Fetal surgical therapy for severe congenital malformations

Background: Over the past two decades the diagnosis of life threatening con genital malformations has evolved rapidly. Sophisticated and powerful new i maging and sampling techniques have stripped the veil of mystery from the o nce secretive fetus. Early detection and close follow-up of the fetus with congenital malformations have allowed us to define their natural history, d etermine the clinical features that affect clinical outcome, and plan manag ement approaches to improve prognosis. Fetal surgical intervention is the l ogical culmination of the progress in fetal diagnosis. The purpose of this article is to describe the current techniques and recent advances in prenat al diagnosis and fetal intervention of severe congenital malformation. Material and Methods: A complete review of the literature and our own exper ience concerning fetal surgery was performed. Results: Although most prenatally diagnosed malformations are best managed by appropriate medical and surgical therapy after maternal transport and pl anned delivery at a tertiary care center, an expanding number of simple ana tomical abnormalities with predictable, lethal consequences have been succe ssfully corrected before birth. A malformation amenable to prenatal surgica l intervention must fulfill a number of conditions. It must be severe enoug h to warrant the risks associated with in utero treatment and must be relia bly detectable before birth. Additionally, the pathophysiology must be reve rsible by fetal surgery, significantly improving the prognosis over postnat al treatment. Many technical intricacies of open fetal surgery have been so lved, but pre-term labor and premature rupture of membranes remain a omnipr esent risks to both the mother and the fetus. To reduce maternal morbidity and the risk of prematurity we developed minimally invasive techniques to t reat the fetus prenatally. Current indications of fetal surgery include the treatment of congenital diaphragmatic hernia, cystic adenomatoid malformat ion of the lung, sacrococcygeal teratoma, obstructive uropathy, twin-to-twi n-transfusion-syndrome and myelomeningocele. Minimally invasive surgical te chniques (FETENDO) have significantly lessened the incidence of preterm lab or and promise to extend the indications for fetal surgical intervention. Conclusions: Fetal surgical therapy for severe congenital malformations may improve the outcome of selected patients. The development of FETENDO will in all probability reduce the importance of open fetal surgery in the futur e.