case_images: PMC11821711_01
case_images: Preoperative imaging work-up: fine-cut computed tomography (CT), CT angiography (CTA) and brain magnetic resonance image (MRI). a Coronal 3D-reconstruction from CT scan demonstrated a large bony deformity in the right portion of the anterior calvarium (arrow) and the right superior orbital rim (asterisk). The white dashed circle highlights the planned bifrontal craniectomy. b Sagittal 3D-reconstruction from CTA showed a large intracranial pedunculated mass (arrow) within the anterior and middle cranial fossae. c Coronal 3D-reconstruction from CTA revealed the anatomical relationship between the intracranial mass and the major cerebral arteries. CTA did not identify any significant arterial feeders to embolize prior to surgery. d Axial CT scan with bone window settings revealed complete obliteration of the frontal sinus due to the bony growth (asterisk), along with disease extension into its anterior and posterior wall (arrows). e Axial T2-FLAIR MRI highlighted hyperintense signals in the periventricular area and around the intracranial lesion (asterisks). The lesion's size had remained stable in comparison with the previous imaging; however, the FLAIR signal change represented a new finding. f Sagittal T1-postcontrast MRI demonstrated an extensive, irregularly enhancing lesion involving the frontal, sphenoidal, and ethmoidal regions, causing severe cerebral mass effect, particularly on the corpus callosum (arrow)
case_images: 701_2025_6438_Fig1_HTML.jpg
case_images: PMC11821711_01_701_2025_6438_Fig1_HTML.jpg
case_images: Fig1
case_images: Imaging revealed extensive craniofacial FD involving the frontal, ethmoid, sphenoid bones, as well as a large intracranial mass in the anterior and middle cranial fossae, causing cerebral mass effect (Fig. 1).
case_images: First surgery images. a Intraoperative picture showing the surgical cavity after the bifrontal craniectomy (dashed circle), drilling of the superficial lesion, and partial debulking of the intracranial mass (asterisk). b Axial CT scan after the first surgery demonstrated the removal of the superficial disease and intra-tumoral debulking of the pedunculated mass
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case_images: PMC11821711_01_701_2025_6438_Fig2_HTML.jpg
case_images: Fig2
case_images: The galea and skin were meticulously closed over the bony defect (Fig. 2a). A fine-cut CT scan was performed to create a 3D custom-made polyetheretherketone (PEEK) cranioplasty based on the margins of the superficial bony defect (Fig. 2b).
case_images: Second surgery images. a Intraoperative picture demonstrated the placement of the custom-made PEEK cranioplasty, fixed with multiple titanium miniplates, perfectly fitting the cranial defect. b Axial CT scan after the second surgery showed the removal of the extensive FD lesion, brain decompression, and reconstruction of the anterior calvarium. c Axial CT scan with bone settings window highlighted the residual disease
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case_images: PMC11821711_01_701_2025_6438_Fig3_HTML.jpg
case_images: Fig3
case_images: We reconstructed the anterior calvarium with the patient-specific implant, which fit the superficial defect perfectly (Fig. 3a), with no issues arising from skin retraction during placement. After the two-stage surgery, the patient improved significantly, and postoperative CT scan confirmed a gross-total resection of the intracranial mass and frontal bone vault, with cerebral decompression (Fig. 3b).
case_images: Follow-up images. a Axial CT scan 5-months after surgery demonstrated dilatation of the lateral ventricles and pseudomeningocele. The white star highlights the cerebrospinal fluid collection around the cranioplasty. The patient underwent a ventriculoperitoneal shunt placement. b Axial CT scan after 4-years showed significant decrease in ventricular size, and stable postoperative changes. c Axial CT scan with bone window settings revealed that the untreated disease remained stable
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case_images: PMC11821711_01_701_2025_6438_Fig4_HTML.jpg
case_images: Fig4
case_images: After 4 years, the patient remained neurologically stable, with no disease recurrence or symptoms from the untreated area (Fig. 4).