A review of the management of single-suture craniosynostosis, past, present, and future

J Neurosurg Pediatr. 2019 Dec 1;24(6):622-631. doi: 10.3171/2019.7.PEDS18585.


Background: Craniosynostosis is a condition in which 2 or more of the skull bones fuse prematurely. The spectrum of the disorder most commonly involves the closure of a single suture in the skull, but it can also involve syndromic diagnoses in which multiple skull bones and/or bones outside of the cranium are affected. Craniosynostosis can result in cosmetic deformity as well as potential limitations in brain growth and development, and the neurocognitive impact of the condition is just starting to be studied more thoroughly. Our knowledge regarding the genetics of this condition has also evolved substantially. In this review, the authors explore the medical and surgical advancements in understanding and treating this condition over the past century, with a focus on how the diagnosis and treatment have evolved.

Methods: In this review article, the authors, who are the leaders of a craniofacial team at a major academic pediatric hospital, focus on single-suture craniosynostosis (SSC) affecting the 6 major cranial sutures and discuss the evolution of the treatment of SSC from its early history in modern medicine through the current state of the art and future trends. This discussion is based on the authors' broad experience and a comprehensive review of the literature.

Summary: The management of SSC has evolved substantially over the past 100 years. There have been major advances in technology and medical knowledge that have allowed for safer treatment of this condition through the use of newer techniques and technologies in the fields of surgery, anesthesia, and critical care. The use of less invasive surgical techniques along with other innovations has led to improved outcomes in SSC patients. The future of SSC treatment will likely be guided by elucidation of the causes of neurocognitive delay in these children and assessment of how the timing and type of surgery can mitigate adverse outcomes.

Keywords: SSC = single-suture craniosynostosis; UCS = unilateral coronal synostosis; anesthesia; cognitive; cosmetic; craniofacial; craniosynostosis; endoscopic.

Publication types

  • Review

MeSH terms

  • Cognitive Dysfunction / etiology
  • Cranial Sutures
  • Craniosynostoses / complications
  • Craniosynostoses / diagnostic imaging
  • Craniosynostoses / genetics
  • Craniosynostoses / surgery*
  • Developmental Disabilities / etiology
  • Humans
  • Neuroendoscopy / methods
  • Neurosurgical Procedures / methods*
  • Physical Appearance, Body
  • Plastic Surgery Procedures / methods*
  • Time Factors