Growing skull fracture in a child with Ehlers-Danlos syndrome: case report and literature review

Childs Nerv Syst. 2023 Sep;39(9):2399-2405. doi: 10.1007/s00381-023-06035-7. Epub 2023 Jun 21.

Abstract

Introduction: Growing skull fracture (GSF) is a rare complication of head trauma in the pediatric population, commonly observed in children younger than 3 years.

Discussion: In this report, the authors describe a case of a 3-year-old male child, with clinical features of Ehlers-Danlos syndrome (EDS), who developed a GSF in frontal bone after a crib fall, treated with duraplasty and cranioplasty with autologous craft. Here, pertinent literature was reviewed with an emphasis on surgical techniques, and correlation with the mentioned syndrome.

Conclusion: This is the first case of GSF in association with EDS in the literature. The relevance of the case described concerns the rarity of the condition itself, the atypical presentation, and the intraoperative findings, which showed the important fragility of the dura mater, probably due to EDS. Therefore, this syndrome, besides having influenced the pathogenesis, was also a challenging factor in the surgical treatment.

Keywords: Craniofacial surgery; Ehlers-Danlos syndrome; Growing skull fractures; Pediatrics.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Craniocerebral Trauma* / complications
  • Ehlers-Danlos Syndrome* / complications
  • Ehlers-Danlos Syndrome* / surgery
  • Frontal Bone / surgery
  • Humans
  • Male
  • Skull Fractures* / complications
  • Skull Fractures* / diagnostic imaging