关键词: Hunter syndrome MPS II peroneal nerve release tarsal tunnel release

Mesh : Humans Male Adolescent Mucopolysaccharidosis II / surgery complications Tarsal Tunnel Syndrome / surgery etiology Peroneal Neuropathies / etiology surgery Peroneal Nerve / surgery Nerve Compression Syndromes / surgery etiology

来  源:   DOI:

Abstract:
BACKGROUND: Children with Hunter syndrome have a high prevalence of nerve compression syndromes given the buildup of glycosaminoglycans in the tendon sheaths and soft tissue structures. These are often comorbid with orthopedic conditions given joint and tendon contractures due to the same pathology. While carpal tunnel syndrome and surgical treatment has been well-reported in this population, the literature on lower extremity nerve compression syndromes and their treatment in Hunter syndrome is sparse.
METHODS: We report the case of a 13-year-old male with a history of Hunter syndrome who presented with toe-walking and tenderness over the peroneal and tarsal tunnel areas. He underwent bilateral common peroneal nerve and tarsal tunnel releases, with findings of severe nerve compression and hypertrophied soft tissue structures demonstrating fibromuscular scarring on pathology. Post-operatively, the patient\'s family reported subjective improvement in lower extremity mobility and plantar flexion.
CONCLUSIONS: In this case, peroneal and tarsal nerve compression were diagnosed clinically and treated effectively with surgical release and postoperative ankle casting. Given the wide differential of common comorbid orthopedic conditions in Hunter syndrome and the lack of validated electrodiagnostic normative values in this population, the history and physical examination and consideration of nerve compression syndromes are tantamount for successful workup and treatment of gait abnormalities in the child with Hunter syndrome.
摘要:
背景:考虑到肌腱鞘和软组织结构中糖胺聚糖的积聚,亨特综合征儿童的神经压迫综合征患病率很高。由于相同的病理,关节和肌腱挛缩通常与骨科疾病并存。虽然腕管综合征和手术治疗在这个人群中已经得到了很好的报道,关于下肢神经压迫综合征及其在亨特综合征中的治疗的文献很少。
方法:我们报告了一个有亨特综合征病史的13岁男性病例,该病例表现为在腓骨和髌骨隧道区域的脚趾行走和压痛。他接受了双侧腓总神经和髌骨隧道松解术,发现严重的神经压迫和肥大的软组织结构,在病理学上显示纤维肌肉瘢痕。术后,患者家属报告主观上下肢活动能力和足底屈曲改善。
结论:在这种情况下,临床诊断为腓骨和tal神经受压,并通过手术松解术和术后踝关节铸造有效治疗。鉴于亨特综合征中常见的骨科合并症差异很大,并且该人群中缺乏经过验证的电诊断规范值,病史和体格检查以及神经压迫综合征的考虑等同于成功的检查和治疗Hunter综合征患儿的步态异常。
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