It is important for people with VHL to discuss a possible pregnancy with their doctor and medical team, including what might happen if tumors grow during pregnancy. Since it is preferable not to use tests that involve radiation while pregnant for fear of harming the baby, it is best for testing to be performed in advance. It is also important to discuss possible risk factors before making the decision to get pregnant. Pregnant women with VHL should watch for symptoms and report anything to the doctor. Pregnancy is accompanied by multiple changes in the body. While some are normal in any pregnancy, they can be of particular concern for someone with VHL.

• Vomiting and headaches: This will take more watching than for most pregnant people, since these can also be signs of brain and spinal tumors. Do not ignore or discount them, particularly if they are excessive or persistent. A little morning sickness is normal as the amount of vomiting is variable within a pregnancy. You should always check with your medical team if there is cause for concern.

• Doubling of blood volume: If you have a hemangioblastoma in the brain, spinal cord or retina, this increased blood flow may expand the tumor for a period of time during the pregnancy. Some pregnant VHL patients have reported worsening of symptoms during the pregnancy followed by a lessening of symptoms after delivery. In some cases, the expansion took mild or non-existent symptoms and expanded them to a critical level.

• Possibility of triggering an existing pheochromocytoma (pheo): It is important to have a thorough test for a pheo before planning a pregnancy, or as soon as you are pregnant, and especially before going through the birthing process. An active pheo can be life-threatening to you and your baby. Be sure to get checked—and rechecked—for a pheo during the pregnancy to avoid these complications. Pheo symptoms can be overlooked during pregnancy, with the assumption that high blood pressure is due to preeclampsia or another issue. Undiagnosed pheos can increase risk of parental death. This higher parental mortality arises from pheo-related difficulties in controlling blood pressure. For example, elevated blood pressure can result in the premature separation of the placenta from the uterus, posing a life-threatening problem for the parent and the fetus. Pheos have been safely removed during some stages of pregnancy, but it is preferable to remove them prior to pregnancy.

• Additional strain to your spinal column due to the extra weight of the fetus: Depending on what cysts or tumors are already present in the spinal cord, this additional stress may cause a worsening of symptoms

• Increased fluid load on your kidneys: You need to make sure that your kidney function is normal so that your kidneys will serve you and your baby well. Because some changes from pregnancy can mask symptoms and signs of tumors, it is important to know what is going on before those changes begin. The surveillance guidelines regarding pregnancy include

• Going for a generalized physical exam, with a medical history taken, as well as blood pressure and heart rate checked, prior to conception

• Having an abdominal, brain, and spine MRI—with and without contrast—prior to conception. If already pregnant, any necessary MRIs should be performed WITHOUT contrast only

• Getting tested for pheochromocytomas, via a plasma free metanephrines test (blood) or urinary free metanephrines test (24-hour urine) prior to conception

• Undergoing a dilated retinal exam for hemangioblastomas prior to conception, and every 6-12 months thereafter.[IS1]

Regarding anesthesia during labor, there is a theoretical risk that spinal hemangioblastomas may rupture with anesthesia; however, very few VHL lesions are in the lumbar region of the spine. Thus, if the hemangioblastomas are not in the lumbar region, the risk during epidural anesthesia is likely low. It is important to have imaging done before administering anesthesia. Some anesthesiologists will not offer epidurals to patients who have spinal hemangioblastomas. General anesthesia appears to be safe when used in an emergency. Approximately 2–3 months after the baby is born, have another thorough check-up to evaluate any changes in your own health. New symptoms or complications of central nervous system (CNS) lesions could occur postpartum and thus the patient with VHL should be examined carefully, especially if any new symptoms arise.

Women with VHL who have just given birth should resume scans within 2-3 months postpartum, provided she is asymptomatic.

If symptoms exist (i.e. headaches) then immediate evaluation is recommended.

Research has shown that a very small percentage of gadolinium-based contrast is excreted into the breast milk and absorbed by the infant’s gut. The available data suggest that it is safe for the mother and infant to continue breast-feeding after receiving such an agent. That being said, out of an abundance of caution for the infant, the mother may wish to abstain from breast-feeding from the time of contrast administration for a period of 12 to 24 hours. The mother should express and discard breast milk from both breasts after contrast administration until breast feeding resumes. In anticipation of this, she may wish to use a breast pump to obtain milk before the contrast-enhanced study to feed the infant during the 24- hour period following the examination.