Craniosynostosis: Causes, Symptoms, Diagnosis, Treatment, and Prevention
Baby's skull shape is affected by craniosynostosis, a condition in which sutures (the joints between the skull bones) fuse too early, before the brain is fully developed. As a result, the head may have an abnormal shape and, in some cases, brain function and growth can be affected.
Causes of Craniosynostosis
The exact cause of craniosynostosis is unknown, but it may be related to genetic factors, environmental factors, or a combination of both. Occasionally, babies are born with craniosynostosis as part of a genetic syndrome, such as Apert syndrome, Crouzon syndrome, or Pfeiffer syndrome, which can also cause physical or developmental problems. It is also possible for babies to develop craniosynostosis from random mutations in genes, or from external factors such as maternal smoking, infections, or medications during pregnancy.
Symptoms of Craniosynostosis
There is a misshapen head as the main symptom of craniosynostosis, which may become more apparent at birth or in the first few months of life. The shape of the head depends on the affected suture. As an example, a fused sagittal suture (which runs from the front of the head to the back) may result in a long and narrow head. The forehead may be flat on one side and bulging on the other if one of the coronal sutures (the ones that run from the ears to the top of the head) is fused. Other symptoms may include:
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A raised, hard ridge along the affected suture
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A small or absent soft spot (fontanel) on the top of the head
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A difference in the size or shape of the eyes or ears
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A facial asymmetry or deformity
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A delayed or impaired development of motor skills, speech, or cognition
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A headache, vomiting, irritability, or vision problems due to increased pressure in the skull
Diagnosis of Craniosynostosis
A doctor can diagnose craniosynostosis by examining the baby's head and face, as well as measuring the circumference and comparing it to normal growth charts. To confirm the diagnosis and assess the severity of the condition, the doctor may order imaging tests, such as X-rays, CT scans, or MRI scans. In addition, the doctor may perform genetic tests to identify underlying syndromes or mutations.
Treatment of Craniosynostosis
Although surgery is the main treatment for craniosynostosis, not all affected children will require it. Several factors influence the decision whether or not to perform surgery, including the type and severity of craniosynostosis, the child's age and health, as well as the potential risks and benefits. Surgery is done to correct the head shape and create enough space for the brain to grow normally. Surgery is usually done within the first year of life, when the skull bones are still soft and flexible. The surgery may involve:
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Making an incision in the scalp and removing the fused suture
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Reshaping and repositioning the skull bones with plates, screws, or wires
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Closing the incision with stitches or staples
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Applying a bandage or a helmet to protect the head and promote healing
Children may need to be hospitalized for a few days and have regular follow-up visits after the surgery. The procedure may take several hours and require general anesthesia. In order to mold the head into a normal shape, the child may also need to wear a helmet or headband for several months after the surgery.
Prevention of Craniosynostosis
There is no sure way to prevent craniosynostosis, as the cause is often unknown or genetic. However, some possible preventive measures include:
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Avoiding smoking, drinking alcohol, or using drugs during pregnancy
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Taking prenatal vitamins and folic acid supplements as recommended by the doctor
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Having regular prenatal check-ups and ultrasounds to monitor the baby’s growth and development
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Seeking medical attention if the baby has any signs or symptoms of craniosynostosis
Summary
As the name suggests, craniosynostosis affects a baby's skull shape. It occurs when one or more sutures (the joints between the skull bones) fuse before the brain is fully developed, causing the head to have an abnormal shape and, in some cases, affecting its growth and function. Surgery is the primary treatment for craniosynostosis, but not all affected children will need it. The goal of surgery is to correct the shape of the head and allow enough space for the brain to grow normally. A physical examination, imaging tests, and genetic tests can diagnose craniosynostosis. Since the cause of craniosynostosis is often unknown or genetic, there is no sure way to prevent it. Preventative measures include avoiding smoking, drinking alcohol, or using drugs during pregnancy, taking prenatal vitamins and folic acid supplements, undergoing regular prenatal checks and ultrasounds, and seeking medical attention if the baby shows any signs or symptoms of craniosynostosis.
