Poland-mbius syndrome

General: Rare congenital disorder that is a combination of Mbius syndrome and Poland anomaly; has been proposed that Mbius syndrome, the Poland anomaly, and the Klippel-Feil defect all result from a transient interruption during the sixth week of gestation in the development of the subclavian artery and its branches.

Ocular: Bilateral sixth nerve paralysis; no movement of upper lid in horizontal gaze; no globe retraction during adduction; vertical nystagmus; diplopia; chronic keratitis; corneal ulcer.

Clinical: Paresis of the sixth and seventh cranial nerves; bilateral absence of pectoralis muscle and ipsilateral hand and digit anomalies; chronic drooling; speech difficulties; masklike facial expression.

Bavinck JN, Weaver DD. Subclavian artery disruption sequence: hypothesis of a vascular etiology from Poland, Klippel-Feil and Mobius anomalies. Am J Med Genet 1986; 23:903-918.

Gadoth N, et al. Mobius syndrome and limb abnormalities. J Pediatr Ophthalmol 1977; 14:134-l38.

Hopper KD, et al. Poland-Mobius syndrome. South Med J 1985; 78:523-527.

Wishnick MM, et al. Mobius syndrome and limb abnormalities with dominant inheritance. Ophthalmic Pediatr Gen 1983; 2:277-281.