Megalocornea Treatment & Management

  • Author: Thomas A Oetting, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: May 21, 2010
 

Medical Care

Medical care for patients with megalocornea includes correction of refractive error and thorough evaluation for findings of anterior megalophthalmos.

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Surgical Care

Surgical amelioration of glaucoma and cataract is performed when necessary.

Cataract is common with megalocornea, and the cataract surgery is difficult. The zonules supporting the lens capsule surrounding the cataract are often weak. Weakened zonules make it more difficult to support an artificial intraocular lens (IOL). This leads to increased surgical complications, such as vitreous loss and loss of lens material to the back of the eye during cataract surgery.[1]

The large size of the anterior segment makes IOL selection difficult as standard IOLs are too small. A standard lens will often not work. Oetting and Newsom at the University of Iowa reported on the use of a special IOL called the Artisan lens (see the image below) that clips onto the iris.[2]

Megalocornea patient with Artisan lens. Megalocornea patient with Artisan lens.

Because this IOL clips onto the iris rather than fitting into the angle, it allows for a safe and stable placement, even with the large anterior segment. Basti and colleagues reported using suture based iris fixation to allow placement of a standard IOL in these large eyes.[3]

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Consultations

Pediatric developmental specialist consultation may be indicated.

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Contributor Information and Disclosures
Author

Thomas A Oetting, MD  Professor, Residency Program Director, Department of Ophthalmology, University of Iowa Carver College of Medicine

Thomas A Oetting, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Mark A Hendrix, MD  Consulting Staff, Department of Ophthalmology, Suburban Hospital, Shady Grove Hospital

Mark A Hendrix, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Fernando H Murillo-Lopez, MD  Senior Surgeon, Unidad Privada de Oftalmologia CEMES

Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

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.

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching

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.

References

  1. Saatci AO, Soylev M, Kavukcu S, Durak I, Saatci I, Memisoglu B. Bilateral megalocornea with unilateral lens subluxation. Ophthalmic Genet. Mar 1997;18(1):35-8. [Medline].

  2. Oetting TA, Newsom TH. Bilateral Artisan lens for aphakia and megalocornea: Long-term follow-up. J Cataract Refract Surg. Mar 2006;32(3):526-8. [Medline].

  3. Basti S, Koch DD. Secondary peripheral iris suture fixation of an acrylic IOL in megalocornea. J Cataract Refract Surg. Jan 2005;31(1):7; author reply 8. [Medline].

  4. Chang DF. Siepser slipknot for McCannel iris-suture fixation of subluxated intraocular lenses. J Cataract Refract Surg. Jun 2004;30(6):1170-6. [Medline].

  5. Kraft SP, Judisch GF, Grayson DM. Megalocornea: a clinical and echographic study of an autosomal dominant pedigree. J Pediatr Ophthalmol Strabismus. Sep-Oct 1984;21(5):190-3. [Medline].

  6. Mackey DA, Buttery RG, Wise GM, Denton MJ. Description of X-linked megalocornea with identification of the gene locus. Arch Ophthalmol. Jun 1991;109(6):829-33. [Medline].

  7. Maumenee IH. The cornea in connective tissue diseases. Ophthalmology. Oct 1978;85(10):1014-7. [Medline].

  8. Meire FM. Megalocornea. Clinical and genetic aspects. Doc Ophthalmol. 1994;87(1):1-121. [Medline].

  9. Meire FM, Bleeker-Wagemakers EM, Oehler M, Gal A, Delleman JW. X-linked megalocornea. Ocular findings and linkage analysis. Ophthalmic Paediatr Genet. Sep 1991;12(3):153-7. [Medline].

  10. Meire FM, Delleman JW. Biometry in X linked megalocornea: pathognomonic findings. Br J Ophthalmol. Oct 1994;78(10):781-5. [Medline].

  11. OMIM. Online Mendelian Inheritance in Man, OMIM(TM). McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University (Baltimore, MD) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, MD), 2000. Available at: http://www.ncbi.nlm.gov/Omim/.

  12. Skuta GL, Sugar J, Ericson ES. Corneal endothelial cell measurements in megalocornea. Arch Ophthalmol. Jan 1983;101(1):51-3. [Medline].

  13. Srivastava AK, McMillan S, Jermak C, Shomaker M, Copeland-Yates SA, et al. Integrated STS/YAC physical, genetic, and transcript map of human Xq21.3 to q23/q24 (DXS1203-DXS1059). Genomics. Jun 1 1999;58(2):188-201. [Medline].

  14. Starck T, Hersh PS, Kenyon KR. Corneal dysgeneses, dystrophies, and degenerations. In: Principles and Practice of Ophthalmology. Vol 2. 2000:695-696.

  15. Wood WJ, Green WR, Marr WG. Megalocornea: A clinico-pathologic clinical case report. Md State Med J. Jul 1974;23(7):57-60. [Medline].

  16. Assia EI, Segev F, Michaeli A. Cataract surgery in megalocornea Comparison of 2 surgical approaches in a single patient. J Cataract Refract Surg. Dec 2009;35(12):2042-6. [Medline].

 
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Central mosaic dystrophy sometimes noted in megalocornea.
Megalocornea.
Megalocornea patient with Artisan lens.
B-scan ultrasound showing a large flat iris.
Aphakic megalocornea patient with 15 mm white to white.
 
 
 
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