Hemorrhagic Moyamoya Disease in Children: Clinical, Angiographic features, and Long-Term Surgical Outcome.

BACKGROUND AND PURPOSE: Here, we describe the clinical, angiographic characteristics, and long-term surgical outcome of hemorrhagic moyamoya disease in children. METHODS: We retrospectively collected 374 consecutive children with moyamoya disease (hemorrhagic 30 and ischemic 344) between 2004 and 2012 in our hospital. The clinical and radiological characteristics of the hemorrhagic patients were retrospectively described and analyzed. All the hemorrhagic patients underwent encephalo-duro-arterio-synangiosis procedure. Digital subtraction angiography was performed to evaluate the efficacy of vascularization. Clinical follow-up outcomes were obtained through clinical visits, telephone, or letter interview. RESULTS: In our study, the ratio of female to male patients in the hemorrhagic group was significantly higher than the ischemic group (2:1 versus 0.9:1; P<0.05). The most frequent hemorrhagic location was intraventricular hemorrhage (n=22, 73%). In addition, significantly greater dilatation of the anterior choroidal artery and the posterior communicating artery were seen in the hemorrhagic group (P<0.05). Good or fair vascularization were observed in all the 15 children with digital subtraction angiography follow-up. Clinical outcomes showed that 25 of 30 (83%) patients had no disability (modified Rankin scale score, 0 and 1); 1 patient (3.3%) died of recurrent hemorrhagic stroke. CONCLUSIONS: The presence of anterior choroidal artery and posterior communicating artery dilation may be associated with the bleeding episode in the children with hemorrhagic moyamoya disease. The encephalo-duro-arterio-synangiosis surgery can effectively increase the cerebral blood flow in children, which may decrease the incidence of recurrent hemorrhage.

Dissection-related carotid-cavernous fistula (CCF) following surgical revascularization of chronic internal carotid artery occlusion: a new subtype of CCF and proposed management.

BACKGROUND: The development of carotid-cavernous fistulas (CCFs) during surgical recanalization of chronic internal carotid artery occlusion (ICAO) may be secondary to severe ICA dissection rather than a focal tear of the cavernous ICA seen in typical traumatic CCFs. The purpose of this study is to investigate the causal relationship between the CCFs and severe ICA dissections and to characterize technical outcomes after treatment with stenting. METHODS: Five patients underwent treatment with self-expanding stents due to intraprocedural CCF and ICA dissection following surgical removal of ICAO plaque. The stents were telescopically placed via true channel of the dissection. Safety of the procedure was evaluated with 30-day stroke and death rate. Procedural success was determined by the efficacy of CCF obliteration and ICAO recanalization with angiography. RESULTS: All CCFs were associated with spiral and long segmental dissection from the cervical to cavernous ICA. After stenting, successful dissection reconstruction with TICI 3 was achieved in all patients, with complete (n = 4) or partial CCF (n = 1) obliteration. No patient had CCF syndrome, stroke, or death during follow-up of 6 to 37 months; but one patient had pulsatile tinnitus, which resolved 1 year later. Angiography at 6 to 24 months demonstrated CCF obliteration in all 5 patients and durable ICA patency in 4 patients. CONCLUSIONS: Intraprocedural CCFs with spiral and cervical-to-cavernous ICA dissection during ICAO surgery are dissection-related because of successful obliteration after stenting for dissection reconstruction. Self-expanding stenting through true channel of the dissection, serving as implanting stent-autograft, may be an optimal therapy for the atypical CCF complication from ICAO surgery.

A complex cavernous sinus dural arteriovenous fistula secondary to covered stent placement for a traumatic carotid artery-cavernous sinus fistula: case report.

Authors present the case of a patient with a direct carotid artery-cavernous sinus fistula caused by head trauma in whom a self-expanding covered stent was successfully used to obliterate the fistula. However, at the 9-month follow-up an angiogram revealed a complex caroticocavernous fistula that was completely obliterated with Onyx 18 transarterially.

Intracranial Aneurysms Associated with Moyamoya Disease in Children: Clinical Features and Long-Term Surgical Outcome.

BACKGROUND: Moyamoya disease (MMD) in children was rarely associated with intracranial aneurysms. The purpose of this study was to report the clinical characteristics and long-term surgical outcomes of pediatric intracranial aneurysms accompanied with MMD. METHODS: Between October 2002 and October 2013, our department treated 9 pediatric MMD patients (aged ≤17 years) with intracranial aneurysms. Clinical and angiographic features, treatment selection, as well as follow-up information were obtained and analyzed. The efficacy of vascularization and the changes of intracranial aneurysms were evaluated with digital subtraction angiography (DSA). We also collected 7 previously published reports to analyze the characteristics of this rare condition. RESULTS: In our series of 9 patients, 7 were male. The mean age was 11 ± 3.4 years (range 5-16). Seven patients presented with intracranial hemorrhage as the initial manifestation, while 2 patients suffered transient ischemic attacks. The most common aneurysm location was the posterior choroidal artery (4, 44.4%). One anterior choroidal artery aneurysm was completely embolized with Onyx (ev3, Irvine, California, USA). One posterior choroidal artery aneurysm failed due to inaccessibility to the parent artery. Bilateral encephalo-duro-arterio-synangiosis (EDAS) surgery was performed for all the children. During the follow-up period of 6.4 ± 2.2 years (range 3-11), spontaneous occlusion of aneurysm was observed in 4 children, including 1 child with middle cerebral artery aneurysm, 1 with lenticulostriate artery aneurysm, and 2 with posterior choroidal artery aneurysm. Good or fair vascularization was observed in all the 9 children with DSA follow-up. No patients suffered intracranial hemorrhage during the follow-up period. CONCLUSIONS: The long-term survey showed EDAS surgery could effectively increase the cerebral blood flow and maintain good outcomes in children, which may further result in the disappearance of the intracranial aneurysms and decrease the incidence of recurrent hemorrhage.

Difference in Angiographic Characteristics Between Hemorrhagic And Nonhemorrhagic Hemispheres Associated with Hemorrhage Risk of Moyamoya Disease in Adults: A Self-Controlled Study.

BACKGROUND: Here, we compare the angiographic characteristics of hemorrhagic and nonhemorrhagic hemispheres within the same adult patient with moyamoya disease (MMD) and identify the possible risk factors for initial and recurrent hemorrhage during the long-term follow-up period. METHODS: We retrospectively collected and analyzed the clinical and angiographic data of 145 consecutive adults with hemorrhagic MMD between 2004 and 2011. Separate angiographic characteristics of the hemorrhagic and nonhemorrhagic hemispheres were analyzed based on digital subtraction angiography. Multivariate logistic regression analysis was used to study the risk factors related to initial hemorrhage. In addition, clinical follow-up for at least 5 years was obtained in all the 145 patients. Cox regression analysis was used to determine the predictors for the subsequent recurrent hemorrhagic strokes. RESULTS: With regard to the 145 consecutive patients in our cohort, multivariate analysis indicated that the dilation grade of anterior choroidal artery (odds ratio [OR], 2.449; 95% confidence interval [CI], 1.617-3.862), the dilation grade of the posterior communicating artery (PComA) (OR, 1.491; 95% CI, 1.071-2.075), and the involvement of the posterior cerebral artery (OR, 4.623; 95% CI, 1.273-16.792) were significantly associated with initial hemorrhage. After a median follow-up of 6.33 ± 1.81 years, 20 of the 145 patients (13.8%) developed 22 recurrent episodes of hemorrhage. No significant correlation was found between rebleeding and the above significant factors associated with the initial hemorrhage. CONCLUSIONS: Compared with the nonhemorrhagic hemispheres, hemorrhagic hemispheres are more prone to recurrent hemorrhage. Our case-control study showed the dilation of the anterior choroidal artery or posterior communicating artery, as well as the involvement of the posterior cerebral artery, is associated with the initial hemorrhage of MMD, but not for the episode of recurrent hemorrhage. Longer and more detailed clinical and angiographic follow-up are still needed to delineate the specific mechanism underlying the recurrent hemorrhage in hemorrhagic MMD.