Cavernous Malformations and Hemorrhage Risk

2012 ◽  
pp. 141-146 ◽  
Author(s):  
Douglas Kondziolka ◽  
Edward A. Monaco III ◽  
L. Dade Lunsford
Keyword(s):  
Cavernous Malformations ◽  
Hemorrhage Risk

A benchmark approach to hemorrhage risk management of cavernous malformations

Neurology ◽  
2018 ◽  
Vol 90 (10) ◽  
pp. e856-e863 ◽  
Author(s):  
Sepide Kashefiolasl ◽  
Markus Bruder ◽  
Nina Brawanski ◽  
Eva Herrmann ◽  
Volker Seifert ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariable Analysis ◽  
Patient Characteristics ◽  
Young Age ◽  
Anatomical Location ◽  
Separate Analysis ◽  
Developmental Venous Anomaly ◽  
Cavernous Malformations ◽  
Risk Of Rupture ◽  
Hemorrhage Risk

ObjectiveDespite the low annual risk of hemorrhage associated with a cavernous malformation (CM) (0.6%–1.1% per year), the risk of rehemorrhage rate and severity of neurologic deficits is significantly higher; therefore, we aimed to evaluate the rupture risk of CMs depending on various factors.MethodsWe retrospectively analyzed medical records of all patients with CM admitted to our institution between 1999 and April 2016. Cavernoma volume, location of the lesion, existence of a developmental venous anomaly (DVA), number of cavernomas, and patient characteristics (sex, age, hypertension, and antithrombotic therapy) were assessed.ResultsOne hundred fifty-four patients with CM were included; 89 (58%) ruptured CMs were identified. In statistical univariable analysis, the existence of a DVA was significantly higher in the ruptured cavernoma group (p < 0.001; odds ratio [OR] 4.6). A multivariable analysis of all included independent risk factors designated young age (<45 years) (p < 0.05; OR 2.2), infratentorial location (p < 0.01; OR 2.9), and existence of a DVA (p < 0.0001; OR 4.7) with significantly higher risk of rupture in our patient cohort. A separate analysis of these anatomical locations, supratentorial vs infratentorial, indicated that the existence of a DVA (p < 0.01; OR 4.16) in ruptured supratentorial cases and CM volume (≥1 cm3) (p < 0.0001; OR 3.5) in ruptured infratentorial cases were significant independent predictors for hemorrhage.ConclusionsYoung age (<45 years), infratentorial location, and the presence of a DVA are associated with a higher hemorrhage risk. CM volume (≥1 cm3) and the existence of a DVA were independently in accordance with the anatomical location high risk factors for CM rupture.

Hemorrhage Risk of Untreated Isolated Cerebral Cavernous Malformations

2019 ◽  
Vol 131 ◽  
pp. e557-e561 ◽  
Author(s):  
Kathryn N. Kearns ◽  
Ching-Jen Chen ◽  
Kaan Yagmurlu ◽  
Stepan Capek ◽  
Thomas J. Buell ◽  
...  
Keyword(s):  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Hemorrhage Risk

scholarly journals Cavernous Malformation of Brainstem, Thalamus, and Basal Ganglia

Neurosurgery ◽  
2012 ◽  
Vol 72 (4) ◽  
pp. 573-589 ◽  
Author(s):  
Paritosh Pandey ◽  
Erick M. Westbroek ◽  
Peter A. Gooderham ◽  
Gary K. Steinberg
Keyword(s):  
Basal Ganglia ◽  
Neurological Deficits ◽  
Term Outcome ◽  
Cavernous Malformations ◽  
Long Term Outcome ◽  
Brain Malformations ◽  
Hemorrhage Risk ◽  
Clinical Records

Abstract BACKGROUND: Cavernous malformations (CMs) in deep locations account for 9% to 35% of brain malformations and are surgically challenging. OBJECTIVE: To study the clinical features and outcomes following surgery for deep CMs and the complication of hypertrophic olivary degeneration (HOD). METHODS: Clinical records, radiological findings, operative details, and complications of 176 patients with deep CMs were reviewed retrospectively. RESULTS: Of 176 patients with 179 CMs, 136 CMs were in the brainstem, 27 in the basal ganglia, and 16 in the thalamus. Cranial nerve deficits (51.1%), hemiparesis (40.9%), numbness (34.7%), and cerebellar symptoms (38.6%) presented most commonly. Hemorrhage presented in 172 patients (70 single, 102 multiple). The annual retrospective hemorrhage rate was 5.1% (assuming CMs are congenital with uniform hemorrhage risk throughout life); the rebleed rate was 31.5%/patient per year. Surgical approach depended on the proximity of the CM to the pial or ependymal surface. Postoperatively, 121 patients (68.8%) had no new neurological deficits. Follow-up occurred in 170 patients. Delayed postoperative HOD developed in 9/134 (6.7%) patients with brainstem CMs. HOD occurred predominantly following surgery for pontine CMs (9/10 patients). Three patients with HOD had palatal myoclonus, nystagmus, and oscillopsia, whereas 1 patient each had limb tremor and hemiballismus. At follow-up, 105 patients (61.8%) improved, 44 (25.9%) were unchanged, and 19 (11.2%) worsened neurologically. Good preoperative modified Rankin Score (98.2% vs 54.5%, P = .001) and single hemorrhage (89% vs 77.3%, P &lt; .05) were predictive of good long-term outcome. CONCLUSION: Symptomatic deep CMs can be resected with acceptable morbidity and outcomes. Good preoperative modified Rankin Score and single hemorrhage are predictors of good long-term outcome.

scholarly journals Influence of Pregnancy on Hemorrhage Risk in Women With Cerebral and Spinal Cavernous Malformations

Stroke ◽  
2021 ◽  
Vol 52 (2) ◽  
pp. 434-441
Author(s):  
Nycole K. Joseph ◽  
Shivram Kumar ◽  
Robert D. Brown ◽  
Giuseppe Lanzino ◽  
Kelly D. Flemming
Keyword(s):  
Spinal Cord ◽  
Neurological Complications ◽  
Reproductive Age ◽  
Childbearing Age ◽  
Cavernous Malformations ◽  
Familial Form ◽  
Hemorrhage Risk ◽  
Risk Of Hemorrhage ◽  
Record Review

Background and Purpose: Pregnancy as a hemorrhage risk factor in women with cavernous malformations (CMs) is controversial. We describe prospective hemorrhage risk in women who become pregnant after an established CM diagnosis. Methods: Beginning in 2015, we recruited consecutive patients with radiologically confirmed CM of the brain or spinal cord to participate in a prospective registry. Participants underwent a baseline and annual medical record review, imaging review, and annual surveys to ascertain pregnancies, deliveries, and neurological complications. We collected prospective hemorrhage data on reproductive age women. We calculated prospective hemorrhage rates on childbearing age patients based on the number of hemorrhages occurring post-CM diagnosis during pregnancy and nonpregnancy, divided by the years of follow-up and censored at last follow-up, age 46, or surgery in sporadic-form CM. The hemorrhage rates were compared and the 95% CI presented with a P value of <0.05. Results: Of 160 women, 90 presented with CM under the age of 46 (average age 31.6 years; 25.6% familial form; 46.7% with hemorrhage; 24.4% brain stem location). These 90 patients had 136 pregnancies before CM diagnosis; 36 pregnancies occurred at or after the diagnosis of CM. Four patients had a hemorrhage while pregnant or postpartum leading to the first CM diagnosis. During 402.6 years of follow-up while not pregnant, 42 hemorrhages occurred prospectively yielding a 10.4% per year (95% CI, 7.5–14.0) risk of hemorrhage while not pregnant. No hemorrhages occurred during 32 prospective pregnancies (26 live births including 1 twinset and 7 nonviable fetuses) after CM diagnosis during 26.9 years of pregnancy time yielding a rate of 0% per year (95% CI, 0–13.6). We found no statistical difference in the rates of hemorrhage during pregnant and nonpregnant time ( P =0.09). No hemorrhage occurred during delivery. Conclusions: Our prospective data suggest that pregnancy does not increase the risk of hemorrhage in women with a known brain or spinal cord CM and that vaginal delivery is safe in appropriate candidates.

Clinical course of untreated pediatric brainstem cavernous malformations: hemorrhage risk and functional recovery

2014 ◽  
Vol 13 (5) ◽  
pp. 471-483 ◽  
Author(s):  
Da Li ◽  
Shu-Yu Hao ◽  
Jie Tang ◽  
Xin-Ru Xiao ◽  
Gui-Jun Jia ◽  
...  
Keyword(s):  
Complete Recovery ◽  
Lesion Size ◽  
Full Recovery ◽  
Functional Evaluation ◽  
Cavernous Malformations ◽  
Clinical Appearance ◽  
Perilesional Edema ◽  
Hemorrhage Risk ◽  
The Mean

Object The aim of this study was to investigate the clinical appearance of untreated pediatric brainstem cavernous malformations (CMs) and to identify the hemorrhage risks and functional outcomes. Methods All pediatric patients with a diagnosis of brainstem CM between 1985 and 2012 were registered. The clinical chart and radiographs were recorded, and follow-up evaluations were obtained prospectively. Results A total of 85 patients (69.4% male) were included with a mean age of 12.7 years. Sixty-seven patients (78.8%) had prior hemorrhage, and 6 patients (7.1%) were asymptomatic. There were 15 midbrain lesions, 53 pons lesions, and 17 medulla lesions. The mean lesion size was 1.9 cm. During a total of 401.6 patient-years of follow-up, 47 hemorrhages occurred in 37 patients, and the annual hemorrhage rate was 11.7% per patient-year. The mean hemorrhage interval was 47.8 months. The hemorrhage risk declined over time, especially after the first 2 years. Both a lesion size ≥ 2 cm (hazard ratio [HR] 2.122, p = 0.037) and the presence of perilesional edema (HR 2.192, p = 0.039) predicted future hemorrhage and were associated with a high annual hemorrhage rate. The hemorrhage-free survival at 6 months was 85.7%, and at 1, 5, 10, and 15 years was 71.5%, 49.4%, 27.5%, and 13.7%, respectively. At the most recent functional evaluation, 33 patients (38.8%) had improved, 32 (37.6%) had stabilized, and 20 (23.5%) had worsened, without any deaths. Twenty-two patients (25.9%) obtained a full recovery. Prospective hemorrhage (HR 0.191, p = 0.003) was the adverse predictor for full recovery. Full recovery primarily occurred within the first 12 months, after which the chance of full recovery decreased. The cumulative percentage of complete recovery at 6 months was 32.7%, and at 1, 3, and 5 years was 40.8%, 43.6%, and 49.2%, respectively. Conclusions In this study the hemorrhage rate was relatively high in pediatric brainstem CMs, although the functional outcome was acceptable. The decline in hemorrhage risk and the identified adverse predictors in this study were helpful for clinicians and patients when deciding on treatment. Referral bias and the insufficient follow-up period of the study were highlighted as limitations.

Aggressive course of multiple de novo cavernous malformations

2011 ◽  
Vol 115 (6) ◽  
pp. 1175-1178 ◽  
Author(s):  
Kelly D. Flemming ◽  
George K. Bovis ◽  
Fredric B. Meyer
Keyword(s):  
De Novo ◽  
Clinical Symptoms ◽  
Cavernous Malformation ◽  
Correct Diagnosis ◽  
Cavernous Malformations ◽  
History Of ◽  
Hemorrhage Risk ◽  
Hemorrhagic Lesion ◽  
One Year ◽  
Risk Of Hemorrhage

The risk of hemorrhage from an intracerebral cavernous malformation has been estimated at 2%–4% per year. In patients with multiple cavernous malformations, typically there are 1 or 2 dominant lesions that result in symptoms. This report highlights an unusual case of recurrent hemorrhage from de novo cavernous malformations. This 35-year-old man had a generalized seizure in 2007. Magnetic resonance imaging performed at the time showed multiple hemorrhagic lesions suggestive of cavernous malformations. Two years later, the patient had clinical symptoms referable to a midbrain hemorrhage. This lesion was not present on 2007 standard and gradient echo images. One year later, the patient had another clinical hemorrhage at the cervical medullary junction. This lesion was also not present on earlier imaging. Genetic testing was negative for the known familial types of cavernous malformation. A lesion was biopsied to ensure correct diagnosis, and the results were pathologically consistent with a cavernous malformation. The patient had a fourth clinical hemorrhage in 2011 from a separate lesion. All hemorrhage symptoms were mild, and he returned to normal functioning and work after each hemorrhage. This case highlights several unusual features of the known natural history of intracerebral cavernous malformations. In this case, resection of the hemorrhagic lesion would not have altered future hemorrhage risk since each new hemorrhage was from a de novo lesion.

scholarly journals Hemorrhage from cerebral cavernous malformations: a systematic pooled analysis

2017 ◽  
Vol 126 (4) ◽  
pp. 1079-1087 ◽  
Author(s):  
Bradley A. Gross ◽  
Rose Du
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Natural History ◽  
Cavernous Malformation ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Pooled Analysis ◽  
Cavernous Malformations ◽  
Natural History Studies ◽  
Hemorrhage Risk

OBJECTIVE The aim of this paper is to define an overall cavernous malformation (CM) hemorrhage rate and risk factors for hemorrhage. METHODS The authors performed a systematic, pooled analysis via the PubMed database through October 2015 using the terms “cavernoma,” “cavernous malformation,” “natural history,” “bleeding,” and “hemorrhage.” English-language studies providing annual rates and/or risk factors for CM hemorrhage were included. Data extraction, performed independently by the authors, included demographic data, hemorrhage rates, and hemorrhage risk factors. RESULTS Across 12 natural history studies with 1610 patients, the mean age at presentation was 42.7 years old and 52% of patients (95% CI 49%–55%) were female. Presentation modality was seizure in 30% (95% CI 25%–35%), hemorrhage in 26% (95% CI 17%–37%), incidental in 17% (95% CI 9%–31%), and focal deficits only in 16% of cases (95% CI 11%–23%). CM location was lobar in 66% (95% CI 61%–70%), brainstem in 18% (95% CI 13%–24%), deep supratentorial in 8% (95% CI 6%–10%), and cerebellar in 8% (95% CI 5%–11%). Pooling 7 studies that did not assume CM presence since birth, the annual hemorrhage rate was 2.5% per patient-year over 5081.2 patient-years of follow-up (95% CI 1.3%–5.1%). Pooling hazard ratios across 5 studies that evaluated hemorrhage risk factors, prior CM hemorrhage was a significant risk factor for hemorrhage (HR 3.73, 95% CI 1.26–11.1; p = 0.02) while younger age, female sex, deep location, size, multiplicity, and associated developmental venous anomalies (DVAs) were not. CONCLUSIONS Although limited by the heterogeneity of incorporated reports and selection bias, this study found prior hemorrhage to be a significant risk factor for CM bleeding, while age, sex, CM location, size, multiplicity, and associated DVAs were not. Future natural history studies should compound annual hemorrhage rate with prospective seizure and nonhemorrhagic neurological deficit rates.

Small Cavernous Malformation Presenting with Medically Refractory Epilepsy

2019 ◽  
pp. 259-266
Author(s):  
John R. Williams ◽  
Gabrielle A. White-Dzuro ◽  
Michael R. Levitt ◽  
Andrew L. Ko
Keyword(s):  
Surgical Resection ◽  
Seizure Frequency ◽  
Medical Management ◽  
Seizure Control ◽  
Refractory Epilepsy ◽  
Cavernous Malformation ◽  
Cerebral Cavernous Malformations ◽  
Cavernous Malformations ◽  
Hemorrhage Risk ◽  
Medically Refractory Epilepsy

Abstract: Cerebral cavernous malformations are a rare but important cause of medically refractory epilepsy. Although medical management is important in seizure control, surgical resection remains the most effective method of both reducing seizure frequency and eliminating hemorrhage risk. The prognosis for seizure control with resection is excellent. Adjacent hemosiderin-stained brain is resected unless the cortical location is highly eloquent. A key part of the management is the decision as to when to offer surgery. Radiosurgery is generally felt to be an ineffective treatment for cavernous malformations. This chapter discusses the diagnosis, presurgical workup, and operative nuances that should be considered when treating this disease.

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