Möbius and Möbius-like patients: etiology, diagnosis, and treatment options
Section snippets
History
The unusual combination of developmental facial paralysis and restriction of horizontal eye movement was first described by v. Graefe in 1880 [1].
The German neurologist Paul Julius Möbius was the first to compare his own cases with case reports from Chrisholm [2] and Haarlan [3]. He suggested that the bilateral paralysis of the 7th and 6th cranial nerves might be an independent pathological entity with the presumptive term the infantile Kernschwund [4], [5]. His name was later used as an eponym.
Clinical findings
The paralysis of the orbicularis oculi muscle is the first sign of Mobius' syndrome and can be observed soon after birth; however, the condition is unfamiliar within the pediatric specialty, so diagnosis is made in later ages. Upon further development it becomes noticeable that the child does not smile or show other features of nonverbal communication. The mask-like faces are the dominant clinical finding associated with drooling and indistinct speech. If the facial paralysis is incomplete, the
Differential diagnosis
Physicians should consider the following possible differential diagnoses: glossopalatine-ankylosis syndrome, hypoglossal-hypodactyly syndrome, and Charlie-M syndrome [23]. In addition, there is a growing list of unrelated conditions with bilateral facial diplegia that must be considered [42], [43]. The combination of multiple cranial nerve deficiencies is also found in patients with CHARGE association [44].
Epidemiology
Facial paralysis is uncommon in newborns; the incidence rate has been reported to be between 0.2% and 6.9% [45]. Möbius' syndrome is very rare; in the past 120 years, approximately 320 cases have been reported in the literature. Most of these are smaller cohorts focusing on exceptional findings. The incidence in the population has not been determined yet [34]. The majority of cases occur sporadically, but undiagnosed cases are highly possible. Despite the apparent clinical features, the
Classification
Möbius' syndrome presents heterogeneously. The great variety of clinical findings dictates the need for a classification system. A clinical classification by Tentamy and McKusik [22] describes three groups: (1) isolated VI and VII cranial nerve palsy; (2) VI and VII cranial nerve palsy in association with arthrogryposis multiplex congenita; and (3) VI and VII cranial nerve palsies with associated limb anomalies. Based on neuropathological observations in one case and evaluation of 14 additional
Etiology
Because of the variety of clinical findings the etiology of Mobius' syndrome is still unknown. It remains unknown whether the developmental mishap is dysplastic or degenerative. Pathological findings differ between a primary ectodermal dysplasia of the midbrain with secondary mesodermal defect [50], [51] and a primary mesodermal defect [52], [53]. Besides myopathy [53], findings have pointed toward a peripheral neuropathy [54]. MRI, CT scans, and autopsy materials have shown calcification in
Treatment
Surgical treatment of Möbius patients focuses mainly on correction of limb and ocular deficiencies (eg, talipes correction and strabismus surgery). The inability of Möbius patients to express happiness, sadness, or anger through nonverbal communication can result in severe introversion and reclusive personality [82], [83], [84]. Because of their expressionless appearance and speech impairment, these patients are often considered to be mentally retarded [85], [86].
The restoration of even a small
Summary
The surgical goal in Möbius patients is far more modest and differs from patients with unilateral developmental facial paralysis. It is impossible to restore a true smile in these mask-like, expressionless faces. Despite sophisticated microneurovascular transplantations, movement can only be restored along one vector and enhanced firmness in the cheeks, thus multiple differentiated facial animation is not achievable.
A detailed neurological evaluation can identify possible motor donors or
Acknowledgements
The authors express their gratitude to Diane Mocion for providing medical illustrations and David Beck for photographs.
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Physiopathologic Bases of Moebius Syndrome: Combining Genetic, Vascular, and Teratogenic Theories
2024, Pediatric NeurologyModified gracilis muscle flap in facial reanimation: U-shaped design
2023, Journal of Plastic, Reconstructive and Aesthetic SurgeryTransfer of the deep temporal nerve for eyelid reconstruction in Mobius syndrome – an anatomic feasibility study and proposed surgical approach
2022, Journal of Plastic, Reconstructive and Aesthetic SurgeryCitation Excerpt :Mobius syndrome is a form of bilateral congenital facial palsy that occurs in approximately 1 in 50,000 live births.2 In addition to the facial nerve, several other cranial nerves may be variably affected.3 A particular challenge to provide dynamic reconstruction of the facial musculature for these patients is to find an expendable motor nerve to power the introduction of new musculature.
Long-term results of facial animation surgery in patients with Moebius syndrome
2020, Journal of Cranio-Maxillofacial SurgeryCitation Excerpt :However, facial palsy is the feature most frequently addressed, because of its associations with severe functional, cosmetic, and social impairments. For patients with Moebius syndrome, it is impossible to communicate emotions (for example, by smiling) (Terzis and Noah, 2002). Over the past 20 years, facial animation surgery featuring a gracilis neuromuscular transplant has become the gold standard procedure.
Diagnosis and treatment of speech disorders in children with Moebius syndrome
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Early surgical treatment with free muscle transfer has been reported as the first line of treatment for these patients [6,7]. Terzis and Noah [8] created a classification system: Classic MS: Complete bilateral facial and abducens nerve paralysis; incomplete MS: Clinical picture of MS with some residual motor function on one side of the face, and Moebius-like: Unilateral facial paralysis, but additional cranial nerve palsies are present. Some reports have supported the statement that the state of the art for treating patients with MS is facial reanimation by gracilis muscle free transfer.
Plastic and Reconstructive Surgery
2019, A Practice of Anesthesia for Infants and Children