POLAND-MOBIUS SYNDROME-THE POSSIBLE COMMON DEVELOPMENTAL PATHOGENESIS

Dr. Sujata Alawani*

ABSTRACT

Introduction: Mobius syndrome is a rare disorder characterised by congenital non-progressive unilateral or bilateral VI and VII cranial nerve paresis with or without involvement of other cranial nerves. Poland syndrome is another rare congenital abnormality of the chest wall, characterised by unilateral partial or total absence of the pectoralis major muscle and ipsilateral symbrachydactyly. An infant with features of both Mobius syndrome and Poland syndrome is presented in this case report. Case Report: Two days old baby was brought with complains of drooling of milk on feeding. On examination, baby had left sided facial palsy, bilateral abducent nerve palsy, absent right pectoralis major muscle and homolateral symbrachydactyly. Baby also had congenital talipes equino varus of left foot. This work conforms to the values laid down in the Declaration of Helsinki (1964). The protocol of this study has been approved by the relevant ethical committee related to our institution in which it was performed. All subjects gave full informed consent to participate in this study. Discussion: The etiology of these syndromes is multifactorial. Both these syndromes have been attributed to vascular disruption sequence. The recurrent presentation of combination of these two syndromes provides further evidence to a possible common developmental pathogenesis referred to as the subclavian artery disruption sequence.

Keywords: Mobius syndrome characterized pectoralis major muscle and homolateral symbrachydactyly.