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Subarachnoid hemorrhage (SAH) is one of the most severe neurosurgical diseases in which systemic management is important from the acute phase to the chronic phase. To date, several reports have documented SAH with intra-abdominal hemorrhage. As a cause of intra-abdominal hemorrhage, segmental arterial mediolysis (SAM) was first described in 1976.1 It is also gaining attention as a cause of intra-abdominal hemorrhage associated with SAH. The clinical course of such a patient is presented along with a review of the relevant literature.
Illustrative Case
A 60-year-old woman collapsed suddenly at home and was brought to our institution by ambulance. She had a past history of unilateral severe renal atrophy and hypertension. On arrival, her consciousness was E1V1M3, and her pupils were isocoric, normal in size. There were no meaningful findings, including no anemia, on laboratory examination. Electrocardiography showed no findings suggestive of Takotsubo cardiomyopathy. Head computed tomography (CT) showed SAH, intraventricular hemorrhage, and intracerebral hematoma in the right frontal lobe with acute hydrocephalus, suggesting the presence of an anterior cerebral artery aneurysm on the right side (Fig. 1). After the head CT, whole-body CT was performed to screen for pneumonia due to COVID-19. However, immediately after these scans, the patient’s blood pressure decreased suddenly, and her breathing deteriorated. Endotracheal intubation was subsequently performed, and continuous administration of noradrenaline was started. Whole-body CT showed hematoma from the perirenal space to the intrapelvic space on the right side. Additional contrast-enhanced CT showed an abnormal bright-signal spot suggesting extravasation in the hematoma cavity, and one of the abdominal arteries that ran forward was considered the responsible artery although head CT angiography was postponed because of her poor condition (Fig. 2). Emergency ventriculostomy was conducted first to decrease intracranial pressure when her vital signs recovered slightly. However, she was still in need of catecholamine, and vital signs remained unstable. In this case, it was determined that endovascular embolization of the intra-abdominal hemorrhage was necessary first to stabilize the vital sign. Abdominal angiography showed a pseudoaneurysm of the right ovarian artery (Fig. 3A–C). This aneurysm was consistent with a diagnosis of SAM related to a ruptured cerebral aneurysm. The responsible aneurysm causing intra-abdominal bleeding was obliterated using gelatin sponge and pushable coil embolization although extravasation occurred during the procedure (Fig. 3D and E). Immediately after treatment, the patient’s vital signs worsened again. Because she then became comatose and had dilated pupils, intracranial vascular evaluation and the treatment of SAH were cancelled. Although there was no radiographic proof regarding the presence of a cerebral aneurysm, the initial head CT showing thick SAH in the interhemispheric fissure with intracerebral hemorrhage in the right frontal base strongly suggested a ruptured anterior cerebral artery aneurysm. Cone-beam CT showed no increase of the intracranial hematoma, but the cortical sulci seemed to be very tight, indicating extremely high intracranial pressure. Unfortunately, the patient died on day 3 of hospitalization.
Initial CT on admission (A–C) shows SAH and intracerebral hematoma in the frontal lobe with acute hydrocephalus and intraventricular hemorrhage, suggesting a ruptured anterior cerebral artery aneurysm.
A: Abdominal plain CT on admission shows massive intra-abdominal hematoma on the right side. B: Abdominal contrast-enhanced CT in the arterial phase demonstrates a spot sign in the center of the hematoma (white arrow). C: Abdominal contrast-enhanced CT in the venous phase demonstrates a high-density area that has increased in size, suggesting the source of the bleed (black arrow). D: Abdominal contrast-enhanced coronal CT shows hematoma from the perirenal area to the intrapelvic space, and the elongated right abdominal artery descends through the hematoma (black arrows).
Digital subtraction angiography, right ovarian artery injection (A–C), demonstrates the pseudoaneurysm with contrast retention in the midportion of the ovarian artery (black arrows). D: Microcatheter injection during the embolization procedure shows extravasation from the pseudoaneurysm (black circle). E: The ovarian artery is successfully obliterated using gelatin sponge and pushable coil embolization (black arrows).
Discussion
Observations
The concept of SAM was first described by Slavin et al. in 1976.1 The common sites were reported to be the superior mesenteric artery (53.1%), hepatic artery (44.8%), celiac artery (35.7%), renal artery (25.9%), splenic artery (24.5%), cerebral vessels (13.3%), and inferior mesenteric artery (10.5%), and multiple lesions were reported in 62.2%.2 Although the definitive diagnosis is supposed to be made pathologically, it is difficult to harvest the specimen routinely in clinical practice. Therefore, clinical diagnostic criteria have been proposed based on imaging, laboratory data, and clinical symptoms.3 Imaging features include dissection, a spindle-shaped aneurysm, and occlusion. Other differential diagnoses to consider for SAM include fibromuscular dysplasia, polyarteritis nodosa, antineutrophil cytoplasmic antibody–associated vasculitis, giant cell arteritis, Takayasu disease, Behçet disease, mycotic aneurysm, type IV Ehlers-Danlos syndrome, Marfan syndrome, neurofibromatosis, pseudoxanthoma elasticum, atherosclerosis, and Kawasaki disease.3 The histopathological features of SAM include (1) a residual medial island with aneurysmal segmentation and fibrinous gaps due to vacuolization and lysis of the tunica media and (2) a medial gap with rupture of the intima while the dilated adventitia is preserved and no inflammation in the vessel wall or atherosclerosis of the vessel wall.4 Our patient also had an intra-abdominal hemorrhage due to the ruptured ovarian artery aneurysm. She was already semicomatose on arrival, and it was not possible to confirm her clinical symptoms, detailed neurological findings, or laboratory data necessary for differential diagnosis. The possibility that a preexisting ovarian aneurysm caused intra-abdominal bleeding due to blood pressure fluctuation on arrival cannot be ruled out; however, ovarian aneurysm rupture is extremely rare. Only 25 cases were reported from 1963 to 2014, 18 (72%) of which were associated with pregnancy.5 In the present case, abdominal angiography showed a pseudoaneurysm of the ovarian artery. The patient was not pregnant, and abdominal bleeding occurred after SAH. Therefore, a clinical diagnosis of intra-abdominal hemorrhage associated with SAM was made. To the best of our knowledge, a total of 14 similar cases have been reported, including the present patient (Table 1).6–18 Multiple aneurysms were found both intracranially and extracranially. The Iocation of ruptured cerebral aneurysms was the anterior circulation in 58.8% of cases and the posterior circulation in 41.2% of cases. The time from onset of SAH to onset of intra-abdominal hemorrhage varied from 0 to 30 days. Interestingly, 3 (21.4%) of 14 patients had both intracranial and intra-abdominal hemorrhage on the same day. The clinical manifestations at the time of onset included hypovolemic shock in 6 patients, abdominal pain in 3 patients, and impaired consciousness in 2 patients. Intra-abdominal hemorrhage due to SAM was most frequently observed in the splenic artery and superior mesenteric artery (16.7%), followed by the renal artery and hepatic artery (12.5%). Because there have been no reports describing SAM involving the ovarian artery, this may be the first case of bleeding from this vessel. Previous reports indicate that various therapeutic approaches for SAM have been adopted, including coil embolization (28%), abdominal organ surgery (24%), open arterial repair (21%), and medical management (20%). Case-specific treatment modalities yielded symptom relief in most (91%) patients, with a mortality rate of 7%.2 In patients with SAH attributed to SAM, 4 of 14 patients (28.6%) died even after treatment for SAM (Table 1). These data indicate that patients with SAM associated with SAH often have a poor prognosis. Although the cause of SAM remains controversial, several authors suggested that a catecholamine surge may be relevant to intra-abdominal bleeding as well as Takotsubo cardiomyopathy.19 One article proposed that norepinephrine is the cause of SAM, reporting that SAM was found in the renal arteries, intrahepatic arteries, and coronary arteries in dogs injected with ractopamine (a β2-adrenergic agonist). Ractopamine causes the release of norepinephrine from the peripheral nervous system.20 Catecholamines are sometimes used to treat vasoconstriction, and some reports have suggested that this may also be a cause of the development of SAM. When a sudden worsening of the vital signs occurs, it is important to consider the possibility of not only Takotsubo cardiomyopathy but also intra-abdominal hemorrhage. In addition to routine examination for cerebrovascular diseases, systemic examination should be performed as soon as possible.
Literature regarding intra-abdominal hemorrhage caused by SAM associated with aneurysmal SAH
| Authors & Year | Age (yrs)/Sex | WFNS Grade | Cerebral Aneurysm | Tx for Cerebral Aneurysm | Symptoms of SAM | Location of SAM | Interval Btwn Ab Hemorrhage & SAH | Path Dx of SAM | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Isla et al., 198816 | 68/F | II | A com a | Clipping | NA | NA | 1 mo | No | Improved |
| Fuse et al., 199618 | 56/F | II | Lt ICA, rt MCA | Clipping | Ab pain | Celiac a, gastric a | 16 days | No | Improved |
| Sakata et al., 200211 | 48/M | V | Rt VA, lt ICA | No | Impaired consciousness | Superior mesenteric a, renal a, external iliac a | NA | Yes | Died |
| Soga et al., 200913 | 73/F | V | NA | No | Impaired consciousness | Superior mesenteric a | NA | Yes | Died |
| Matsuda et al., 201210 | 59/M | I | Rt proximal ACA, rt distal ACA, lt VA | Coating | NA | Splenic a, renal a, gastroduodenal a | NA | No | Improved |
| Stetler et al., 201214 | 59/F | NA | Rt P com a | Coiling | Hypovolemic shock, ab pain | Hepatic a | Postop day 3 | No | Improved |
| Cooke et al., 20136 | unknown (45–55/M) | V | Lt VA | Coiling | Incidental | Mammary a, renal a | NA | No | Improved |
| Shinoda et al., 201612 | 47/M | V | Lt VA | Coiling | Hypovolemic shock, ab pain | Middle colic a | 8 days | Yes | Improved |
| Welch et al., 201717 | 61/M | NA | Posterior spinal a | Embolization | NA | Splenic a, posterior spinal a | Same day | No | Improved |
| Hellstern et al., 20188 | 30/M | IV | BA, bilat ICA | Coiling & flow diverter | Hypovolemic shock | Splenic a, hepatic a | Same day | Yes | Improved |
| Hayashi et al., 20187 | 49/F | NA | Lt ICA | Clipping | NA | Splenic a, hepatic a, superior mesenteric a, gastroduodenal a | 4 days | No | Improved |
| Isaji et al., 20189 | 45/M | II | Rt VA | Coiling | Hypovolemic shock | Middle colic a | 8 days | No | Improved |
| Tanaka et al., 202015 | 60/M | II | Lt ICA | High-flow bypass & trapping of C2 segment | Hypovolemic shock | Superior mesenteric a | 12 days | Yes | Died |
| Present case | 60/F | V | Rt ACA suspected | No | Hypovolemic shock | Ovarian a | Same day | No | Died |
a = artery; ab = abdominal; ACA = anterior cerebral artery; A com a = anterior communicating artery; BA = basilar artery; Dx = diagnosis; NA = not applicable; ICA = internal carotid artery; MCA = middle cerebral artery; Path = pathological; P com a = posterior communicating artery; SAH = subarachnoid hemorrhage; SAM = segmental arterial mediolysis; Tx = treatment; VA = vertebral artery; WFNS = World Federation of Neurosurgical Societies.
Lessons
Although it was possible to incidentally detect intra-abdominal hemorrhage in our patient, SAH and intra-abdominal hemorrhage can occur almost simultaneously. It is necessary to keep in mind the possibility of intra-abdominal hemorrhage when vital signs deteriorate during the acute care of a patient with SAH. Prompt diagnosis and appropriate treatment are of great importance.
Disclosure
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
Author Contributions
Conception and design: Tao, Matsubara, Uno. Acquisition of data: Matsubara, Kinoshita, Fukunaga, Yamamoto. Analysis and interpretation of data: Tao, Yamamoto. Drafting the article: Tao, Matsubara. Critically revising the article: Tao, Yagi. Reviewed submitted version of manuscript: Tao, Matsubara. Administrative/technical/material support: Matsubara, Fukunaga, Yamamoto. Study supervision: Matsubara, Uno.
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