====== Möbius Syndrome ======
    * Described by Möbius as “Congenital Facial Diplegia” with bilateral abducens palsy in 1888
    * Diplegia= symmetrical bilateral palsy
====Cause====
  * Pathogenesis is unclear
  * Deletion/translocation in long arm of chromosome 13 in a few families
  * 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, brachydactyly, agenesis of digits, clubfoot
  * Swallowing and speech abnormalites from cranial V, IX and X palsies
  * Craniofacial abnormalities
    * Micrognathia, Microstomia, Ear abnormalities, Bifid uvula, cleft palate
  * 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>strabismus syndrome}}