Cavernous Malformations of the Brainstem Presenting in Childhood: Surgical Experience in 40 Patients

Abla, Adib A.; Leković, Gregory P.; Garrett, Mark P.; Wilson, David A.; Nakaji, Peter; Bristol, Ruth E.; Spetzler, Robert F.

doi:10.1227/neu.0b013e3181f8d1b2

Cited by 77 publications

(83 citation statements)

References 18 publications

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“…Furthermore, studies differed in their hemorrhage definitions, the assumption of a congenital lesion, and neurological function evaluation methods. 1,2,4,14,24,30,35 In our study, we performed a prospective analysis of retrospectively collected data on the cohort of pediatric brainstem CMs with a stringent definition of hemorrhage and found that the annual hemorrhage rate was calculated to be 11.7% per patientyear, that both the presence of perilesional edema and a larger lesion size predicted poorer hemorrhagefree survival and a higher annual hemorrhage rate, and that prospective hemorrhage prevented complete neurological recovery (Fig. 2).…”

Section: Discussionmentioning

confidence: 89%

“…1,2,36,48 Most previous studies included both adult and pediatric patients, were limited by their retrospective nature, or encountered inherent referral biases in surgical series that compromised the comparability between studies and obscured the natural history of pediatric brainstem CMs. Furthermore, studies differed in their hemorrhage definitions, the assumption of a congenital lesion, and neurological function evaluation methods.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

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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“…28,49,68 Preoperative hemorrhage risks are still the subject of controversy and have been insufficiently studied. Several factors have been correlated with hemorrhage, including sex (female), 6,9,51,63,69 age, 3,6,12,43 lesion location (deep-seated lesions), 17,41,44,56 large lesion size, 43 a history of previous ictus, 6,7,25,28,[40][41][42][43]56,57,66 and associated DVA. 1 A prospective, population-based cohort study demonstrated that female sex was a risk factor for the recurrence for cerebral CMs, but the annual rates of recurrence were not significantly different for brainstem lesions and lesions in other locations.…”

Section: Natural Historymentioning

confidence: 99%

“…3,4,31,32,54 Therefore, we retrospectively reviewed a single-center series of 242 cases of brainstem CM to determine the pre-and postoperative hemorrhage risk factors, clinical characteristics based on the location of the lesions, and long-term functional outcomes.…”

mentioning

confidence: 99%

Hemorrhage risk, surgical management, and functional outcome of brainstem cavernous malformations

Yang

Hao

et al. 2013

JNS

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Object The aim of this study was to evaluate the pre- and postoperative rehemorrhage risk, neurological function outcome, and prognostic factors of surgically treated brainstem cavernous malformations (CMs) with long-term follow-up. Methods The authors conducted a retrospective review of the clinical data from 242 patients with brainstem CMs that were surgically treated between 1999 and 2010. Patient charts, imaging findings, and outcomes were examined. Results The study included 242 patients, with a male-to-female ratio of 1.3 and mean age of 32.6 years. The mean modified Rankin Scale scores on admission, at discharge, at 3 and 6 months after surgery, and at recent evaluation were 2.2, 2.6, 2.3, 1.8, and 1.5, respectively. The preoperative calculated annual hemorrhage and rehemorrhage rates were 5.0% and 60.9%, respectively. The complete resection rate was 95%. Surgical morbidity occurred in 112 patients (46.3%). Eighty-five patients (35.1%) demonstrated worsened condition immediately after surgery; 34 (41.0%) and 51 (61.4%) of these patients recovered to their baseline level within 3 and 6 months after surgery, respectively. At a mean follow-up of 89.4 months, the patients' condition had improved in 147 cases (60.7%), was unchanged in 70 cases (28.9%), and had worsened in 25 cases (10.3%). A total of 8 hemorrhages occurred in 6 patients, and the postoperative annual hemorrhage rate was 0.4%. Permanent morbidity remained in 65 patients (26.9%). The adverse factors for preoperative rehemorrhage were age ≥ 50 years, size ≥ 2 cm, and perilesional edema. The risk factors for postoperative hemorrhage were developmental venous anomaly and incomplete resection. The independent adverse factors for long-term outcome were increased age, multiple hemorrhages, ventral-seated lesions, and poor preoperative status. Favorable, complete improvement in the postoperative deficits over time was correlated with good preoperative neurological function and continuing improvement thereafter. Conclusions Favorable long-term outcomes and significantly low postoperative annual hemorrhage rates were achieved via surgery. Total resection should be attempted with an aim of minimal injury to neurological function; however, postoperative deficits can improve during the postoperative course. Close follow-up with radiological examination is proposed for patients with adverse factors predictive of rehemorrhage.

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“…Surgical excision of cavernous malformations relieves mass effect on surrounding brain and may be effective in controlling seizures, especially if surrounding hemosiderin-stained tissue is removed. Unfortunately, removal of lesions in the brain stem does not necessarily eliminate the risk of hemorrhage: there is a growing awareness of a moderately high rate of recurrence in this region and the spinal cord [233]. Whether recurrence is related to further growth of residual malformation or development of a new lesion remains to be determined.…”

Section: Prognosismentioning

confidence: 99%

Central Nervous System Vascular Malformations

Davidson¹,

Stoodley²

2012

Congenital Anomalies - Case Studies and Mechanisms

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Cavernous Malformations of the Brainstem Presenting in Childhood: Surgical Experience in 40 Patients

Cited by 77 publications

References 18 publications

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

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

Hemorrhage risk, surgical management, and functional outcome of brainstem cavernous malformations

Central Nervous System Vascular Malformations

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