Rare cause of oculomotor palsy

Case report: Oculomotor palsy (OcP) is a uni- or bilateral injury of the third cranial nerve and usually the first symptom of intracranial pressure or a chiasmatic tumor. Hemangiomas are the most frequent benign neoplasms in childhood and predominantly located in skin. An intracranial manifestation of hemangioma is rare and especially occurs in children with multiple cutaneous hemangiomas. We present the case of a teenager with oculomotor palsy and intracranial hemangioma in optic chiasm.

A 17-year old girl initially presents 16 months ago with progressive OcP and amaurosis of her right and progressive visual reduction of her left eye (current acuity 0.15). Funduscopic examination offers optic atrophy of both eyes. No cutaneous hemangioma is found in clinical examination. In cMRI, an extended, parasellar, extra-axial tumor with homogeneous contrast accumulation is detected. After stereotactially guided biopsy hemangioma with positive proof of VEGF is established histologically. Primary, surgical intervention cannot be done because of tumor size, therefore a treatment with propranolol is started to protect the remaining visual acuity of 0.15. No reduction of tumor size can be achieved. Therefore after 3 months additional medication with prednisolone is started which lead to an initial tumor reduction in cMRI. Secondary, cortisone-induced brain atrophy is detected. Thus by reference the tumor size to brain volume, no significant tumor reduction is achieved. Therefore a therapy with bevacizumab was induced, which was stopped after 3 months of treatment without beneficial effect. About 15 months after beginning of any therapy the decision of a surgical extirpation of hemangioma with embolization is made as ultimate ratio. In postoperative course she develops vasospasms with temporary left-sided hemiplegia. Furthermore, she loses the remaining visual acuity of her left eye.

This case indicates that there is no defined treatment strategy of an intracranial hemangioma. The therapy depends on the symptoms and remains individual. The optimal timing of the surgical intervention is now under discussion.