What Is Craniosynostosis (Skull Shape Disorder) in Infants, What Are Its Causes, Symptoms and Treatment Methods?

An infant's skull is made up of several bones held together by strong tissues, along with the gaps between these bones. These extra gaps provide the space needed for the baby's brain to grow. At around 2 years of age, the baby's skull bones gradually begin to fuse and the gaps turn into bone. However, in some babies the skull bones fuse too early. This early fusion is a condition that can lead to health problems.

What Is Craniosynostosis (Skull Shape Disorder)?

The skull of a newborn consists of several cartilages connected to one another. There are usually some gaps between these skull cartilages, and over time these gaps close. The closing of the cartilages continues until about the age of 2 on average. A skull shape disorder can occur when the skull cartilages fuse earlier than they should. This disorder is called craniosynostosis. Craniosynostosis occurs when one or more of the gaps close too early in an infant. Craniosynostosis is a defect seen in infancy. The gaps between the cartilages allow the skull to grow as the baby's brain grows. When the gaps close and the skull cartilages fuse too early, skull growth in that area stops. As the brain reaches its normal size, the skull takes on an abnormal appearance over time. However, if too many cartilages close early, serious conditions such as increased pressure inside the skull can also develop. There are 4 types of craniosynostosis. These types are:

Sagittal Synostosis: It occurs when the suture running from front to back along the top of the head (the joint areas between the skull bones) closes early. It is a common type of craniosynostosis. It causes the baby's skull to be narrower. The skull appears long toward the back and narrow from side to side.

Coronal Synostosis: It is the early closure of the suture in the forehead area. A flatter brow and forehead structure develops on the affected side. On the affected side, not only the forehead but also the structure of the eye may appear different.

Metopic Synostosis: It occurs when the metopic suture, a suture running from the baby's fontanelle to its nose, closes early. It may cause a bulge running from the forehead to the nose, and it can also cause the eyes to be very close together.

Lambdoid Synostosis: It occurs due to early closure of the lambdoid suture, which runs along the back of the head. It is rarer than the other types. It may cause one part of the head to appear flatter and the ears to not sit in alignment.

What Are the Symptoms of Craniosynostosis (Skull Shape Disorder)?

In places where the skull cartilages fuse too early, the baby's head may stop growing. In other areas where the cartilages have not fused, the baby's head continues to grow. As a result, the heads of babies with craniosynostosis usually have an asymmetrical appearance. However, if the baby has several cartilages that close too early, there may not be enough space for the brain to grow. As a result, these babies may experience increased pressure inside the skull. The symptoms seen in babies with craniosynostosis are:

Bulging of the fontanelle

Sleeping longer than normal

Prominent veins on the head

Loud crying

Projectile vomiting

Increase in head circumference

Developmental delay

What Are the Causes of Craniosynostosis (Skull Shape Disorder)?

In most babies, the cause of craniosynostosis is not fully known. In some cases, craniosynostosis may occur due to genetic abnormalities such as Apert syndrome, Carpenter syndrome, Crouzon syndrome, Pfeiffer syndrome and Saethre-Chotzen syndrome. However, most babies with craniosynostosis do not have a genetic syndrome. It is also thought that craniosynostosis may develop depending on the mother's environmental factors, the foods she consumed, alcohol and cigarette use, and medications she used during pregnancy.

How Is Craniosynostosis (Skull Shape Disorder) Diagnosed?

Craniosynostosis may be detected during routine ultrasound checks in pregnancy, although this process can often take several weeks after birth. After birth, detection becomes easier during physical examinations performed by a doctor. The process can also be sped up with routine head circumference measurements. If the head circumference is larger than it should be, the doctor may request some tests for follow-up. X-rays and computed tomography make it easier to image bones and cartilages and allow disorders to be detected. At the same time, genetic tests and blood tests may be needed when necessary. Starting treatment as soon as possible is very important to prevent permanent damage. Therefore, diagnosis in a short time and at an early stage helps speed up treatment. If diagnosis and treatment are delayed, permanent damage may occur. Usually, the first sign of craniosynostosis is an abnormally shaped skull. Genetic tests are not used to confirm this diagnosis. The purpose of their use is to investigate whether a genetic syndrome causing the skull shape disorder is present.

How Is Craniosynostosis (Skull Shape Disorder) Surgery Performed?

If craniosynostosis is diagnosed after the baby's birth and it is not mild, surgery is recommended as soon as possible. The reason surgery is recommended early is that the bones are soft and flexible enough to be correctly repositioned. The aim of craniosynostosis surgery is to relieve the pressure of the incorrectly fused bones on the brain and, at the same time, correct the physical appearance. Two types of craniosynostosis surgery can be performed by surgeons. The types of craniosynostosis surgery are:

Open Surgery: Open surgery is an operation in which the surgeon makes an incision in the baby's scalp. Through the incision, the surgeon shapes the skull bones into a circular form. The surgeon removes the skull fragments near the fused suture and reshapes them, making room for the brain to grow. Open surgery may last up to six hours, and blood transfusion may generally be required due to blood loss during the operation. After completing the surgery, the baby needs to be closely monitored in the intensive care unit for one night. Most children are moved to a regular hospital room one day after surgery. Shaping with open surgery is generally recommended for babies older than 6 months. The reason is that the bones are flexible enough to be moved but thick enough to keep their new shape.

Closed Endoscopic Procedure: Endoscopic craniosynostosis surgery offers a less invasive option for treating craniosynostosis. Smaller incisions, only large enough for medical instruments to pass through, are made in the scalp compared with open surgery. An endoscope tube is inserted through these incisions for imaging and for moving the bones. The surgeon can move the bones using this tube. This method is used especially in the 2-4 month period, when the bones are soft enough to be moved with an endoscope. However, because the bones of babies continue to grow during this period, a medical helmet may need to be worn for a period of time.

Frequently Asked Questions About Craniosynostosis (Skull Shape Disorder) in Infants

A newborn baby's skull consists of several soft, movable bone plates that allow passage through the birth canal. As these bones grow after birth, they gradually fuse with each other. In pediatric craniosynostosis, a baby's skull bones fuse too early, which can restrict brain growth and lead to an abnormal head shape. Treatment of craniosynostosis usually requires surgery to relieve any pressure on the brain and allow the brain to grow. The best time for craniosynostosis surgery is generally before the baby reaches 1 year of age, because the bones of babies' skulls are still soft and easier to shape. Surgery may be recommended at 1 month of age for babies with a severe case of craniosynostosis. There are also mild cases of craniosynostosis that do not require surgery. If craniosynostosis is left untreated, it may lead to the following, along with increased pressure inside the skull and impaired brain development:

Developmental disorders

Intellectual disability

Blindness

Seizures

Babies with mild craniosynostosis may rarely not need surgery. In this case, a medical helmet can help the baby's brain development and correct the shape of the skull. A helmet to shape the skull may also need to be used after an endoscopic procedure, not only after surgery. The surgeon determines the duration of helmet therapy depending on how quickly the skull shape responds to treatment. Two types of surgery are performed for treatment. These surgeries can be performed as open surgery or endoscopically. The use of a shaping helmet outside of surgery may also be considered appropriate. Skull shape disorders are usually noticed during a routine examination by a pediatrician. When this condition is suspected, the patient can be referred to a neurosurgeon and tests can be carried out. Hisar Hospital Intercontinental neurosurgery specialists successfully perform craniosynostosis surgeries. The problems that craniosynostosis may cause vary depending on the type of craniosynostosis the baby has and how severe it is. Most craniosynostosis cases can be treated and do not lead to long-term complications. However, severe craniosynostosis that is not treated can adversely affect the baby's brain growth and development. If craniosynostosis is not treated, permanent head and facial abnormalities, developmental delays and intellectual disability may develop. If a baby's head is frequently turned to one side, flat spots may form on that side of the head. However, there is no proven sleeping position to make babies' head shape more symmetrical. Craniosynostosis is a condition that needs to be diagnosed early. It is very important that the physician performing routine examinations for diagnosis is a specialist in the field. Hisar Hospital Intercontinental has an important role in the diagnosis and treatment of craniosynostosis with its team of expert physicians and surgeons. You can contact us to make an appointment and have your baby's necessary examinations carried out.