Möbius sequence: further in vivo support for the subclavian artery supply disruption sequence.

Möbius sequence consists of a congenital bilateral facial nerve palsy and external ophthalmoplegia often associated with malformations of the limbs and orofacial structures. The pathogenesis of the sequence is a subject of debate. However, a new hypothesis proposes that Möbius sequence results from an interruption of embryonic blood supply (subclavian artery supply disruption sequence). Here we present an infant with bilateral facial nerve palsy (VII), external ophthalmoplegia (IV, VI), paresis of cranial nerves V, IX, X, XI, and XII, absence of the pectoralis major muscle (Poland anomaly), terminal transverse limb defects, and absence of the right diaphragm. Also, he was found to have discrete foci of brainstem calcifications in the region of the dorsal respiratory group on both CT scan and the histologic sections with microscopic evidence of diffuse brainstem "injury." The anomalies and histopathology noted in this infant imply that vascular insufficiency prior to the sixth week of gestation involving the proximal sixth intersegmental artery may result in the manifestations presented in this report and lend further support for the existence of a subclavian artery supply disruption sequence.

Variation in the origin of the inferior alveolar nerve.

The inferior alveolar nerve and maxillary artery were studied in 40 human heads through infratemporal dissection; in one specimen, a unusual variation in the origin of the inferior alveolar nerve and its relationship with the surrounding structures was observed. The inferior alveolar nerve originated from the mandibular nerve by two roots and the second part of the maxillary artery was incorporated between them. An embryologic origin of this variation and its clinical implications is discussed. Because the maxillary artery ran between the two roots of the inferior alveolar nerve, and the nerve was fixed between the foramen ovale and mandibular foramen, tension and compression of the nerve from arterial pulsation could cause mandibular neuralgia.

Prenatal sonographic evidence supporting an in utero developmental etiology of Möbius sequence.

Möbius sequence features masklike facies with sixth and seventh nerve palsy and frequently micrognathia thought to result from a neuromuscular deficiency in early movement of the mandible. Failure to thrive in infancy is commonly due to feeding and aspiration difficulties. We present the first description of prenatal sonographic findings associated with this lesion, which also support an in utero developmental etiology of this rare condition.