Craniosynostosis
Craniosynostosis is a condition in which one or more of the sutures in a baby's skull fuse too early, affecting the shape of the head and, in some cases, normal brain growth. Early evaluation by a pediatric neurosurgeon helps determine which treatment approach is most appropriate.
Quick Answer
Craniosynostosis occurs when one or more of the fibrous joints (sutures) between the bones of a baby’s skull fuse too early. Because an infant’s brain grows rapidly during the first years of life, premature suture closure can change the shape of the head and, in some cases, affect normal brain growth. Treatment depends on which suture is involved, your child’s age, and the severity of the condition.
Key Takeaways
- Craniosynostosis occurs when one or more skull sutures fuse before the brain has finished growing.
- The condition changes the shape of a baby's head and may affect brain development if left untreated.
- Premature closure of different sutures produces different characteristic head shapes.
- Treatment depends on your child's age, the affected suture, and the severity of the condition.
- Early evaluation by a pediatric neurosurgeon provides the greatest range of treatment options.
Craniosynostosis at a Glance
| Type | Premature fusion of one or more skull sutures |
|---|---|
| Who it affects | Infants, usually diagnosed during the first year of life |
| Common signs | Abnormal head shape, a raised ridge over a suture, uneven forehead, or slowing head growth |
| Common types | Sagittal, coronal, metopic, and lambdoid craniosynostosis |
| Diagnosis | Physical examination and, when needed, low-dose CT imaging |
| Treatment | Endoscopic strip craniectomy or cranial vault remodeling, depending on the child's age, anatomy, and affected suture(s) |
| Long-term outlook | Most children experience excellent outcomes with early diagnosis and appropriate treatment |
Understanding Craniosynostosis
A baby’s skull is made up of several bones connected by flexible joints called sutures. These sutures normally remain open during infancy and early childhood, allowing the skull to expand as the brain grows rapidly.
Craniosynostosis occurs when one or more of these sutures fuse too early. Because the affected suture can no longer grow with the skull, the skull grows in other directions, resulting in an abnormal head shape. In some children, premature suture fusion may also increase pressure inside the skull and affect normal brain growth.
The Different Types of Craniosynostosis
The appearance of a child’s head depends on which suture closes prematurely.
Sagittal Craniosynostosis
Premature closure of the sagittal suture results in a long, narrow head shape (scaphocephaly).
Coronal Craniosynostosis
Fusion of one coronal suture causes flattening of one side of the forehead and elevation of the eye socket. When both coronal sutures are involved, the head may appear short and wide.
Metopic Craniosynostosis
Premature fusion of the metopic suture creates a triangular-shaped forehead (trigonocephaly) and may cause the eyes to appear closer together.
Lambdoid Craniosynostosis
The rarest form. It affects the back of the skull and can sometimes resemble positional plagiocephaly, making careful evaluation important.
Craniosynostosis vs. Positional Plagiocephaly
Many babies develop a flat spot on the back or side of the head from spending time in one position. This is called positional plagiocephaly and is much more common than craniosynostosis.
Craniosynostosis
Caused by one or more skull sutures closing too early. The abnormal head shape is related to how the skull grows around the fused suture and may require surgical treatment.
Positional Plagiocephaly
Develops when external pressure causes flattening of the skull while the sutures remain open. Treatment depends on the baby’s age and severity and does not involve surgery to open a fused suture.
Because the two conditions can sometimes look similar, evaluation by a pediatric neurosurgeon can help establish the correct diagnosis.
Why Early Evaluation Matters
Early diagnosis provides the greatest range of treatment options. Some infants may be candidates for minimally invasive surgery when diagnosed during the first few months of life, while older infants may benefit from different surgical approaches.
Prompt evaluation also helps ensure that babies with craniosynostosis receive the most appropriate treatment while avoiding unnecessary procedures for infants with positional plagiocephaly.
Signs & Symptoms
The most noticeable sign of craniosynostosis is an unusual head shape caused by one or more skull sutures closing too early. The specific shape depends on which suture is affected and may become more noticeable as a baby grows.
Some children may also develop changes in head growth or, in more severe cases, symptoms related to increased pressure inside the skull.
What Parents May Notice
- Abnormal or asymmetric head shape
- A raised ridge along a skull suture
- Flattening or prominence in one area of the head
- Uneven positioning of the eyes or ears
- Slower-than-expected head growth
- Changes in the size or feel of the soft spot
When to Seek Evaluation
- Your baby’s head shape seems unusual or becomes more pronounced over time
- You notice a firm ridge along a skull suture
- Head growth appears slower than expected
- The head shape does not improve with growth or repositioning
- You’re unsure whether the head shape may be craniosynostosis or positional plagiocephaly
Diagnosis and Evaluation
Diagnosing craniosynostosis begins with a thorough physical examination and review of your child’s medical history. In many cases, an experienced pediatric neurosurgeon can identify the affected suture based on your baby’s head shape and physical examination alone.
The evaluation helps determine whether the abnormal head shape is caused by craniosynostosis or a more common condition such as positional plagiocephaly, and whether additional testing is needed.
Physical Examination
Your pediatric neurosurgeon will evaluate:
- The shape and symmetry of your child’s head
- Head circumference and growth over time
- The position of the forehead, eyes, and ears
- Any raised ridges along the skull sutures
- Signs that more than one suture may be involved
Imaging Studies
Imaging is not always necessary. When additional detail is needed to confirm the diagnosis or plan treatment, your child’s care team may recommend imaging such as low-dose CT with 3D reconstruction.
Evaluating for Associated Conditions
Most children with craniosynostosis involving a single suture do not have an underlying genetic syndrome. When multiple sutures are involved or other findings suggest a syndromic form of craniosynostosis, your child’s care team may recommend additional evaluation or consultation with specialists.
Determining the Best Treatment
Treatment recommendations depend on your child’s age, the affected suture or sutures, the severity of the skull deformity, and whether there are signs of increased pressure inside the skull.
Early evaluation is important because your child’s age can affect which surgical approaches are available.
Treatment Options
Treatment for craniosynostosis depends on your child’s age, the affected suture, the severity of the skull deformity, and whether the condition is affecting normal brain growth or increasing pressure within the skull. Some infants benefit from early minimally invasive surgery, while others require a more traditional reconstructive approach.
The goal of treatment is to create adequate space for normal brain growth, improve the shape of the skull, and reduce the risk of future complications.
Endoscopic Surgery
Some infants diagnosed during the first few months of life may be candidates for endoscopic strip craniectomy, a minimally invasive procedure performed through small incisions.
Often followed by: Several months of custom helmet therapy to help guide normal skull growth.
Cranial Vault Remodeling
Older infants and children, or those with more complex forms of craniosynostosis, may benefit from cranial vault remodeling. During this procedure, the bones of the skull are reshaped and repositioned to create more space for the growing brain while improving the overall shape of the head.
May be recommended based on: Your child’s age, anatomy, and the specific sutures involved.
Long-Term Outlook and Follow-Up
Most children with craniosynostosis go on to have normal brain development and healthy, active lives. Outcomes are best when the condition is recognized early and treatment is tailored to the child’s age, affected sutures, and individual needs.
After treatment, your child will continue to be monitored as they grow. Follow-up visits allow the pediatric neurosurgery team to evaluate skull growth, neurological development, healing, and overall head shape. Children with syndromic or more complex forms of craniosynostosis may require additional monitoring or care from other specialists.
For most children, treatment is followed by routine long-term monitoring rather than ongoing surgical care.
The Diagnosis-to-Treatment Pathway
1. Evaluation
Your pediatric neurosurgeon evaluates your baby's head shape, growth, medical history, and physical examination.
2. Diagnosis
The affected suture is identified, and craniosynostosis is distinguished from positional plagiocephaly.
3. Imaging
Low-dose CT imaging or other studies may be obtained when needed to confirm the diagnosis and assist with treatment planning.
4. Personalized Treatment Planning
Your child's age, the affected suture, and the severity of the condition are considered when developing an individualized treatment plan.
5. Treatment
Depending on your child's age and diagnosis, treatment may include observation, endoscopic strip craniectomy, or cranial vault remodeling.
6. Long-Term Follow-Up
Regular follow-up visits monitor skull growth, healing, neurological development, and long-term outcomes.
Frequently Asked Questions
Craniosynostosis is a condition in which one or more of the joints (sutures) between the bones of a baby’s skull fuse too early. This changes the way the skull grows and can affect the shape of the head and, in some cases, normal brain growth.
Most cases occur sporadically, meaning there is no known cause. Less commonly, craniosynostosis is associated with a genetic syndrome or affects multiple skull sutures. Your pediatric neurosurgeon will determine whether additional evaluation is recommended.
Craniosynostosis occurs when a skull suture closes too early. Positional plagiocephaly is caused by external pressure on the skull and does not involve premature suture fusion. Because the two conditions can appear similar, evaluation by a pediatric neurosurgeon is important to establish the correct diagnosis.
Not every child requires surgery. Treatment depends on your child’s age, which suture is affected, the severity of the skull deformity, and whether normal brain growth may be affected. Your pediatric neurosurgeon will recommend the most appropriate treatment for your child.
Endoscopic strip craniectomy is a minimally invasive procedure performed through small incisions to remove the fused suture and allow the skull to grow more normally. It is typically performed during the first few months of life and is followed by helmet therapy.
Cranial vault remodeling is a reconstructive procedure that reshapes and repositions the bones of the skull. It is often recommended for older infants or children with more complex forms of craniosynostosis.
Helmet therapy is commonly recommended after endoscopic strip craniectomy to help guide normal skull growth. It is not typically required after cranial vault remodeling, although recommendations vary depending on the procedure and your child’s individual needs.
With early diagnosis and appropriate treatment, most children with craniosynostosis experience excellent outcomes. Many achieve normal brain development, participate fully in school and activities, and lead healthy, active lives.
Craniosynostosis Specialists at Atlantic Brain and Spine
Kate T. Carroll, MD, is a fellowship-trained pediatric neurosurgeon providing specialized evaluation and treatment for infants and children with craniosynostosis.
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