Morrison David G, Elsas Frederick J, Descartes Maria
The Children's Hospital of Alabama, Department of Ophthalmology, University of Alabama at Birmingham, Birmingham, AL 35205, USA.
J AAPOS. 2005 Apr;9(2):166-8. doi: 10.1016/j.jaapos.2004.12.017.
Isotretinoin (RA), used for the treatment of cystic acne, is a powerful teratogen, causing craniofacial dysmorphisms and neural tube defects. We present two patients with RA embryopathy and oculomotor nerve synkinesis.
Retrospective review of patient records.
Two patients presented with third nerve synkinesis and fetal RA exposure. Both had marked elevation of the upper eyelids on adduction such that the lid fissures alternately opened and closed on gaze from side to side. Both patients showed typical dysmorphisms of RA embryopathy. The first patient had complete agenesis of the cerebellar vermix and died at 2 years. The second patient had restricted extraocular muscles in one eye and was exotropic and hypotropic.
Both patients demonstrated simultaneous innervation of the medial rectus and levator palpebrae muscles causing coincident lid elevation in adduction. This evidence of oculomotor nerve synkinesis is consistent with animal studies showing abnormalities in the formation of cranial nerve ganglia following fetal RA exposure.
RA is a powerful teratogen. These patients provide additional clinical evidence of its influence on neural migration during early development.
异维甲酸（RA）用于治疗囊肿性痤疮，是一种强效致畸剂，可导致颅面部畸形和神经管缺陷。我们报告两例患有RA胚胎病和动眼神经联带运动的患者。
对患者记录进行回顾性研究。
两名患者出现动眼神经联带运动且胎儿期暴露于RA。两人在内收时上睑均明显上抬，以至于在左右注视时睑裂交替开闭。两名患者均表现出典型的RA胚胎病畸形。首例患者小脑蚓部完全缺如，2岁时死亡。第二例患者一只眼睛的眼外肌受限，有外斜视和下斜视。
两名患者均表现出内直肌和提上睑肌同时受神经支配，导致内收时上睑同时上抬。这种动眼神经联带运动的证据与动物研究一致，后者显示胎儿期暴露于RA后颅神经节形成异常。
RA是一种强效致畸剂。这些患者为其在早期发育过程中对神经迁移的影响提供了额外的临床证据。
