CONSENT
Written informed consent was obtained from the patient to publish this
report in accordance with the journal’s patient consent policy.
KEY CLINICAL MESSAGE
We should always consider the possibility of segmental arterial
mediolysis (SAM) in case of shock after subarachnoid hemorrhage (SAH).
In addition, SAH with cerebral vasospasm may be associated with SAM,
suggesting that intra-abdominal hemorrhage due to SAM may occur during
cerebral vasospasm.
ABSTRACT
A man in his 50s with no significant past medical history developed
subarachnoid hemorrhage due to ruptured left middle cerebral artery
aneurysm. 9th hospital day, he experienced a ruptured
visceral aneurysm with segmental arterial mediolysis, and we
successfully treated with transarterial embolization using metallic
coils.
KEYWORDS
segmental arterial mediolysis, subarachnoid hemorrhage,cerebral vasospasm, visceral artery aneurysm, intracranial aneurysm,
intraabdominal hemorrhage
1 INTRODUCTION
Segmental arterial mediolysis (SAM) is a noninflammatory,
nonatherosclerotic disease that causes segmental lysis of the outer
arterial media. 1 It can result in separation of the
media from the adventitia leading to dissecting
aneurysms.1 Most commonly,an aneurysm forms in the
large abdominal aortic branches, and its rupture causes intra-abdominal
hemorrhage. 2 The cerebral arteries can also be
involved, but SAM is not yet well known in the neurological field. In
rare cases, SAM has been reported to occur in conjunction with
subarachnoid hemorrhage (SAH). We report a case of intra-abdominal
bleeding due to SAM during the cerebral spasm after SAH, which was
successfully treated with coil embolization of an intraperitoneal
aneurysm.
2 CASE HISTORY
A 59-year-old man with no significant past medical history suddenly lost
consciousness after onset of a severe headache. He presented to the
emergency department at our hospital. On admission, his Glasgow Coma
Scale was E3V2M5. A clinical diagnosis of grade Ⅳ was made on the World
Federation of Neurological Surgeons [WFNS] scale. Computed
tomography (CT) scan showed SAH from the basal cistern to the left
Sylvian fissure (Fisher group 3). Three-dimensional CT angiography
revealed an aneurysm (6.7 mm x 6.2mm x 4.9 mm) with blebs was found in
the middle cerebral artery (M2) bifurcation of the left MCA. (Figure 1)
Non-contrast enhanced CT of the chest and abdomen for screening showed
no abnormalities. We diagnosed SAH
due to ruptured left MCA aneurysm and performed clipping for the
aneurysm on the same day. External
decompression was also added because of the elevated cerebral pressure.
Intraoperative findings indicated
that the aneurysm was a saccular appearance.
(Figure 2) To prevent cerebral
vasospasm, spinal drainage was started postoperatively and
administration of ozagrel sodium 80 mg/day and fasudil hydrochloride
hydrate 30 mg x 3/day was started.
8 days after onset, the cerebral
angiography showed narrowing at the left M2 due to cerebral vasospasm
and delayed blood flow in peripheral
vessels.
(Figure 3) Therefore, low-
molecular-weight dextran was added to maintain cerebral blood flow, and
blood pressure was maintained at a higher level. (Figure 4)
9th hospital day, when we performed tracheostomy for
respiratory management due to prolonged disturbance of consciousness, he
suddenly lapsed into hypovolemic shock state.
The head CT showed no new
intracranial hemorrhage or infarction. However, non-contrast and
contrast-enhanced CT of the chest and abdomen showed bloody ascites and
abnormal dilatation of the superior mesenteric artery and anterior
superior pancreatoduodenal artery (ASPDA), suspected intraperitoneal
hemorrhage from the same site.
(Figure 5)
We determined that the patient was
in hemorrhagic shock due to abdominal hemorrhage. He was administered
catecholamine, blood transfusion, and insertion of an aortic balloon
pumping to stabilize his circulation.
An
emergency abdominal angiography revealed a beaded appearance of the
ASPDA. (Figure 6) The ruptured ASPDA pseudoaneurysm was successfully
treated by transarterial coil embolization. (Figure 7) The immunological
tests performed to investigate the cause of the disease showed that
proteinase-3 antineutrophil cytoplasmic antibody (PR3ANCA) was mildly
elevated. Antinuclear antibodies, anticardiolipin antibodies, and
myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPOANCA) were
negative, and complement titers, C3 and C4, were not elevated.
10 days after the SAH, the CT
revealed severe left MCA territory infarction due to cerebral vasospasm
and we performed conservative treatment for the patient. On the 44th
day, the patient had good cerebral pressure control and underwent
cranioplasty. The patient was transferred to a rehabilitation hospital
on the 77 days, and was discharged home with a modified Rankin Scale 3.
3 DISCUSSION
SAM is first reported by Slavin
in 1976, based on pathological examination of cases of intra-abdominal
hemorrhage due to abdominal aneurysms. 3 There are two
main types of arteries: the elastic arteries, and the muscular arteries.
Elastic arteries consist of elastic tissue in the tunica media and
include the aorta and common carotid artery. Muscular arteries contain
smooth muscle cells in the tunica media and include cerebral arteries
and visceral arteries. 4 SAM occurs in muscular
arteries because of segmental lysis of the arterial tunica media smooth
muscle cells. 2It often shows findings of a spindle aneurysm or dissection.5-10 The definitive diagnosis of SAM is based on
pathological findings, the clinical diagnosis depends on the exclusion
of other vasculitis and the finding of beaded vessel irregularities,
dissection, or aneurysm on angiography. Specifically, the criteria are
(1) middle-aged and elderly patients, (2) exclusion of underlying
diseases such as inflammatory changes or atherosclerotic changes, (3)
sudden onset of intra-abdominal bleeding, and (4) presence of bead-like
changes in blood vessels on angiography. 11Differential diseases include
atherosclerotic disease, systemic vasculitis (Behcet’s disease,
polyarteritis nodosa), fibromuscular dysplasia (FMD), Ehlers–Danlos
disease type IV, mycotic aneurysms, cystic medial necrosis and Marfan
syndrome. 1
In this case, the patient was a
middle-aged man in his 50s with no history of collagen disease or other
inflammatory or atherosclerotic diseases. Laboratory blood samples
showed no elevated inflammatory response, and MPOANCA and complement
titers were normal. Only PR3ANCA was elevated in immunological tests,
which required differentiation from granulomatous polyangiitis. It was
not characteristic because of no findings of upper respiratory tract
symptoms, pulmonary symptoms, or nephritis. 9thhospital day, the patient developed sudden intra-abdominal hemorrhage,
and abdominal angiography showed beaded vasodilatation confined to the
ASPDA. Based on the above, SAM was diagnosed because it met the criteria
for clinical diagnosis.
The
etiology of SAM is still unclear, but some authors believe that
vasoactive substances such as norepinephrine are involved and cause
vasoconstriction. 12,13 When SAH occurs, the
activation of the sympathetic nervous system causes a surge of
catecholamines, which stimulate α1 receptors widely distributed in
smooth muscle cells of the tunica media.12 Because
systemic norepinephrine increases approximately threefold within 48
hours of onset, an intra-abdominal aneurysm may have formed at an early
stage after the onset of SAH in this case. 14 The
blood pressure was maintained at a high level during the management of
cerebral vasospasm and the blood pressure further increased due to
stimulation associated with tracheostomy. Therefore, it may have
contributed to the aneurysm rupture.
Including this case, 14 cases of intraperitoneal hemorrhage due to SAM
after SAH have been reported. 2, 5-10, 15-20 (Table 1)
There are many reports from Japan. This is because of the high incidence
of SAH in Japan compared to the worldwide.21 Intracranial cerebral aneurysms included 6
dissecting aneurysms, 2 blistering aneurysms, and 6 saccular aneurysms.
In addition including this case,
11 patients had intraperitoneal hemorrhage during the cerebral vasospasm
period after SAH. The cerebral vasospasm often begins 3-4 days after
onset, peaks at 7-10 days, and lasts until about 14-21 days.22 This is the period when SAM may occur. The blood
pressure is often maintained high and antiplatelet medications are used
for cerebral vasospasm management. It may contribute to intraperitoneal
hemorrhage.
The
cerebral infarction caused by cerebral vasospasm was reported in 5
cases. SAH with cerebral vasospasm may be prone to intra-abdominal
vasospasm as well, which may lead to aneurysm formation. Therefore, SAM
should be considered when cerebral vasospasm occurs after SAH. In
addition, in many cases, the
patients had a good level of consciousness before the intra-abdominal
hemorrhage and were able to report abdominal symptoms. Therefore, they
suspected abdominal disease from the beginning and were able to examine
the patients. On the other hand, our patient was unconsciousness due to
severe SAH, making it difficult to differentiate the cause of shock.
Recognizing SAM can lead to early treatment and save their lives.
4 CONCLUSION
SAH with cerebral vasospasm may be associated with SAM, suggesting that
intra-abdominal hemorrhage due to SAM may occur during cerebral
vasospasm. SAM should be considered in the treatment of SAH in patients
with impaired consciousness and inability to complain of abdominal pain.
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FIGURES AND TABLES
Figure 1. Three-dimensional computed tomography (CT) angiography showing
an aneurysm (arrow) with blebs at the M2 bifurcation of the left middle
cerebral artery.
Figure 2. Intraoperative photograph showing a saccular aneurysm (arrow)
at the M2 bifurcation of the left middle cerebral artery.
Figure 3. The cerebral angiography indicating narrowing at the left M2
(arrow) due to cerebral vasospasm.
Figure 4. This chart explaining the clinical course of a patient with
various anticonvulsant treatments and the development of segmental
arterial mediolysis (SAM).
Figure 5. Axial view of the abdominal contrast-enhanced computed
tomography (CT) scan revealing abnormal band dilatation of the anterior
superior pancreatoduodenal artery (ASPDA) (arrow).
Figure 6. Anterior superior
pancreatoduodenal artery (ASPDA) angiography revealing
pseudoaneurysm in the ASPDA. (arrow).
Figure 7. Anterior superior pancreatoduodenal artery (ASPDA) angiography
illustrating coil embolization (arrow) of the pseudoaneurysm.
Figure 1