Sacrococcygeal Teratoma (SCT) is the most common tumor of the newborn,
View larger image »which occurs in 1 out of every 35,000 to 40,000 live births. Most fetal sacrococcygeal teratomas are not likely to be malignant, and the prognosis tends to be good after resection.
However, prenatally diagnosed sacrococcygeal teratoma associated with fetal hydrops can be rapidly fatal in-utero or can lead to polyhydramnios and premature delivery. The high-output cardiac failure is related to “vascular steal” from the high blood flow through the tumor. Fetal hydrops and placentomegaly may also jeopardize maternal health through the “maternal mirror” syndrome in which the mother’s condition mirrors that of the sick fetus.
In most instances, the abnormal size of the uterus
Fetal sacrococcygeal teratoma ultrasound. © CHOP/CFDT
View larger image »leads to a diagnosis of fetal Sacrococcygeal Teratoma (SCT) by ultrasound. The discrepancy in size can be due to a massive tumor or to polyhydramnios. Less common presentations include maternal preeclampsia. Fetal Sacrococcygeal Teratoma may be cystic, solid or mixed in its sonographic appearance. The heterogeneous appearance of the mass may be due to mixed areas of tumor necrosis, cystic degeneration, hemorrhage or calcification.
In our experience, fetal hydrops associated with sacrococcygeal teratoma is rapidly progressive and nearly always fatal.
Fetal sacrococcygeal teratoma MRI. © CHOP/CFDT
View larger image »Discussing the implications of the ultrasound findings when a sacrococcygeal teratoma is initially diagnosed can provide parents with information on options to consider if hydrops evolves, a potentially fatal situation.
This diagram shows possible Sacrococcygeal Teratoma treatment options for sacrococcygeal teratoma.
Exposure of 26-week fetus through hysterotomy revealing sacrococcygeal teratoma (SCT).
Closure of skin flaps after resection.
The tumor "steals" blood from fetal circulation, causing the heart to work extra hard and making cardiac failure possible. Cardiac failure is manifest as "hydrops," an accumulation of fluid in the body of the fetus. The association of fetal hydrops and Sacrococcygeal Teratoma is usually fatal, and always fatal prior to 30 weeks gestation.
For the mother, there is the risk of “maternal mirror syndrome” in which the mom’s condition parallels that of the sick fetus.
Other risks include:
Fetal hydrops (excess fluid retention from high output cardiac failure) is the sole indication for fetal surgery. If the condition of the fetus is stable with no high output cardiac failure, there will be regular ultrasound monitoring. If the Sacrococcygeal Teratoma is small, a vaginal delivery at term may be planned. However, if the SCT is large or if there is an excess of amniotic fluid, an early Cesarean section is planned.
If the condition of the fetus (fetal hydrops) places him or her in jeopardy, immediate intervention is recommended. If the fetus is mature, there will be an emergency Cesarean section. If the fetus is not ready to be born, fetal surgery to remove the Sacrococcygeal tumor will be recommended.
General anesthesia is administered to provide complete pain relief for the mother. Although general anesthesia will alleviate most of the pain of the fetus, additional anesthesia is provided to the fetus directly during the operation. Once the mother is asleep, the surgeon will make an incision similar to that of a Cesarean section and will lift the uterus partly out of the abdomen. Next, the surgeon will open the uterus and expose the baby's Sacrococcygeal tumor. The surgeon will remove the tumor and close the wound in the baby's back. The uterus is then closed, placed back inside the maternal abdomen, and the abdominal incision repaired. Mothers recover in the hospital for several days.
Updated: June 2011