Introduction:
Sacrococcygeal teratoma is a rare tumor that develops at the tailbone (coccyx) of the fetus. It occurs in one out of every 40,000 live births. The tumor develops from the cells of the reproductive system. The cells of the reproductive system grow abnormally, giving rise to the tumor. Most cases of sacrococcygeal tumors are mild and can be treated after delivery. However, in a few cases, the tumor grows rapidly, requiring treatment during pregnancy. The tumors have several blood vessels coming through them. Girl babies are affected more commonly than males. The tumor can grow to a great extent but is non-cancerous. At times these tumors may grow at the back or inwards towards the child's stomach. According to their location and severity, the tumors can be categorized as:
Type 1 Tumors - The tumors are at the outer surface of the body, attached to the tailbone.
Type 2 Tumors - These tumors are spread both externally and internally.
Type 3 Tumors - These tumors are visible on the outside but are present mostly inside the abdomen.
Type 4 Tumors - These tumors are not visible outside but are present inside the body of the baby.
What Are the Symptoms Associated With Sacrococcygeal Teratoma?
Sacrococcygeal teratoma varies in size. Most of these tumors feed on the blood of the baby, making them grow faster. This puts a lot of pressure on the heart, and it has to work harder.
There is an increase in the amniotic fluid, leading to its accumulation in other body parts.
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Hydrops - A condition where there is excessive fluid build-up in the tissues and organs of the baby leading to swelling or edema. This can be fatal to both the baby and the mother.
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Urinary blockage.
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Bleeding from the tumor.
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Maternal Mirror Syndrome - This condition occurs in association with excessive fetal hydrops, where the condition is similar to that of the fetus. The syndrome may still occur even after the removal of the sacrococcygeal tumor. This puts her at risk of preterm delivery. As a result of hyperdynamic heart functioning, the mother usually develops symptoms that are similar to pre-eclampsia:
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High blood pressure.
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Swelling of the hands and feet.
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Presence of protein in the urine.
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Accumulation of fluid in the lungs.
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Excessive weight gain over a short period.
How Is a Sacrococcygeal Teratoma Diagnosed?
An abnormally sized uterus could possibly suggest the presence of a sacrococcygeal teratoma. The large size could be due to a big tumor or excessive accumulation of the amniotic fluid. A sacrococcygeal tumor can be diagnosed through the following diagnostic modalities:
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Ultrasound - Sacrococcygeal teratoma can be detected in the second-trimester ultrasound. It appears as a mass of tissue at the bottom of the fetus.
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Blood Test - A routine blood test may detect high levels of maternal alpha-fetoprotein.
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Targeted prenatal ultrasound - A a fetal anomaly scan done during pregnancy to study the development and anatomy of the fetus.
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Fetal MRI (magnetic resonance imaging) - An imaging technique used to obtain detailed images of the fetus.
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Fetal Echocardiogram - This echocardiogram examines the heart of the fetus and evaluates how much strain is put on the fetus's heart.
What Are the Risks and Complications Associated With Sacrococcygeal Teratoma?
A sacrococcygeal tumor can become life-threatening to both the mother and baby in association with fetal hydrops. In certain severe cases, the teratoma feeds on the fetal blood, putting pressure on the heart to work laboriously, leading to a risk of heart failure. The condition of the mother is similar to the fetus. The mother becomes sick with signs of preeclampsia or toxemia.
How Is a Sacrococcygeal Teratoma Treated?
Pregnancy Care:
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Most sacrococcygeal teratomas are resected post-birth, hence delivery arrangements must be made in specialized hospitals with expertise in pediatric surgery.
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The small or medium-sized tumors should be examined regularly with ultrasound to make sure it does not grow further and that there is no increase in the blood flow.
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Large tumors should be routinely monitored between 18 to 28 weeks of pregnancy for the growth of the tumor, excessive blood flow to the tumor, and heart failure. In some cases, the fetus can develop heart failure as a result of heavy blood flow through the tumor. Such a fetus is an ideal candidate for intervention.
Fetal Intervention:
Fetus intervention is carried out in cases where there is a chance of heart failure. Fetal intervention before 32 weeks of pregnancy is advised to reverse fetal heart failure. Open fetal surgery is performed, and the sacrococcygeal tumor is resected. Whereas in case the pregnancy is past 32 weeks, the fetus may be delivered for intensive management post-birth. Most of the fetus interventions are minimally invasive that does not require cutting the uterus open. Surgery is carried out with the help of a needle that is inserted through the mother's abdomen into the blood vessels feeding the tumor. Which radiofrequency waves are used to destroy the blood vessels. This puts a stop to the tumor growth, thus reversing heart failure. In another method, the blood flow to the tumor is stopped with the help of drug injection such as alcohol, causing the blood to clot. However, none of them have been found to be universally effective. Fetal intervention is not indicated in women with advanced hydrops, thickened placenta, or pre-eclampsia, as these are irreversible situations.
Treatment Post-birth:
A sacrococcygeal teratoma bigger than ten centimeters in diameter requires a cesarean delivery. The baby is kept in the intensive care unit till the time they are stable for the surgery. Small tumors can be resected without much difficulty, but large ones can be very difficult to remove. Blood transfusions and intensive care is required by the babies for some time. The kids should be monitored annually for increased levels of alpha-fetoprotein levels, which could suggest a recurrence of the tumor. Resection of a huge tumor can lead to long-term problems such as difficulty in urination and passing stool.
Conclusion:
The sacrococcygeal tumor is a tumor of the fetus that is located at the base of the tailbone. These tumors can grow to a large extent but are usually not cancerous. They can cause trouble to the mother and are usually surgically resected post-birth. It is diagnosed accidentally in a maternal blood test carried out at 16 weeks showing an increased alpha-fetoprotein or in an ultrasound. The uterus increases in size due to excess accumulation of amniotic fluid. The prognosis after tumor resection is good, with a few long-term problems, such as difficulty in passing urine and motion.
