Congenital Exotropia Clinical Presentation
- Author: Mounir Bashour, MD, CM, FRCS(C), PhD, FACS; Chief Editor: Hampton Roy Sr, MD more...
History
By definition, children present when they are younger than 12 months with a constant outward deviation of the eyes.
Physical
- Unlike other neurologic forms of exotropia, there should be no pupillary or lid involvement. Although craniofacial syndromes can be seen with congenital exotropia, there should be no ptosis or pupillary mydriasis (see Oculomotor Nerve Palsy).
- The eyes should appear diverging with no limitation of adduction.
- Over time, a preference may occur with one eye used consistently for fixation; then, the other eye will develop amblyopia.
- As many as 60% of patients may develop oblique muscle dysfunction, dissociated vertical deviation, and amblyopia. Nystagmus is rare.
Causes
There is a familial predisposition suggestive of an autosomal dominant pattern with incomplete penetrance.
Hunter DG, Kelly JB, Buffenn AN, et al. Long-term outcome of uncomplicated infantile exotropia. J AAPOS. Dec 2001;5(6):352-6. [Medline].
Saunders RA, Trivedi RH. Sensory results after lateral rectus muscle recession for intermittent exotropia operated before two years of age. J AAPOS. Apr 2008;12(2):132-5. [Medline].
Biglan AW, Davis JS, Cheng KP, et al. Infantile exotropia. J Pediatr Ophthalmol Strabismus. Mar-Apr 1996;33(2):79-84. [Medline].
Brodsky MC, Baker RS, Hamed LM. Pediatric Neuro-ophthalmology. 1996.
Hunter DG, Ellis FJ. Prevalence of systemic and ocular disease in infantile exotropia: comparison with infantile esotropia. Ophthalmology. Oct 1999;106(10):1951-6. [Medline].
Matsuo T, Yamane T, Ohtsuki H. Heredity versus abnormalities in pregnancy and delivery as risk factors for different types of comitant strabismus. J Pediatr Ophthalmol Strabismus. Mar-Apr 2001;38(2):78-82. [Medline].
Mohney BG, Huffaker RK. Common forms of childhood exotropia. Ophthalmology. Nov 2003;110(11):2093-6. [Medline].
von Noorden GK. Binocular Vision and Binocular Motility: Theory and Management of Strabismus. 1996.
Wright KW, Buckley EG, Del Monte MA. Pediatric Ophthalmology and Strabismus. 1995.

