MOBIUS SEQUENCE - FURTHER IN-VIVO SUPPORT FOR THE SUBCLAVIAN ARTERY SUPPLY DISRUPTION SEQUENCE

Mobius sequence 'consists of a congenital bilateral facial nerve palsy and external ophthalmoplegia often associated with malformations of t he limbs and orofacial structures. The pathogenesis of the sequence is a subject of debate. However, a new hypothesis proposes that Mobius s equence results from an interruption of embryonic blood supply (subcla vian artery supply disruption sequence). Here we present an infant wit h bilateral facial nerve palsy (VII), external ophthalmoplegia (IV, VI ), paresis of cranial nerves V, IX, X, XI, and XII, absence of the pec toralis major muscle (Poland anomaly), terminal transverse limb defect s, and absence of the right diaphragm. Also, he was found to have disc rete foci of brainstem calcifications in the region of the dorsal resp iratory group on both CT scan and the histologic sections with microsc opic evidence of diffuse brainstem ''injury.'' The anomalies and histo pathology noted in this infant imply that vascular insufficiency prior to the sixth week of gestation involving the proximal sixth intersegm ental artery may result in the manifestations presented in this report and lend further support for the existence of a subclavian artery sup ply disruption sequence. (C) 1993 Wiley-Liss, Inc.