An Anxious Boy with Moebius Syndrome: A Case Report

INTRODUCTION

Moebius syndrome (MS) is a rare, congenital, non-progressive neurological condition characterised by weakness or paralysis of multiple cranial nerves. The sixth(Abducens) and seventh (Facial) cranial nerves are most frequently affected resulting in classical clinical presentations of (1) “mask- like” facies due to lack of facial expression, usually bilaterally and (2) limited abduction of one or both eyes in affected individuals. MS affects both genders equally and occurs in all ethnicities. The exact incidence of MS is unknown. The aetiology of MS remains unspecified but may be due to genetic and/or environmental factors.  Intellectual disabilities have been reported in approximately 10-15% of cases. Common orofacial manifestations include microstomia, micrognathia, cleft palate or high vault palate, deep fissured tongue, bifid uvula, hypodontia, hypoplastic teeth, hypoplastic upper lip, incompetent lips and dental caries. These manifestations could lead to various dental issues requiring multidisciplinary management. This case report presents the long-term dental management of an anxious boy with underlying MS.

CASE DESCRIPTION

The patient was initially referred to the Department of Paediatric Dentistry in 2013 when he was 2 years 5 months old for management of gingival bleeding.  He was diagnosed with severe early childhood caries and generalised gingivitis and undergone his first comprehensive dental treatment (CDT) under General anaesthesia (GA). Subsequently, he was on our periodic dental reviews. Unfortunately, due to persistent poor parental motivation, he was consistently categorised as high caries risk with poor oral hygiene. In addition to his short attention span and anxious behaviour, chair-side dental management was not a viable long-term treatment option. Therefore, he underwent two more CDTs under GAs in 2015 and 2020. His MS features include mask-like face and unable to abduct eyes, micrognathia, incompetence lips and hypoplastic upper lip.  Intra-oral examination revealed poor oral hygiene, dental caries, fissured tongue and Class II division I malocclusion with an overjet of 8mm and deep bite. For his long-term dental plan, he may benefit from Orthodontic treatment. However, he is currently not a suitable candidate due to his poor oral hygiene and anxious behaviour. Thus, he continues to be on our periodic preventive follow up.

LEARNING POINT/ CONCLUSION

As with all Paediatric dental patients, basic preventive measures and periodic follow ups are paramount in MS patients not only to maintain good oral health and quality of life but to ameliorate patient’s attitude towards dental treatment and subsequently future multidisciplinary dental treatment.