Parry-Romberg Syndrome and an Associated Complex Vascular Lesion Managed With Hybrid Strategies: A Case Report.

background and importanceParry-Romberg syndrome (PRS) is a condition of unknown etiology, most commonly characterized by progressive facial hemi-atrophy due to breakdown of the underlying skin and soft tissues. PRS has been associated with neurologic disease such as intracranial aneurysms and vascular malformations. However, its exact role in the incidence of neurovascular disease remains unknown. CLINICAL PRESENTATION: We present a case of a complex fusiform aneurysm involving the A1 segment of the left anterior cerebral artery in a 21-year-old man with PRS. The patient initially presented with a left giant fusiform/dissecting internal carotid artery aneurysm initially managed with overlapping flow diverters. His post-flow diverter treatment course was confounded by progressive growth of the aneurysm with worsening mass effect on the optic chiasm leading to complete visual loss in the left eye and a right hemifield cut. To manage the aneurysm recurrence, retreatment with surgical bypass-supported carotid occlusion was performed. Thereafter, visual symptoms improved, but complete normalization of vision was not achieved. The patient remained clinically stable for several years until follow-up imaging demonstrated a novel adjacent recurrence involving the ipsilateral A1 segment, previously noted to be angiographically normal. The patient underwent superselective angiography followed by endovascular occlusion of the new fusiform lesion. DISCUSSION: The case presented here illustrates the hybrid roles of endovascular and surgical treatment modalities in treating complex vascular lesions. The recurrence of the lesion following initial treatment strategies introduces questions about how the nature and development of the lesion affected the success of treatment at each stage. Considerations for the efficacy of each stage of treatment in this case include the patients underlying connective tissue disorder, the pediatric etiology of the lesion, and the timing of each treatment option given these factors.

conclusionIn addition to understanding the biological behavior of flow diversion for complex intracranial aneurysms, the role of PRS in the incidence of neurovascular disease should also be considered when applicable. More research into the mechanisms of PRS in neurovascular disease is needed.