Elective ventilation to facilitate organ donation in infants with anencephaly: perinatal professionals' views and an ethical analysis.

AIM: Following the elective ventilation and referral for organ donation of an infant with anencephaly, we sought local perinatal professionals' views of this practice. METHODS: Anonymous online survey: demographics, ethical viewpoints and potential public/maternal perceptions (standard 5-part Likert scale and free text). DEMOGRAPHICS: 49 replies (38 female): 4 obstetricians, 14 neonatologists, 6 foetal clinicians, 23 nurses, 1 anaesthetist and 1 reproductive specialist. EXPERIENCE: 0.5-33 years (average 12). Twenty-one had experience of anencephalic delivery, and 10 reported pregnancy continued for religious reasons. ETHICS: (i) 73% thought anencephalic donation acceptable, of which 64% supported elective ventilation, 20% neutral and 16% disagreed. (ii) Provision of treatments not in infant's strict best interest to facilitate donation: 22% strongly agreed, 36% agreed, 33% neutral and 9% disagreed. (iii) Accept ventilation to permit donation if societal benefit: 53% agreed, 33% neutral and 13% disagreed. (iv) Public opinion: 59% disagreed anencephalic donation would harm public opinion about donation and 19% agreed. CONCLUSION: We found a supportive local environment for donation in the setting of anencephaly, including support for elective ventilation. Given this, and our ethical analysis, we recommend provision of organ donation information as part of palliative care counselling for women carrying a foetus with a condition likely to be fatal in infancy.

Monoamniotic twin pregnancy discordant for lethal open cranial defect: management dilemmas.

OBJECTIVE: To report the sonographic findings, prenatal courses, and management options in four cases of monoamniotic twin pregnancy complicated by a discordant, lethal open cranial vault defect. METHODS: Cases were collected from two Latin American fetal medicine referral centers after the sonographic detection of a monoamniotic twin pregnancy in which one fetus was structurally normal and the other had a lethal open cranial vault defect. Three abnormal fetuses had acrania-anencephaly sequence and one had a large occipital encephalocele. The diagnosis was confirmed at referral, at which time umbilical cord entanglement was also diagnosed with color Doppler ultrasound in all cases. RESULTS: Two cases were managed with selective termination, one of which required referral to a fetal surgery center abroad because this option was illegal in Chile. The other case underwent radiofrequency ablation of the fetal intra-abdominal umbilical vessels after the corresponding ethics and legal committees approved the procedure. In both cases, the non-anomalous twin was born after 35 weeks and had no perinatal complications. Expectant management was performed in the other two cases, resulting in the death of the normal twin at 25 weeks. CONCLUSIONS: Monoamniotic twin pregnancy discordant for severe cranial vault defect is a serious condition with a high risk of fetal demise of the structurally normal fetus. Our experience, although limited, suggests that aggressive management with selective termination of the abnormal twin and transection of its umbilical cord in these cases may prevent the demise of the structurally normal co-twin.

Concepts in the neurosurgical care of patients with spinal neural tube defects: An embryologic approach.

BACKGROUND: The neural tube defects (NTDs) are a heterogeneous group of structural birth defects that arise from a complex array of multiple genetic and environmental factors and adversely affect the structure and function of the brain and spinal cord. Spinal NTDs are clinically more common than cranial NTDs. There remains a significant gap in linking the multiple NTD phenotypes to current genomic understanding. METHODS: This article summarizes the neurosurgical clinical approach to spinal NTDs by correlating each step of embryonic development of the human nervous system with key management concepts for defects that arise at that step. RESULTS: The NTDs are broadly classified as open or closed. Open defects include myelomeningocele (MMC), encephalocele, and anencephaly. Closed defects are also known as occult spinal dysraphism and are characterized by intact skin over the spinal defect. They are more common and often cause neurologic decline from tethered cord syndrome. Failure of primary neurulation gives rise to open myelomeningocele (MMC). Surgical closure of an open MMC focuses on realigning the tissue layers that failed to separate during neurulation. In utero closure is a promising recent technique. Chronic neurosurgical management largely focuses treating hydrocephalus. The Chiari II malformation is uniformly present in MMC patients and may cause brainstem dysfunction. Tethered spinal cord may progressively impair normal neurologic function but typically responds well to surgical untethering. CONCLUSIONS: Surgical closure of MMC centers on approximated realignment of embryologically disordered neural tissue. Clinical surgical management decisions in the spinal NTDs remains challenging but standardized principles have emerged.

Medical futility in the natural attitude.

Medical futility has a long history going back to Plato but continues to be a controversial topic. Patients, families, and health professionals are faced with decisions about which treatments and interventions may be futile, but such questions as who decides; how do competing values get resolved; what value is placed on human life; how are decisions balanced according to reason and, in some circumstances, faith; and who decides effect, benefit, and burdens of treatments are often difficult to answer. The naïve reality of medical futility is explored in the natural attitude to expose presumptions and facts related to both physiologic (fact-based) and evaluative (value-based) futility components. Highlights from the bioethical and clinical literature, a review of 3 landmark cases, and implications for nursing practice are presented. This natural attitude description could serve as what ought to be bracketed for a future phenomenology.

What is left of futility? The convergence of anencephaly and the Emergency Medical Treatment and Active Labor Act.

In 1986, Congress passed legislation intended to prevent hospitals from "dumping" patients who were unable to pay for their care. This legislation was appended to the Consolidated Omnibus Budget Reconciliation Act as the Emergency Medical Treatment and Active Labor Act (EMTALA). The EMTALA imposes two sets of duties on all hospitals that receive Medicare funds. Since 98% of hospitals in the United States participate in the Medicare program, EMTALA applies to nearly all hospitals in the United States. In short, hospitals with an emergency department must provide an appropriate screening examination to any patient who requests treatment. The purpose of the medical screening examination is to determine whether an "emergency medical condition" exists. If an emergency medical condition is discovered, the hospital must either provide treatment sufficient to stabilize the patient's condition or transfer the patient to another medical facility in an acceptable fashion.

Twin pregnancies discordant for anencephaly-management, pregnancy outcome and review of literature.

AIMS AND METHODS: To examine the prognosis of conservative management in twin pregnancies discordant for anencephaly, we review the literature and report on four of our own cases, which were analyzed retrospectively. RESULTS: In the University Hospital of Erlangen, six twin pregnancies discrepant for anencephaly were observed during an 8-year period (1992-2000). Four of these pregnancies were managed conservatively. Preterm delivery occurred in all of the cases, but the unaffected fetuses all had favorable long-term outcome. Delayed interval delivery was performed in two cases due to extreme immaturity in the healthy fetus. CONCLUSIONS: Our data show that in twin pregnancies with early diagnosis of anencephaly in one fetus, the risk of premature delivery of the healthy fetus may be more increased than is already known in the literature. Therefore, selective fetocide of the anencephalic fetus is a management option, at least in dichorionic pregnancies, to prevent polyhydramnios and to reduce the risk of preterm delivery.

Anencephalic infants as sources of organs. Issues and implications for nurses.

This article discusses anencephalic infants as potential sources of organs for infants and young children. Ethical issues relating to screening pregnant women, parental consent for organ donation, and the postnatal care of anencephalic infants are addressed. Professional responsibilities are identified to enable nurses to act ethically when confronted by these issues in caring for mothers and their infants.

A committee consults: the care of an anencephalic infant.

KIE: A hospital ethics committee's deliberations concerning the care of an anencephalic newborn are summarized. Because the defect was irreversible and rapidly lethal, resuscitation and mechanical ventilation were deemed not medically or legally required. The issue of providing fluids and nutrition was complicated: while tube feeding was possible, the infant was beginning to aspirate glucose water, in itself a possible cause of death. Intravenous (IV) feeding might be painful, but could an infant without higher brain structures experience pain? The committee also considered the emotional impact of withholding such treatment on the nurses and family. In the end, no consensus was reached on the issue of withholding fluids and nutrition. The author concludes that the priority concerns, when a decision is made to withhold support from a terminally ill infant, are to avoid pain to the infant and to provide emotional support to the family and staff.^ieng

Ethical issues in organ retrieval from anencephalic infants.

A number of ethical issues have been created by the possibility of transplanting organs from anencephalic infants to other children who need an organ in order to live. The issues of moral justification of the practice, adequacy of informed consent procedures involved, and the potential constraints on female integrity are explored and the relevant arguments for and against transplantation efforts are discussed. Nurses are encouraged to become knowledgeable about the issues and to enter into public discussion of the issues as policy initiatives are proposed.

Monochorionic and dichorionic twin pregnancies discordant for fetal anencephaly: a systematic review of prenatal management options.

The aim of this study was to evaluate the effect of selective feticide (SF) compared to expectant management (EM) on perinatal outcome in dichorionic and monochorionic twins discordant for anencephaly. For this purpose, we conducted a systematic review of literature and added ten unpublished cases. As a result, we found that in dichorionic twins, mean gestational age (GA) at birth in the SF group was 38.0 weeks versus 34.9 weeks (P = 0.0002). Mean birth weight was 2922 g in the SF group versus 2474 g (P = 0.03). In monochorionic twins, mean GA at birth was 35.2 weeks versus 32.7 weeks (P = 0.1). Mean birth weight was 2711 g versus 1667 g (P = 0.0001). We conclude that while SF does not reduce perinatal mortality, it does result in significantly longer gestations and higher birth weight, and appears to be the management of choice in dichorionic twins discordant for anencephaly. In monochorionic twins, SF also increases birth weight, but in view of the complexity of this group, no clear recommendations can be made.

Dilemmas in the management of twins discordant for anencephaly diagnosed at 11 + 0 to 13 + 6 weeks of gestation.

OBJECTIVE: To help develop an evidence-based approach to the best management of twin pregnancies discordant for anencephaly. METHODS: We retrospectively examined the management and outcome of 18 pregnancies discordant for anencephaly diagnosed at 11 + 0 to 13 + 6 weeks of gestation in our center. We combined these data with those from other publications. In total, there were 44 dichorionic pregnancies that were managed expectantly (n = 35) or by selective feticide (n = 9) and 19 monochorionic pregnancies that were managed expectantly. We also reviewed the literature to ascertain the outcome of monochorionic twin pregnancies undergoing cord occlusion. RESULTS: In the 35 dichorionic pregnancies that were managed expectantly, 20 (57.1%) developed polyhydramnios at 25-31 weeks; 13 were managed expectantly, five had amniodrainage and two had selective feticide. In 34 of the 35 cases the non-anencephalic twin was liveborn at a median gestation of 36 (range, 28-39) weeks and in six (17.6%) of these it was born before 33 weeks. In the dichorionic pregnancies that had selective feticide, there was one miscarriage and eight (88.9%) live births at a median gestation of 37 (range, 30-40) weeks and in one (12.5%) of these it was born before 33 weeks. In the monochorionic pregnancies, four (21.1%) anencephalic fetuses died at 20-32 weeks and in three of these the normal co-twin also died. In the 16 (84.2%) cases resulting in the live birth of the normal twin, delivery occurred at a median gestation of 33 (range, 27-39) weeks and in six (37.5%) of these it was before 33 weeks. Ultrasound-guided bipolar cord coagulation in 92 pregnancies, mostly complicated by twin reversed arterial perfusion sequence or severe twin-to-twin transfusion syndrome, was associated with a survival rate of 77.2% and early preterm delivery rate of 31.0%. CONCLUSION: Dichorionic twins discordant for anencephaly are best managed with serial ultrasound examinations for early diagnosis of polyhydramnios, which can then be treated either by amniodrainage or selective feticide. In monochorionic twins it is uncertain whether the best management is expectant or by cord occlusion.

Monoamniotic twins discordant for anencephaly managed conservatively with good outcomes: two case reports and a review of the literature.

Monoamniotic twin pregnancy discordant for anencephaly (MATDA) is a rare occurrence with only seven prior reported cases. Selective termination has been advocated in managing discordant monoamniotic twins. We report two cases managed expectantly with good outcomes and review other previously reported cases. The first case was a primigravid woman diagnosed with MATDA at 18 weeks. She was managed expectantly until 32 + 5 weeks when a Cesarean section was performed for preterm labor. The surviving female infant weighed 1610 g. The second case was a multigravid woman who was diagnosed with MATDA at 17 + 5 weeks and was managed as an outpatient. An emergency Cesarean section was performed at 31 weeks for non-reassuring monitoring and the surviving male infant weighed 1790 g. In both cases, the survivors were discharged home in good condition. A review of these two cases and those in the literature suggests that expectant management should be considered among management options for this rare condition.

Prolonged survival of two anencephalic infants.

Two infants with anencephaly survived for 7 and 10 months without the need for prolonged assisted mechanical ventilation. One infant prospectively fulfilled all four criteria of the Medical Task Force on Anencephaly, making diagnosis almost certain. Prolonged survival of anencephalic infants is important not only to provide adequate counseling, but also for its relevance to organ transplantation.

Etiology and management of monoamniotic twin discordant for anencephaly.

A case of monoamniotic monochorionic (MAMC) female twins, with one twin being anencephalic, and the co-twin being normal, is reported. Twin to twin transfusion syndrome was also noted. The reasons for anencephaly in association with monozygotic (MZ) twinning are discussed. The management of such a case is presented.