Aneurysmal bone cysts (ABCs) are an uncommon entity predominantly encountered in the pediatric population. The skull is rarely involved, but these cysts have been reported to arise in the skull base. Traditional treatment has been with surgery alone; however, there is a gathering body of literature that reports alternative treatments that can achieve long-term disease-free survival. However, these therapies are predominantly directed at peripheral skeletal lesions. To the authors’ knowledge, this report is the first to describe long-term follow-up of the efficacy of Gamma Knife stereotactic radiosurgery for treatment of ABC residuum in the skull base that resulted in long-term patient stability and likely ABC obliteration.
George H. Tse, Feng Y. Jiang, Matthias W. R. Radatz, Saurabh Sinha and Hesham Zaki
Lee Walton, Anna Hampshire, Paul Vaughan, David M. C. Forster, Andras A. Kemeny and Matthias W. R. Radatz
✓ The purpose of this paper was to note a potential source of error in magnetic resonance (MR) imaging. Magnetic resonance images were acquired for stereotactic planning for GKS of a vestibular schwannoma in a female patient. The images were acquired using three-dimensional sequence, which has been shown to produce minimal distortion effects.
The images were transferred to the planning workstation, but the coronal images were rejected.
By examination of the raw data and reconstruction of sagittal images through the localizer side plate, it was clearly seen that the image of the square localizer system was grossly distorted.
The patient was returned to the MR imager for further studies and a metal clasp on her brassiere was identified as the cause of the distortion.
M. Yashar S. Kalani, Michael T. Lawton and Robert F. Spetzler
Eduard B. Dinca, Patricia de Lacy, John Yianni, Jeremy Rowe, Matthias W. R. Radatz, Daniel Preotiuc-Pietro and Andras A. Kemeny
The authors present their 25-year experience in treating pediatric arteriovenous malformations (AVMs) to allow comparisons with other historic studies and data in adults.
Data were collected from a prospectively maintained departmental database selected for age and supplemented by case note review and telephone interviews as appropriate.
Three hundred sixty-three patients, ages 1–16 years (mean ± SD, 12 ± 3.2 years), underwent 410 treatments; 4 had planned 2-stage treatments and 43 were retreated subsequent to an initial partial response. Fifty-eight percent received general anesthesia for the procedure. Sixteen percent had previously undergone embolization. The most common presenting symptoms were as follows: hemorrhage (80.2%), epilepsy (8.3%; overall seizure prevalence 19.9%), and migrainous headaches (6.3%). Only 0.28% of the AVMs were incidental findings. The mean lesion volume was 3.75 ± 5.3 cm3 (range 0.01–32.8 cm3), with a median Spetzler-Martin grade of III (range I–V). The mean peripheral (therapeutic) dose was 22.7 ± 2.3 Gy (range 15–25 Gy), corresponding to a mean maximum dose of 43.6 ± 6 Gy (range 25–51.4 Gy).
The obliteration rate was 71.3% in patients who received one treatment and 62.5% for retreated patients, with a mean obliteration time of 32.4 and 79.6 months, respectively. The overall obliteration rate was 82.7%. No follow-up data are as yet available for the 4 patients who underwent the staged treatments. Only 4 patients received peripheral doses below 20 Gy, and the AVM was obliterated in 3 of these patients. The other patients received 20, 22.5, or 25 Gy and had obliteration rates of 82.6%, 77.7%, and 86.3%, respectively. The bleeding rate postradiosurgery was 2.2%, and the cumulative complication rate was 3.6%, with radionecrosis being the most common complication (1.1%).
Surprisingly, there was no correlation (p = 0.43) between outcome and radiosurgical dose when that dose was between 20 and 25 Gy, thus suggesting that the lower of these 2 doses may be effective. Radiosurgery for pediatric AVM is safe and effective.
Lee Walton, Anna Hampshire, Anthony Roper, Patrick Mitchell, Paul Vaughan, David M. C. Forster, Andras A. Kemeny and Matthias W. R. Radatz
Object. One of the limiting factors in gamma knife radiosurgery is the restriction to one treatment imposed by the fixed stereotactic frame. The ability, in selected cases, to remove the frame and replace it on a subsequent occasion in the same location would facilitate fractionated treatments and provide flexibility in the timing of treatment delivery. It is the purpose of this work to investigate techniques for frame fixation and for essential verification of frame position once it has been reapplied.
Methods. A technique is proposed that requires four surgical self-tapping screws to be inserted into the skull. Aluminum pins are inserted through the frame pillars and are tightened against the head of the screws, providing a firm fixation of the frame. Pin lengths are recorded on gauges to ensure reproducibility of the position. In phantom tests, test objects were localized (using the angiographic localizer) before and after each of five frame removal/reapplications to test the reproducibility of frame position. The mean error in the observed target coordinates was 0.3 mm and the maximum error observed was 0.7 mm, indicating that the frame can be reapplied with some confidence.
Repetition of bubble skull measurements has been investigated as a means of verifying that the frame was repositioned correctly; however, reproducibility of patient measurements was found to be poor even when no frame movement had occurred. In contrast, the use of a radiotherapy simulator to obtain repeated lateral and anteroposterior projections of the head was shown to be capable of detecting frame movements of as little as 1 mm.
Conclusions. Using this technique of frame application facilitates the reapplication of the frame with an accuracy of plus or minus 0.7 mm. Bubble measurements are inadequate for the detection of frame movement. Simple techniques in which a radiotherapy simulator is used can verify correct frame placement and indicate frame movements of less than 1 mm.
Radiosurgery and cavernous malformations
Jason Sheehan and David Schlesinger
Gábor Nagy, Adam Razak, Jeremy G. Rowe, Timothy J. Hodgson, Stuart C. Coley, Matthias W. R. Radatz, Umang J. Patel and Andras A. Kemeny
The role of radiosurgery in the treatment of cavernous malformations (CMs) remains controversial. It is frequently recommended only for inoperable lesions that have bled at least twice. Rehemorrhage can carry a substantial risk of morbidity, however. The authors reviewed their practice of treating deep-seated inoperable CMs to assess the complication rate of radiosurgery, the impact that radiosurgery might have on rebleeding, and whether a more active, earlier intervention is justified in managing this condition.
The authors performed a retrospective analysis of 113 patients with 79 brainstem and 39 thalamic/basal ganglia CMs treated with Gamma Knife surgery. Lesions were stratified into 2 groups: those that might be lower risk with no more than 1 symptomatic bleed before radiosurgical treatment and those deemed high risk with multiple symptomatic hemorrhages before treatment.
Forty-one CMs had multiple symptomatic hemorrhages before radiosurgery with a first-ever bleed rate of 2.9% per lesion per year, a rebleed rate of 30.5% per lesion per year, and a median time of 1.5 years between the first and second bleeds. In this group the rebleed rate decreased to 15% for the first 2 years after radiosurgery and declined further to 2.4% thereafter. Pretreatment multiple bleeds led to persistent deficits in 72% of the patients.
Seventy-seven CMs had no more than 1 symptomatic bleed before radiosurgery, making for a lifetime bleed rate of 2.2% per lesion per year. The short period between the presenting bleed and treatment (median 1 year) makes the natural history in this group uncertain. The rate of hemorrhage in the first 2 years after treatment was 5.1%, and 1.3% thereafter. Pretreatment hemorrhages resulted in permanent deficits in 43% of the patients in this group, a rate significantly lower than in the multiple-bleeds group (p < 0.001).
Posttreatment hemorrhages led to persistent deficits in only 7.3% of the patients. Permanent adverse radiation effects were rare (7.3%) and minor in both groups.
Stereotactic radiosurgery is a safe management strategy for CMs in eloquent sites with the marked advantage of reducing rebleed risks in patients with repeated pretreatment hemorrhages. The benefit in treating CMs with a single bleed is less clear. Note, however, that repeated hemorrhage carries a significant risk of increased morbidity far in excess of any radiosurgery-related morbidity, and the authors assert that this finding justifies the early active management of deep-seated CMs.
Gábor Nagy, Wendy Burkitt, Stuart S. Stokes, Debapriya Bhattacharyya, John Yianni, Jeremy G. Rowe, Andras A. Kemeny and Matthias W. R. Radatz
Long-term benefits of radiosurgery (RS) applying modern protocols to treat cavernous malformations (CMs) remain unclear as critics may consider the decrease in the rebleed rate generally observed 2 years after RS as a reflection of the lesion’s natural history. The authors adopted an early intention-to-treat attitude since rehemorrhage from deep-seated CMs ultimately leads to stepwise neurological deterioration. The safety of this early policy was previously demonstrated. Here, the authors revisit their current practice in a larger population with a longer follow-up time to assess the long-term effects of RS in the context of current knowledge on the natural history of CMs.
The authors conducted a retrospective analysis of 210 patients with 210 hemorrhagic CMs located in the brainstem, thalamus, or basal ganglia and treated with Gamma Knife RS between 1995 and 2014. Two hundred six patients had available follow-up, which was a median of 5.5 years (range 1–20 years). The median age was 37 years (0.5–77 years) at presentation and 43 (2–78) at treatment. One hundred twenty-seven CMs had bled once and 83 had had multiple hemorrhages prior to treatment.
The lifetime annual bleed rate of CMs having a single hemorrhage prior to treatment was 2.4% per lesion. The hemorrhage rate stabilized at 1.1% after a temporary increase of 4.3% within the first 2 years after RS. The annual pretreatment hemorrhage rate was 2.8% for the lesions having multiple bleeds prior to RS with a pretreatment rebleed rate of 20.7% and with a modest gradual decrease within the first 5 years and remaining stable at 11.55% thereafter. The rebleed rate fell to 7.9% for the first 2 years after RS and declined further to 1.3% thereafter, which was significantly lower than the long-term pretreatment rebleed risk. The rate of hemorrhage-free survival remained 86.4% and 75.1% (1 patient each) at 20 years after RS in the single- and multiple-bleed groups, respectively.
Pretreatment hemorrhages resulted in permanent deficits in 48.8% of the cases with a single bleed and in 77.1% of the cases with multiple bleeds. Both the rate and severity of deficits were significantly lower in the first group. Only mild and a low rate of permanent neurological deficits were caused either by posttreatment hemorrhages (7.4%) or by radiation (7.2%). The rate of persistent morbidity in the single-bleed group remained significantly lower at the end of the study than pretreatment morbidity in the multiple-bleed group (OR 2.9, 95% CI 1.6–5.3). Lesion-specific mortality was < 1%.
The hemorrhage rate of CMs after RS remained low after the first 2 years during the longer follow-up period. The benefit of early treatment appears to be confirmed by the study results as repeated hemorrhages carry the risk of significantly higher cumulative morbidity than the morbidity associated with RS.
Gábor Nagy, Stuart S. Stokes, Loránd G. Erőss, Debapriya Bhattacharyya, John Yianni, Jeremy G. Rowe, Andras A. Kemeny and Matthias W. R. Radatz
The role of radiosurgery (RS) in treating superficial cavernous malformations (CMs) is insufficiently studied in part because of the disappointing results of early experimental attempts as compared to the mostly safe and effective microsurgery. Nonetheless, because of lesion- or treatment-specific factors, a therapeutic alternative may be required. In this study, the authors aimed to assess the safety of RS in treating superficial CMs and to analyze its long-term effect on hemorrhage rates and epilepsy control.
The authors conducted a retrospective analysis of 96 patients with 109 CMs located in the cerebral or cerebellar hemispheres and treated with RS between 1995 and 2014. A median of 15 Gy (range 10–25 Gy) was given to the 50% prescription isodose level, lesion volume was 604 mm3 (4–8300 mm3), and the prescription isodose volume was 638.5 mm3 (4–9500 mm3). Outcomes were compared to those of 206 deep-seated lesions reported on in another study. Ninety-five patients had available follow-up, which was a median of 7 years (1–21 years). Median patient age was 42 years (0.5–77) at presentation and 45 (3–80) at treatment. Seventy-one CMs presented with symptomatic hemorrhage, and 52 caused seizures.
In the nonhemorrhagic group (37 lesions), one bleed occurred during the follow-up period, for an annual bleed rate of 0.4% per lesion. The lifetime annual bleed rate of CMs having a single hemorrhage prior to treatment was 2.5%. The rebleed rate in the single-bleed group decreased from 1.8% within the first 2 years after RS to 0.7% thereafter. The pretreatment rebleed rate for lesions having multiple bleeds prior to RS was 14.15%, which fell to 3.85% for the first 2 years after RS and declined to 1.3% thereafter. Multivariate analysis showed younger age, deep lesion location, and multiple pretreatment hemorrhages as significant predictors of posttreatment hemorrhage.
Pretreatment hemorrhages led to permanent deficits in 41.4% of the cases with a single bleed and in 46.1% of cases with multiple bleeds. Only mild (modified Rankin Scale score 1) and a low rate of permanent neurological deficits were caused either by posttreatment hemorrhages (4.3%) or by radiation (2%).
The rate of improvement in epilepsy was 84.9% after RS in patients with at least one seizure prior to treatment, not depending on the presence of hemorrhage or the time interval between presentation and treatment. Favorable outcome occurred in 81% of patients whose seizures were not controlled with antiepileptic medication prior to RS.
Radiosurgery for superficial CMs is safe and appears to be effective, offering a real treatment alternative to surgery for selected patients. Given their relatively benign natural history, superficial CMs require further study to verify the long-term benefit of RS over the lesions’ natural history.