craniosynostosis image

Craniosynostosis – the premature fusion of one or more of the cranial sutures – is a condition that can appear in infancy. Though it affects 1 in every 2,000 infants, the cause of the condition remains unknown. Craniosynostosis can cause significant deformities in the shape of the skull and, in severe cases, can even prevent proper brain development.

The only treatment available today for the repair of Craniosynostosis is surgical intervention by neurosurgical specialists. Herzliya Medical Center is a private hospital that is home to top Israeli neurosurgeons, among them leading experts in the treatment of Craniosynostosis in Israel and in the world, with many years of experience, who have successfully performed many of these operations.

What is Craniosynostosis?

The human skull is composed of 26 different bones. During the first two years of life, these bones remain flexible and dynamic to allow for the gradual development of the brain, which grows rapidly during this period. Essentially it is the rate of brain growth that determines the rate of skull growth, as well as its shape and appearance.

The flexibility of the bones of the skull is made possible by their natural separations at areas known as sutures and fontanels. The sutures are the areas where the different bones of the skull meet and the fontanels are the junctions of the various sutures. There are two main fontanels in the skull – the anterior fontanel and the posterior fontanel.

Over the first two years of life, as brain growth progresses rapidly (from an average head circumference at birth of 35 cm to a circumference of roughly 47 cm at 12 months), the connections between the bones – the sutures – are made out of soft tissue that ensures the skull flexibility. This tissue hardens gradually until it becomes solid bone and a continuous part of the skull.

Craniosynostosis the name for a range of conditions related to the development of the skull with a single common denominator: early and rapid fusion of one or more of the cranial sutures, which directly affects the normal development of the skull. Since the bones of the skull cannot develop properly at the location where the suture has fused prematurely, they “compensate” through increased growth and development at another location, resulting in deformities in the shape of the skull. Although the condition is mostly cosmetic and aesthetic, in severe cases it can also affect brain development.

To this day, it remains unclear why the condition develops. Sometimes, Craniosynostosis can appear as part of a known chromosomal aberration , but in many cases it occurs as a distinct entity with no apparent cause and not as a part of any syndrome.

In addition, Craniosynostosis usually is not a hereditary problem: even if a parent or sibling suffered from the condition as an infant, the chance that an additional family member will develop Craniosynostosis is very low.

Craniosynostosis is typically characterized by a visible deformity of the skull. The deformity is directly affected by the location of the prematurely fused suture such that in most cases, the child’s appearance alone is sufficient to determine the location of the fused suture.

  • A long, narrow skull indicates premature fusion of the sagittal suture (which traverses the skull from front to back).
  • A skull that is asymmetric when comparing the two sides indicates fusion of one of the coronal sutures (sutures at the side of the skull).
  • A short skull that is flat at the front and has a tendency to grow vertically indicates fusion of both coronal sutures.
  • A “triangular” skull – trigonocephaly indicates fusion of the metopic suture (located between the frontal bones which form the forehead).

The deformity can also be identified by measuring the child’s head circumference and comparing it to standard growth curves, allowing the condition to be detected even in the absence of a readily visible deformity. Note that in rare cases, Craniosynostosis can be accompanied by additional symptoms, such as:

  • Headache
  • Loss of appetite
  • Vomiting
  • Developmental delay

The final diagnosis can be confirmed by the medical team at Herzliya Medical Center, using the most advanced imaging technology. This cutting-edge imaging technology ensures accurate diagnosis of the condition and measurement of all parameters relevant for treatment.

In the majority of cases of Craniosynostosis, the treatment is surgical. Prof. Shlomo Constantini, a pediatric neurosurgical specialist, performs these operations, with assistance from his expert medical team, with whom he has been working in cooperation for many years.

Prof. Constantini is considered a world renowned neurosurgeon in the field of pediatric Neurosurgery and one of the world experts in performing neurosurgical procedures in infants and children, among them: brain and spinal tumors, congenital disorders of the brain and spine, and of course, repair of skull structure deformities in newborns, including Craniosynostosis. Prof. Constantini’s rich résumé includes thousands of pediatric neurosurgical operations, including a long list of Craniosynostosis operations of all types. He serves as a senior physician at the Neurosurgical Center for Excellence at Herzliya Medical Center, and has, for many years, served as the director of the Department of Pediatric Neurosurgery at one of the largest public hospitals in Israel.

The consensus is that surgery for the repair of Craniosynostosis should be performed at early age, typically before infant reaches eight months, both due to the fact that the bones of the skull are still relatively soft, and in order to avoid impaired brain development. The operation requires between two and six hours, depending on the particular characteristics of the condition, and is performed under general anesthesia.

There are several types of surgery for the repair of Craniosynostosis, depending on the location and number of prematurely fused sutures. In all of the operations, however, the surgeon operates on the skull only, without involvement of the brain tissues, while inserting absorbable plates or sutures at the site to ensure stability of the repair.

The main surgeries for the treatment of Craniosynostosis are as follows:

  • Surgery for the repair of the metopic synostosis (forehead) is typically performed for cosmetic reasons only. During the operation, the surgeon adjusts the shape of the skull after opening the fused suture and reshaping the fronthead and orbital rim. This operation is typically performed before the infant reaches six months.
  • Surgery to repair one of the coronal synostosis (side of the head) is performed in a similar manner and within the same age window and in the event that both coronal sutures have fused prematurely can include the repair of both simultaneously. Premature fusion of the coronal sutures is one of the only forms of craniosynostosis considered to risk normal brain development treatment.
  • Surgery to repair the sagittal synostosis (middle of the skull) typically requires only expansion of the suture. This is an operation typically performed at a very early age, before the child reaches of three and a half months.
  • Surgery to repair the lambdoid suture (back of the skull): This rare operation is typically performed by the age of 8 months, and is also considered to be a mainly cosmetic surgery. Note that it many instances, deformation of the skull occurs even in the absence of craniosynostosis, a condition that resolves itself over time with no need for surgery.
  • Surgery to simultaneously treat the premature fusion of multiple sutures: This condition can result in an increase in intracranial pressure and significant impairment to normal brain development and activity. Therefore, surgical intervention is considered to be urgent and is performed over a relatively wide range of ages.

The main goal is to create a familiar and soothing environment for your child. We recommend that you bring some toys that your child likes and clothing that he is used to wearing. Meeting the medical team and the anesthesiologist prior to the operation will also help, and will reduce the feeling of strangeness and nervous feelings that tend to arise.

Your child will be required to fast for four to six hours prior to the operation. The medical team will be with you throughout the entire process, from your child’s admission to the hospital, through the operation and during your child’s post-operational recovery as well as at the follow-up visits after. They will be available to answer any questions you might have and will be happy to help you with anything you need.

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