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Secondary Congenital Glaucoma Workup

  • Author: Inci Irak Dersu, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 14, 2014
 

Laboratory Studies

See the list below:

  • Aniridia: Chromosome analysis and genetic counseling are important parts of the workup.
  • Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities.
  • Many other systemic anomaly – associated glaucoma conditions require appropriate workup including genetic analysis, laboratory studies, and imaging to diagnose and manage the patient's systemic diseases.
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Imaging Studies

See the list below:

  • Neurofibromatosis
    • The most serious complication of neurofibromatosis type 1 (NF-1) is optic nerve and/or chiasm glioma (in 15% of patients).
    • An MRI of the orbit and brain is needed to screen for the tumor.
  • Sturge-Weber syndrome
    • In Sturge-Weber syndrome, calcium deposits located predominantly in the occipital lobe of the brain parenchyma can be detected by a CT scan. These deposits follow the cerebral convolutions and give the appearance of a railroad track.
    • Angiomatous malformations, decreased cerebral volume, and increased choroidal plexus volume are the other findings of Sturge-Weber syndrome. An MRI can delineate these findings better than a CT scan.
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Other Tests

See the list below:

  • Peters anomaly
    • B-scan is needed to evaluate intraocular structures that are obstructed by the corneal opacity.
    • Electrophysiologic tests occasionally are needed to evaluate the visual potential of the eye prior to making decision on intervention.
  • Nanophthalmos: Pachymetry, A- and B-scan ultrasonography, and ultrasound biomicroscopy (UBM) are useful in helping to establish a diagnosis.
  • All glaucoma types
    • Pachymetry readings are important in all types of glaucoma, including childhood glaucoma, to adjust for IOP readings.
    • In a small study, the mean central corneal thickness of children with different types of childhood glaucoma was measured. According to this study, in 34 children with glaucoma, IOP was overestimated by 3 mm Hg or more in 41.2% of them. In children with Sturge-Weber syndrome, the mean central corneal thickness was 591.9 +/- 23.1 µm, and, in children with aniridia, the mean central corneal thickness was 754.5 +/- 92.6 µm.[1]
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Contributor Information and Disclosures
Author

Inci Irak Dersu, MD, MPH Associate Professor of Clinical Ophthalmology, State University of New York Downstate College of Medicine; Attending Physician, SUNY Downstate Medical Center, Kings County Hospital, and VA Harbor Health Care System

Inci Irak Dersu, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Martin B Wax, MD Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Research and Development, Head, Ophthalmology Discovery Research and Preclinical Sciences, Alcon Laboratories, Inc

Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Society for Neuroscience

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, American Society for Laser Medicine and Surgery, American Academy of Ophthalmology, American Medical Association

Disclosure: Nothing to disclose.

References
  1. Lopes JE, Wilson RR, Alvim HS, Shields CL, Shields JA, Calhoun J, et al. Central corneal thickness in pediatric glaucoma. J Pediatr Ophthalmol Strabismus. 2007 Mar-Apr. 44(2):112-7. [Medline].

  2. Iwach AG, Hoskins HD Jr, Hetherington J Jr, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. 1990 Jul. 97(7):904-9. [Medline].

  3. Agarwal HC, Sandramouli S, Sihota R, Sood NN. Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. 1993 Jun. 24(6):399-402. [Medline].

  4. Kargi SH, Koc F, Biglan AW, Davis JS. Visual acuity in children with glaucoma. Ophthalmology. 2006 Feb. 113(2):229-38. [Medline].

  5. Yang LL, Lambert SR, Lynn MJ, Stulting RD. Surgical management of glaucoma in infants and children with Peters' anomaly: long-term structural and functional outcome. Ophthalmology. 2004 Jan. 111(1):112-7. [Medline].

  6. Allingham R, Damji K, Freedman S, Moroi S, Shafranov G. Developmental glaucomas with associated anomalies. Shields' Textbook of Glaucoma. 5th ed. Philadelphia, PA 19106: Lippincott Williams & Wilkins; 2005. 252-271.

  7. Cantor LB. Glaucoma associated with congenital disorders. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 931-960.

  8. Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, Archer SM, Musch DC, Schertzer RM, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. 2003 Jul. 110(7):1361-3. [Medline].

  9. Facts and Comparisons. Drug Facts and Comparisons. St Louis; 1999.

  10. Freedman S, Walton D. Glaucoma in infants and children. Nelson L, Olitsky S, eds. Harley's Pediatric Ophthalmology. 5th ed. Philadelphia, PA 19106: Lippincott Williams & Wilkins; 2005. Chapter 14, 285-304.

  11. Hittner HM. Aniridia. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 869-884.

  12. Kirwan JF, Shah P, Khaw PT. Diode laser cyclophotocoagulation: role in the management of refractory pediatric glaucomas. Ophthalmology. 2002 Feb. 109(2):316-23. [Medline].

  13. Schottenstein EM. Peter's anomaly. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2.: 897-903.

  14. Shields MB. Axenfeld-Rieger syndrome. Ritch R, ed. The Glaucomas. St Louis, Mo: Mosby; 1989. Vol 2: 885-95.

  15. Singh OS. Nanophthalmos guidelines for diagnosis and therapy. Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 2000. Vol 4: 2846-2859.

  16. Walsh J, Muldoon T. Glaucoma associated with retinal vitreoretinal disorders. Ritch R, Shield MB, Krupin T, eds. The Glaucomas. 1996. Vol 2: 1055-1071.

  17. Weiss JS, Ritch R. Glaucoma in the phakomatoses. Ritch R, ed. The Glaucomas. St Louis: Mosby; 1989. Vol 2: 905-29.

  18. Wilson ME, Buckley EG, Kivlin JD. Pediatric Ophthalmology and Strabismus. AAO, Basic and Clinical Science Course. 1998. 6:330-345.

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Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.
Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient is aged 8 months; in Image B (right), patient is aged 8 years.
Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.
 
 
 
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