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Hemorrhagic type Moyamoya disease
Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196 – S201
Hemorrhagic type Moyamoya disease Naokatsu Saeki a,*, Susumu Nakazaki a, Motoo Kubota a, Akira Yamaura a, Seiichirou Hoshi b, Souichi Sunada b, Kenro Sunami b a
Department of Neurological Surgery, Chiba Uni6ersity, School of Medicine, 1 -8 -1 Inohana, Chuhohku Chibashi, Chiba 260, Japan b Department of Neurological Surgery, Kawatetsu Chiba Hospital, Chiba, Japan
Abstract The clinical picture of hemorrhagic type Moyamoya disease was analyzed in 20 cases. Hematoma at the basal ganglia was noted in 40% of cases, intraventricular hemorrhage (IVH) in 30%, thalamic hemorrhage with ventricular rupture in 15% and subcortical hemorrhage in 5%. The location was undetermined in two cases (10%) due to bleeding in the pre-computed tomography (CT) era. The frequencies shown above were correlated well to previous reports. In magnetic resonance imaging (MRI) performed 1 year or more after IVH, the primary bleeding site was demonstrated at the lateral wall of lateral ventricle, in proton density weighted and T2 weighted images. MRI can detect the site of old bleeding points and its chronological change if the study is repeated. In a follow-up period of 6.2 years, 35% of the cases had rebleeding once or twice. The second bleeding occured seven times and the third twice. IVH occurred five times and the most common, basal ganglia hematoma three times while thalamic hemorrhage once. As a result, the rate of good outcome was 60% after the first bleeding and 40% after rebleedings. The mortality rate was 5% after the first and 25% after rebleedings. Factors related to rebleedings and their poorer outcome are sex (with women being more susceptible), massive ICH and early recurrence. Rebleedings worsened the outcome. Therefore, prevention of rebleeding is important. From a therapeutic viewpoint, although a close relation between rebleeding and untreated hypertension could not be established, blood pressure control is critical at the both acute and chronic stages. Bypass surgery for bleeding type of Moyamoya disease seems to be less promising than ischemic type, even though a definite answer may not be obtained from our small number of cases. © 1997 Elsevier Science B.V. Keywords: Moyamoya disease; Hemorrhage; Intraventricular hemorrhage; Magnetic resonance imaging; Bypass surgery
1. Introduction Hemorrhagic type Moyamoya disease has different clinical profiles from ischemic type, since it is more common among adults, efficacy of revascularization is uncertain and prognosis is poorer [1 – 3]. In this paper, clinical features of hemorrhagic type Moyamoya disease are analyzed based on our own experience, in
* Corresponding author. Tel.: +81 43 2227171; fax: + 81 43 2250573. 0303-8467/97/$17.00 © 1997 Elsevier Science B.V. All rights reserved. PII S 0 3 0 3 - 8 4 6 7 ( 9 7 ) 0 0 0 7 9 - 6
order to more clearly elucidate its natural course and search for better treatment modalities for this type.
2. Cases This paper deals with 20 patients of hemorrhagic type Moyamoya disease, experienced in Chiba University and its affiliated hospitals after the computed tomography (CT) era. The CT scans in two patients who bled in pre-CT era, were not available. Male to female ratio was 6:14. Hemorrhagic type was more common
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
S197
among women. Average age at the initial onset was 42.2 years (11–75 years). Follow-up period was 6.2 years (14 days–27 years) (Table 1).
3. Results In 20 cases, the total number of bleeding was 29. Of these CT scan was available for 27 times. Intraventricular hemorrhage (IVH) occured 11 times (37.9%), basal ganglia hematoma 11 times (37.9%), thalamic hemorrhage four times (13.7%), subcortical hemorrhage once (3.4%) and unknown twice (6.9%), respectively (Table 1).
Fig. 2. Case 17 in Table 2. Microaneurysm, 2 mm in diameter, was found at the left distal anterior choroidal artery (arrow). This was supposed to be related to intravantricular hemorrhage. This disappeared in the following angiography 6 months later.
3.1. First bleeding Common symptoms were motor weakness, headache and conscious disturbance in the first bleeding. Basal ganglia hematoma was in eight cases (40%); IVH, six cases (30%); thalamic hemorrhage, three cases (15%); subcortical hemorrhage, one case (5%); and unknown, two cases (10%).
3.2. Illustrati6e cases
Fig. 1. (a) Case 2 in Table 1. Left basal ganglia hematoma. (b) Case 17 in Table 2. Intraventricular hemorrhage. (c) Case 13 in Table 1. Left thalamic hemorrhage with ventricular rupture.
A 37 year-old man presented with left putaminal hemorrhage. Right hemihypesthesia was the initial symptom, which continued for 2 weeks. There was no history of hypertension. The angiographical grade was three. Since PET scan showed normal CBF and OEF, no revascularizing procedure was carried out. In the next 8 year follow-up period, no further episode has been noted. (Fig. 1a). A 44 year-old woman without hypertension history complained of headache. CT showed IVH (Fig. 1b) Angiographical grade was four. In addition, microaneurysm, 2 mm in diameter, was found at the distal anterior choroidal artery on the left side which disappeared in a follow-up angiography 6 months later (Fig. 2). An 11 year-old girl complained of headache and subsequently became semicomatose in the following 7 h. CT showed left thalamic hemorrhage with ventricular rupture. Angiographical grade was four. She was treated conservatively and discharged 3 months later. She has been moderately disabled in the following 5 years (Fig. 1c).
44 37 67 24 32 59 63 50 51 28 23 55 11
1 2 3 4 5 6 7 8 9 10 11 12 13
F M F M F M M F F F M F F
Sex
Lt hemiparesis Lt hemiparesis Headache Hemihypesthesia Lt hemiparesis Headache Headache Rt hemiparesis Rt hemiparesis Headache Lt hemiparesis Lt hemiparesis Lt hemihypesthesia Consciousness disturbance Consciousness disturbance Rt hemiparesis
Initial symptoms and signs Rt basal ganglia Rt basal ganglia Lt basal ganglia (SAH) Lt basal ganglia Rt thalamus (IVH) IVH IVH IVH Lt basal ganglia IVH Rt parietal lobe subcortical Rt basal ganglia Lt thalamus (IVH)
CT findings bleeding site (−) (−) EMS (−) (−) (−) (−) V-P shunt Hematoma removal (−) Hematoma removal (−) V-P shunt
Surgical treatment 2 – 6/12% 7 2 6/12% 3 9 6 7 7/12% 9/12% 4 — 5
Follow up period (years) G G G G G G G SD V MD MD D MD
Outcome (−) (−) (−) (+) (−) (+) (+) (+) (−) (−) (−) (+) (−)
Hypertension
Age
34
47
38
25
75
44
36
Case
14
15
16
17
18
19
20
M
F
F
F
F
F
F
Sex
(1) 1979 (2) 1992 13 years
Rt hemiparesis Coma
Consciousness disturbance
6/12 years
(2) 1990
Consciousness disturbance Lt hemiparesis Consciousness disturbance
Headache Headache Rt hemiparesis
Headache consciousness disturbance Rt hemiparesis
Headache consciousness Disturbance
Lt hemiparesis
14 days
18 years 8 years
2 years
2 years
(1) 1989
(2) 1987
(1) 1987
(1) 1968 (2) 1987 (3) 1995
(1) 1980 (2) 1982
(1) 1980 (2) 1982
Lt hemiparesis consciousness Disturbance
(2) 1986 (3) 1986
11 years 17 days
Headache
(1) 1975
Year of bleeding Interval of bleed- Initial symptoms and signs ings
Table 2 Clinical course of rebleeding cases
Lt basal ganglia Rt basal ganglia (IVH)
Lt basal ganglia (frontal) Lt basla ganglia (IVH)
Rt thalamus (IVH SAH) Lt basal ganglia (IVH)
(−) IVH IVH
IVH Lt Thalamus (IVH)
IVH IVH
IVH IVH
(−)
CT findings
Hematoma removal (−)
Drainage
(−)
(−)
Drainage
(−) EDAS
Ventricular drainage V-P shunt
(−) (−)
(−) Ventricular drainage EDAS
Surgical treatment
13 years
4 years
14 days
27 years
2 years
2 years
20 years
Follow up period
SD D
SD
MD
D
SD
G G G
G D
G D
MD SD
G
(−)
(+)
(+)
(−)
(−)
(−)
(+)
Outcome Hypertension
M, male; F, female; Lt, left; Rt, right; SAH, subarachnoid hemorrhage; IVH, intraventricular hemorrhage; G, good recovery; MD, moderate disability; SD, severe disability; V, vegetative state; D, death.
Age
Case
Table 1 Clinical course of single bleeding cases S198 N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
S199
Fig. 3. Case 18 in Table 2. (a) Right thalamic hemorrhage with subarachnoid and IVH in the first attack. (b) Huge left basal ganglia hematoma with IVH in the second attack. In this case, bleeding points are different.
Fig. 4. Seven cases with intraventricular hemorrhage. Responsible bleeding points at the paraventricular areas were noted (arrows), which are highly characteristic of IVH in Moyamoya disease.
Secondary IVH following thalamic or basal ganglia hemorrhage was noted in three cases out of 20 (15%). Secondary SAH was noted in two cases (10%) on CT. No primary SAH was noted.
3.3. Rebleeding Rebleeding occurred in seven cases 35%, during 6.2 year follow-up period. Of these six cases were women
S200
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
and one male case. Rebleeding was also more common among women. Rebleeding occurred nine times. The second bleeding seven times and the third occured twice. IVH was five times and the most common, basal ganglia hematoma three times and thalamic hemorrhage once (Table 2).
type (IVH, basal ganglia or thalamic hemorrhage) was the same except for one case. Death after IVH occured in one case, while death after ICH occured in three cases. Outcome after ICH was worse than after IVH, because the main cause of death was massive hematoma compressing vital brain structures.
3.4. Illustrati6e cases
3.5. Surgical treatment
A 75 year-old woman suffered from right thalamic hemorrhage. Angiographical grade was three. Another hemorrhage occurred 14 days later at the left basal ganglia and she died a week later. In this case, rebleeding occurred in the different place even at the contralateral hemisphere (Fig. 3). Interval between bleedings was various, 14 days to 18 years. In case 14, IVH recurred in 17 days and case 18 in 14 days. Both cases had poor outcome. Hemorrhagic
Surgical treatment was VP shunt and ventricular drainage in seven cases and hematoma removal in two cases. Revascularizing operations were EDAS in two cases and EMS in one case. In case 17 with EDAS, IVH recurred 8 years after the operation.
3.6. Hypertension Relation between rebleeding and hypertension was analyzed. In the case of no obvious history related to hypertension previous to bleeding, blood pressure at the outpatient clinic was referred. Hypertension was noted in five of 13 single bleeding cases, while three of seven cases were noted with rebleeding episodes. From our analysis, no significant relation was noted between hypertension and rebleeding.
3.7. Outcome The rate of good recovery is 60% after the first and decreased to 40% after the rebleedings. The mortality rate was 5% after the first and 25% after rebleedings. Rebleedings make the outcome worse and therefore prevention of rebleeding is important (Table 1).
3.8. Neuroradiological features
Fig. 5. Case 6 in Table 1. (a) Intraventricular hemorrhage. With close observation, the lateral wall of left lateral ventricle is suspected to be a responsible bleeding point. (b) CT scan 6 years after bleeding does not reveal any bleeding origin. (c) and (d) MRI 6 years after bleeding. In both proton density weighted and T2 weighted images, high intensity lesions are noted to show the origin of old bleeding point.
CT findings of IVH in Moyamoya disease showed diagnostically common characteristics. They seemed to be pure IVH. However, with a careful look at lateral ventricular wall on CTs, we could find thin parenchymal high density areas which was supposed to be the original bleeding point. In seven initial primary IVH cases, this finding was commonly seen and highly diagnostic of IVH due to Moyamoya disease (Fig. 4). MRI offered a hint as to the location of the original bleeding point even years after the initial bleeding. Case 6 had IVH mainly on the left side, which became unrecognized 3 months later on CTs. MRI a year later still showed high intensity change in particular on T2 and PD weighted images (Fig. 5) MRI more clearly showed the original bleeding point. This was diagnostically highly characteristic of previous IVH due to Moyamoya disease.
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
4. Discussion Factors related to rebleeding and its poorer outcome were sex (women being more susceptible), massive ICH and early recurrence. The last factor emphasizes the importance of blood pressure control in the acute stage. From our data, recurrent bleeding is commonly the same type (IVH or ICH) and occurs at a different place even on the contralateral hemisphere. This is the reason why prophylactic revascularizing operation should be performed bilaterally. Is revascularizing procedure effective to prevent rebleeding? In our series, of the three operated cases one had rebleeding. However this question remains controversial and unanswered. Yonekawa and Aoki separately reported the negative data in their experience [1,3]. Wanifuchi raised supportive opinion for this surgery [2]. Bypass surgery for hemorrhagic type of
S201
Moyamoya disease seems to be less promising than ischemic type, even though a definite answer is not obtained from our small number of cases.
References [1] Aoki N. Cerebrovascular bypass surgery for the treatment of Moyamoya disease: Unsatisfactory outcome in patients presenting with intracranial hemorrhage. Surg Neurol 1993;40:327–72. [2] Wanifuchi H, Takeshita M, Izawa M, Aoki N, Kagawa M. Management of adult Moyamoya disease. Neurologia MedicoChirugica 1993;33:300 – 5. [3] Yonekawa Y, Yamashita K, Taki W, Kikuchi H. Clinical features of hemorrhagic type of moyamoya disease: Special emphasis on cases with rebleeding, In: Annual Report of the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya disease) of the Ministry of Health and Welfare, Japan (Chairman Kikuchi H.). 1988:81 – 88.
.
Hemorrhagic type Moyamoya disease Naokatsu Saeki a,*, Susumu Nakazaki a, Motoo Kubota a, Akira Yamaura a, Seiichirou Hoshi b, Souichi Sunada b, Kenro Sunami b a
Department of Neurological Surgery, Chiba Uni6ersity, School of Medicine, 1 -8 -1 Inohana, Chuhohku Chibashi, Chiba 260, Japan b Department of Neurological Surgery, Kawatetsu Chiba Hospital, Chiba, Japan
Abstract The clinical picture of hemorrhagic type Moyamoya disease was analyzed in 20 cases. Hematoma at the basal ganglia was noted in 40% of cases, intraventricular hemorrhage (IVH) in 30%, thalamic hemorrhage with ventricular rupture in 15% and subcortical hemorrhage in 5%. The location was undetermined in two cases (10%) due to bleeding in the pre-computed tomography (CT) era. The frequencies shown above were correlated well to previous reports. In magnetic resonance imaging (MRI) performed 1 year or more after IVH, the primary bleeding site was demonstrated at the lateral wall of lateral ventricle, in proton density weighted and T2 weighted images. MRI can detect the site of old bleeding points and its chronological change if the study is repeated. In a follow-up period of 6.2 years, 35% of the cases had rebleeding once or twice. The second bleeding occured seven times and the third twice. IVH occurred five times and the most common, basal ganglia hematoma three times while thalamic hemorrhage once. As a result, the rate of good outcome was 60% after the first bleeding and 40% after rebleedings. The mortality rate was 5% after the first and 25% after rebleedings. Factors related to rebleedings and their poorer outcome are sex (with women being more susceptible), massive ICH and early recurrence. Rebleedings worsened the outcome. Therefore, prevention of rebleeding is important. From a therapeutic viewpoint, although a close relation between rebleeding and untreated hypertension could not be established, blood pressure control is critical at the both acute and chronic stages. Bypass surgery for bleeding type of Moyamoya disease seems to be less promising than ischemic type, even though a definite answer may not be obtained from our small number of cases. © 1997 Elsevier Science B.V. Keywords: Moyamoya disease; Hemorrhage; Intraventricular hemorrhage; Magnetic resonance imaging; Bypass surgery
1. Introduction Hemorrhagic type Moyamoya disease has different clinical profiles from ischemic type, since it is more common among adults, efficacy of revascularization is uncertain and prognosis is poorer [1 – 3]. In this paper, clinical features of hemorrhagic type Moyamoya disease are analyzed based on our own experience, in
* Corresponding author. Tel.: +81 43 2227171; fax: + 81 43 2250573. 0303-8467/97/$17.00 © 1997 Elsevier Science B.V. All rights reserved. PII S 0 3 0 3 - 8 4 6 7 ( 9 7 ) 0 0 0 7 9 - 6
order to more clearly elucidate its natural course and search for better treatment modalities for this type.
2. Cases This paper deals with 20 patients of hemorrhagic type Moyamoya disease, experienced in Chiba University and its affiliated hospitals after the computed tomography (CT) era. The CT scans in two patients who bled in pre-CT era, were not available. Male to female ratio was 6:14. Hemorrhagic type was more common
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
S197
among women. Average age at the initial onset was 42.2 years (11–75 years). Follow-up period was 6.2 years (14 days–27 years) (Table 1).
3. Results In 20 cases, the total number of bleeding was 29. Of these CT scan was available for 27 times. Intraventricular hemorrhage (IVH) occured 11 times (37.9%), basal ganglia hematoma 11 times (37.9%), thalamic hemorrhage four times (13.7%), subcortical hemorrhage once (3.4%) and unknown twice (6.9%), respectively (Table 1).
Fig. 2. Case 17 in Table 2. Microaneurysm, 2 mm in diameter, was found at the left distal anterior choroidal artery (arrow). This was supposed to be related to intravantricular hemorrhage. This disappeared in the following angiography 6 months later.
3.1. First bleeding Common symptoms were motor weakness, headache and conscious disturbance in the first bleeding. Basal ganglia hematoma was in eight cases (40%); IVH, six cases (30%); thalamic hemorrhage, three cases (15%); subcortical hemorrhage, one case (5%); and unknown, two cases (10%).
3.2. Illustrati6e cases
Fig. 1. (a) Case 2 in Table 1. Left basal ganglia hematoma. (b) Case 17 in Table 2. Intraventricular hemorrhage. (c) Case 13 in Table 1. Left thalamic hemorrhage with ventricular rupture.
A 37 year-old man presented with left putaminal hemorrhage. Right hemihypesthesia was the initial symptom, which continued for 2 weeks. There was no history of hypertension. The angiographical grade was three. Since PET scan showed normal CBF and OEF, no revascularizing procedure was carried out. In the next 8 year follow-up period, no further episode has been noted. (Fig. 1a). A 44 year-old woman without hypertension history complained of headache. CT showed IVH (Fig. 1b) Angiographical grade was four. In addition, microaneurysm, 2 mm in diameter, was found at the distal anterior choroidal artery on the left side which disappeared in a follow-up angiography 6 months later (Fig. 2). An 11 year-old girl complained of headache and subsequently became semicomatose in the following 7 h. CT showed left thalamic hemorrhage with ventricular rupture. Angiographical grade was four. She was treated conservatively and discharged 3 months later. She has been moderately disabled in the following 5 years (Fig. 1c).
44 37 67 24 32 59 63 50 51 28 23 55 11
1 2 3 4 5 6 7 8 9 10 11 12 13
F M F M F M M F F F M F F
Sex
Lt hemiparesis Lt hemiparesis Headache Hemihypesthesia Lt hemiparesis Headache Headache Rt hemiparesis Rt hemiparesis Headache Lt hemiparesis Lt hemiparesis Lt hemihypesthesia Consciousness disturbance Consciousness disturbance Rt hemiparesis
Initial symptoms and signs Rt basal ganglia Rt basal ganglia Lt basal ganglia (SAH) Lt basal ganglia Rt thalamus (IVH) IVH IVH IVH Lt basal ganglia IVH Rt parietal lobe subcortical Rt basal ganglia Lt thalamus (IVH)
CT findings bleeding site (−) (−) EMS (−) (−) (−) (−) V-P shunt Hematoma removal (−) Hematoma removal (−) V-P shunt
Surgical treatment 2 – 6/12% 7 2 6/12% 3 9 6 7 7/12% 9/12% 4 — 5
Follow up period (years) G G G G G G G SD V MD MD D MD
Outcome (−) (−) (−) (+) (−) (+) (+) (+) (−) (−) (−) (+) (−)
Hypertension
Age
34
47
38
25
75
44
36
Case
14
15
16
17
18
19
20
M
F
F
F
F
F
F
Sex
(1) 1979 (2) 1992 13 years
Rt hemiparesis Coma
Consciousness disturbance
6/12 years
(2) 1990
Consciousness disturbance Lt hemiparesis Consciousness disturbance
Headache Headache Rt hemiparesis
Headache consciousness disturbance Rt hemiparesis
Headache consciousness Disturbance
Lt hemiparesis
14 days
18 years 8 years
2 years
2 years
(1) 1989
(2) 1987
(1) 1987
(1) 1968 (2) 1987 (3) 1995
(1) 1980 (2) 1982
(1) 1980 (2) 1982
Lt hemiparesis consciousness Disturbance
(2) 1986 (3) 1986
11 years 17 days
Headache
(1) 1975
Year of bleeding Interval of bleed- Initial symptoms and signs ings
Table 2 Clinical course of rebleeding cases
Lt basal ganglia Rt basal ganglia (IVH)
Lt basal ganglia (frontal) Lt basla ganglia (IVH)
Rt thalamus (IVH SAH) Lt basal ganglia (IVH)
(−) IVH IVH
IVH Lt Thalamus (IVH)
IVH IVH
IVH IVH
(−)
CT findings
Hematoma removal (−)
Drainage
(−)
(−)
Drainage
(−) EDAS
Ventricular drainage V-P shunt
(−) (−)
(−) Ventricular drainage EDAS
Surgical treatment
13 years
4 years
14 days
27 years
2 years
2 years
20 years
Follow up period
SD D
SD
MD
D
SD
G G G
G D
G D
MD SD
G
(−)
(+)
(+)
(−)
(−)
(−)
(+)
Outcome Hypertension
M, male; F, female; Lt, left; Rt, right; SAH, subarachnoid hemorrhage; IVH, intraventricular hemorrhage; G, good recovery; MD, moderate disability; SD, severe disability; V, vegetative state; D, death.
Age
Case
Table 1 Clinical course of single bleeding cases S198 N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
S199
Fig. 3. Case 18 in Table 2. (a) Right thalamic hemorrhage with subarachnoid and IVH in the first attack. (b) Huge left basal ganglia hematoma with IVH in the second attack. In this case, bleeding points are different.
Fig. 4. Seven cases with intraventricular hemorrhage. Responsible bleeding points at the paraventricular areas were noted (arrows), which are highly characteristic of IVH in Moyamoya disease.
Secondary IVH following thalamic or basal ganglia hemorrhage was noted in three cases out of 20 (15%). Secondary SAH was noted in two cases (10%) on CT. No primary SAH was noted.
3.3. Rebleeding Rebleeding occurred in seven cases 35%, during 6.2 year follow-up period. Of these six cases were women
S200
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
and one male case. Rebleeding was also more common among women. Rebleeding occurred nine times. The second bleeding seven times and the third occured twice. IVH was five times and the most common, basal ganglia hematoma three times and thalamic hemorrhage once (Table 2).
type (IVH, basal ganglia or thalamic hemorrhage) was the same except for one case. Death after IVH occured in one case, while death after ICH occured in three cases. Outcome after ICH was worse than after IVH, because the main cause of death was massive hematoma compressing vital brain structures.
3.4. Illustrati6e cases
3.5. Surgical treatment
A 75 year-old woman suffered from right thalamic hemorrhage. Angiographical grade was three. Another hemorrhage occurred 14 days later at the left basal ganglia and she died a week later. In this case, rebleeding occurred in the different place even at the contralateral hemisphere (Fig. 3). Interval between bleedings was various, 14 days to 18 years. In case 14, IVH recurred in 17 days and case 18 in 14 days. Both cases had poor outcome. Hemorrhagic
Surgical treatment was VP shunt and ventricular drainage in seven cases and hematoma removal in two cases. Revascularizing operations were EDAS in two cases and EMS in one case. In case 17 with EDAS, IVH recurred 8 years after the operation.
3.6. Hypertension Relation between rebleeding and hypertension was analyzed. In the case of no obvious history related to hypertension previous to bleeding, blood pressure at the outpatient clinic was referred. Hypertension was noted in five of 13 single bleeding cases, while three of seven cases were noted with rebleeding episodes. From our analysis, no significant relation was noted between hypertension and rebleeding.
3.7. Outcome The rate of good recovery is 60% after the first and decreased to 40% after the rebleedings. The mortality rate was 5% after the first and 25% after rebleedings. Rebleedings make the outcome worse and therefore prevention of rebleeding is important (Table 1).
3.8. Neuroradiological features
Fig. 5. Case 6 in Table 1. (a) Intraventricular hemorrhage. With close observation, the lateral wall of left lateral ventricle is suspected to be a responsible bleeding point. (b) CT scan 6 years after bleeding does not reveal any bleeding origin. (c) and (d) MRI 6 years after bleeding. In both proton density weighted and T2 weighted images, high intensity lesions are noted to show the origin of old bleeding point.
CT findings of IVH in Moyamoya disease showed diagnostically common characteristics. They seemed to be pure IVH. However, with a careful look at lateral ventricular wall on CTs, we could find thin parenchymal high density areas which was supposed to be the original bleeding point. In seven initial primary IVH cases, this finding was commonly seen and highly diagnostic of IVH due to Moyamoya disease (Fig. 4). MRI offered a hint as to the location of the original bleeding point even years after the initial bleeding. Case 6 had IVH mainly on the left side, which became unrecognized 3 months later on CTs. MRI a year later still showed high intensity change in particular on T2 and PD weighted images (Fig. 5) MRI more clearly showed the original bleeding point. This was diagnostically highly characteristic of previous IVH due to Moyamoya disease.
N. Saeki et al. / Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S196–S201
4. Discussion Factors related to rebleeding and its poorer outcome were sex (women being more susceptible), massive ICH and early recurrence. The last factor emphasizes the importance of blood pressure control in the acute stage. From our data, recurrent bleeding is commonly the same type (IVH or ICH) and occurs at a different place even on the contralateral hemisphere. This is the reason why prophylactic revascularizing operation should be performed bilaterally. Is revascularizing procedure effective to prevent rebleeding? In our series, of the three operated cases one had rebleeding. However this question remains controversial and unanswered. Yonekawa and Aoki separately reported the negative data in their experience [1,3]. Wanifuchi raised supportive opinion for this surgery [2]. Bypass surgery for hemorrhagic type of
S201
Moyamoya disease seems to be less promising than ischemic type, even though a definite answer is not obtained from our small number of cases.
References [1] Aoki N. Cerebrovascular bypass surgery for the treatment of Moyamoya disease: Unsatisfactory outcome in patients presenting with intracranial hemorrhage. Surg Neurol 1993;40:327–72. [2] Wanifuchi H, Takeshita M, Izawa M, Aoki N, Kagawa M. Management of adult Moyamoya disease. Neurologia MedicoChirugica 1993;33:300 – 5. [3] Yonekawa Y, Yamashita K, Taki W, Kikuchi H. Clinical features of hemorrhagic type of moyamoya disease: Special emphasis on cases with rebleeding, In: Annual Report of the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya disease) of the Ministry of Health and Welfare, Japan (Chairman Kikuchi H.). 1988:81 – 88.
.