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Primary megalocornea: clinical features for differentiation from infantile glaucoma
1Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
Journal of Pediatric Ophthalmology and Strabismus
|February 21, 2004
Summary
Megalocornea in children presents with clear, enlarged corneas and normal intraocular pressures (IOPs), distinct from infantile glaucoma. Key findings include deep anterior chambers and iris abnormalities, aiding diagnosis.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Background:
- Abnormally enlarged corneas in children necessitate differentiation between conditions like megalocornea and infantile glaucoma.
- Infantile glaucoma is characterized by elevated intraocular pressure (IOP), corneal edema, and specific structural changes.
Purpose of the Study:
- To delineate the ocular findings in hereditary megalocornea.
- To aid in distinguishing megalocornea from infantile glaucoma in pediatric patients with enlarged corneas.
Main Methods:
- Retrospective review of clinical findings in four boys diagnosed with megalocornea.
- Analysis of ocular examination results, including corneal size, anterior chamber depth, iris characteristics, and IOP.
Main Results:
- Megalocornea cases exhibited clear, enlarged corneas, deep anterior chambers, posterior iris bowing, and normal IOPs.
- Iris transillumination (6/8 eyes) and pigment dispersion (4/8 eyes) were observed; pigment dispersion appeared acquired.
- Absence of Descemet's membrane breaks and sex-linked recessive inheritance patterns were noted, differentiating from infantile glaucoma's autosomal recessive inheritance.
Conclusions:
- Hereditary megalocornea has distinct clinical features, including normal IOP and specific iris/anterior segment findings.
- Corneal size asymmetry can be present in megalocornea and does not rule out the diagnosis.