Acute
Subdural
Hematoma
with
Moyamoya
in Young
Patient
Disease
Case Report— —
Shigekazu
TAKEUCHI, Ryuichi
Department
Akimichi
TANAKA
and
ICHIKAWA, Hiroyuki
Tetsuo
KOIKE,
ARAI*
of Neurosurgery, Brain Research Institute, Niigata University, Niigata, *Department of Neurosurgery , Kuwana Hospital, Niigata
Abstract
A 17-year-oldboy with known moyamoya diseasedevelopedan acute subdural hematoma after a mild head trauma. He had been confined to a wheelchairwith contracture in the upper and lower extremi ties due to juvenile rheumatoid arthritis since age 1 year. He had undergone encephalo-duro-arterio synangiosis(EDAS)on the right and encephalo-myo-synangiosis(EMS)on the left at 13years of age. He was admitted with headache, nausea, and vomiting after a fall from his wheelchair at age 17. Computed tomography on admission showed a large acute subdural hematoma in the right fronto temporal region but no bleeding at the EDAS or EMS sites. Cerebral angiography 12 weeksafter the head trauma revealed a remarkable reduction in the spontaneous transdural external-internalcarotid anastomoses in the right frontal region. The acute subdural hematoma was probably caused by rup ture of the spontaneous transdural anastomoses. Key words: moyamoya disease, subdural hematoma, head injury, encephalo-duro-arterio-synangiosis, encephalo-myo-synangiosis, anastomoses
Introduction
Case
Moyamoya disease is characterized angiographically by progressive occlusion of cerebral arteries, abnor mal vascular networks in the basal ganglia, leptome ningeal anastomoses, and multiple transdural exter nal-internal carotid anastomoses. Moyamoya dis ease usually manifests clinically as ischemic episodes in children and intracranial hemorrhage in adults. Modern computed tomographic (CT) scanning clear ly shows that most intracranial hemorrhages are in tracerebral or intraventricular.1) Despite the presence of multiple transdural arterial anastomoses, associ ated acute or subacute subdural hematoma rarely occurs. 2-1,1,12) Here, we describe a young adult with an acute subdural hematoma due to the rupture of spontaneous transdural external-internal carotid anastomoses after a mild head trauma.
Received 1991
April
3,
1991;
Accepted
September
2,
Report
This 17-year-old boy had suffered from a juvenile type of rheumatoid arthritis in the upper and lower extremities since 1 year old, requiring a wheelchair because of gradual contracture in the hip, knee, and ankle joints. On August 26, 1978 (at 9 years old), he suddenly developed clonic convulsion of the left upper extremity for over 15 minutes, followed by hemiparesis on the left, dysarthria, alexia, and dyscalculia. He was slowly recovering from these symptoms when he presented at the Department of Neurosurgery, Brain Research Institute, Niigata University on July 12, 1982. Neurological examina tion revealed mental deterioration, weakness of the left upper extremity, and contracture of the upper and lower extremities. A CT scan showed a large low-density lesion in the right frontal lobe. Cerebral angiograms demonstrated typical findings of moya moya disease, including moderate transdural exter nal-internal carotid anastomoses in the right frontal lobe.
Fig.
1
CT scans
on January
12, 1983,
showing
multiple
low-density
lesions.
On January 5, 1983 (at 13 years old), he was ad mitted to Niigata University Hospital for surgical management of the moyamoya disease. CT scans showed low-density lesions in the bilateral frontal lobes (Fig. 1). Encephalo-duro-arterio-synangiosis (EDAS)6) was performed on the right on January 14, and encephalo-myo-synangiosis (EMS)") on the left on January 27. EMS was the surgical treatment of choice for child patients with moyamoya disease at that time, but the right EDAS was performed to avoid injuring spontaneous transdural arterial anastomoses. General convulsion, transient pseudo bulbar paresis, and transient weakness of the right upper extremity occurred a few days postoperative ly. He became independent gradually, and attended a disabled children's school. Cerebral angiograms on August 5, 1983 showed slight revascularization of the brain via the right EDAS and marked revas cularization via the left EMS (Fig. 2). He received 1500 mg acetylsalicylic acid daily to treat the rheu matoid arthritis pre and postoperatively. He con tinued to be relatively well without convulsion or ischemic attack for 4 years. On February 3, 1987 (at 17 years old), he fell from a wheelchair and hit the left temporal region on the floor without loss of consciousness. He com plained of only slight left temporalgia. He fell again and hit the right frontotemporal region on the floor without loss of consciousness on February 5, 1987. He was admitted to the Department of Neurosur gery, Kuwana Hospital with complaints of head ache, nausea, and vomiting on the same day. Neuro logical examination revealed mental deterioration, contracture, and deformity in the upper and lower extremities which were present before the head trau ma, but no new deficits. Skull x-ray films showed no fracture except the operative craniotomy. CT
Fig. 2
left: Right external carotid angiogram, lateral projection, 6 months after EDAS, showing slight filling of the middle cerebral arteries via the EDAS in the parietal region, and spon taneous, multiple transdural external-internal carotid anastomoses in the frontal region. right: Left external carotid angiogram, lateral projection, 6 months after EMS, showing remarkable filling of the middle cerebral arteries via the EMS.
scans demonstrated a large acute subdural hema toma in the right frontotemporal region and a small cerebral contusion in the right temporal region with a slight mass effect (Fig. 3). There was no bleeding or cerebral contusion at the right EDAS or the left EMS sites. He was treated conservatively with symptomatic improvement, and was discharged on March 12, 1987. Cerebral angiograms on April 27, 1987 showed that revascularization via the operative sites was unchanged, but the spontaneous transdu ral arterial anastomoses in the right frontal region were remarkably reduced compared with the angio grams on August 5, 1983 (Fig. 4).
Fig. 3
CT
scans on February
5, 1987,
showing
a large
acute
subdural
poral region and a small cerebral contusion in the right temporal cerebral contusion at the right EDAS or left EMS sites.
Fig. 4
Right external carotid angiogram, lateral pro jection, 12 weeks after the head trauma, show ing markedly decreased transdural arterial anastomoses in the frontal region.
Discussion Children with moyamoya disease usually develop spontaneous transdural external-internal carotid anastomoses. Acute or subacute subdural hematoma has occurred in a few adults with this disease.2.3,5,'.'2) However, only one child patient with acute subdural hematoma has been reported.41 Taveras12 reported an adult with a subdural hematoma following ven triculography with considerable subdural air. He suspected that separation of the brain caused by the subdural air resulted in rupture of the transdural arterial channels. Mori et al.') described an adult case of moyamoya syndrome with acute subdural and in tracerebral hematomas from different bleeding
hematoma region.
in the right There
frontotem
is no bleeding
or
sources. They thought that distortion of the brain by the intracerebral hematoma caused rupture of an aneurysmal dilatation in a transdural anastomo sis. These authors emphasized the importance of shearing of enlarged transdural arterial channels as a significant source for subdural bleeding. In our case, there was a low-density lesion in the right frontal lobe, where the transdural anastomoses were located. Probably, the transdural anastomoses at the old cerebral infarction site were easily injured by a shearing force even in mild head trauma because of the brain atrophy. Nakagawa et al.') and Sonobe et al. 10)reported juvenile cases of chronic subdural hematoma developing after EMS for moyamoya disease. They pointed out that postoperative administration of acetylsalicylic acid was a factor in the development of the chronic subdural hematoma. In our case, acetylsalicylic acid was administered for rheumatoid arthritis. The maximum rate of secondary platelet aggregation with addition of 10,uM adenosine diphosphate and 2 Mg collagen/ml of plasma was 61 and 23%, respectively, using platelet-rich plasma with 300 x 104 platelets on admission to Kuwana Hospital. Although other coagulation tests were normal on admission, this reduction in platelet ag gregation may have been a factor enlarging the acute subdural hematoma after the head trauma. Sayama et al.') reported the autopsy of a 16-year old boy with moyamoya disease who received an ex ternal carotid-internal carotid bypass and EMS, and died of a severe cerebral contusion and an acute sub dural hematoma caused by a traffic accident. The traumatic damage and hemorrhage were most drastic in the area corresponding to the EMS. In our case, there was no bleeding or cerebral contusion at the
EMS and EDAS sites. Impact is unlikely to damage indirect revascularization in moyamoya disease such as EMS and EDAS easily, because the muscle and the galea spontaneously attach to the brain surface postoperatively. The spontaneous transdural exter nal-internal carotid anastomoses are more likely to be injured.
2)
3)
4)
5)
6)
Aoki N, Mizutani H: Does moyamoya disease cause subarachnoid hemorrhage? Review of 54 cases with intracranial hemorrhage confirmed by computerized tomography. J Neurosurg 60: 348-353, 1984 Handa H, Tani E, Kajikawa H, Sato K, Yamashita J, Osaka K, Haebara H, Tamegai M, Kyogoku M: Clinicopathological study on an adult case with cerebral arterial rete. No To Shinkei 21: 181-191, 1969 (in Japanese) Kawakami K, Takahashi S, Sonobe M, Koshu K, Hirota S, Kusunose M: Subacute subdural hematoma associated with moyamoya phenomenon. A case report. No Shinkei Geka 16: 205-209, 1988 (in Japanese) Maki Y, Nakada Y: Autopsy case of hemangio matous malformation of bilateral internal carotid artery at base of brain. No To Shinkei 17: 764-766, 1965 (in Japanese)
Matsumura M, Nojiri K: Resolved chronic subdural hematoma associated with acute subdural hematoma in moyamoya disease. Case report. Neurol Med Chir (Tokyo) 24: 622-627, 1984 (in Japanese) Matsushima
Y, Fukai
N, Tanaka
K, Tsuruoka
S,
Y, Aoyagi
ment
of moyamoya
nary
7)
8)
References 1)
Inaba
9)
10)
11)
12)
report.
Surg
M, Ohno
K: A new
disease Neurol
surgical
in children:
15: 313-320,
treat
A prelimi 1981
Mori K, Miyazaki H, Yasunaga A, Tanaka K, Ono H: A case of moyamoya syndrome with subdural and intracerebral hematoma due to different bleeding sources. Acta Med Nagasaki 24: 48-54, 1979 Nakagawa Y, Shimoyama M, Kashiwaba T, Suzuki Y, Gotoh S, Miyasaka K, Takei H, Gotoh S, Ohtsuka K, Abe H, Tsuru M: Reconstructive opera tion of moyamoya disease and its problems. No Shinkei Geka 9: 305-314, 1981 (in Japanese) Sayama I, Fukasawa H, Yasui N, Suzuki A: Child with moyamoya disease after bypass surgery. Report of an autopsy case. Neurol Med Chir (Tokyo) 25: 975-980, 1985 (in Japanese) Sonobe M, Takahashi S, Kubota Y, Shirane R: Chronic subdural hematoma developing after EMS for moyamoya disease. No Shinkei Geka 10: 857 859, 1982 (in Japanese) Takeuchi S, Tsuchida T, Kobayashi K, Fukuda M, Ishii R, Tanaka R, Ito J: Treatment of moyamoya disease by temporal muscle graft ‘encephalo-myo synangiosis.’ Childs Brain 10: - 1-15, 1983 Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults. Amer J Roentgen 106: 235-268, 1969
Address
reprint
ment
of
Niigata 951,
requests
to:
S. Takeuchi,
Neurosurgery, University,
Japan.
1-757
Brain
M.D.,
Research
Asahimachi-dori,
Depart Institute, Niigata
Subdural
Hematoma
with
Moyamoya
in Young
Patient
Disease
Case Report— —
Shigekazu
TAKEUCHI, Ryuichi
Department
Akimichi
TANAKA
and
ICHIKAWA, Hiroyuki
Tetsuo
KOIKE,
ARAI*
of Neurosurgery, Brain Research Institute, Niigata University, Niigata, *Department of Neurosurgery , Kuwana Hospital, Niigata
Abstract
A 17-year-oldboy with known moyamoya diseasedevelopedan acute subdural hematoma after a mild head trauma. He had been confined to a wheelchairwith contracture in the upper and lower extremi ties due to juvenile rheumatoid arthritis since age 1 year. He had undergone encephalo-duro-arterio synangiosis(EDAS)on the right and encephalo-myo-synangiosis(EMS)on the left at 13years of age. He was admitted with headache, nausea, and vomiting after a fall from his wheelchair at age 17. Computed tomography on admission showed a large acute subdural hematoma in the right fronto temporal region but no bleeding at the EDAS or EMS sites. Cerebral angiography 12 weeksafter the head trauma revealed a remarkable reduction in the spontaneous transdural external-internalcarotid anastomoses in the right frontal region. The acute subdural hematoma was probably caused by rup ture of the spontaneous transdural anastomoses. Key words: moyamoya disease, subdural hematoma, head injury, encephalo-duro-arterio-synangiosis, encephalo-myo-synangiosis, anastomoses
Introduction
Case
Moyamoya disease is characterized angiographically by progressive occlusion of cerebral arteries, abnor mal vascular networks in the basal ganglia, leptome ningeal anastomoses, and multiple transdural exter nal-internal carotid anastomoses. Moyamoya dis ease usually manifests clinically as ischemic episodes in children and intracranial hemorrhage in adults. Modern computed tomographic (CT) scanning clear ly shows that most intracranial hemorrhages are in tracerebral or intraventricular.1) Despite the presence of multiple transdural arterial anastomoses, associ ated acute or subacute subdural hematoma rarely occurs. 2-1,1,12) Here, we describe a young adult with an acute subdural hematoma due to the rupture of spontaneous transdural external-internal carotid anastomoses after a mild head trauma.
Received 1991
April
3,
1991;
Accepted
September
2,
Report
This 17-year-old boy had suffered from a juvenile type of rheumatoid arthritis in the upper and lower extremities since 1 year old, requiring a wheelchair because of gradual contracture in the hip, knee, and ankle joints. On August 26, 1978 (at 9 years old), he suddenly developed clonic convulsion of the left upper extremity for over 15 minutes, followed by hemiparesis on the left, dysarthria, alexia, and dyscalculia. He was slowly recovering from these symptoms when he presented at the Department of Neurosurgery, Brain Research Institute, Niigata University on July 12, 1982. Neurological examina tion revealed mental deterioration, weakness of the left upper extremity, and contracture of the upper and lower extremities. A CT scan showed a large low-density lesion in the right frontal lobe. Cerebral angiograms demonstrated typical findings of moya moya disease, including moderate transdural exter nal-internal carotid anastomoses in the right frontal lobe.
Fig.
1
CT scans
on January
12, 1983,
showing
multiple
low-density
lesions.
On January 5, 1983 (at 13 years old), he was ad mitted to Niigata University Hospital for surgical management of the moyamoya disease. CT scans showed low-density lesions in the bilateral frontal lobes (Fig. 1). Encephalo-duro-arterio-synangiosis (EDAS)6) was performed on the right on January 14, and encephalo-myo-synangiosis (EMS)") on the left on January 27. EMS was the surgical treatment of choice for child patients with moyamoya disease at that time, but the right EDAS was performed to avoid injuring spontaneous transdural arterial anastomoses. General convulsion, transient pseudo bulbar paresis, and transient weakness of the right upper extremity occurred a few days postoperative ly. He became independent gradually, and attended a disabled children's school. Cerebral angiograms on August 5, 1983 showed slight revascularization of the brain via the right EDAS and marked revas cularization via the left EMS (Fig. 2). He received 1500 mg acetylsalicylic acid daily to treat the rheu matoid arthritis pre and postoperatively. He con tinued to be relatively well without convulsion or ischemic attack for 4 years. On February 3, 1987 (at 17 years old), he fell from a wheelchair and hit the left temporal region on the floor without loss of consciousness. He com plained of only slight left temporalgia. He fell again and hit the right frontotemporal region on the floor without loss of consciousness on February 5, 1987. He was admitted to the Department of Neurosur gery, Kuwana Hospital with complaints of head ache, nausea, and vomiting on the same day. Neuro logical examination revealed mental deterioration, contracture, and deformity in the upper and lower extremities which were present before the head trau ma, but no new deficits. Skull x-ray films showed no fracture except the operative craniotomy. CT
Fig. 2
left: Right external carotid angiogram, lateral projection, 6 months after EDAS, showing slight filling of the middle cerebral arteries via the EDAS in the parietal region, and spon taneous, multiple transdural external-internal carotid anastomoses in the frontal region. right: Left external carotid angiogram, lateral projection, 6 months after EMS, showing remarkable filling of the middle cerebral arteries via the EMS.
scans demonstrated a large acute subdural hema toma in the right frontotemporal region and a small cerebral contusion in the right temporal region with a slight mass effect (Fig. 3). There was no bleeding or cerebral contusion at the right EDAS or the left EMS sites. He was treated conservatively with symptomatic improvement, and was discharged on March 12, 1987. Cerebral angiograms on April 27, 1987 showed that revascularization via the operative sites was unchanged, but the spontaneous transdu ral arterial anastomoses in the right frontal region were remarkably reduced compared with the angio grams on August 5, 1983 (Fig. 4).
Fig. 3
CT
scans on February
5, 1987,
showing
a large
acute
subdural
poral region and a small cerebral contusion in the right temporal cerebral contusion at the right EDAS or left EMS sites.
Fig. 4
Right external carotid angiogram, lateral pro jection, 12 weeks after the head trauma, show ing markedly decreased transdural arterial anastomoses in the frontal region.
Discussion Children with moyamoya disease usually develop spontaneous transdural external-internal carotid anastomoses. Acute or subacute subdural hematoma has occurred in a few adults with this disease.2.3,5,'.'2) However, only one child patient with acute subdural hematoma has been reported.41 Taveras12 reported an adult with a subdural hematoma following ven triculography with considerable subdural air. He suspected that separation of the brain caused by the subdural air resulted in rupture of the transdural arterial channels. Mori et al.') described an adult case of moyamoya syndrome with acute subdural and in tracerebral hematomas from different bleeding
hematoma region.
in the right There
frontotem
is no bleeding
or
sources. They thought that distortion of the brain by the intracerebral hematoma caused rupture of an aneurysmal dilatation in a transdural anastomo sis. These authors emphasized the importance of shearing of enlarged transdural arterial channels as a significant source for subdural bleeding. In our case, there was a low-density lesion in the right frontal lobe, where the transdural anastomoses were located. Probably, the transdural anastomoses at the old cerebral infarction site were easily injured by a shearing force even in mild head trauma because of the brain atrophy. Nakagawa et al.') and Sonobe et al. 10)reported juvenile cases of chronic subdural hematoma developing after EMS for moyamoya disease. They pointed out that postoperative administration of acetylsalicylic acid was a factor in the development of the chronic subdural hematoma. In our case, acetylsalicylic acid was administered for rheumatoid arthritis. The maximum rate of secondary platelet aggregation with addition of 10,uM adenosine diphosphate and 2 Mg collagen/ml of plasma was 61 and 23%, respectively, using platelet-rich plasma with 300 x 104 platelets on admission to Kuwana Hospital. Although other coagulation tests were normal on admission, this reduction in platelet ag gregation may have been a factor enlarging the acute subdural hematoma after the head trauma. Sayama et al.') reported the autopsy of a 16-year old boy with moyamoya disease who received an ex ternal carotid-internal carotid bypass and EMS, and died of a severe cerebral contusion and an acute sub dural hematoma caused by a traffic accident. The traumatic damage and hemorrhage were most drastic in the area corresponding to the EMS. In our case, there was no bleeding or cerebral contusion at the
EMS and EDAS sites. Impact is unlikely to damage indirect revascularization in moyamoya disease such as EMS and EDAS easily, because the muscle and the galea spontaneously attach to the brain surface postoperatively. The spontaneous transdural exter nal-internal carotid anastomoses are more likely to be injured.
2)
3)
4)
5)
6)
Aoki N, Mizutani H: Does moyamoya disease cause subarachnoid hemorrhage? Review of 54 cases with intracranial hemorrhage confirmed by computerized tomography. J Neurosurg 60: 348-353, 1984 Handa H, Tani E, Kajikawa H, Sato K, Yamashita J, Osaka K, Haebara H, Tamegai M, Kyogoku M: Clinicopathological study on an adult case with cerebral arterial rete. No To Shinkei 21: 181-191, 1969 (in Japanese) Kawakami K, Takahashi S, Sonobe M, Koshu K, Hirota S, Kusunose M: Subacute subdural hematoma associated with moyamoya phenomenon. A case report. No Shinkei Geka 16: 205-209, 1988 (in Japanese) Maki Y, Nakada Y: Autopsy case of hemangio matous malformation of bilateral internal carotid artery at base of brain. No To Shinkei 17: 764-766, 1965 (in Japanese)
Matsumura M, Nojiri K: Resolved chronic subdural hematoma associated with acute subdural hematoma in moyamoya disease. Case report. Neurol Med Chir (Tokyo) 24: 622-627, 1984 (in Japanese) Matsushima
Y, Fukai
N, Tanaka
K, Tsuruoka
S,
Y, Aoyagi
ment
of moyamoya
nary
7)
8)
References 1)
Inaba
9)
10)
11)
12)
report.
Surg
M, Ohno
K: A new
disease Neurol
surgical
in children:
15: 313-320,
treat
A prelimi 1981
Mori K, Miyazaki H, Yasunaga A, Tanaka K, Ono H: A case of moyamoya syndrome with subdural and intracerebral hematoma due to different bleeding sources. Acta Med Nagasaki 24: 48-54, 1979 Nakagawa Y, Shimoyama M, Kashiwaba T, Suzuki Y, Gotoh S, Miyasaka K, Takei H, Gotoh S, Ohtsuka K, Abe H, Tsuru M: Reconstructive opera tion of moyamoya disease and its problems. No Shinkei Geka 9: 305-314, 1981 (in Japanese) Sayama I, Fukasawa H, Yasui N, Suzuki A: Child with moyamoya disease after bypass surgery. Report of an autopsy case. Neurol Med Chir (Tokyo) 25: 975-980, 1985 (in Japanese) Sonobe M, Takahashi S, Kubota Y, Shirane R: Chronic subdural hematoma developing after EMS for moyamoya disease. No Shinkei Geka 10: 857 859, 1982 (in Japanese) Takeuchi S, Tsuchida T, Kobayashi K, Fukuda M, Ishii R, Tanaka R, Ito J: Treatment of moyamoya disease by temporal muscle graft ‘encephalo-myo synangiosis.’ Childs Brain 10: - 1-15, 1983 Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults. Amer J Roentgen 106: 235-268, 1969
Address
reprint
ment
of
Niigata 951,
requests
to:
S. Takeuchi,
Neurosurgery, University,
Japan.
1-757
Brain
M.D.,
Research
Asahimachi-dori,
Depart Institute, Niigata
