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Craniosynostosis

Premature closure of sutures; Synostosis; Plagiocephaly; Scaphocephaly; Fontanelle - craniosynostosis; Soft spot - craniosynostosis

Craniosynostosis is a birth defect in which one or more sutures on a baby's head closes earlier than usual.

The skull of an infant or young child is made up of bony plates that are still growing. The borders at which these plates intersect are called sutures or suture lines. The sutures allow for growth of the skull. They normally close ("fuse") by the time the child is 2 or 3 years old.

Early closing of a suture causes the baby to have an abnormally shaped head. This may limit brain growth.

Images

Skull of a newborn

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Causes

The cause of craniosynostosis is not known. Genes may play a role, but there is usually no family history of the condition. More often, it may be caused by external pressure on a baby's head before birth. Abnormal development of the base of the skull and the membranes around the skull bones is believed to affect the movement and position of the bones as they grow.

In cases when this is passed down through families, it may occur with other health problems, such as seizures, decreased intelligence, and blindness. Genetic disorders commonly linked to craniosynostosis include Crouzon, Apert, Carpenter, Saethre-Chotzen, and Pfeiffer syndromes.

However, most children with craniosynostosis are otherwise healthy and have normal intelligence.

Symptoms

Symptoms depend on the type of craniosynostosis. They may include:

Types of craniosynostosis are:

Exams and Tests

The health care provider will feel the infant's head and perform a physical exam.

The following tests may be done:

Well-child visits are an important part of your child's health care. They allow the provider to regularly check the growth of your infant's head over time. This will help identify any problems early.

Treatment

Surgery is usually needed. It is done while the baby is still an infant. The goals of surgery are:

Outlook (Prognosis)

How well a child does depends on:

Children with this condition who have surgery do well in most cases, especially when the condition is not associated with a genetic syndrome.

Possible Complications

Craniosynostosis results in head deformity that can be severe and permanent if it is not corrected. Complications may include:

When to Contact a Medical Professional

Call your child's provider if your child has:

Related Information

Craniosynostosis repair
Craniosynostosis repair - discharge

References

Centers for Disease Control and Prevention website. Facts about craniosynostosis. www.cdc.gov/ncbddd/birthdefects/craniosynostosis.html. Updated November 1, 2018. Accessed October 24, 2019.

Graham JM, Sanchez-Lara PA. Craniosynostosis: general. In: Graham JM, Sanchez-Lara PA, eds. Smith's Recognizable Patterns of Human Deformation. 4th ed. Philadelphia, PA: Elsevier; 2016:chap 29.

Kinsman SL, Johnston MV. Congenital anomalies of the central nervous system. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 609.

Mandela R, Bellew M, Chumas P, Nash H. Impact of surgery timing for craniosynostosis on neurodevelopmental outcomes: a systematic review. J Neurosurg Pediatr. 2019;23(4):442-454. PMID: 30684935 pubmed.ncbi.nlm.nih.gov/30684935/.

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Review Date: 9/29/2019  

Reviewed By: Kimberly G. Lee, MD, MSc, IBCLC, Clinical Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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