Introduction

Abnormal or asymmetric shape of an infant's head is a common reason for referral to a pediatric neurosurgeon. These anomalies are frequently noted by the child's parents, caretaker, or pediatrician, with a visit to the neurosurgeon frequently scheduled in the first months of life. The two most common diagnoses related to a child's head shape are craniosynostosis and deformational plagiocephaly, which are, respectively, inherited and acquired problems related to cranial sutures.

What are cranial sutures?

Because the child's head needs to squeeze through the birth canal during delivery, humans have evolved with a soft skull composed of many different bony plates that meet at regions called cranial "sutures". These sutures also allow the skull to grow rapidly during the first year of life, at which time the bones start to slowly fuse together.

What is craniosynostosis?

Craniosynostosis, or premature fusion of one or more cranial sutures, is a condition which affects about 1/2000 children. The most common suture to fuse early is the sagittal suture, followed by the coronal, metopic and lambdoid. The premature fusion impedes growth in the region of the suture. As a result, the child develops visible alterations of head shape. In rare instances (about 12%) neurologic abnormalities, such as increased pressure on the brain, develop as a result of suture fusion. Most patients with this condition do not have other, related medical problems. A small percentage have craniofacial syndromes such as Crouzon, Apert, Pfeiffer or Saethre-Chotzen. The standard treatment for craniosynostosis, with rare exceptions, is surgery on the child's skull within the first months to year of life.

What is deformational plagiocephaly?

Deformational plagiocephaly, also known as positional molding, is a much more common cause of head shape anomalies than craniosynostosis. It is caused by laying predominantly on one side of the soft, newborn skull. Like a fruit on a countertop, the area in contact will flatten out over time, causing characteristic changes that can potentially affect the entire skull. The incidence of plagiocephaly seems to have increased dramatically since the American Academy of Pediatrics has instituted the 'back to sleep' program. While the number of cases of sudden infant death syndrome (SIDS) has been reduced by 40%, we are seeing many more patients with flattening of the back of the heads. Treatment for plagiocephaly ranges from parental repositioning of the child in the crib during the first few months of life, to physical therapy if the baby has torticollis (stiff neck), to placing the child in a molding helmet to redirect the external forces on the infant's skull. At Children's Hospital in Boston, we are leaders in the field of craniofacial anomalies in children. Neurosurgery is an integral member of the Craniofacial team, which includes Plastic Surgeons, Oral/maxillofacial surgeons, Dentists and Speech Pathologists. We are innovators in the surgical repair of many craniofacial defects, and have also developed an extensive program for the non-surgical correction of deformational plagiocephaly. Depending on diagnosis, your child will be scheduled either in the Craniofacial Clinic, a joint clinic with many specialists, or directly in our Neurosurgery Clinic.

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