An ultrasound performed on a woman who was 23 weeks pregnant revealed multiple findings suspicious for trisomy 21 syndrome, (Down syndrome). The woman and her husband were devastated, saying they could not possibly raise a child with mental retardation and physical anomalies, and they requested a termination. The obstetrician recommended amniocentesis for chromosomal analysis that would give definitive diagnosis of trisomy 21, and the test was performed. The parents said they planned to terminate the pregnancy if the results of the chromosome analysis confirmed Down syndrome.
Before the results were returned, the woman arrived at the labor and delivery unit with a tender abdomen, purulent discharge from the cervix, and high fever. She appeared to have an acute intrauterine infection from the amniocentesis procedure. Antibiotics were started, but it soon became clear that the woman was becoming septic; the obstetrician on call recommended rapid delivery of the fetus. The woman and her husband again clearly stated that they wanted no resuscitation performed on the infant after delivery. The couple and the physicians agreed that, given the probability of a severely anomalous infant, the plan would be to provide only comfort care measures.
The woman's labor was induced and she delivered a liveborn female infant, surprisingly robust. The infant had a strong cry, kicked vigorously, and was much larger than anticipated. The neonatologists examining the infant found themselves reconsidering their decision to withhold resuscitation. Suddenly the seemingly certain prenatal diagnosis of Down syndrome appeared implausible, given the appearance of a strong infant without apparent anomalies. The NICU team realized that, under any other circumstance, resuscitation measures would be well under way; they became uneasy as they watched the premature infant's forceful kicking and energetic cries. Within minutes to hours the female infant's lungs would tire and she would die without respiratory support.
The physicians announced to the parents their decision to reverse their previous plan to withhold care based on the healthy appearance of the neonate. The neonatologist described the resuscitation measures they planned to begin. The parents were infuriated. "We had an agreement," the father retorted. "My wife and I made it very clear to you that we cannot manage an impaired child. This is our decision to make—we're the parents, and it is your duty to respect our wishes."
Decisions about whether to provide intensive care to periviable infants remain some of the most difficult in neonatology. These decisions do not occur in a vacuum; rather they are complex interactions among parental concerns and rights; societal norms, which may be regional rather than national; and the neonatologist's opinions about viability and medical futility. The case presented is an excellent example of the potential conflicts among these competing demands, and it helps focus some of the issues surrounding prenatal consultation and parental decision making.
So, what are the facts about outcomes of extreme prematurity? We know that below a certain gestational age (approximately 22 weeks), because of immaturity of the major organ systems, death is certain even with aggressive care—hence, providing intensive care is medically futile. With advancing gestational age, the chances of survival increase, though this may come at the cost of significant long-term morbidity, especially in those infants born between 23 and 24 weeks' gestation. For example, in the Vermont Oxford Network (a voluntary network for data collection in more than 650 neonatal intensive care units in the U.S. and abroad), among infants born between 1996 and 2000 with a birth weight of 401 to 500 grams and a mean gestational age of 23.2 weeks, mortality was 83 percent, and survivors often had serious short-term medical complications . The EPICure study reported outcomes for all infants born at a gestational age of 20 to 25 weeks over a 10-month period in 1995 in the U.K. and Ireland. Only 811 of the 4,004 infants (20 percent) received intensive care, and 39 percent of those survived to discharge . Of the survivors, 16.5 percent had ultrasonographic evidence of severe brain injury. Of these surviving infants who were evaluated at 30 months of age, half had a motor, cognitive, or neurosensory disability; in approximately one quarter of the children, the disability was considered severe.
The National Institutes of Child Health and Development Neonatal Research Network recently analyzed outcomes at 18 to 22 months of age of 4,446 infants born between 22 to 25 weeks' gestation at 19 centers in the United States . Of these, 83 percent received intensive care in the form of mechanical ventilation. Of the infants for whom outcome could be determined, 49 percent died, 61 percent died or had profound impairment, and 73 percent died or had impairment (defined as mental retardation, moderate or severe cerebral palsy, blindness, or deafness). Factors in addition to gestational age that were found to affect a favorable outcome to intensive care included being female, exposure to antenatal corticosteroids, singleton gestation, and birth weight. A web-based tool to approximate survival without impairment based on these data is available at the National Institutes of Health web site .
How Neonatologists Act on These Facts
How do neonatologists interpret these data when it comes to decisions in the delivery room about resuscitation of an individual infant? A cross-sectional survey of 149 practicing neonatologists in six New England states queried attitudes about whether intensive care was beneficial at different gestational ages . At or below 23-0/7 weeks gestation, 93 percent of the reporting neonatologists considered treatment futile. In contrast, at 24-1/7 to 24-6/7 weeks and 25-1/7 to 25-6/7 weeks' gestation, 41 percent and 84 percent of respondents, respectively, considered treatment beneficial. When asked to consider parental requests, 91 percent of the neonatologists responding reported that they would resuscitate in the delivery room despite parental requests to withhold treatment if they considered treatment to be clearly beneficial. When respondents considered treatment to be of uncertain benefit, 100 percent reported that they would resuscitate if parents requested, 98 percent reported that they would resuscitate if parents were unsure, and 76 percent reported that they would follow parental requests to withhold. Thus, while parents' requests about their infant's resuscitation influence the neonatologist's decision making in the delivery room, these decisions are also heavily influenced by the physicians' beliefs about the gestational age bounds of clearly beneficial care, which are strikingly variable among physicians.
These data reinforce the need for prenatal consultation with parents prior to the expected delivery of an extremely preterm infant. In the same survey of New England neonatologists, respondents were queried as to the content of prenatal consultation . The results showed that neonatologists consistently discussed the clinical issues anticipated with the expectant parents, but they varied when it came to discussing the social and ethical issues surrounding an extreme preterm birth. Of note, while 77 percent of the neonatologists surveyed indicated they thought that decisions about withholding resuscitation should be made jointly with parents, only 40 percent said that the decision is made by both parties in actual practice.
While it is clear that the consulting neonatologist's beliefs about the benefits of providing intensive care influence how the consultation is performed, it is also evident that how the message is framed to parents influences decisions. In a survey of adult volunteers, a hypothetical vignette of a threatened delivery at a gestational age of 23 weeks was given to participants . Respondents were randomly assigned to receive the same prognostic outcome information framed as either likelihood of survival with lack of disability (positive frame) or the chance of dying and likelihood of disability (negative frame), and asked to decide on resuscitation or comfort care. Overall, 24 percent of respondents chose comfort care, and 76 percent chose resuscitation. More participants chose to provide comfort care rather than resuscitation when the vignette was presented in a negative frame.
In practice, because of the uncertainty surrounding outcomes in periviable infants, after prenatal consultation even, many parents are unable to state definitively whether they desire resuscitation or not. In these circumstances, it is left to the neonatologist to decide whether to intervene in the delivery room. Many neonatologists use their initial assessment of the infant at birth and the response to initial resuscitative efforts to help them decide whether to proceed with further intensive care. In a study of outcomes of infants with a birth weight equal to or less than 750 grams, proxy measures of "how the infant looked" in the delivery room (Apgar scores and heart rate at one and five minutes) "were neither sensitive to nor predictive of death before discharge, survival with neurologic disability, or intact neurologic survival" .
Guiding Principles for Decision Making
Where are we left with these difficult decisions about what to do in the delivery room when a periviable infant is born? First, prenatal consultation should provide the expectant parents with factual information about survival and outcomes, unfettered by the neonatologist's personal beliefs. Second, it is essential that the parents' beliefs and attitudes about quality of life be sought and understood. Finally, respect for the parents as decision makers for their unborn infant must form the basis for these conversations. It must remain clear, however, that after the infant's birth, the neonatologist's first duty is to his or her patient—the newly born infant. While the judgment to offer resuscitation to an individual infant should be heavily influenced by the parents' wishes, if clinical circumstances are found to be different after birth than was expected, the physician must first consider the rights of the baby.
This case presents exactly that dilemma—a prenatal diagnosis which is unconfirmed, and an infant perhaps more mature and vigorous than expected. Here, several errors may have been made which influenced the parental decisions. It is unclear whether the parents were provided a sense of the uncertainty of the diagnosis of trisomy 21 based on the ultrasound findings. Many findings "associated" with an aneuploidy may also be seen in a normal fetus. When the mother developed chorioamnionitis after the amniocentesis, the decision to resuscitate the infant needed to be reconsidered and discussed with the parents in the context of what to do if the diagnosis of trisomy 21 was incorrect. It is possible that the parents, when provided with the full information about the outcomes of extreme prematurity, might have chosen resuscitation in the absence of a chromosomal abnormality.
Lastly, as is true for any prenatal consultation, uncertainty about the gestational age needs to be clarified—it is clear that differences of 1 week of gestation can profoundly alter outcome and influence the decision to provide intensive care. While the neonatologist does indeed have a duty to respect the parents' wishes, he or she also has an obligation to provide care that is, in his or her opinion, beneficial to the baby. I would argue in this case, since the gestational age of the infant is certain to be 23 weeks or less, our knowledge of outcomes would swing the first duty to the parent's strongly expressed wishes for no resuscitation, and, regardless of the condition of the infant after birth, comfort care would be appropriate.
- Lucey JF, Rowan CA, Shiono P, et al. Fetal infants: the fate of 4172 infants with birth weights of 401 to 500 grams–the Vermont Oxford Network experience (1996-2000). Pediatrics. 2004;113(6):1559-1566.
- Costeloe K, Hennessy E, Gibson AT, Marlow N, Wilkinson AR. The EPICure study: outcomes to discharge from hospital for infants born at the threshold of viability. Pediatrics. 2000;106(4):659-671.
- Tyson JE, Parikh NA, Langer J, Green C, Higgins RD. Intensive care for preterm newborns—moving beyond gestational age thresholds. N Engl J Med. 2008;358(16):1672-1681.
NICHD Neonatal Research Network (NRN): Extremely Preterm Birth Outcome Data. National Institute of Child Health and Human Development web site. http://www.nichd.nih.gov/about/org/cdbpm/pp/prog%5Fepbo/. Updated April 17, 2008. Accessed August 26, 2008.
- Peerzada JM, Richardson DK, Burns JP. Delivery room decision-making at the threshold of viability. J Pediatr. 2004;145(4):492-498.
- Bastek TK, Richardson DK, Zupancic JA, Burns JP. Prenatal consultation practices at the border of viability: a regional survey. Pediatrics. 2005;116(2):407-413.
- Haward MF, Murphy RO, Lorenz JM. Message framing and perinatal decisions. Pediatrics. 2008;122(1):109-118.
- Singh J, Fanaroff J, Andrews B, et al. Resuscitation in the "gray zone" of viability: determining physician preferences and predicting infant outcomes. Pediatrics. 2007;120(3):519-526.