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Pediatrics

Types of Craniofacial Disorders & Deformities

Different types of craniosynostosis are:

Sagittal synostosis

Sagittal synostosis is the most common type of craniosynostosis. In this type, the anterior or posterior portion of the sagittal suture closes prematurely;  the resulting compensatory growth causes frontal bossing. This causes your child’s skull to become long and narrow. This is sometimes called scaphocephaly.

Coronal synostosis

Coronal synostosis is the second most common type of synostosis. In this type, one or both of the skull’s coronal sutures closes prematurely, resulting in head and facial asymmetry that gives an infant a wide skull with a forehead that is flat and tall. Your doctor or surgeon will call this shaped head brachycephaly. Surgery is required to open the fused sutures, reshape the head and allow for normal brain and skull growth.

Metopic synostosis

Metopic synostosis is a premature closure of the metopic suture, resulting in a growth restriction of the frontal bones—which leads to a skull malformation known as trigonocephaly. This gives an infant a forehead that often looks pointed or triangular from above. Sometimes you may be able to feel a ridge in the middle of the forehead. Metopic synostosis also leads to facial abnormalities such as hypotelerism, resulting in a decrease in distance between the eyes. The goal of the surgery is to open the suture that is closed and to restore the natural shape of the forehead.

Lambdoid synostosis

Lambdoid synostosis is one of the rarest forms of craniosynostosis. Unilateral lamdoid synostosis results in a flattening of the back of the head on the affected side, as well as compensatory growth of the mastoid process on the same side (ipsilateral mastoid bulge). This leads to a characteristic and unique “tilt” in the cranial base. This differentiates it from positional plagiocephaly, which is an abnormal flattening of the back of the head due to position of the child and not premature closure of suture. Positional plagiocephaly is treated primarily with repositioning of the child and surgery is only required if the lambdoid suture is closed or fused.

Syndromic Craniosyntosis

In some cases craniosynostosis is inherited and part of a genetic syndrome. Though these are extremely rare, an evaluation by a geneticist will help identify if your child has any of these syndromes.

  • Apert Syndrome
    A congenital condition, Apert Sydrome is a defect that occurs in approximately 1 out of every 160,000 to 200,000 live births. Though it can be inherited from a parent with Apert’s Syndrome, it may also be the result of a spontaneous mutation. Apert syndrome is characterized by specific malformations of the skull, midface, hands, and feet. The skull is prematurely fused and unable to grow normally; the midface appears to be sunken.  If your child is diagnosed with Apert’s syndrome, treatment begins right away and continues for many years. Your child may be treated by a craniofacial team that includes a neurosurgeon, plastic surgeon, orthodontist, speech pathologist and audiologist.
  • Crouzon Syndrome
    In Crouzon’s Syndrome, the bones of the skull and face fuse abnormally causing an abnormal skull shape with changes in the facial bones, especially around the eyes and cheeks. There are often jaw problems. The cheeks may appear flat and the eyes may appear too prominent and in some cases, bulging. Crouzon’s syndrome unlike most craniosynostosis syndromes does not involve abnormalities of the hands and feet. In addition, it is common for those with Crouzon’s syndrome to have cervical spine abnormalities, or in some cases, subtle elbow, hand, musculoskeletal or internal organ anomalies. Crouzon syndrom’s is rare, affecting only about 4.5% of craniosynostosis patients. Read more. 
  • Pfeiffer Syndrome
    Linked to two different genes, Pfeiffer’s Syndrome is a type of a craniosynostosis in which children present with wide thumbs and large toes, as well as partially webbed fingers and toes. Children with Pfeiffer’s Syndrome often suffer from hearing loss, as well. Pfeiffer’s Syndrome causes several skull sutures to prematurely fuse, which results in abnormal growth of the skull and face.  Pfeiffer’s Syndrome has been broken down into three subtypes. The subtypes are not always clear as they are based on physical findings. The most common and mildest of the three is type I. Children with Type I usually have normal intelligence. However, Type II and Type III, the more severe cases, cause the most serious medical problems.  Skull shape differentiates Type II and Type III with Type II children presenting a “cloverleaf” skull. Both Types II and III are also associated with a greater risk of early death in childhood. If diagnosed with Pfeiffer syndrome, care begins at birth. This includes multiple operations and treatment in a medical center with a craniofacial team.

Deformities

Positional Plagiocephaly

Positional Plagiocephaly or positional head deformity, is when the back of the head is flattened with corresponding facial asymmetry secondary to prolonged positioning of a child on his/her back. Positional plagiocephaly is treated primarily with repositioning the child so that the non-flat side of the head is placed dependent against the mattress and encourage supervised “tummy time” on firm surfaces when the infant is awake. Rarely, and in severe cases, positional plagiocephaly may require treatment with helmet therapy.

Torticollis

Torticollis can also cause positional head deformity. Torticollis is when a child holds his/her neck towards one side. This tilted position can cause changes to the child’s face and head shape overtime. Physical therapy is often used to help correct abnormal neck position.