Definition: The term craniosnestosis is the name given to the early closure of the joints (sutures) in the skull during pregnancy before birth. Babies are generally born with a head circumference of 36 cm at birth. The head circumference of babies reaches 49 cm by the time they are 2 years old, as many sutures, which are the joints in the skull, or the fontanel area (fontanel) in the front and back, are open, joints are open and fibrotic. However, babies born with craniosynostosis are born with these open and fibrotic joints closed. This growth around the head allows the baby's brain volume to grow and the development of brain functions. Premature closure of the joints in the skull bone around the brain both hinders the baby's brain development and causes aesthetic or cosmetic deformities as a result of head deformity. This group of diseases, which fall under the title of skull, face, nose and forehead deformities, has two types: primary and secondary. Primary type ones occur due to hereditary (genetic) causes or the mother's use of teratogenic drugs (valporate) during pregnancy or unknown reasons. The secondary type is babies with blood and endocrine diseases, vitamin D deficiency or microcephalic (small head circumference). This secondary type is not a surgical disease; it is only diagnosed by determining the cause of the disease and treated medically. Primary type craniosynostosis or skull deformities is a surgical disease. When the skull joints close prematurely, apart from the deformity of the head, the intracerebral pressure increases depending on the number and location of the affected joints. Unfortunately, as the child grows, this increased brain pressure causes inadequate brain development, mental retardation or other brain damage. Among the most common skull deformities, premature closure of the joint in the middle of the skull (sagittal suture) is around 55% and is seen in 1/5000 live births, while the head appearance of these babies is elongated in front-back diameter. The second most common is trigonocephaly, with premature closure of the joint at the front of the skull (metopic suture), and the front part of the head and forehead area of these babies are triangular in shape and cannot be seen. Its frequency is seen in 1/7000 births. Thirdly, premature closure of the joint on the sides of the skull (coronary suture) occurs unilaterally or bilaterally, and the shape of the head here has a more tower-shaped skull appearance. More rarely, it is the unilateral or bilateral closure of the joints at the back of the brain (lambdoid suture). This type of craniosynostosis is very similar to the head shape, especially in babies who are laid down in the same position unilaterally, and their differential diagnosis is very important.
How is skull deformity diagnosed?
Diagnosis of craniosynostoses begins with a special examination by pediatricians, such as the child's head shape at birth, the patency of the fontanel, head circumference measurements, and fontanelle swelling. If there is an irregularity in the head shape and head diameter growth in the monthly child development follow-up, and if there is asymmetrical growth of the head both in the front and back, it is a suspicion of skull deformity. Head deformity and brain development, head circumference and fontanelle measurements should be compared very carefully with the pictures of the child after birth. In cases where skull deformity and craniosynostosis are suspected, the diagnosis and especially its type are confirmed by brain ultrasonography and brain 3D Computed Tomography.
What is the treatment for skull deformity?
There are two types of treatment, medical and surgical. While the secondary type of craniosynostosis or microcephalic babies are treated medically, the primary type is treated surgically.
The purpose of surgical treatment is to increase the intracranial pressure of the closed skull joints or the bones of the fontanelle area, thus preventing the volumetric and physiological development of the brain. In addition to the aim of correcting increased brain pressure, surgical treatment also has a cosmetic or aesthetic purpose: correction of head, face, nose and forehead deformities.
Skull deformity surgical treatment. timing;
1: Endoscopic surgical procedures within the first 3 months of life,
2: Sagittal synostosis vertex skull reconstruction within the first 3 months of life,
3: In frontal and other skull synostoses, 4-12 a years,
4: Hypertelorism surgery is at the age of 4-5,
5: For combined anomalies of the skull, nose, face and forehead, craniofacial advancement surgery is at the age of 5-7.
Conclusion skull deformities (craniosynestoses) are a relatively common disease in the newborn and infancy period. The diagnosis of this disease is sometimes made by mothers who suspect the shape of their babies' heads. Generally, during the monthly follow-up of the babies, the Pediatrician suspects head deformity or lack of head diameter growth. A definitive diagnosis is made by the Neurosurgeon's Neurological and physical examination as well as imaging tests and 3D Computed Tomography examination.
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