Fetal therapy: practical ethical considerations

Progress in prenatal diagnosis can lead to the diagnosis of severe fetal abnormalities for which natural history anticipates a fatal outcome or the development of severe disability despite optimal postnatal care. Intrauterine therapy can be offered in these selected cases. Prenatal diagnosis is the only field of medicine in which termination is an option in the management of severe diseases. Fetal therapy has therefore developed as an alternative to fatalist expectant prenatal management as well as to termination of pregnancy (TOP). There are few standards of fetal care that have gone beyond the stage of equipoise and even fewer have been established based on appropriate studies comparing pre- and postnatal care. Several ethical questions are being raised as fetal surgery develops, including basic Hippocratic principles of patients' autonomy and doctors' duty of competence moving the boundaries between experimental surgery, therapeutic innovation and standard care. In addition, the technical success of a fetal intervention can only rarely fully predict the postnatal outcome. Managing uncertainty regarding long-term morbidity and the possibility for fetal therapy to change the risk of perinatal death into that of severe handicap remains a critical factor affecting women's choice for TOP as an alternative to fetal therapy. Copyright © 2011 John Wiley & Sons, Ltd.     

New and/or improved aspects of fetal surgery

Open fetal surgery through a wide hysterotomy is no longer a real option for prenatal intervention, but a minimally invasive approach has emerged as treatment for a small number of indications. Endoscopic ablation of placental vessels is the preferred treatment for severe twin-to-twin transfusion syndrome and it may be the only chance to salvage the most severe forms of congenital diaphragmatic hernia. Several other indications are currently under review and may become justified in the future, provided that diagnostic accuracy and patient selection become more accurate. Before invasive fetal intervention becomes widely accepted, however, we need to better define outcome. It is no longer acceptable to express results in terms of survival at birth. Survival at discharge and long-term morbidity must be considered as well. Copyright © 2011 John Wiley & Sons, Ltd.     

Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia

Significant advances in the postnatal management of patients with congenital diaphragmatic hernia (CDH) have resulted in a remarkable improvement in survival rates over the past two decades. The success of current postnatal management of CDH patients has rendered fetal intervention to be limited to the most severe cases, and the role for prenatal treatment of CDH patients remains unclear. The adoption of lung-preserving strategies including high-frequency oscillatory ventilation (HFOV) and extracorporeal membrane oxygenation (ECMO) have improved CDH outcomes especially in those patients with significant ventilatory or circulatory compromise. Survival rates of up to 90% are being reported in some high-volume centers. However, the increased survival in CDH patients has been accompanied by an increase in neurological, nutritional and musculoskeletal morbidity among the long-term survivors. This has resulted in the need to provide resources for the long-term follow-up and support of this patient population. In this article, the postnatal management strategies and primary and secondary outcomes of high-volume international pediatric surgical centers will be reviewed. Finally, the role of a multidisciplinary management team for the follow-up of long-term CDH survivors will be discussed. Copyright © 2008 John Wiley & Sons, Ltd.     

Congenital cervical teratoma,associated with agenesis of corpus callosum and a subarachnoid cyst

Cervical teratoma is a neoplasm composed of embryonic tissues with representation of all three germ layers. We report an extremely rare case of fetal cervical teratoma presenting at 24 weeks of gestation. A submaxillary mass and agenesis of corpus callosum were identified on ultrasonography, associated with agenesis of corpus callosum and a subarachnoid cyst. Copyright © 2005 John Wiley & Sons, Ltd.     

Isolated right diaphragmatic eventration mimicking congenital heart disease in utero

Diaphragmatic eventration is the upward displacement of the abdominal viscera secondary to a thin or paralytic diaphragm. Its clinical presentations and radiographic pictures are similar to those of diaphragmatic hernia. Prenatal diagnosis of diaphragmatic eventration is extremely rare. A pregnant woman was referred to us because of abnormal cardiac findings noted at 20 weeks of gestation. A diagnosis of partial anomalous pulmonary venous connection was made on the basis of our findings of right atrial enlargement with an abnormal vascular channel drainage to it. The infant was born via cesarean section at 40 weeks and developed complications of cyanosis immediately after birth. Postnatal imaging studies and surgical findings disclosed right side diaphragmatic eventration with liver and associated vasculature upward displacement into the right pleural cavity. The cardiac structure was otherwise normal. We conclude that when an abnormal vessel tracing and unexplainable cardiac chamber asymmetry is encountered, diaphragmatic eventration should be considered as one of the differential diagnoses. Correct recognition and transferral to the hospital for neonatal assistance may lead to timely and appropriate management of these fetuses. Copyright © 2005 John Wiley & Sons, Ltd.