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.

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.

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.

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.

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.

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.

Distanced perspectives: AIDS, anencephaly, and AHP.

US court decisions guaranteeing life-sustaining care to anencephalic infants have been viewed with disfavor, and sometimes disbelief, by some ethicists who do not believe in the necessity of life-sustaining support for those without cognitive abilities or an independently sustainable future. The distance between these two views--one legal and inclusive, the other medical and specific--seems unbridgeable. This paper reports on a program using multicriterion decision making to define and describe persons in a way which both acknowledges the differences perceived by many as well as those commonalities insisted on in U.S. court decisions. It does this through application of the Analytic Hierarchy Process to a hierarchy of "humanness" criteria, and secondarily through reference to that concept's subset, personhood.

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.

Aspectos éticos relacionados con las decisiones clínicas en neonatología: estudio preliminar / Ethical features related to clinical decisions in neonatology: prliminary report

Background: Personnel working in neonatal intensive care units frequently face difficult ethical problems related to the initation, maintenance or withdrawal of life support therapies. Aim: To assess the importance of ethical issues in the clinical decision making of health care providers in neonatal intensive care units. Material and methods: A questionnaire based on five clinical vignettes that assessed judgments about quality of life, impact of parent's opinions and decision making in emergency situations and with different degrees, of certainty, was designed. Eleven neonatologists and 20 nurses and midwives specialized in neonatology anonymously answered this questionnaire. Results: There was a great inter individual variability in therapeutic approaches in cases with a bad vital and neurological prognosis. In cases of medical emergencies with uncentain diagnoses, bad vital prognosis but neurological indemnity, most professionals coincided in delivering all possible therapeutic options. Parent's opinions had a great impact in medical decisions, except when there was neurological indemnity. Conclusions: The specific responsibilities of the different agents in medical decision making must be delimited. Parents do not have absolute rights over their offspring and physicians must reject useless therapies

Management of twin pregnancies discordant for anencephaly.

OBJECTIVE: To examine options of management and outcome of twin pregnancies discordant for anencephaly. DESIGN: Retrospective study. SETTING: Research Centre for Fetal Medicine. POPULATION: Twenty-four twin pregnancies discordant for anencephaly. METHODS: A computer search was made of our database for twin pregnancies discordant for anencephaly. The data were reviewed for gestation at presentation, chorionicity, management and pregnancy outcome. MAIN OUTCOME MEASURES: Pregnancy outcome in relation to chorionicity and management. RESULTS: There were 13 dichorionic and 11 monochorionic twin pregnancies discordant for anencephaly. In the dichorionic group five pregnancies had selective fetocide at 17 to 21 weeks; one pregnancy resulted in spontaneous abortion but in the others a healthy infant was born at a median gestation of 37 weeks. The other eight dichorionic pregnancies were managed expectantly, but three developed polyhydramios at 26 to 30 weeks; in one case amniodrainage was performed and in another selective fetocide was carried out. In this group the median gestation at delivery was 35 weeks. All 11 monochorionic pregnancies were managed expectantly and in three there was intrauterine death of both fetuses. In the other eight cases the normal twin was liveborn at a median gestation of 34 weeks; in four of these pregnancies polyhydramnios developed and two were managed by amniodrainage. CONCLUSIONS: In monochorionic pregnancies, expectant management is associated with a high rate of intrauterine lethality of the normal twin. In dichorionic pregnancies selective fetocide in the second trimester prevents the development of polyhydramnios and is associated with a lower risk of preterm delivery but can cause miscarriage.

The persistent vegetative state and anencephaly: problematic paradigms for discussing futility and rationing.

Medical futility has been a controversial issue for the last several years. Although anencephalic infants and patients in the persistent vegetative state (PVS) have often been suggested as paradigmatic cases for discussing the issue of medical futility, there is no consensus among physicians or ethicists that treatment of such patients is futile and courts have not supported the futility argument. Focusing on the issue of medical futility will not resolve the management issues surrounding the care of patients in PVS or anencephalic infants. Instead, an analysis of the benefits and burdens of treatments offers a potential solution by demonstrating that the real problem is the dissociation of the benefits and burdens of treatment for such patients. We propose the idea that anencephalic infants and patients in PVS provide an appropriate paradigm for discussing the issue of rationing of health-care resources.

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.