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Craniosynostosis is a condition that can occur in newborns when one or more of the fibrous connections, called cranial sutures, that connect the bones of the skull close prematurely before the baby’s brain fully develops. The closure of these sutures can cause the baby’s head to appear misshapen as it continues to grow. In normal circumstances, the cranial sutures are flexible during infancy, allowing the skull to expand as the brain develops. The meeting point of these sutures is known as a fontanel, which is a soft region located on top of the head. There are several fontanels located throughout the skull, with the anterior fontanel found directly behind a baby’s forehead, followed by the largest one being the posterior fontanel located at the back of the skull. The skull also features two smaller fontanels on each side.
Multiple suture craniosynostosis refers to the premature fusing of more than one of the sutures in a baby’s skull, which differs from the more common premature fusion of just one cranial suture. Syndromic craniosynostosis is an uncommon kind of craniosynostosis that is brought on by specific hereditary disorders.
Surgery is used to treat craniosynostosis and restore the head’s normal form while promoting brain growth. Early detection and therapy provide your baby’s developing brain enough room to grow and develop.
Though severe cases of neurological impairment may result, the majority of kids recover from surgery with satisfactory cosmetic results and normal cognitive development. Early detection and intervention are essential.
Symptoms
Usually present at birth, craniosynostosis symptoms intensify in the first few months of your baby’s life. The number of sutures that are fused and the stage of brain development at which they happen determine the symptoms and severity. Among the warning signs and symptoms are:
- A skull that is malformed, with the shape varying depending on which sutures are troubled.
- The formation of an abnormally altered skull shape and a high, hard ridge along the damaged sutures.
Types of craniosynostosis
There are different types of craniosynostosis, each presenting in a distinct form. One cranial suture is typically fused in the majority of cases. The fusion of several sutures occurs in some complex cases of craniosynostosis. Syndromic craniosynostosis, which refers to multiple suture craniosynostosis, is frequently connected to hereditary disorders.
Depending on which sutures are impacted, different types of craniosynostosis have different names. There are various forms of craniosynostosis.
- Sagittal (scaphocephaly). The sagittal suture, which runs from the front to the rear at the top of the skull, fuses prematurely, causing the head to become long and narrow. Scaphocephaly is the term for this head shape. The type of craniosynostosis that occurs most frequently is sagittal.
- Coronal. The forehead may flatten on the affected side and protrude on the unaffected side as a result of premature fusion of one of the coronal sutures (unicoronal) that run from each ear to the top of the skull. Additionally, the affected side develops an elevated eye socket and a turned-of nose. Bicoronally fusing both coronal sutures causes the head to seem small and wide, with the forehead frequently tipped forward.
- Metopic. The metopic suture extends from the upper part of the nasal bridge, traverses the centerline of the forehead, and continues towards the anterior fontanelle, where it intersects with the sagittal suture. Premature fusion widens the back of the skull and gives the forehead a triangular shape. Trigonocephaly is another name for this head shape.
- Lambdoid. A rare kind of craniosynostosis called lambdoid synostosis affects the lambdoid suture, which runs along the back of the head. It may result in a baby’s head seeming flat on one side, one ear being higher than the other, and the top of the head tilting to one side.
Other head misshapen reasons
In addition to craniosynostosis, there are other factors that can contribute to abnormal head shape in infants. For example, spending too much time lying on one side of the head can cause flattening of the back of the head. To address this issue, regular repositioning or, in severe cases, helmet therapy (cranial orthosis) may be recommended to help reshape the head and restore a more balanced appearance.
During routine check-ups, your child’s healthcare provider will typically monitor the size and shape of their head. If you have concerns about your baby’s head size or shape, it’s important to bring it up with your pediatrician. They can provide further evaluation and guidance on appropriate treatment options if necessary.
Causes
While the exact cause of craniosynostosis is often unknown, genetic issues can sometimes play a role.
- Nonsyndromic craniosynostosis is the kind of craniosynostosis that is most typical. Although the exact etiology is unknown, it is likely a result of both genetic and environmental factors.
- Syndromic craniosynostosis is caused by specific genetic abnormalities that might disrupt a baby’s skull growth, like Apert syndrome, Pfeiffer syndrome, or Crouzon syndrome. These syndromes frequently contain additional physical characteristics and medical issues.
In rare instances, certain circumstances during pregnancy raise a baby’s likelihood of having craniosynostosis. These consist of:
- Drugs for fertility issues, including clomiphene citrate.
- Having thyroid disease while pregnant is known as maternal thyroid disease.
Diagnosis
Specialists who do plastic and reconstructive surgery or pediatric neurosurgery must assess craniosynostosis. The following symptoms could indicate craniosynostosis:
- Physical exam. Your baby’s doctor will feel the suture ridges on its head and look for other facial anomalies like imbalanced features.
- Imaging studies. Your baby’s skull can be imaged using a Computerized Tomography (CT) scan or Magnetic Resonance Imaging (MRI) to see whether any sutures have fused. It’s possible to employ cranial ultrasound imaging. Because they become invisible when fused, fused sutures can be recognized by their absence or by a ridging of the suture line. To take exact measurements of the skull shape, laser scans and photographs may also be employed.
- Genetic testing. Genetic testing may help discover an underlying genetic condition if your healthcare professional suspects one.
Treatment
Treatment for mild cases of craniosynostosis may not be necessary. If the cranial sutures are open and the skull is misshapen. Your doctor may suggest a specially designed helmet to help reshape your baby’s head. The molded helmet in this case can help your baby’s brain develop and correct the shape of the skull.
Surgery, however, is the main form of care for the majority of infants. The kind of craniosynostosis and whether an underlying hereditary abnormality exists determine the kind of surgery to be performed and when. Multiple operations are occasionally necessary.
Surgery is performed to improve your baby’s look, minimize or avoid pressure on the brain, make space for the brain to develop normally, and correct the head shape. Both a planning procedure and surgery are involved in this.
Surgical planning
Imaging investigations can aid in the planning of surgical procedures by surgeons. High-definition 3D CT scans and MRI scans of your baby’s skull are used in virtual surgical planning for the treatment of craniosynostosis to create a customized, computer-simulated surgical plan. To help with the process, specific templates are built based on the virtual surgical plan.
Surgery
The procedure is often carried out by a team that consists of a neurosurgeon (a specialist in brain surgery) and a craniofacial surgeon (a specialist in head and face surgery). Endoscopic or open surgery can be used for surgery. Both treatments have a low risk of complications and typically generate extremely satisfactory cosmetic results.
- Endoscopic surgery. Babies up to 6 months old may be candidates for this minimally invasive procedure. Early surgery is preferred. To enable the baby’s brain to develop normally, the surgeon removes the problematic suture using an endoscope, a lit tube with a camera that is introduced through tiny scalp cuts (incisions). A smaller incision, a one-night hospital stays, and frequently no blood transfusion are all advantages of endoscopic surgery over open surgery.
- Open surgery. Open surgery is typically performed on infants older than 6 months. The afflicted area of the skull is then reshaped after the surgeon makes an incision in the scalp and cranial bones. Absorbable plates and screws are used to fix the skull position. A three-to-four-day hospital stay is customary following open surgery, and blood transfusions are frequently required. The treatment is often one-time, but in complicated situations, the baby’s head shape may need to be corrected through a series of open surgeries.
Helmet therapy
Following a minimally invasive procedure, periodic office visits are required to fit your baby with a succession of helmets that will help form his or her skull. Depending on how quickly the form reacts to treatment, the surgeon will decide how long helmet therapy should last. In most cases, no helmet is required after open surgery.
