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Craniosynostosis- Types, Causes, Symptoms, and Treatment

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Craniosynostosis is a birth defect involving premature fusion of cranial bones resulting in the abnormal shape of the skull. Read below to know more.

Written byDr. Trivedi Himja Kiritbhai

Medically reviewed byDr. Veerabhadrudu Kuncham

Published At October 21, 2022
Reviewed AtAugust 28, 2024

What Is Craniosynostosis?

Skull development takes place in two different phases; neurocranium – the formation of the braincase, which comprises eight bones, and viscerocranium – the formation of the facial skeleton, which comprises 14 bones. Each bone is connected by spaces called sutures.

Craniosynostosis is a congenital developmental disorder where the fibrous joint between the two bones is fused earlier than its normal time in infants. During the neurocranium phase, sutures are closed earlier than they are supposed to in one or many parts of the skull. While the brain is still growing and where sutures are not connected, it causes the skull to grow unusually. As a result, the brain growth of the baby is hampered.

In newborns, the sutures are flexible enough to let the skull expand with the growth of the brain. There are many options available with the advancement of medicine if the condition is diagnosed earlier. Treatment options involve the surgical correction of the head to provide enough space for the brain to grow properly. In some cases, even the non-surgical treatment option, such as helmet therapy, is recommended for the children.

As craniosynostosis disorder causes a developmental hurdle in the growth of the brain, there are chances of a child developing neurological disabilities or damage. Even in a situation like this, early diagnosis can be a real boon for the good health of the child.

What Are the Types of Craniosynostosis?

Usually, craniosynostosis involves a single suture fusion. However, in some cases, different types of fusion are seen. Depending upon where and which suture fusion is taking place, there are types of craniosynostosis, such as:

1. Sagittal Synostosis - Sagittal suture runs from the front of the head to the back of the head and connects both sides of the skull. When the suture joins before time, it causes an elongated skull shape called scaphocephaly.

2. Coronal Synostosis - It involves a coronal suture that runs from both sides of the ears to the top of the head. The appearance of the face concerning the position and shape of the nose and eyes mainly changes.

  • Bicoronal Synostosis - When both sides of the suture fuse together prematurely, it causes a flat head and a wide appearance of the head.

  • Unilateral Synostosis - When either side of the suture fuses, it causes a bulge on the affected side.

3. Lambdoid Synostosis - It is a rare type of synostosis that involves the lambdoid suture that runs along the backside of the skull. The effect of this condition causes a flat head (posterior plagiocephaly), a tilting appearance of the head on one side, and one ear bigger in size than the other.

4. Metopic Synostosis - Metopic suture runs from the bridge of the nasal area to the top of the head connecting with the sagittal suture. The effect of this condition causes a triangle appearance of the head (trigonocephaly), narrow at the front and wide at the back.

What Are the Causes of Craniosynostosis?

In a rare scenario, the disorder is related to a genetic mutation. However, in most pediatric cases, there are no known reasons behind this condition. Etiological factors affecting this disorder are:

Syndromes Affecting the Condition:

  • Apert Syndrome - Abnormality is associated with the head and the face.

  • Crouzon Syndrome - Crouzon syndrome is a mutation of genes affecting eyesight and shallowing of eye sockets.

  • Pfeiffer Syndrome - Mutation causing underdeveloped jaw bone, abnormality of the nose, and shape of the eyes.

  • Muenke Syndrome - Abnormalities of extremities (hand and feet) and hearing loss are the symptoms.

  • Saethre-Chotzen Syndrome - Symptoms are droopy eyelids, abnormal hairline, forehead, and broad nose.

  • Antley-Bixler Syndrome - There is a fusion of arms and an abnormality of facial bones seen.

  • Frontonasal Dysplasia - A flat nose with a missing tip is commonly seen with this syndrome.

What Are the Signs and Symptoms of Craniosynostosis?

There are primary signs like misshapen facial bone, abnormal frontal bone, asymmetrical facial features, and skull. Moreover, premature closure of bone can build up pressure inside the skull due to the development of the brain, causing signs like:

  • Headache.

  • Dizziness.

  • Nausea.

  • Drooping eyelids.

  • Difficulty with eyesight.

  • Hearing loss.

  • Poor feeding.

  • Irritation.

  • High-pitched cry.

What Are the Complications Associated With Craniosynostosis?

If left untreated, increased intracranial pressure can cause many complications in infants, such as:

What Are the Treatment Options for Craniosynostosis?

  • The first line of treatment is diagnosing the condition at the earliest stage and then finding the factors affecting it. Depending on the severity of the condition, there are surgical and non-surgical treatments available.

  • Non-surgical treatments include medical helmet therapy for young ones. Furthermore, the surgical treatment includes traditional open surgery and minimally invasive endoscopic surgery.

  • Open surgery is specifically for children aged up to 11 months with severe conditions and symptoms. The affected bone is removed, reshaped, and placed with the help of plates and screws that dissolves with time. Overnight observation in the ICU and two to three days of rest are required post-surgery.

  • Minimally invasive endoscopic surgery is performed on children younger than three months. In the procedure, with the help of one to two small incisions, a thin tube with a camera on its end is inserted to unlock the fused sutures. Usually, there is no blood loss or swelling involved after surgery. Moreover, the recovery time is comparatively less than open surgery. However, after surgery for a short period, customized cranial helmets or customized spring implants are suggested.

Conclusion

Most babies with craniosynostosis live a long and healthy life if the condition is not that severe. However, some cases show delayed brain and intellectual development due to underlying genetic disorders or prenatal syndromes causing premature fusion of the skull and increased cranial pressure. Even after surgery, the little one should visit the doctors regularly for post-operative checkups.

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Frequently Asked Questions

The severity of Craniosynostosis can vary from one baby to another. It can range from mild to severe. Most babies with the condition are otherwise healthy. Surgery is the recommended treatment option.
Craniosynostosis can occur by itself or other deformities in the craniofacial region. If the condition is left untreated, it can cause severe deformities. They can include head deformities and increased pressure in the brain. Surgery is usually recommended for the condition.
Treatment for Craniosynostosis involves separating the cranial bones and rearranging them. It was treated using surgical methods, with incisions from ear to ear. The affected bones are removed, reshaped if required, and reattached.
The first sign of the condition can involve an abnormally shaped skull. Other signs can be soft spots in the skull and a raised firm edge at the areas where the sutures fused early. The children are otherwise healthy and have normal intelligence. At times, it can have genetic associations.
Typically, the surgery is performed within two to ten months. It depends on the suture to be fused and the best technique for that specific head shape. To get good results, minimally invasive surgery should be done within three months after birth. However, if the condition is mild, the surgery can be prolonged for some more months.
Yes, Craniosynostosis is a birth defect. It is a condition where a baby’s skull bones fuse very early. It happens even before the baby’s brain is fully formed. As a result, as the baby’s brain grows, the skull loses its shape.
The condition is generally diagnosed immediately after the baby is born. In some cases, it is diagnosed later in life. Generally, an abnormal shape in the skull is used to diagnose the condition. Surgery is recommended for the condition.
The condition can remain permanent if not treated appropriately. The deformity in the head can get severe. However, mild conditions might not require surgical intervention. The deformity is less evident as the baby grows older and hair grows over the head.
The position in the mother’s womb can cause the condition. Maternal thyroid issues can be another risk factor. Those pregnant mothers who are in treatment or have thyroid issues have an increased risk. Scanning for anomalies can help to understand the condition in-utero.
Babies with Craniosynostosis have less sleep when compared to normal babies. They show noticeable scalp veins. They can be easily irritated and become fussy. They usually have a high-pitched cry.
Yes, the condition can affect the intelligence quotient (IQ) of the child. This is generally observed as they start school. They usually have lower scores than normal children. This is because the condition can affect the child’s brain development.
The surgery for the condition may last for two to three hours. The child will be in the hospital for recovery and follow-up care. It takes around twelve weeks to recover from the surgery. This is the time taken for the bones to rejoin and regain strength.
There are rare chances that the condition will recur after surgery. This is because the surgery has a high success rate. However, in rare cases, it can recur in the same sutures. It may not recur in the adjacent sutures.
There can be derangement in the visual pathway of children with Craniosynostosis. If not treated appropriately, they can cause permanent blindness. These should be identified and treated earlier in life.
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Dr. Veerabhadrudu Kuncham from iCliniq
Dr. Veerabhadrudu Kuncham

Pediatrics

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