Portal de Periódicos da CAPES

Hypothenar Hammer Syndrome: Rare or Underdiagnosed?

2006; Elsevier BV; Volume: 32; Issue: 3 Linguagem: Inglês

10.1016/j.ejvs.2006.02.013

1532-2165

M. Abudakka, Anand Pillai, Haytham Al-Khaffaf,

Orthopedic Surgery and Rehabilitation

Hypothenar hammer Syndrome (HHS) is a condition characterised by digital ischaemia as a result of repetitive trauma to the hypothenar eminence of the hand. It occurs in people who repeatedly use the palm of the hand as a hammer to push, grind or twist objects. It is a curable and a preventable cause of upper digital ischemia. In this report we present a case of HHS and discuss the causes and pathogenesis of this syndrome. We review the incidence, clinical characteristics, differential diagnosis, investigation and treatment. Hypothenar hammer Syndrome (HHS) is a condition characterised by digital ischaemia as a result of repetitive trauma to the hypothenar eminence of the hand. It occurs in people who repeatedly use the palm of the hand as a hammer to push, grind or twist objects. It is a curable and a preventable cause of upper digital ischemia. In this report we present a case of HHS and discuss the causes and pathogenesis of this syndrome. We review the incidence, clinical characteristics, differential diagnosis, investigation and treatment. A 68 year-old Caucasian male was referred to the vascular outpatient clinic by a dermatologist with gangrenous changes in the tip of the right middle finger. He had already had an amputation of the tip of his right little and left middle fingers after he had developed painful non-healing ulcers. He described a 10 years progressive history of pain and cold intolerance in the fingers of both hands. The gentleman had been working in the machinery industry for more than 30 years. His work required continuous use of spanners, which involved using his unprotected hands to hammer the spanner whenever it didn't yield to ordinary pressure. His medical and family histories were unremarkable. He had smoked for many years but stopped 2 years ago. On examination, the tip of his right middle finger was gangrenous. His right little and left middle finger tips had been amputated. Blood pressure and pulses were normal and equal on both arms. Unfortunately Allen's test was not done at that time. He had a normal FBC, ESR, serum glucose, Urea and Electrolytes, LFT, Antinuclear factor, rheumatoid factor, Cryoglobulins and thrombophilia screen. His C-ANCA titre was positive at 1:1000. Anti-MPO antibodies and anti-PR3 antibodies were negative. Plain radiography of the hand showed no bony abnormality. His recent echocardiogram was normal. Arrangements were made for him to have an amputation of the terminal phalanx of his right middle finger. Two weeks later he had a colour duplex scan which revealed normal blood flow within the subclavian, brachial, radial and ulnar arteries in both arms with damped flow in both palmar arches. Subsequently an angiogram was carried out (Fig. 1, Fig. 2, Fig. 3), and showed that the ulnar arteries on both sides were thready with progressive narrowing and complete occlusion in the lower third. There was a deficient ulnar component of the palmar arch, no digital arteries supplying the amputated stump of the little finger on the right side, and the digital arteries supplying the other fingers were also diseased.Fig. 2Angiogram of the left hand showing a thready ulnar artery with occlusion of the lower third, and deficient ulnar component of the palmar arch.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig. 3Angiogram of the left forearm showing a normal radial artery. The ulnar artery appears normal in the upper two thirds of the forearm and then becomes thready and gradually occluded in the lower third.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Histology of the amputated middle finger showed no evidence of primary vasculitis. In view of his past history and both Duplex scan and angiographic findings a diagnosis of Hypothenar Hammer Syndrome was made. He was recently reviewed in the outpatient clinic and seemed to be managing well. There was no evidence of any further ischaemic changes in either hand (Fig. 4). Therefore a conservative approach has been adopted, particularly as the lesions in his ulnar arteries are not amenable to surgical intervention. The Ulnar artery, as it enters the hand anterior to the flexor retinaculum between the pisiform bone and the hook of hamate (Guyon's canal), is fixed to the surrounding structure over a length of 2–3 cm. Between its deep palmar branch and the beginning of the superficial palmar arch, it is only protected by the skin, subcutaneous tissue, palmaris brevis muscle and superficial apeneurosis.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar, 2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar Frequent blunt trauma to the hypothenar eminence compresses the unprotected ulnar artery against the hook of hamate triggering vasospasm of the artery.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar, 3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 10Conn Jr., J. Bergan J.J. Bell J.L. Hypothenar hammer syndrome: posttraumatic digital ischemia.Surgery. 1970 Dec; 68: 1122-1128Google Scholar Continued trauma causes damage of the arterial intima which encourages platelet aggregation and thrombus formation. Distal embolization of the digital arteries exacerbates ischemia.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 11Zimmerman N.B. Occlusive vascular disorders of the upper extremity.Hand Clin. 1993 Feb; 9: 139-150Google Scholar Less commonly the repetitive blunt trauma results in ulnar artery aneurysm formation.2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar In a more recent study it has been found that over 90% of sufferers have abnormal angiographic features in the contralateral asymptomatic hand, suggesting that this syndrome is more likely to happen in patients with a pre-existing palmar artery fibrodysplasia.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 12Ferris B.L. Taylor Jr., L.M. Oyama K. McLafferty R.B. Edwards J.M. Moneta G.L. et al.Hypothenar hammer syndrome: proposed etiology.J Vasc Surg. 2000 Jan; 31: 104-113Google Scholar Despite the fact that the radial artery is much less commonly affected by a blunt trauma, as it is distant from the point of impact,3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 13Kleinert H.E. Burget G.C. Morgan J.A. Kutz J.E. Atasoy E. Aneurysms of the hand.Arch Surg. 1973; 106: 554-557Google Scholar it has been found that a vasospasm of the radial artery might occur secondary to ulnar artery injury.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 11Zimmerman N.B. Occlusive vascular disorders of the upper extremity.Hand Clin. 1993 Feb; 9: 139-150Google Scholar HHS was first described by Von Rosen6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar, 8Von Rosen S. Ein Fall von Thrombose in der Arteria ulnarix nach Einwirkung von stumpfer Gewalt.Acta Chir Scand. 1934; 73: 500-506Google Scholar in 1934 and since then several cases have been described. The largest cohort study prospectively enrolled 1300 patients, 21 of which had HHS giving an incidence of 1.6%.12Ferris B.L. Taylor Jr., L.M. Oyama K. McLafferty R.B. Edwards J.M. Moneta G.L. et al.Hypothenar hammer syndrome: proposed etiology.J Vasc Surg. 2000 Jan; 31: 104-113Google Scholar Although HHS is widely regarded as a rare condition2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar, 5Liskutin J. Dorffner R. Resinger M. Silberbauer K. Mostbeck G. Hypothenar hammer syndrome.Eur Radiol. 2000; 10: 542Google Scholar, 12Ferris B.L. Taylor Jr., L.M. Oyama K. McLafferty R.B. Edwards J.M. Moneta G.L. et al.Hypothenar hammer syndrome: proposed etiology.J Vasc Surg. 2000 Jan; 31: 104-113Google Scholar this may be because cases are asymptomatic. This was supported by Little and Ferguson,4Little J.M. Ferguson D.A. The incidence of the hypothenar hammer syndrome.Arch Surg. 1972 Nov; 105: 684-685Google Scholar who examined 79 workers who were habitual hypothenar hammerers and found 14% with objective evidence of occlusion. None of them had symptoms of sufficient severity to interfere with work activities. On the other hand a physician survey was conducted that supported the fact that this syndrome is underdiagnosed.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar HHS is an occupational disease that has been described in a number of industries in which the workers use their hands to pound or to push, including carpenters,2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar motor mechanics, metal workers and lathe operators. Furthermore, HHS has also been described in mountain bikers14Applegate K.E. Spiegel P.K. Ulnar artery occlusion in mountain bikers.J Sports Med Phys Fitness. 1995 Sep; 35: 232-234Google Scholar and many other sports including baseball, volley ball,6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar badminton,7Koga Y. Seki T. Caro L.D. Hypothenar hammer syndrome in a young female badminton player. A case report.Am J Sports Med. 1993 Nov–Dec; 21: 890-892Google Scholar tennis,15Noel B. Hayoz D. A tennis player with hand claudication.Vasa. 2000 May; 29: 151-153Google Scholar hand ball, softball, karate, weight lifting and hockey.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar Kaji et al.16Kaji H. Honma H. Usui M. Yasuno Y. Saito K. Hypothenar hammer syndrome in workers occupationally exposed to vibrating tools.J Hand Surg [Br]. 1993 Dec; 18: 761-766Google Scholar found 24 arteriographically diagnosed cases among 293 subjects diagnosed as vibration disease among a population study of 330 workers who were exposed to vibration in mining, forestry and other industries. In this series the right hand was involved in 53% of the cases, the left in 25% and both in 22%. Similarly Conn et al.10Conn Jr., J. Bergan J.J. Bell J.L. Hypothenar hammer syndrome: posttraumatic digital ischemia.Surgery. 1970 Dec; 68: 1122-1128Google Scholar reported bilateral disease in 2 of 13 patients. HHS it is often incorrectly diagnosed,5Liskutin J. Dorffner R. Resinger M. Silberbauer K. Mostbeck G. Hypothenar hammer syndrome.Eur Radiol. 2000; 10: 542Google Scholar or diagnosed at a stage where irreversible consequences have already taken place. Differential diagnosis of upper extremity digital ischemia includes primary Raynaud's disease, Raynaud's phenomenon associated with underlying connective tissue disorder, buerger's disease, vasculitis, arterial emboli from a cardiac source, atherosclerosis with secondary thrombosis, thoracic outlet obstruction and hypothenar hammer syndrome.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar, 2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar HHS presentation maybe initially confused with that of Raynaud's phenomenon. Therefore initial exclusion of other causes of Raynaud's phenomenon should be performed, such as scleroderma, systemic lupus erythematosus, or rheumatic disease. Distinguishing manifestations of HHS from classic Raynaud's phenomenon were summarised by Spencer Green and colleagues (Table 1).18Spencer-Green G. Morgan G.J. Brown L. FitzGerald O. Hypothenar hammer syndrome: an occupational cause of Raynaud's phenomenon.J Rheumatol. 1987 Oct; 14: 1048-1051Google Scholar Supporting the above mentioned distinguishing factors; Pineda19Pineda C.J. Weisman M.H. Bookstein J.J. Saltzstein S.L. Hypothenar hammer syndrome. Form of reversible Raynaud's phenomenon.Am J Med. 1985 Nov; 79: 561-570Google Scholar has stated that although pallor and cyanosis may appear, it is notable that hyperaemic redness was absent.Table 1HHS; distinction from classic Raynaud's phenomenon18Spencer-Green G. Morgan G.J. Brown L. FitzGerald O. Hypothenar hammer syndrome: an occupational cause of Raynaud's phenomenon.J Rheumatol. 1987 Oct; 14: 1048-1051Google Scholar■Male preponderance.■Occupational history of repetitive hand and wrist trauma.■Asymmetric distribution.■Absence of the hyperaemic phase.■Diminished ulnar/radial pulses.■Digital ulcers in areas supplied by affected vessel. Open table in a new tab While Allen's test maybe useful in diagnosis, Kaji et al.16Kaji H. Honma H. Usui M. Yasuno Y. Saito K. Hypothenar hammer syndrome in workers occupationally exposed to vibrating tools.J Hand Surg [Br]. 1993 Dec; 18: 761-766Google Scholar found it to be negative in 17% of their cases. Doppler examination can be helpful.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar Taute et al.20Taute B.M. Behrmann C. Cappeller W.A. Podhaisky H. Ultrasound image of the hypothenar hammer syndrome.Ultraschall Med. 1998 Oct; 19: 220-224Google Scholar found that colour duplex sonography enabled distinction between HHS and other causes of digital ischemia. Arteriography has been described as the gold standard test, which will differentiate HHS from other vascular abnormalities in the hand.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar, 2Van de Walle P.M. Moll F.L. De Smet A.A. The hypothenar hammer syndrome: update and literature review.Acta Chir Belg. 1998 Jun; 98: 116-119Google Scholar, 3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar It is worth mentioning that 3 papers have reviewed HHS attributed to vibration exposure. Noel et al.17Noel B. Holtz J. Savolainen H. Depairon M. Hand-arm vibration syndrome with proximal ulnar artery occlusion.Vasa. 1998 Aug; 27: 176-178Google Scholar reported a HHS case with 25 years exposure to vibration. Lee and Evans23Lee E.H. Evans J.G. Vibration-induced white finger disease: a case report.Can J Surg. 1984 Sep; 27: 513-514Google Scholar reported a single case from Canada, with a vibration-induced white finger, but with a positive Allen's test and arteriographically confirmed absence of the superficial palmar arch. Kaji et al, also reported 24 cases of HHS among 330 vibration-exposed workers. Hand-Arm vibration syndrome (HAVS) classically presents with Raynaud's phenomenon.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar HHS usually affects the digits mainly supplied by the superficial palmar arch (medial 3 digits), while the lateral 2 digits are unlikely to be affected as the deep palmar arch is complete in 97% of the HHS cases.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar Therefore, it is highly likely that HHS might present in a similar pattern to that of HAVS or a Raynaud's like symptoms. However the absence of typical hyperaemic flush in HHS is of distinguishing value along with a positive Allen's test.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar In our case the patient lost the terminal phalanx of the right little, middle and left middle fingers which is consistent with the pattern of HHS described above. Most authorities suggest conservative management should be tried before interventional treatment is planned. This includes cessation of the offending activity and the avoidance of exacerbating factors.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 21McLafferty R.B. Edwards J.M. Taylor Jr., L.M. Porter J.M. Diagnosis and long-term clinical outcome in patients diagnosed with hand ischemia.J Vasc Surg. 1995 Oct; 22 ([discussion 367–369]): 361-367Google Scholar Smoking cessation is essential,6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar, 22Spittell P.C. Spittell J.A. Occlusive arterial disease of the hand due to repetitive blunt trauma: a review with illustrative cases.Int J Cardiol. 1993 Mar; 38: 281-292Google Scholar low-lipid diet is also important and repeated venesections to reduce smoking induced polycythemia has also been suggested.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar, 24Wieczorek I. Farber A. Alexander K. Hypothenar hammer syndrome successfully managed with intravenous prostaglandin E1 and heparin and with correction of the thrombogenic risk profile. A case report.Angiology. 1996 Nov; 47: 1111-1116Google Scholar Anti-platelet therapy should also be considered3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar and intra-venous heparin and prostaglandin E1 maybe useful.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar Vasodilators, such as calcium channels blockers and cervical sympathectomy may also be helpful although the latter may not be beneficial if collateral vessels are already maximally vasodilated.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar Surgical options include segmental ulnar artery excision with vein grafting in patients with severe symptoms and poor collateral circulation in whom conservative management has failed.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar, 3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar, 11Zimmerman N.B. Occlusive vascular disorders of the upper extremity.Hand Clin. 1993 Feb; 9: 139-150Google Scholar In cases with ulnar artery aneurysms, Ulnar artery ligation to prevent further digital embolisations has been described.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar Sometimes resection of the aneurysm and end-to-end anastomosis of the Ulnar artery may be necessary.5Liskutin J. Dorffner R. Resinger M. Silberbauer K. Mostbeck G. Hypothenar hammer syndrome.Eur Radiol. 2000; 10: 542Google Scholar Finally amputation of ulcerated necrotic finger tips maybe required in advanced cases.3Tsavellas G. Huang A. Ranaboldo C.J. Soft-tissue case 42. Hypothenar hammer syndrome.Can J Surg. 2001 Dec; 44 (466–467): 409Google Scholar In conclusion, HHS is not as rare as it is thought to be.6Cooke R.A. Hypothenar hammer syndrome: a discrete syndrome to be distinguished from hand-arm vibration syndrome.Occup Med (Lond). 2003 Aug; 53: 320-324Google Scholar, 9Gaylis H. Kushlick A.R. The hypothenar hammer syndrome.S Afr Med J. 1976 Jan 31; 50: 125-127Google Scholar It is a curable and a preventable cause of upper limb digital ischemia.1Wernick R. Smith D.L. Bilateral hypothenar hammer syndrome: an unusual and preventable cause of digital ischemia.Am J Emerg Med. 1989 May; 7: 302-306Google Scholar Occupational or recreational trauma maybe not volunteered by these patients, therefore such history should be carefully obtained and investigations should be conducted before irreversible consequences take place.