Sturge-Weber syndrome (SWS) is a neurocutaneous disorder presenting with a facial port-wine stain, along with an occipital leptomeningeal angiomatosis that is typically located ipsilateral to the stain. In this paper, the authors present a rare case of SWS associated with an arteriovenous malformation (AVM) instead of an angiomatosis in the ipsilateral occipital lobe. While the patient was in the care of the authors, the AVM progressively enlarged, and was accompanied by progressive stenoocclusive changes of the venous system. The resulting brain edema finally brought about a serious neurological condition 13 years after the initial diagnosis. Transarterial embolization and medical treatments decreased the edema. Subsequently, however, a large intraparenchymal cyst appeared, aggravating the patient's motor weakness. Aspiration of the cyst ameliorated these symptoms. The analysis of the fluid from the cyst revealed that it contained a very high concentration of protein. Although there is no proven pathogenic mechanism to explain these protein concentrations and the enlargement of the AVM, the authors hypothesize that the progressive edema resulted from a synergic augmentation of the inflow from the AVM and the progressive obstruction of venous drainage that is a hallmark of SWS. The formation of the cyst probably resulted from the blood vessel hyperpermeability that is inherent to SWS.
Kazuhiko Nishino, Yasushi Ito, Takatoshi Sorimachi, Junsuke Shimbo and Yukihiko Fujii
Kazuhiko Nishino, Yasushi Ito, Hitoshi Hasegawa, Bumpei Kikuchi, Junsuke Shimbo, Keiko Kitazawa and Yukihiko Fujii
Transvenous embolization (TVE) for the treatment of a cavernous sinus (CS) dural arteriovenous fistula (DAVF) occasionally causes cranial nerve palsy (CNP). Overpacking of coils is considered to result in CNP. The purpose of this study was to analyze the association of TVE-induced CNP with the volume and location of coils activated in the CS.
Thirty-one patients with CS DAVFs (33 lesions) underwent TVE.
Cranial nerve palsy occurred or was aggravated in 13 cases (39.4%; CNP group). The cumulative volume of activated coils was significantly greater in the CNP group (0.241 ± 0.172 cm3) than in the non-CNP group (0.119 ± 0.075 cm3; p < 0.05). Of those lesions with > 0.2 cm3 of coil volume, 77.8% showed immediate aggravation or a new occurrence of CNP after TVE. Five lesions treated with a smaller volume of coils showed a delayed worsening or occurrence of CNP. In cases with induced oculomotor nerve palsy, coils had been densely packed in the superolateral part of the anterior CS. Dense packing in the lateral portion of the posterior CS frequently induced abducent nerve palsy. Although patients harboring lesions with a greater coil volume required a longer recovery time, newly developed or aggravated CNP, related to 84.6% of the lesions, resolved completely.
The cumulative volume and specific locations of coils in the CS correlated with TVE-induced CNP. Overpacking appeared to be the predominant cause of CNP; however, for CNP in cases involving smaller coil volumes, an alternative mechanism may be involved.