Moyamoya disease (MMD) is a cerebrovascular disease of unknown etiology characterized by stenotic and occlusive arterial changes of the anterior circulation, with subsequent proliferative development of arterial collateralization. In spite of there being limited understanding of the clear etiology of MMD, surgical revascularization for MMD is considered the standard treatment to prevent further stroke. While the use of surgical revascularization to prevent future hemorrhagic stroke in MMD is still controversial, it is considered effective in the case of ischemic stroke. This article presents a review of the current surgical management of MMD based on an analysis of the most recent data from peer-reviewed articles and opinion based on personal experience with surgical revascularization in the treatment of MMD.

OBJECTIVE Currently, there is no established standard regarding the ideal number of external ventricular drain (EVD) clamp trials performed before ventriculoperitoneal (VP) shunt insertion following nontraumatic subarachnoid hemorrhage (SAH). In this study, the authors aimed to evaluate this relationship. METHODS A retrospective review of all patients presenting with SAH between July 2007 and December 2016 was performed. Patients with SAH who had received an EVD within the first 24 hours of hospital admission and had undergone at least 1 clamp trial prior to EVD removal were eligible for inclusion in the study. Patient demographics, clinical presentations, SAH etiologies and grades, clamp trial data, hospital lengths of stay, and functional outcomes were recorded. RESULTS One hundred fourteen patients with nontraumatic SAH complicated by posthemorrhagic hydrocephalus were included in the study. The median patient age was 57 years (range 28-90 years), with a male/female ratio of 1:1.7. A ruptured aneurysm was the underlying etiology of SAH in 79.8% of patients. A majority of patients (69.4%) had a Hunt and Hess grade III-V on admission. The median number of clamp trials performed was 2 (range 1-6). A VP shunt was required in 40.4% of patients. In those who underwent 2 and 3 clamp trials, 60% and 38.9%, respectively, did not require subsequent VP shunt placement. CONCLUSIONS Surgical placement of a VP shunt is associated with complications. Clamp trials are routinely performed before making the decision to insert a shunt. In the present study, the authors found that a significant percentage of patients passed their second and third clamp trials without requiring subsequent shunt insertion. These data support performing multiple clamp trials prior to shunt placement.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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