megalocornea


Also found in: Acronyms.

megalocornea

 [meg″ah-lo-kor´ne-ah]
a developmental anomaly of the cornea, which is of abnormal size at birth and continues to grow, sometimes reaching a diameter of 14 or 15 mm in the adult.

ker·a·to·glo·bus

(ker'ă-tō-glō'bŭs),
Congenital anomaly consisting of an enlarged anterior segment of the eye.
[kerato- + L. globus, ball]

megalocornea

An X-linked recessive, nonprogressive enlargement of the cornea to 13 mm or greater, which is present at birth; 90% of cases occur in males. The cornea and the limbus are enlarged but histologically normal. Associated findings include anterior megalophthalmos, enlarged ciliary ring with zonular stretching (leading to phacodonesis), ectopia lentis, iridodonesis, iris stromal hypoplasia, posterior embryotoxon, transillumination defects, Krukenberg spindles, trabecular meshwork pigmentation, posterior embryotoxon, Rieger anomaly, goniodysgenesis and cataracts. It is usually isolated, but may be associated with other ocular and systemic findings.

megalocornea

A non-progressive enlargement of the cornea (more than 13 mm in diameter) without significant change in corneal thickness and normal clarity and function. Intraocular pressure is normal and high myopia and astigmatism often occur. It is usually transmitted as an X-linked recessive trait. Some systemic associations include Marfan's syndrome, Apert's syndrome, Down's syndrome, Weil-Marchesani syndrome and osteogenesis imperfecta. See cornea plana; keratoglobus; microcornea.
References in periodicals archive ?
Slit lamp examination revealed megalocornea, measuring vertically 13 mm and horizontally 14 mm in both eyes.
INTRODUCTION: Pyle's disease is a rare familial metaphysical dysplastic disorder with few reports worldwide (1-4) associated with megalocornea is too rare.
On routine examination, we found she also suffers from right sided megalocornea. We done routine skiagram firstly in the traumatic knee, we found typical Erlenmeyer flask sign (fig 5) without any fracture.
Based on clinical features, radiological findings and histopathological examination we made the diagnosis of Pyle's metaphysical dysplasia with megalocornea which is a rare association.
In this case there was no spinal deformity (3,4,5) but jaw was involved with additional unilateral megalocornea which is usually bilateral (9).