and your child’s specific health needs are the primary guides for care [1][2].

Shifting Perspectives on Surgery

Medical experts now emphasize that a Trisomy 18 diagnosis alone is not a reason to automatically deny surgery [3][4]. Instead, doctors are encouraged to evaluate each child case-by-case, looking at the severity of their heart or organ issues and their overall stability [4][5]. While surgeries can improve the chance of a child being discharged from the hospital, they do not “cure” the underlying chromosomal condition, and they come with significant medical burdens [6][7].

Cardiac (Heart) Surgery

About 80% of children with Trisomy 18 have heart defects, such as a Ventricular Septal Defect (VSD), which is a hole between the lower chambers of the heart [8].

  • Potential Benefits: Retrospective observational studies suggest that in selected cases, heart surgery can be associated with higher survival rates and a higher likelihood of the child being discharged to go home [7][9]. One study found an association between surgery and reaching the first birthday [10]. It is vital to recognize that children selected for surgery are often already more stable or have more treatable anatomy, so this observational comparison does not prove that surgery alone caused the survival difference.
  • Risks and Morbidity: Surgery is high-risk for these infants. Operative mortality (death during or shortly after surgery) has been reported between 8% and 15% in different selected cohorts [11][12]. Complications are common, occurring in over 50% of operations in some registries, and can include dangerous heart rhythms (arrhythmias), severe infections, and the need for a breathing machine for a long time [11][9].
  • Key Factors: Children who are already on a ventilator (breathing machine) before surgery have much higher odds of operative death [11]. Decisions are often based on individualized risk estimates by the treating congenital-heart team [4].

Non-Cardiac Surgeries and Procedures

Beyond the heart, other procedures may be considered to help with daily care or stability, but they require a clear understanding of informed consent:

  • Gastrostomy Tube (G-tube): A small tube placed through the skin into the stomach to provide nutrition [13]. While it can support nutrition, it does not guarantee adequate calories, prevent reflux, eliminate aspiration, or guarantee a child is stable enough for discharge; it involves procedural and ongoing caregiving burdens [6][14].
  • Tracheostomy: A surgical opening in the neck to provide a direct airway [13]. This is generally considered for selected chronic airway obstruction or prolonged ventilatory dependence; it is not simply a usual treatment for apnea [15][16]. It requires intensive home-care requirements and specialist assessment.
  • Esophageal Atresia Repair: If a child is born without a connection between the throat and stomach, surgery may be required to allow for feeding and to prevent saliva from entering the lungs [17][18].

Intensive Management vs. Comfort-Directed Care

You have the right to work with your medical team to choose the pathway that best aligns with your family’s values, understanding that clinicians also weigh interventions based on medical benefit and harm.

  1. Intensive Pathway: This involves pursuing surgeries and intensive care (NICU) with the goal of prolonging life and, if possible, bringing the child home [1]. It may involve long hospital stays and multiple procedures.
  2. Comfort-Directed Plan: This focuses on the quality of life, minimizing pain, and maximizing time spent together as a family, often at home [19][20]. In this pathway, medical interventions are chosen based on whether they improve comfort (like medicine for breathing distress) rather than just to extend life [19].

Integrating Support

Palliative care clinicians are essential partners regardless of which path you choose [19]. They can support either intensive treatment, selective treatment, or comfort-only care [21][22]. A time-limited trial is another option, where intensive treatment is tried for a documented, set period to see how the baby responds before making long-term decisions [1].

Ultimately, the goal is to create a plan where your child is not “over-treated” in a way that causes unnecessary suffering, nor “under-treated” based on a label alone [3][23].

Common questions in this guide

Does a Trisomy 18 diagnosis automatically rule out surgery?
No. Shared decision-making considers the child's heart or organ problems, medical stability, expected benefits and burdens, and the family's goals rather than using the diagnosis alone to deny surgery.
Can heart surgery help a child with Trisomy 18 live longer or go home?
In selected children, observational studies have found that heart surgery is associated with higher survival and a greater chance of hospital discharge, and one study found an association with reaching the first birthday. These studies do not prove that surgery itself caused the better outcomes because children chosen for surgery may already be more stable or have more treatable heart anatomy.
How risky is heart surgery for a child with Trisomy 18?
It is high-risk: death during or shortly after surgery has been reported in about 8% to 15% of selected groups, and more than half of operations in some registries had complications. Possible problems include abnormal heart rhythms, severe infection, and needing a breathing machine for a prolonged time; being on a ventilator before surgery raises the risk.
What should families know about a G-tube or tracheostomy?
A G-tube can deliver nutrition but does not guarantee enough calories, prevent reflux or aspiration, or make discharge home possible. A tracheostomy can provide a direct airway for selected children with chronic airway blockage or prolonged dependence on a ventilator, but it requires intensive home care and is not a routine treatment for apnea. Both decisions should account for procedure risks and caregiving responsibilities.
What is the difference between intensive treatment and comfort-directed care?
Intensive care may include NICU treatment and surgery with goals of prolonging life and possibly bringing the child home. Comfort-directed care prioritizes relief of pain and breathing distress, quality of life, and time together, choosing interventions for comfort rather than life extension alone. Palliative care can support either approach.
How does a time-limited trial of intensive care work?
The team and family agree in advance on a defined period of intensive treatment and specific goals, such as improved stability or reduced need for medical support. They then review the child's response and use that information to decide whether to continue, change, or stop intensive interventions. This can help families make decisions without committing immediately to a major long-term plan.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What are the specific risks of this procedure for my child, including the likelihood of needing a breathing tube (mechanical ventilation) for a long time afterward?
  2. 2.How much experience does this surgical team have in performing this specific operation on children with Trisomy 18?
  3. 3.If we choose surgery, what are the chances my child will be able to come home, and what kind of medical equipment (like oxygen or feeding pumps) will we need at home?
  4. 4.How will this surgery change my child's daily comfort and quality of life?
  5. 5.What is the 'plan B' if the surgery is not successful or if my child experiences a serious complication like an arrhythmia or infection?
  6. 6.Can we set specific goals for a 'time-limited trial' of intensive care to see how my child responds before committing to a major surgery?

Questions For You

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References

References (23)
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    Shared decision making and the pathways approach in the prenatal and postnatal management of the trisomy 13 and trisomy 18 syndromes.

    Andrews SE, Downey AG, Showalter DS, et al.

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    Cardiac Surgical Repair Should Be Offered to Infants with Trisomy 18, Interrupted Aortic Arch and Ventricular Septal Defect.

    Kavarana MN

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    The American Association for Thoracic Surgery (AATS) 2023 Expert Consensus Document: Recommendation for the care of children with trisomy 13 or trisomy 18 and a congenital heart defect.

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This page provides general information about medical and surgical decisions for children with Trisomy 18 and does not constitute medical advice. Discuss your child's individual risks, goals, and care options with the treating medical and palliative care teams.

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