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Patrick J. McDonald, Charles C. Matouk, Blake Papsin and James T. Rutka

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Ajay Malhotra, Xiao Wu, Timothy Miller, Charles C. Matouk, Pina Sanelli and Dheeraj Gandhi

OBJECTIVE

Both endovascular coiling and the Pipeline embolization device (PED) have been shown to be safe and clinically effective for treatment of small (< 10 mm) aneurysms. The authors conducted a comparative effectiveness analysis to compare the utility of these treatment methods in terms of health benefits.

METHODS

A decision-analytical study was performed with Markov modeling methods to simulate patients with small unruptured aneurysms undergoing endovascular coiling, stent-assisted coiling (SAC), or PED placement for treatment. Input probabilities were derived from prior literature, and 1-way, 2-way, and probabilistic sensitivity analyses were performed to assess model and input parameter uncertainty.

RESULTS

The base case calculation for a 50-year-old man reveals PED to have a higher health benefit (17.48 quality-adjusted life years [QALYs]) than coiling (17.44 QALYs) or SAC (17.36 QALYs). PED is the better option in 6020 of the 10,000 iterations in probabilistic sensitivity analysis. When the retreatment rate of PED is lower than 9.53%, and the coiling retreatment is higher than 15.6%, PED is the better strategy. In the 2-way sensitivity analysis varying the retreatment rates from both treatment modalities, when the retreatment rate of PED is approximately 14% lower than the retreatment rate of coiling, PED is the more favorable treatment strategy. Otherwise, coiling is more effective. SAC may be better than PED when the unfavorable outcome risk of SAC is lower than 70% of its reported current value.

CONCLUSIONS

With the increasing use of PEDs for treatment of small unruptured aneurysms, the current study indicates that these devices may have higher health benefits due to lower rates of retreatment compared to both simple coiling and stent-assisted techniques. Longer follow-up studies are needed to document the rates of recurrence and retreatment after coiling and PED to assess the cost-effectiveness of these strategies.

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Daniel Duran, Philipp Karschnia, Jonathan R. Gaillard, Jason K. Karimy, Mark W. Youngblood, Michael L. DiLuna, Charles C. Matouk, Beverly Aagaard-Kienitz, Edward R. Smith, Darren B. Orbach, Georges Rodesch, Alejandro Berenstein, Murat Gunel and Kristopher T. Kahle

Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation–arteriovenous malformation syndrome (RASA1) and hereditary hemorrhagic telangiectasia (ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients’ families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances in the diagnosis and treatment of patients with VOGM.

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Christoph J. Griessenauer, Christopher S. Ogilvy, Nimer Adeeb, Adam A. Dmytriw, Paul M. Foreman, Hussain Shallwani, Nicola Limbucci, Salvatore Mangiafico, Ashish Kumar, Caterina Michelozzi, Timo Krings, Vitor Mendes Pereira, Charles C. Matouk, Mark R. Harrigan, Hakeem J. Shakir, Adnan H. Siddiqui, Elad I. Levy, Leonardo Renieri, Thomas R. Marotta, Christophe Cognard and Ajith J. Thomas

OBJECTIVE

Flow diversion for posterior circulation aneurysms performed using the Pipeline embolization device (PED) constitutes an increasingly common off-label use for otherwise untreatable aneurysms. The safety and efficacy of this treatment modality has not been assessed in a multicenter study.

METHODS

A retrospective review of prospectively maintained databases at 8 academic institutions was performed for the years 2009 to 2016 to identify patients with posterior circulation aneurysms treated with PED placement.

RESULTS

A total of 129 consecutive patients underwent 129 procedures to treat 131 aneurysms; 29 dissecting, 53 fusiform, and 49 saccular lesions were included. At a median follow-up of 11 months, complete and near-complete occlusion was recorded in 78.1%. Dissecting aneurysms had the highest occlusion rate and fusiform the lowest. Major complications were most frequent in fusiform aneurysms, whereas minor complications occurred most commonly in saccular aneurysms. In patients with saccular aneurysms, clopidogrel responders had a lower complication rate than did clopidogrel nonresponders. The majority of dissecting aneurysms were treated in the immediate or acute phase following subarachnoid hemorrhage, a circumstance that contributed to the highest mortality rate in those aneurysms.

CONCLUSIONS

In the largest series to date, fusiform aneurysms were found to have the lowest occlusion rate and the highest frequency of major complications. Dissecting aneurysms, frequently treated in the setting of subarachnoid hemorrhage, occluded most often and had a low complication rate. Saccular aneurysms were associated with predominantly minor complications, particularly in clopidogrel nonresponders.