De Pellegrin Maurizio, Marcucci Lorenzo, Brogioni Lorenzo, Prati Giovanni
Pediatric Orthopedic and Traumatology Unit, San Raffaele Hospital, 20132 Milan, Italy.
Department of Orthopedic and Traumatology, San Raffaele Hospital, 20132 Milan, Italy.
Children (Basel). 2021 Apr 19;8(4):310. doi: 10.3390/children8040310.
Moebius syndrome (MS) is a rare disease, with paralysis of the VI and VII cranial nerves, frequently associated with clubfoot (CF). The aim of this study was to evaluate surgical treatment of CF in MS, providing its peculiarities. Between 1990 and 2019, we collected data of 11 MS patients with unilateral ( = 5) or bilateral ( = 6) CF, for a total of 17 feet (9R,8L). Six patients (3M,3F) for a total of 10 feet (6R,4L) were treated elsewhere, performing first surgery at an average age of nine months, and in our hospital for relapse surgery at an average age of 4.5 years (Group 1). Five patients (3M, 2F), for a total of seven feet (3R,4L), were primarily treated in our hospital with a peritalar release according to McKay at an average age of 9.4 months (Group 2). Diméglio score was used to assess CF severity. Three questionnaires were submitted for evaluation of subjective and functional results: American Orthopedics Foot and Ankle Society for Hindfoot (AOFAS), Foot and Ankle Outcome Score (FAOS), and Foot and Ankle Ability Measure (FAAM). Average AOFAS/FAOS/FAMM scores were 82.8, 84.8, and 82.3 for Group 1, and 93.2, 94.7, and 95.1 for Group 2 at an average follow-up of 16.9 and 13.3 years, respectively. The average Diméglio score improved from 15.5 to 4.8 in the long-term follow-up in Group 1 and from 14.6 to 3.8 in Group 2. The comparison between the groups showed better results for AOFAS, FAOS, and FAAM scores for Group 2, particularly for pain, function, and foot alignment and for the post-surgical Diméglio score. CF in MS is more severe and presented a higher relapse rate (58.8%) than idiopathic CF. Peritalar release showed no relapse and better subjective and functional results in the long-term follow-up compared to other surgical techniques.
莫比乌斯综合征（MS）是一种罕见疾病，伴有第六和第七颅神经麻痹，常与马蹄内翻足（CF）相关。本研究的目的是评估MS中CF的手术治疗，并阐述其特点。1990年至2019年间，我们收集了11例患有单侧（=5）或双侧（=6）CF的MS患者的数据，共计17只脚（9只右脚，8只左脚）。6例患者（3例男性，3例女性），共计10只脚（6只右脚，4只左脚）在其他地方接受了治疗，首次手术的平均年龄为9个月，在我院进行复发手术的平均年龄为4.5岁（第一组）。5例患者（3例男性，2例女性），共计7只脚（3只右脚，4只左脚）在我院接受了首次治疗，根据麦凯方法进行距周松解，平均年龄为9.4个月（第二组）。采用迪梅廖评分评估CF的严重程度。提交了三份问卷以评估主观和功能结果：美国足踝外科协会后足评分（AOFAS）、足踝结果评分（FAOS）和足踝能力测量（FAAM）。第一组的平均AOFAS/FAOS/FAMM评分分别为82.8、84.8和82.3，第二组在平均随访16.9年和13.3年时分别为93.2、94.7和95.1。在长期随访中，第一组的平均迪梅廖评分从15.5提高到4.8，第二组从14.6提高到3.8。两组之间的比较显示，第二组的AOFAS、FAOS和FAAM评分结果更好，尤其是在疼痛、功能、足部对线方面以及术后迪梅廖评分方面。MS中的CF比特发性CF更严重，复发率更高（58.8%）。与其他手术技术相比，距周松解在长期随访中未出现复发，主观和功能结果更好。
