Infantile cranial fasciitis: case-based review and operative technique

Childs Nerv Syst. 2017 Jun;33(6):899-908. doi: 10.1007/s00381-017-3417-y. Epub 2017 Apr 27.

Abstract

Background: Cranial fasciitis (CF) is an uncommon benign primary lesion of the skull that typically affects the pediatric age group. Due to the rarity of CF, no prospective studies exist. Earliest description of this condition dates to 1980. The limited scientific and clinical literature regarding CF is dominated by case reports. For these reasons, questions pertaining to the true incidence, genetic risk factors, prognosis, and long-term outcome remain unanswered.

Discussion: Clinically, CF presents as a firm, painless, growing scalp mass that is typically not considered in the differential diagnosis. Preoperative pathognomonic signs and symptoms are absent, and imaging features are often nonspecific. Treatment is typically through complete surgical resection, at which time histopathological examination confirms the diagnosis of CF. Reconstruction of the skull defect in the child is critical. Autograft techniques help maintain a rigid construct that integrates with the native skull while preserving its continued ability to grow. Generally, a good outcome is observed with complete resection.

Exemplary case: We report a case of CF in an infant with emphasis on operative nuances and early follow-up results.

Conclusion: CF is a rare fibroproliferative disease that has a poorly defined incidence and long-term follow-up. Due to its locally invasive nature and nonspecific presentation, CF is often difficult to differentiate from malignancies and infections. Complete surgical resection is the best approach for diagnosis and cure. Its occult clinical presentation often allows it to achieve considerable growth, leaving a sizeable skull defect following resection. Since CF presents in the pediatric population, allograft reconstruction is preferred over titanium mesh or other synthetic materials to allow osseous integration and continued uninterrupted skull growth.

Keywords: Cranial fasciitis; Fibroproliferative; Primary skull lesion; Rib resection.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Fasciitis / diagnostic imaging*
  • Fasciitis / surgery*
  • Female
  • Humans
  • Infant
  • Male
  • Neurosurgical Procedures / methods*
  • Skull / diagnostic imaging*
  • Skull / surgery*