Muenke Syndrome Treatment

Muenke syndrome is the most common craniosynostosis syndrome, occurring in approximately one in 30,000 births. It primarily affects the bones of the skull.

Cause of Muenke Syndrome

Muenke syndrome is caused by a specific gene mutation. In most cases, this mutation arises randomly. We do not yet fully understand what causes this mutation. However, it does not appear that the mutation occurs as a result of something either parent does or fails to do. Most parents who have a baby with Muenke syndrome likely have normal genes.

Children with Muenke syndrome can pass the condition on to their children, however. Only one copy of the gene needs to be passed on for the new baby to have this syndrome.  A parent with Muenke syndrome has a 50-50 chance of having a baby that also has this condition. Anyone with Muenke syndrome who wishes to have children should consider meeting with a geneticist in order to discuss the risks and make a thoughtful, informed decision.

Characteristic Traits

Children with Muenke syndrome characteristically have fusion of one or both of the coronal sutures – specialized expansion joints in the skull which allow rapid growth of the front part of the skull in the first years of life. In 70 percent of cases, children with Muenke syndrome have both coronal sutures fused. In the remainder of cases, only one of the sutures is involved.  Fusion of one or both of the coronal sutures leads to restricted skull growth and a very characteristic skull shape. 

In addition to coronal craniosynostosis, a small number of children with this syndrome may also have hearing loss related to the sensory nerves.

Diagnosis

Most often, the diagnosis of Muenke syndrome is suspected based on an abnormal skull shape and a diagnosis of coronal craniosynostosis. The suspected diagnosis is confirmed by a blood test to check for a gene mutation. We recommend that all children with coronal craniosynostosis see a geneticist to evaluate for Muenke syndrome.

Surgical Treatment

The treatment of Muenke syndrome is focused on correction of the abnormal skull shape and mirrors the treatment of coronal craniosynostosis. Surgical correction is most often done between 6 and 12 months of age.

Following surgery, your baby will spend at least one night in the pediatric intensive care unit prior to being transferred to a pediatric bed. You can anticipate your baby will spend anywhere from 2-5 days in the hospital following surgery.