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| + | ====== Möbius Syndrome ====== | ||
| + | * Described by Möbius as “Congenital Facial Diplegia” with bilateral abducens palsy in 1888 | ||
| + | * Diplegia= symmetrical bilateral palsy | ||
| + | ====Cause==== | ||
| + | * Pathogenesis is unclear | ||
| + | * Deletion/ | ||
| + | * Timing of insult 4-6 weeks gestation when cranial nerve nuclei are rapidly developing | ||
| + | * Trauma, illness or toxic exposure | ||
| + | ====Clinical Features==== | ||
| + | * 6th and 7th nerve palsies | ||
| + | * Usually bilateral but may be asymmetric | ||
| + | * Esotropia most common | ||
| + | * “Mask-like” facies | ||
| + | * If incomplete palsy- upper division of facial nerve involved | ||
| + | ====Other Ocular features: | ||
| + | * Small palpebral fissures | ||
| + | * Epicanthal folds | ||
| + | * Hypertelorism | ||
| + | * Exposure or neurotrophic keratitis | ||
| + | * Situs inversus of retinal vessels | ||
| + | * Entropion | ||
| + | * Ptosis | ||
| + | * Head tilt | ||
| + | * Amblyopia | ||
| + | * Gaze palsy | ||
| + | ====Other Systemic Features==== | ||
| + | * Extremities | ||
| + | * Syndactyly, polydactyly, | ||
| + | * Swallowing and speech abnormalites from cranial V, IX and X palsies | ||
| + | * Craniofacial abnormalities | ||
| + | * Micrognathia, | ||
| + | * Dextrocardia | ||
| + | * Defective musculature | ||
| + | * Missing pectoral and trapezius muscles | ||
| + | * Absence of sternal head of pectoralis major | ||
| + | * Rib defects | ||
| + | * Tongue hypoplasia | ||
| + | * Mild Mental retardation | ||
| + | ===First signs:=== | ||
| + | * Difficulty sucking, drooling, incomplete closure of eyelids | ||
| + | * Lack of smiling response | ||
| + | * Other cranial nerve abnormalities: | ||
| + | * V, IX,X and XII can be involved | ||
| + | * Craniofacial abnormalities | ||
| + | ====Evaluation==== | ||
| + | * Strabismus (Esotropia) | ||
| + | * Amblyopia | ||
| + | * Corneal health | ||
| + | * Pediatric Genetics evaluation | ||
| + | * Craniofacial abnormalities in differential diagnosis | ||
| + | * Nager syndrome (acrofacial dysostosis) | ||
| + | * Neuromuscular disease in differential diagnosis | ||
| + | * Facioscapulohumeral (FSH) muscular dystrophy- shoulders primarily affected | ||
| + | * Congenital or infantile myotonic dystrophy (slow relaxation of muscles after contraction) | ||
| + | * Muscle wasting, cataracts, heart conduction defects, endocrine abnormalities | ||
| + | * Charcot-Marie-Tooth disease | ||
| + | * progressive loss of muscle and touch sensation in extremities | ||
| + | * Usually in late childhood or early adulthood | ||
| + | * Often first present with foot drop and claw toe | ||
| + | ====Strabismus Management==== | ||
| + | * Abnormal Extraocular muscles | ||
| + | * Hypoplasia, aplasia and fibrous bands | ||
| + | * Forced duction testing | ||
| + | * Vertical Rectus transposition | ||
| + | * Medial rectus botox (more effective if muscle not tight) | ||
| + | * Medial rectus recession | ||
| + | |||
| + | {{tag> | ||