Diffuse Cerebral Edema After Moyamoya Disease-Related Intracerebral Hemorrhage: A Case Report

2020 ◽  
pp. 194187442098061
Author(s):  
Alvin S. Das ◽  
Robert W. Regenhardt ◽  
Nirav Patel ◽  
Steven K. Feske ◽  
Matthew B. Bevers ◽  
...  

Moyamoya disease (MMD) is a rare, progressive occlusive disease characterized by bilateral internal carotid artery hypoplasia that often presents with ischemic stroke and intracerebral hemorrhage (ICH). Although MMD-related ICH is generally managed similarly to spontaneous ICH, we present a case in which standard management strategies may have led to an unprecedented devastating outcome. A 37-year-old female without any previous medical history presented with headache and right-sided weakness. A computed tomography (CT) scan revealed a large left basal ganglia ICH. Vessel imaging revealed diffuse narrowing of the entire anterior circulation with prominent leptomeningeal collaterals consistent with MMD. The patient’s systolic blood pressure was kept under 140 mmHg. During the hospitalization, she became hypocarbic while being trialed on pressure support ventilation. Several hours later, she developed fixed and dilated pupils. Repeat CT head showed new diffuse cerebral edema with tonsillar herniation. Despite hyperosmolar therapy, paralytics, pentobarbital, and cerebrospinal fluid diversion, no improvement was noted. Unfortunately, brain MRI revealed multifocal brainstem infarcts with superimposed Duret hemorrhages. Herein, we report diffuse cerebral edema as a complication of MMD-related ICH. We hypothesize that disruptions of delicate cerebral autoregulatory mechanisms led to extensive hypoxic-ischemic injury. In the setting of ICH, aggressive blood pressure management coupled with relative hypocapnia may have likely caused vasoconstriction of poorly compliant arteries leading to worsened cerebral blood flow and ischemia. Therefore, because of its complex pathophysiology, strict adherence to eucapnia should be maintained in MMD-related ICH.

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Szu-Ju Chen ◽  
Hsin-Hsi Tsai ◽  
Li-Kai Tsai ◽  
Ya-Fang Chen ◽  
Sung-Chun Tang ◽  
...  

Background: Intensive blood pressure (BP) reduction is regarded as the gold standard therapy for acute intracerebral hemorrhage (ICH), but its associations to cerebral hypoperfusion and ischemic lesions have been suspected. This study aims to investigate the impact of acute BP reduction on the development of acute ischemic lesions (AILs) at border zone (BZ) areas in patients with hypertensive ICH. Methods: We enrolled patients with acute hypertensive ICH (hemorrhagic lesions restricted to deep region [Strictly deep-ICH] or located in mixed lobar and deep areas [Mixed-ICH]) who received brain MRI within 7 days after ICH onset. BZ AILs were defined as lesions locating at BZ areas that were hyperintense on DWI sequence and hypointense on ADC series (figure). Acute SBP change was the difference between the initial SBP and the SBP recorded at 24 hours after ICH onset. Results: Of the 274 enrolled patients (62.5 ± 12.7 years old, 65% male), 11 subjects had BZ AILs. Compared to patients without BZ AILs, patients with lesions had wider amount of acute SBP reduction (71.7 ± 33.6 vs. 43.0 ± 32.2 mmHg, P = 0.023), more lobar and deep microbleeds (MB) and larger white matter hyperintensity volume (all p < 0.05). Using ROC curve analysis, acute SBP drop at more than 54mmHg was linked to the occurrence of AILs (sensitivity 82%, specificity 64%, P = 0.002). In multiple logistic regression model, acute SBP decline at above 54mmHg (OR 11.45, 95%CI 2.06 - 63.49, P = 0.005) and higher deep MB burden ( P = 0.032) raised the risk of AILs after adjustment for age, sex, and image markers of cerebral small vessel disease. In subgroup analysis, larger acute SBP drop remained to be an independent risk factor for development of AILs in patients with Mixed-ICH ( P = 0.008), but not in patients with strictly deep-ICH ( P = 0.715). Conclusion: Acute SBP change in hypertensive ICH, especially in Mixed-ICH, increases the risk of AILs at BZ areas, showing widespread microangiopathy that is vulnerable to rapid BP dysregulation to ischemia.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Shyam Prabhakaran ◽  
David S Liebeskind ◽  
George Cotsonis ◽  
Azhar Nizam ◽  
Edward Feldmann ◽  
...  

Introduction: Prior studies have evaluated risks factors for recurrent stroke in patients with symptomatic intracranial atherosclerotic disease (ICAD). However, few reports have assessed risk factors for early infarct recurrence in the territory distal to the symptomatic artery. Methods: We analyzed data from patients who underwent study-paid brain MRI at 6-8 weeks after enrollment in the ongoing MyRIAD study, an NIH/NINDS funded prospective multicenter observational study of patients with recent ( < 21 days) stroke or TIA (recurrent or with DWI) caused by ICAD 50-99% without planned angioplasty/stenting. The outcome of interest was new infarcts on brain MRI (on DWI or FLAIR) at 6-8 weeks compared to qualifying brain MRI at time of index stroke or TIA. Qualifying events and clinical and radiographic outcomes are centrally ascertained by 2 independent reviewers. We used logistic regression to identify independent clinical predictors of new infarct in the territory of the symptomatic artery. Results: Among 84 (80%) of 105 enrolled patients in MyRIAD with 6-8 week MRI, the mean age was 63.6 + 12.4 years, 83.1% have stenosis 70-99%, and 51.2% had history of diabetes; those who underwent MRI did not differ from those who did not undergo MRI. A new DWI/FLAIR infarct in the territory of the symptomatic artery was noted in 26.2%. Those with recurrent infarcts were younger (57.7 vs. 65.7 years, p=0.009), more likely to have diabetes (71.4% vs. 44.3%, p=0.043), have greater degree of stenosis (82.5% vs. 76.0%, p=0.099), and have greater decline in systolic blood pressure (SBP) from enrollment to 6-8 week follow-up (+5 vs. -6.2 mm Hg, p=0.074). In adjusted analyses, age (aOR 0.922, 95% CI 0.869-0.979) and change in SBP (aOR 0.967, 95% CI 0.937-0.997) were related to new infarct in the territory. Conclusions: Early recurrent infarcts occur in more than one-quarter of patients with symptomatic ICAD, may be a suitable biomarker of disease activity, and add to the subclinical burden of this high-risk disease. Given the association between SBP lowering and infarct recurrence, studies of early blood pressure management strategies, including extended permissive hypertension, may be warranted in this population.


2020 ◽  
pp. 1-8
Author(s):  
Ryosuke Tashiro ◽  
Miki Fujimura ◽  
Masahito Katsuki ◽  
Taketo Nishizawa ◽  
Yasutake Tomata ◽  
...  

OBJECTIVESuperficial temporal artery–middle cerebral artery (STA-MCA) anastomosis is the standard surgical management for moyamoya disease (MMD), whereas cerebral hyperperfusion (CHP) is one of the potential complications of this procedure that can result in delayed intracerebral hemorrhage and/or neurological deterioration. Recent advances in perioperative management in the early postoperative period have significantly reduced the risk of CHP syndrome, but delayed intracerebral hemorrhage and prolonged/delayed CHP are still major clinical issues. The clinical implication of RNF213 gene polymorphism c.14576G>A (rs112735431), a susceptibility variant for MMD, includes early disease onset and a more severe form of MMD, but its significance in perioperative pathology is unknown. Thus, the authors investigated the role of RNF213 polymorphism in perioperative hemodynamics after STA-MCA anastomosis for MMD.METHODSAmong 96 consecutive adult patients with MMD comprising 105 hemispheres who underwent serial quantitative cerebral blood flow (CBF) analysis by N-isopropyl-p-[123I]iodoamphetamine SPECT after STA-MCA anastomosis, 66 patients consented to genetic analysis of RNF213. Patients were routinely maintained under strict blood pressure control during and after surgery. The local CBF values were quantified at the vascular territory supplied by the bypass on postoperative days (PODs) 1 and 7. The authors defined the radiological CHP phenomenon as a local CBF increase of more than 150% compared with the preoperative values, and then they investigated the correlation between RNF213 polymorphism and the development of CHP.RESULTSCHP at POD 1 was observed in 23 hemispheres (23/73 hemispheres [31.5%]), and its incidence was not statistically different between groups (15/41 [36.6%] in RNF213-mutant group vs 8/32 [25.0%] in RNF213–wild type (WT) group; p = 0.321). CHP on POD 7, which is a relatively late period of the CHP phenomenon in MMD, was evident in 9 patients (9/73 hemispheres [12.3%]) after STA-MCA anastomosis. This prolonged/delayed CHP was exclusively observed in the RNF213-mutant group (9/41 [22.0%] in the RNF213-mutant group vs 0/32 [0.0%] in the RNF213-WT group; p = 0.004). Multivariate analysis revealed that RNF213 polymorphism was significantly associated with CBF increase on POD 7 (OR 5.47, 95% CI 1.06–28.35; p = 0.043).CONCLUSIONSProlonged/delayed CHP after revascularization surgery was exclusively found in the RNF213-mutant group. Although the exact mechanism underlying the contribution of RNF213 polymorphism to the prolonged/delayed CBF increase in patients with MMD is unclear, the current study suggests that genetic analysis of RNF213 is useful for predicting the perioperative pathology of patients with MMD.


2020 ◽  
Vol 5 (4) ◽  
pp. 2473011420S0040
Author(s):  
Ryan G. Rogero ◽  
Emmanuel M. Illical ◽  
Daniel Corr ◽  
Steven M. Raikin ◽  
James Krieg ◽  
...  

Category: Ankle; Trauma Introduction/Purpose: With an increasing frequency of syndesmotic fixation during ankle fracture ORIF and no current gold standard management protocol, it is important for surgeons to understand the frequency and usage patterns of the various techniques among other orthopaedic surgeons. The purposes of this study are to determine how orthopaedic surgeons currently manage ankle fractures with concomitant syndesmotic disruption and to identify surgeon demographics predictive of syndesmotic management. Methods: An 18-question survey, including 10 specific syndesmotic management questions was sent to the Orthopaedic Trauma Association (OTA) and Canadian Orthopaedic Association (COA), as well as sent to email addresses of foot and ankle-fellowship trained surgeons. Surgeon demographic questions included years, country, and type of practice, fellowship(s) completed, setting of ankle fracture surgery, and number of ankle fractures operated on per year. Multinomial regression analysis was performed to determine if surgeon demographics were predictive of syndesmotic management. Results: One-hundred ten orthopaedic surgeons completed our survey. Selected predictors of syndesmotic management included: private practice with academic appointments (0.077 [0.007, 0.834]; p=0.035) being predictive of not using screws through an ORIF plate; foot & ankle fellowship (9.981 [1.787, 55.764]; p=0.009) and trauma fellowship (6.644 [1.302, 33.916]; p=0.023) predictive of utilizing screws through a plate; no fellowship (14.886 [1.226, 180.695]; p=0.034) predictive of only using 1 screw; and surgeons practicing in the U.S. were more likely to not use screws across just 3 cortices (0.031 [0.810, 3.660]; p=0.009). Additionally, among those utilizing suture-button devices, foot & ankle fellowship-trained surgeons were more likely to implement suture-button through plate (7.676 [1.286, 45.806]; p=0.025). Conclusion: Several surgeon factors influence decision making in the management of ankle fractures with syndesmotic disruption. This study raises awareness of differences in management strategies that should be used for further discussion when determining a potential gold standard for management of these complex injuries.


Sign in / Sign up

Export Citation Format

Share Document