eMedicine Specialties > Ophthalmology > Intraocular Pressure
Glaucoma, Secondary Congenital: Differential Diagnoses & Workup
Updated: Feb 15, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
| Aniridia | Nystagmus, Congenital |
| Dermoid, Limbal | Peters Anomaly |
| Glaucoma, Juvenile | |
| Glaucoma, Primary Congenital | |
| Megalocornea |
Other Problems to Be Considered
Differential diagnosis of aniridia
Axenfeld-Rieger
Iridoschisis
Iridocorneal endothelial syndrome
Congenital iris hypoplasiaDifferential diagnosis of Peters anomaly
Congenital glaucoma
Sclerocornea
Forceps injury
Dermoid
Congenital hereditary stromal dystrophy
Congenital hereditary endothelial dystrophy
Mucopolysaccharidosis
Workup
Laboratory Studies
- Aniridia: Chromosome analysis and genetic counseling are important parts of the workup.
- Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities.
Imaging Studies
- 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.
Other Tests
- 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
More on Glaucoma, Secondary Congenital |
| Overview: Glaucoma, Secondary Congenital |
 Differential Diagnoses & Workup: Glaucoma, Secondary Congenital |
| Treatment & Medication: Glaucoma, Secondary Congenital |
| Follow-up: Glaucoma, Secondary Congenital |
| Multimedia: Glaucoma, Secondary Congenital |
| References |
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References
Lopes JE, Wilson RR, Alvim HS, Shields CL, Shields JA, Calhoun J, et al. Central corneal thickness in pediatric glaucoma. J Pediatr Ophthalmol Strabismus. Mar-Apr 2007;44(2):112-7. [Medline].
Kirwan JF, Shah P, Khaw PT. Diode laser cyclophotocoagulation: role in the management of refractory pediatric glaucomas. Ophthalmology. Feb 2002;109(2):316-23. [Medline].
Iwach AG, Hoskins HD Jr, Hetherington J Jr, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. Jul 1990;97(7):904-9. [Medline].
Agarwal HC, Sandramouli S, Sihota R, Sood NN. Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. Jun 1993;24(6):399-402. [Medline].
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. Jan 2004;111(1):112-7. [Medline].
Cantor LB. Glaucoma associated with congenital disorders. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:931-960.
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. Jul 2003;110(7):1361-3. [Medline].
Facts and Comparisons. Drug Facts and Comparisons. St Louis; 1999.
Hittner HM. Aniridia. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:869-884.
Schottenstein EM. Peter's anomaly. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:897-903.
Shields MB. Textbook of Glaucoma. 3rd ed. Baltimore: Williams & Wilkins; 1992:235-257, 348-351.
Shields MB. Axenfeld-Rieger syndrome. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis, Mo: Mosby; 1989:885-95.
Singh OS. Nanophthalmos guidelines for diagnosis and therapy. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 4. 2000:2846-2859.
Walsh J, Muldoon T. Glaucoma associated with retinal vitreoretinal disorders. In: Ritch R, Shield MB, Krupin T. The Glaucomas. 2. 1996:1055-1071.
Weiss JS, Ritch R. Glaucoma in the phakomatoses. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:905-29.
Wilson ME, Buckley EG, Kivlin JD. Pediatric Ophthalmology and Strabismus. AAO, Basic and Clinical Science Course. 1998;6:330-345.
Further Reading
Keywords
secondary congenital glaucoma, developmental glaucoma, open angle, closed angle, vision loss, visual deficit, congenital ocular anomalies, congenital ocular abnormalities, systemic anomalies
