Almost all children undergoing skull-remodeling surgery for craniosynostosis require blood transfusions. Studies have shown that the average amount of blood lost during craniosynostosis repairs can sometime approach a child’s total blood volume. A recent study, examining the most serious potential complications of craniosynostosis surgery estimated that the risk of a child not surviving the operation is roughly 0.1%, or about1 in 1,000. While it is likely that these risks are probably slightly lower for teams that do more of these operations (and slightly higher for those that do fewer operations), one thing appears clear: the most common cause of reported deaths is directly related to excess blood loss. At our center in Dallas, we believe that a focus on blood loss can further improve the safety of these operations. One of the many ways we do this for our own patients is to increase the amount of blood in their body before the operation is performed.

Erythropoietin is a hormone made in our kidneys that acts a messenger to ramp up production of red blood cells in the bone marrow. When this hormone (called Procrit in the U.S.) is given as injections, it stimulates the body to make more red blood cells. In 2002, our center published a study that showed that children who received Procrit before craniosynostosis surgery only required blood transfusions about 50% of the time, whereas those children who did not receive Procrit had an almost a100% transfusion rate. We, and a few other centers around the world, have been recommending that infants receive Procrit before surgery. However, like any other medication, there are both risks and benefits associated with getting Procrit. Complications reported with this drug include a process called pure red cell aplasia, which is an allergic reaction causing the body to stop making blood. This complication has been linked to a substance that used to be a component of the stoppers in bottles specifically made in Europe; however, to my knowledge this complication has never been reported to occur in a child. Other complications include a faster growth of cancer cells (but this drug has never been reported to cause cancer) and a higher incidence of heart attacks and strokes in older patients with diabetes. Finally, Procrit has been shown to cause an increased risk of developing blood clots in the legs during surgery (going from 2% to almost 5%, for adults undergoing spine surgery). It appears that the primary risk of Procrit is related to this drugs ability to make the blood more likely to form blood clots in adults. For these reasons, the FDA recommended that Procrit have a “black box” warning label. However, studies suggest that children seem to be protected against developing these blood clots in their legs during surgery. In fact, we believe that if the blood is more likely to clot in infants getting Procrit, this could actually be beneficial during skull surgery, by limiting blood loss. In 2012, we participated in a 3 center study (Pittsburgh, Dallas, UCLA) looking at 369 children who received Procrit for craniosynostosis surgery and found that none of these children showed any signs of complications from blood clots, which provides some reassuring safety data.

The ability to prevent a child from receiving someone else’s blood during surgery obviously eliminates the risks associated with these types of blood transfusions. Most parents worry about their child getting HIV, or AIDS, from blood transfusions. However, the risks of a child becoming infected with a viral infection are extremely low. The more common risks associated with blood transfusions are: transfusion reactions and a serious lung injury called TRALI. In addition, there are studies that have suggested that the the risk of developing a surgical infection may be higher for those receiving a blood transfusion (the theory is that getting someone else’s blood lowers the strength of the immune system).

We believe that starting an operation with a higher blood level places a safety net under the child; this is because the child will have more blood to lose before putting him, or her, at that ultimate risk. However, as children get older than 18-months of age, their total blood volumes are larger and the benefits of Procrit seem become less; therefore, we typically do not recommend giving this drug to older todlers. With every decision surgeons make there are risks associated with doing that treatment and risks of not doing that treatment.  Doctors need to educate themseleves as much as possible about both sides and use their best judgment to chart the safest course for each individual patient. At the current time, we believe that the evidence shows that it is better to give Procrit before infant skull surgery than to not give it.

For a further discussion on this topic you can email, write or call Dr. Fearon:

Jeffrey A. Fearon, MD
The Craniofacial Center
7777 Forest Lane, Suite C-700
Dallas, Texas 75230