Introduction
This is a rare birth defect whereby the joints between the bones of an infant close prematurely before the brain is fully formed. It is a condition where a baby is born with an abnormal shaped skull. The premature closure of the baby’s bone sutures changes the skull’s growth pattern and this leads to abnormal head shapes and facial features. This condition causes a baby to be born with a deformed or an abnormal shaped skull. This skull deformity needs to be attended to since it causes problems with normal brain and skull growth in infants.
There are four main types of craniosynostosis. The most common type is sagittal synostosis (scaphocephaly). This type of craniosynostosis affects sagittal suture which is the main suture on the top of the head (Samwel 44). When the sutures close prematurely, instead of the head growing wide, it forces it to grow narrow and long. Babies with this type of craniosynostosis.have broad foreheads and it affect mainly boys. The second type of craniosynostosis is called the frontal plagiocephaly. This craniosynostosis affects the suture running from ear to ear on the top of the head. It involves the right or left side of the coronal suture which runs from ear to ear, it mostly affects girls than boys. The third type is lambdoid craniosynostosis, where the suture that runs at the back of the head cracks causing the area to flatten. Jimenez et al asserts that this is the rarest craniosynostosis of all (410). Lastly, there is trigonocephaly also known as metopic synostosis. This type of craniosynostosis is very rare and mainly affects males than females. This type of synostosis affects the sutures close to the forehead and ranges from mild to severe. Babies with this type develop a triangular sculp. Craniosynostosis can either be syndromic or non-syndromic.
Causes
The underlying cause of craniosynostosis is unknown although many experts have linked it to genetic factors. Syndromic craniosynostosis is caused by gene mutations. Gene mutations results from one of the bodily functions not working properly. Examples of genes that mutate and cause craniosynostosis are Twist, FGFR1, FGFR2, and FGFR3. Researchers have come up with a series of findings on the causes of non-syndromic craniosynostosis. One of the theories is that cell defects in the sutures lead them to close prematurely. Another theory is fetal constraints during pregnancy, when pressure is put on the baby’s head while in the womb during pregnancy, the sutures will be closed because the plates of the bones might be pushed together in the process. More research reveals that maternal smoking and exposure to amino-containing drugs can also cause craniosynostosis. Craniosynostosis occurs in one out of 2000 births and mainly affects males than female. There are syndromes that are said to cause craniosynostosis, they include; apert syndrome, chotzen, crouzon, carpenter and Pfeiffer syndromes.
Symptoms
The appearance of the shape of the head of infants affected by this condition is always noticeable. Infants with craniosynostosis have the following symptoms;
-Poor feeding
-They have a high pitched cry due to the pain the feel
-Increased irritability, they will always feel irritations in the affected areas
-They experience projectile vomiting
-They have unending seizures
-Slower growth rate of the head compared to the rest of the body
-Most of them have a bulging fontanel and distorted skull shape
Craniosynostosis can be diagnosed at birth or may be observed as the child grows (Coumoul and Deng 290). When a child has this condition, their development is very slow and others the head does not grow at all.
Treatment
The main typical recommended treatment for craniosynostosis is surgery. Surgery is needed to separate the fused bones in order to give the brain enough space to grow. Surgery is carried out by a two team of surgeons, the neurosurgeon who is a nervous system and brain specialist and a cranio-facial surgeon, a specialist who deals with face, head and jaws surgery. Barry et al states that surgery should be done to correct the head and face deformation in the infants (118). The surgery helps to reduce pressure on the brain and to create enough space in the child’s skull in order to allow the brain to grow properly. It also helps to improve the child’s head appearance since it is always deformed.
Surgery should be carried out in the first 12 months after a child’s birth. If a child is not treated, they will end up developing more skull complications and this can affect the functioning of the rest of the body. If not treated, it can also lead to vision loss and a child can also develop ICP (Inter-cranial pressure).
When surgery is done, the child grows normally as long as the condition is not in any way associated with any genetic syndrome that causes craniosynostosis. Craniosynostosis treatment is mainly based on a child’s age and overall medical and health history. It can also be based on the extent of craniosynostosis in the child and the child’s tolerance for certain medications. According to Panchal and Venus, after surgery, a child may experience fever, vomiting, headache, fatigue, redness and swellings in the incision areas (2035). The child will grow normally after a successful surgery has been done.
Conclusion
Craniosynostosis is a condition in which a baby is born with a skull that is abnormally shaped. Craniosynostosis can either be syndromic or non-syndromic. Babies with craniosynostosis can be treated by surgery being performed on them to reshape their skulls. If not treated, craniosynostosis has adverse effects and can lead to lead to vision loss and a child can also develop ICP (Intercranial pressure).
Works Cited
Samwel, Pruzansky. Craniosynostosis: Diagnosis, evaluation, and Management. New York: Raven Press, 1986. Print.
Barry, Jones, David, Dunaway, Robert, Hayward and Robert, Evans. The Clinical management of Craniosynostosis. Cambridge: Cambridge University Press, 2004. Print.
Coumoul, Xavier, and Deng, Chu-Xia. "Roles of FGF receptors in mammalian development and congenital diseases." Birth Defects Research Part C: Embryo Today: Reviews 69 (2003): 286–304. Print.
Jimenez, David, et al. "Endoscopy-assisted wide-vertex craniotomy, barrel stave osteotomies, and postoperative helmet molding therapy in the management of sagittal suture craniosynostosis." Journal of Neurosurgery (Pediatrics 5) 100 (2002): 407–17. Print.
Panchal, Jayesh, and Venus, Uttchin. "Management of Craniosynostosis." Plastic & Reconstructive Surgery 111 (2003): 2032–49. Print.
