Extremely preterm infants are at increased risk for developing an intraventricular hemorrhage in the first few days of life. Higher grades of intraventricular hemorrhage, referred to as severe intraventricular hemorrhage (sIVH), are associated with increased risk of adverse outcomes such as cerebral palsy, other neurodevelopmental impairments, and even death.1,2 Infants who develop sIVH often have significant cardiorespiratory disease as well. These concurrent clinical factors make it difficult to determine if early death after a diagnosis of sIVH is directly related to the hemorrhage, other clinical factors, or a decision to withdraw support.
In this issue of Pediatrics, McCauley et al3 examine whether sIVH is independently associated with death. They analyzed retrospective data from >32 000 infants born at 22 to 29 weeks’ gestation during a 14-year period and hospitalized in one of 242 Pediatrix Medical Group NICUs. In multivariate analysis, the authors found that infants who required mechanical ventilation soon after birth and were subsequently diagnosed with sIVH were less likely to have been exposed to antenatal steroids, more premature, and more likely to require high frequency ventilation and at least 1 vasopressor in the first few days of life. These infants died earlier than those without sIVH (median 7.5 vs 13.5 days), and in infants who survived beyond 30 days of age, sIVH was associated with a greater mortality risk. Using a Cox proportional hazards model, the authors concluded that sIVH was independently associated with mortality in their cohort. Additionally, the authors interpreted regional differences in survival as suggesting that the presence of sIVH played a major role in the decision-making process to withdraw care, regardless of the overall severity of illness.
When a critically ill preterm infant develops sIVH, the risk of death, as well as the potential for significant long-term neurodevelopmental challenges, should be discussed with the parents. These discussions allow parents to work with the medical team to determine if continuing, limiting, or withdrawing life-sustaining medical treatment (LSMT) is in the best interest for their child. Each situation is unique, because each family has their own set of values and desires regarding the quality of life they envision for their infant and family. These conversations with families are often not well documented in the medical record, and therefore, when doing a retrospective review, it can be difficult to ascertain a specific reasoning for a parent’s decision to continue, limit, or withdraw support.4
Decision-making for a critically ill preterm infant is challenging for both parents and providers. The recommended approach in this situation is shared decision-making between the parents and medical team.5–7 It is, therefore, unclear why McCauley et al speculate that the increased mortality rate among infants with sIVH is the result of provider’s influence, because they did not collect data regarding provider–parent interactions preceding the infant’s death. They also did not collect data on the clinical course leading up to the infant’s death, so it is unknown how many of these deaths were the direct result of withdrawal of support, limitation of support, the infant’s underlying physiologic instability, or some combination thereof.
The authors conclude that regional differences in survival suggest that provider and/or parental decision-making influenced the decision to withdraw LSMT for patients with sIVH. They base this conclusion on the fact that providers’ beliefs and practices have been shown to underlie regional variation in the intensity of care for adult patients with end-stage disease processes; it would be important to know the extent to which this applies or does not apply in the NICU setting. Until we do, the authors’ conclusion remains highly speculative.
No one has a crystal ball to predict what the future will hold for an infant with sIVH, and despite a wealth of data regarding outcomes, each case is uniquely different. The fact that extremely preterm infants with sIVH have a greater mortality risk has been well-recognized for many years, and in their study, McCauley et al suggest that sIVH is an independent risk factor. However, the authors’ speculation that this is primarily due to limitation or withdrawal of LSMT is arguable; the current study is limited by its retrospective design, restricted data set, and post hoc analysis. Nevertheless, the authors raise several important questions. How prevalent is limitation of LSMT in infants with sIVH, how are those decisions being made, and how informed are the parents when making such decisions, given the limited prognostic value of cranial ultrasound findings? Some questions may be difficult or even impossible to answer, because of the limitations of medical documentation and the complexity of such decision-making. As McCauley et al conclude, better prognostic tools will help. More important, providers must strive to be transparent and objective when helping parents make such difficult decisions.
Opinions expressed in these commentaries are those of the authors and not necessarily those of the American Academy of Pediatrics or its Committees
FUNDING: No external funding.
COMPANION PAPER: A companion to this article can be found online at www.pediatrics.org/cgi/doi/10.1542/2020-1584.
POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.