Abstract
Introduction Congenital microphthalmos can either occur alone (simple
microphthalmos) or be associated with other ocular malformations, such as
sclerocornea or cataract (complex microphthalmos). As this is a rare condition,
there are no uniform recommendations for treatment.
Material and Methods Retrospective case series of 103 patients or a total
of 114 eyes with congenital microphthalmos, with reporting of age, sex, visual
acuity, pupil reaction, axial length, horizontal width of the palpebral fissure,
type of therapy performed and complications.
Results All patients would have been able to be fitted with a prosthesis
primarily. The size of the palpebral fissure depended on the underlying
findings: “bilateral microphthalmos” < “microphthalmos and healthy fellow
eye” < “microphthalmos and fellow anophthalmos”. In order to assess visual
(residual) function in an infant in the first weeks or months of life, the
pupillary response is of the upmost importance in deciding on therapy,
especially in unilateral disease, and as assessed with the indirect light
response of the healthy eye. In about half of the cases, conservative prosthetic
treatment was sufficient. After the successful initial fitting of a prosthesis,
the prosthesis was enlarged according to the ocularistʼs instructions. If the
eye length difference was so large that symmetry could not be achieved even with
a double-walled prosthesis, volume filling with retrobulbar implanted
self-swelling pellet expanders (osmed GmbH, Ilmenau) was offered. In almost one
third of the patients, no surgical therapy or prosthetic treatment was
performed. The reason for this was usually the presence of minimal visual
function of the microphthalmos – ranging from light perception to hand
movements.
Conclusions In the case of visual function of the microphthalmos, surgical
measures should not be indicated or only with extreme caution, since the
preservation of the existing visual acuity must be regarded as having priority
over the cosmetic findings. In cases of asymmetry or underdeveloped palpebral
fissure, therapy can be started early in the first year of life without fear of
resulting complications.
Key words
paediatric ophthalmology - plastic surgery - microphthalmos - prosthesis - expander
