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Fibrous dysplasia of the skull in children
C Di Rocco1, E Marchese, F Velardi
1Institute of Neurosurgery, Catholic University, Rome, Italy.
Insights
Early diagnosis of skull fibrous dysplasia in children is possible through painless bony bulging. Surgical intervention for pediatric skull fibrous dysplasia is explored, considering unpredictable courses and reconstruction challenges.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Skeletal Dysplasias
Background:
- Skull fibrous dysplasia in children often presents as painless, progressive bony bulging, enabling early diagnosis.
- Despite its benign nature, fibrous dysplasia can have unpredictable clinical courses with potentially severe and irreversible symptoms.
- The role of early surgical correction in pediatric skull fibrous dysplasia is debated due to the complexities of skull reconstruction after extensive bone excision.
Purpose of the Study:
- To evaluate the surgical treatment outcomes for pediatric skull fibrous dysplasia.
- To compare surgical approaches based on lesion localization and extent.
- To assess technical methodologies, cosmetic results, and long-term follow-up in pediatric patients.
Main Methods:
- Surgical treatment of 9 pediatric patients (ages 7-14) with skull fibrous dysplasia.
- Patients categorized into two groups based on disease localization: Group 1 (hair-covered cranium/fronto-orbital region) and Group 2 (multizonal skull involvement including cranial base).
- Discussion of surgical options, technical procedures, cosmetic outcomes, and long-term follow-up.
Main Results:
- Detailed surgical strategies were applied based on disease extent and location in pediatric patients.
- Cosmetic results and long-term follow-up data were collected for both patient groups.
- The study provides insights into the surgical management of skull fibrous dysplasia in children.
Conclusions:
- Surgical intervention for pediatric skull fibrous dysplasia requires careful consideration of lesion characteristics and reconstruction challenges.
- Tailored surgical approaches can yield favorable cosmetic and functional outcomes.
- Long-term follow-up is crucial for assessing the efficacy of surgical treatment in this pediatric population.
Abstract:
Early diagnosis of skull fibrous dysplasia in children is relatively easy, based on a history of painless progressive bony bulging. The therapeutic approach is still controversial, due to the benignity of the lesion. However, the clinical course may be unpredictable, with sudden appearance of symptoms, some of which can be important and irreversible. In pediatric patients, the possibility that an early surgical correction might positively interfere with the natural history of the lesion has to be evaluated by taking into account the obvious difficulties that will be encountered in reconstructing the skull after a wide excision of the pathologic bone. In the present report, we describe our personal experience on the surgical treatment of 9 children, ranging in age between 7 and 14 years. The patients were subdivided into two groups, according to the localization and extent of the disease. Patients in group 1 presented an involvement of the hair-covered cranium and/or the fronto-orbital region. Group 2 patients presented with a multizonal involvement of the skull, including the central cranial base (pterygoid, sphenoid, petrous and mastoid bone). The different surgical options, chosen for the two groups of patients, are discussed, together with the technical methodologies utilized, the cosmetic results and the long-term follow-up.