The co-occurrence of a brain arteriovenous malformation, Moyamoya Disease, and intracranial aneurysm is exceedingly rare. We report the third case of this disease constellation, and the first where the aneurysm arises from the Moyamoya collateral vessel. We review the relevant literature and discuss proposed pathophysiological mechanisms and clinical implications.
Objective The study aimed to determine long-term outcomes in patients with intraoperative electrical conduction block in an anatomically intact facial nerve (FN).
Patients and Methods Single center retrospective review of prospectively collected database of all vestibular schwannoma surgeries between January 1, 2008 and August 25, 2015. Operative notes were reviewed and patients with anatomically intact FNs, but complete conduction block at the end of surgery were included for analysis.
Results In total, 371 patients had vestibular schwannoma surgery of which 18 met inclusion criteria. Mean follow-up was 34.28 months and average tumor size was 28.00 mm. Seventeen patients had House-Brackmann Grade VI facial palsy immediately postoperatively and one patient was grade V. At 1 year, three patients remained grade VI (17%), two improved to grade V (11%), seven to grade IV (39%), six to grade III (33%), and one patient to grade II (6%). On extended follow-up, five patients (28%) had additional 1 to 2 score improvement in facial function. Subset analysis revealed no correlation of tumor size, vascularity, adherence to nerve, operative approach, extent of resection, splaying of FN, and recurrent tumor or sporadic tumors to the extent of FN recovery.
Conclusion Intraoperative conduction block does not condemn a patient to permanent FN palsy. There is potential for a degree of recovery comparable with those undergoing nerve grafting. Our data do not clearly support a policy of same-surgery or early-postoperative primary nerve grafting in the event of a complete conduction block, and instead we favor monitoring for recovery in an anatomically intact nerve.
Colorectal cancer (CRC) is the second most common cause of cancer-related death in the United States comprising 7.9% of all new cancer diagnoses and 8.6% of all cancer deaths. The combined 5-year relative survival rate for all stages is 65.1% but in its most aggressive form, stage 4 CRC has a 5-year relative survival rate of just 15.1%. For most with stage 4 CRC, treatment is palliative not curative, with the goal to prolong overall survival and maintain an acceptable quality of life.The identification of unique cancer genomic and biologic markers allows patient-specific treatment options. Treatment of stage 4 CRC consists of systemic therapy with chemotherapeutic agents, surgical resection if feasible, potentially including resection of metastasis, palliative radiation in select settings, and targeted therapy toward growth factors. Despite advances in surgical and medical management, metastatic CRC remains a challenging clinical problem associated with poor prognosis and low overall survival.
Neurofibromatosis Type 2 (NF2) is a rare autosomal dominant disease caused by pathological variants of the tumor suppressor gene NF2 classically manifesting as bilateral vestibular schwannoma, intracranial and spinal neoplasms such as meningioma and ependymoma, and posterior subcapsular and cortical wedge lens opacities, with an average onset of 18-24 years of age. Approximately 50% of patients inherit the disorder from an affected parent with the remainder resulting from de novo mutations or genetic mosaicism. The most common variants of NF2 are due to C>T transitions resulting in nonsense pathological variants. Individuals with splice-site variants of NF2 display varied phenotypes. Here we present a family with NF2 as the result of an apparently de novo c.885+1G>A splicing variant in exon 9 of the NF2 gene not previously described in the literature. This variant appears to be associated with an extremely mild phenotype with regards to vestibular schwannomas, other schwannoma disease and meningioma, instead exhibiting a delayed presentation with a predilection for ependymomas. The non-classical presentation of this NF2 splicing variant illustrates the importance of keeping NF2 in the differential diagnosis in patients with multiple spinal ependymomas with delayed onset and may warrant genetic testing for NF2 variants in such patients.
The natural history and optimal treatment—of previously ruptured anterior communicating artery (ACOM) aneurysms that recur is unclear. This study determines whether our management decisions of endovascular treatment vs open surgery is correct with respect to recurrence and rebleeding rates.
A retrospective observational study. All patients presenting to a single centre with a subarachnoid haemorrhage (SAH) secondary to an ACOM aneurysm were included. Data was collected from November 2012 to September 2018. Data collected included baseline demographics, aneurysm characteristics, management, complications, follow-up imaging, and outcomes.
A total of 137 patients were included in the study. A total of 113 aneurysms were coiled and 19 were clipped. The indication for clipping was due to aneurysm morphology or the presence of a haematoma exerting mass effect. Rates of vasospasm, infarction, cerebrospinal fluid (CSF) diversion, rebleed, length of stay, and functional outcome were not significantly different in the 2 cohorts. Residual and recurrence was higher in the coiled cohort n = 46 vs clipping n = 2 (P = .0140) with over a quarter of these patients requiring further treatment at a median time of 16 mo. In a subgroup group analysis of coiled aneurysms, there was no statistical differences in rates of rebleed or functional outcome in those that recurred/had a residual and those that did not.
Patients treated with coiling have an increased risk of recurrence vs those treated with clipping. However, the risk of rebleed was not statistically significant. The prevention and impact of recurrence and residual aneurysms remain incompletely understood. Hence, treatment decisions should be considered carefully in a multidisciplinary setting.
False localizing signs involving cranial nerves are rare, even more so when involving the trigeminal nerve. Here we present the first case of trigeminal V2 sensory loss as a false localizing sign. The sensory dysfunction was caused by a large contralateral cystic vestibular schwannoma and subsequently improved after tumor resection. The clinical and radiographic features are described, and proposed mechanisms for this false localizing sign are discussed.
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