Vanishing Bone Disease of the Skull Base: A Review of Rare Entity

Mohamed Aziz Hermassi*, Ala Belhaj, Dorsaf Nouri, Abdelhafidh Slimane, Insaf Dkhil and Khansa Abderrahmen

Department of Neurosurgery, National Institute of Neurology, Tunisia Department of Radiology, National Institute of Neurology, Tunisia

^*Correspondance to: Mohamed Aziz Hermassi 

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Abstract:

Introduction: Vanishing bone diseaseis a very rare idiopathic disorder of the skeletal system of unknown etiology. The pathogenetic mechanisms of the disease remain unclear. Any part of the skeleton can be involved and the cranial localization is extremely uncommon. This case aims to highlight the diagnostic process and management of this extremely rare entity. Case Report: A 23-year-old female, presented with asymptomatic skull defect over the left temporooccipital region that progressively enlarged over a period of 2 years. Computed tomography revealed a left temporal osteolyticlesion, centered on the mastoidboneextending to the ipsilateral occipital bone and clivus. MRI features showed a medullary replacement process with a component whose enhancement suggests a venolymphatic vascular nature. Open bone biopsy confirmed the diagnosis of Gorham-Stout disease. We chose the conservative treatment and the results were good. Clinical Discussion: Vanishing bone disease of the skull base is an extremely rare entity characterized by destruction of osseous matrix and proliferation of vascular structures with benign origin.The skull is among the least common locations of involvement. The diagnosis of the cranial localization of the syndrome is very challenging and it is confirmed by the histopathological analysis of the lesions. Its management is still an object of research, there is no consensus regarding the treatment of the skull localization and its prognosis is still unpredictable. Conclusion: Gorham-Stout disease of the skull base presents unique diagnostic and therapeutic challenges. Physicians should be aware of the existence of this rare entity, early diagnosis and a multidisciplinary approach to management, including surgical and pharmacologic interventions, are crucial in optimizing outcomes.

Keywords:

Gorham-Stout; Osteolysis; Vanishing bone disease; Skull base; Diagnosis; Case report

Cite the Article:

Hermassi MA, Belhaj A, Nouri D, Slimane A, Dkhil I, Abderrahmen K. Vanishing Bone Disease of the Skull Base: A Review of Rare Entity. Clin Case Rep Int. 2024; 8: 1714..