scholarly journals Moyamoya Disease in Infancy: Application of a Novel Indirect Revascularization Technique for Bihemispheric Cortical Synangiosis

Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Danielle DeBacker Dang ◽  
John V Dang ◽  
Duaa AbdelHameid ◽  
Seth Mansinghani ◽  
Leon E Moores
Keyword(s):  
Moyamoya Disease ◽  
Literature Search ◽  
Case Series ◽  
Combined Approach ◽  
Pial Surface ◽  
Frontal Pole ◽  
Safety And Efficacy ◽  
Indirect Revascularization ◽  
Cerebral Arteriopathy ◽  
Scalp Incision

Abstract INTRODUCTION Moyamoya disease (MMD) is a rare progressive cerebral arteriopathy characterized by nonatherosclerotic steno-occlusive lesions of the Circle of Willis. Presentation in infancy is rare and usually presents with ischemic stroke. We present a 7-mo old female with bilateral MMD who presented for indirect revascularization after 2 successive strokes in multiple vascular territories. METHODS We demonstrate a novel indirect revascularization technique in infant MMD whereby the patient's multifocal ischemic burden and STA caliber precluded arterial and myosynangiosis. We instead utilized bihemispheric pericranium for revascularization of bilateral oligemic cerebrum. Through a bicoronal scalp incision and inverted T-shaped pericranial incision, large anteriorly-based pericranial flaps were applied to the exposed pial surface using bilateral hemicraniotomies. Synangiosis extended from the frontal pole into parieto-occipital territories. We additionally performed a literature search on bypass approaches used for infant MMD. RESULTS A total of 10 infant MMD cases have been reported in the literature. Our case represents the only use of a pericranial graft in an infant without a combined approach. Pial synangiosis, EDAS, and conservative management represent techniques described in this population. Two case series utilized pericranial flaps with EDAS, however, no infants were included nor did revascularization extend beyond traditional margins. Our patient remains stroke and seizure-free for at least 16-months. She is ambulating independently and meeting her pediatric milestones. CONCLUSION Indirect revascularization is favored in pediatric MMD with approaches primarily utilizing native vessel donor grafts. Bilateral revascularization is performed in a delayed, sequential fashion if indicated. Further, collateralization is limited by the anatomical graft parameters, making hemispheric pathology difficult to comprehensively treat with these approaches. Our case demonstrates the safety and efficacy of utilizing a large bihemispheric pericranial flap on its native pedicle with bilateral hemicraniotomies for revascularization of multiple cerebrovascular territories in severe infant MMD. Our technique also allows for preservation of critical native vessels should the need for reoperation arise.

So Much Writing, So Little Science: A Review of 37 Years of Literature on Edetate Sodium Chelation Therapy

1993 ◽  
Vol 27 (12) ◽  
pp. 1504-1509 ◽  
Author(s):  
Michael T. Grier ◽  
David G. Meyers
Keyword(s):  
Clinical Trials ◽  
Clinical Practice ◽  
Literature Search ◽  
Chelation Therapy ◽  
Case Reports ◽  
Scientific Evidence ◽  
Case Series ◽  
Mechanisms Of Action ◽  
Tetraacetic Acid ◽  
Safety And Efficacy

OBJECTIVE: To determine the safety and efficacy of edetate sodium (ethylenediamine tetraacetic acid; EDTA) chelation therapy for atherosclerosis. METHODS: Literature search using MEDLINE, encompassing 1966 through May 1993. Further references were obtained from articles and books, and from citations obtained from the American Academy of Medical Preventics. RESULTS: 16 case reports or case series, 2 longitudinal studies, and 3 clinical trials were reviewed, along with testimonials cited in 19 books. CONCLUSIONS: Little valid scientific evidence is available. Although the postulated mechanisms of action for EDTA are biologically plausible and EDTA appears to be safe, it has not been proven effective. Indeed, the best evidence shows it to be ineffective. Therefore, EDTA chelation therapy should not be used in clinical practice to treat atherosclerosis.

scholarly journals ACTA2 Cerebral Arteriopathy: Not Just a Puff of Smoke

2018 ◽  
Vol 46 (3-4) ◽  
pp. 159-169 ◽  
Author(s):  
Joshua A. Cuoco ◽  
Christopher M. Busch ◽  
Brendan J. Klein ◽  
Michael J. Benko ◽  
Rachel Stein ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Moyamoya Disease ◽  
Literature Search ◽  
Case Reports ◽  
Smooth Muscle Actin ◽  
Muscle Dysfunction ◽  
Missense Mutations ◽  
Radiographic Findings ◽  
Cerebral Arteriopathy ◽  
Smooth Muscle Dysfunction

Background: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. Summary: We performed a literature search using PubMed with the key words “ACTA2 mutation,” “ACTA2 cerebral arteriopathy,” and “multisystemic smooth muscle dysfunction syndrome.” Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.

scholarly journals Direct versus indirect revascularization procedures for moyamoya disease: a comparative effectiveness study

2017 ◽  
Vol 126 (5) ◽  
pp. 1523-1529 ◽  
Author(s):  
Luke Macyszyn ◽  
Mark Attiah ◽  
Tracy S. Ma ◽  
Zarina Ali ◽  
Ryan Faught ◽  
...  
Keyword(s):  
Decision Analysis ◽  
Moyamoya Disease ◽  
Literature Search ◽  
Definitive Treatment ◽  
Decision Analysis Model ◽  
Life Years ◽  
Significant Difference ◽  
Indirect Revascularization ◽  
Revascularization Procedures

OBJECTIVEMoyamoya disease (MMD) is a chronic cerebrovascular disease that can lead to devastating neurological outcomes. Surgical intervention is the definitive treatment, with direct, indirect, and combined revascularization procedures currently employed by surgeons. The optimal surgical approach, however, remains unclear. In this decision analysis, the authors compared the effectiveness of revascularization procedures in both adult and pediatric patients with MMD.METHODSA comprehensive literature search was performed for studies of MMD. Using complication and success rates from the literature, the authors constructed a decision analysis model for treatment using a direct and indirect revascularization technique. Utility values for the various outcomes and complications were extracted from the literature examining preferences in similar clinical conditions. Sensitivity analysis was performed.RESULTSA structured literature search yielded 33 studies involving 4197 cases. Cases were divided into adult and pediatric populations. These were further subdivided into 3 different treatment groups: indirect, direct, and combined revascularization procedures. In the pediatric population at 5- and 10-year follow-up, there was no significant difference between indirect and combination procedures, but both were superior to direct revascularization. In adults at 4-year follow-up, indirect was superior to direct revascularization.CONCLUSIONSIn the absence of factors that dictate a specific approach, the present decision analysis suggests that direct revascularization procedures are inferior in terms of quality-adjusted life years in both adults at 4 years and children at 5 and 10 years postoperatively, respectively. These findings were statistically significant (p < 0.001 in all cases), suggesting that indirect and combination procedures may offer optimal results at long-term follow-up.

Clinical Features and Surgical Outcomes of Patients With Moyamoya Disease and the Homozygous RNF213 p.R4810K Variant

2019 ◽  
Vol 34 (13) ◽  
pp. 793-800 ◽  
Author(s):  
Qian Zhang ◽  
Peicong Ge ◽  
Yonggang Ma ◽  
Dong Zhang ◽  
Rong Wang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Surgical Outcomes ◽  
Carotid Arteries ◽  
Susceptibility Gene ◽  
Case Series ◽  
External Carotid ◽  
First Case ◽  
Disease Case ◽  
Indirect Revascularization

The ring-finger protein 213 ( RNF213) gene is a major susceptibility gene for moyamoya disease. The homozygote of the p.R4810K variant on RNF213 exhibits an early onset age and severe form of moyamoya disease. We report 4 unrelated pediatric moyamoya disease cases with the homozygous p.R4810K variant and the long-term surgical outcomes. Published reports on surgical outcome of moyamoya disease case with the homozygous p.R4810K variant were reviewed. Cerebral angiography revealed classic angiographic findings of moyamoya disease in 7 hemispheres of the 4 children. All patients underwent successful indirect revascularization. Abundant collateral blood flow from the external carotid arteries to the internal carotid arteries was observed in all bypass procedures by angiography. Improvements in symptoms and cerebral blood volume were observed in all patients at long-term follow-up. This report is the first case series in the literature on the surgical management of these patients. These cases highlight the effectiveness of indirect revascularization for moyamoya disease patients with the homozygous p.R4810K variant. Early diagnosis and treatment are crucial to avoid irreversible neurologic deficits in these patients.

scholarly journals Safety and efficacy of cold‐water immersion in the treatment of older patients with heat stroke: a case series

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Chikao Ito ◽  
Isao Takahashi ◽  
Miyuki Kasuya ◽  
Kyoji Oe ◽  
Masahito Uchino ◽  
...  
Keyword(s):  
Older Patients ◽  
Cold Water ◽  
Water Immersion ◽  
Heat Stroke ◽  
Case Series ◽  
Cold Water Immersion ◽  
Safety And Efficacy

Poster 59 Moyamoya Disease and Rehabilitation Outcomes: A Case Series

PM&R ◽  
2011 ◽  
Vol 3 ◽  
pp. S197-S197
Author(s):  
Mallikarjuna Nallegowda ◽  
Ziyad Ayyoub ◽  
Murray Brandstater ◽  
Jared Myers ◽  
Jiangnan Wang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Case Series ◽  
Rehabilitation Outcomes

scholarly journals Sinus Floor Augmentation—Associated Surgical Ciliated Cysts: Case Series and a Systematic Review of the Literature

2021 ◽  
Vol 11 (4) ◽  
pp. 1903
Author(s):  
Adrian Kahn ◽  
Shlomo Matalon ◽  
Rahaf Bassam Salem ◽  
Lazar Kats ◽  
Liat Chaushu ◽  
...  
Keyword(s):  
Systematic Review ◽  
Microscopic Examination ◽  
Literature Search ◽  
Case Series ◽  
Systematic Literature Search ◽  
Review Of The Literature ◽  
Inclusion Criteria ◽  
Sinus Floor Augmentation ◽  
Sinus Floor

This study aimed to characterize the demographic and clinical features of underreported surgical ciliated cysts developing after sinus floor augmentation, based on a series of cases from our files and a systematic review of the literature. A series of five cases (four patients) of microscopically confirmed surgical ciliated cysts following sinus floor augmentation procedures from our files are described. A systematic literature search (1991–2020) with strict clinical-, radiological- and microscopic-based exclusion and inclusion criteria was performed to detect additional similar cases. The systematic review revealed only five cases that fulfilled the inclusion criteria. Altogether, surgical ciliated cysts associated with sinus floor augmentation have been rarely reported in the literature, and have not been characterized either demographically or clinically. Graft materials were diverse, implants were placed simultaneously, or up to two years post-augmentation. The associated surgical ciliated cysts developed between 0.5 and 10 years post-augmentation. Although limited in its extent, this study is the first series to characterize possible underreported sequelae of surgical ciliated cysts associated with sinus floor augmentation. It emphasizes the need for long post-operative follow-up and confirmation of lesion by microscopic examination.

Cerebral arteriopathy in ACTA2 mutations: a spectrum of disease highlighted by a case of variable penetrance in two siblings

2021 ◽  
pp. 1-6
Author(s):  
Christine Tschoe ◽  
Teddy E. Kim ◽  
Kyle M. Fargen ◽  
Stacey Q. Wolfe
Keyword(s):  
Moyamoya Disease ◽  
De Novo ◽  
Early Recognition ◽  
Smooth Muscle Actin ◽  
Amino Acid Replacement ◽  
Single Mutation ◽  
Cerebral Arteriopathy ◽  
And Performance ◽  
Actin Protein ◽  
Revascularization Procedures

Until recently, cerebral arteriopathy due to heterozygous mutations of the ACTA2 gene was considered a variant of moyamoya disease. However, radiographic analysis of patients with these mutations reveals a distinctive angiographic appearance from that seen in moyamoya disease. Several heterozygous missense ACTA2 mutations have been implicated in the development of this distinct cerebrovascular entity; however, the penetrance and systemic manifestations of these mutations vary based on the location of the amino acid replacement within the α–smooth muscle actin protein. The severity of the phenotype may also differ among patients within a single mutation type. There is limited literature on the safety and efficacy of revascularization procedures for ACTA2 arteriopathy, which have been limited to those patients with known Arg179His mutations. The authors provide a review of the breadth of mutations within the ACTA2 literature and report a case of two siblings with de novo ACTA2 Arg258Cys mutations with differing clinical courses, highlighting the utility of indirect revascularization with 8-year follow-up data. This case highlights the importance of early recognition of the angiographic appearance of ACTA2 cerebral arteriopathy and performance of genetic testing, as the location of the mutation impacts clinical presentation and outcomes.

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