Management of Severe Cerebral Compression in Extensive Craniofacial Fibrous Dysplasia

Introduction: Fibrous dysplasia (FD) is a benign disorder marked by progressive substitution of normal bone with irregular fibro-connective tissue, resulting in expansion, fractures, pain, and functional impairment of the affected skeletal area. Although fibrous dysplasia can affect any bone of the body, craniofacial involvement is very common.

Objective: Despite surgical intervention is the primary treatment modality in craniofacial fibrous dysplasia, there is ongoing debate regarding optimal indications, techniques, and timing. Several authors agree on the importance of making a larger reconstruction procedure with placement of surgical implants rather than less aggressive recontouring procedures to decrease the rates of recurrence. We aim to demonstrate the feasibility of surgically treating extensive craniofacial lesions in two stages to maximize disease removal rates and reduce the risk of perioperative complications.

Materials and Methods: We report a case of a 46-year-old woman with severe craniofacial polyostotic fibrous dysplasia involving frontal, ethmoid, sphenoid bones, and nasal cavity with extensive intracranial component causing bilateral frontal lobes and lateral ventricles compression and executive function deficit.

Results: We planned a two-stage surgery with both demolitive and reconstructive purposes. During the first surgery, we removed a large portion of the tumor, mostly in its extracranial component, to alleviate the patient's symptoms and define the extent of the bone defect for subsequent treatment. During the second surgical intervention, we enlarged the tumor removal into its intracranial portion to maximize decompression of the brain parenchyma, and subsequently implanted a custom-made bone prosthesis based on the previously defined superficial defect.

Conclusion: Two-staged surgical strategy successfully mitigated the risks associated with a single extensive surgery, particularly the potential for high blood loss and those inherent to the prolonged operative time. This approach also allows for planning a custom-made cranioplasty during the second stage, based on the extent of the lesion removal on the first intervention.

Fig. 1 Preoperative 3D reconstruction CT scan shows fibrous dysplasia lesion affecting the anterior portion of the calvarium and anterior skull base with its extensive intracranial component.

Fig. 2 Preoperative T1-postcontrast MRI image demonstrates fibrous dysplasia lesion involving frontal, ethmoid, and sphenoid bones causing severe and bilateral frontal lobes, corpus callosum, and lateral ventricles compression.

Fig. 3 Intraoperative imaging of second surgery showing the surgical field before the reconstructive phase.

Fig. 4 Intraoperative imaging of second surgery after placement of PEEK implant that perfectly fit over the patient’s bone defect. Postoperative imaging at a 4 years of follow-up. CT image demonstrates stable postoperative change without complications.

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Artikel online veröffentlicht:
07. Februar 2025