Occult cerebrovascular malformations after irradiation

OBJECTIVE: It has recently been found that patients receiving cerebral irradiation can develop hemorrhagic dysangiogeneses simulating occult vascular malformations. To analyze this connection, we report on five patients with occult cerebrovascular malformations occurring after ''standard'' or focused irradiation performed for brain tumors in four patients and for a deep-seated cavernous angioma in one patient. METHODS: All lesions were within the radiation ports. The time interval between irradiation and the detection of the occult vascular malformations varied from 3 to 9 years; the ratio of female to male patients was 4:1. Four patients were <15 years old when first irradiated. Four patients presented with acute symptoms (headache, vomiting, focal signs) and one was asymptomatic when the lesions were first detected. Serial magnetic resonance imaging scans were available in four patients and a computed tomographic scan in the other patient. RESULTS: The initial appearance was that of a hypointense T1-T2 focus; magnetic resonance imaging then revealed focal or multifocal T1 hyperintensity and T2 mixed signal intensity followed by a late ring of decreased signal intensity. Four patients were operated on and one was under neuroradiological monitoring. Histological features of these lesions included clusters of closely packed vascular spaces resembling cavernous malformations sometimes associated with a thrombosed thick-walled vein with intense hemosiderin deposition and fibroblastic proliferation; telangiectasic changes were also seen in the adjacent brain. CONCLUSION: Increased awareness of occult cerebrovascular malformations is necessary, because their occurrence is not infrequent and they have hemorrhagic potential. Children receiving cerebral irradiation are at greater risk of this complication.