CHAPTER
2
HEMORRHAGE
2.1 | Hypertensive Hemorrhage |
Case History
A 54-year-old woman developed a headache and collapsed at home. She was unconscious on examination.
Diagnosis: Hypertensive Hemorrhage
Images 2.1A–2.1C: Axial, coronal, and sagittal CT images demonstrate a massive, left basal ganglia hemorrhage with intraventricular extension involving the fourth ventricle (red arrow). Image 2.1D: Gross pathology of basal ganglia hemorrhage with intraventricular extension.
Introduction
Intraparenchymal hemorrhage (IPH) comprises between 10% and 20% of strokes. The pathology is due to the rupture of small, penetrating arteries that have been weakened due to chronic hypertension (HTN), which is the biggest modifiable risk factor in IPH. Chronic HTN leads to lipohyalinosis in the small, penetrating arteries of the brain.
Low serum cholesterol also increases the risk of IPH, particularly in patients with HTN. IPH is more common in Asians and African Americans. They are also more common in men than in women. This is likely due to increased rates of HTN in these populations.
The use of anticoagulation is the most common iatrogenic cause of IPH. The overall risk for IPH in a patient on warfarin is about 0.5% to 1.0% annually, and this is substantially increased in patients with an international normalized ratio (INR) greater than 2. The additional use of aspirin, HTN, advanced age, and the presence of cerebral amyloid angiopathy (CAA) increase the hemorrhage risk for patients on anticoagulant therapy. These are usually devastating hemorrhages and are often fatal.
Excessive alcohol consumption and smoking increase the risk, as does the use of sympathomimetic drugs such as cocaine or amphetamines. The substances may cause a vasculitis or produce acute rises in blood pressure (BP) leading to the rupture of preexisting vascular abnormalities.
Clinical Presentation
On clinical grounds alone, hypertensive IPH cannot reliably be distinguished from ischemic infarcts, though there may be some general differences. Unlike ischemic strokes, which tend to have an immediate onset, IPH progresses over 30 to 90 minutes. Hematoma expansion is unusual after 24 hours, and its occurrence portends a worse prognosis. IPH is more commonly associated with decreased levels of consciousness and headache as well as nausea and vomiting compared to ischemic strokes, due to the mass effect of a rapidly expanding hematoma and increased intracranial pressure (ICP). Finally, the clinical picture produced by intracerebral hemorrhages is somewhat more variable than those produced by ischemic events, as hemorrhages do not respect well-demarcated vascular territories.
Common locations for hypertensive IPH are the basal ganglia (in particular, the putamen), thalamus, pons, and cerebellum.
Basal ganglia: The basal ganglia, the putamen in particular, is the most common location for IPHs. Patients present with contralateral hemiplegia and sensory deficits. Larger hemorrhages may cause aphasias or neglect syndrome (depending on which side of the brain is involved), visual disturbances, and a decreased level of consciousness progressing to coma, especially if there is mass effect on the brainstem. Large hemorrhages may extend into the ventricular system causing a decreased level of consciousness, severe headache, and nausea and vomiting. Eventually, hydrocephalus may result leading to long-term cognitive impairment.
Thalamus: Thalamic hemorrhages present with contralateral sensory deficits and hemiplegia due to mass effect on the corticospinal tract as it runs through the posterior limb of the internal capsule. Aphasias or neglect syndrome may result. Larger thalamic hemorrhages may also extend into the ventricular system or produce mass effect on the upper brainstem causing disturbances of vertical gaze.
Pons: Pontine hemorrhages are devastating and often fatal events. They are due to the rupture of penetrating vessels that emerge from the basilar artery and travel into the pons. Patients are quickly rendered quadriplegic with rigidity and decerebrate posturing. They are typically comatose and with minimal brainstem reflexes. The pupils are typically pinpoint. The overall prognosis is uniformly poor, especially once coma has developed. Most patients do not survive, and those who do rarely have meaningful recovery. In certain cases, if the initial hemorrhage is very small or is due to an underlying vascular disorder, some degree of recovery is possible.
Images 2.1E–2.1G: Axial, sagittal, and coronal CT images demonstrate hemorrhage in the right putamen. Image 2.1H: Gross pathology of basal ganglia hemorrhage.
Images 2.1I and 2.1J: Axial CT images demonstrate hemorrhage in the right and left thalami in two different patients. Mass effect on the midbrain is obvious in Image 2.1I.
Cerebellum: Cerebellar hemorrhages comprise about 10% of all IPHs. They present with headache, ataxia, vertigo/dizziness, and vomiting. Patients may develop acute, lethal obstructive hydrocephalus due to occlusion of the fourth ventricle.
Radiographic Appearance and Diagnosis
CT scans are the imaging modality of choice for patients with a suspected IPH. They are 100% sensitive and specific for acute hypertensive hemorrhages.
MRIs are used to evaluate for any underlying pathology that may have led to IPH. The appearance of a hemorrhage depends on the timing of the MRI and the sequence used (T1-weighted vs. T2-weighted); see Table 2.1.1. This appearance reflects the underlying components of the hemorrhage. Gradient echo sequences of MRI are best for detecting degraded blood of older hemorrhages.
Images 2.1K–2.1M: Axial, coronal, and sagittal CT images demonstrate hemorrhage in the pons with extension into the fourth ventricle. Image 2.1N: Gross pathology of pontine hemorrhage.
Image 2.1O: Axial CT image demonstrates hemorrhage in the cerebellum with extension into the fourth ventricle (red arrow). The yellow arrow shows the enlargement of the temporal horns of the lateral ventricle.
Table 2.1.1 The Appearance of Blood on MRIs
Image 2.1P: Axial CT image demonstrates a large hemorrhage in the left thalamus with intraventricular extension. Images 2.1Q–2.1S: Axial T2-weighted images are shown on day 1, day 21, and day 120.
Treatment
Patients should be admitted to a monitored setting with close monitoring of respiratory and circulatory status. Patients unable to safely protect their airways due to brainstem dysfunction or depressed levels of consciousness should have mechanical ventilation. The most common cause of decline in patients with IPH is early hematoma expansion, which occurs in about 40% of patients.
There is no primary therapy of the hematoma itself with demonstrated improved outcomes. A trial of activated factor VIIa reduced the size of hematoma expansion, but failed to have an impact on patient outcome. Patients on anticoagulation should be given vitamin K and either fresh frozen plasma or recombinant factor VIIa. At present, there is no evidence to support the use of platelet transfusions in patients taking antiplatelet medications.
Patients with large hematomas and poor neurological statuses benefit from placement of intraventricular monitors to measure ICP. Patients with elevated ICPs can have fluid drained from their intraventricular catheters. Other interventions to decrease brain edema, such as mannitol or hyperventilation, should be used in patients with impending herniation as a bridge to definitive treatment.
The American Heart Association guidelines for treating elevated BP are as follows:
Image 2.1T: Axial CT image demonstrates cerebellar hemorrhage. Image 2.1U: Axial CT image after evacuation of the blood products.
If systolic BP is greater than 200 mmHg or mean arterial pressure (MAP) is greater than 150 mmHg, then consider aggressive reduction of BP with continuous intravenous infusion with frequent BP (every 5 minutes) checks.
If systolic BP is greater than 180 mmHg or MAP is greater than 130 mmHg and there is evidence or suspicion of elevated ICP, then consider monitoring of ICP and reducing BP using intermittent or continuous intravenous medications to maintain a cerebral perfusion pressure of 60-80 mmHg.
If systolic BP is greater than 180 or MAP is greater than 130 mmHg and there is NO evidence or suspicion of elevated ICP, then consider modest reduction of BP (target MAP of 110 mmHg or target BP of 160/90 mmHg) with BP checks every 15 minutes.
Careful attention to prevention of complications in critically ill patients such as infection, deep vein thrombosis, and electrolyte disturbances is essential. Antiepileptic medications are suggested only in those patients who have suffered seizures, which is more common in patients with lobar hemorrhages.
“Fatal gastroenteritis” is the term given to nausea and vomiting due to unrecognized cerebellar pathology that eventually kills the patient. Relieving increased ICP by means of an external ventricular drain can be lifesaving. Hematomas larger than 3 cm in diameter are at most risk for neurological decline, and neurosurgical evacuation of the hematoma is required in such patients. Patients with smaller hematomas should be monitored closely both clinically and radiographically for hematoma expansion.
References
1. Elliott J, Smith M. The acute management of intracerebral hemorrhage: a clinical review. Anesth Analg. May 2010;110(5):1419–1427.
2. Flower O, Smith M. The acute management of intracerebral hemorrhage. Curr Opin Crit Care. April 2011;17(2):106–114.
3. Manno EM. Update on intracerebral hemorrhage. Continuum (Minneap Minn). June 2012;18(3):598–610.
4. Adeoye O, Broderick JP. Advances in the management of intracerebral hemorrhage. Nat Rev Neurol. November 2010;6(11):593–601.
5. Hemphill JC 3rd, Greenberg SM, Anderson CS, et al. Guidelines for the management of spontaneous intracerebral hemorrhage: A guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. July 2015;46(7):2032–2060.
Unless otherwise stated, all pathology images in this chapter are from the website http://medicine.stonybrookmedicine.edu/pathology/neuropathology and are reproduced with permission of the author, Roberta J. Seidman, MD, Associate Professor. Unauthorized reproduction is prohibited.
2.2 | Lobar Hemorrhages |
Case History
A 75-year-old man developed a sudden onset headache and left homonymous hemianopsia.
Diagnosis: Lobar Hemorrhages Due to Amyloid Angiopathy
Images 2.2A–2.2C: Axial CT images demonstrate an acute hemorrhage in the right occipital lobe. Image 2.2D: Gross pathology of a lobar hemorrhage.
Introduction
Hemorrhages within the lobes of the brain adjacent to the cortex are called lobar hemorrhages. They are most common in elderly, nonhypertensive patients and are associated with CAA. This occurs when there is an abnormal accumulation of beta-amyloid protein in the arterioles of the cortex and meninges. There are typically simultaneous hemorrhages or multiple hemorrhages of different ages. For unclear reasons, such hemorrhages are much more common in the parietal and occipital lobes. The basal ganglia, brainstem, and cerebellum are typically spared.
The risk of lobar hemorrhage is increased in patients who have the e2 or e4 allele of the apolipoprotein E gene and is independent of the risk of HTN.
Clinical Presentation
Patients present with a headache and rapid progression of focal neurological deficits, which depend on the location of the bleed. Patients may lose consciousness if there is mass effect on the brainstem or a rapid rise in ICP.
Images 2.2E–2.2H: Axial CT images demonstrate hemorrhage in the frontal, temporal lobes, parietal, and occipital lobes in four different patients.
Radiographic Appearance and Diagnosis
CT scans are 100% sensitive for lobar hemorrhages.
The diagnosis of CAA can only be definitively made with pathological examination of blood vessels, though a probable diagnosis can be made in the appropriate clinical setting with supportive evidence on imaging. The amyloid protein stains brightly with Congo red dye and demonstrates yellow-green birefringence under polarized light.
Treatment
There is no definitive treatment, but antiplatelet agents and anticoagulation should be avoided. In certain cases, surgical evacuation of the hematoma may be considered especially if there is life-threatening mass effect, though there is little evidence to support this practice. Patients should be admitted to a monitored setting with close monitoring of respiratory and circulatory status.
Images 2.2I and 2.2J: Histopathology of cerebral amyloid angiopathy showing deposition of amyloid (red) in hyalinized vessel walls on Congo red staining and birefringence in polarized light (image credit Marvin101).
Images 2.2K and 2.2L: Axial CT images demonstrate hemorrhage in the left temporal lobe before and after evacuation of the blood.
References
1. Li XQ, Su DF, Chen HS, Fang Q. Clinical neuropathological analysis of 10 cases of cerebral amyloid angiopathy-related cerebral lobar hemorrhage. J Korean Neurosurg Soc. July 2015;58(1):30–35.
2. Mehndiratta P, Manjila S, Ostergard T, et al. Cerebral amyloid angiopathy-associated intracerebral hemorrhage: pathology and management. Neurosurg Focus. April 2012;32(4):E7.
3. Izumihara A, Suzuki M, Ishihara T. Recurrence and extension of lobar hemorrhage related to cerebral amyloid angiopathy: multivariate analysis of clinical risk factors. Surg Neurol. August 2005;64(2):160–164.
4. Thanvi B, Robinson T. Sporadic cerebral amyloid angiopathy—an important cause of cerebral haemorrhage in older people. Age Ageing. November 2006;35(6):565–571.
Unless otherwise stated, all pathology images in this chapter are from the website http://neuropathology-web.org and are reproduced with permission of the author, Dr. Dimitri Agamanolis. Unauthorized reproduction is prohibited.
2.3 | Amyloid Beta Related Angiitis |
Case History
A 65-year-old otherwise healthy man developed progressive dementia.
Diagnosis: Amyloid Beta Related Angiitis
Images 2.3A and 2.3B: Axial FLAIR images demonstrate extensive white matter disease, mainly in the left temporal and bilateral occipital lobes. Images 2.3C and 2.3D: Axial gradient echo images demonstrate extensive punctate foci of old hemorrhage scattered throughout the brain parenchyma, though primarily in the occipital and temporal lobes.
Introduction
Amyloid beta related angiitis (ABRA) is characterized by transmural infiltration of vessel walls by lymphocytes and macrophages with the formation of granulomas and multinucleated giant cells in the background of CAA.
It is a vasculitis of small- and medium-sized leptomeningeal arteries and is thought to be an immune reaction to beta amyloid in blood vessel walls.
Clinical Presentation
Patients present with a combination of subacute dementia, seizures, focal neurological signs, and headaches. Patients present at a younger age than those with noninflammatory CAA.
Radiographic Appearance
Imaging reveals white matter hyperintensities, usually asymmetric, and microbleeds, best seen on gradient echo or T2-weighted MRI. The microbleeds are in a distribution distinct from the white matter lesions, and there is a correlation between the clinical course and the size of the lesions. The lesions shrink with improvement in symptoms and enlarge with recurrences. A brain biopsy is needed for definitive diagnosis.
Treatment
It is treated with immunosuppressants.
Image 2.3E: Congo red stain demonstrating amyloid deposits in blood vessel walls (image credit Dr. Seema Shroff, Fellow, New York University Langone Medical Center [NYULMC]).
References
1. Tschampa HJ, Niehusmann P, Marek M, Mueller CA, Kuchelmeister K, Urbach H. MRI in amyloid beta-related brain angiitis. Neurology. July 2009;73(3):247.
2. Danve A, Grafe M, Deodhar A. Amyloid beta-related angiitis—a case report and comprehensive review of literature of 94 cases. Semin Arthritis Rheum. August 2014;44(1):86–92.
2.4 | Hemorrhagic Conversion of Ischemic Stroke |
Case History
A 71-year-old woman developed a seizure and obtundation after receiving tissue plasminogen activator (t-PA) for an ischemic stroke.
Diagnosis: Hemorrhagic Conversion of Ischemic Stroke
Images 2.4A and 2.4B: Axial CT images demonstrate a large hemorrhage within a right posterior cerebral artery infarct. Image 2.4C: Gross pathology demonstrates hemorrhagic infarction.
Introduction
Ischemic stroke, especially after the administration of t-PA, is an important cause of IPH. The rate of symptomatic hemorrhage with the use of t-PA in ischemic hemorrhages is 6% and increases with deviations from the standard t-PA protocol, larger strokes, cortical strokes, strokes due to atrial fibrillation, advanced age, low platelet count, and hyperglycemia. Of these, large infarct size is the greatest risk factor.
Two types of hemorrhages are seen after strokes: small petechial hemorrhages, called hemorrhagic infarctions, and larger hematomas into the ischemic bed, called parenchymal hematomas.
Petechial hemorrhages are seen in about 50% of large cerebral infarcts, while larger parenchymal hematomas are less common. In patients who have received thrombolytic therapy, parenchymal hematomas usually occur in the first 24 hours. In untreated patients they occur within the first four days.
Clinical Presentation
Petechial hemorrhages are asymptomatic and of no clinical consequence. They can be considered part of the natural evolution of large ischemic strokes due to reperfusion in the vascular bed of the infarct.
Larger parenchymal hematomas can result in rapid, significant deterioration. The clinical presentation depends on the area into which the hemorrhage occurs as well as its size. Patients with parenchymal hematomas have worsened outcomes and mortality.
Radiographic Appearance
CT scan is the imaging modality of choice for patients with a suspected hemorrhagic conversion. Petechial hemorrhages appear as small hyperdensities. They typically occur in the gray matter due to robust collateral circulation.
Images 2.4D and 2.4E: Axial CT scans demonstrate petechial hemorrhages within a right middle cerebral artery infarct.
As seen in Images 2.4A and 2.4B, larger parenchymal hematomas may fill the entire ischemic deficit with mass effect on the surrounding brain tissue and secondary ischemia.
Treatment
Proven treatment is limited to supportive measures and minimizing risk of further bleed or expansion.
References
1. Zhang J, Yang Y, Sun H, Xing Y. Hemorrhagic transformation after cerebral infarction: current concepts and challenges. Ann Transl Med. August 2014; 2(8):81.
2. Sussman ES, Connolly ES Jr. Hemorrhagic transformation: a review of the rate of hemorrhage in the major clinical trials of acute ischemic stroke. Front Neurol. June 2013;4:69.
Unless otherwise stated, all pathology images in this chapter are from the website http://medicine.stonybrookmedicine.edu/pathology/neuropathology and are reproduced with permission of the author, Roberta J. Seidman, MD, Associate Professor. Unauthorized reproduction is prohibited.
Unless otherwise stated, all pathology images in this chapter are from the website http://medicine.stonybrookmedicine.edu/pathology/neuropathology and are reproduced with permission of the author, Roberta J. Seidman, MD, Associate Professor. Unauthorized reproduction is prohibited.
2.5 | Hemorrhagic Tumor |
Case History
A 76-year-old woman presented with a headache and deficits in her right visual field.
Diagnosis: Hemorrhagic Tumor
Image 2.5A: Axial CT image demonstrates hemorrhage in the left parietal lobe. Image 2.5B: Contrast-enhanced axial T1-weighted image demonstrates an underlying tumor. Image 2.5C: Gross pathology of a hemorrhagic tumor with extension into the ventricles (image credit www.wikidoc.org via Professor Peter Anderson, DVM, PhD, and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology).
Introduction
High-grade primary brain neoplasms may hemorrhage into the necrotic center of the tumor, and this can be the presenting symptom of the tumor. Hemorrhage occurs in about 8% to 9% of glioblastomas and is the presenting symptom in about 2%. In children, medulloblastomas are commonly associated with hemorrhage. Lower grade neoplasms are much less likely to hemorrhage.
Other primary central nervous system (CNS) neoplasms that are associated with hemorrhage include oligodendrogliomas, craniopharyngiomas, vestibular schwannomas, ependymomas, pituitary adenomas, and choroid plexus carcinomas.
Certain metastatic tumors are prone to hemorrhage. The tumors with the highest propensity to hemorrhage are melanoma, renal cell carcinoma, choriocarcinoma, and thyroid cancer. Hemorrhage is the presenting symptom in about half of these tumors. Because breast and lung cancers are much more common overall, hemorrhagic metastases are most likely due to these tumors.
Image 2.5D: Contrast-enhanced axial T1-weighted image demonstrates a small enhancing lesion in the right temporal lobe in a patient with known melanoma. Image 2.5E: Axial CT image demonstrates a large, fatal hemorrhage in the same patient several weeks later.
Clinical Presentation
Patients develop headache and other symptoms of increased ICP due to the sudden increase in mass effect. Further symptoms depend on the area into which the hemorrhage occurs.
Radiographic Appearance
CT is the modality of choice for patients with a suspected hemorrhage. Oftentimes, the underlying tumor is obscured by blood products, and a biopsy or repeat MRI may be needed to find the tumor.
Within the supratentorial structures, hemorrhagic metastases may be indistinguishable from primary brain tumors. However, in the cerebellum the overwhelming majority of tumors are metastatic in adults.
Treatment
Treatment is directed toward the underlying tumor.
References
1. Lieu AS, Hwang SL, Howng SL, Chai CY. Brain tumors with hemorrhage. J Formos Med Assoc. May 1999;98(5):365–367.
2. Wakai S, Yamakawa K, Manaka S, Takakura K. Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. Neurosurgery. 1982;10:437–444.
2.6 | Spinal Epidural Hematoma |
Case History
A 46-year-old woman presented with the abrupt onset of a severe, stabbing pain in her neck and weakness of her arms and legs.
Diagnosis: Spinal Epidural Hematoma
Images 2.6A and 2.6B: Sagittal and axial T1-weighted images of the cervical spine demonstrate an epidural hematoma (red arrows) with compression of the cervical spinal cord.
Introduction
A spontaneous spinal epidural hematoma (SEDH) is an accumulation of blood in the potential space between the dura and bone with the dorsal aspect of the thoracolumbar region most commonly involved. The epidural venous plexus is usually involved, although arterial sources of hemorrhage may also occur, and expansion is limited to a few vertebral levels.
Overall, it is a very rare condition. It most commonly presents in the fourth or fifth decade. They may occur spontaneously or in patients with impaired coagulation. In patients with impaired coagulation, a lumbar puncture may precipitate an epidural hematoma. Tumors and traumatic injuries are also predisposing factors.
Clinical Presentation
SEDHs are characterized by the acute onset of severe, stabbing pain, often in a radicular pattern, followed by signs of myelopathy depending on the location of the bleed and the speed at which it develops. There will be variable weakness and sensory loss below the level of the lesion.
Radiographic Appearance and Diagnosis
MRI best delineates the location and extent of SEDHs. It typically shows a well-circumscribed, biconvex hematoma in the epidural space. In the first 24 hours, the hematoma is isointense on T1-weighted images with a heterogeneous high signal on T2-weighted images. After 24 hours, the blood is hyperintense on T1-weighted images, and isointense to cerebrospinal fluid (CSF) on T2-weighted images.
Treatment
Prompt diagnosis and early surgical intervention (within 36–48 hours) with decompressive laminectomy and hematoma removal is the definitive treatment. This can prevent severe and permanent neurological injury in patients without preoperative deficits.
References
1. Ng WH, Lim CC, Ng PY, Tan KK. Spinal epidural hematoma: MRI-aided diagnosis. J Clin Neuroscience. 2002;9:92–94.
2. Liu Z, Jiao Q, Xu J, Wang X, Li S, You C. Spontaneous spinal epidural hematoma: analysis of 23 cases. Surg Neurol. March 2008;69(3):253–260.
3. Groen RJ. Non-operative treatment of spontaneous spinal epidural hematomas: a review of the literature and a comparison with operative cases. Acta Neurochir (Wien). February 2004;146(2):103–110.
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