Genetic and Rare Diseases Information Center. Centers for Disease Control and Prevention. Syndromic craniosynostosis: Unique management considerations. Craniosynostosis: Clinical presentation, genetics, and prenatal diagnosis. Identifying the misshapen head: Craniosynostosis and related disorders. Craniosynostosis and positional plagiocephaly (infant). If untreated, increased intracranial pressure can cause: But babies with an underlying syndrome may develop increased intracranial pressure if their skulls don't expand enough to make room for their growing brains. The risk of increased pressure inside the skull (intracranial pressure) from simple craniosynostosis is small if the suture and head shape are fixed surgically. If untreated, craniosynostosis may cause, for example: These syndromes usually also include other physical features and health problems. Syndromic craniosynostosis is caused by certain genetic syndromes, such as Apert syndrome, Pfeiffer syndrome or Crouzon syndrome, which can affect a baby's skull development.Its cause is unknown, although it's thought to be a combination of genes and environmental factors. Nonsyndromic craniosynostosis is the most common type of craniosynostosis.Often the cause of craniosynostosis is not known, but sometimes it's related to genetic disorders. Talk to your pediatrician if you have concerns about your baby's head growth or shape. Your health care provider will routinely monitor your child's head growth at well-child visits. This can be treated with regular position changes, or if significant, with helmet therapy (cranial orthosis) to help reshape the head to a more balanced appearance. For example, if the back of your baby's head appears flattened, it could be the result of spending too much time lying on one side of the head. It may cause one side of a baby's head to appear flat, one ear to be higher than the other ear and tilting of the top of the head to one side.Ī misshapen head doesn't always indicate craniosynostosis. Lambdoid synostosis is a rare type of craniosynostosis that involves the lambdoid suture, which runs along the back of the head. This head shape is also called trigonocephaly. Premature fusion gives the forehead a triangular appearance and widens the back part of the head. The metopic suture runs from the top of the bridge of the nose up through the midline of the forehead to the anterior fontanel and the sagittal suture. When both coronal sutures fuse prematurely (bicoronal), the head has a short and wide appearance, often with the forehead tilted forward. It also leads to turning of the nose and a raised eye socket on the affected side. Premature fusion of one of the coronal sutures (unicoronal) that run from each ear to the top of the skull may cause the forehead to flatten on the affected side and bulge on the unaffected side. Sagittal craniosynostosis is the most common type of craniosynostosis. Premature fusion of the sagittal suture that runs from the front to the back at the top of the skull forces the head to grow long and narrow. The term given to each type of craniosynostosis depends on what sutures are affected. Multiple suture craniosynostosis is usually linked to genetic syndromes and is called syndromic craniosynostosis. Some complex forms of craniosynostosis involve the fusion of multiple sutures. Most involve the fusion of a single cranial suture. There are several types of craniosynostosis. Development of a raised, hard ridge along affected sutures, with a change in the shape of the head that's not typical.A misshapen skull, with the shape depending on which of the sutures are affected.Signs and severity depend on how many sutures are fused and when in brain development the fusion occurs. The signs of craniosynostosis are usually noticeable at birth, but they become more apparent during the first few months of your baby's life. Early diagnosis and treatment allow your baby's brain adequate space to grow and develop.Īlthough neurological damage can occur in severe cases, most children develop as expected in their ability to think and reason (cognitive development) and have good cosmetic results after surgery. Treating craniosynostosis involves surgery to correct the shape of the head and allow for brain growth. In rare cases, craniosynostosis is caused by certain genetic syndromes (syndromic craniosynostosis). Each side of the skull has a tiny fontanel.Ĭraniosynostosis usually involves premature fusion of a single cranial suture, but it can involve more than one of the sutures in a baby's skull (multiple suture craniosynostosis). The next largest fontanel is at the back (posterior). The anterior fontanel is the soft spot felt just behind a baby's forehead. In the front of the skull, the sutures meet in the large soft spot (fontanel) on top of the head. Usually, during infancy the sutures remain flexible, allowing a baby's skull to expand as the brain grows.
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