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Abstract #15836 Published in IGR 2-3
Megalophthalmus anterior: a genealogical essay on 12 patients from four generations
Pletz C; Hentsch RKlinische Monatsblätter für Augenheilkunde 2000; 217: 284-288
BACKGROUND: The debate about whether megalophthalmus anterior is a diagnosis that needs to be distinguished clearly from buphthalmus, or whether there are indeed connections between the two disorders, is just as topical today as it was when the disorder was first described. With the aid of a genealogical examination, the symptoms of megalophthalmus anterior and the way in which they differ to those of buphthalmus will be shown. PATIENTS AND METHODS: Twelve individuals making up an age span of over four generations formed the genealogical tree. In seven of the family members, the subjective refraction, the javal, the applanation values and the diameter of the cornea, both vertically and horizontally, were established, together with the length of the axis of the eye and the depth of the anterior chamber measured partly biometrically. In addition, a detailed slit-lamp examination of the anterior section was carried out, together with a gonioscopy and a funduscopy. A 30-2 cental visual field test was carried out on four of the subjects who bore the symptoms, using the Humphrey apparatus and evaluated. RESULTS: The megalophthalmus anterior which used to be known by the name megalocornea globosa Kaiser-Gronholm, not only involves a megalocornea with a vertical corneal diameter of between 12.5 and 14.5 mm, but also enlargement of the whole anterior section of the eye with iridodonesis and lentodonesis. In addition, through the existing series of examinations, the following characteristics of megalophthalmus were found: symmetrical differences between each side, global front to the cornea with partly thinning in the central region, crocodile chagrin, no sign of glaucoma (normal intraocular pressure, a practically normal angle of the iris, no excavatio papillae, no loss in the field of vision) and recessive x-chromosomal heredity. CONCLUSIONS: In order to eliminate buphthalmus and to diagnose megalophthalmus anterior, the difficulty in correctly analyzing the results seems to lie in the lack of glaucoma, in the findings in the anterior section, and in the condition's hereditary tendency.LA: German
Dr. C. Pletz, Augenkliniek, Carl-Thiem-Kliniek, Cottbus, Akademische Lehrkrankenhaus Charite Berlin, Thiemstrasse 111, 03048 Cottbus, Germany
Classification:
10 Differential diagnosis e.g. anterior and posterior ischemic optic neuropathy
