Uemura Takuya, Okada Mitsuhiro, Terai Hidetomi, Yokoi Takuya, Onode Ema, Shintani Kosuke, Konishi Sadahiko, Nakamura Hiroaki
Department of Orthopaedic Surgery, Osaka General Hospital of West Japan Railway Company, Osaka, Japan.
Department of Orthopaedic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan.
Plast Reconstr Surg Glob Open. 2020 Nov 10;8(11):e3251. doi: 10.1097/GOX.0000000000003251. eCollection 2020 Nov.
Although carpal tunnel syndrome (CTS) is exceedingly rare in children, its prevalence in those with Hunter syndrome, mucopolysaccharidosis type II, is high. With the advent of hematopoietic stem cell transplantation and enzyme replacement therapy, the survival of patients with Hunter syndrome has dramatically improved. With improved longevity in these patients, CTS continues to progress with age. However, most patients with Hunter syndrome with CTS have generally been treated with an open carpal tunnel release (OCTR) only, without considering the severity. Here, we present a mid-term follow-up of a 16-year-old patient with Hunter syndrome associated with severe bilateral CTS successfully treated by the simultaneous opposition tendon transfer with an OCTR to improve the thumb function. Intraoperatively, the median nerve was constricted and flattened with congestion by the transverse carpal ligament. External and internal neurolysis of the scarred median nerve were performed and found epineural fibrosis and tethered epineurium. An intraneural lipoma of the left median nerve was especially resected with epineurotomy. During neurolysis and tendon transfer, the soft tissue was very viscous, a characteristic of mucopolysaccharidoses. Transferring the tension of the palmaris longus tendon to the abductor pollicis brevis for the thumb palmar abduction should be stronger than routine adult patients because the soft tissue such as the tendon excursion is stickier and more contracted in patients with Hunter syndrome. Postoperatively, a thumb spica splint was applied for 3 weeks, and then active motion exercises were cautiously started to prevent joint contracture. Early recognition and surgical intervention for CTS are essential in patients with Hunter syndrome.
尽管腕管综合征（CTS）在儿童中极为罕见，但在患有亨特综合征（黏多糖贮积症II型）的儿童中其患病率却很高。随着造血干细胞移植和酶替代疗法的出现，亨特综合征患者的生存率有了显著提高。随着这些患者寿命的延长，CTS会随着年龄的增长而持续进展。然而，大多数患有亨特综合征且伴有CTS的患者通常仅接受开放性腕管松解术（OCTR）治疗，而未考虑病情的严重程度。在此，我们报告一例16岁患有亨特综合征并伴有严重双侧CTS的患者的中期随访情况，该患者通过同时进行OCTR联合对掌肌腱转移术成功治疗，以改善拇指功能。术中，正中神经被腕横韧带压迫，变窄且充血。对瘢痕化的正中神经进行了内外神经松解，发现存在神经外膜纤维化和神经外膜粘连。尤其对左侧正中神经的神经内脂肪瘤进行了切除并切开神经外膜。在神经松解和肌腱转移过程中，软组织非常黏滞，这是黏多糖贮积症的一个特征。由于亨特综合征患者的肌腱滑动等软组织更黏滞且收缩更明显，将掌长肌腱的张力转移至拇短展肌以实现拇指掌侧外展时，其张力应比常规成年患者更强。术后，应用拇指人字形夹板固定3周，然后谨慎地开始主动活动锻炼以防止关节挛缩。对于亨特综合征患者，早期识别和对CTS进行手术干预至关重要。
