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, Hitoshi Hasegawa, Kenichi Morita, Masafumi Fukuda, Yasushi Ito, Yukihiko Fujii and Mitsuya Sato
Arteriovenous malformations (AVMs) in the cerebellopontine angle cistern (CPAC) are specific lesions that can cause neurovascular compression syndromes as well as intracranial hemorrhage. Although case reports describing the CPAC AVMs, especially those presenting with trigeminal neuralgia (TN), have been accumulating by degrees, the pathophysiology of CPAC AVMs remains obscure. The authors' purpose in the present study was to evaluate the clinical and radiographic features of CPAC AVMs as well as the treatment options.
This study defined a CPAC AVM as a small AVM predominantly located in the CPAC with minimal extension into the pial surface of the brainstem and closely associated with cranial nerves. All patients with CPAC AVMs treated in the authors' affiliated hospitals over a 16-year period were retrospectively identified. Clinical charts, imaging studies, and treatment options were evaluated.
Ten patients (6 men and 4 women), ranging in age from 56 to 77 years (mean 65.6 years), were diagnosed with CPAC AVMs according to the authors' definition. Six patients presented with hemorrhage, 3 with TN, and the remaining patient developed a hemorrhage subsequent to TN. Seven AVMs were associated with the trigeminal nerve (Group V), and 3 with the facial-vestibulocochlear nerve complex (Group VII–VIII). All patients in Group VII–VIII presented with the hemorrhage instead of hemifacial spasm. Regarding angioarchitecture, the intrinsic pontine arteries provided the blood supply for all CPAC AVMs in Group V. In addition, 5 of 7 AVMs with hemorrhagic episodes accompanied flow-related aneurysms, although no aneurysm was detected in patients with TN alone. With respect to treatment, all patients with hemorrhagic presentation underwent Gamma Knife surgery (GKS), resulting in favorable outcomes except for 1 patient who experienced rebleeding after GKS, which was caused by the repeated rupture of a feeder aneurysm. The AVMs causing TN were managed with surgery, GKS, or a combination, according to the nidus-nerve relationship. All patients eventually obtained pain relief.
Clinical symptoms caused by CPAC AVMs occur at an older age compared with AVMs in other locations; CPAC AVMs also have distinctive angioarchitectures according to their location in the CPAC. Although GKS is likely to be an effective treatment option for the CPAC AVMs with hemorrhagic presentations, it seems ideal to obliterate the flow-related aneurysms before performing GKS, although this is frequently challenging. For CPAC AVMs with TN, it is important to evaluate the nidus-nerve relationship before treatment, and GKS is especially useful for patients who do not require urgent pain relief.
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.
Junichi Yoshimura, Yoshihiro Tsukamoto, Masakazu Sano, Hitoshi Hasegawa, Kazuhiko Nishino, Akihiko Saito, Masafumi Fukuda, Kouichirou Okamoto and Yukihiko Fujii
The authors report a rare case of a huge hypervascular tentorial cavernous angioma treated with preoperative endovascular embolization, followed by successful gross-total removal. A 15-year-old girl presented with scintillation, diplopia, and papilledema. Computed tomography and MRI studies revealed a huge irregularly shaped tumor located in the right occipital and suboccipital regions. The tumor, which had both intra- and extradural components, showed marked enhancement and invasion of the overlying occipital bone. Angiography revealed marked tumor stain, with blood supply mainly from a large branch of the left posterior meningeal artery. Therefore, this lesion was diagnosed as a tentorium-based extraaxial tumor. For differential diagnosis, meningioma, hemangiopericytoma, and malignant skull tumor were considered. Tumor feeders were endovascularly embolized with particles of polyvinyl alcohol. On the following day, the tumor was safely gross totally removed with minimum blood loss. Histopathological examination confirmed the diagnosis of cavernous angioma. To date, there have been no reports of tentorium-based cavernous angiomas endovascularly embolized preoperatively. A tentorial cavernous angioma is most likely to show massive intraoperative bleeding. Therefore, preoperative embolization appears to be quite useful for safe maximum resection. Hence, the authors assert that the differential diagnosis of tentorium-based tumors should include tentorial cavernous angioma, for which preoperative endovascular embolization should be considered.