Craniosynostosis
What is Craniosynostosis?
Craniosynostosis
What is Craniosynostosis?
Craniosynostosis
Types of Craniosynostosis
Metopic Craniosynostosis
Trigonocephaly
Metopic craniosynostosis is a condition that occurs when the metopic suture, the seam running from the top of the nose to the upper forehead, fuses too early. This early fusion prevents normal skull growth, resulting in a distinctive triangular-shaped forehead, known as trigonocephaly. In addition to cosmetic concerns, metopic craniosynostosis can sometimes impact brain growth, making early diagnosis and treatment critical.
Signs and Symptoms of Metopic Craniosynostosis
The most common sign of metopic craniosynostosis is a noticeable ridge running down the forehead, often accompanied by a narrowing of the forehead and wider spacing between the eyes. Parents may also notice that their child’s head appears more pointed or triangular. In severe cases, pressure on the brain can lead to developmental delays, though this is rare. If you observe these signs in your child, it is important to consult with a specialist for evaluation.
Causes of Metopic Craniosynostosis
The exact cause of metopic craniosynostosis is not fully understood, though it is believed to result from a combination of genetic and environmental factors. While some cases may be linked to syndromes or genetic conditions, most cases occur sporadically, without a clear hereditary link. Regardless of the cause, early diagnosis can ensure the best possible outcomes for your child.
Treatment Options
The primary treatment for metopic craniosynostosis is surgery, which is usually recommended to improve both the appearance of the skull and allow for normal brain development. Depending on the severity of the case and the child’s age, different surgical approaches may be considered. For infants younger than four months, less invasive procedures such as endoscopic suturectomy may be an option, followed by helmet therapy to help shape the skull. For older children or more severe cases, a cranial vault remodeling surgery may be required to reshape the forehead and skull.
Prognosis and Long-Term Outcomes
With prompt and appropriate surgical treatment, children with metopic craniosynostosis generally experience excellent outcomes. After surgery, most children go on to have normal brain development and a more typical head shape. Long-term follow-up care is important to monitor the child’s development and ensure optimal recovery.
Expert Care at the NJ Craniosynostosis Center
At the NJ Craniosynostosis Center, we are experts at treating metopic craniosynostosis. Our team of specialists works closely with families to create a tailored treatment plan that addresses the unique needs of each patient. From diagnosis to post-operative care, we are with you every step of the way, ensuring the best possible outcome for your child.
If your child has been diagnosed with metopic craniosynostosis, or if you are concerned about the shape of your child’s head, we are here to help. Contact us today to schedule a consultation and learn more about your treatment options.
Sagittal Craniosynostosis
Scaphocephaly
Sagittal craniosynostosis is the most common form of craniosynostosis. It occurs when the sagittal suture, which runs from the front to the back of the skull, closes prematurely. This early closure causes the skull to grow abnormally, resulting in a long, narrow head shape known as scaphocephaly. While the condition may seem purely cosmetic, untreated sagittal craniosynostosis can sometimes lead to increased pressure on the brain, making early diagnosis and treatment essential.
Signs and Symptoms of Sagittal Craniosynostosis
The most noticeable sign of sagittal craniosynostosis is an elongated and narrow head shape, with a prominent forehead and a ridge running along the top of the head where the suture has fused. Parents may also observe a narrowing of the sides of the head, giving it a boat-like appearance. In severe cases, increased intracranial pressure can develop, which may lead to headaches, developmental delays, or vision problems. If you notice any of these signs or symptoms in your child, it is important to seek a professional evaluation as soon as possible.
Causes of Sagittal Craniosynostosis
Like other forms of craniosynostosis, the exact cause of sagittal craniosynostosis is not fully understood. In most cases, it occurs sporadically, without a family history or an associated genetic syndrome. However, some cases may be linked to genetic conditions or environmental factors during pregnancy. Regardless of the cause, early intervention can help prevent complications and improve outcomes for your child.
Like other forms of craniosynostosis, the exact cause of sagittal craniosynostosis is not fully understood. In most cases, it occurs sporadically, without a family history or an associated genetic syndrome. However, some cases may be linked to genetic conditions or environmental factors during pregnancy. Regardless of the cause, early intervention can help prevent complications and improve outcomes for your child.
Treatment for Sagittal Craniosynostosis
The primary treatment for sagittal craniosynostosis is surgery, which aims to correct the abnormal head shape and create space for the brain to grow properly. Different surgical options are available Depending on the child’s age and severity of the condition. For infants under four months of age, endoscopic-assisted surgery followed by helmet therapy may be an option. This minimally invasive procedure can achieve excellent results with a shorter recovery time. A more comprehensive cranial vault remodeling surgery may be necessary for older children or more severe cases to reshape the skull and relieve any pressure on the brain.
Prognosis and Long-Term Outlook
With timely surgical intervention, the long-term outlook for children with sagittal craniosynostosis is very positive. Most children go on to have normal brain development, with significant improvements in head shape and appearance. Follow-up care is crucial to ensure proper healing and monitor for any signs of increased intracranial pressure or developmental issues.
Expert Care at the NJ Craniosynostosis Center
At the NJ Craniosynostosis Center, we understand the concerns and anxieties that come with a craniosynostosis diagnosis. Dr. Ciminello and his team are experts at treating sagittal craniosynostosis. Our practice works closely with neurosurgeons, pediatricians, and specialists to provide comprehensive care tailored to each child’s unique needs. We strive to ensure that families feel informed and supported every step of the way.
If your child has been diagnosed with sagittal craniosynostosis, or you are concerned about their head shape, we are here to help. Contact us today to schedule a consultation and learn more about your treatment options.
Brachycephaly & Anterior Plagiocephaly
Coronal and Bicoronal Craniosynostosis
Coronal craniosynostosis occurs when one or both of the coronal sutures, which run from the top of the head down to the ears, fuse prematurely. When only one side is affected, it is known as unilateral coronal craniosynostosis, while the fusion of both coronal sutures is called bicoronal craniosynostosis and the head shape is sometimes referred to as brachycephaly. These conditions can significantly impact the shape of the skull and face, leading to asymmetry and other potential complications if left untreated.
Signs and Symptoms of Coronal and Bicoronal Craniosynostosis
In unilateral coronal craniosynostosis, the most common signs are an asymmetrical forehead, with one side appearing flatter than the other, and a misalignment of the eye sockets, causing one eye to appear higher than the other. In bicoronal craniosynostosis, both sides of the forehead may appear flat, giving the head a short and broad appearance. In addition to the cosmetic effects, some children may experience developmental delays or increased intracranial pressure, making early intervention crucial.
Causes of Coronal and Bicoronal Craniosynostosis
Coronal craniosynostosis can occur sporadically, without a clear genetic cause, but it is also associated with several genetic syndromes, such as Crouzon, Apert, and Pfeiffer syndromes. Bicoronal craniosynostosis is more frequently linked to these genetic conditions. In either case, a specialist evaluation is necessary to determine the exact cause and tailor the treatment plan to your child’s specific needs.
Treatment for Coronal and Bicoronal Craniosynostosis
Surgical intervention is the primary treatment for both coronal and bicoronal craniosynostosis, aiming to correct the shape of the skull and allow for normal brain growth. For children diagnosed early, endoscopic-assisted surgery may be an option, especially in cases of unilateral coronal craniosynostosis. However, more commonly, open cranial vault remodeling or fronto-orbital advancement is performed to reshape the forehead and correct the facial asymmetry. In bicoronal cases, more extensive surgery may be needed to address the broader shape of the skull.
Prognosis and Long-Term Outcomes
With timely surgery, children with coronal or bicoronal craniosynostosis generally experience excellent outcomes. The shape of the head and face improves significantly, and long-term brain development is typically unaffected. Post-operative follow-up care is important to monitor the child’s progress and ensure proper healing.
Expert Care at the NJ Craniosynostosis Center
At the NJ Craniosynostosis Center, Dr. Ciminello and his team are experts at treating coronal and bicoronal craniosynostosis. We work closely with neurosurgeons, pediatricians, and other specialists to develop a comprehensive treatment plan that addresses the unique needs of your child. From diagnosis to post-surgical care, we are with you every step of the way, ensuring the best possible outcome.
If you are concerned about your child’s head shape, or have received a diagnosis of coronal or bicoronal craniosynostosis, we are here to help. Contact us today to schedule a consultation and learn more about your options.
Lambdoid Craniosynostosis
Posterior Plagiocephaly
Lambdoid craniosynostosis is a rare form of craniosynostosis that occurs when the lambdoid suture, located at the back of the skull, fuses prematurely. This condition can cause an asymmetrical flattening of the back of the head, which is distinct from positional plagiocephaly, a more common condition where the head flattens due to external pressure. Lambdoid craniosynostosis can lead to further cranial deformities and, in severe cases, increased intracranial pressure, necessitating early intervention.
Signs and Symptoms of Lambdoid Craniosynostosis
The primary sign of lambdoid craniosynostosis is flattening at the back of the head, usually on one side (unilateral). This can cause the base of the skull to tilt, resulting in a characteristic “tilted” appearance of the ear on the affected side. The opposite side of the skull may become more prominent as the brain grows in an uneven shape. In severe cases, children may experience developmental delays or symptoms related to increased intracranial pressure, such as irritability and vomiting.
Causes of Lambdoid Craniosynostosis
Lambdoid craniosynostosis is usually sporadic, meaning it occurs without a known genetic cause. However, it can occasionally be linked to certain genetic conditions or syndromes. A thorough evaluation by specialists, including genetic testing, may be necessary to determine the cause of the condition and develop an appropriate treatment plan.
Treatment for Lambdoid Craniosynostosis
Surgery is the recommended treatment for lambdoid craniosynostosis, with the goal of correcting the skull shape and relieving any pressure on the brain. Depending on the severity and timing of diagnosis, endoscopic-assisted surgery may be an option for infants, offering a less invasive approach with smaller incisions. However, most cases are treated with open cranial vault remodeling, a procedure that reshapes the back of the skull to restore symmetry and promote normal brain growth.
Prognosis and Long-Term Outcomes
Children who undergo timely surgery for lambdoid craniosynostosis generally have excellent outcomes. The shape of the skull is corrected, and brain growth is not affected in the long term. Follow-up care is essential to monitor the child’s development and ensure proper healing after surgery. With the right treatment, children with lambdoid craniosynostosis can go on to live healthy, normal lives.
Expert Care at the NJ Craniosynostosis Center
At the NJ Craniosynostosis Center, Dr. Ciminello and his team are experts in treating lambdoid craniosynostosis. Our multidisciplinary approach ensures that each child receives personalized care, from initial diagnosis through post-surgical follow-up. We work closely with neurosurgeons, geneticists, and pediatric specialists to provide comprehensive care tailored to your child’s needs. Our mission is to deliver the best possible outcome for every child we treat.
If you suspect your child may have lambdoid craniosynostosis or have already received a diagnosis, the NJ Craniosynostosis Center is here to help. Contact us today to schedule a consultation and learn more about how we can assist you on your journey to ensuring your child’s health and well-being.
Syndromic & Non-syndromic craniosynostosis
Syndromic & Non-syndromic craniosynostosis
Craniosynostosis can occur in two distinct forms: syndromic and non-syndromic. Non-syndromic craniosynostosis is the more common form, occurring when one or more sutures in the skull close prematurely without being linked to any other underlying medical conditions. In non-syndromic cases, the fusion of the sutures typically only affects the shape of the head, and with timely treatment, most children go on to develop normally. While the exact cause of non-syndromic craniosynostosis is often unknown, it is believed to involve both genetic and environmental factors.
PLAGIOCEPHALY VS CRANIOSYNOSTOSIS
PLAGIOCEPHALY VS CRANIOSYNOSTOSIS
the genetics of craniosynostosis
Craniosynostosis can sometimes have a genetic component, meaning it may be passed down from parent to child or caused by changes (mutations) in certain genes. While many cases of craniosynostosis are classified as non-syndromic and occur without any known genetic cause, syndromic craniosynostosis is linked to specific genetic syndromes such as Crouzon, Apert, and Pfeiffer syndromes. These genetic syndromes not only cause craniosynostosis but can also affect other parts of the body, including the face, hands, and feet. For families with a history of craniosynostosis or other craniofacial conditions, genetic testing and counseling may be recommended to better understand the likelihood of craniosynostosis occurring in future children.
Common genes affected include: FGFR2, FGFR3, TWIST1, EFNB1, CDC45 and SMO
At the NJ Craniosynostosis Center, we work closely with genetic specialists to evaluate the potential genetic causes of craniosynostosis in each child. For families dealing with syndromic craniosynostosis, we collaborate with a multidisciplinary team to provide a comprehensive care plan that addresses both the cranial deformities and other related medical issues. Understanding the genetic component of craniosynostosis can help guide treatment and provide families with valuable information about their child’s condition. We ensure that families feel informed and supported every step of the way.
treatment options for craniosynostosis
Endoscopic Suturectomy
Learn MoreCranial Vault Remodeling
Learn MoreDistraction Osteogenesis
Learn MoreFronto-Orbital Advancement
Learn More
Frequently asked questions
Welcome to our FAQ section, where we answer some of the most common questions parents have about craniosynostosis.
Contact usWhat causes craniosynostosis?
The exact cause of craniosynostosis is not always clear. In most cases, it occurs sporadically, meaning there is no known cause. However, some cases are linked to genetic factors or syndromes like Apert, Crouzon, or Pfeiffer syndromes. If there is a family history of craniosynostosis or other craniofacial conditions, genetic testing may be recommended.
How common is craniosynostosis?
Craniosynostosis is relatively rare, affecting about 1 in every 2,000 to 2,500 live births. The condition can affect one or more of the skull sutures, and it can occur as an isolated issue (non-syndromic) or as part of a genetic syndrome (syndromic).
What are the risks of leaving craniosynostosis untreated?
If craniosynostosis is not treated, the fused sutures can restrict the normal growth of the skull, leading to increased pressure on the brain. This can result in developmental delays, cognitive problems, vision issues, and, in severe cases, permanent neurological damage. Early treatment can help prevent these complications.
At what age should craniosynostosis be treated?
Ideally, craniosynostosis should be treated in infancy, typically between 3 and 12 months of age. The specific timing depends on the severity and type of craniosynostosis. Early intervention leads to better outcomes, as the skull is more malleable and can be reshaped more easily during this stage.
Will craniosynostosis affect my child’s brain development?
If left untreated, craniosynostosis can potentially lead to increased pressure on the brain, which may result in developmental delays or neurological issues. However, with early diagnosis and appropriate treatment, most children go on to have normal brain development and live healthy lives.
