Van Heest A E, House J, Krivit W, Walker K
Department of Orthopaedic Surgery, University of Minnesota, Minneapolis 55455, USA.
J Hand Surg Am. 1998 Mar;23(2):236-43. doi: 10.1016/S0363-5023(98)80120-2.
The role of surgical intervention for carpal tunnel syndrome (CTS) and trigger digits in children with mucopolysaccharide storage disorders (MPSDs) has not been clearly defined, particularly as the treatment of the underlying disease has advanced to include bone marrow transplantation. This study reviews our experience in the treatment of CTS and trigger digits in 22 children with MPSDs who were evaluated for CTS by electromyographic (EMG)/nerve conduction velocity (NCV) testing. Seventeen children were diagnosed with CTS by EMG/NCV testing and were treated with bilateral open surgical release with or without flexor tenosynovectomy. The EMG/NCV testing revealed normal results in 5 patients who are subsequently being monitored. Forty-five digits in 8 children were diagnosed clinically with trigger digits. Nineteen digits were treated by annular pulley release alone. Twenty-six digits were treated by annular pulley release with partial flexor digitorum superficialis tendon resection. The average age at the time of hand surgery was 6.3 years, and at the time of follow-up, 9.6 years. Postoperative EMG/NCV testing in 7 children showed 1 with improvement and 6 with normalization. None of the patients undergoing carpal tunnel release went on to develop thenar atrophy or absent sensibility, as has been reported in untreated cases. Patients were evaluated for triggering digits both by preoperative tendon palpation and by intraoperative flexor tendon excursion at the time of open carpal tunnel release. All patients undergoing trigger release had improved active digital flexion seen at the final follow-up visit. Because of the very high incidence of CTS and trigger digits in this population, the authors currently recommend routine screening of EMG/NCV for all children with MPSDs. Early surgical intervention for nerve compression and stenosing flexor tenosynovitis can maximize hand function in these children.
手术干预在黏多糖贮积症（MPSD）患儿腕管综合征（CTS）和扳机指治疗中的作用尚未明确界定，尤其是随着基础疾病的治疗进展到包括骨髓移植。本研究回顾了我们对22例MPSD患儿CTS和扳机指的治疗经验，这些患儿通过肌电图（EMG）/神经传导速度（NCV）测试评估CTS。17例患儿经EMG/NCV测试诊断为CTS，接受了双侧开放性手术松解，部分患儿还进行了屈肌腱鞘切除术。5例患者的EMG/NCV测试结果正常，随后进行监测。8例患儿的45个手指临床诊断为扳机指。19个手指仅接受了环形滑车松解术。26个手指接受了环形滑车松解术并部分切除指浅屈肌腱。手部手术时的平均年龄为6.3岁，随访时为9.6岁。7例患儿术后的EMG/NCV测试显示，1例改善，6例恢复正常。与未治疗病例的报道不同，接受腕管松解术的患者均未出现鱼际肌萎缩或感觉缺失。通过术前肌腱触诊和开放性腕管松解术时的术中屈肌腱活动度评估扳机指患者。所有接受扳机指松解术的患者在末次随访时均出现主动手指屈曲改善。由于该人群中CTS和扳机指的发病率非常高，作者目前建议对所有MPSD患儿进行常规EMG/NCV筛查。早期手术干预神经压迫和狭窄性屈肌腱鞘炎可使这些患儿的手部功能最大化。
