Index score at age 30 months. And, as shown in Table 16-3, surgery was associated with a small but significant risk for pla cental abruption and maternal pulmonary edema. Moreover, nearly half were delivered before 34 weeks, which signiicantly increased the risk for respiratory distress syndrome (Adzick, 201l). Long-term surveillance data have only recently become cele repair prior to the MOMS trial. At a median follow-up of 10 years, these children have higher rates of behavioral problems and adverse executive functioning compared with population norms (Danzer, 2016).
Since publication of the MOMS findings, fetal myelomeningocele surgery rates have grown. Expansion of centers ofering this procedure has raised concerns about the importance of training and ongoing experience, adherence to the MOMS research criteria, and need for a registry to ensure that future eforts have similar success rates (Cohen, 2014; Vrecenak, 20l3).
In the past, if hydrops developed in a fetus with a large pulmonary sequestration or cystic adenomatoid malformation without a dominant cyst, open fetal surgery with lobectomy was the only treatment available other than preterm delivery. Most thoracic masses are small and have a benign prognosis, and larger masses are generally treated with corticosteroids (p. 318). Fetal surgery is generally reserved for cases prior to 32 weeks in which hydrops is developing, and in selected cases, the survival rate following open lobectomy approximates 60 percent (Vrecenak, 20l3). Use of the ex-utero intrapartum treatment procedure in the treatment of fetal lung masses at delivery is discussed later on page 327.
This germ cell tumor has a prevalence of approximately 1 per 28,000 births (Derikx, 2006; Swamy, 2008). Sonographically, a sacrococcygeal teratoma (SCT) is a solid and/or cystic mass that arises from the anterior sacrum (Fig. 16-3). Fetal MR imaging can aid evaluation of the extent of the internal tumor component. he mass may grow rapidly, usually extending inferiorly and externally (Fig. 10-18, p. 196). Hydramnios is common, and hydrops may develop from high-output cardiac failure, either as a consequence of tumor vascularity or secondary to bleeding within the tumor and resultant anemia. Mirror syndrome-maternal preeclampsia developing along with fetal hydrops-may occur in this setting (Chap. 15, p. 312).
FIGURE 16-3 Fetal surgery for sacrococcygeal teratoma resection. Following laparotomy and hysterotomy, the caudal portion of the fetus has been delivered onto the surgical field. The tumor is held by the surgeon's hand. (Used with permission from Dr. Timothy M. Crombleholme.)