Craniosynostosis is the early closure of one or more bones in the skull from the seam (suture). The skull consists of five separate plates. Craniosynostosis limits the growth of these discrete bones and causes plates to fuse and form a ridge. It is recommended to see a specialist for an examination as soon as possible. There are different types of craniosynostosis: sagittal, coronal, metopic, and lambdoid. Infants diagnosed with craniosynostosis usually require surgical intervention and should be urgently seen by a pediatric neurosurgeon or craniofacial specialist for further examination and treatment. Early diagnosis can be vital.
Active STARband helmets are preferred to correct the deformation after the sutures have been released in surgery. STARband helmets must be used as a continuation of the treatment after endoscopic craniectomy surgery. This less invasive technique, which requires about 45 minutes of surgery time, is performed without opening the skull completely and allows the patient to be discharged the next day.
All over the world, active STARband helmets are the preferred devices to use after endoscopy-assisted (closed) surgery. STARband helmets are also the most trusted devices due to their results in correcting the skull. Active STARband helmets may also be needed, although rarely, in open CVR surgery for proper skull growth and protection purposes or when adequate correction is not achieved with surgery.
Often the cause of the craniosynostosis is unknown, but in some cases, it is based on genetic disorders.
Non-syndromic craniosynostosis is the most common type of craniosynostosis. It occurs every two to three thousand births (1/2000-1/3000). Moreover, single suture closure is seen mostly in non-syndromic craniosynostosis. Although the cause is unknown, it is thought that it occurs as the result of a combination of genetic and environmental factors. Non-syndromic, single suture synostoses are divided into four and their frequency rates are as follows:
• 40-55% Sagittal Suture Synostosis (Scaphocephaly)
• 20-25% Coronal Suture Synostosis (Anterior Plagiocephaly)
• 5-15% Metopic Suture Synostosis (Trigonocephaly)
• 1-5% Lambdoid Suture Synostosis (Posterior Plagiocephaly)
Syndromic craniosynostosis occurs due to certain genetic syndromes that affect the skull development of infants, such as Apert syndrome, Pfeiffer syndrome or Crouzon syndrome. In general, more than one suture is closed. A small percentage of those diagnosed with craniosynostosis are syndromic. Syndromic craniosynostosis cases are usually diagnosed early by both family and health care professionals compared to non-syndromic, due to their genetically significant characteristics. Frequency rates in birth are as follows:
• Apert Syndrome 1/60,000
• Crouzon Syndrome 1/25,000
• Pfeiffer Syndrome 1/100,000
• Jackson-Weiss Syndrome Not Known
• Muenke Syndrome 1/30,000
• Saethre-Chotzen Syndrome 1/25,000-1/50,000
• Carpenter Syndrome 1/1,000,000
Craniosynostosis is usually recognized at birth. However, a few months after birth, it becomes much more apparent. It may also be noticed months later by the family or health care professionals in cases of a partial closure of the suture. It can also be noticed during prenatal monitoring, such as an ultrasound.
The first sign of craniosynostosis is a head-shaped abnormality. The skull takes its shape depending on which suture is closed. Especially in simple synostosis, which is more common and where the single suture is closed, the skull continues to grow towards the side where the sutures are open. It causes significant deformity in the head. Cases of craniosynostosis are easy to distinguish because they show certain head shape features. Depending on the type of craniosynostosis, facial asymmetry shows itself in certain forms as protrusions or flatness of the forehead or behind the head. Normal head circumference development can be observed in measurements of skull growth. Therefore, even if the head shape deformity and facial asymmetry are noticed, it is thought by some that these deformities will improve in time. This line of thinking is a misconception by some. It should also be noted that, no growth or slow development of the head is a sign of craniosynostosis and occurs especially in multiple suture closures.
In addition, deformational head shape abnormalities improve with the start of repositioning. However, the head shape deformity in infants with synostosis does not respond to traditional repositioning. Deformation continues to increase day by day. Sometimes sutures close after birth. The best way to monitor these babies is through frequent quantitative and visual comparison of the head shape from various aspects with the STARscanner and the SmartSoc 3D scanning systems. Along with asymmetry of the head, the severity and progression of facial asymmetry can be monitored. The STARscanner and the SmartSoc are non-invasive. They only take the surface of the head shape in 3D without going under the skin. Unlike tomography and MRI, no matter how many times a scan is taken, there is no harm done to the infants. With the use of STARscanner and SmartSoc systems, infants do not need to be exposed to radiation and put to sleep with anesthesia.
Another sign is the obvious height that is felt when the closed suture is touched. Two skull plates push together to form a hard ridge.
In addition to these symptoms in the suture or sutures, another sign is the absence of an anterior fontanel.
If it is left untreated, craniosynostosis causes permanent deformations in the skull and face. Moreover, there may be psychological and social effects such as poor self-confidence and social isolation.
If the head shape and suture are addressed with surgery, there is no risk of intracranial pressure with simple craniosynostosis. On the other hand, in infants with complex craniosynostosis (especially in infants with the underlying syndrome), the pressure inside the skull may increase if the brain does not have enough space to grow. If increased intracranial pressure is not treated, it may cause:
• Developmental delay
• Impairment of apprehension
• Lack of energy or interest
• Impaired eye movement
• In rare cases, it may cause death.
Lance S. Governale, MD. Craniosynostosis. Published:July 22, 2015 DOI:https://doi.org/10.1016/j.pediatrneurol.2015.07.006
Mark. S. Diaz et al. Identifying the Misshapen Head: Craniosynostosis and Related Disorders. Pediatrics (2020) 146 (3): e2020015511. https://doi.org/10.1542/peds.2020-015511