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MOBIUS SYNDROME

Harish S Hosalkar*, Ad Kulkarni**,Mg Yagnik***, Hetal Shah+, Priti Gujar++
*Senior Registrar; **Intern; ***Consultant Hon HOD, Paediatric Orthopaedics; +Lecturer, Dept. of Anaesthesia; ++Lecturer, Dept. of Radiology, Bai Jerbai Wadia Hospital for Children, Parel, Mumbai.

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Mobius syndrome is a rare syndrome, the chief features of which are mask like facies with loss of abduction of eyes and partial or complete facial nerve palsy with associated other anomalies. A brief review of a case of this syndrome and its management is presented.

 

INTRODUCTION

The distinctive features of mobius syndrome are congenital facial paralysis and abduction weakness, but limbs, chestwall, spine and soft tissue can also be involved. [1] We report a case of an infant with mobius syndrome.

CASE REPORT

Two month male child presented to our clinic with bilateral talipes equinovarus. He was first born child of normal, unrelated parents, the perinatal history was normal with no significant family history.

On examination, child had following features : Mask - like asymmetric facies with unilateral incomplete VII and also VI nerve palsy. There was hypodactyly of index and middle finger of right hand, pectoralis major hypoplasia, bilateral talipes equinovarus and hypoglossia. Suckling reflex was poor, leading to feeding difficulties.

Clinico-radiological assessment was done to rule out major cardiac and solid organ anomalies. X-rays of club foot revealed the severity of it and ruled out any bony pathology. MRI of brain revealed no hypoplasia of central brainstem nuclei.

Repeated manipulation under general anaesthesia was carried out to treat the club foot. Recovery was uneventful.

Consultation with ENT and ophthalmology specialist was taken for managing cranial nerve anomalies.

DISCUSSION

Mobius syndrome first described by Mobius in 1888 includes congenital facial paresis and eye abduction weakness as characteristic stigmata of syndrome. The facial palsy is commonly bilateral, frequently asymmetric and often incomplete tending to spare lower face and platysma. Abduction defect may be unilateral or bilateral and esotropia is common.

Aetiology is unknown. Whether the primary defect is maldevelopment of cranial nerve nuclei, hypoplasia of muscles or a combination of central and peripheral factors is unclear. Gestational factors such as trauma, illness and intake of various drugs such as misoprostol for abortion. [2]

Associated developmental defects include ptosis, palatal lingual palsy, hearing defects, pectoral and lingual muscle defects, micrognathia, syndactyly, supernumerary digits or absence of hands, feet, finger or toes. About one third of patients have talipes equinovarus, which might be the consequence of neurological deficiency relative to early foot movement.

Recently Abramson DL, Cohen MM, et al have prepared a CLUFT system (C - cranial nerve, L - lower limb, U - upper limb, F - face, T - thorax) for categorization and comparison of mobius patient for phenotypic and management outcome studies. [3]

In conclusion mobius syndrome is commonly of sporadic occurrence in an otherwise normal family, although rare instances of autosomal dominant inheritance with variable expression have been reported.

REFERENCES

1. Richard RM. The mobius syndrome. J Bone Joint Surg, 1953; 41-A : 473.
2. NEJM, Jun 25, 1988; 338 (26) : 1881-5.
3. Plastic and Reconstructive Surgery, Sept., 1998; 102 (4) : 961-7.

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