Groot Annabel L W, Kuijten Maayke M P, Remmers Jelmer, Gilani Asra, Mourits Daphne L, Kraal-Biezen Elke, de Graaf Pim, Zwijnenburg Petra J, Moll Annette C, Tan Stevie, Saeed Peerooz, Hartong Dyonne T
Department of Ophthalmology, Amsterdam Orbital Center, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.
Department of Ophthalmology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands.
Acta Ophthalmol. 2020 Aug;98(5):514-520. doi: 10.1111/aos.14364. Epub 2020 Feb 25.
Current clinical classifications do not distinguish between the severity of the MICrophthalmia/Anophthalmia (MICA) spectrum with regard to treatment urgency. We aim to provide parameters for distinguishing mild, moderate and severe MICA using clinical and biometrical characteristics.
We performed a single-centre, cross-sectional analysis of prospective cohort of 58 MICA children from September 2013 to February 2018 seen at the Amsterdam University Medical Center, The Netherlands. All patients with a visible underdeveloped globe were included. We performed full ophthalmic evaluation including horizontal palpebral fissure length, axial length by ultrasound and/or MRI measurements, paediatric and genetic evaluation. Cases were subdivided based on clinical characteristics. Biometrical data were used to calculate the relative axial length (rAL) and the relative horizontal palpebral fissure length (rHPF) compared with the healthy contralateral eye for unilateral cases.
In previously untreated patients, a strong correlation exists between rAL and rHPF, distinguishing between severe, moderate and mild subjects using rAL of 0-45%, 45-75% and 75%-100%, respectively. Clinical subgroups were randomly dispersed throughout the scatterplot.
Current classifications lack clinical implications for MICA patients. We suggest measuring eyelid length and axial length to classify the severity and determine treatment strategy. The 'severe' group has obvious asymmetry and abnormal socket configuration for which therapy should quickly be initiated; the 'moderately' affected group has normal socket anatomy with a microphthalmic eye with disturbing asymmetry for which treatment should be initiated within months of development; the 'mild' group has a slightly smaller axial length or less obvious eyelid asymmetry for which reconstructive correction is possible, but expansive conformer treatment is unnecessary.
目前的临床分类在治疗紧迫性方面未区分小眼症/无眼症（MICA）谱系的严重程度。我们旨在提供利用临床和生物测量特征区分轻度、中度和重度MICA的参数。
我们对2013年9月至2018年2月在荷兰阿姆斯特丹大学医学中心就诊的58例MICA儿童的前瞻性队列进行了单中心横断面分析。纳入所有可见眼球发育不全的患者。我们进行了全面的眼科评估，包括水平睑裂长度、超声和/或MRI测量的眼轴长度、儿科和基因评估。根据临床特征对病例进行细分。对于单侧病例，生物测量数据用于计算与健康对侧眼相比的相对眼轴长度（rAL）和相对水平睑裂长度（rHPF）。
在既往未治疗的患者中，rAL和rHPF之间存在强相关性，分别使用0 - 45%、45 - 75%和75% - 100%的rAL区分重度、中度和轻度受试者。临床亚组随机分布在散点图中。
目前的分类对MICA患者缺乏临床指导意义。我们建议测量眼睑长度和眼轴长度以对严重程度进行分类并确定治疗策略。“重度”组有明显的不对称和异常的眼窝结构，应迅速开始治疗；“中度”受影响组眼窝解剖结构正常，有小眼症且不对称令人困扰，应在病情发展数月内开始治疗；“轻度”组眼轴长度略小或眼睑不对称不太明显，可进行重建矫正，但无需进行扩张性结膜囊扩张器治疗。
