Ruptured
Cerebral
Aneurysm
with Coarctation
Associated
of the Aorta
Report of Two Cases— — Toru
SERIZAWA, Masaru
Akira
ODAKI,
Department
SATOH,
Hiroshi
Akihiro
MIYATA,
NAKAMURA
of Neurosurgery,
and
Shigeki
Yoshiroh
Chiba Emergency
KOBAYASHI,
WATANABE
Medical Center,
Chiba
Abstract Two
cases
of ruptured
cerebral
aneurysms
were successfully
aneurysm
should
before
aortic
Key words: surgical
be treated
repair,
aneurysm
clipped as early
if the general
ruptured
cerebral
associated
in the acute as possible,
condition
aneurysm,
with coarctation
stage and
prior
unruptured
of the patient coarctation
of the aorta
to correction
are presented.
of the coarctation.
aneurysm
should
The
Ruptured
also
be treated
allows.
of the aorta,
multiple
aneurysms,
indication
Introduction
Table
1
Incidence with
of
cerebral
aortic
coarctation
associated
aneurysm
Cerebral aneurysm is associated with coarctation of the aorta in 2-10% of cases, 2.7% of which die from aneurysmal rupture.') However, the incidence of coarctation of the aorta among cerebral aneurysm patients is very low, ranging from 0.19 to 1.9% (mean, 0.45%) and in our institute 0.22% (Table 1) 3,4,9,11-14) Here, we present two cases of ruptured cerebral aneurysm with coarctation of the aorta, in which the ruptured cerebral aneurysms were successfully clip ped during the acute phase of subarachnoid hemor rhage. The clinical features, and timing and order of surgical intervention for the ruptured cerebral aneurysm and coarctation of the aorta in addition to accompanying unruptured cerebral aneurysm are dis cussed. Case
Reports
left paresthesia 1 year before. Intravenous digital sub traction angiograms at a local hospital had revealed a right middle cerebral artery (MCA) aneurysm, but she refused surgery. On admission to our hospital, she was semicomatose and exhibited left decerebrated posture (Hunt & Kosnik grade IV). The right pupil was dilated and fixed to light while the left was nor
Case 1: A 33-year-old female presented on August 15, 1989 with consciousness disturbance. She had a history of hypertension due to coarctation of the aor ta 2 years before, and sudden severe headache and Received Author's
April present
30,
1991;
address:
Accepted T. Serizawa, Japan.
December M.D.,
3, 1991
Department
of Neurosurgery
, Chiba
University
School
of Medicine,
Chiba,
mal. Blood pressure was 270/160 mmHg, with no discrepancy between the right and the left upper ex tremities. The bilateral femoral pulses were very fee ble and delayed. A chest x-ray film demonstrated no obvious rib notch. Computed tomographic (CT) scans demonstrated subarachnoid hemorrhage with right Sylvian hematoma (Fig. 1). Right direct carotid angiograms showed the large right MCA aneurysm and two right distal anterior cerebral artery (ACA) aneurysms (Fig. 2 left). Nine hours after onset, a right decompressive frontotemporal craniectomy and a neck clipping of the MCA aneurysm were per formed. Two weeks later, she became stuporous with left hemiparesis. Postoperative angiograms by Seldinger's method via the right brachial artery on September 24 visualized only the right carotid, right vertebral, and aortic arteries due to severe elongation
Fig. 2
Case 1. left: Right carotid angiogram, reveal ing a large right MCA aneurysm (arrows) and two right distal ACA aneurysms (arrowheads). right: Postoperative left carotid angiogram, revealing a left IC-PCoA aneurysm and a left distal ACA aneurysm (arrowheads).
Fig. 3
Case 1. Aortograms via the right brachial artery 40 days after onset, demonstrating the long stenosis from the descending aorta to the abdominal aorta (arrow), inoperable because of extension below the renal arteries.
(Fig. 3). Direct left carotid angiograms demonstrated a left internal carotid-posterior communicating artery (IC-PCoA) aneurysm and a distal left ACA aneurysm (Fig. 2 right). The left IC-PCoA aneurysm and the three distal ACA aneurysms were clipped and cranioplasty carried out on October 4. Her postoperative course was uneventful sient ventricular dilatation.
Fig.
1
Case
1.
CT
subarachnoid hematoma gesting
scans
on
admission,
hemorrhage with
rupture
severe of right
except for tran
and midline MCA
showing
intra-Sylvian shift,
aneurysm.
sug
She was transferred for cardiovascular treatment with mild confusion and left hemiparesis on November 30. However, the aortic coarctation could not be corrected because of extension beyond the renal artery. She is now at home with mild left hemiparesis. Case 2: A 15-year-old boy was admitted on December 10, 1990 with sudden severe headache. A chest x-ray film had disclosed cardiomegaly when he was 9 years old. Sudden onset of severe headache had occurred at 12 years old. On admission, his blood pressure was 130/90 mmHg in the bilateral upper extremities. The bilateral femoral pulses were relatively weak. His con sciousness was drowsy and confused (Hunt & Kosnik grade III), and mild left hemiparesis was present. A chest x-ray film did not demonstrate the rib notch.
CT scans demonstrated
subarachnoid
hemorrhage,
predominantly in the interhemispheric fissure and basal cistern, intraventricular hemorrhage in all ven tricles, and intracerebral hematoma in the right fron tal lobe (Fig. 4). Right carotid angiograms showed a large anterior communicating artery (ACoA) aneu rysm (Fig. 5). Eight hours later, a right decompres sive frontotemporal craniectomy and aneurysmal neck clipping were performed under hypothermic anesthesia. Two weeks later, he became confused and disinhibited. On January 18, 1991, cerebral angio grams by the Seldinger's method via the right femoral artery encountered some difficulties due to aortic elongation and stenosis, and demonstrated no residual aneurysm. Aortograms at the same time demonstrated moderate coarctation of the descend ing aorta (Fig. 6). The pressure gradient was 60 mmHg. Cranioplasty was performed on January 21. He was discharged without neurological deficits on January 30. The coarctation was repaired 6 months later.
Fig. 5
Case 2. Right carotid angiogram via the right femoral artery, revealing a large ACoA aneurysm (arrow).
Fig. 6
Case 2. Aortogram via the right artery, demonstrating coarctation descending aorta (arrow).
Discussion Ruptured
cerebral
aneurysm
associated
with coarcta
femoral of the
tion of the aorta has three characteristic clinical features. 1,2,4,1,13,14) First, aneurysmal rupture with co arctation occurs much earlier in life (mean, 25 yrs) than without coarctation (mean, 50-54 yrs).2) The incidence of bleeding from cerebral aneurysm in young patients with coarctation ranges from 12 to 23%,6 8) suggesting that coarctation of the aorta is a very important cause of subarachnoid hemorrhage in youth. Second, the incidence of multiple aneurysms is higher (30%) than in the absence of coarctation
Fig. 4
Case 2. CT scans marked subarachnoid
on admission, hemorrhage
terhemispheric
and
bral hematoma intraventricular
in the right hemorrhage
suggesting
rupture
basal
cisterns, frontal in all
of the ACoA
showing in the in intracere lobe, and ventricles,
aneurysm.
(20%), 12)requiring complete cerebral angiography. Brackett and Morants2) recommended angiography via the brachial artery for selective catheterization of cerebral arteries, and for identifying aortic lesions. However, the more rapid combination of left direct carotid and right retrograde brachial angiography is the first choice for preoperative examination in the acute phase, because selective catheterization via the
brachial artery might be very difficult and that via the femoral artery often impossible to pass through the aortic stenosis. Finally, the mortality due to ruptured cerebral aneurysm associated with coarctation is much higher (50-75%), mainly because of rebleeding from the cerebral aneurysm.',") Sedzimir et al. ") and Schwartz and Baronofsky10) treated the coarctation before the ruptured aneurysm. This order, however, has a high risk of perioperative rebleeding from the cerebral aneurysm and aggressive induced hypertensive therapy against vasospasm is unsafe. Recent ad vances in anesthesia, perioperative management, and neurosurgical techniques allow surgical treatment of the ruptured cerebral aneurysm in the acute phase in the presence of untreated coarctation with relative safety. Therefore, the ruptured aneurysm should be treated as early as possible before correcting the co arctation. Generally, unruptured aneurysms should also be treated before aortic repair. However, if heart failure is apparent or clipping of the unrup tured aneurysm is difficult, the coarctation should be repaired first. Early surgery for ruptured aneurysm requires several special postoperative precautions. A most im portant precaution is to maintain the blood pressure high enough to prevent ischemic complications due to vasospasm, which can worsen the prognosis after clipping of ruptured cerebral aneurysm even in young patients like ours. However, induced hyper tension might cause heart failure, cerebral swelling, and rupture of accompanying unruptured aneurysm. Cardiac function was evaluated with pulmonary capillary wedge pressure in Case 1 and central ve nous pressure in Case 2. External decompression and ventricular drainage were very effective for controlling intracranial pressure during induced hy pertension therapy in both cases, although Case 1 required hyperosmolar agents for a short time post operatively. Blood pressure was easily controlled in our cases by continuous intravenous administration of low doses of nicardipine or dopamine. When the blood pressure must be kept low, care should be to prevent ischemia of the kidney or the spinal cord. 2,4,5,7,9-11,14) The urinary output and blood pres sure or at least the arterial pulsation and motor function of the lower extremities must be checked every hour. Both our cases had hematoma, in the Sylvian fissure in Case 1, and in the right frontal lobe in Case 2. Such intracerebral hematoma due to ruptured cerebral aneurysm with coarctation has not been previously described. These hematomas might be in cidental or due to hypertension caused by coarcta
tion or subarachnoid adhesions minor bleeding from the cerebral
caused by previous aneurysm. This sug
gests that the intracerebral hematoma with coarcta tion previously reported' 6) might have been caused by cerebral aneurysmal rupture.
References 1)
2)
3)
4)
5)
6) 7)
8)
9)
10)
11)
12) 13) 14)
Abbott ME: Coarctation of the aorta of adult type II. A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or oblitera tion of the descending arch in subjects above the age of two years. Amer Heart J 3: 574-618, 1928 Brackett CE, Morants RA: Special problems associ ated with subarachnoid hemorrhage. No Shinkei Geka 2: 1807-1808, 1973 (in Japanese) DuBoulay GH: Some observation on the natural history of intracranial aneurysms. Brit J Radiol 38: 721-757, 1965 Fukuda H, Sako K, Yonemasu Y: Coarctation of the descending aorta with aneurysm of the anterior com municating artery. Surg Neurol 23: 382-392, 1985 Hino H, Maruyama H, Inomata H: A case of spinal artery presenting transverse myelopathy associated with coarctation of the aorta. Clin Neurol 29: 1009 1012, 1989 Matoson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23: 575-583, 1965 Ohta K, Iwasa H, Kurokawa N, Oguro K, Masuzawa T: Ruptured aneurysms associated with pulseless diseases. Report of three cases. Surgery for Cerebral Stroke 16: 68-71, 1988 (in Japanese) Patel AN, Richardson AE: Ruptured intracranial aneurysms in the first two decades of life. A study of 58 patients. J Neurosurg 35: 571-576, 1971 Robinson RG: Coarctation of the aorta and cerebral aneurysms. Report of two cases. J Neurosurg 26: 527-531, 1967 Schwartz MJ, Baronofsky ID: Ruptured intracranial aneurysm associated with coarctation of the aorta. Amer J Cardiol 6: 982-988, 1960 Sedzimir CB, Jones EW, Hamilton D, Edowards R: Management of coarctation of aorta and bleeding in tracranial aneurysm in pediatric cases. Neuro paediatrie 4: 124-133, 1973 Stehbens WE: Cerebral aneurysms and congenital ab normalities. Aust Ann Med 11: 102-112, 1962 Walton JN: Subarachnoid Haemorrhage. Edin burgh, E & S Livingstone, 1956, 350 pp Yokota A, Kodama T, Matsukado Y: Cerebral aneurysm associated with coarctation of the aorta. No To Shinkei 29: 221-226, 1977 (in Japanese)
Address reprint requests to: T. Serizawa, M.D., Depart ment of Neurosurgery, Chiba University School of Medicine, Japan.
1-8-1
Inohana,
Chuo-ku,
Chiba
260,
Cerebral
Aneurysm
with Coarctation
Associated
of the Aorta
Report of Two Cases— — Toru
SERIZAWA, Masaru
Akira
ODAKI,
Department
SATOH,
Hiroshi
Akihiro
MIYATA,
NAKAMURA
of Neurosurgery,
and
Shigeki
Yoshiroh
Chiba Emergency
KOBAYASHI,
WATANABE
Medical Center,
Chiba
Abstract Two
cases
of ruptured
cerebral
aneurysms
were successfully
aneurysm
should
before
aortic
Key words: surgical
be treated
repair,
aneurysm
clipped as early
if the general
ruptured
cerebral
associated
in the acute as possible,
condition
aneurysm,
with coarctation
stage and
prior
unruptured
of the patient coarctation
of the aorta
to correction
are presented.
of the coarctation.
aneurysm
should
The
Ruptured
also
be treated
allows.
of the aorta,
multiple
aneurysms,
indication
Introduction
Table
1
Incidence with
of
cerebral
aortic
coarctation
associated
aneurysm
Cerebral aneurysm is associated with coarctation of the aorta in 2-10% of cases, 2.7% of which die from aneurysmal rupture.') However, the incidence of coarctation of the aorta among cerebral aneurysm patients is very low, ranging from 0.19 to 1.9% (mean, 0.45%) and in our institute 0.22% (Table 1) 3,4,9,11-14) Here, we present two cases of ruptured cerebral aneurysm with coarctation of the aorta, in which the ruptured cerebral aneurysms were successfully clip ped during the acute phase of subarachnoid hemor rhage. The clinical features, and timing and order of surgical intervention for the ruptured cerebral aneurysm and coarctation of the aorta in addition to accompanying unruptured cerebral aneurysm are dis cussed. Case
Reports
left paresthesia 1 year before. Intravenous digital sub traction angiograms at a local hospital had revealed a right middle cerebral artery (MCA) aneurysm, but she refused surgery. On admission to our hospital, she was semicomatose and exhibited left decerebrated posture (Hunt & Kosnik grade IV). The right pupil was dilated and fixed to light while the left was nor
Case 1: A 33-year-old female presented on August 15, 1989 with consciousness disturbance. She had a history of hypertension due to coarctation of the aor ta 2 years before, and sudden severe headache and Received Author's
April present
30,
1991;
address:
Accepted T. Serizawa, Japan.
December M.D.,
3, 1991
Department
of Neurosurgery
, Chiba
University
School
of Medicine,
Chiba,
mal. Blood pressure was 270/160 mmHg, with no discrepancy between the right and the left upper ex tremities. The bilateral femoral pulses were very fee ble and delayed. A chest x-ray film demonstrated no obvious rib notch. Computed tomographic (CT) scans demonstrated subarachnoid hemorrhage with right Sylvian hematoma (Fig. 1). Right direct carotid angiograms showed the large right MCA aneurysm and two right distal anterior cerebral artery (ACA) aneurysms (Fig. 2 left). Nine hours after onset, a right decompressive frontotemporal craniectomy and a neck clipping of the MCA aneurysm were per formed. Two weeks later, she became stuporous with left hemiparesis. Postoperative angiograms by Seldinger's method via the right brachial artery on September 24 visualized only the right carotid, right vertebral, and aortic arteries due to severe elongation
Fig. 2
Case 1. left: Right carotid angiogram, reveal ing a large right MCA aneurysm (arrows) and two right distal ACA aneurysms (arrowheads). right: Postoperative left carotid angiogram, revealing a left IC-PCoA aneurysm and a left distal ACA aneurysm (arrowheads).
Fig. 3
Case 1. Aortograms via the right brachial artery 40 days after onset, demonstrating the long stenosis from the descending aorta to the abdominal aorta (arrow), inoperable because of extension below the renal arteries.
(Fig. 3). Direct left carotid angiograms demonstrated a left internal carotid-posterior communicating artery (IC-PCoA) aneurysm and a distal left ACA aneurysm (Fig. 2 right). The left IC-PCoA aneurysm and the three distal ACA aneurysms were clipped and cranioplasty carried out on October 4. Her postoperative course was uneventful sient ventricular dilatation.
Fig.
1
Case
1.
CT
subarachnoid hematoma gesting
scans
on
admission,
hemorrhage with
rupture
severe of right
except for tran
and midline MCA
showing
intra-Sylvian shift,
aneurysm.
sug
She was transferred for cardiovascular treatment with mild confusion and left hemiparesis on November 30. However, the aortic coarctation could not be corrected because of extension beyond the renal artery. She is now at home with mild left hemiparesis. Case 2: A 15-year-old boy was admitted on December 10, 1990 with sudden severe headache. A chest x-ray film had disclosed cardiomegaly when he was 9 years old. Sudden onset of severe headache had occurred at 12 years old. On admission, his blood pressure was 130/90 mmHg in the bilateral upper extremities. The bilateral femoral pulses were relatively weak. His con sciousness was drowsy and confused (Hunt & Kosnik grade III), and mild left hemiparesis was present. A chest x-ray film did not demonstrate the rib notch.
CT scans demonstrated
subarachnoid
hemorrhage,
predominantly in the interhemispheric fissure and basal cistern, intraventricular hemorrhage in all ven tricles, and intracerebral hematoma in the right fron tal lobe (Fig. 4). Right carotid angiograms showed a large anterior communicating artery (ACoA) aneu rysm (Fig. 5). Eight hours later, a right decompres sive frontotemporal craniectomy and aneurysmal neck clipping were performed under hypothermic anesthesia. Two weeks later, he became confused and disinhibited. On January 18, 1991, cerebral angio grams by the Seldinger's method via the right femoral artery encountered some difficulties due to aortic elongation and stenosis, and demonstrated no residual aneurysm. Aortograms at the same time demonstrated moderate coarctation of the descend ing aorta (Fig. 6). The pressure gradient was 60 mmHg. Cranioplasty was performed on January 21. He was discharged without neurological deficits on January 30. The coarctation was repaired 6 months later.
Fig. 5
Case 2. Right carotid angiogram via the right femoral artery, revealing a large ACoA aneurysm (arrow).
Fig. 6
Case 2. Aortogram via the right artery, demonstrating coarctation descending aorta (arrow).
Discussion Ruptured
cerebral
aneurysm
associated
with coarcta
femoral of the
tion of the aorta has three characteristic clinical features. 1,2,4,1,13,14) First, aneurysmal rupture with co arctation occurs much earlier in life (mean, 25 yrs) than without coarctation (mean, 50-54 yrs).2) The incidence of bleeding from cerebral aneurysm in young patients with coarctation ranges from 12 to 23%,6 8) suggesting that coarctation of the aorta is a very important cause of subarachnoid hemorrhage in youth. Second, the incidence of multiple aneurysms is higher (30%) than in the absence of coarctation
Fig. 4
Case 2. CT scans marked subarachnoid
on admission, hemorrhage
terhemispheric
and
bral hematoma intraventricular
in the right hemorrhage
suggesting
rupture
basal
cisterns, frontal in all
of the ACoA
showing in the in intracere lobe, and ventricles,
aneurysm.
(20%), 12)requiring complete cerebral angiography. Brackett and Morants2) recommended angiography via the brachial artery for selective catheterization of cerebral arteries, and for identifying aortic lesions. However, the more rapid combination of left direct carotid and right retrograde brachial angiography is the first choice for preoperative examination in the acute phase, because selective catheterization via the
brachial artery might be very difficult and that via the femoral artery often impossible to pass through the aortic stenosis. Finally, the mortality due to ruptured cerebral aneurysm associated with coarctation is much higher (50-75%), mainly because of rebleeding from the cerebral aneurysm.',") Sedzimir et al. ") and Schwartz and Baronofsky10) treated the coarctation before the ruptured aneurysm. This order, however, has a high risk of perioperative rebleeding from the cerebral aneurysm and aggressive induced hypertensive therapy against vasospasm is unsafe. Recent ad vances in anesthesia, perioperative management, and neurosurgical techniques allow surgical treatment of the ruptured cerebral aneurysm in the acute phase in the presence of untreated coarctation with relative safety. Therefore, the ruptured aneurysm should be treated as early as possible before correcting the co arctation. Generally, unruptured aneurysms should also be treated before aortic repair. However, if heart failure is apparent or clipping of the unrup tured aneurysm is difficult, the coarctation should be repaired first. Early surgery for ruptured aneurysm requires several special postoperative precautions. A most im portant precaution is to maintain the blood pressure high enough to prevent ischemic complications due to vasospasm, which can worsen the prognosis after clipping of ruptured cerebral aneurysm even in young patients like ours. However, induced hyper tension might cause heart failure, cerebral swelling, and rupture of accompanying unruptured aneurysm. Cardiac function was evaluated with pulmonary capillary wedge pressure in Case 1 and central ve nous pressure in Case 2. External decompression and ventricular drainage were very effective for controlling intracranial pressure during induced hy pertension therapy in both cases, although Case 1 required hyperosmolar agents for a short time post operatively. Blood pressure was easily controlled in our cases by continuous intravenous administration of low doses of nicardipine or dopamine. When the blood pressure must be kept low, care should be to prevent ischemia of the kidney or the spinal cord. 2,4,5,7,9-11,14) The urinary output and blood pres sure or at least the arterial pulsation and motor function of the lower extremities must be checked every hour. Both our cases had hematoma, in the Sylvian fissure in Case 1, and in the right frontal lobe in Case 2. Such intracerebral hematoma due to ruptured cerebral aneurysm with coarctation has not been previously described. These hematomas might be in cidental or due to hypertension caused by coarcta
tion or subarachnoid adhesions minor bleeding from the cerebral
caused by previous aneurysm. This sug
gests that the intracerebral hematoma with coarcta tion previously reported' 6) might have been caused by cerebral aneurysmal rupture.
References 1)
2)
3)
4)
5)
6) 7)
8)
9)
10)
11)
12) 13) 14)
Abbott ME: Coarctation of the aorta of adult type II. A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or oblitera tion of the descending arch in subjects above the age of two years. Amer Heart J 3: 574-618, 1928 Brackett CE, Morants RA: Special problems associ ated with subarachnoid hemorrhage. No Shinkei Geka 2: 1807-1808, 1973 (in Japanese) DuBoulay GH: Some observation on the natural history of intracranial aneurysms. Brit J Radiol 38: 721-757, 1965 Fukuda H, Sako K, Yonemasu Y: Coarctation of the descending aorta with aneurysm of the anterior com municating artery. Surg Neurol 23: 382-392, 1985 Hino H, Maruyama H, Inomata H: A case of spinal artery presenting transverse myelopathy associated with coarctation of the aorta. Clin Neurol 29: 1009 1012, 1989 Matoson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23: 575-583, 1965 Ohta K, Iwasa H, Kurokawa N, Oguro K, Masuzawa T: Ruptured aneurysms associated with pulseless diseases. Report of three cases. Surgery for Cerebral Stroke 16: 68-71, 1988 (in Japanese) Patel AN, Richardson AE: Ruptured intracranial aneurysms in the first two decades of life. A study of 58 patients. J Neurosurg 35: 571-576, 1971 Robinson RG: Coarctation of the aorta and cerebral aneurysms. Report of two cases. J Neurosurg 26: 527-531, 1967 Schwartz MJ, Baronofsky ID: Ruptured intracranial aneurysm associated with coarctation of the aorta. Amer J Cardiol 6: 982-988, 1960 Sedzimir CB, Jones EW, Hamilton D, Edowards R: Management of coarctation of aorta and bleeding in tracranial aneurysm in pediatric cases. Neuro paediatrie 4: 124-133, 1973 Stehbens WE: Cerebral aneurysms and congenital ab normalities. Aust Ann Med 11: 102-112, 1962 Walton JN: Subarachnoid Haemorrhage. Edin burgh, E & S Livingstone, 1956, 350 pp Yokota A, Kodama T, Matsukado Y: Cerebral aneurysm associated with coarctation of the aorta. No To Shinkei 29: 221-226, 1977 (in Japanese)
Address reprint requests to: T. Serizawa, M.D., Depart ment of Neurosurgery, Chiba University School of Medicine, Japan.
1-8-1
Inohana,
Chuo-ku,
Chiba
260,
