Spontaneous Intraventricular Hemorrhage And Diagnosis And Therapeutic Strategies Of Intracranial Aneurysms Associated With Moyamoya Disease

Primary intraventricular hemorrhage (PIVH)is a neurological disorder, bleeding in the ventricular system without a discernable parenchymal fragment.The prognosis of patients with PIVH has been reported to be better than the prognosis of the patients with a diagnosis of secondary intraventricular hemorrhage.In the treatment of spontaneous intraventricular hemorrhage cases,With immediate Lateral ventricle drainage and management of hydrocephalus and intracranial pressure, more patients can obtain satisfactory curative effectBut there are still a part of patients experiencing further hemorrhage and death.And in this part of the cases of bleeding again, it maybe include Moyamoya disease, intracranial aneurysms associated with Moyamoya disease, arteriovenous malformations and so on.Moyamoya disease more common in eastern Asia, including China. Incidence of the disease is about (0.35 0.94) /100000. With the people’s understanding of moyamoya disease increasing and the wide application of CTA and MRA and DSA, there is an upward trend in number of cases. Moyamoya disease can be characterized by ischemia, hemorrhage, epilepsy and so on. About half of the adult patients with moyamoya disease was bleeding,which was their main performance. In recent years, studies have shown that the latter is the main cause for bleeding and rebleeding of the patient with Moyamoya disease.Initially, the aneurysms associated with Moyamoya disease were divided as major artery aneurysm and peripheral artery aneurysm.This classification limit neurosurgeon to further understand mechanism and clinical characteristic of intracranial aneurysms associated with Moyamoya disease, harmful for diagnosis and treatment. In 1996,a retrospective analysis of 111 patients with aneurysms associated with Moyamoya disease is presented. These 111 cases had 131 aneurysms.lt includes three types.around the circle of Willis(major artery), in the basal ganglia (so called Moyamoya vessels) and on the collateral vessels(the distal part of collateral anastomosis).The article shows a new point about clinical characteristics and classification concerning aneurysms associated with Moyamoya disease. Peripheral artery aneurysms were further divided into aneurysms in the basal ganglia and aneurysms on the collateral vessels. In recent years, the literature also reports aneurysms in the dura mater, cannot be ignored.Major artery aneurysms express as subarachnoid hemorrhage in many cases,so they won the high degree of attention.Diagnosed by CTA,MRA or DSA and treated by endovascular embolization,overwhelming majority of major artery aneurysms acquire satisfied effect as well as ideal follow-up results.However,the diagnosis, treatment and prognosis of peripheral artery aneurysms remains patchy.Aneurysms in basal ganglia express as basal ganglia hemorrhage in many cases, often mistaken for hypertensive cerebral hemorrhage, undertaken by conservative treatment without further checks. Although the diagnosis of bleeding reason unclear or wrong, the treatment effect could be satisfied. The serious problem is derived from the processing of aneurysms on the collateral vessels, is the most dangerous point of aneurysm associated with Moyamoya disease, which could be easy overlooked. Aneurysms on the collateral vessels express as intraventricular hemorrhage in many cases.With sparing application of accessory examinationm, intraventricular hemorrhage is considered to be the cause of hypertension or rupture of tiny blood vessels.Assuming relatively light Symptoms, patients could recover quickly with intraventricular external drainage or lumbar puncture treatment.Thus, bleeding reason and etiological treatment were ignored,as a result,it got serious consequences.After definite diagnosis,many peripheral artery aneurysms gained successful treatment with endovascular embolization or surgical treatment.But it should not be ignored that some patient sufferred from the absence of postoperative neurological function.At the same time, on one hand,the aneurysms can disappear after conservative treatment.On the other hand, some aneurysms ruptured again after conservative treatment.Therefore,consensus on treatment of peripheral artery aneurysms has not yet been formed, still need to explore.This study based on clinical cases of intraventricular hemorrhage caused by aneurysms associated with Moyamoya disease.On one hand,do some research to analyze the causes of spontaneous intraventricular hemorrhage.On the other hand,to investigate the classification and therapeutic Strategies of aneurysms associated with Moyamoya disease.Part 1 Analysis of Pathogenesis of Spontaneous Intraventricular HemorrhageBackground Primary intraventricular hemorrhage (PIVH)is a neurological disorder, bleeding in the ventricular system without a discernable parenchymal fragmentPrimary IVH in adults is comprising only 3.1% of all intracranial hemorrhages. Many neurosurgeon hold the view that hypertension is a major cause of spontaneous intraventricular hemorrhage.With ventricular drainage or lumbar puncture, especially thrombolytic drugs making blood clots dissolve and discharge, patients got rapid recovery.With traditional treatment,more patients can obtain satisfactory curative effect.But there are still a part of patients experiencing further hemorrhage and death.lt is not enough to simply handle the intraventricular hematoma, must further reason for bleeding and corresponding treatment.Objective This study analysis the causes of spontaneous intraventricular hemorrhage, and puts forward relevant treatment strategies.Methods 23 patients with spontaneous intraventricular hemorrhage were included without intraventricular hemorrhage associated with trauma from June 2011 to June 2014.CT alone was done in 6 patients, CT and DSA were done in 17 patients.Summarizing the bleeding risk factors, clinical characteristics, imaging examination, this study analyse the cause of bleeding and finish follow-up work.Results Case analysis of intraventricular hemorrhage shows several features:(1)Of the 23 patients,12 (52.17%) cases has been clear about the cause of bleeding derived from cerebrovascular disease. Moyamoya disease was most common predisposing factor (26.08%) including aneurysms associated with Moyamoya disease (13.08%) followed by arterio-venous malformations (AVMs) (13.08%),arteriovenous fistula(AVF)(4.34%),intracranial aneurysm(4.34%)and sinus thrombosis (4.34%). Hypertension is about 17.39%.(2)seventeen underwent DSA examination,12 cases with cerebrovascular disease, positive rate is 70.59%;All cases were divided into two groups, young groups (age<40), positive rate was 87.5% (7/8), the older group (age> 40), the positive rate was 55.56% (5/9);(3)Four patients with bleeding again, including 2 cases with moyamoya disease and 2 cases without DSA examination.The 4 cases all failed to deal with reason of bleeding.Conclusions Spontaneous intraventricular hemorrhage mostly comes from acute cerebrovascular disease.According to these cases,Moyamoya disease is the most important cause,followed by hypertension and arteriovenous malformation.In the group of age ess than or equal to 60 of spontaneous intraventricular hemorrhage cases, the positive rate of cerebrovascular disease is as high as 73.3%.Diagnosis of hypertension intraventricular hemorrhage have to rule out the factors of cerebrovascular disease. Spontaneous intraventricular hemorrhage should undergo regular cerebrovascular related inspection.Part 2 Classification and Therapeutic Strategies of Intracranial Aneurysms Associated with Moyamoya DiseaseBackground In recent years, studies have shown that aneurysms associated Moyamoya disease is the main cause for bleeding and rebleeding of the patient with Moyamoya disease.Initially, the aneurysms associated with Moyamoya disease were divided as major artery aneurysm and peripheral artery aneurysm.At that time,he major artery aneurysm only can be treatde by surgical operation.However,the peripheral artery aneurysms, are thought to a troublie,whose treatment is difficult and high risk.It is very easy to damage the main nerve, so conservative treatment would be taken.In 1996,a retrospective analysis of 111 patients with aneurysms associated with Moyamoya disease is presented. These 111 cases had 131 aneurysms.It includes three types:around the circle of Willis, in the basal ganglia and on the collateral vessels.The article shows a new point about clinical characteristics and classification concerning aneurysms associated with Moyamoya disease. At present,Major artery aneurysms express as subarachnoid hemorrhage in many cases,so they won the high degree of attention.Treated by endovascular embolization,overwhelming majority of major artery aneurysms acquire satisfied effect as well as ideal follow-up results.Aneurysms in basal ganglia and on the collateral vessels could be diagnosed by experienced neurosurgeon. However, many cases were overlooked and misdiagnosed, as a result, treatment outcomes could be terrible, especially when it comes to patients with ventricular hemorrhage. Treatments include conservative treatment, endovascular embolization and surgery. Consensus on treatment of peripheral artery aneurysms has not yet been formed. Treatment of aneurysms remains patchy. In recent years, the literature also reports aneurysms in the dura mater, whose treatment faces challenges. So there are a lot of problems during the treatment.Objective Summarizing diagnosis, treatment and follow-up results of aneurysms associated hemorrhagic Moyamoya disease, this study analyses characteristics of the aneurysms and assesses the risk of rupture of different types of aneurysms,and tries to regulate the diagnosis and treatment of aneurysms associated with Moyamoya disease.Methods Basing on guidelines for diagnosis and indications,ten patients (12 aneurysms) with aneurysms associated with hemorrhagic moyamoya disease were included from June 2011 to June 2014. They all accept CT and DSA examination.According to the different characteristics and the location of the aneurysm, they got different treatments by a single team of cerebrovascular disease.Of the 12 aneurysms diagnosed by DSA,7 were found distributed around the circle of Willis,2 in the basal ganglia and 3 on the collateral vessels.Nine aneurysms were ruptured and 3 were unruptured. Six of the 7 aneurysms around the circle of Willis were treated by endovascular embolization or surgical clipping, while the remaining 1 case was treated conservatively.Two aneurysms in the basal ganglia were managed with conservative treatment.Of the 3 aneurysms on the collateral vessels,2 were occluded by 33% Glubran glue injection,1 was treated conservatively.Results Treatment of aneurysms associated with Moyamoya disease:(1)Six aneurysms around the circle of Willis were successfully and completely occluded or clipped.The other 1 patient was managed with conservative treatment and the aneurysm was stable.Two aneurysms in the basal ganglia were followed up conservatively and disappeared 3 and 8 months later,respectively.Two of the 3 aneurysms on the collateral vessels got complete embolization.The patient treated conservatively experienced a second episode of bleeding and died 14 days after the first hemorrhage. (2) Outcome was assessed using the modified Rankin Scale (mRS) when patients discharged, 0 point in 5 cases,1 in 2 cases,2 in 1 case,3 in 1 case,4 in 0 case and 6 in 1 case. (3) The follow-up period of the 9 patients was between 2 to 36 months. Re-examination by DSA revealed no recurrence of the aneurysms occluded or clipped and the aneurysms disappearing spontaneously.The aneurysm treated conservatively remained stationary.Conclusions Aneurysms associated with moyamoya disease are known to occur around the circle of Willis, in the basal ganglia, and on the collateral vessels. We recommend endovascular embolization or surgical intervention for aneurysms around the circle of Willis and on the collateral vessels. However, it should be treated conservatively for aneurysms found in the basal ganglia.