Session 5: Case based discussions with expert panel, Q & As

Cherubism is a rare, genetic, fibro-osseous dysplasia which presents with progressive bilateral upper and lower jaw enlargement, with typical facial characteristics. First described by Jones in 1933, there are about 300 reported cases in the literature, the facial features bearing a passing resemblance to a biblical cherub.Features include remodelling of normal bone to fibrous tissue with cyst formation, leading to early tooth loss, mandibular and maxillary enlargement. Orbital involvement is very rare, tending to present in advanced cases, leading to globe dislocation, ophthalmoplegia, optic nerve compression and macular striae and scarring. Where patients present with significant globe displacement and/or optic neuropathy, mass debulking of the lesion can be successful, as described in this case.

Three challenging cases of sphenoid wing meningiomas are presented. Each posed a unique challenge in either the diagnosis (case 1), symptom control (case 2) or management (cases 1- 3).Case 1 presents a 35 year old gentleman with a history of a medulloblastoma operated on as a child and who received whole brain radiotherapy. He presented with proptosis. His MRI scan showed an extrinsic lesion centred around the left sphenoid wing which had both intra cranial and intra orbital components.Case 2 presents a 38 year old lady with bilateral sphenoid wing meningiomas who underwent multiple surgeries over the years but unfortunately still lost her vision on the right. She also developed hypopituitarism and trigeminal neuralgia as a result of the tumour.Case 3 presents a 41 year old lady who experienced proptosis in the immediate post-partum period that caused disabling diplopia. MRI revealed a sphenoid wing meningioma with a predominantly intra osseous component.These cases each underwent a different management strategy despite having a common diagnosis. This presentation highlights the need for individualised patient care for patients with sphenoid wing meningiomas. Collaboration between the different specialties is key for success.