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Anophthalmos: Treatment & Medication

Author: Nick Mamalis, MD, Professor, Director of Ophthalmic Pathology, Department of Ophthalmology, University of Utah School of Medicine; Director of Intermountain Ocular Research Center
Coauthor(s): Jacob W Brubaker, MD, Pre-residency Fellowship in Ocular Pathology, University of Utah
Contributor Information and Disclosures

Updated: Sep 25, 2007

Treatment

Medical Care

  • Ocular/orbital
    • A solid conformer may be placed in the orbit to stimulate bony orbital growth and to enlarge the orbital cavity in an attempt to attain normal proportions.
    • Progressively increasing the size of conformers will often help to increase the size of the orbit.
    • An ocular prosthesis may be fitted over the conformer to improve the appearance.
    • In patients with unilateral anophthalmos, they and their families should be aware that the target proportions of a reconstructed orbit are not planned to exactly mirror that of a healthy orbit. In all likelihood, a perfectly normal-looking orbit will not be achieved.

Surgical Care

  • Inflatable expander
    • If conformers are not tolerated or are unsuccessful, an inflatable expander may be placed surgically.
    • The expander works best if placed relatively early in life, especially within the first year.
    • The inflatable silicone expander is placed surgically deep into the orbit and is accessible by a tube placed in the lateral orbital rim.
    • The expander can be gradually filled with liquid (eg, saline) on a weekly or biweekly basis.
    • The advantage of an inflatable expander is that it may allow more rapid and extensive orbital tissue expansion as compared with solid conformers.
  • Self-expanding hydrophilic, osmotic expanders3
    • A new possible treatment is the use of self-inflating expanders.
    • Hydrophilic expanders are placed in their dry, contracted state.  The expanders then expand gradually to their full size via osmotic absorption of surrounding tissue fluid.
    • This method offers the benefit of controllable self-expansion, without the necessity of repeated fittings of solid conformers or surgical placement of external tubing required for inflatable expanders.
    • Long-term biocompatibility studies have not been completed, but early results are promising.
  • Eyelid surgery
    • The increase in the size of a conformer is often limited by shortening of the eyelids in the palpebral fissure, which do not permit passage of a large conformer. The horizontal length of the palpebral fissure may be increased surgically by performing a lateral canthotomy or cantholysis.
    • An additional method to lengthen the eyelids can be accomplished by a combination of skin, mucosal, or cartilage grafts.
  • Orbital surgery
    • If conformers and expanders are unsuccessful, the bony orbit may be expanded surgically. This method is preferred in cases of late referral or insufficient orbital volume.
    • The orbit can be expanded in 3 different directions, as follows: laterally, inferiorly, and superiorly.
    • Surgical expansion of the orbit can be accomplished by dividing the bony orbital rim into 3 parts in a stepwise fashion.
    • Cranial bone grafts may be used to augment deficient orbital contours.
    • Lastly, a bicoronal approach through the scalp may be necessary when the orbital roof has to be elevated.

Consultations

  • Anophthalmos causes serious psychological problems due to not only the absence of an eye but also the disfigurement of the orbital socket and the eyelids. Psychological counseling or consultation may be warranted for these children.

More on Anophthalmos

Overview: Anophthalmos
Differential Diagnoses & Workup: Anophthalmos
Treatment & Medication: Anophthalmos
Follow-up: Anophthalmos
Multimedia: Anophthalmos
References
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References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. 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].

  7. Kennedy RE. The effect of early enucleation on the orbit; in animals and humans. Am J Ophthalmol. Aug 1965;60:277-306. [Medline].

  8. 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].

  9. 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].

  10. Mustarde JE. The orbital rim. In: Mustarde JC, Jancsous IT, eds. Plastic Surgery in Infancy and Children. Edinburgh: Churchill Livingston; 1988:150-155.

  11. Putterman AM. Ocular socket problems. In: Waltman SR, Keates RH, Hoyt CS, eds. Surgery of the Eye. New York: Churchill Livingston; 1988:749-758.

  12. Roy FH, ed. Ocular Differential Diagnosis. 6th ed. Baltimore, MD: Lippincott, Williams & Wilkins; 1997:263.

  13. Tucker SM, Sapp N, Collin R. Orbital expansion of the congenitally anophthalmic socket. Br J Ophthalmol. Jul 1995;79(7):667-71. [Medline].

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Further Reading

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Keywords

primary anophthalmos, true anophthalmos, extreme microphthalmos, anophthalmia, microphthalmia, microphthalmic eye, small eye syndrome, small orbit, malformed globe, enucleation, surgical removal of eye, orbital implant, prosthetic eye, artificial eye, conformers

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Contributor Information and Disclosures

Author

Nick Mamalis, MD, Professor, Director of Ophthalmic Pathology, Department of Ophthalmology, University of Utah School of Medicine; Director of Intermountain Ocular Research Center
Nick Mamalis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and American Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Jacob W Brubaker, MD, Pre-residency Fellowship in Ocular Pathology, University of Utah
Jacob W Brubaker, MD is a member of the following medical societies: American Medical Student Association/Foundation
Disclosure: Nothing to disclose.

Medical Editor

Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado
Ron W Pelton, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Colorado Medical Society, Utah Medical Association, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal, and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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