An analysis of the natural history of cavernous malformations

Kim, Dong-Seok; Park, Yong-Gou; Choi, Jina; Chung, Seungsoo; Lee, Kyu-Chang

doi:10.1016/s0090-3019(96)00425-9

Cited by 253 publications

(166 citation statements)

References 29 publications

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“…Many studies have identified the adverse effects of female sex, 1,19,22 prior hemorrhage, 1,3,9,14,15,20 location (nonlobar, 6 infratentorial, 18 or deep), 20 and age (< 40 years) 1 ; the risk factors of female sex and prior hemorrhage were consistent with our results. However, a study of the natural history of brainstem CMs by Kupersmith et al 16 failed to confirm these rehemorrhage risk factors.…”

Section: Hemorrhage Risksupporting

confidence: 90%

Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations

Li¹,

Hao²,

Jia³

et al. 2014

JNS

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Object Cerebral cavernous malformations have been studied widely, but the natural history of brainstem cavernous malformations (CMs) is not well defined, and hemorrhages caused by brainstem CMs are devastating. The goal of this study was to quantify the hemorrhage risks and functional outcomes of patients with brainstem CMs. Methods This prospective, longitudinal, cohort study included patients with brainstem CMs diagnosed between 1985 and 2012. The clinical courses of all patients were recorded. Predictors of hemorrhage and the overall untreated outcomes were evaluated. Results A total of 331 patients (46.5% female) were included, with a mean follow-up duration of 6.5 years. The annual hemorrhage rates in patients initially presenting with hemorrhage with (n = 215) or without (n = 34) focal neurological deficits were 15.9% and 12.4%, respectively. However, the annual hemorrhage rate was 8.7% in patients initially presenting without hemorrhage (n = 82). The risk factors for hemorrhage were female sex (hazard ratio [HR] 1.445, p = 0.041), prior hemorrhage (HR 1.277, p = 0.029), and perilesional edema (HR 1.830, p = 0.002). Overall, neurological function at the most recent assessment was improved compared with neurological function at diagnosis. Additionally, 307 patients (92.7%) improved or stabilized, 268 (81.0%) lived independently, and 95 (28.7%) completely recovered. Predictors favoring complete recovery were no prospective hemorrhage (HR 1.958, p = 0.001), younger age (HR 1.268, p = 0.001), and small lesion size (HR 1.578, p = 0.004). Conclusions Patients' initial presentation predicts their prospective annual hemorrhage rate. This study suggests that several strong risk factors for hemorrhage and predictors of brainstem CM outcomes may enable clinicians to evaluate the potential hemorrhage risks of their patients and design personalized treatments.

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Section: Hemorrhage Risksupporting

confidence: 90%

Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations

Li¹,

Hao²,

Jia³

et al. 2014

JNS

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“…4,21 Furthermore, the overall annual hemorrhage rate was usually influenced by strict 40,45 or lax 3,29,41 hemorrhage definitions, DVA, referral pattern, and familial lesions. 4,15,28,32,40,50 Through comparing the included studies concerning the natural history of cerebral CMs (Table 9) 3,4,6,13,15,21,23,28-32,40,41,45,50 and based on the aforementioned risk factors, it was interesting to find that AlShahi et al, 6 Labauge et al, 31 and Robinson et al, 45 who reported the lowest rates (0.5%, 0.6%, and 0.7% per lesionyear, respectively), had the longest followup duration (8.8 years), the highest percentage of asymptomatic patients (100%), and the lowest percentage of patients presenting with hemorrhage (9.1%). It was also noteworthy that Moriarity et al, 40 with the highest hemorrhage rate per patientyear (3.1%) of the studies with a nonfamilial pattern of CMs, had the highest percentage of female patients (64.7%) and extremely few asymptomatic patients (1.5%).…”

Section: Hemorrhage Riskmentioning

confidence: 99%

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

Li¹,

Hao²,

Tang³

et al. 2014

PED

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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.

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“…13,70 Among the general population, the reported prevalence of CNS cavernous malformations is 0.4%-0.6%. 16,25,31,48,52,56 These lesions most often occupy the intracranial (mainly supratentorial) compartment. 10,16,25,33,52,66 By contrast, spinal cord cavernous malformations are very rare entities, accounting for 5%-12% of intraspinal vascular malformations.…”

mentioning

confidence: 99%

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

Badhiwala¹,

Farrokhyar

Alhazzani

et al. 2014

SPI

117

118

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Object Information pertaining to the natural history of intramedullary spinal cord cavernous malformations (ISCCMs) and patient outcomes after surgery is scarce. To evaluate factors associated with favorable outcomes for patients with surgically and conservatively managed ISCCMs, the authors performed a systematic review and metaanalysis of the literature. In addition, they included their single-center series of ISCCMs. Methods The authors searched MEDLINE, EMBASE, CINAHL, Google Scholar, and The Cochrane Library for studies published through June 2013 that reported cases of ISCCMs. Data from all eligible studies were used to examine the epidemiology, clinical features, and neurological outcomes of patients with surgically managed and conservatively treated ISCCMs. To evaluate several variables as predictors of favorable neurological outcomes, the authors conducted a meta-analysis of individual patient data and performed univariate and multivariate logistic regression analyses. Variables included patient age, patient sex, lesion spinal level, lesion size, cerebral cavernomas, family history of cavernous malformations, clinical course, presenting symptoms, treatment strategy (operative or conservative), symptom duration, surgical approach, spinal location, and extent of resection. In addition, they performed a meta-analysis to determine a pooled estimate of the annual hemorrhage rate of ISCCMs. Results Eligibility criteria were met by 40 studies, totaling 632 patients, including the authors' institutional series of 24 patients. Mean patient age was 39.1 years (range 2–80 years), and the male-to-female ratio was 1.1:1. Spinal levels of cavernomas were cervical (38%), cervicothoracic (2.4%), thoracic (55.2%), thoracolumbar (0.6%), lumbar (2.1%), and conus medullaris (1.7%). Average cavernoma size was 9.2 mm. Associated cerebral cavernomas occurred in 16.5% of patients, and a family history of cavernous malformation was found for 11.9% of evaluated patients. Clinical course was acute with stepwise progression for 45.4% of patients and slowly progressive for 54.6%. Symptoms were motor (60.5%), sensory (57.8%), pain (33.8%), bladder and/or bowel (23.6%), respiratory distress (0.5%), or absent (asymptomatic; 0.9%). The calculated pooled annual rate of hemorrhage was 2.1% (95% CI 1.3%–3.3%). Most (89.9%) patients underwent resection, and 10.1% underwent conservative management (observation). Outcomes were better for those who underwent resection than for those who underwent conservative management (OR 2.79, 95% CI 1.46–5.33, p = 0.002). A positive correlation with improved neurological outcomes was found for resection within 3 months of symptom onset (OR 2.11, 95% CI 1.31–3.41, p = 0.002), hemilaminectomy approach (OR 3.20, 95% CI 1.16–8.86, p = 0.03), and gross-total resection (OR 3.61, 95% CI 1.24–10.52, p = 0.02). Better outcomes were predicted by an acute clinical course (OR 1.72, 95% CI 1.10–2.68, p = 0.02) and motor symptoms (OR 1.76, 95% CI 1.08–2.86, p = 0.02); poor neurological recovery was predicted by sensory symptoms (OR 0.58, 95% CI 0.35–0.98, p = 0.04). Rates of neurological improvement after resection were no higher for patients with superficial ISCCMs than for those with deep-seated ISCCMs (OR 1.36, 95% CI 0.71–2.60, p = 0.36). Conclusions Intramedullary spinal cord cavernous malformations tend to be clinically progressive. The authors' findings support an operative management plan for patients with a symptomatic ISCCM. Surgical goals include gross-total resection through a more minimally invasive hemilaminectomy approach within 3 months of presentation.

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An analysis of the natural history of cavernous malformations

Cited by 253 publications

References 29 publications

Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations

Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations

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

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

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