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Those Who Forget Their History Are Condemned to Repeat It
DIAGNOSTIC DILEMMA Aimee K. Zaas, MD, Section Editor
Those Who Forget Their History Are Condemned to Repeat It Theresa Rowe, DO, Roy C. Ziegelstein, MD, Joyce Jones, MD Department of Medicine, Johns Hopkins Bayview Medical Center, Johns Hopkins University School of Medicine, Baltimore, Md.
PRESENTATION “Those who forget their history are condemned to repeat it.” This sentence, often attributed to the philosopher George Santayana, is actually a misquotation of his comment, “Those who cannot remember the past are condemned to repeat it.”1 However, the misquotation may be more apropos in this case. A 47-year-old, right-handed male truck driver who enjoyed working on automobile engines in his spare time was admitted with pain, numbness, and pallor of the right 4th and 5th fingers, exacerbated by exposure to the cold. The previous night, when washing his hands after working on an engine, he had noticed that the 2 fingers were cold, painful, and pale. They remained that way throughout the night without significant color or temperature change. He denied any fevers, fatigue, recent weight loss, or trauma to his right hand. A year earlier, the patient had experienced an almost identical episode involving the 4th and 5th digits of his left hand. He had been admitted to another hospital on that occasion and could recall no trauma to the left hand at that time. An angiogram had revealed occlusion of the left ulnar artery at the terminus of the palmar arch. An evaluation for a hypercoagulable state had revealed that he was heterozygous for Factor V Leiden. A transesophageal echocardiogram had shown a patent foramen ovale, which was subsequently closed percutaneously. He had been started on antiplatelet therapy and had been well until the present symptoms developed. The patient’s history also was notable for dyslipidemia, obesity, and mild coronary artery disease, diagnosed by cardiac catheterization several years earlier. He had stopped Funding: The authors did not receive pharmaceutical industry funds or any other funding in preparation for this manuscript or any related research. Conflicts of Interest: The authors have no conflicts of interest to disclose. Authorship: All authors had access to the data and played a role in writing the manuscript. Requests for reprints should be addressed to Theresa A. Rowe, DO, Johns Hopkins Bayview Medical Center, 4940 Eastern Avenue, Baltimore, MD 21224-2780. E-mail address: [email protected]
0002-9343/$ -see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.amjmed.2010.05.004
smoking cigarettes 24 years earlier. He reported no use of alcohol or illicit substances. He was taking pravastatin and aspirin daily. He had no family history of hypercoagulability disorders or rheumatologic diseases.
ASSESSMENT The patient did not appear to be in distress. He was afebrile and had a blood pressure of 133/84 mm Hg in both arms, a pulse of 64 beats per minute, and a respiration rate of 18 breaths per minute. His oxygen saturation was 99% on room air. His cardiac and lung exams were unremarkable without carotid bruits. His right 4th and 5th digits felt cool compared to his other fingers. No abnormalities were noted in his left hand. His radial and brachial pulses were ⫹2 bilaterally, and his right ulnar pulse was ⫹1. His left ulnar pulse was not palpable but was detected by Doppler ultrasonography. Laboratory findings were significant for a white blood cell count of 6.8 ⫻ 103 cells/mm3, a hematocrit of 40%, and a platelet count of 158 ⫻ 103 cells/mm3. His comprehensive metabolic panel and prothrombin and activated partial thromboplastin times were normal. The Westergren erythrocyte sedimentation rate was 6 mm/h, and the C-reactive protein level was 0.1 mg/dL. A test for antinuclear antibody was negative, and rheumatoid factor was normal. The homocysteine level was 25.9 mol/L (normal, 4.0 – 15.2 mol/L). A computed tomography (CT) angiogram showed occlusion of the right ulnar artery approximately 5 cm above the wrist (Figure 1). Two days later, a second CT angiogram showed restoration of flow in the distal one-third of the ulnar artery near the wrist, coinciding with an improvement in the patient’s symptoms (Figure 2). A transthoracic echocardiogram with agitated saline contrast injection did not reveal an intracardiac shunt.
DIAGNOSIS Although the numbness and paresthesias of the patient’s right 4th and 5th fingers suggested a possible ulnar neuropathy, the pallor in the fingers pointed to a vascular cause;
Rowe et al
Forgotten History
Figure 1 Reconstructed 3-dimensional (3D) image of the right upper extremity showing occlusion of the right ulnar artery approximately 5 cm above the wrist (arrow).
this was confirmed by the CT angiogram. Because the patient repeatedly denied trauma to the hand or wrist, the differential diagnosis consisted of embolus, vasculitis, or thrombosis involving the ulnar artery or a systemic hypercoagulable state. An embolus to the ulnar artery seemed unlikely because the patient had no history of atrial fibrillation and because of the successful patent foramen ovale closure 1 year earlier. He had no systemic signs or symptoms suggesting a medium or large vessel vasculitis, and his rheumatologic panel was negative, with normal inflammatory markers. Ulnar artery thrombosis secondary to atherosclerotic vascular disease seemed unlikely because the ulnar artery is not generally affected by atherosclerosis.2 Although a systemic hypercoagulable state, such as antiphospholipid antibody syndrome, paroxysmal nocturnal hemoglobinuria, or hyperhomocysteinemia, can cause arterial thrombosis, the patient’s previous tests for a hypercoaguable state had yielded entirely negative or normal results. His mild homocysteine elevation seemed unlikely to be related to the ulnar artery occlusion. Although he was heterozygous for factor V Leiden, which is associated with an increased risk of venous thromboembolic disease, whether this mutation has any relation to arterial thrombosis is unclear. Because the patient frequently performed mechanical work on automobile engines, and because other potential causes of ulnar artery occlusion seemed unlikely, we felt that his symptoms were probably a result of unrecognized trauma to the ulnar artery and were most consistent with hypothenar hammer syndrome.
797 The patient was discharged from the hospital, advised to continue taking aspirin and pravastatin, and strongly urged to take precautions against any trauma to the heel of the hand. Folic acid was added to treat mild hyperhomocysteinemia. At his follow-up appointment several weeks after discharge, the patient rather sheepishly admitted that he had just had an important realization. The previous evening, he had been working on a car engine in his garage when he had begun to hit a wrench with the hypothenar eminence of his right hand. At that point, he had immediately realized that he often used his hand as a makeshift hammer, although this fact had escaped his notice when he was questioned during his hospitalization. With the history of trauma established, a diagnosis of hypothenar hammer syndrome was made. This syndrome occurs when repeated trauma to the heel of the hand causes occlusion of the superficial palmar branch of the ulnar artery. People with this syndrome often describe using the ulnar aspect of their hand to strike objects. This repetitive trauma causes damage to the distal ulnar artery as it is compressed against the hook of the hamate bone, resulting in arterial thrombosis or aneurysm formation.3-5 As in our patient, symptoms commonly include digital pain, coldness, pallor, and cyanosis. The condition is typically seen in young men who have occupational or recreational exposure to repetitive hand trauma, such as mechanics, carpenters, and participants in certain sports.7 In a study of 21 randomly selected autopsies, the degree of intimal hyperplasia in the distal ulnar arteries was significantly greater in the right
Figure 2 Reconstructed 3D image of the right upper extremity showing redemonstration of the distal ulnar artery.
798
The American Journal of Medicine, Vol 123, No 9, September 2010
hands of men than in their left hands or in the hands of women.2
MANAGEMENT Conservative management is typically recommended, as it was in this case. Most importantly, patients are encouraged to avoid any trauma to the hand. (In one case series, approximately one-third of patients with signs of hypothenar hammer syndrome recurrence had continued traumatic exposure to the hypothenar eminence.7) Smoking cessation and treatment of dyslipidemia and arterial hypertension also are recommended.3,7 Although controversial, some clinicians recommend calcium channel blockers, antiplatelet agents, or anticoagulation therapy. Patients whose symptoms do not respond to medical treatment and who have an identifiable anatomic predisposition to this syndrome may benefit from reconstructive surgical intervention.4 While hypothenar hammer syndrome is generally considered rare, it may be under-diagnosed. Indeed, in a study by Little and Ferguson, 14% of habitual “hand” hammerers had evidence of the condition.5 Hypothenar hammer syndrome is not well recognized among primary care physicians, and its symptoms can be non-specific and easily confused with those of other well-known conditions affecting the hand, such as ulnar neuropathy, Raynaud phenomenon, vasculitis, or arterial emboli from a cardiac source.6,7 In one case series, 1.13% of cases referred for evaluation of
Raynaud phenomenon had evidence of hypothenar hammer syndrome.7 The key to the diagnosis is a history of repeated trauma to the hypothenar eminence. The clinician must carefully elicit any history of trauma to the hand, as the patient may initially be unaware of such a history. Indeed, the use of one’s hand as a makeshift hammer might be so commonplace among some individuals that they might disregard it as “trauma.” In the present case, failure to elicit a history of trauma to the hypothenar eminence delayed the diagnosis. Of greater importance, had the history come to light a year earlier, the patient might have been spared the procedure to close his patent foramen ovale.
References 1. Santayana G. The Life of Reason Vol. 1: Reason in Common Sense. London, London Constable; 1905. 2. Stone JR. Intimal hyperplasia in the distal ulnar artery: influence of gender and implications for the hypothenar hammer syndrome. Cardiovasc Pathol. 2004;13:20-25. 3. Ablett CT, Hackett LA. Hypothenar hammer syndrome: case reports and brief review. Clin Med Res. 2008;6:3-8. 4. Lifchez S, Higgins, J. Long-term results of surgical treatment for hypothenar hammer syndrome. Plast Reconstr Surg. 2009;124:210-216. 5. Little JM, Ferguson DA. The incidence of the hypothenar hammer syndrome. Arch Surg. 1972;105:684-685. 6. Abudakka M, Pillai A, Al-Khaffaf H. Hypothenar hammer syndrome: rare or underdiagnosed? Eur J Vasc Endovasc Surg. 2006;32:257-260. 7. Marie I, Herve= F, Primard E, et al. Long-term follow-up of hypothenar hammer syndrome. Medicine. 2007;86:334-343.
Those Who Forget Their History Are Condemned to Repeat It Theresa Rowe, DO, Roy C. Ziegelstein, MD, Joyce Jones, MD Department of Medicine, Johns Hopkins Bayview Medical Center, Johns Hopkins University School of Medicine, Baltimore, Md.
PRESENTATION “Those who forget their history are condemned to repeat it.” This sentence, often attributed to the philosopher George Santayana, is actually a misquotation of his comment, “Those who cannot remember the past are condemned to repeat it.”1 However, the misquotation may be more apropos in this case. A 47-year-old, right-handed male truck driver who enjoyed working on automobile engines in his spare time was admitted with pain, numbness, and pallor of the right 4th and 5th fingers, exacerbated by exposure to the cold. The previous night, when washing his hands after working on an engine, he had noticed that the 2 fingers were cold, painful, and pale. They remained that way throughout the night without significant color or temperature change. He denied any fevers, fatigue, recent weight loss, or trauma to his right hand. A year earlier, the patient had experienced an almost identical episode involving the 4th and 5th digits of his left hand. He had been admitted to another hospital on that occasion and could recall no trauma to the left hand at that time. An angiogram had revealed occlusion of the left ulnar artery at the terminus of the palmar arch. An evaluation for a hypercoagulable state had revealed that he was heterozygous for Factor V Leiden. A transesophageal echocardiogram had shown a patent foramen ovale, which was subsequently closed percutaneously. He had been started on antiplatelet therapy and had been well until the present symptoms developed. The patient’s history also was notable for dyslipidemia, obesity, and mild coronary artery disease, diagnosed by cardiac catheterization several years earlier. He had stopped Funding: The authors did not receive pharmaceutical industry funds or any other funding in preparation for this manuscript or any related research. Conflicts of Interest: The authors have no conflicts of interest to disclose. Authorship: All authors had access to the data and played a role in writing the manuscript. Requests for reprints should be addressed to Theresa A. Rowe, DO, Johns Hopkins Bayview Medical Center, 4940 Eastern Avenue, Baltimore, MD 21224-2780. E-mail address: [email protected]
0002-9343/$ -see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.amjmed.2010.05.004
smoking cigarettes 24 years earlier. He reported no use of alcohol or illicit substances. He was taking pravastatin and aspirin daily. He had no family history of hypercoagulability disorders or rheumatologic diseases.
ASSESSMENT The patient did not appear to be in distress. He was afebrile and had a blood pressure of 133/84 mm Hg in both arms, a pulse of 64 beats per minute, and a respiration rate of 18 breaths per minute. His oxygen saturation was 99% on room air. His cardiac and lung exams were unremarkable without carotid bruits. His right 4th and 5th digits felt cool compared to his other fingers. No abnormalities were noted in his left hand. His radial and brachial pulses were ⫹2 bilaterally, and his right ulnar pulse was ⫹1. His left ulnar pulse was not palpable but was detected by Doppler ultrasonography. Laboratory findings were significant for a white blood cell count of 6.8 ⫻ 103 cells/mm3, a hematocrit of 40%, and a platelet count of 158 ⫻ 103 cells/mm3. His comprehensive metabolic panel and prothrombin and activated partial thromboplastin times were normal. The Westergren erythrocyte sedimentation rate was 6 mm/h, and the C-reactive protein level was 0.1 mg/dL. A test for antinuclear antibody was negative, and rheumatoid factor was normal. The homocysteine level was 25.9 mol/L (normal, 4.0 – 15.2 mol/L). A computed tomography (CT) angiogram showed occlusion of the right ulnar artery approximately 5 cm above the wrist (Figure 1). Two days later, a second CT angiogram showed restoration of flow in the distal one-third of the ulnar artery near the wrist, coinciding with an improvement in the patient’s symptoms (Figure 2). A transthoracic echocardiogram with agitated saline contrast injection did not reveal an intracardiac shunt.
DIAGNOSIS Although the numbness and paresthesias of the patient’s right 4th and 5th fingers suggested a possible ulnar neuropathy, the pallor in the fingers pointed to a vascular cause;
Rowe et al
Forgotten History
Figure 1 Reconstructed 3-dimensional (3D) image of the right upper extremity showing occlusion of the right ulnar artery approximately 5 cm above the wrist (arrow).
this was confirmed by the CT angiogram. Because the patient repeatedly denied trauma to the hand or wrist, the differential diagnosis consisted of embolus, vasculitis, or thrombosis involving the ulnar artery or a systemic hypercoagulable state. An embolus to the ulnar artery seemed unlikely because the patient had no history of atrial fibrillation and because of the successful patent foramen ovale closure 1 year earlier. He had no systemic signs or symptoms suggesting a medium or large vessel vasculitis, and his rheumatologic panel was negative, with normal inflammatory markers. Ulnar artery thrombosis secondary to atherosclerotic vascular disease seemed unlikely because the ulnar artery is not generally affected by atherosclerosis.2 Although a systemic hypercoagulable state, such as antiphospholipid antibody syndrome, paroxysmal nocturnal hemoglobinuria, or hyperhomocysteinemia, can cause arterial thrombosis, the patient’s previous tests for a hypercoaguable state had yielded entirely negative or normal results. His mild homocysteine elevation seemed unlikely to be related to the ulnar artery occlusion. Although he was heterozygous for factor V Leiden, which is associated with an increased risk of venous thromboembolic disease, whether this mutation has any relation to arterial thrombosis is unclear. Because the patient frequently performed mechanical work on automobile engines, and because other potential causes of ulnar artery occlusion seemed unlikely, we felt that his symptoms were probably a result of unrecognized trauma to the ulnar artery and were most consistent with hypothenar hammer syndrome.
797 The patient was discharged from the hospital, advised to continue taking aspirin and pravastatin, and strongly urged to take precautions against any trauma to the heel of the hand. Folic acid was added to treat mild hyperhomocysteinemia. At his follow-up appointment several weeks after discharge, the patient rather sheepishly admitted that he had just had an important realization. The previous evening, he had been working on a car engine in his garage when he had begun to hit a wrench with the hypothenar eminence of his right hand. At that point, he had immediately realized that he often used his hand as a makeshift hammer, although this fact had escaped his notice when he was questioned during his hospitalization. With the history of trauma established, a diagnosis of hypothenar hammer syndrome was made. This syndrome occurs when repeated trauma to the heel of the hand causes occlusion of the superficial palmar branch of the ulnar artery. People with this syndrome often describe using the ulnar aspect of their hand to strike objects. This repetitive trauma causes damage to the distal ulnar artery as it is compressed against the hook of the hamate bone, resulting in arterial thrombosis or aneurysm formation.3-5 As in our patient, symptoms commonly include digital pain, coldness, pallor, and cyanosis. The condition is typically seen in young men who have occupational or recreational exposure to repetitive hand trauma, such as mechanics, carpenters, and participants in certain sports.7 In a study of 21 randomly selected autopsies, the degree of intimal hyperplasia in the distal ulnar arteries was significantly greater in the right
Figure 2 Reconstructed 3D image of the right upper extremity showing redemonstration of the distal ulnar artery.
798
The American Journal of Medicine, Vol 123, No 9, September 2010
hands of men than in their left hands or in the hands of women.2
MANAGEMENT Conservative management is typically recommended, as it was in this case. Most importantly, patients are encouraged to avoid any trauma to the hand. (In one case series, approximately one-third of patients with signs of hypothenar hammer syndrome recurrence had continued traumatic exposure to the hypothenar eminence.7) Smoking cessation and treatment of dyslipidemia and arterial hypertension also are recommended.3,7 Although controversial, some clinicians recommend calcium channel blockers, antiplatelet agents, or anticoagulation therapy. Patients whose symptoms do not respond to medical treatment and who have an identifiable anatomic predisposition to this syndrome may benefit from reconstructive surgical intervention.4 While hypothenar hammer syndrome is generally considered rare, it may be under-diagnosed. Indeed, in a study by Little and Ferguson, 14% of habitual “hand” hammerers had evidence of the condition.5 Hypothenar hammer syndrome is not well recognized among primary care physicians, and its symptoms can be non-specific and easily confused with those of other well-known conditions affecting the hand, such as ulnar neuropathy, Raynaud phenomenon, vasculitis, or arterial emboli from a cardiac source.6,7 In one case series, 1.13% of cases referred for evaluation of
Raynaud phenomenon had evidence of hypothenar hammer syndrome.7 The key to the diagnosis is a history of repeated trauma to the hypothenar eminence. The clinician must carefully elicit any history of trauma to the hand, as the patient may initially be unaware of such a history. Indeed, the use of one’s hand as a makeshift hammer might be so commonplace among some individuals that they might disregard it as “trauma.” In the present case, failure to elicit a history of trauma to the hypothenar eminence delayed the diagnosis. Of greater importance, had the history come to light a year earlier, the patient might have been spared the procedure to close his patent foramen ovale.
References 1. Santayana G. The Life of Reason Vol. 1: Reason in Common Sense. London, London Constable; 1905. 2. Stone JR. Intimal hyperplasia in the distal ulnar artery: influence of gender and implications for the hypothenar hammer syndrome. Cardiovasc Pathol. 2004;13:20-25. 3. Ablett CT, Hackett LA. Hypothenar hammer syndrome: case reports and brief review. Clin Med Res. 2008;6:3-8. 4. Lifchez S, Higgins, J. Long-term results of surgical treatment for hypothenar hammer syndrome. Plast Reconstr Surg. 2009;124:210-216. 5. Little JM, Ferguson DA. The incidence of the hypothenar hammer syndrome. Arch Surg. 1972;105:684-685. 6. Abudakka M, Pillai A, Al-Khaffaf H. Hypothenar hammer syndrome: rare or underdiagnosed? Eur J Vasc Endovasc Surg. 2006;32:257-260. 7. Marie I, Herve= F, Primard E, et al. Long-term follow-up of hypothenar hammer syndrome. Medicine. 2007;86:334-343.