Complications
- Significant cosmetic deformities are often seen if the anophthalmic orbit is not treated early.
- Even after proper treatment using conformers, expanders, or surgical treatment, results are often cosmetically disappointing.
- Fitted prostheses are completely immobile.
- The eyelids often show significant malformations and are shortened and immobile.
Prognosis
- True anophthalmos is considered a pediatric ocular emergency.
- As the growth and development of the bony orbit are dependent on the growth of the globe, the absence of an eye or an extremely microphthalmic eye impedes the proper development of the orbit.
- The small bony cavity not only is a cosmetic deformity but also does not allow proper fitting of a prosthesis.
- Even with proper early treatment, results are often disappointing in patients with anophthalmos.
Patient Education
- Inform the child with anophthalmos as well as the family that the treatment of this condition could be a long and complicated one.
- Multiple conformers, expanders, and surgical treatments may be necessary to create an adequate-sized orbital cavity for placement of a proper-fitting prosthesis. In addition, multiple eyelid and conjunctival surgeries may be necessary throughout the child's life.
Shaw GM, Carmichael SL, Yang W, Harris JA, Finnell RH, Lammer EJ. Epidemiologic characteristics of anophthalmia and bilateral microphthalmia among 2.5 million births in California, 1989-1997. Am J Med Genet A. Aug 15 2005;137(1):36-40. [Medline].
Boyd PA, Haeusler M, Barisic I. EUROCAT Report 9: Surveillance of congenital anomalies in Europe 1980-2008. Birth Defects Res A Clin Mol Teratol. Mar 2011;91 Suppl 1:S1. [Medline].
Shah SP, Taylor AE, Sowden JC, Ragge NK, Russell-Eggitt I, Rahi JS. Anophthalmos, microphthalmos, and typical coloboma in the United Kingdom: a prospective study of incidence and risk. Invest Ophthalmol Vis Sci. Jan 2011;52(1):558-64. [Medline].
Roy FH, ed. Ocular Differential Diagnosis. 6th ed. Baltimore, MD: Lippincott, Williams & Wilkins; 1997:263.
Kennedy RE. The effect of early enucleation on the orbit; in animals and humans. Am J Ophthalmol. Aug 1965;60:277-306. [Medline].
Putterman AM. Ocular socket problems. In: Waltman SR, Keates RH, Hoyt CS, eds. Surgery of the Eye. New York: Churchill Livingston; 1988:749-758.
Ahmad ME, Dada R, Dada T, Kucheria K. 14q(22) deletion in a familial case of anophthalmia with polydactyly. Am J Med Genet A. Jul 1 2003;120(1):117-22. [Medline].
Hoover-Fong JE, Cai J, Cargile CB, Thomas GH, Patel A, Griffin CA. Facial dysgenesis: a novel facial syndrome with chromosome 7 deletion p15.1-21.1. Am J Med Genet A. Feb 15 2003;117A(1):47-56. [Medline].
Bakrania P, Robinson DO, Bunyan DJ, et al. SOX2 anophthalmia syndrome: 12 new cases demonstrating broader phenotype and high frequency of large gene deletions. Br J Ophthalmol. Nov 2007;91(11):1471-6. [Medline].
Bardakjian TM, Schneider A. The genetics of anophthalmia and microphthalmia. Curr Opin Ophthalmol. Sep 2011;22(5):309-13. [Medline].
Albernaz VS, Castillo M, Hudgins PA, Mukherji SK. Imaging findings in patients with clinical anophthalmos. AJNR Am J Neuroradiol. Mar 1997;18(3):555-61. [Medline].
Marchac D, Cophignon J, Achard E, Dufourmentel C. Orbital expansion for anophthalmia and micro-orbitism. Plast Reconstr Surg. Apr 1977;59(4):486-91. [Medline].
Mustarde JE. The orbital rim. In: Mustarde JC, Jancsous IT, eds. Plastic Surgery in Infancy and Children. Edinburgh: Churchill Livingston; 1988:150-155.
Tucker SM, Sapp N, Collin R. Orbital expansion of the congenitally anophthalmic socket. Br J Ophthalmol. Jul 1995;79(7):667-71. [Medline].
Krastinova D, Kelly MB, Mihaylova M. Surgical management of the anophthalmic orbit, part 1: congenital. Plast Reconstr Surg. Sep 15 2001;108(4):817-26. [Medline].
Quaranta-Leoni FM. Treatment of the anophthalmic socket. Curr Opin Ophthalmol. Sep 2008;19(5):422-7. [Medline].
Gundlach KK, Guthoff RF, Hingst VH, Schittkowski MP, Bier UC. Expansion of the socket and orbit for congenital clinical anophthalmia. Plast Reconstr Surg. Oct 2005;116(5):1214-22. [Medline].
Cepela MA, Nunery WR, Martin RT. Stimulation of orbital growth by the use of expandable implants in the anophthalmic cat orbit. Ophthal Plast Reconstr Surg. 1992;8(3):157-67; discussion 168-9. [Medline].
Tse DT, Pinchuk L, Davis S, Falcone SF, Lee W, Acosta AC, et al. Evaluation of an integrated orbital tissue expander in an anophthalmic feline model. Am J Ophthalmol. Feb 2007;143(2):317-327. [Medline].
Tse DT, Abdulhafez M, Orozco MA, Tse JD, Azab AO, Pinchuk L. Evaluation of an integrated orbital tissue expander in congenital anophthalmos: report of preliminary clinical experience. Am J Ophthalmol. Mar 2011;151(3):470-82.e1. [Medline].
Quaranta-Leoni FM. Congenital anophthalmia: current concepts in management. Curr Opin Ophthalmol. Sep 2011;22(5):380-4. [Medline].
Mazzoli RA, Raymond WR 4th, Ainbinder DJ, Hansen EA. Use of self-expanding, hydrophilic osmotic expanders (hydrogel) in the reconstruction of congenital clinical anophthalmos. Curr Opin Ophthalmol. Oct 2004;15(5):426-31. [Medline].
Kotlus BS, Dryden RM. Correction of anophthalmic enophthalmos with injectable calcium hydroxylapatite (Radiesse). Ophthal Plast Reconstr Surg. Jul-Aug 2007;23(4):313-4. [Medline].

