Abteilung Augenheilkunde, Bereich Strabologie, Neuroophthalmologie und okuloplastische Chirurgie, Universitätsmedizin Göttingen, Deutschland.
Klinik und Poliklinik für Augenheilkunde, Universität Rostock, Deutschland.
Klin Monbl Augenheilkd. 2022 Jan;239(1):64-72. doi: 10.1055/a-1685-5002. Epub 2022 Feb 4.
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.
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.
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 ocularist's 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.
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.
先天性小眼球可单独发生（单纯性小眼球）或与其他眼部畸形相关，如巩膜硬化或白内障（复杂性小眼球）。由于这是一种罕见的情况，因此没有统一的治疗建议。
回顾性病例系列研究了 103 名患者或总共 114 只患有先天性小眼球的患者，报告了年龄、性别、视力、瞳孔反应、眼轴长度、睑裂水平宽度、所进行的治疗类型和并发症。
所有患者最初都可以佩戴义眼。睑裂的大小取决于潜在的发现：“双侧小眼球”<“小眼球和健康对侧眼”<“小眼球和对侧无眼球”。为了评估婴儿在生命的最初几周或几个月内的视觉（残留）功能，瞳孔反应对于决定治疗至关重要，尤其是在单侧疾病中，可以通过健康眼的间接光反应来评估。在大约一半的情况下，保守的义眼治疗就足够了。成功地初次佩戴义眼后，根据义眼师的指示扩大义眼。如果眼轴长度差异如此之大，即使使用双层义眼也无法实现对称，则提供眶内植入自膨胀颗粒扩张器（osmed GmbH，Ilmenau）进行容积填充。几乎三分之一的患者未进行手术治疗或义眼治疗。这样做的原因通常是小眼球的视力功能非常有限——从光感到手动。
在小眼球存在视力功能的情况下，不应或仅应谨慎地进行手术治疗，因为必须优先考虑保留现有的视力，而不是美容效果。在不对称或睑裂未发育的情况下，可以在生命的第一年及早开始治疗，而不必担心由此产生的并发症。
