Paradoxical symptomatic cerebral blood flow decreases after combined revascularization surgery for patients with pediatric moyamoya disease: illustrative case

BACKGROUND Transient neurological deficits (TNDs) develop after cerebral revascularization in patients with moyamoya disease (MMD). The authors report a rare pediatric MMD case with extensive decreased cerebral blood flow (CBF) and prolonged TNDs after combined revascularization. OBSERVATIONS A 9-year-old boy presented with transient left upper limb weakness, and MMD was diagnosed. A right-sided combined surgery was performed. Two years after the surgery, frequent but transient facial (right-sided) and upper limb weakness appeared. The left internal carotid artery terminal stenosis had progressed. Therefore, a left combined revascularization was performed. The patient’s motor aphasia and right upper limb weakness persisted for approximately 10 days after surgery. Magnetic resonance angiography showed that the direct bypass was patent, but extensive decreases in left CBF were observed using single photon emission tomography. With adequate fluid therapy and blood pressure control, the neurological symptoms eventually disappeared, and CBF improved. LESSONS The environment of cerebral hemodynamics is heterogeneous after cerebral revascularization for MMD, and the exact mechanism of CBF decreases was not identified. TNDs are significantly associated with the onset of stroke during the early postoperative period. Therefore, appropriate treatment is desired after determining complex cerebral hemodynamics using CBF studies.

INDIRECT CEREBRAL REVASCULARIZATION ON OPTHALMIC ARTERY BY USING A DRUG-ELUTING BALLON FOR SUSPECTED MOYAMOYA DISEASE

Moyamoya disease (MMD) is a rare idiopathic progressive vaso-occlusive disease causing multiple occlusion of cerebral vessels lead to ischemic stroke. Asian population is the most common race to be affected. We present a male patient 33-years old with suspected MMD with right hemiparesis and neurocognitive changes. On digital substraction angiography (DSA) there was appearance of ‘puff of smoke’ on his right hemisphere, stenosis middle cerebral arteries M1 bilaterally, stenosis of right opthalmic artery (OA), stenosis of left anterior cerebral artery (ACA) and aplasia of right ACA. Ballon angioplasty was performed on right OA that supply the contralateral symptomatic stenosis area (left A1) indirectly through anterior ethmoidal artery and anterior falcine artery (OA-ACA collateral). While no guidelines for the management of MMD, cerebral revascularization by using drug-eluting ballon (DEB) in right opthalmica artery is potentially effective treatment that could allow the brain to have good blood supply (gives good collateral to both ACA), reduces burden of the fragile moya-moya vessels to be ruptured followed by improvement of clinical results. Patient’s selection by understanding the stage, its progressivity and collateral formation are crucial before decision is made.

Motor excitability in bilateral moyamoya vasculopathy and the impact of revascularization

OBJECTIVE Motor cortical dysfunction has been shown to be reversible in patients with unilateral atherosclerotic disease after cerebral revascularization. Moyamoya vasculopathy (MMV) is a rare bilateral stenoocclusive cerebrovascular disease. The aim of this study was to analyze the corticospinal excitability and the role of bypass surgery in restoring cortical motor function in patients by using navigated transcranial magnetic stimulation (nTMS). METHODS Patients with bilateral MMV who met the criteria for cerebral revascularization were prospectively included. Corticospinal excitability, cortical representation area, and intracortical inhibition and facilitation were assessed by nTMS for a small hand muscle (first dorsal interosseous) before and after revascularization. The clinically and/or hemodynamically more severely affected hemisphere was operated first as the leading hemisphere. Intra- and interhemispheric differences were analyzed before and after direct or combined revascularization. RESULTS A total of 30 patients with bilateral MMV were examined by nTMS prior to and after revascularization surgery. The corticospinal excitability was higher in the leading hemisphere compared with the non-leading hemisphere prior to revascularization. This hyperexcitability was normalized after revascularization as demonstrated in the resting motor threshold ratio of the hemispheres (preoperative median 0.97 [IQR 0.89–1.08], postoperative median 1.02 [IQR 0.94–1.22]; relative effect = 0.61, p = 0.03). In paired-pulse paradigms, a tendency for a weaker inhibition of the leading hemisphere was observed compared with the non-leading hemisphere. Importantly, the paired paradigm also demonstrated approximation of excitability patterns between the two hemispheres after surgery. CONCLUSIONS The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.

Cerebral Bypass for Aneurysms in the Era of Flow Diversion: Single-Surgeon Case Series

BACKGROUND With recent advances in endovascular treatments of brain aneurysms such as flow diverters, the role of cerebral revascularization needs to be re-evaluated. OBJECTIVE To evaluate the contemporary indications and outcomes of cerebral revascularization for brain aneurysms. METHODS A retrospective evaluation of a prospectively maintained database was performed to review clinical and imaging data of all the patients who underwent cerebral revascularization for brain aneurysms over the past 10 yr. RESULTS Among 174 cerebral revascularizations, 40 (in 36 patients) were done for the treatment of aneurysms. In total, 9 patients underwent combined endovascular treatment and surgical revascularization. Immediate aneurysm occlusion was achieved in 30 patients (83.3%). Immediate postoperative bypass patency was confirmed in 33 patients (92%). Postoperative neurological deficit was observed in 4 patients (11.1%). There were 2 mortalities in the postoperative period. Aneurysm total occlusion rate was 91% at 1 yr. Thirty patients had 1 yr clinical and radiological follow-up. Clinical evaluations showed modified Rankin Scale 2 or less in 25 patients at 1 yr. Bypass patency was confirmed in 27 (90%). Patients with fair/poor outcome were all in the subarachnoid hemorrhage group. Twenty-one patients had follow-up studies for 3 yr or beyond with no evidence of stroke or aneurysm recurrence. CONCLUSION Our results support that cerebral revascularization can be regarded as a viable and durable treatment option for these challenging aneurysms with acceptable morbidity. Cerebral bypass should be offered in selected cases where standard endovascular or surgical treatment is not efficacious or curative.

Case Report: Cerebral Revascularization in a Child With Mucopolysaccharidosis Type I

Mucopolysaccharidosis (MPS) type I is a rare lysosomal storage disorder caused by an accumulation of glycosaminoglycans (GAGs) resulting in multisystem disease. Neurological morbidity includes hydrocephalus, spinal cord compression, and cognitive decline. While many neurological symptoms have been described, stroke is not a widely-recognized manifestation of MPS I. Accordingly, patients with MPS I are not routinely evaluated for stroke, and there are no guidelines for managing stroke in patients with this disease. We report the case of a child diagnosed with MPS I who presented with overt stroke and repeated neurological symptoms with imaging findings for severe ventriculomegaly, infarction, and bilateral terminal carotid artery stenosis. Direct intracranial pressure evaluation proved negative for hydrocephalus. The patient was subsequently treated with cerebral revascularization and at a 3-year follow-up, the patient reported no further neurological events or new ischemia on cerebral imaging. Cerebral arteriopathy in patients with MPS I may be associated with GAG accumulation within the cerebrovascular system and may predispose patients to recurrent strokes. However, further studies are required to elucidate the etiology of cerebrovascular arteriopathy in the setting of MPS I. Although the natural history of steno-occlusive arteriopathy in patients with MPS I remains unclear, our findings suggest that cerebral revascularization is a safe treatment option that may mitigate the risk of future strokes and should be strongly considered within the overall management guidelines for patients with MPS I.

Case Report: Successful Cerebral Revascularization and Cardiac Transplant in a 16-Year-Old Male With Syndromic BRCC3-Related Moyamoya Angiopathy

Background:BRCC3/MTCP1 deletions are associated with a rare familial moyamoya angiopathy with extracranial manifestations.Case: We report the case of an adolescent male presenting with progressive and symptomatic moyamoya angiopathy and severe dilated cardiomyopathy caused by a hemizygous deletion of BRCC3/MTCP1. He was treated for renovascular hypertension by left kidney homograft and right nephrectomy in infancy and had other syndromic features, including cryptorchidism, growth hormone deficiency, and facial dysmorphism. Due to worsening of the neurological and cardiac condition, he was treated by a direct superficial temporal artery to middle cerebral artery bypass to enable successful cardiac transplant without cerebral damage.Conclusions:BRCC3-related moyamoya is a devastating disease with severe heart and brain complications. This case shows that aggressive management with cerebral revascularization to allow cardiac transplant is feasible and efficient despite end-stage heart failure.

Double-barrel STA-MCA bypass for cerebral revascularization: lessons learned from a 10-year experience

OBJECTIVE In select patients, extracranial-intracranial (EC-IC) bypass remains an important tool for cerebral revascularization. Traditionally, superficial temporal artery–middle cerebral artery (STA-MCA) bypass was performed using one limb of the STA only. In an attempt to augment flow and to direct flow to different ischemic areas of the brain, the authors adopted a “double-barrel” technique in which both branches of the STA are used to revascularize distinct MCA territories. METHODS A series of consecutive double-barrel STA-MCA bypasses performed between 2010 and 2020 were reviewed. Each anastomosis was directed to augment flow to a territory most at risk based on preoperative perfusion studies, cerebral angiography, and intraoperative indocyanine green data. CT perfusion and CTA were routinely used to evaluate postoperative augmentation and graft patency. Patient perioperative outcomes, surgical complications, and modified Rankin Scale (mRS) scores at the last follow-up were reported. RESULTS Forty-four patients (16 males, 28 females) successfully underwent double-barrel STA-MCA bypass on 54 cerebral hemispheres: 28 operations were for moyamoya disease, 23 for atherosclerotic disease refractory to medical therapy, 2 for complex cerebral aneurysms, and 1 for carotid occlusion as a sequela of cavernous meningioma growth. Ten patients underwent multiple operations, 9 of whom had moyamoya disease/syndrome, with the subsequent operation on the contralateral hemisphere. The average patient age at surgery was 45.1 years (range 14–73 years), with a mean follow-up time of 22.1 months. Intraoperative graft patency was confirmed in 100% of cases, and 101 (98.1%) of the 103 anastomoses with imaging follow-up were patent. Perfusion to the revascularized hemisphere was improved in 88.2% of cases. Perioperative ischemic and hemorrhagic complications occurred in 8 procedures (2 were asymptomatic), whereas remote ischemic and hemorrhagic events occurred in 7 cases. There was no mortality in the series, and the mean patient mRS scores were 1.72 at presentation and 1.15 at the last follow-up. CONCLUSIONS The high rates of intraoperative and postoperative patency support the feasibility of dual-anastomosis STA-MCA bypass for revascularization. The perioperative complication rate is not significantly different from that of single-anastomosis bypass. The functional outcomes at follow-up and perfusion improvement postoperatively support the efficacy and safety of this method as a treatment strategy.