Ghanem I, Zeller R, Seringe R
Université René Descartes, Paris V.
Rev Chir Orthop Reparatrice Appar Mot. 1996;82(2):152-60.
Although Charcot-Marie-Tooth disease (CMT) is known to be the most common neuromuscular cause of pes cavovarus, other paralytic deformities of the foot may be present with hereditary motor and sensory neuropathies (HMSN). The purpose of our review is to analyze these foot deformities and to assess the results of the different therapeutic methods used.
We evaluated 66 patients who had HMSN and had a total of 127 foot deformities. Fifty three patients had CMT, 6 patients Déjerine Sottas disease (DS), and 7 patients had an unclassified HMSN. The average age at diagnosis was of 9 years and 11 months. There were 35 males and 31 females. The deformity was unilateral in 5 cases. In 50 bilateral cases, the severity of the deformity was not similar in both feet. In three bilateral cases, the deformity was completely asymmetrical. The chief complaint was mainly deformity in all cases, followed by subtalar or ankle instability in 57 cases. There were 105 cases of cavus or cavovarus, and 22 cases or valgus or planovalgus deformity (8 of which changed spontaneously to cavovarus). A non surgical treatment was undertaken in 57 cases for minor deformity. Soft tissue release with or without osteotomies was done in 39 cases, and triple arthrodesis in the remaining 31 cases. A clawtoe deformity was treated operatively in 14 cases.
The mean follow-up period was 6 years and 9 months for non operated feet and 7 years and 10 months for operated feet (all of the surgically treated feet were reviewed after the end of growth). Three patients of the non operated group and 8 patients of the surgically treated feet underwent triple arthrodesis for a recurrence of the deformity. Thirty nine per cent of the feet which underwent triple arthrodesis (a total of 42 feet) were considered to have fair or bad result at 6 years and 3 months follow-up period.
The foot deformity in HMSN is of a wide variety. A valgus flat foot is not uncommon, especially in DS and unclassified neuropathies. The young age of the patient is not a contrindication to surgical management. Even if minor previous surgeries do not always succeed in avoiding recurrence of the deformity, they nevertheless prepare the foot fort a possible triple arthrodesis, that will be done in better anatomical conditions.
虽然已知夏科-马里-图斯病（CMT）是高弓足内翻最常见的神经肌肉病因，但足部的其他麻痹性畸形也可能与遗传性运动和感觉神经病（HMSN）相关。我们综述的目的是分析这些足部畸形，并评估所采用的不同治疗方法的效果。
我们评估了66例患有HMSN且共有127处足部畸形的患者。53例患有CMT，6例患有德热里纳-索塔斯病（DS），7例患有未分类的HMSN。诊断时的平均年龄为9岁11个月。男性35例，女性31例。畸形单侧出现5例。在50例双侧畸形病例中，双足畸形的严重程度不相似。在3例双侧畸形病例中，畸形完全不对称。所有病例的主要诉求主要是畸形，其次是57例距下关节或踝关节不稳。有105例高弓足或高弓足内翻，22例外翻或扁平外翻畸形（其中8例自发转变为高弓足内翻）。57例轻度畸形患者采用非手术治疗。39例进行了软组织松解，可伴或不伴截骨术，其余31例进行了三关节融合术。14例爪形趾畸形接受了手术治疗。
未手术足部的平均随访期为6年9个月，手术足部为7年10个月（所有接受手术治疗的足部在生长结束后进行复查）。未手术组有3例患者，手术治疗的足部有8例患者因畸形复发接受了三关节融合术。在6年3个月的随访期，接受三关节融合术的足部（共42只）中有39%被认为效果一般或较差。
HMSN中的足部畸形多种多样。外翻扁平足并不少见，尤其是在DS和未分类的神经病中。患者年龄小并非手术治疗的禁忌证。即使先前的小手术并不总能成功避免畸形复发，但它们仍可为足部后续可能进行的三关节融合术创造条件，且三关节融合术将在更好的解剖条件下进行。
