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Case Report: Headache in a Postpartum Patient With Essential Thrombocytosis
Case
At presentation, the patient was in no apparent distress and had normal vital signs, including normal blood pressure. On physical examination, she was alert and oriented, with unremarkable head, neck, fundoscopic, cardiac, pulmonary, skin, and neurological examinations. Laboratory results were only notable for a platelet count of 677 x 109/L. The remainder of the complete blood count, chemistry, and coagulation panels was all within normal limits. A noncontrast computed tomography (CT) of the brain was unremarkable. She was treated symptomatically with ketorolac, diphenhydramine, and metoclopramide, but received no relief.
Based on the patient’s history of ET and postpartum state, there was a high suspicion for cerebral venous thrombosis (CVT). Magnetic resonance imaging/magnetic resonance venography (MRI/MRV) of the brain was obtained, which revealed near-complete thrombosis of the vein of Galen and straight sinus (Figure). Neurology and hematology services were consulted emergently, and the patient was started on a heparin drip and admitted to the neurosurgical intensive care unit. Hydroxyurea was also given for cytoreduction, and platelets normalized to 305 x 109/L within 1 day of treatment. The patient’s neurological examination remained nonfocal, her headache gradually resolved, and she was discharged on hospital day 3 on oral anticoagulants.
Discussion
Essential thrombocytosis is a chronic myeloproliferative disorder characterized by clonal proliferation of the megakaryocyte cell line due to a defect in a pluripotent hematopoietic stem cell.1 The estimated annual incidence is 2.5 per 100,000 individuals.2 It affects patients of both genders, most commonly between ages 50 to 70 years.3
Up to 25% of patients with ET experience neurological complications, mainly occlusive strokes, chronic headache, and dizziness.4 Since pregnancy also increases thrombogenicity, and CVT occurs in 12 per 100,000 deliveries,5 it should be considered in any pregnant or postpartum patient with neurological symptoms.
Diagnosis of ET
Essential thrombocytosis is a diagnosis of exclusion, and no clinical or laboratory finding is diagnostic.1 Although patients with ET are at increased risk for bleeding, these events usually involve mucosal and cutaneous sites and are clinically insignificant. Thrombotic complications account for the majority of the morbidity and mortality associated with ET and can be both arterial and venous. In one case series, roughly 50% patients with ET experienced a thrombotic event within 9 years from diagnosis.6 Thrombotic complications are seen most commonly in patients younger than age 55 years at diagnosis. Common sites of thrombosis include the deep veins of the lower extremities, pulmonary vessels, hepatic vein, portal vein, digital microvasculature, and placenta.1
Despite the high rate of thrombotic complications in ET, there is little data on neurological complications and few reports of CVT in patients with ET.7-9 In one chart review of 70 patients with ET, researchers identified 18 patients who developed neurological complications,4 the most common of which was cerebrovascular accident/transient ischemic attack. Only one patient in the series had a CVT. The majority of patients with neurological events were female and no clinical or laboratory parameter predicted the risk of neurological event.
Thrombocytosis
Thrombocytosis is classified as either primary/essential or secondary/reactive. The majority of thrombocytosis is secondary/reactive and is caused by a variety of inflammatory conditions, such as infection and malignancy. Unlike ET, secondary thrombocytosis is not associated with any bleeding or thrombotic complications and does not require direct treatment.1
Treatment of ET
The decision to treat ET is based on clinical parameters and is usually reserved for patients at high-risk of thrombosis, including those with history of thrombosis or platelets >1,500 x 109/L.1 The first-line agent for treatment of essential thrombocytosis, hydroxyurea has been shown to decrease thrombotic episodes.10 Other cytoreductive agents include anagrilide and interferon-α, the use of which is considered safe in pregnancy.1
Cerebral Venous Thrombosis
Cerebral venous thrombosis is a rare but potentially fatal condition. Patients may present with headache (95%), seizures (47%), and vision changes (41%), and may or may not have focal or generalized neurological deficits.11 Thrombosis can lead to venous obstruction, resulting in increased intracranial pressure and ultimately cerebral herniation and death. Predisposing risk factors for CVT include thrombophilic and procoagulant disorders, maxillofacial infections, trauma, malignancy, and vasculitides. Among female patients with CVT, studies show approximately 50% were on oral contraceptives and 20% were pregnant or in the postpartum period at the time of the event. Nearly half of patients with CVT have multiple risk factors.12
Diagnosis of CVT
Diagnosis of CVT requires a high clinical suspicion. Lumbar puncture usually reveals high opening pressure with normal cerebrospinal fluid analysis.13 The classic finding of CVT on a contrast-enhanced CT scan is the “empty delta sign,” which is a central hypointensity within the superior sagittal sinus secondary due to slow/absent flow surrounded by contrast enhancement in a triangular shape. An ischemic infarction crossing arterial boundaries or near a venous sinus—with or without a hemorrhagic component—is also suggestive of CVT. However, CT scans are read as normal or indeterminate in up to 30% patients.14 Moreover, while CT venography may visualize the cerebral venous system, there is a false-negative rate of 25%.15 Therefore, MRI with MRV is the gold standard for diagnosis.
Pregnancy and CVT
As previously noted, pregnant or postpartum patients are at an increased risk of developing CVT. During pregnancy, decreased levels of protein S inhibitors and increased levels of fibrinogen, clotting factors, and protein C inhibitors lead to increased thrombogenicity.16 Older maternal age, pregnancy-related hypertension, peripartum infections, and cesarean delivery also increase the risk of CVT.16
Patients with CVT associated with pregnancy have more acute onset of symptoms and neurological findings, and 2% to 10% less mortality than patients with CVT secondary to other etiologies. Cerebral venous thrombosis should be considered in the differential diagnosis of any pregnant or postpartum patient presenting with neurological symptoms.
Treatment of CVT
Regardless of underlying etiology or symptom duration, all patients with CVT should receive anticoagulation therapy. Unfractionated or low-molecular weight heparin has been shown to decrease thrombus propagation, increase the rate of recanalization, and improve long-term outcomes—even in the presence of intracranial hemorrhage.17 In the 30% to 40% of patients with CVT who present with intracranial hemorrhage, anticoagulation decreases mortality and is not associated with new or enlarged bleeding.17-19 Patients who continue to deteriorate despite anticoagulation therapy may benefit from endovascular thrombolysis or decompressive hemicraniectomy.20 In a study of patients with CVT, 80% had full recovery, 6% had minor disability, and 14% had a poor outcome.12
Conclusion
This case describes a patient with two risk factors for CVT, a life-threatening condition. It should be considered in patients with ET and/or pregnant and postpartum patients presenting with headache or other neurological symptoms. Magnetic resonance venography is the diagnostic imaging of choice as the CVT may not be visible on CT. All patients with thrombosis and ET should receive anticoagulation therapy and hydroxyurea to prevent cerebral herniation and death.
Dr Canders is a resident, department of emergency medicine, David Geffen School of Medicine, University of California, Los Angeles. Dr Taira is a clinical assistant professor, department of emergency medicine, Los Angeles County Hospital/University of Southern California, Los Angeles.
Disclosure: The authors report no financial disclosures or conflicts of interests related to this article.
- Schafer AI. Thrombocytosis and thrombocythemia. Blood Rev. 2001;15(4):159-166.
- Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995. Am J Hematol. 1999;61():10-15.
- Tefferi A. Recent progress in the pathogenesis and management of essential thrombocythemia. Leuk Res. 2001;25(5):369-377.
- Kesler A, Ellis MH, Manor Y, Gadoth N, Lishner M. Neurological complications of essential thrombocytosis (ET). Acta Neurol Scand. 2000;102(5):299-302.
- Cantú C, Barinagarrementeria F. Cerebral venous thrombosis associated with pregnancy and puerperium: Review of 67 cases. Stroke. 1993;24(12):1880-1884.
- Bazzan M, Tamponi G, Schinco P, Vaccarino A, Foli C, Gallone G, et al. Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia. Ann Hematol. 1999;78(12):539-543.
- Walther EU, Tiecks FP, Haberl RL. Cranial sinus thrombosis associated with essential thrombocythemia followed by heparin-associated thrombocytopenia. Neurology. 1996;47(1):300-301.
- McDonald TD, Tatemichi TK, Kranzler SJ, Chi L, Hilal SK, Mohr JP. Thrombosis of the superior sagittal sinus associated with essential thrombocytosis followed by MRI during anticoagulant therapy. Neurology. 1989;39(11):1554-1555.
- Iob I, Scanarini M, Andrioli GC, Pardatscher K. Thrombosis of the superior sagittal sinus associated with idiopathic thrombocytosis. Surg Neurol. 1979;11(6):439-41.
- Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995;332(17):1132-1136.
- de Bruijn SF, de Haan RJ, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. For The Cerebral Venous Sinus Thrombosis Study Group. J Neurol Neurosurg Psychiatry. 2001;70(1):105-108.
- Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35(3):664-670.
- DeLashaw MR, Vizioli TL, Jr, Counselman FL. Headache and seizure in a young woman postpartum. J Emerg Med. 2005;29(3):289-293.
- Bousser MG. Cerebral venous thrombosis: diagnosis and management. J Neurol. 2000;247(4):252-258.
- Grover M. Cerebral venous thrombosis as a cause of acute headache. J Am Board Fam Pract. 2004;17(4):295-298.
- Martinelli I, Sacchi E, Landi G, Taioli E, Duca F, Mannucci PM. High risk of cerebral venous thrombosis in carriers of prothrombic-gene mutation and in users of oral contraceptives. N Engl J Med. 1988;338(25):1793-1797.
- Star M, Flaster M. Advances and controversies in the management of cerebral venous thrombosis. Neurol Clin. 2013;31(3):765-783.
- Einhäupl KM, Villringer A, Meister W, Mehraein S, Garner C, Pellkofer M, et al. Heparin treatment in sinus venous thrombosis. Lancet. 1991;338(8767):597-600.
- Preter M, Tzourio C, Ameri A, Bousser MG. Long-term prognosis in cerebral venous thrombosis: follow-up of 77 patients. Stroke 1996;27(2):243-246.
- Coutinho JM, Stam J. How to treat cerebral venous and sinus thrombosis. J Thromb Haemost. 2010;8(5):877-883.
Case
At presentation, the patient was in no apparent distress and had normal vital signs, including normal blood pressure. On physical examination, she was alert and oriented, with unremarkable head, neck, fundoscopic, cardiac, pulmonary, skin, and neurological examinations. Laboratory results were only notable for a platelet count of 677 x 109/L. The remainder of the complete blood count, chemistry, and coagulation panels was all within normal limits. A noncontrast computed tomography (CT) of the brain was unremarkable. She was treated symptomatically with ketorolac, diphenhydramine, and metoclopramide, but received no relief.
Based on the patient’s history of ET and postpartum state, there was a high suspicion for cerebral venous thrombosis (CVT). Magnetic resonance imaging/magnetic resonance venography (MRI/MRV) of the brain was obtained, which revealed near-complete thrombosis of the vein of Galen and straight sinus (Figure). Neurology and hematology services were consulted emergently, and the patient was started on a heparin drip and admitted to the neurosurgical intensive care unit. Hydroxyurea was also given for cytoreduction, and platelets normalized to 305 x 109/L within 1 day of treatment. The patient’s neurological examination remained nonfocal, her headache gradually resolved, and she was discharged on hospital day 3 on oral anticoagulants.
Discussion
Essential thrombocytosis is a chronic myeloproliferative disorder characterized by clonal proliferation of the megakaryocyte cell line due to a defect in a pluripotent hematopoietic stem cell.1 The estimated annual incidence is 2.5 per 100,000 individuals.2 It affects patients of both genders, most commonly between ages 50 to 70 years.3
Up to 25% of patients with ET experience neurological complications, mainly occlusive strokes, chronic headache, and dizziness.4 Since pregnancy also increases thrombogenicity, and CVT occurs in 12 per 100,000 deliveries,5 it should be considered in any pregnant or postpartum patient with neurological symptoms.
Diagnosis of ET
Essential thrombocytosis is a diagnosis of exclusion, and no clinical or laboratory finding is diagnostic.1 Although patients with ET are at increased risk for bleeding, these events usually involve mucosal and cutaneous sites and are clinically insignificant. Thrombotic complications account for the majority of the morbidity and mortality associated with ET and can be both arterial and venous. In one case series, roughly 50% patients with ET experienced a thrombotic event within 9 years from diagnosis.6 Thrombotic complications are seen most commonly in patients younger than age 55 years at diagnosis. Common sites of thrombosis include the deep veins of the lower extremities, pulmonary vessels, hepatic vein, portal vein, digital microvasculature, and placenta.1
Despite the high rate of thrombotic complications in ET, there is little data on neurological complications and few reports of CVT in patients with ET.7-9 In one chart review of 70 patients with ET, researchers identified 18 patients who developed neurological complications,4 the most common of which was cerebrovascular accident/transient ischemic attack. Only one patient in the series had a CVT. The majority of patients with neurological events were female and no clinical or laboratory parameter predicted the risk of neurological event.
Thrombocytosis
Thrombocytosis is classified as either primary/essential or secondary/reactive. The majority of thrombocytosis is secondary/reactive and is caused by a variety of inflammatory conditions, such as infection and malignancy. Unlike ET, secondary thrombocytosis is not associated with any bleeding or thrombotic complications and does not require direct treatment.1
Treatment of ET
The decision to treat ET is based on clinical parameters and is usually reserved for patients at high-risk of thrombosis, including those with history of thrombosis or platelets >1,500 x 109/L.1 The first-line agent for treatment of essential thrombocytosis, hydroxyurea has been shown to decrease thrombotic episodes.10 Other cytoreductive agents include anagrilide and interferon-α, the use of which is considered safe in pregnancy.1
Cerebral Venous Thrombosis
Cerebral venous thrombosis is a rare but potentially fatal condition. Patients may present with headache (95%), seizures (47%), and vision changes (41%), and may or may not have focal or generalized neurological deficits.11 Thrombosis can lead to venous obstruction, resulting in increased intracranial pressure and ultimately cerebral herniation and death. Predisposing risk factors for CVT include thrombophilic and procoagulant disorders, maxillofacial infections, trauma, malignancy, and vasculitides. Among female patients with CVT, studies show approximately 50% were on oral contraceptives and 20% were pregnant or in the postpartum period at the time of the event. Nearly half of patients with CVT have multiple risk factors.12
Diagnosis of CVT
Diagnosis of CVT requires a high clinical suspicion. Lumbar puncture usually reveals high opening pressure with normal cerebrospinal fluid analysis.13 The classic finding of CVT on a contrast-enhanced CT scan is the “empty delta sign,” which is a central hypointensity within the superior sagittal sinus secondary due to slow/absent flow surrounded by contrast enhancement in a triangular shape. An ischemic infarction crossing arterial boundaries or near a venous sinus—with or without a hemorrhagic component—is also suggestive of CVT. However, CT scans are read as normal or indeterminate in up to 30% patients.14 Moreover, while CT venography may visualize the cerebral venous system, there is a false-negative rate of 25%.15 Therefore, MRI with MRV is the gold standard for diagnosis.
Pregnancy and CVT
As previously noted, pregnant or postpartum patients are at an increased risk of developing CVT. During pregnancy, decreased levels of protein S inhibitors and increased levels of fibrinogen, clotting factors, and protein C inhibitors lead to increased thrombogenicity.16 Older maternal age, pregnancy-related hypertension, peripartum infections, and cesarean delivery also increase the risk of CVT.16
Patients with CVT associated with pregnancy have more acute onset of symptoms and neurological findings, and 2% to 10% less mortality than patients with CVT secondary to other etiologies. Cerebral venous thrombosis should be considered in the differential diagnosis of any pregnant or postpartum patient presenting with neurological symptoms.
Treatment of CVT
Regardless of underlying etiology or symptom duration, all patients with CVT should receive anticoagulation therapy. Unfractionated or low-molecular weight heparin has been shown to decrease thrombus propagation, increase the rate of recanalization, and improve long-term outcomes—even in the presence of intracranial hemorrhage.17 In the 30% to 40% of patients with CVT who present with intracranial hemorrhage, anticoagulation decreases mortality and is not associated with new or enlarged bleeding.17-19 Patients who continue to deteriorate despite anticoagulation therapy may benefit from endovascular thrombolysis or decompressive hemicraniectomy.20 In a study of patients with CVT, 80% had full recovery, 6% had minor disability, and 14% had a poor outcome.12
Conclusion
This case describes a patient with two risk factors for CVT, a life-threatening condition. It should be considered in patients with ET and/or pregnant and postpartum patients presenting with headache or other neurological symptoms. Magnetic resonance venography is the diagnostic imaging of choice as the CVT may not be visible on CT. All patients with thrombosis and ET should receive anticoagulation therapy and hydroxyurea to prevent cerebral herniation and death.
Dr Canders is a resident, department of emergency medicine, David Geffen School of Medicine, University of California, Los Angeles. Dr Taira is a clinical assistant professor, department of emergency medicine, Los Angeles County Hospital/University of Southern California, Los Angeles.
Disclosure: The authors report no financial disclosures or conflicts of interests related to this article.
Case
At presentation, the patient was in no apparent distress and had normal vital signs, including normal blood pressure. On physical examination, she was alert and oriented, with unremarkable head, neck, fundoscopic, cardiac, pulmonary, skin, and neurological examinations. Laboratory results were only notable for a platelet count of 677 x 109/L. The remainder of the complete blood count, chemistry, and coagulation panels was all within normal limits. A noncontrast computed tomography (CT) of the brain was unremarkable. She was treated symptomatically with ketorolac, diphenhydramine, and metoclopramide, but received no relief.
Based on the patient’s history of ET and postpartum state, there was a high suspicion for cerebral venous thrombosis (CVT). Magnetic resonance imaging/magnetic resonance venography (MRI/MRV) of the brain was obtained, which revealed near-complete thrombosis of the vein of Galen and straight sinus (Figure). Neurology and hematology services were consulted emergently, and the patient was started on a heparin drip and admitted to the neurosurgical intensive care unit. Hydroxyurea was also given for cytoreduction, and platelets normalized to 305 x 109/L within 1 day of treatment. The patient’s neurological examination remained nonfocal, her headache gradually resolved, and she was discharged on hospital day 3 on oral anticoagulants.
Discussion
Essential thrombocytosis is a chronic myeloproliferative disorder characterized by clonal proliferation of the megakaryocyte cell line due to a defect in a pluripotent hematopoietic stem cell.1 The estimated annual incidence is 2.5 per 100,000 individuals.2 It affects patients of both genders, most commonly between ages 50 to 70 years.3
Up to 25% of patients with ET experience neurological complications, mainly occlusive strokes, chronic headache, and dizziness.4 Since pregnancy also increases thrombogenicity, and CVT occurs in 12 per 100,000 deliveries,5 it should be considered in any pregnant or postpartum patient with neurological symptoms.
Diagnosis of ET
Essential thrombocytosis is a diagnosis of exclusion, and no clinical or laboratory finding is diagnostic.1 Although patients with ET are at increased risk for bleeding, these events usually involve mucosal and cutaneous sites and are clinically insignificant. Thrombotic complications account for the majority of the morbidity and mortality associated with ET and can be both arterial and venous. In one case series, roughly 50% patients with ET experienced a thrombotic event within 9 years from diagnosis.6 Thrombotic complications are seen most commonly in patients younger than age 55 years at diagnosis. Common sites of thrombosis include the deep veins of the lower extremities, pulmonary vessels, hepatic vein, portal vein, digital microvasculature, and placenta.1
Despite the high rate of thrombotic complications in ET, there is little data on neurological complications and few reports of CVT in patients with ET.7-9 In one chart review of 70 patients with ET, researchers identified 18 patients who developed neurological complications,4 the most common of which was cerebrovascular accident/transient ischemic attack. Only one patient in the series had a CVT. The majority of patients with neurological events were female and no clinical or laboratory parameter predicted the risk of neurological event.
Thrombocytosis
Thrombocytosis is classified as either primary/essential or secondary/reactive. The majority of thrombocytosis is secondary/reactive and is caused by a variety of inflammatory conditions, such as infection and malignancy. Unlike ET, secondary thrombocytosis is not associated with any bleeding or thrombotic complications and does not require direct treatment.1
Treatment of ET
The decision to treat ET is based on clinical parameters and is usually reserved for patients at high-risk of thrombosis, including those with history of thrombosis or platelets >1,500 x 109/L.1 The first-line agent for treatment of essential thrombocytosis, hydroxyurea has been shown to decrease thrombotic episodes.10 Other cytoreductive agents include anagrilide and interferon-α, the use of which is considered safe in pregnancy.1
Cerebral Venous Thrombosis
Cerebral venous thrombosis is a rare but potentially fatal condition. Patients may present with headache (95%), seizures (47%), and vision changes (41%), and may or may not have focal or generalized neurological deficits.11 Thrombosis can lead to venous obstruction, resulting in increased intracranial pressure and ultimately cerebral herniation and death. Predisposing risk factors for CVT include thrombophilic and procoagulant disorders, maxillofacial infections, trauma, malignancy, and vasculitides. Among female patients with CVT, studies show approximately 50% were on oral contraceptives and 20% were pregnant or in the postpartum period at the time of the event. Nearly half of patients with CVT have multiple risk factors.12
Diagnosis of CVT
Diagnosis of CVT requires a high clinical suspicion. Lumbar puncture usually reveals high opening pressure with normal cerebrospinal fluid analysis.13 The classic finding of CVT on a contrast-enhanced CT scan is the “empty delta sign,” which is a central hypointensity within the superior sagittal sinus secondary due to slow/absent flow surrounded by contrast enhancement in a triangular shape. An ischemic infarction crossing arterial boundaries or near a venous sinus—with or without a hemorrhagic component—is also suggestive of CVT. However, CT scans are read as normal or indeterminate in up to 30% patients.14 Moreover, while CT venography may visualize the cerebral venous system, there is a false-negative rate of 25%.15 Therefore, MRI with MRV is the gold standard for diagnosis.
Pregnancy and CVT
As previously noted, pregnant or postpartum patients are at an increased risk of developing CVT. During pregnancy, decreased levels of protein S inhibitors and increased levels of fibrinogen, clotting factors, and protein C inhibitors lead to increased thrombogenicity.16 Older maternal age, pregnancy-related hypertension, peripartum infections, and cesarean delivery also increase the risk of CVT.16
Patients with CVT associated with pregnancy have more acute onset of symptoms and neurological findings, and 2% to 10% less mortality than patients with CVT secondary to other etiologies. Cerebral venous thrombosis should be considered in the differential diagnosis of any pregnant or postpartum patient presenting with neurological symptoms.
Treatment of CVT
Regardless of underlying etiology or symptom duration, all patients with CVT should receive anticoagulation therapy. Unfractionated or low-molecular weight heparin has been shown to decrease thrombus propagation, increase the rate of recanalization, and improve long-term outcomes—even in the presence of intracranial hemorrhage.17 In the 30% to 40% of patients with CVT who present with intracranial hemorrhage, anticoagulation decreases mortality and is not associated with new or enlarged bleeding.17-19 Patients who continue to deteriorate despite anticoagulation therapy may benefit from endovascular thrombolysis or decompressive hemicraniectomy.20 In a study of patients with CVT, 80% had full recovery, 6% had minor disability, and 14% had a poor outcome.12
Conclusion
This case describes a patient with two risk factors for CVT, a life-threatening condition. It should be considered in patients with ET and/or pregnant and postpartum patients presenting with headache or other neurological symptoms. Magnetic resonance venography is the diagnostic imaging of choice as the CVT may not be visible on CT. All patients with thrombosis and ET should receive anticoagulation therapy and hydroxyurea to prevent cerebral herniation and death.
Dr Canders is a resident, department of emergency medicine, David Geffen School of Medicine, University of California, Los Angeles. Dr Taira is a clinical assistant professor, department of emergency medicine, Los Angeles County Hospital/University of Southern California, Los Angeles.
Disclosure: The authors report no financial disclosures or conflicts of interests related to this article.
- Schafer AI. Thrombocytosis and thrombocythemia. Blood Rev. 2001;15(4):159-166.
- Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995. Am J Hematol. 1999;61():10-15.
- Tefferi A. Recent progress in the pathogenesis and management of essential thrombocythemia. Leuk Res. 2001;25(5):369-377.
- Kesler A, Ellis MH, Manor Y, Gadoth N, Lishner M. Neurological complications of essential thrombocytosis (ET). Acta Neurol Scand. 2000;102(5):299-302.
- Cantú C, Barinagarrementeria F. Cerebral venous thrombosis associated with pregnancy and puerperium: Review of 67 cases. Stroke. 1993;24(12):1880-1884.
- Bazzan M, Tamponi G, Schinco P, Vaccarino A, Foli C, Gallone G, et al. Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia. Ann Hematol. 1999;78(12):539-543.
- Walther EU, Tiecks FP, Haberl RL. Cranial sinus thrombosis associated with essential thrombocythemia followed by heparin-associated thrombocytopenia. Neurology. 1996;47(1):300-301.
- McDonald TD, Tatemichi TK, Kranzler SJ, Chi L, Hilal SK, Mohr JP. Thrombosis of the superior sagittal sinus associated with essential thrombocytosis followed by MRI during anticoagulant therapy. Neurology. 1989;39(11):1554-1555.
- Iob I, Scanarini M, Andrioli GC, Pardatscher K. Thrombosis of the superior sagittal sinus associated with idiopathic thrombocytosis. Surg Neurol. 1979;11(6):439-41.
- Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995;332(17):1132-1136.
- de Bruijn SF, de Haan RJ, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. For The Cerebral Venous Sinus Thrombosis Study Group. J Neurol Neurosurg Psychiatry. 2001;70(1):105-108.
- Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35(3):664-670.
- DeLashaw MR, Vizioli TL, Jr, Counselman FL. Headache and seizure in a young woman postpartum. J Emerg Med. 2005;29(3):289-293.
- Bousser MG. Cerebral venous thrombosis: diagnosis and management. J Neurol. 2000;247(4):252-258.
- Grover M. Cerebral venous thrombosis as a cause of acute headache. J Am Board Fam Pract. 2004;17(4):295-298.
- Martinelli I, Sacchi E, Landi G, Taioli E, Duca F, Mannucci PM. High risk of cerebral venous thrombosis in carriers of prothrombic-gene mutation and in users of oral contraceptives. N Engl J Med. 1988;338(25):1793-1797.
- Star M, Flaster M. Advances and controversies in the management of cerebral venous thrombosis. Neurol Clin. 2013;31(3):765-783.
- Einhäupl KM, Villringer A, Meister W, Mehraein S, Garner C, Pellkofer M, et al. Heparin treatment in sinus venous thrombosis. Lancet. 1991;338(8767):597-600.
- Preter M, Tzourio C, Ameri A, Bousser MG. Long-term prognosis in cerebral venous thrombosis: follow-up of 77 patients. Stroke 1996;27(2):243-246.
- Coutinho JM, Stam J. How to treat cerebral venous and sinus thrombosis. J Thromb Haemost. 2010;8(5):877-883.
- Schafer AI. Thrombocytosis and thrombocythemia. Blood Rev. 2001;15(4):159-166.
- Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995. Am J Hematol. 1999;61():10-15.
- Tefferi A. Recent progress in the pathogenesis and management of essential thrombocythemia. Leuk Res. 2001;25(5):369-377.
- Kesler A, Ellis MH, Manor Y, Gadoth N, Lishner M. Neurological complications of essential thrombocytosis (ET). Acta Neurol Scand. 2000;102(5):299-302.
- Cantú C, Barinagarrementeria F. Cerebral venous thrombosis associated with pregnancy and puerperium: Review of 67 cases. Stroke. 1993;24(12):1880-1884.
- Bazzan M, Tamponi G, Schinco P, Vaccarino A, Foli C, Gallone G, et al. Thrombosis-free survival and life expectancy in 187 consecutive patients with essential thrombocythemia. Ann Hematol. 1999;78(12):539-543.
- Walther EU, Tiecks FP, Haberl RL. Cranial sinus thrombosis associated with essential thrombocythemia followed by heparin-associated thrombocytopenia. Neurology. 1996;47(1):300-301.
- McDonald TD, Tatemichi TK, Kranzler SJ, Chi L, Hilal SK, Mohr JP. Thrombosis of the superior sagittal sinus associated with essential thrombocytosis followed by MRI during anticoagulant therapy. Neurology. 1989;39(11):1554-1555.
- Iob I, Scanarini M, Andrioli GC, Pardatscher K. Thrombosis of the superior sagittal sinus associated with idiopathic thrombocytosis. Surg Neurol. 1979;11(6):439-41.
- Cortelazzo S, Finazzi G, Ruggeri M, Vestri O, Galli M, Rodeghiero F, et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995;332(17):1132-1136.
- de Bruijn SF, de Haan RJ, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. For The Cerebral Venous Sinus Thrombosis Study Group. J Neurol Neurosurg Psychiatry. 2001;70(1):105-108.
- Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35(3):664-670.
- DeLashaw MR, Vizioli TL, Jr, Counselman FL. Headache and seizure in a young woman postpartum. J Emerg Med. 2005;29(3):289-293.
- Bousser MG. Cerebral venous thrombosis: diagnosis and management. J Neurol. 2000;247(4):252-258.
- Grover M. Cerebral venous thrombosis as a cause of acute headache. J Am Board Fam Pract. 2004;17(4):295-298.
- Martinelli I, Sacchi E, Landi G, Taioli E, Duca F, Mannucci PM. High risk of cerebral venous thrombosis in carriers of prothrombic-gene mutation and in users of oral contraceptives. N Engl J Med. 1988;338(25):1793-1797.
- Star M, Flaster M. Advances and controversies in the management of cerebral venous thrombosis. Neurol Clin. 2013;31(3):765-783.
- Einhäupl KM, Villringer A, Meister W, Mehraein S, Garner C, Pellkofer M, et al. Heparin treatment in sinus venous thrombosis. Lancet. 1991;338(8767):597-600.
- Preter M, Tzourio C, Ameri A, Bousser MG. Long-term prognosis in cerebral venous thrombosis: follow-up of 77 patients. Stroke 1996;27(2):243-246.
- Coutinho JM, Stam J. How to treat cerebral venous and sinus thrombosis. J Thromb Haemost. 2010;8(5):877-883.
An 18-year-old woman with hepatic cysts
An 18-year-old woman presents with 3 days of epigastric abdominal pain, with no fever or constitutional symptoms. She was born in the United States and reports yearly trips since age 3 to her family’s farm in a rural area of Mexico, where she is exposed to dogs and horses.
Ultrasonography reveals two large hepatic cysts measuring 5.8 × 6.8 × 5.4 cm and 5.3 × 4.9 × 7 cm, with thickened walls and internal debris (Figure 1). The debris moves to dependent areas when the patient is asked to move onto her side.
Laboratory values at the time of presentation are as follows:
- White blood cell count 11.9 × 109/L (reference range 4.5–11.0), with 20% eosinophils
- Alkaline phosphatase 116 U/L (30–100)
- Total protein 7.3 g/dL (6.0–8.0)
- Albumin 4.3 g/dL (3.5–5.0)
- Aspartate aminotransferase (AST) 19 U/L (10–40)
- Alanine aminotransferase (ALT) 18 U/L (5–40)
- Total bilirubin 0.2 mg/dL (0.3–1.2)
- Direct bilirubin 0.1 mg/dL (0.1–0.3)
- Echinococcus antibody (IgG) testing is positive.
CYSTIC ECHINOCOCCOSIS
The two clinically relevant species of Echinococcus that cause human infection are E granulosus (in cystic echinococcosis) and E multilocularis (in alveolar echinococcosis). Based on clinical and radiographic findings, hepatic hydatid disease from cystic echinococcosis can usually be differentiated from the alveolar form.
E granulosus is a parasitic tapeworm that requires an intermediate host (sheep, goats, cows) and a definite host (dogs, foxes, and related species) for its life cycle. Humans become infected when they ingest food contaminated with feces that contain the eggs of the tapeworm or when they handle carnivorous animals, usually dogs, and accidentally ingest the tapeworm eggs. Once ingested, the egg releases an oncosphere that penetrates the intestinal wall, enters the circulation, and develops into a cyst, most often in the liver and the lungs.1 Human-to-human transmission does not occur.2
Hydatid cysts grow slowly, at a rate of 1 to 50 mm per year,3 so most patients remain asymptomatic for several years. Symptoms occur when a cyst ruptures or impinges on structures.3 Fever and constitutional symptoms usually occur only if there is rupture or bacterial superinfection of the cyst. Tests of liver function tend to be normal unless a cyst obstructs biliary flow. Eosinophilia occurs in 25% of patients.1 Eosinophilia along with the abrupt onset of abdominal pain suggests cyst rupture.
Making the diagnosis
Diagnosis is made by characteristic ultrasonographic findings and by serologic testing. Antibody assays for Echinococcus include indirect hemagglutination, enzyme-linked immunosorbent assay, and latex agglutination. However, these serologic antibody assays for immunoglobulin G cross-react to different echinococcal species as well as to other helminthic infections. Specific serologic studies such as an enzyme-linked immunosorbent assay for E multilocularis are available to confirm the species of Echinococcus but are only used to distinguish cystic echinococcosis from alveolar echinococcosis.
Treatment options
Treatment options include surgery, percutaneous procedures, drug therapy, and observation.
Currently, there is no clear consensus on treatment. To guide treatment decisions, the World Health Organization Informal Working Group on Echinococcosis (WHO-IWGE) recommends management of hepatic hydatid cysts based on classification, size, symptoms, location, and available resources.3
Two percutaneous treatments are aspiration, injection, and re-aspiration to destroy the germinal matrix, and percutaneous therapy to destroy the endocyst. Percutaneous aspiration, injection, and re-aspiration is increasingly used as the first-line treatment for single or easily accessible cysts and for patients who cannot undergo surgery. Surgery is considered for multiple cysts, large cysts, and cysts not easily accessible with a percutaneous technique.3 Complication rates and length of hospital stay with percutaneous aspiration are lower than with surgery.4 Observation is recommended for small, asymptomatic, inactive cysts.
Leakage of cyst contents during surgical or percutaneous intervention or spontaneous rupture can cause a recurrence,5 and anaphylaxis is a potential complication of cyst rupture.1 For this reason, giving oral albendazole (Albenza) is recommended before any intervention. Sterilization of the cyst contents with a protoscolicidal agent (20% NaCl) before evacuation of cyst contents is also standard practice.
The rate of cyst recurrence is 16.2% with open surgery and 3.5% with percutaneous intervention.6 A higher incidence of recurrence in patients who undergo surgical cystectomy likely reflects the more complicated and active nature of the cysts in patients who undergo surgery.6
Albendazole is the drug of choice for hepatic hydatid disease.3 The optimal duration of treatment is unclear but should be guided by a combination of clinical response, medication side effects, serologic titers, and imaging. The most common adverse effects of albendazole are hepatotoxicity, abdominal pain, and nausea.
OUR PATIENT’S DIAGNOSIS AND TREATMENT
In our patient, ultrasonography confirmed the diagnosis of cystic echinococcosis by the finding of active anechoic cysts with echogenic internal debris and with a well-delineated cyst wall. The WHO-IWGE classification was CE1, ie, active anechoic cysts with internal echogenic debris.
Our patient underwent surgical rather than percutaneous cystectomy because of concern about possible cyst leakage, since she had presented with the acute onset of pain and eosinophilia. We were also concerned about the subdiaphragmatic location of the larger cyst, which could have been difficult to reach percutaneously.
Open total pericystectomy involved opening the cyst cavity, sterilizing the contents with 20% NaCl, evacuating the cyst contents, and removing the cyst tissue. Two large cysts were excised and sent for histologic examination, which confirmed E granulosus. Percutaneous aspiration was necessary 4 months later because of a recurrence, and albendazole 400 mg twice daily was continued for another 5 months. Ultrasonography 3 years later showed no evidence of echinococcal cysts, and her antibody titers remain undetectable.
- McManus DP, Gray DJ, Zhang W, Yang Y. Diagnosis, treatment, and management of echinococcosis. BMJ 2012; 344:e3866.
- McManus DP, Zhang W, Li J, Bartley PB. Echinococcosis. Lancet 2003; 362:1295–1304.
- Brunetti E, Kern P, Vuitton DA; Writing Panel for the WHO-IWGE. Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop 2010; 114:1–16.
- Khuroo MS, Wani NA, Javid G, et al. Percutaneous drainage compared with surgery for hepatic hydatid cysts. N Engl J Med 1997; 337:881–887.
- Kayaalp C, Sengul N, Akoglu M. Importance of cyst content in hydatid liver surgery. Arch Surg 2002; 137:159–163.
- Yagci G, Ustunsoz B, Kaymakcioglu N, et al. Results of surgical, laparoscopic, and percutaneous treatment for hydatid disease of the liver: 10 years experience with 355 patients. World J Surg 2005; 29:1670–1679.
An 18-year-old woman presents with 3 days of epigastric abdominal pain, with no fever or constitutional symptoms. She was born in the United States and reports yearly trips since age 3 to her family’s farm in a rural area of Mexico, where she is exposed to dogs and horses.
Ultrasonography reveals two large hepatic cysts measuring 5.8 × 6.8 × 5.4 cm and 5.3 × 4.9 × 7 cm, with thickened walls and internal debris (Figure 1). The debris moves to dependent areas when the patient is asked to move onto her side.
Laboratory values at the time of presentation are as follows:
- White blood cell count 11.9 × 109/L (reference range 4.5–11.0), with 20% eosinophils
- Alkaline phosphatase 116 U/L (30–100)
- Total protein 7.3 g/dL (6.0–8.0)
- Albumin 4.3 g/dL (3.5–5.0)
- Aspartate aminotransferase (AST) 19 U/L (10–40)
- Alanine aminotransferase (ALT) 18 U/L (5–40)
- Total bilirubin 0.2 mg/dL (0.3–1.2)
- Direct bilirubin 0.1 mg/dL (0.1–0.3)
- Echinococcus antibody (IgG) testing is positive.
CYSTIC ECHINOCOCCOSIS
The two clinically relevant species of Echinococcus that cause human infection are E granulosus (in cystic echinococcosis) and E multilocularis (in alveolar echinococcosis). Based on clinical and radiographic findings, hepatic hydatid disease from cystic echinococcosis can usually be differentiated from the alveolar form.
E granulosus is a parasitic tapeworm that requires an intermediate host (sheep, goats, cows) and a definite host (dogs, foxes, and related species) for its life cycle. Humans become infected when they ingest food contaminated with feces that contain the eggs of the tapeworm or when they handle carnivorous animals, usually dogs, and accidentally ingest the tapeworm eggs. Once ingested, the egg releases an oncosphere that penetrates the intestinal wall, enters the circulation, and develops into a cyst, most often in the liver and the lungs.1 Human-to-human transmission does not occur.2
Hydatid cysts grow slowly, at a rate of 1 to 50 mm per year,3 so most patients remain asymptomatic for several years. Symptoms occur when a cyst ruptures or impinges on structures.3 Fever and constitutional symptoms usually occur only if there is rupture or bacterial superinfection of the cyst. Tests of liver function tend to be normal unless a cyst obstructs biliary flow. Eosinophilia occurs in 25% of patients.1 Eosinophilia along with the abrupt onset of abdominal pain suggests cyst rupture.
Making the diagnosis
Diagnosis is made by characteristic ultrasonographic findings and by serologic testing. Antibody assays for Echinococcus include indirect hemagglutination, enzyme-linked immunosorbent assay, and latex agglutination. However, these serologic antibody assays for immunoglobulin G cross-react to different echinococcal species as well as to other helminthic infections. Specific serologic studies such as an enzyme-linked immunosorbent assay for E multilocularis are available to confirm the species of Echinococcus but are only used to distinguish cystic echinococcosis from alveolar echinococcosis.
Treatment options
Treatment options include surgery, percutaneous procedures, drug therapy, and observation.
Currently, there is no clear consensus on treatment. To guide treatment decisions, the World Health Organization Informal Working Group on Echinococcosis (WHO-IWGE) recommends management of hepatic hydatid cysts based on classification, size, symptoms, location, and available resources.3
Two percutaneous treatments are aspiration, injection, and re-aspiration to destroy the germinal matrix, and percutaneous therapy to destroy the endocyst. Percutaneous aspiration, injection, and re-aspiration is increasingly used as the first-line treatment for single or easily accessible cysts and for patients who cannot undergo surgery. Surgery is considered for multiple cysts, large cysts, and cysts not easily accessible with a percutaneous technique.3 Complication rates and length of hospital stay with percutaneous aspiration are lower than with surgery.4 Observation is recommended for small, asymptomatic, inactive cysts.
Leakage of cyst contents during surgical or percutaneous intervention or spontaneous rupture can cause a recurrence,5 and anaphylaxis is a potential complication of cyst rupture.1 For this reason, giving oral albendazole (Albenza) is recommended before any intervention. Sterilization of the cyst contents with a protoscolicidal agent (20% NaCl) before evacuation of cyst contents is also standard practice.
The rate of cyst recurrence is 16.2% with open surgery and 3.5% with percutaneous intervention.6 A higher incidence of recurrence in patients who undergo surgical cystectomy likely reflects the more complicated and active nature of the cysts in patients who undergo surgery.6
Albendazole is the drug of choice for hepatic hydatid disease.3 The optimal duration of treatment is unclear but should be guided by a combination of clinical response, medication side effects, serologic titers, and imaging. The most common adverse effects of albendazole are hepatotoxicity, abdominal pain, and nausea.
OUR PATIENT’S DIAGNOSIS AND TREATMENT
In our patient, ultrasonography confirmed the diagnosis of cystic echinococcosis by the finding of active anechoic cysts with echogenic internal debris and with a well-delineated cyst wall. The WHO-IWGE classification was CE1, ie, active anechoic cysts with internal echogenic debris.
Our patient underwent surgical rather than percutaneous cystectomy because of concern about possible cyst leakage, since she had presented with the acute onset of pain and eosinophilia. We were also concerned about the subdiaphragmatic location of the larger cyst, which could have been difficult to reach percutaneously.
Open total pericystectomy involved opening the cyst cavity, sterilizing the contents with 20% NaCl, evacuating the cyst contents, and removing the cyst tissue. Two large cysts were excised and sent for histologic examination, which confirmed E granulosus. Percutaneous aspiration was necessary 4 months later because of a recurrence, and albendazole 400 mg twice daily was continued for another 5 months. Ultrasonography 3 years later showed no evidence of echinococcal cysts, and her antibody titers remain undetectable.
An 18-year-old woman presents with 3 days of epigastric abdominal pain, with no fever or constitutional symptoms. She was born in the United States and reports yearly trips since age 3 to her family’s farm in a rural area of Mexico, where she is exposed to dogs and horses.
Ultrasonography reveals two large hepatic cysts measuring 5.8 × 6.8 × 5.4 cm and 5.3 × 4.9 × 7 cm, with thickened walls and internal debris (Figure 1). The debris moves to dependent areas when the patient is asked to move onto her side.
Laboratory values at the time of presentation are as follows:
- White blood cell count 11.9 × 109/L (reference range 4.5–11.0), with 20% eosinophils
- Alkaline phosphatase 116 U/L (30–100)
- Total protein 7.3 g/dL (6.0–8.0)
- Albumin 4.3 g/dL (3.5–5.0)
- Aspartate aminotransferase (AST) 19 U/L (10–40)
- Alanine aminotransferase (ALT) 18 U/L (5–40)
- Total bilirubin 0.2 mg/dL (0.3–1.2)
- Direct bilirubin 0.1 mg/dL (0.1–0.3)
- Echinococcus antibody (IgG) testing is positive.
CYSTIC ECHINOCOCCOSIS
The two clinically relevant species of Echinococcus that cause human infection are E granulosus (in cystic echinococcosis) and E multilocularis (in alveolar echinococcosis). Based on clinical and radiographic findings, hepatic hydatid disease from cystic echinococcosis can usually be differentiated from the alveolar form.
E granulosus is a parasitic tapeworm that requires an intermediate host (sheep, goats, cows) and a definite host (dogs, foxes, and related species) for its life cycle. Humans become infected when they ingest food contaminated with feces that contain the eggs of the tapeworm or when they handle carnivorous animals, usually dogs, and accidentally ingest the tapeworm eggs. Once ingested, the egg releases an oncosphere that penetrates the intestinal wall, enters the circulation, and develops into a cyst, most often in the liver and the lungs.1 Human-to-human transmission does not occur.2
Hydatid cysts grow slowly, at a rate of 1 to 50 mm per year,3 so most patients remain asymptomatic for several years. Symptoms occur when a cyst ruptures or impinges on structures.3 Fever and constitutional symptoms usually occur only if there is rupture or bacterial superinfection of the cyst. Tests of liver function tend to be normal unless a cyst obstructs biliary flow. Eosinophilia occurs in 25% of patients.1 Eosinophilia along with the abrupt onset of abdominal pain suggests cyst rupture.
Making the diagnosis
Diagnosis is made by characteristic ultrasonographic findings and by serologic testing. Antibody assays for Echinococcus include indirect hemagglutination, enzyme-linked immunosorbent assay, and latex agglutination. However, these serologic antibody assays for immunoglobulin G cross-react to different echinococcal species as well as to other helminthic infections. Specific serologic studies such as an enzyme-linked immunosorbent assay for E multilocularis are available to confirm the species of Echinococcus but are only used to distinguish cystic echinococcosis from alveolar echinococcosis.
Treatment options
Treatment options include surgery, percutaneous procedures, drug therapy, and observation.
Currently, there is no clear consensus on treatment. To guide treatment decisions, the World Health Organization Informal Working Group on Echinococcosis (WHO-IWGE) recommends management of hepatic hydatid cysts based on classification, size, symptoms, location, and available resources.3
Two percutaneous treatments are aspiration, injection, and re-aspiration to destroy the germinal matrix, and percutaneous therapy to destroy the endocyst. Percutaneous aspiration, injection, and re-aspiration is increasingly used as the first-line treatment for single or easily accessible cysts and for patients who cannot undergo surgery. Surgery is considered for multiple cysts, large cysts, and cysts not easily accessible with a percutaneous technique.3 Complication rates and length of hospital stay with percutaneous aspiration are lower than with surgery.4 Observation is recommended for small, asymptomatic, inactive cysts.
Leakage of cyst contents during surgical or percutaneous intervention or spontaneous rupture can cause a recurrence,5 and anaphylaxis is a potential complication of cyst rupture.1 For this reason, giving oral albendazole (Albenza) is recommended before any intervention. Sterilization of the cyst contents with a protoscolicidal agent (20% NaCl) before evacuation of cyst contents is also standard practice.
The rate of cyst recurrence is 16.2% with open surgery and 3.5% with percutaneous intervention.6 A higher incidence of recurrence in patients who undergo surgical cystectomy likely reflects the more complicated and active nature of the cysts in patients who undergo surgery.6
Albendazole is the drug of choice for hepatic hydatid disease.3 The optimal duration of treatment is unclear but should be guided by a combination of clinical response, medication side effects, serologic titers, and imaging. The most common adverse effects of albendazole are hepatotoxicity, abdominal pain, and nausea.
OUR PATIENT’S DIAGNOSIS AND TREATMENT
In our patient, ultrasonography confirmed the diagnosis of cystic echinococcosis by the finding of active anechoic cysts with echogenic internal debris and with a well-delineated cyst wall. The WHO-IWGE classification was CE1, ie, active anechoic cysts with internal echogenic debris.
Our patient underwent surgical rather than percutaneous cystectomy because of concern about possible cyst leakage, since she had presented with the acute onset of pain and eosinophilia. We were also concerned about the subdiaphragmatic location of the larger cyst, which could have been difficult to reach percutaneously.
Open total pericystectomy involved opening the cyst cavity, sterilizing the contents with 20% NaCl, evacuating the cyst contents, and removing the cyst tissue. Two large cysts were excised and sent for histologic examination, which confirmed E granulosus. Percutaneous aspiration was necessary 4 months later because of a recurrence, and albendazole 400 mg twice daily was continued for another 5 months. Ultrasonography 3 years later showed no evidence of echinococcal cysts, and her antibody titers remain undetectable.
- McManus DP, Gray DJ, Zhang W, Yang Y. Diagnosis, treatment, and management of echinococcosis. BMJ 2012; 344:e3866.
- McManus DP, Zhang W, Li J, Bartley PB. Echinococcosis. Lancet 2003; 362:1295–1304.
- Brunetti E, Kern P, Vuitton DA; Writing Panel for the WHO-IWGE. Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop 2010; 114:1–16.
- Khuroo MS, Wani NA, Javid G, et al. Percutaneous drainage compared with surgery for hepatic hydatid cysts. N Engl J Med 1997; 337:881–887.
- Kayaalp C, Sengul N, Akoglu M. Importance of cyst content in hydatid liver surgery. Arch Surg 2002; 137:159–163.
- Yagci G, Ustunsoz B, Kaymakcioglu N, et al. Results of surgical, laparoscopic, and percutaneous treatment for hydatid disease of the liver: 10 years experience with 355 patients. World J Surg 2005; 29:1670–1679.
- McManus DP, Gray DJ, Zhang W, Yang Y. Diagnosis, treatment, and management of echinococcosis. BMJ 2012; 344:e3866.
- McManus DP, Zhang W, Li J, Bartley PB. Echinococcosis. Lancet 2003; 362:1295–1304.
- Brunetti E, Kern P, Vuitton DA; Writing Panel for the WHO-IWGE. Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop 2010; 114:1–16.
- Khuroo MS, Wani NA, Javid G, et al. Percutaneous drainage compared with surgery for hepatic hydatid cysts. N Engl J Med 1997; 337:881–887.
- Kayaalp C, Sengul N, Akoglu M. Importance of cyst content in hydatid liver surgery. Arch Surg 2002; 137:159–163.
- Yagci G, Ustunsoz B, Kaymakcioglu N, et al. Results of surgical, laparoscopic, and percutaneous treatment for hydatid disease of the liver: 10 years experience with 355 patients. World J Surg 2005; 29:1670–1679.
Syncope during a pharmacologic nuclear stress test
A 60-year-old woman was referred for pharmacologic nuclear stress testing before treatment for breast cancer. She had hypertension, diabetes mellitus, coronary artery disease, and a remote history of stroke, and she was taking clonidine (Catapres), labetalol (Normodyne, Trandate), furosemide (Lasix), hydralazine, valsartan (Diovan), insulin, and the aspirin-dipyridamole combination Aggrenox. Her vital signs and electrocardiogram before the stress test were normal.
The stress test was started with a standard protocol of adenosine (Adenoscan) infused intravenously over 4 minutes. For the first 2 minutes, she was stable and had no symptoms, but then sinus pauses and second-degree atrioventricular block type 2 developed, after which her heart stopped beating (Figure 1). The infusion was immediately stopped, but she became unresponsive and remained pulseless.
Cardiopulmonary resuscitation was started, aminophylline 100 mg was given intravenously, and she regained a pulse and blood pressure within a few minutes. She was then transferred to the emergency room, where she returned to her baseline clinical and neurologic status without symptoms.
AN UNRECOGNIZED DRUG INTERACTION
Asystole occurred in this patient because of the interaction of intravenous adenosine with the dipyridamole in the medication Aggrenox. Although adenosine, given during pharmacologic stress testing, is known to interact with various medications, the potential for this interaction may be overlooked if the culprit is present in a combination drug. Aggrenox is commonly given for secondary stroke prevention and should be discontinued before pharmacologic nuclear stress testing.
Pharmacologic stress testing involves two commonly used stress agents, adenosine and regadenoson (Lexiscan), which cause coronary vasodilation through their action on A2A receptors in the heart. Coronary vasodilation results in flow heterogeneity in the region of a stenotic artery, which can be detected with nuclear perfusion agents. In addition, adenosine has a short-lived effect on the A1 receptors that block atrioventricular conduction.1
Dipyridamole (Persantine) is contraindicated when either adenosine or regadenoson is used. Dipyridamole enhances the effect of exogenous and endogenous adenosine by inhibiting its uptake by cardiac cells, thus enhancing the action of these coronary vasodilators.2 Atrioventricular block is common during adenosine stress testing but is transient because adenosine has a short half-life (< 10 seconds), and complete heart block or asystole, as seen in this patient, is rare. Giving intravenous adenosine or regadenoson to patients on dipyridamole may have a marked effect on adenosine receptors, so that profound bradycardia and even asystole leading to cardiac collapse may occur. No data are available on the specific interaction of dipyridamole and regadenoson.
Even though the pharmacodynamics of the interaction between dipyridamole and adenosine are known,3 few reports are available detailing serious adverse events. The contraindication to pharmacologic stress testing in patients taking dipyridamole is noted in the American Society of Nuclear Cardiology Guidelines for stress protocols,4 which advise discontinuing dipyridamole-containing drugs at least 48 hours before the use of adenosine or regadenoson. Similarly, the American Heart Association guidelines5 for the management of supraventricular tachycardia recommend an initial dose of 3 mg of adenosine rather than 6 mg in patients who have been taking dipyridamole.
The dose of aminophylline for reversing the adverse effects of adenosine or regadenoson is 50 to 250 mg intravenously over 30 to 60 seconds. But since these adverse effects are short-lived once the infusion is stopped, aminophylline is usually given only if the adverse effects are severe, as in this patient.
Pharmacologic nuclear stress testing with adenosine receptor agonists (eg, adenosine or regadenoson) is contraindicated in patients taking dipyridamole or the combination pill Aggrenox because of the potential for profound bradyarrhythmias or asystole.
- Zoghbi GJ, Iskandrian AE. Selective adenosine agonists and myocardial perfusion imaging. J Nucl Cardiol 2012; 19:126–141.
- Lerman BB, Wesley RC, Belardinelli L. Electrophysiologic effects of dipyridamole on atrioventricular nodal conduction and supraventricular tachycardia. Role of endogenous adenosine. Circulation 1989; 80:1536–1543.
- Biaggioni I, Onrot J, Hollister AS, Robertson D. Cardiovascular effects of adenosine infusion in man and their modulation by dipyridamole. Life Sci 1986; 39:2229–2236.
- Henzlova MJ, Cerqueira MD, Mahmarian JJ, Yao SS; Quality Assurance Committee of the American Society of Nuclear Cardiology. Stress protocols and tracers. J Nucl Cardiol 2006; 13:e80–e90.
- ECC Committee, Subcommittees and Task Forces of the American Heart Association. 2005 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Part 7.3: management of symptomatic bradycardia and tachycardia. Circulation 2005; 112(suppl 24):IV67–IV77.
A 60-year-old woman was referred for pharmacologic nuclear stress testing before treatment for breast cancer. She had hypertension, diabetes mellitus, coronary artery disease, and a remote history of stroke, and she was taking clonidine (Catapres), labetalol (Normodyne, Trandate), furosemide (Lasix), hydralazine, valsartan (Diovan), insulin, and the aspirin-dipyridamole combination Aggrenox. Her vital signs and electrocardiogram before the stress test were normal.
The stress test was started with a standard protocol of adenosine (Adenoscan) infused intravenously over 4 minutes. For the first 2 minutes, she was stable and had no symptoms, but then sinus pauses and second-degree atrioventricular block type 2 developed, after which her heart stopped beating (Figure 1). The infusion was immediately stopped, but she became unresponsive and remained pulseless.
Cardiopulmonary resuscitation was started, aminophylline 100 mg was given intravenously, and she regained a pulse and blood pressure within a few minutes. She was then transferred to the emergency room, where she returned to her baseline clinical and neurologic status without symptoms.
AN UNRECOGNIZED DRUG INTERACTION
Asystole occurred in this patient because of the interaction of intravenous adenosine with the dipyridamole in the medication Aggrenox. Although adenosine, given during pharmacologic stress testing, is known to interact with various medications, the potential for this interaction may be overlooked if the culprit is present in a combination drug. Aggrenox is commonly given for secondary stroke prevention and should be discontinued before pharmacologic nuclear stress testing.
Pharmacologic stress testing involves two commonly used stress agents, adenosine and regadenoson (Lexiscan), which cause coronary vasodilation through their action on A2A receptors in the heart. Coronary vasodilation results in flow heterogeneity in the region of a stenotic artery, which can be detected with nuclear perfusion agents. In addition, adenosine has a short-lived effect on the A1 receptors that block atrioventricular conduction.1
Dipyridamole (Persantine) is contraindicated when either adenosine or regadenoson is used. Dipyridamole enhances the effect of exogenous and endogenous adenosine by inhibiting its uptake by cardiac cells, thus enhancing the action of these coronary vasodilators.2 Atrioventricular block is common during adenosine stress testing but is transient because adenosine has a short half-life (< 10 seconds), and complete heart block or asystole, as seen in this patient, is rare. Giving intravenous adenosine or regadenoson to patients on dipyridamole may have a marked effect on adenosine receptors, so that profound bradycardia and even asystole leading to cardiac collapse may occur. No data are available on the specific interaction of dipyridamole and regadenoson.
Even though the pharmacodynamics of the interaction between dipyridamole and adenosine are known,3 few reports are available detailing serious adverse events. The contraindication to pharmacologic stress testing in patients taking dipyridamole is noted in the American Society of Nuclear Cardiology Guidelines for stress protocols,4 which advise discontinuing dipyridamole-containing drugs at least 48 hours before the use of adenosine or regadenoson. Similarly, the American Heart Association guidelines5 for the management of supraventricular tachycardia recommend an initial dose of 3 mg of adenosine rather than 6 mg in patients who have been taking dipyridamole.
The dose of aminophylline for reversing the adverse effects of adenosine or regadenoson is 50 to 250 mg intravenously over 30 to 60 seconds. But since these adverse effects are short-lived once the infusion is stopped, aminophylline is usually given only if the adverse effects are severe, as in this patient.
Pharmacologic nuclear stress testing with adenosine receptor agonists (eg, adenosine or regadenoson) is contraindicated in patients taking dipyridamole or the combination pill Aggrenox because of the potential for profound bradyarrhythmias or asystole.
A 60-year-old woman was referred for pharmacologic nuclear stress testing before treatment for breast cancer. She had hypertension, diabetes mellitus, coronary artery disease, and a remote history of stroke, and she was taking clonidine (Catapres), labetalol (Normodyne, Trandate), furosemide (Lasix), hydralazine, valsartan (Diovan), insulin, and the aspirin-dipyridamole combination Aggrenox. Her vital signs and electrocardiogram before the stress test were normal.
The stress test was started with a standard protocol of adenosine (Adenoscan) infused intravenously over 4 minutes. For the first 2 minutes, she was stable and had no symptoms, but then sinus pauses and second-degree atrioventricular block type 2 developed, after which her heart stopped beating (Figure 1). The infusion was immediately stopped, but she became unresponsive and remained pulseless.
Cardiopulmonary resuscitation was started, aminophylline 100 mg was given intravenously, and she regained a pulse and blood pressure within a few minutes. She was then transferred to the emergency room, where she returned to her baseline clinical and neurologic status without symptoms.
AN UNRECOGNIZED DRUG INTERACTION
Asystole occurred in this patient because of the interaction of intravenous adenosine with the dipyridamole in the medication Aggrenox. Although adenosine, given during pharmacologic stress testing, is known to interact with various medications, the potential for this interaction may be overlooked if the culprit is present in a combination drug. Aggrenox is commonly given for secondary stroke prevention and should be discontinued before pharmacologic nuclear stress testing.
Pharmacologic stress testing involves two commonly used stress agents, adenosine and regadenoson (Lexiscan), which cause coronary vasodilation through their action on A2A receptors in the heart. Coronary vasodilation results in flow heterogeneity in the region of a stenotic artery, which can be detected with nuclear perfusion agents. In addition, adenosine has a short-lived effect on the A1 receptors that block atrioventricular conduction.1
Dipyridamole (Persantine) is contraindicated when either adenosine or regadenoson is used. Dipyridamole enhances the effect of exogenous and endogenous adenosine by inhibiting its uptake by cardiac cells, thus enhancing the action of these coronary vasodilators.2 Atrioventricular block is common during adenosine stress testing but is transient because adenosine has a short half-life (< 10 seconds), and complete heart block or asystole, as seen in this patient, is rare. Giving intravenous adenosine or regadenoson to patients on dipyridamole may have a marked effect on adenosine receptors, so that profound bradycardia and even asystole leading to cardiac collapse may occur. No data are available on the specific interaction of dipyridamole and regadenoson.
Even though the pharmacodynamics of the interaction between dipyridamole and adenosine are known,3 few reports are available detailing serious adverse events. The contraindication to pharmacologic stress testing in patients taking dipyridamole is noted in the American Society of Nuclear Cardiology Guidelines for stress protocols,4 which advise discontinuing dipyridamole-containing drugs at least 48 hours before the use of adenosine or regadenoson. Similarly, the American Heart Association guidelines5 for the management of supraventricular tachycardia recommend an initial dose of 3 mg of adenosine rather than 6 mg in patients who have been taking dipyridamole.
The dose of aminophylline for reversing the adverse effects of adenosine or regadenoson is 50 to 250 mg intravenously over 30 to 60 seconds. But since these adverse effects are short-lived once the infusion is stopped, aminophylline is usually given only if the adverse effects are severe, as in this patient.
Pharmacologic nuclear stress testing with adenosine receptor agonists (eg, adenosine or regadenoson) is contraindicated in patients taking dipyridamole or the combination pill Aggrenox because of the potential for profound bradyarrhythmias or asystole.
- Zoghbi GJ, Iskandrian AE. Selective adenosine agonists and myocardial perfusion imaging. J Nucl Cardiol 2012; 19:126–141.
- Lerman BB, Wesley RC, Belardinelli L. Electrophysiologic effects of dipyridamole on atrioventricular nodal conduction and supraventricular tachycardia. Role of endogenous adenosine. Circulation 1989; 80:1536–1543.
- Biaggioni I, Onrot J, Hollister AS, Robertson D. Cardiovascular effects of adenosine infusion in man and their modulation by dipyridamole. Life Sci 1986; 39:2229–2236.
- Henzlova MJ, Cerqueira MD, Mahmarian JJ, Yao SS; Quality Assurance Committee of the American Society of Nuclear Cardiology. Stress protocols and tracers. J Nucl Cardiol 2006; 13:e80–e90.
- ECC Committee, Subcommittees and Task Forces of the American Heart Association. 2005 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Part 7.3: management of symptomatic bradycardia and tachycardia. Circulation 2005; 112(suppl 24):IV67–IV77.
- Zoghbi GJ, Iskandrian AE. Selective adenosine agonists and myocardial perfusion imaging. J Nucl Cardiol 2012; 19:126–141.
- Lerman BB, Wesley RC, Belardinelli L. Electrophysiologic effects of dipyridamole on atrioventricular nodal conduction and supraventricular tachycardia. Role of endogenous adenosine. Circulation 1989; 80:1536–1543.
- Biaggioni I, Onrot J, Hollister AS, Robertson D. Cardiovascular effects of adenosine infusion in man and their modulation by dipyridamole. Life Sci 1986; 39:2229–2236.
- Henzlova MJ, Cerqueira MD, Mahmarian JJ, Yao SS; Quality Assurance Committee of the American Society of Nuclear Cardiology. Stress protocols and tracers. J Nucl Cardiol 2006; 13:e80–e90.
- ECC Committee, Subcommittees and Task Forces of the American Heart Association. 2005 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Part 7.3: management of symptomatic bradycardia and tachycardia. Circulation 2005; 112(suppl 24):IV67–IV77.
Stress cardiac magnetic resonance feasible and prognostic in obese patients
Stress cardiac magnetic resonance is highly feasible and prognostically useful in obese patients, a population in which stress imaging methods are limited, according to a study of nearly 300 patients reported online in JACC: Cardiovascular Imaging on April 9.
Dr. Ravi V. Shah of Harvard Medical School, Boston, and his colleagues, said stress echocardiography and nuclear perfusion can be challenging in obese patients, and PET has issues around ionizing radiation and cost, but the use of stress cardiac magnetic resonance had also been limited by concerns about claustrophobia and safety monitoring.
In this feasibility study in 285 patients with a mean body mass index of 35.4 kg/m2, the primary outcome was a composite of cardiac death or MI (MACE). During a mean follow-up of 2.1 years, 19 patients died, 7 from cardiovascular causes.
The incidence of MACE increased with both inducible ischemia and late gadolinium enhancement (LGE). The patients with no evidence of inducible ischemia, infarction, or LGE had a very low annualized MACE rate of 0.3%, while those who had no inducible ischemia who did have LGE had a rate of 2.4%. The MACE rate jumped significantly, to 6.3%, in patients with inducible ischemia and no LGE, and further yet in patients with evidence of both to 6.7%.
Diabetes, age, prior MI, prior revascularization, and reduced left ventricular ejection fraction were all associated with MACE in the study.
The investigators noted that only 13 (5%) of patients failed to complete the study protocol because of claustrophobia, intolerance to the stress agent, or poor gating, and sedation was required in 19 (7%) of patients.
However diagnostic-quality imaging was achieved in more than 89% of patients (JACC Cardiovasc. Imaging 2014 [dx.doi.org/10.1016/j.jcmg.2013.11.011]).
These results "confirm, in an obese population, that inducible ischemia and LGE by stress perfusion CMR are robust markers of risk even in those patients without a clinical history of prior infarction," the authors concluded.
Researchers declared grants and awards from the American Heart Association, the National Institutes of Health and the Alberta Heritage Foundation for Medical Research, and one author declared research support from Astellas Pharma US.
Stress SPECT imaging has been problematic in obese patients, often yielding poor quality images. This study by Dr. Shah and his coworkers clearly shows the prognostic value of stress cardiac magnetic resonance in obese individuals.
Those patients with absence of both ischemia and late gadolinium enhancement had an excellent prognosis during follow-up. It should be pointed out that PET myocardial perfusion imaging is also effective in obese patients with a similar event-free survival in those with normal studies. As the authors note, however, PET imaging is associated with some radiation exposure, whereas CMR imaging has no radiation exposure to the patient.
CMR use in this setting is limited, however, as some patients require sedation because of claustrophobia, and the technique is not readily available to all cardiac imaging laboratories.
Dr. George A. Beller is chief of the cardiovascular division at the University of Virginia Health System in Charlottesville. He has no financial conflicts of interest.
Stress SPECT imaging has been problematic in obese patients, often yielding poor quality images. This study by Dr. Shah and his coworkers clearly shows the prognostic value of stress cardiac magnetic resonance in obese individuals.
Those patients with absence of both ischemia and late gadolinium enhancement had an excellent prognosis during follow-up. It should be pointed out that PET myocardial perfusion imaging is also effective in obese patients with a similar event-free survival in those with normal studies. As the authors note, however, PET imaging is associated with some radiation exposure, whereas CMR imaging has no radiation exposure to the patient.
CMR use in this setting is limited, however, as some patients require sedation because of claustrophobia, and the technique is not readily available to all cardiac imaging laboratories.
Dr. George A. Beller is chief of the cardiovascular division at the University of Virginia Health System in Charlottesville. He has no financial conflicts of interest.
Stress SPECT imaging has been problematic in obese patients, often yielding poor quality images. This study by Dr. Shah and his coworkers clearly shows the prognostic value of stress cardiac magnetic resonance in obese individuals.
Those patients with absence of both ischemia and late gadolinium enhancement had an excellent prognosis during follow-up. It should be pointed out that PET myocardial perfusion imaging is also effective in obese patients with a similar event-free survival in those with normal studies. As the authors note, however, PET imaging is associated with some radiation exposure, whereas CMR imaging has no radiation exposure to the patient.
CMR use in this setting is limited, however, as some patients require sedation because of claustrophobia, and the technique is not readily available to all cardiac imaging laboratories.
Dr. George A. Beller is chief of the cardiovascular division at the University of Virginia Health System in Charlottesville. He has no financial conflicts of interest.
Stress cardiac magnetic resonance is highly feasible and prognostically useful in obese patients, a population in which stress imaging methods are limited, according to a study of nearly 300 patients reported online in JACC: Cardiovascular Imaging on April 9.
Dr. Ravi V. Shah of Harvard Medical School, Boston, and his colleagues, said stress echocardiography and nuclear perfusion can be challenging in obese patients, and PET has issues around ionizing radiation and cost, but the use of stress cardiac magnetic resonance had also been limited by concerns about claustrophobia and safety monitoring.
In this feasibility study in 285 patients with a mean body mass index of 35.4 kg/m2, the primary outcome was a composite of cardiac death or MI (MACE). During a mean follow-up of 2.1 years, 19 patients died, 7 from cardiovascular causes.
The incidence of MACE increased with both inducible ischemia and late gadolinium enhancement (LGE). The patients with no evidence of inducible ischemia, infarction, or LGE had a very low annualized MACE rate of 0.3%, while those who had no inducible ischemia who did have LGE had a rate of 2.4%. The MACE rate jumped significantly, to 6.3%, in patients with inducible ischemia and no LGE, and further yet in patients with evidence of both to 6.7%.
Diabetes, age, prior MI, prior revascularization, and reduced left ventricular ejection fraction were all associated with MACE in the study.
The investigators noted that only 13 (5%) of patients failed to complete the study protocol because of claustrophobia, intolerance to the stress agent, or poor gating, and sedation was required in 19 (7%) of patients.
However diagnostic-quality imaging was achieved in more than 89% of patients (JACC Cardiovasc. Imaging 2014 [dx.doi.org/10.1016/j.jcmg.2013.11.011]).
These results "confirm, in an obese population, that inducible ischemia and LGE by stress perfusion CMR are robust markers of risk even in those patients without a clinical history of prior infarction," the authors concluded.
Researchers declared grants and awards from the American Heart Association, the National Institutes of Health and the Alberta Heritage Foundation for Medical Research, and one author declared research support from Astellas Pharma US.
Stress cardiac magnetic resonance is highly feasible and prognostically useful in obese patients, a population in which stress imaging methods are limited, according to a study of nearly 300 patients reported online in JACC: Cardiovascular Imaging on April 9.
Dr. Ravi V. Shah of Harvard Medical School, Boston, and his colleagues, said stress echocardiography and nuclear perfusion can be challenging in obese patients, and PET has issues around ionizing radiation and cost, but the use of stress cardiac magnetic resonance had also been limited by concerns about claustrophobia and safety monitoring.
In this feasibility study in 285 patients with a mean body mass index of 35.4 kg/m2, the primary outcome was a composite of cardiac death or MI (MACE). During a mean follow-up of 2.1 years, 19 patients died, 7 from cardiovascular causes.
The incidence of MACE increased with both inducible ischemia and late gadolinium enhancement (LGE). The patients with no evidence of inducible ischemia, infarction, or LGE had a very low annualized MACE rate of 0.3%, while those who had no inducible ischemia who did have LGE had a rate of 2.4%. The MACE rate jumped significantly, to 6.3%, in patients with inducible ischemia and no LGE, and further yet in patients with evidence of both to 6.7%.
Diabetes, age, prior MI, prior revascularization, and reduced left ventricular ejection fraction were all associated with MACE in the study.
The investigators noted that only 13 (5%) of patients failed to complete the study protocol because of claustrophobia, intolerance to the stress agent, or poor gating, and sedation was required in 19 (7%) of patients.
However diagnostic-quality imaging was achieved in more than 89% of patients (JACC Cardiovasc. Imaging 2014 [dx.doi.org/10.1016/j.jcmg.2013.11.011]).
These results "confirm, in an obese population, that inducible ischemia and LGE by stress perfusion CMR are robust markers of risk even in those patients without a clinical history of prior infarction," the authors concluded.
Researchers declared grants and awards from the American Heart Association, the National Institutes of Health and the Alberta Heritage Foundation for Medical Research, and one author declared research support from Astellas Pharma US.
FROM JACC CARDIOVASCULAR IMAGING
Major finding: Obese individuals with no evidence of inducible ischemia or LGE in stress CMR are at significantly lower annualized risk of MACE (0.3%), compared with those with evidence of both (6.7%).
Data source: Cohort study in 285 patients with a mean body mass index of 35.4 kg/m2.
Disclosures: Researchers declared grants and awards from the American Heart Association, the National Institutes of Health, and the Alberta Heritage Foundation for Medical Research; one author declared research support from Astellas Pharma US.
An intravenous drug user with persistent dyspnea and lung infiltrates
A 58-year-old-man with a history of intravenous drug abuse, chronic hepatitis C, and anxiety presented to our emergency department twice in 4 weeks with progressive dyspnea and night sweats. He was a nonsmoker and had been an electrician for 15 years.
The first time he came in, chest radiography revealed bilateral reticulonodular infiltrates in the lung bases. He was treated with intravenous ceftriaxone (Rocephin) and azithromycin (Zithromax) for presumed community-acquired pneumonia and was then sent home on a 10-day course of oral amoxicillin-clavulanate (Augmentin). The antibiotics did not improve his symptoms, and 3 weeks later he presented again to the emergency department.
On his second presentation, he was in respiratory distress (oxygen saturation 78% on room air) and was afebrile and tachypneic. Physical examination revealed numerous injection marks or “tracks” on the skin of both arms, and auscultation revealed diminished intensity of breath sounds in both lung bases.
Repeat chest radiography demonstrated that the infiltrates were still there. Computed tomography was ordered and showed mild centrilobular emphysematous changes in both lungs, bibasilar opacifications, and a mass-like lesion (3.3 × 1.9 cm) in the right lower lobe (Figure 1).
He subsequently underwent bronchoscopy, which showed no endobronchial abnormalities. Transbronchial lung biopsy was performed, and histopathologic analysis of the specimen (Figure 2) revealed rodlike, birefringent crystals under polarized light, with an extensive foreign-body giant-cell reaction outside pulmonary capillaries, suggestive of intravascular pulmonary talcosis. Blood and sputum cultures were negative for pathologic organisms. Bronchoalveolar lavage samples were negative for pathologic organisms and malignant cells.
On further questioning, the patient revealed that he intravenously injected various drugs intended for oral use, such as crushed meperidine (Demerol), methylphenidate (Ritalin), and methadone tablets.
Pulmonary function tests indicated a severe obstructive pattern. The predicted forced expiratory volume in the first second of expiration (FEV1) was 25%, and the ratio of FEV1 to forced vital capacity was 27%.
Transthoracic echocardiography revealed mild pulmonary hypertension with a right ventricular systolic pressure of 28 mm Hg at rest.
Based on the results of the histologic examination, a diagnosis of intravascular pulmonary talcosis was made. Antibiotics were discontinued, and treatment with albuterol and ipratropium bromide (Combivent) inhalers was started. The patient remained oxygen-dependent at the time of hospital discharge.
INTRAVASCULAR PULMONARY TALCOSIS
Intravascular pulmonary talcosis is seen predominantly in those who chronically inject intravenous drugs intended for oral use.1,2
Many oral medications contain talc as a filler and lubricant to prevent the tablet from sticking to equipment during the manufacturing process. When oral medications containing talc are crushed, dissolved in water, and injected intravenously, the talc crystals and other particles lodge in the pulmonary vascular bed, resulting in microscopic pulmonary embolizations.
Over time, these particles migrate to the pulmonary interstitium and incite a foreign-body granulomatous reaction, which may be associated with progressive pulmonary fibrosis. The severity of this immune reaction and fibrosis may vary; hence, some patients remain asymptomatic, whereas some present with dyspnea from extensive fibrosis and pulmonary hypertension.
Persistent dyspnea along with persistent infiltrates on chest imaging in an intravenous drug abuser should prompt suspicion for intravascular pulmonary talcosis as well as consideration of other diagnoses, such as pneumonia, malignancy, and septic pulmonary emboli.
There is no established treatment for intravascular pulmonary talcosis; treatment is often supportive. A few studies and case reports have indicated varied success with systemic and inhaled corticosteroids.3–5 In extreme cases, lung transplantation may be necessary; however, this would require a comprehensive psychiatric assessment to minimize the risk of addiction relapse after transplantation.
- Arnett EN, Battle WE, Russo JV, Roberts WC. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension. Am J Med 1976; 60:711–718.
- Griffith CC, Raval JS, Nichols L. Intravascular talcosis due to intravenous drug use is an underrecognized cause of pulmonary hypertension. Pulm Med 2012; 2012:617531.
- Chau CH, Yew WW, Lee J. Inhaled budesonide in the treatment of talc-induced pulmonary granulomatosis. Respiration 2003; 70:439.
- Gysbrechts C, Michiels E, Verbeken E, et al. Interstitial lung disease more than 40 years after a 5 year occupational exposure to talc. Eur Respir J 1998; 11:1412–1415.
- Marchiori E, Lourenço S, Gasparetto TD, Zanetti G, Mano CM, Nobre LF. Pulmonary talcosis: imaging findings. Lung 2010; 188:165–171.
A 58-year-old-man with a history of intravenous drug abuse, chronic hepatitis C, and anxiety presented to our emergency department twice in 4 weeks with progressive dyspnea and night sweats. He was a nonsmoker and had been an electrician for 15 years.
The first time he came in, chest radiography revealed bilateral reticulonodular infiltrates in the lung bases. He was treated with intravenous ceftriaxone (Rocephin) and azithromycin (Zithromax) for presumed community-acquired pneumonia and was then sent home on a 10-day course of oral amoxicillin-clavulanate (Augmentin). The antibiotics did not improve his symptoms, and 3 weeks later he presented again to the emergency department.
On his second presentation, he was in respiratory distress (oxygen saturation 78% on room air) and was afebrile and tachypneic. Physical examination revealed numerous injection marks or “tracks” on the skin of both arms, and auscultation revealed diminished intensity of breath sounds in both lung bases.
Repeat chest radiography demonstrated that the infiltrates were still there. Computed tomography was ordered and showed mild centrilobular emphysematous changes in both lungs, bibasilar opacifications, and a mass-like lesion (3.3 × 1.9 cm) in the right lower lobe (Figure 1).
He subsequently underwent bronchoscopy, which showed no endobronchial abnormalities. Transbronchial lung biopsy was performed, and histopathologic analysis of the specimen (Figure 2) revealed rodlike, birefringent crystals under polarized light, with an extensive foreign-body giant-cell reaction outside pulmonary capillaries, suggestive of intravascular pulmonary talcosis. Blood and sputum cultures were negative for pathologic organisms. Bronchoalveolar lavage samples were negative for pathologic organisms and malignant cells.
On further questioning, the patient revealed that he intravenously injected various drugs intended for oral use, such as crushed meperidine (Demerol), methylphenidate (Ritalin), and methadone tablets.
Pulmonary function tests indicated a severe obstructive pattern. The predicted forced expiratory volume in the first second of expiration (FEV1) was 25%, and the ratio of FEV1 to forced vital capacity was 27%.
Transthoracic echocardiography revealed mild pulmonary hypertension with a right ventricular systolic pressure of 28 mm Hg at rest.
Based on the results of the histologic examination, a diagnosis of intravascular pulmonary talcosis was made. Antibiotics were discontinued, and treatment with albuterol and ipratropium bromide (Combivent) inhalers was started. The patient remained oxygen-dependent at the time of hospital discharge.
INTRAVASCULAR PULMONARY TALCOSIS
Intravascular pulmonary talcosis is seen predominantly in those who chronically inject intravenous drugs intended for oral use.1,2
Many oral medications contain talc as a filler and lubricant to prevent the tablet from sticking to equipment during the manufacturing process. When oral medications containing talc are crushed, dissolved in water, and injected intravenously, the talc crystals and other particles lodge in the pulmonary vascular bed, resulting in microscopic pulmonary embolizations.
Over time, these particles migrate to the pulmonary interstitium and incite a foreign-body granulomatous reaction, which may be associated with progressive pulmonary fibrosis. The severity of this immune reaction and fibrosis may vary; hence, some patients remain asymptomatic, whereas some present with dyspnea from extensive fibrosis and pulmonary hypertension.
Persistent dyspnea along with persistent infiltrates on chest imaging in an intravenous drug abuser should prompt suspicion for intravascular pulmonary talcosis as well as consideration of other diagnoses, such as pneumonia, malignancy, and septic pulmonary emboli.
There is no established treatment for intravascular pulmonary talcosis; treatment is often supportive. A few studies and case reports have indicated varied success with systemic and inhaled corticosteroids.3–5 In extreme cases, lung transplantation may be necessary; however, this would require a comprehensive psychiatric assessment to minimize the risk of addiction relapse after transplantation.
A 58-year-old-man with a history of intravenous drug abuse, chronic hepatitis C, and anxiety presented to our emergency department twice in 4 weeks with progressive dyspnea and night sweats. He was a nonsmoker and had been an electrician for 15 years.
The first time he came in, chest radiography revealed bilateral reticulonodular infiltrates in the lung bases. He was treated with intravenous ceftriaxone (Rocephin) and azithromycin (Zithromax) for presumed community-acquired pneumonia and was then sent home on a 10-day course of oral amoxicillin-clavulanate (Augmentin). The antibiotics did not improve his symptoms, and 3 weeks later he presented again to the emergency department.
On his second presentation, he was in respiratory distress (oxygen saturation 78% on room air) and was afebrile and tachypneic. Physical examination revealed numerous injection marks or “tracks” on the skin of both arms, and auscultation revealed diminished intensity of breath sounds in both lung bases.
Repeat chest radiography demonstrated that the infiltrates were still there. Computed tomography was ordered and showed mild centrilobular emphysematous changes in both lungs, bibasilar opacifications, and a mass-like lesion (3.3 × 1.9 cm) in the right lower lobe (Figure 1).
He subsequently underwent bronchoscopy, which showed no endobronchial abnormalities. Transbronchial lung biopsy was performed, and histopathologic analysis of the specimen (Figure 2) revealed rodlike, birefringent crystals under polarized light, with an extensive foreign-body giant-cell reaction outside pulmonary capillaries, suggestive of intravascular pulmonary talcosis. Blood and sputum cultures were negative for pathologic organisms. Bronchoalveolar lavage samples were negative for pathologic organisms and malignant cells.
On further questioning, the patient revealed that he intravenously injected various drugs intended for oral use, such as crushed meperidine (Demerol), methylphenidate (Ritalin), and methadone tablets.
Pulmonary function tests indicated a severe obstructive pattern. The predicted forced expiratory volume in the first second of expiration (FEV1) was 25%, and the ratio of FEV1 to forced vital capacity was 27%.
Transthoracic echocardiography revealed mild pulmonary hypertension with a right ventricular systolic pressure of 28 mm Hg at rest.
Based on the results of the histologic examination, a diagnosis of intravascular pulmonary talcosis was made. Antibiotics were discontinued, and treatment with albuterol and ipratropium bromide (Combivent) inhalers was started. The patient remained oxygen-dependent at the time of hospital discharge.
INTRAVASCULAR PULMONARY TALCOSIS
Intravascular pulmonary talcosis is seen predominantly in those who chronically inject intravenous drugs intended for oral use.1,2
Many oral medications contain talc as a filler and lubricant to prevent the tablet from sticking to equipment during the manufacturing process. When oral medications containing talc are crushed, dissolved in water, and injected intravenously, the talc crystals and other particles lodge in the pulmonary vascular bed, resulting in microscopic pulmonary embolizations.
Over time, these particles migrate to the pulmonary interstitium and incite a foreign-body granulomatous reaction, which may be associated with progressive pulmonary fibrosis. The severity of this immune reaction and fibrosis may vary; hence, some patients remain asymptomatic, whereas some present with dyspnea from extensive fibrosis and pulmonary hypertension.
Persistent dyspnea along with persistent infiltrates on chest imaging in an intravenous drug abuser should prompt suspicion for intravascular pulmonary talcosis as well as consideration of other diagnoses, such as pneumonia, malignancy, and septic pulmonary emboli.
There is no established treatment for intravascular pulmonary talcosis; treatment is often supportive. A few studies and case reports have indicated varied success with systemic and inhaled corticosteroids.3–5 In extreme cases, lung transplantation may be necessary; however, this would require a comprehensive psychiatric assessment to minimize the risk of addiction relapse after transplantation.
- Arnett EN, Battle WE, Russo JV, Roberts WC. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension. Am J Med 1976; 60:711–718.
- Griffith CC, Raval JS, Nichols L. Intravascular talcosis due to intravenous drug use is an underrecognized cause of pulmonary hypertension. Pulm Med 2012; 2012:617531.
- Chau CH, Yew WW, Lee J. Inhaled budesonide in the treatment of talc-induced pulmonary granulomatosis. Respiration 2003; 70:439.
- Gysbrechts C, Michiels E, Verbeken E, et al. Interstitial lung disease more than 40 years after a 5 year occupational exposure to talc. Eur Respir J 1998; 11:1412–1415.
- Marchiori E, Lourenço S, Gasparetto TD, Zanetti G, Mano CM, Nobre LF. Pulmonary talcosis: imaging findings. Lung 2010; 188:165–171.
- Arnett EN, Battle WE, Russo JV, Roberts WC. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension. Am J Med 1976; 60:711–718.
- Griffith CC, Raval JS, Nichols L. Intravascular talcosis due to intravenous drug use is an underrecognized cause of pulmonary hypertension. Pulm Med 2012; 2012:617531.
- Chau CH, Yew WW, Lee J. Inhaled budesonide in the treatment of talc-induced pulmonary granulomatosis. Respiration 2003; 70:439.
- Gysbrechts C, Michiels E, Verbeken E, et al. Interstitial lung disease more than 40 years after a 5 year occupational exposure to talc. Eur Respir J 1998; 11:1412–1415.
- Marchiori E, Lourenço S, Gasparetto TD, Zanetti G, Mano CM, Nobre LF. Pulmonary talcosis: imaging findings. Lung 2010; 188:165–171.
Pediatric Orthopedic Imaging: More Isn’t Always Better
Three excellent instructional cases from Dr. Lawrence Wells and colleagues from
the Children’s Hospital of Philadelphia follow in this E-Focus on Imaging in Pediatric Orthopedics of the February issue of The American Journal of Orthopedics (AJO). These cases highlight the important role of imaging in the practice of pediatric orthopedics, particularly its usefulness in problem solving for conditions that are difficult to diagnose clinically. Given the wide array of imaging techniques currently available, there is a tendency for surgeons to over-investigate. But more isn’t always better.
For example, while magnetic resonance (MR) imaging has the well-known advantages of avoidance of the potential hazards of ionizing radiation, multiplanar imaging capability, and superior soft-tissue contrast and resolution, the relatively long time period for acquisition of MR images make it relatively user-unfriendly for imaging in children. Movement artifacts can be a big problem, leading to image degradation and interpretation difficulties. For young children, having to administer heavy sedation or general anesthesia often negates the benefits of this diagnostic technique. Multidetector computed tomography (CT) produces images of excellent quality and resolution, particularly of bone. However, the price to pay for the thinner contiguous slices that enable production of the beautiful reformatted 2-dimensional sagittal and coronal images, and the stunning 3-dimensional
(3D) images, is a markedly increased radiation dose to the young patient.
It appears that the solution lies in a return to basic principles of good clinical practice. As illustrated by these 3 pediatric orthopedic cases in this month’s AJO, formulating a provisional diagnosis and short list of differential diagnoses starts with a well-taken and detailed clinical history and a meticulous physical examination. Simple hematologic investigations should be interpreted in light of the clinical findings. Imaging should be reserved for problem solving and should not be considered as a screening tool. There must be an imaging plan that aims to
address the following questions: Is there a lesion? If so, what and where exactly is it? And how can I best treat this patient’s condition—in this respect, is imaging really necessary?
For orthopedic problems, the time-honored radiograph still remains the initial imaging investigation in today’s practice. Too often, more expensive and advanced imaging modalities are requested first, even when the diagnosis can be made on
the basis of the plain film. This is poor clinical practice, and it reflects a lack of training and common sense. Radiographs are readily available, technically easy to perform, and give an overview of bone and joint lesions. It is the imaging investigation of choice for the detection of fractures and dislocations and also for the diagnosis of bone tumors and many other bone conditions. CT should be considered a supplementary examination to radiographs and is helpful when radiographs are equivocal or findings are subtle. CT is particularly suited for complex skeletal anatomy, for example, the spine, scapula, pelvis, and hindfoot.
In pediatric patients, reconstructed 3D CT images are useful for sorting out congenital spinal deformities.
For children and adolescents, ultrasonography can be used in place of MR imaging for many indications, particularly for assessing superficial structures such as tendons, muscles, ligaments, blood vessels, and other soft tissues. However, performing musculoskeletal ultrasonography well entails a rather long and steep
learning curve before technical expertise can be achieved. More advanced techniques such as MR imaging, nuclear medicine imaging, and imaging-guided interventional procedures should be used sparingly.
In fact, less may be better. If in doubt, pause before asking for more imaging and do consult your friendly neighborhood musculoskeletal radiologist.
Three excellent instructional cases from Dr. Lawrence Wells and colleagues from
the Children’s Hospital of Philadelphia follow in this E-Focus on Imaging in Pediatric Orthopedics of the February issue of The American Journal of Orthopedics (AJO). These cases highlight the important role of imaging in the practice of pediatric orthopedics, particularly its usefulness in problem solving for conditions that are difficult to diagnose clinically. Given the wide array of imaging techniques currently available, there is a tendency for surgeons to over-investigate. But more isn’t always better.
For example, while magnetic resonance (MR) imaging has the well-known advantages of avoidance of the potential hazards of ionizing radiation, multiplanar imaging capability, and superior soft-tissue contrast and resolution, the relatively long time period for acquisition of MR images make it relatively user-unfriendly for imaging in children. Movement artifacts can be a big problem, leading to image degradation and interpretation difficulties. For young children, having to administer heavy sedation or general anesthesia often negates the benefits of this diagnostic technique. Multidetector computed tomography (CT) produces images of excellent quality and resolution, particularly of bone. However, the price to pay for the thinner contiguous slices that enable production of the beautiful reformatted 2-dimensional sagittal and coronal images, and the stunning 3-dimensional
(3D) images, is a markedly increased radiation dose to the young patient.
It appears that the solution lies in a return to basic principles of good clinical practice. As illustrated by these 3 pediatric orthopedic cases in this month’s AJO, formulating a provisional diagnosis and short list of differential diagnoses starts with a well-taken and detailed clinical history and a meticulous physical examination. Simple hematologic investigations should be interpreted in light of the clinical findings. Imaging should be reserved for problem solving and should not be considered as a screening tool. There must be an imaging plan that aims to
address the following questions: Is there a lesion? If so, what and where exactly is it? And how can I best treat this patient’s condition—in this respect, is imaging really necessary?
For orthopedic problems, the time-honored radiograph still remains the initial imaging investigation in today’s practice. Too often, more expensive and advanced imaging modalities are requested first, even when the diagnosis can be made on
the basis of the plain film. This is poor clinical practice, and it reflects a lack of training and common sense. Radiographs are readily available, technically easy to perform, and give an overview of bone and joint lesions. It is the imaging investigation of choice for the detection of fractures and dislocations and also for the diagnosis of bone tumors and many other bone conditions. CT should be considered a supplementary examination to radiographs and is helpful when radiographs are equivocal or findings are subtle. CT is particularly suited for complex skeletal anatomy, for example, the spine, scapula, pelvis, and hindfoot.
In pediatric patients, reconstructed 3D CT images are useful for sorting out congenital spinal deformities.
For children and adolescents, ultrasonography can be used in place of MR imaging for many indications, particularly for assessing superficial structures such as tendons, muscles, ligaments, blood vessels, and other soft tissues. However, performing musculoskeletal ultrasonography well entails a rather long and steep
learning curve before technical expertise can be achieved. More advanced techniques such as MR imaging, nuclear medicine imaging, and imaging-guided interventional procedures should be used sparingly.
In fact, less may be better. If in doubt, pause before asking for more imaging and do consult your friendly neighborhood musculoskeletal radiologist.
Three excellent instructional cases from Dr. Lawrence Wells and colleagues from
the Children’s Hospital of Philadelphia follow in this E-Focus on Imaging in Pediatric Orthopedics of the February issue of The American Journal of Orthopedics (AJO). These cases highlight the important role of imaging in the practice of pediatric orthopedics, particularly its usefulness in problem solving for conditions that are difficult to diagnose clinically. Given the wide array of imaging techniques currently available, there is a tendency for surgeons to over-investigate. But more isn’t always better.
For example, while magnetic resonance (MR) imaging has the well-known advantages of avoidance of the potential hazards of ionizing radiation, multiplanar imaging capability, and superior soft-tissue contrast and resolution, the relatively long time period for acquisition of MR images make it relatively user-unfriendly for imaging in children. Movement artifacts can be a big problem, leading to image degradation and interpretation difficulties. For young children, having to administer heavy sedation or general anesthesia often negates the benefits of this diagnostic technique. Multidetector computed tomography (CT) produces images of excellent quality and resolution, particularly of bone. However, the price to pay for the thinner contiguous slices that enable production of the beautiful reformatted 2-dimensional sagittal and coronal images, and the stunning 3-dimensional
(3D) images, is a markedly increased radiation dose to the young patient.
It appears that the solution lies in a return to basic principles of good clinical practice. As illustrated by these 3 pediatric orthopedic cases in this month’s AJO, formulating a provisional diagnosis and short list of differential diagnoses starts with a well-taken and detailed clinical history and a meticulous physical examination. Simple hematologic investigations should be interpreted in light of the clinical findings. Imaging should be reserved for problem solving and should not be considered as a screening tool. There must be an imaging plan that aims to
address the following questions: Is there a lesion? If so, what and where exactly is it? And how can I best treat this patient’s condition—in this respect, is imaging really necessary?
For orthopedic problems, the time-honored radiograph still remains the initial imaging investigation in today’s practice. Too often, more expensive and advanced imaging modalities are requested first, even when the diagnosis can be made on
the basis of the plain film. This is poor clinical practice, and it reflects a lack of training and common sense. Radiographs are readily available, technically easy to perform, and give an overview of bone and joint lesions. It is the imaging investigation of choice for the detection of fractures and dislocations and also for the diagnosis of bone tumors and many other bone conditions. CT should be considered a supplementary examination to radiographs and is helpful when radiographs are equivocal or findings are subtle. CT is particularly suited for complex skeletal anatomy, for example, the spine, scapula, pelvis, and hindfoot.
In pediatric patients, reconstructed 3D CT images are useful for sorting out congenital spinal deformities.
For children and adolescents, ultrasonography can be used in place of MR imaging for many indications, particularly for assessing superficial structures such as tendons, muscles, ligaments, blood vessels, and other soft tissues. However, performing musculoskeletal ultrasonography well entails a rather long and steep
learning curve before technical expertise can be achieved. More advanced techniques such as MR imaging, nuclear medicine imaging, and imaging-guided interventional procedures should be used sparingly.
In fact, less may be better. If in doubt, pause before asking for more imaging and do consult your friendly neighborhood musculoskeletal radiologist.
Tip of the Iceberg: Subtle Findings on Traumatic Knee Radiographs Portend Significant Injury
Using Computed Tomography to Assess Proximal Humerus Fractures
Transcranial ultrasound method outdoes echocardiography for finding PFO
SAN DIEGO – Transcranial Doppler saline studies were superior to transesophageal echocardiography in diagnosing a hole in the heart, according to a prospective study of patients who had a cryptogenic stroke.
Researchers analyzed data from 340 patients with cryptogenic stroke suspected of paradoxical embolism who had had their right-left shunt confirmed with transcranial Doppler saline study (TCDSS). Transesophageal echocardiography (TEE) failed to show a shunt such as patent foramen ovale (PFO) in 15% of the cases.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
Courtesy Dr. J. David Spence, Robarts Research Institute
"I was surprised by the number," said Dr. J. David Spence, senior researcher of the study, who presented the findings at the International Stroke Conference, sponsored by the American Heart Association. "I had realized that some of the patients with shunts were missed by TEE, ... but I was surprised by how often that happened."
Nearly a quarter of the population has a PFO, and 4.0%-5.5% of strokes are due to paradoxical embolism through a right-left shunt. But patching the shunts isn’t the simple answer, because the procedure is not without complications, and so far, the studies haven’t been able to make a strong case for the procedure.
TCDSS is an ultrasound method in which 1 mL of tiny bubbles is injected into the vein and if detected in the brain, could give clues to a right-left shunt. The method is safer, the equipment is cheaper, and the training is faster than for TEE, Dr. Spence said.
He added that one reason that TCDSS may be more sensitive than TEE is that sedation for TEE may prevent an adequate Valsalva maneuver. He also showed in a video that detecting the bubbles in TCDSS is rather straightforward.
Given the findings of his study, Dr. Spence said that there’s a need to identify which PFO patients are more likely to have paradoxical embolism and are more likely to respond to patching.
One solution is paying attention to the clinical clues of paradoxical embolism (J. Neurol. Sci. 2008;275:121-7). And Dr. Spence’s recent findings may add another tool to help with this prediction.
Aside from the superiority of TCDSS to TEE in identifying PFO, Dr. Spence and his colleagues found that TCDSS was also better for risk stratification of PFOs. The analysis found that 25% of the shunts that were missed on TEE were high-grade shunts (Spencer grade 3 or higher). Patients with a shunt grade of 3 or higher were significantly more likely to have a stroke or transient ischemic attack in the 4-year follow-up period (P = .028), said Dr. Spence, director of the Stroke Prevention & Atherosclerosis Research Centre at the University of Western Ontario, London. This was not predicted by the presence of shunt on TEE, nor was it predicted by mobile atrial septum or septal aneurysm. TEE missed nearly 46% of grade 1 shunts, 32% of grade 2, 13% of grade 3, 7% of grade 4, and almost 5% of grade 5.
"This doesn’t mean that everyone should go pack up their TEE machines, because we still need it for diagnosis of other cardioembolism," said Dr. Spence. "But these techniques can be complementary."
He added that it’s too soon to use these findings as grounds for change in clinical practice, and there’s a need for further studies such as randomized trials.
The majority (62%) of the study’s 340 patients were female. The patients had a mean age of 53 years and underwent follow-up for a median of 420 days. All the patients, who visited the center between 2000 and 2013, had cryptogenic stroke and were suspected of having paradoxical embolism. A total of 280 cases had TEE data available.
Dr. Spence said he had no disclosures relevant to the study.
On Twitter @naseemsmiller
SAN DIEGO – Transcranial Doppler saline studies were superior to transesophageal echocardiography in diagnosing a hole in the heart, according to a prospective study of patients who had a cryptogenic stroke.
Researchers analyzed data from 340 patients with cryptogenic stroke suspected of paradoxical embolism who had had their right-left shunt confirmed with transcranial Doppler saline study (TCDSS). Transesophageal echocardiography (TEE) failed to show a shunt such as patent foramen ovale (PFO) in 15% of the cases.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
Courtesy Dr. J. David Spence, Robarts Research Institute
"I was surprised by the number," said Dr. J. David Spence, senior researcher of the study, who presented the findings at the International Stroke Conference, sponsored by the American Heart Association. "I had realized that some of the patients with shunts were missed by TEE, ... but I was surprised by how often that happened."
Nearly a quarter of the population has a PFO, and 4.0%-5.5% of strokes are due to paradoxical embolism through a right-left shunt. But patching the shunts isn’t the simple answer, because the procedure is not without complications, and so far, the studies haven’t been able to make a strong case for the procedure.
TCDSS is an ultrasound method in which 1 mL of tiny bubbles is injected into the vein and if detected in the brain, could give clues to a right-left shunt. The method is safer, the equipment is cheaper, and the training is faster than for TEE, Dr. Spence said.
He added that one reason that TCDSS may be more sensitive than TEE is that sedation for TEE may prevent an adequate Valsalva maneuver. He also showed in a video that detecting the bubbles in TCDSS is rather straightforward.
Given the findings of his study, Dr. Spence said that there’s a need to identify which PFO patients are more likely to have paradoxical embolism and are more likely to respond to patching.
One solution is paying attention to the clinical clues of paradoxical embolism (J. Neurol. Sci. 2008;275:121-7). And Dr. Spence’s recent findings may add another tool to help with this prediction.
Aside from the superiority of TCDSS to TEE in identifying PFO, Dr. Spence and his colleagues found that TCDSS was also better for risk stratification of PFOs. The analysis found that 25% of the shunts that were missed on TEE were high-grade shunts (Spencer grade 3 or higher). Patients with a shunt grade of 3 or higher were significantly more likely to have a stroke or transient ischemic attack in the 4-year follow-up period (P = .028), said Dr. Spence, director of the Stroke Prevention & Atherosclerosis Research Centre at the University of Western Ontario, London. This was not predicted by the presence of shunt on TEE, nor was it predicted by mobile atrial septum or septal aneurysm. TEE missed nearly 46% of grade 1 shunts, 32% of grade 2, 13% of grade 3, 7% of grade 4, and almost 5% of grade 5.
"This doesn’t mean that everyone should go pack up their TEE machines, because we still need it for diagnosis of other cardioembolism," said Dr. Spence. "But these techniques can be complementary."
He added that it’s too soon to use these findings as grounds for change in clinical practice, and there’s a need for further studies such as randomized trials.
The majority (62%) of the study’s 340 patients were female. The patients had a mean age of 53 years and underwent follow-up for a median of 420 days. All the patients, who visited the center between 2000 and 2013, had cryptogenic stroke and were suspected of having paradoxical embolism. A total of 280 cases had TEE data available.
Dr. Spence said he had no disclosures relevant to the study.
On Twitter @naseemsmiller
SAN DIEGO – Transcranial Doppler saline studies were superior to transesophageal echocardiography in diagnosing a hole in the heart, according to a prospective study of patients who had a cryptogenic stroke.
Researchers analyzed data from 340 patients with cryptogenic stroke suspected of paradoxical embolism who had had their right-left shunt confirmed with transcranial Doppler saline study (TCDSS). Transesophageal echocardiography (TEE) failed to show a shunt such as patent foramen ovale (PFO) in 15% of the cases.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
Courtesy Dr. J. David Spence, Robarts Research Institute
"I was surprised by the number," said Dr. J. David Spence, senior researcher of the study, who presented the findings at the International Stroke Conference, sponsored by the American Heart Association. "I had realized that some of the patients with shunts were missed by TEE, ... but I was surprised by how often that happened."
Nearly a quarter of the population has a PFO, and 4.0%-5.5% of strokes are due to paradoxical embolism through a right-left shunt. But patching the shunts isn’t the simple answer, because the procedure is not without complications, and so far, the studies haven’t been able to make a strong case for the procedure.
TCDSS is an ultrasound method in which 1 mL of tiny bubbles is injected into the vein and if detected in the brain, could give clues to a right-left shunt. The method is safer, the equipment is cheaper, and the training is faster than for TEE, Dr. Spence said.
He added that one reason that TCDSS may be more sensitive than TEE is that sedation for TEE may prevent an adequate Valsalva maneuver. He also showed in a video that detecting the bubbles in TCDSS is rather straightforward.
Given the findings of his study, Dr. Spence said that there’s a need to identify which PFO patients are more likely to have paradoxical embolism and are more likely to respond to patching.
One solution is paying attention to the clinical clues of paradoxical embolism (J. Neurol. Sci. 2008;275:121-7). And Dr. Spence’s recent findings may add another tool to help with this prediction.
Aside from the superiority of TCDSS to TEE in identifying PFO, Dr. Spence and his colleagues found that TCDSS was also better for risk stratification of PFOs. The analysis found that 25% of the shunts that were missed on TEE were high-grade shunts (Spencer grade 3 or higher). Patients with a shunt grade of 3 or higher were significantly more likely to have a stroke or transient ischemic attack in the 4-year follow-up period (P = .028), said Dr. Spence, director of the Stroke Prevention & Atherosclerosis Research Centre at the University of Western Ontario, London. This was not predicted by the presence of shunt on TEE, nor was it predicted by mobile atrial septum or septal aneurysm. TEE missed nearly 46% of grade 1 shunts, 32% of grade 2, 13% of grade 3, 7% of grade 4, and almost 5% of grade 5.
"This doesn’t mean that everyone should go pack up their TEE machines, because we still need it for diagnosis of other cardioembolism," said Dr. Spence. "But these techniques can be complementary."
He added that it’s too soon to use these findings as grounds for change in clinical practice, and there’s a need for further studies such as randomized trials.
The majority (62%) of the study’s 340 patients were female. The patients had a mean age of 53 years and underwent follow-up for a median of 420 days. All the patients, who visited the center between 2000 and 2013, had cryptogenic stroke and were suspected of having paradoxical embolism. A total of 280 cases had TEE data available.
Dr. Spence said he had no disclosures relevant to the study.
On Twitter @naseemsmiller
AT THE INTERNATIONAL STROKE CONFERENCE
Major finding: Echocardiography (TEE) failed to show right-to-left shunts, such as patent foramen ovale, which were identified by TCDSS, in 15% of the cases.
Data source: Prospective study of 340 patients who had a cryptogenic stroke.
Disclosures: Dr. Spence had no disclosures relevant to the study.
