The premature fusion of the sutures of the bones of the skull is termed as craniosynostosis. Early fusion of the sutures distorts and restricts the growth of the skull. An increased cranial pressure growth is caused by the cranial growth restrictions that can even result in impede intellectual development and vision problems. Also abnormal shape of the head cause behavioral problems and is also connected with low self-esteem. The treatment of these deformities may ease cranial pressure.
Craniosynostosis always appear in isolation and is also present in some syndromes that include Crouzon, Carpenters, Saethre-Chotzen, Apert, Opitz-C and Pfeiffer.
Types of Craniosynostosis
Craniosynostosis can be categorized into many types. These types are known by different names that depend upon on which suture is involved. This include –
Saggital Synostosis (Scaphocephaly): Scaphocephaly is the premature fusion of the saggital suture. A head which is narrow from ear to ear and is elongated from front to back is termed as Scaphocephaly. The condition of Scaphocephaly can be corrected with the help of different techniques. The purpose of every treatment is to obtain a more normal shape that includes making the skull wider from side to side and shortening the skull from front to back. This enables for more normal growth. Techniques used for each patient is different. Endoscopic approach or Calvarial Vault Remodeling (CVR) surgery can also be used.
Metopic Synostosis (Trigobocephaly) : This type is the fusion of the forehead (metopic) suture. This suture travels through the top of the head reaching down the middle of the forehead, near to the nose. Its early closure can cause major ridge running down the forehead. In this the forehead looks pointed similar to a triangle having closely placed eye.
Coronal Synostosis (Plagiocephaly): This type includes fusion of either left or right side of the coronal suture running from ear to ear. This is termed as coronal synostosis that leads to normal forehead but stops the growth of the brow.
Unicoronal Craniosynostosis: When there is a premature fusion of one of the coronal sutures then it causes asymmetric forehead and brow. This is considered as the second most common type of synostosis. As compared to right, left side unicoronal synostosis is more common.
Symptoms of Craniosynostosis
- Increased pressure inside the skull such as intracranial pressure
- A distorted skull having the shape that depend upon which of the cranial sutures are affected
- Development of a hard or raised ridge near the affected sutures
- No or slow growth of the head as the baby grows
- Abnormal disappearing or feeling of a soft spot on the skull of the baby
Causes of Non-Syndromic Craniosynostosis
Non-syndromic craniosynostosis has no known cause but is associated with few theories. The presence of a cell defect in the sutures can cause early fusing. According to second theory, when a child in the womb assumes an irregular position if the pressure is placed on the head of the baby. Due to this, bone plates can be pushed together may cause the sutures to fuse.
Causes of Syndromic Craniosynostosis
Syndromic craniosynostosis can be caused by genetic mutations. Some of the examples of genetic mutations include-
- FGFR2
- TWIST
- FGFR1
- FGFR3
Diagnosis of Craniosynostosis
Genetic testing: If the misshapen skull of the baby is due to hereditary syndrome, then genetic testing may help in recognizing the syndrome. A blood sample is generally required for performing genetic tests. A doctor may take sample of baby’s skin, hair or other tissue depending upon the type of abnormality.
A physical exam: The head of the baby is examined for seeing any abnormalities like suture ridges. A doctor also examines facial deformities.
Imaging studies: Computerized tomography (CT) or x-rays are performed for examining the skull of the baby. This test will show whether or not any sutures have fused.
Treatment of Craniosynostosis
The treatment depends upon following conditions –
- Type of craniosynostosis
- The age, overall health and medical history of the child
- The power of tolerance for particular procedures and medications
- Extent of the craniosynostosis
The only treatment for craniosynostosis is surgery. Open surgery is performed for reshaping the skull of the child. An incision is made at the top of the head. Then the affected suture is removed by a neurosurgeon. After this, the skull bones are given a more normal shape by a craniofacial plastic surgeon. Screws and plates are used for holding the new shape till the time bone heals. The screws and plates are made up of special material that absolutely dissolves in 1-2 years. Only one surgery is required if a baby has single suture craniosynostosis. More than one surgery is required if a child has craniosynostosis caused by craniofacial syndromes.
Endoscopic strip craniectomy
If only lambdoid or sagittal suture is fused, then a baby has other option. In this option, a strip of the bone is taken out along the top of the skull by the means of small incisions. A baby is required to wear a helmet for at least 3 months after the surgery in order to mold the head into a more normal shape. Benefits of endoscopic strip craniectomy include –
- Shorter hospital stay
- Less scarring
- Less time for performing the surgery
- Less bleeding that reduces the chance of a blood transfusion
Cost of Craniosynostosis treatment
Craniosynostosis in India is very good option due to multiple benefits; the surgery is done very safely and also at a very less cost in India. India is renowned all the world for its medical infrastructure, advanced treatment facilities and expert surgeons that makes surgery very comfortable and successful.