黏多糖贮积症II型（亨特综合征）背景下的腕管综合征
Carpal Tunnel Syndrome in the Setting of Mucopolysaccharidosis II (Hunter Syndrome).
作者信息
Argenta Anne E, Davit Alexander
机构信息
Department of Plastic Surgery, Medical College of Wisconsin, Milwaukee, Wis.; and Department of Plastic Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pa.
出版信息
Plast Reconstr Surg Glob Open. 2017 Aug 24;5(8):e1477. doi: 10.1097/GOX.0000000000001477. eCollection 2017 Aug.
BACKGROUND
Carpal tunnel syndrome (CTS) is a rare finding in children, but heavily represented in pediatric patients with mucopolysaccharidoses. Diagnosis is a challenge due to lack of the stereotypical symptomatic complaints and relies on examination and objective nerve conduction studies.
METHODS
We present a case of delayed presentation of CTS in a 12-year-old boy with Hunter syndrome, followed by a review of the literature.
RESULTS
Patient Z.D. presented with minimal reported CTS symptoms but advanced median nerve damage on electromyography. He underwent bilateral carpal tunnel release with median nerve neurolysis and flexor tenosynovectomies. Intraoperative examination demonstrated the presence of a "waist sign" of the median nerve and moderate flexor tenosynovial hypertrophy bilaterally. Parents reported mild subjective improvement of dexterity and fine motor skills postoperatively.
CONCLUSION
To optimize functional outcome, routine screening for CTS and intervention at an early age are emphasized in the mucopolysaccharidoses population.
背景
腕管综合征（CTS）在儿童中较为罕见，但在患有黏多糖贮积症的儿科患者中却很常见。由于缺乏典型的症状性主诉，诊断具有挑战性，且依赖于检查和客观的神经传导研究。
方法
我们报告一例12岁患有亨特综合征的男孩延迟出现CTS的病例，并对相关文献进行综述。
结果
患者Z.D.报告的CTS症状轻微，但肌电图显示正中神经有严重损伤。他接受了双侧腕管松解术，同时进行了正中神经神经松解术和屈肌腱鞘切除术。术中检查发现双侧正中神经存在“束腰征”以及中度屈肌腱鞘肥厚。家长报告术后患儿的灵活性和精细运动技能有轻度主观改善。
结论
为优化功能结局，强调对黏多糖贮积症患者人群进行CTS的常规筛查并在早期进行干预。
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