Abstract
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 (n = 5) or bilateral (n = 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
Highlights
Moebius syndrome (MS) is a rare non-progressive congenital disease, characterized by a complete or partial paralysis of the abducens (CN VI) and facial (CN VII) cranial nerves, either unilateral or bilateral, frequently accompanied by the dysfunction of other cranial nerves and by different types of musculoskeletal malformations [1,2].The prevalence of MS is estimated to be 1/250,000 live births, with equal incidence in both sexes
The 10 relapsed feet were primarily treated with manipulations and casts in all cases, with medial release (MR) in 8 cases, and with Achilles tendon lengthening (ATL) and posterior release (PR) in all cases (Figure 1)
Despite the small number involved in the study, Group 2 showed no relapse and better subjective and functional results in the long-term follow-up compared to other surgical techniques
Summary
Moebius syndrome (MS) is a rare non-progressive congenital disease, characterized by a complete or partial paralysis of the abducens (CN VI) and facial (CN VII) cranial nerves, either unilateral or bilateral, frequently accompanied by the dysfunction of other cranial nerves and by different types of musculoskeletal malformations [1,2].The prevalence of MS is estimated to be 1/250,000 live births, with equal incidence in both sexes. A peculiar clinical sign of MS patients is the complete or partial absence of facial mimicry; we speak of “children without smile”. In MS, orthopedic concerns are reported in the literature with an incidence of 60%, among which clubfoot (CF) is the most relevant condition, present in 32% of patients against around 1 in 1000 births in the general population. They represent, together with facial and eye problems, the most invalidating aspects for patients [2]
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