Marie Isabelle, Hervé Fabien, Primard Etienne, Cailleux Nicole, Levesque Hervé
From Department of Internal Medicine (IM, FH, NC, HL) and Department of Radiology (EP), Rouen University Hospital, Rouen, France.
Medicine (Baltimore). 2007 Nov;86(6):334-343. doi: 10.1097/MD.0b013e31815c95d3.
Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. This prompted us to conduct the current retrospective study to 1) evaluate the prevalence of HHS in patients with Raynaud phenomenon and 2) assess the short-term and long-term outcome in patients with HHS. From 1990 to 2006, 4148 consecutive patients were referred to the Department of Internal Medicine at the University of Rouen medical center for evaluation of Raynaud phenomenon using nailfold capillaroscopy. HHS was diagnosed in 47 of these 4148 patients (1.13% of cases).Forty-three patients (91.5%) had occupational exposure to repetitive palmar trauma. The more common occupations were factory worker (21.3%), mason (12.8%), carpenter (10.6%), and metal worker (10.6%); the mean duration of occupational exposure to repetitive palmar trauma at HHS diagnosis was 21 years. One patient (2.1%) had recreational exposure (aikido training) to repetitive trauma of the palmar ulnar artery, and 3 other patients (6.4%) developed HHS related to a single direct injury to the hypothenar area. Clinical manifestations were more often unilateral (87.2%) involving the dominant hand (93%). HHS complications included digital ischemic symptoms (ischemia: n = 21, necrosis: n = 20) and irritation of the sensory branch of the ulnar nerve (n = 11). In HHS patients, angiography demonstrated occlusion of the ulnar artery in the area of the Guyon space (59.6%), aneurysm of the ulnar artery in the area of the Guyon space (40.4%), and embolic multiple occlusions of the digital arteries (57.4%). All patients were advised to change their occupational exposure. They were given vasodilators, including calcium channel blocker (n = 37) and buflomedil (n = 12); 36 patients (76.6%) also received oral platelet aggregation inhibitors. Twenty-one patients with digital ischemia/necrosis were further given hemodilution therapy to reduce the hematocrit level to 35%. In 3 patients with HHS-related digital necrosis who exhibited partial improvement with vasodilators, prostacyclin analog therapy (a 5-day regimen of intravenous prostacyclin analog) was instituted, resulting in complete healing of digital ulcer in these 3 patients. Other conservative treatment options included controlling risk factors (smoking cessation, low-lipid diet, therapy for arterial hypertension) and careful local wound care of fingers in the 20 patients with digital necrosis. Only 2 patients, exhibiting digital necrosis and multiple digital artery occlusions, with nonthrombotic ulnar artery aneurysm underwent reconstructive surgery, that is, resection of the aneurysm with end-to-end anastomosis of the ulnar artery. The median length of follow-up in patients with HHS was 15.9 months. Thirteen patients (27.7%) exhibited clinical recurrences of HHS; the median time of HHS recurrence onset was 11 months. Outcome of HHS relapse was favorable with conservative measures in all cases. Awareness of HHS is required to increase suspicion of the disorder so that further exposure to risk factors like repetitive hypothenar trauma can be avoided for these patients; this is of great importance for their overall prognosis. We found favorable outcomes in most patients after conservative measures were initiated; therefore we suggest that surgery may be undertaken in the subgroup of patients who exhibit partial improvement while receiving conservative therapy. Finally, because we observed recurrence of HHS in 27.7% of patients, we note that HHS patients require close follow-up, including both regular and systematic physical vascular examination.
小鱼际锤状指综合征(HHS)是继发性雷诺现象的一种罕见形式,主要发生在将手部小鱼际用作锤子的人群中;钩骨钩撞击Guyon管内尺动脉的掌浅支,导致尺动脉闭塞和/或动脉瘤形成。在HHS患者中,掌侧尺动脉的此类损伤可能导致手部严重的血管功能不全,并伴有指动脉闭塞。迄今为止,仅有少数系列研究分析了HHS患者的长期预后。这促使我们开展当前的回顾性研究,以1)评估雷诺现象患者中HHS的患病率,以及2)评估HHS患者的短期和长期预后。1990年至2006年,连续4148例患者因雷诺现象被转诊至鲁昂大学医学中心内科,接受甲襞毛细血管镜检查评估。这4148例患者中有47例被诊断为HHS(占病例的1.13%)。43例患者(91.5%)有职业性反复掌部创伤暴露。较常见的职业有工厂工人(21.3%)、泥瓦匠(12.8%)、木匠(10.6%)和金属工人(10.6%);诊断HHS时职业性反复掌部创伤暴露的平均时长为21年。1例患者(2.1%)有娱乐性(合气道训练)反复掌侧尺动脉创伤暴露,另外3例患者(6.4%)因小鱼际区域单次直接损伤而发生HHS。临床表现多为单侧(87.2%),累及优势手(93%)。HHS并发症包括手指缺血症状(缺血:n = 21,坏死:n = 20)和尺神经感觉支激惹(n = 11)。在HHS患者中,血管造影显示Guyon管区域尺动脉闭塞(59.6%)、Guyon管区域尺动脉动脉瘤(40.4%)以及指动脉栓塞性多处闭塞(57.4%)。所有患者均被建议改变职业暴露。给予他们血管扩张剂,包括钙通道阻滞剂(n = 37)和丁咯地尔(n = 12);36例患者(76.6%)还接受了口服血小板聚集抑制剂治疗。21例手指缺血/坏死患者进一步接受血液稀释治疗,将血细胞比容水平降至35%。在3例HHS相关手指坏死且使用血管扩张剂后有部分改善的患者中,采用了前列环素类似物治疗(静脉注射前列环素类似物5天疗程),这3例患者的手指溃疡完全愈合。其他保守治疗选择包括控制危险因素(戒烟、低脂饮食、治疗动脉高血压)以及对20例手指坏死患者的手指进行仔细的局部伤口护理。仅有2例表现为手指坏死和多处指动脉闭塞且伴有非血栓性尺动脉瘤的患者接受了重建手术,即切除动脉瘤并行尺动脉端端吻合术。HHS患者的中位随访时长为15.9个月。13例患者(27.7%)出现HHS临床复发;HHS复发开始的中位时间为11个月。所有病例中HHS复发的预后采用保守措施均良好。需要提高对HHS的认识,以增加对该疾病的怀疑,从而避免这些患者进一步暴露于如反复小鱼际创伤等危险因素;这对他们的总体预后至关重要。我们发现大多数患者在采取保守措施后预后良好;因此我们建议,对于在接受保守治疗时仅有部分改善的患者亚组可考虑手术治疗。最后,由于我们观察到27.7%的患者出现HHS复发,我们指出HHS患者需要密切随访,包括定期和系统的身体血管检查。