MacDougal B, Weeks P M, Wray R C
Plast Reconstr Surg. 1977 Feb;59(2):260-3.
Patients with Hurler's syndrome (MPS-1H), I-cell disease (ML-II) and pseudo-Hurler's syndrome (ML-III) had median nerve compression and triggering of the fingers which limited finger extension. To our knowledge, this combination has not been reported previously in patients with mucopolysaccharidoses and related disorders. In all of our 3 cases the median nerve was compressed by thickened flexor tenosynovium. Synovectomy and resection of the volar carpal ligament improved the hand function in all, including the mentally retarded patient with Hurler's syndrome. Release of the fibroosseous tunnel in two patients was followed by an increased range of motion (but not full extension). A fourth patient, without a mucopolysaccharide storage disorder, also had the combination of trigger finger and carpal tunnel syndrome.
患有胡尔勒综合征(黏多糖贮积症Ⅰ型-H)、I型细胞病(黏脂贮积症Ⅱ型)和假胡尔勒综合征(黏脂贮积症Ⅲ型)的患者出现正中神经受压以及手指卡顿,这限制了手指伸展。据我们所知,这种情况此前在黏多糖贮积症及相关疾病患者中尚未有过报道。在我们所有3例病例中,正中神经均被增厚的屈肌腱鞘膜压迫。滑膜切除术和掌侧腕横韧带切除术改善了所有人的手部功能,包括患有胡尔勒综合征的智障患者。两名患者松解纤维骨性隧道后,活动范围增加(但未完全伸展)。第四例患者没有黏多糖贮积症,也出现了扳机指和腕管综合征的组合。