[Hereditary anterior megalophthalmus--a genealogical study of 12 patients in 4 generations].
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.


METHODS
Twelve individuals making up an age span 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, along with the length of the axis of the eye and the depth of the anterior chamber partly measured biometrically. In addition, a detailed slitlamp examination of the anterior section was carried out, together with a gonioscopy and a funduscopy. On four of these who bore the symptoms, a 30-2 cental visual field test was carried out using the Humphrey apparatus and evaluated.


RESULTS
The megalophthalmus anterior which used to be known by the name megalocornea globosa Kaiser-Grönholm, involves not only a megalocornea with a vertical diameter of the cornea of between 12.5-14.5 mm, but also an 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, a global front of the coenea with partly thining in the central region, crocodile chagrin, no sign of glaucoma (normal intaocular 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 analysing the results seems to lie in the lack of glaucoma, in the findings in the anterior section and in the conditions hereditary tendency.
Pletz
C
C
Augenklinik im Carl-Thiem-Klinikum Cottbus, Akademisches Lehrkrankenhaus der Charité Berlin, Thiemstrasse 111, 03048 Cottbus.
Hentsch
R
R
D000293:Adolescent; D000328:Adult; D002387:Cataract Extraction; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000002:abnormalities* / Q000473:pathology; D005124:Eye Abnormalities / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012729:Sex Chromosome Aberrations / Q000235:genetics; D014960:X Chromosome