Learn About Craniosynostosis

What is the definition of 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.

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What are the alternative names for Craniosynostosis?

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

What are the causes of Craniosynostosis?

The cause of craniosynostosis is not known. Genes may play a role, but there is usually no family history of the condition. 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.

What are the symptoms of Craniosynostosis?

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

  • No "soft spot" (fontanelle) on the newborn's skull
  • A raised hard ridge along the affected sutures
  • Unusual head shape
  • Slow or no increase in the head size over time as the baby grows

Types of craniosynostosis are:

  • Sagittal synostosis (scaphocephaly) is the most common type. It affects the main suture on the very top of the head. The early closing forces the head to grow long and narrow, instead of wide. Babies with this type tend to have a broad forehead. It is more common in boys than girls.
  • Frontal plagiocephaly is the next most common type. It affects the suture that runs from ear to ear on the top of the head. It usually occurs on just one side, causing a flattened forehead, raised eyebrow, and prominent ear on that side. The baby's nose may also appear to be pulled toward that side. This is more common in girls than in boys.
  • Metopic synostosis is a rare form that affects the suture close to the forehead. The child's head shape may be described as trigonocephaly, because the top of the head appears triangular, with a narrow or pointed forehead. It may range from mild to severe.
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What are the current treatments for Craniosynostosis?

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

  • Relieve any pressure on the brain.
  • Make sure there is enough room in the skull to allow the brain to properly grow.
  • Improve the appearance of the child's head.
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What is the outlook (prognosis) for Craniosynostosis?

How well a child does depends on:

  • How many sutures are involved
  • The child's overall health

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

What are the possible complications of Craniosynostosis?

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

  • Increased intracranial pressure
  • Seizures
  • Developmental delay
When should I contact a medical professional for Craniosynostosis?

Call your child's provider if your child has:

  • Unusual head shape
  • Problems with growth
  • Unusual raised ridges on the skull
Skull of a newborn
What are the latest Craniosynostosis Clinical Trials?
GROWing Up With Rare GENEtic Syndromes ….When Children With Complex Genetic Syndromes Reach Adult Age

Summary: Introduction Rare complex syndromes Patients with complex genetic syndromes, by definition, have combined medical problems affecting multiple organ systems, and intellectual disability is often part of the syndrome. During childhood, patients with rare genetic syndromes receive multidisciplinary and specialized medical care; they usually receive medical care from 3-4 medical specialists. Increased...

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Comparison of the Rate of Preoperative Haemoglobin After Administration of Epoetin Alpha Associated With an Oral Medical Supplementation Versus Intravenous Before Surgery of Craniosynostosis at the Child

Summary: Oral iron is commonly used in conjunction with EPO preoperatively for hemorrhagic surgeries in children and especially in the surgery of craniosynostosis. The bioavailability of oral iron is low and compliance with treatment is inconsistent. The aim of this study is to evaluate whether the use of ferric carboxymaltose by injection, which has a much better bioavailability, would make it possible to...

What are the Latest Advances for Craniosynostosis?
Frontofacial Reconstruction Technique Modification With Preservation of Blood Supply to the Monobloc Segment.
Utility of Preoperative Helmet Molding Therapy in Patients With Isolated Sagittal Craniosynostosis.
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Who are the sources who wrote this article ?

Published Date: November 09, 2021
Published 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.

What are the references for this article ?

Centers for Disease Control and Prevention website. Facts about craniosynostosis. www.cdc.gov/ncbddd/birthdefects/craniosynostosis.html . Updated October 23, 2020. Accessed January 19, 2022.

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/.