Synchronous polyostotic fibrous dysplasia and meningiomatosis: a case report on the challenging treatment for severe intracranial hypertension

Background: Louis Lichtenstein first reported fibrous dysplasia in 1938. It is a congenital skeletal anomaly characterized by dysregulated bone remodeling leading to expansive fibro-osseous lesions. In its origin, there is a mutation in the GNAS1 gene that leads to abnormal fibroblasts and inordinate function of skeleton progenitor cells. Despite being extremely rare, there is growing evidence of an association between fibrous dysplasia and co-existing meningiomas, though there is still no known etiopathological factor that explains this coexistence, making it all the more important to report the rare occasions where it occurs. Case Description: We report a unique case of a 36-year-old female with polyostotic fibrous dysplasia and concurrent multiple meningiomas, culminating in severe refractory intracranial hypertension. Despite initial conservative management, progressive neurological deterioration prompted neurointensive care and, initially, a non-invasive approach for intracranial pressure treatment. Standard strategies such as profound sedation, neuromuscular blockade and frequent administration of hypertonic saline and mannitol, and rescue options like thiopental boluses and hyperventilation periods revealed themselves to not being sufficient. This prompted two complex neurosurgical interventions. The first one targeted to remove the apoplectic left parasagittal meningioma, thus attempting to rescind the cycle of edema and intracranial hypertension, and the second one with a decompressive craniotomy forethought. Conclusions: Through multidisciplinary collaboration, including tailored neuromonitoring and surgical approach, the patient’s complex condition was addressed attaining neurological recovery. This case underscores the necessity for individualized therapeutic approaches and interdisciplinary cooperation in managing rare and complex neurosurgical pathologies and their complications.