Head Shape Abnormalities

Learn what to expect during your infant’s development and when to be concerned about their head shape or head size.

Quick Facts

There are 3 main types of head shape abnormalities in babies: Positional plagiocephaly, Craniosynostosis and Syndromic craniosynostosis.
Doctors will perform a thorough exam to determine the issue. They may use medical imaging such as X-rays or CT scans as well as consult with other specialists.
Early evaluation is important to determine the cause and plan a course of treatment.
Timely treatment can address many cases of abnormal head shapes in babies, allowing for optimal growth and development.

Types of Head Shape Abnormalities

The main types of head shape abnormalities in babies are positional plagiocephaly, craniosynostosis and syndromic craniosynostosis.

Positional plagiocephaly (flat head syndrome)

Positional plagiocephaly is also known as flat head syndrome. This is the most common cause for head shape abnormalities in babies.

Positional plagiocephaly is a cosmetic flattening, often seen on the back of the head. It occurs when the baby’s skull bones are rearranged by an external cause. It most commonly develops when babies spend too much time on their backs, whether in a crib, car seat or stroller. If they lie in a particular position for extended periods of time, it can result in the skull flattening on one side of the head.

This is a non-life-threatening, non-surgical condition. There is nothing wrong with the underlying brain development, and the head shape does not affect long-term development or cause delays in developmental milestones.

Common causes of positional plagiocephaly

Infants who have a preference to turn their neck towards one side over the other
History of trauma or injury to the neck at birth
The use of vacuum or forceps during delivery, or any other reported birth trauma
If they have a preference to breastfeed from one breast over the other
Infants who do not like “tummy time” or do not spend very much time each day on their tummy
Young children or children with motor delays who are not yet able to roll or sit unsupported
Children who have head sizes on the higher end of the growth chart
Babies born early (prematurely), or who were hospitalized in the Neonatal or Pediatric ICU for a prolonged period
Abnormal in-utero positioning (such as breech position) or multiple-gestation pregnancy

Craniosynostosis

Craniosynostosis is a condition in which the sutures (joints) between the skull bones close prematurely. This can result in an abnormal head shape and restrict brain growth. If left untreated, it can cause increased pressure inside the head, skull shape deformities, and difficulty with vision.

Syndromic craniosynostosis

Children with this condition have skull fusion and face and limb deformities. This condition also affects the shape and placement of the eyes and the development of the jaw.

Syndromic craniosynostosis can lead to the following conditions:

Apert syndrome: Children with Apert syndrome have underdeveloped facial features, pressure in the skull, bulging eyes, and webbed fingers or toes.
Crouzon syndrome. Children with Crouzon syndrome may have skull fusion, underdeveloped facial features and mild bulging eyes.
Pfeiffer syndrome. Children with Pfeiffer syndrome may have crowded teeth, broad thumbs and toes, and an unusual forehead or nose shape.
Saethre-Chotzen syndrome. In addition to affecting the position of the eyes, Saethre-Chotzen syndrome can affect the position of the nose. Children with this condition can also have low hairlines and short or fused fingers.

Diagnosis

Early evaluation is important to determine the cause and plan a course of treatment. A physical assessment is the first step your pediatrician will take to help differentiate between craniosynostosis and plagiocephaly.

If a diagnosis cannot be made on physical exam alone, imaging can be used to obtain better pictures of the skull. The quickest and easiest imaging option is an X-ray which shows if the sutures have closed. At Riley Children's, a CT scan can also be utilized to provide images with a 3D reconstruction of the skull. CT scans show not only the bone and sutures, but also how the brain fits inside the skull. These images will show the Neurosurgery team if the sutures remain open, have closed or if there are concerns for early closure of the sutures.

Treatment

Treatment varies depending on the condition. Early recognition is important because it allows for more treatment options, like minimally invasive surgeries. Riley Children’s offers many advanced surgeries, such as endoscopic and open repair, distraction techniques, total vault remodeling and spring assisted craniotomy.

How to treat positional plagiocephaly

Repositioning therapy

Since positional plagiocephaly is caused by external forces, the best treatment is to start repositioning therapy at home as soon as possible. Your baby should lie on the side of the head that is not flattened. Once your baby spends more time off the flattened area and changes their head position more frequently, you will start to see improvement in the head shape as the brain and skull continue to grow.

The American Academy of Pediatrics recommends tummy time two to three times each day. Start with three to five minutes, then gradually increase the time and frequency.

As soon as they are able, help and encourage your baby to sit and play upright. Try to keep your baby off the back of the head as much as possible. Move toys and lights on the floor, play area or car seat to their less-favored side. This encourages them to lie on the non-flat side to see those items and helps to promote neck range of motion.

It is not recommended to stop Back to Sleep (AAP). Continue to place infants “back-to-sleep” for SIDS prevention, but allow more belly and upright time during their awake times while monitored by an adult.

Physical therapy

If your child has a preference to lay on one side of their head, it may be important for them to be evaluated by physical therapy or the Indiana First Steps Program.

Infants with torticollis have a condition where the neck muscles cause the head to twist to one side. Typically, these babies need physical therapy to stretch and straighten the neck. In these cases, head flattening typically improves through the treatment of torticollis.

Cranial molding therapy

Your child's doctor may suggest a special helmet in severe cases. Cranial molding therapy (also known as helmet therapy) helps distribute pressure on your baby’s head and prevents it from becoming flatter. This is a noninvasive form of management for severe plagiocephaly that can be considered after treatment failure with physical therapy and conservative management.

How to treat craniosynostosis

If a CT scan shows craniosynostosis, surgery to separate the skull bones is advised. This procedure relieves pressure in the skull so the brain can expand and grow to a normal shape. Doctors typically perform reconstructive surgery before the baby turns one.

How to treat syndromic craniosynostosis

Multiple surgeries are necessary to correct the condition as your child grows and develops. Once again, early treatment leads to the best outcomes.

Your Treatment Team

The Cleft and Craniofacial Anomalies Program at Riley Children's Health is highly skilled in performing these treatments and delicate surgeries. Your treatment team may consist of experts from these departments:

Neurosurgery: Our expert doctors who treat brain and spinal issues can address some conditions alone. These are usually the operations that occur before 3 months of age.
Plastic surgery: Assist with operations after 3 months of age and all forms of syndromic craniosynostosis.
Genetics: Certain conditions are linked to genetic issues, so patients and families may be referred for more tests to better understand their child’s condition.
Ophthalmology: The eye doctor examines vision, eye alignment and signs of increased brain pressure. If left untreated, this can cause vision loss.
Pulmonary: Some types of craniosynostosis can cause changes in the face and breathing passages. The pulmonary doctor helps assess and treat these conditions.

Support Services & Resources

Visit these websites for more information on head shape abnormalities:

We offer a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Children's Craniofacial Association

This nonprofit organization addresses the medical, financial, psychosocial, emotional and educational concerns related to head shape abnormalities, including craniosynostosis, Apert syndrome, Crouzon syndrome, Pfeiffer syndrome and Saethre-Chotzen syndrome.

FACES: The National Craniofacial Association

This nonprofit organization provides education and support for families of children living with craniosynostosis.

MedlinePlus

This U.S. government site explains the causes, symptoms, diagnosis and treatment of craniosynostosis.

Locations

In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.