Development of moyamoya arteriopathy following treatment of intracranial tumors: clinical and radiographic characterization.

OBJECTIVE: Moyamoya arteriopathy can develop in patients with brain tumors, particularly when associated with neurofibromatosis type 1 (NF1) or cranial irradiation. The present study aimed to analyze the clinical outcomes of moyamoya after brain tumor treatment and elucidate the effect of revascularization on brain tumors. METHODS: The authors retrospectively reviewed clinical and radiographic findings in 27 patients with brain tumors who developed moyamoya requiring revascularization surgery between January 1985 and June 2017 at a single institution. The long-term clinical and neuroimaging-based outcomes were analyzed. RESULTS: Among 27 patients, 22 patients underwent radiotherapy, and 12 patients had NF1. The mean ages at diagnosis of brain tumors and moyamoya were 4.4 years and 10.3 years, respectively. The mean interval between radiotherapy and moyamoya diagnosis was 4.0 years. The mean follow-up period after revascularization surgery was 8.5 years. Among 46 affected hemispheres in 27 patients, the patients who underwent radiotherapy (30 hemispheres in 22 patients) had a higher incidence of Suzuki stage 5 or 6 (20% [6/30] vs 0% [0/8]) and infarction (63.6% [14/22] vs 0% [0/5]) compared with patients without radiotherapy (8 hemispheres in 5 patients). After revascularization, stroke occurred in 4 patients, and 6 hemispheres showed Matsushima grade C, all of which occurred in patients with a history of radiotherapy. The residual brain tumors progressed in 4 of 21 patients (19%) after revascularization, comparable to the progression rates of brain tumors without revascularization in previous literature. CONCLUSIONS: Patients with brain tumors can develop moyamoya that exhibits characteristic clinical and radiographic features of idiopathic MMD. Moyamoya associated with cranial irradiation has a higher incidence of stroke with less capacity for revascularization, requiring thorough evaluations and timely treatment. Revascularization does not appear to have any effect on the progression of existing brain tumors.

A national analysis of 9655 pediatric cerebrovascular malformations: effect of hospital volume on outcomes.

OBJECTIVE: Comprehensive multicenter data on the surgical treatment of pediatric cerebrovascular malformations (CVMs) in the US are lacking. The goal of this study was to identify national trends in patient demographics and assess the effect of hospital case volume on outcomes. METHODS: Admissions for CVMs (1997-2012) were identified from the nationwide Kids' Inpatient Database. Admissions with and without craniotomy were reviewed separately. Patients were categorized by whether they were treated at low-, medium-, or high-volume centers (< 10, 10-40, > 40 cases/year, respectively). A generalized linear model was used to evaluate the association of hospital pediatric CVM case volume and clinical variables assessing outcomes. RESULTS: Among the 9655 patients, 1828 underwent craniotomy and 7827 did not. Patient age and race differed in the two groups, as did the rate of private medical payers. High-volume hospitals had fewer nonroutine discharges (11.2% [high] vs 16.4% [medium] vs 22.3% [low], p = 0.0001). For admissions requiring craniotomy, total charges ($106,282 [high] vs $126,215 [medium] vs $134,978 [low], p < 0.001) and complication rates (0.09% [high] vs 0.11% [medium] vs 0.16% [low], p = 0.001) were lower in high-volume centers. CONCLUSIONS: This study revealed that further investigation may be needed regarding barriers to surgical treatment of pediatric CVMs. The authors found that surgical treatment of pediatric CVM at high-volume centers is associated with significantly fewer complications, better dispositions, and lower costs, but for noncraniotomy patients, low-volume centers had lower rates of complications and death and lower costs. These findings may support the consideration of appropriate referral of CVM patients requiring surgery or with intracranial hemorrhage toward high-volume, specialized centers.

A study of pediatric cerebral arteriovenous malformations: clinical presentation, radiological features, and long-term functional and educational outcomes with predictors of sustained neurological deficits.

OBJECTIVE: Large experiences with the treatment of pediatric arteriovenous malformations (AVMs) remain relatively rare, with limited data on presentation, treatment, and long-term functional outcomes. Because of the expected long lifespan of children, caregivers are especially interested in outcome measures that assess quality of life. The authors' intention was to describe the long-term functional outcomes of pediatric patients who undergo AVM surgery and to identify predictors of sustained neurological deficits. METHODS: The authors analyzed a 21-year retrospective cohort of pediatric patients with intracranial AVMs treated with microsurgery at two institutions. The primary outcome was a persistent neurological deficit at last follow-up. Secondary outcome measures included modified Rankin Scale (mRS) score and independent living. RESULTS: Overall, 97 patients (mean age 11.1 ± 4.5 years; 56% female) were treated surgically for intracranial AVMs (mean follow-up 77.5 months). Sixty-four patients (66%) presented with hemorrhage, and 45 patients (46%) had neurological deficits at presentation. Radiologically, 39% of lesions were Spetzler-Martin grade II. Thirty-seven patients (38%) with persistent neurological deficits at last follow-up were compared with those without deficits; there were no differences in patient age, presenting Glasgow Coma Scale score, AVM size, surgical blood loss, or duration of follow-up. Multivariate analysis demonstrated that a focal neurological deficit on presentation, AVM size > 3 cm, and lesions in eloquent cortex were independent predictors of persistent neurological deficits at long-term follow-up. Overall, 92% of the children had an mRS score ≤ 2 on long-term follow-up. CONCLUSIONS: Pediatric patients with AVMs treated with microsurgical resection have good functional and radiological outcomes. There is a high rate (38%) of persistent neurological deficits, which were independently predicted by preoperative deficits, AVMs > 3 cm, and lesions located in eloquent cortex. This information can be useful in counseling families on the likelihood of long-term neurological deficits after cerebral AVM surgery.

Multimodal treatment approach in a patient with multiple intracranial myxomatous aneurysms.

The most common primary cardiac tumor is myxoma, typically originating in the left atrium. Emboli to the central nervous system can cause cerebral infarction or, rarely, seed tumor growth within vessel walls, causing myxomatous aneurysms. Fewer than 60 myxomatous aneurysms have been reported, including 2 cases in children. Here, the authors describe 2 different growing myxomatous aneurysms in a child successfully managed using a combined multidisciplinary approach. A 12-year-old boy developed a sudden headache, diplopia, gait instability, and speech difficulty. Magnetic resonance imaging revealed a left parietal hemorrhage and multifocal cerebral infarction, suspicious for an embolic etiology. A cardiac myxoma was identified in the left atrium and resected. Follow-up cranial vasculature imaging demonstrated multiple intracranial myxomatous aneurysms. These lesions were followed up, and serial imaging identified marked growth of 2 of them (right occipital and left parietal), prompting invasive intervention. The deep occipital lesion was better suited to endovascular treatment, while the superficial parietal lesion was amenable to resection. The patient underwent embolization of an enlarging fusiform aneurysm of the distal right posterior cerebral artery, followed by a left parietal craniotomy for a lesion of the distal left middle cerebral artery. Both procedures were performed without complications and achieved successful obliteration of the lesions, as confirmed by catheter angiography at the 30-month follow-up. To the authors' knowledge, this report illustrates the first combined endovascular and open surgical treatment of 2 myxomatous aneurysms in a single patient. While acknowledging the rarity of this condition, this report illustrates the clinical manifestations and treatment challenges posed by myxoma and details a successful strategy that could be employed in similar scenarios.