I. INTRODUCTION. Extremely low birth weight (ELBW; birth weight <1,000 g) infants are a unique group of patients in the neonatal intensive care unit (NICU). Because these infants are so physiologically immature, they are extremely sensitive to small changes in respiratory management, blood pressure, fluid administration, nutrition, and virtually all other aspects of care. The optimal way to care for these infants continues to be determined by ongoing research. However, the most effective care based on currently available evidence is best ensured through the implementation of standardized protocols for the care of the ELBW infant within individual NICUs. One approach is outlined in Table 13.1. Uniformity of approach within an institution and a commitment to provide and evaluate care in a collaborative manner across professional disciplines may be the most important aspects of such protocols.

II. PRENATAL CONSIDERATIONS. If possible, extremely premature infants should be delivered in a facility with a high-risk obstetrical service and a level 3 or 4 NICU; the value of this practice in preventing mortality and morbidity in ELBW infants has been demonstrated in several studies. The safety of maternal transport must of course be weighed against the risks of infant transport (see Chapter 17). Prenatal administration of glucocorticoids to the mother, even if there is not time for a full course, reduces the risk of respiratory distress syndrome (RDS) and other sequelae of prematurity and is strongly recommended.

A. Neonatology consultation. If delivery of an extremely premature infant is threatened, a neonatologist should consult with the parents, preferably with

the obstetrician present. There are no reliable systems or prognostic scores that allow one to make firm predictions about a particular case, in part because outcome is also affected by variable practices and beliefs about aggressive resuscitation of these infants. The most useful current data is based on a study of ELBWs born in NICUs participating in the Eunice Shriver National Institute of Child Health and Human Development (NICHD) Neonatal Network. This study reported that survival free from neurodevelopmental disability for infants born between 22 and 25 weeks of gestation was dependent not only on completed weeks of gestation but also on (i) sex, (ii) birth weight, (iii) exposure to antenatal corticosteroids, and (iv) singleton or multiple gestation. Using these data, the NICHD developed a web-based tool to estimate the likelihood of survival with and without severe neurosensory disability (https://www.nichd.nih.gov/about/org/der/branches/ppb/programs/epbo/Pages/epbo_case.aspx). To use the tool, data are entered in each of the five categories (estimated gestational age and birth weight, sex, exposure to antenatal glucocorticoids, and singleton or multiple birth). The tool calculates outcome estimates for survival and survival with moderate or severe disabilities. It is helpful to use this estimator tool as a guide, tempered
by the experience in the individual institution, during antenatal discussions with parents. A general approach to consultation is as follows:

1. Survival. To most parents, the impending delivery of an extremely premature infant is frightening, and their initial concern almost always focuses on the likelihood of infant survival. Recent studies have reported that survival is possible at gestational age as low as 22 weeks. The NICHD network reported survival rates of 6% at 22 completed weeks, 26% at 23 weeks, and 55% and 72% at 24 and 25 weeks, respectively. Other studies have reported even higher survival rates, even at 22 weeks. Assessments based solely on best obstetrical estimate of gestational age do not allow for the impact of other factors, whereas those based on birth weight (a more accurately determined parameter), don’t fully account for the impact of growth restriction. The use of the NICHD estimator allows the consultant to estimate the impact and interaction between gestational maturity, weight, and the other identified critical factors. Although extremely helpful as a starting point, at least two important cautions should be considered in individual cases. First, birth weight has to be estimated for purposes of antenatal discussion, although reliable estimates are often available from ultrasonographic examinations, assuming a technically adequate examination can be performed. If this information is not known, gestational age estimates for appropriate for gestational age (AGA) fetuses can be roughly converted as follows: 600 g = 24 weeks; 750 g = 25 weeks; 850 g = 26 weeks; 1,000 g = 27 weeks. Second, there may be important additional information in individual cases that will significantly impact prognosis, such as the presence of anomalies, infection, chronic growth restriction, or evidence of deteriorating status before birth. Clinical experience must be used to guide interpretation of the impact of such factors.

2. For antenatal counseling, it may also be important to interpret published data in the light of local results. The best obstetrical estimate of gestational age may vary between institutions, and local practices and capabilities may significantly affect both mortality and morbidity in ELBW infants. Within individual institutions, practitioners tend to agree on the gestational age at which an infant has any hope of survival, and this can in effect make prognostication a self-fulfilling prophecy. In counseling, practitioners must avoid simply perpetuating local dogma but at the same time remain cognizant of the current institutional capabilities.

In discussions with parents, it is important to attempt to reach a collaborative decision about what course of treatment would be best for their baby. We advocate attempting resuscitation of all newborns who are potentially viable, but recognize that the personal views of parents regarding what might be an acceptable outcome for their child will vary, and thereby impact decisions about offering resuscitation. Currently, we inform them that resuscitation at birth has been technically feasible at gestational age as low as about 22 weeks and a birth weight as low as about 500 g. In an individual case, the superimposition of medical problems other than prematurity may make survival extremely unlikely or impossible even at higher gestational ages. In counseling parents, we
stress that within these parameters, delivery room resuscitation alone has a high (but not absolute) chance of success, but that this in no way guarantees survival beyond these early minutes. Studies show that decisions based on the apparent condition at birth are unreliable in terms of viability or long-term outcome. We also note that the initiation of intensive care in no way mandates that it be continued if it is later determined to be futile or very likely to result in a poor long-term outcome. We assure parents that initial resuscitation is always followed by frequent reassessment in the NICU and discussions with them and that intensive support may be appropriately withdrawn if the degree of immaturity results in no response to therapy or if catastrophic and irreversible complications occur. Parents are counseled that the period of highest vulnerability may last several weeks in infants of lowest gestational ages. Once all these components are discussed, a recommendation can be made regarding an approach to initial resuscitation.

If parents disagree with this recommendation, differences may be resolved by ensuring that they understand the medical information and that their views and concerns are understood as well as recognition of their central role in determining appropriate care for their child. Almost always, a consensus on a plan of care can be reached, but if an impasse continues, consultation from the institutional ethics service may be warranted (see Chapter 19).

3. Morbidity. Care decisions and parental expectations must be based not only on estimates of survival but also on information about likely shortand long-term prognosis. Before delivery, particular attention is paid to the problems that might appear at birth or shortly thereafter. We explain the risk of RDS and the potential need for ventilatory support. Increasingly, support includes continuous positive airway pressure (CPAP) alone, but mechanical ventilation, at least for a short period, is still required for a significant percentage of infants at the lowest gestational ages. Parents should also be informed of the likelihood of infection at birth depending on perinatal risk factors as well as any plan to screen for it and begin empiric antibiotic therapy while final culture results are pending.

4. Potential morbidity. During prenatal consultation, it is generally recommended to avoid giving parents detailed information on every potential sequela of extreme prematurity because they may be too overwhelmed to process extensive information during this time. We do specifically discuss those problems that are most likely to occur in many ELBW infants or will be screened for during hospitalization. These include apnea of prematurity, intraventricular hemorrhage (IVH), nosocomial sepsis (or evaluations for possible sepsis), and feeding difficulties as well as long-term sensory disabilities. We make a point of briefly discussing the risks of retinopathy of prematurity and subsequent visual deficits and the need for hearing screening and the potential for hearing loss. These complications are not diagnosed until late in the hospital course, but we find that giving parents some perspective on the entire hospitalization is helpful to them.

5. Parents’ desires. In most instances, parents are the best surrogate decision makers for their child. We believe that, within each institution, there should be a uniform approach to parental demands for attempting or withholding resuscitation at very low gestational ages. The best practice is to formulate decisions in concert with parents, after providing them with clear, realistic, and factual information about the possibilities for success of therapy and its long-term outcome.

During the consultation, the neonatologist should try to understand parental wishes about resuscitative efforts and subsequent support especially when chances for infant survival are slim. When counseling parents around an expected birth at <24 weeks, we specifically offer them the choice of limiting delivery room interventions to those designed to ensure comfort alone if they feel that the prognosis appears too bleak for their child. We encourage them to voice their understanding of the planned approach and their expectations for their soon-to-be born child. We reassure them that the strength of their wishes does help guide caregivers in determining whether and how long to continue resuscitation attempts. Through this approach, the parents’ role in decision making as well as the limitations of that role is clarified. In practice, parents’ wishes about resuscitation are central to decision making when the gestational age is <24 completed weeks. At 25 weeks and above, in the absence of other factors, we very strongly advocate for attempting resuscitation and make this clear to parents.

III. DELIVERY ROOM CARE. The pediatric team should include an experienced pediatrician or neonatologist, particularly when the fetus is of <26 weeks’ gestational age. The approach to resuscitation is similar to that in more mature infants (see Chapter 4). Special attention should be paid to the following: