Loudot C, Fogliarini C, Baeteman C, Mancini J, Girard N, Denis D
Service d'ophtalmologie, Hôpital Nord, Marseille.
J Fr Ophtalmol. 2007 Dec;30(10):998-1001. doi: 10.1016/s0181-5512(07)79276-8.
Morning Glory syndrome, characterized by an enlarged dysplasic optic disc with glial tissue, is one of the congenital anomalies of the optic nerve. This syndrome is rare, prevalent in the girls, and generally unilateral. It can be revealed with nystagmus, strabismus, or amblyopia.
We report the clinical observation of a 2.5-year-old girl, referred for the diagnosis of Morning Glory syndrome in the left eye with severe amblyopia (1/10 Rossano 1/20) and esotropia. This syndrome has associated central nervous system anomalies with a basal encephalocele. Treated with functional amblyopia therapy, visual acuity was 7/10 Rossano 1/2 after 1 year.
Rehabilitation on functional amblyopia in organic optic nerve anomalies is essential. A child with a Morning Glory syndrome, detected during the period of sensory maturation, must be treated with occlusion therapy, followed by maintenance treatment. This part of the treatment can prevent deep amblyopia. Moreover, regular ophthalmologic follow-up to detect complications of retinal detachment and multidisciplinary follow-up to detect a cytogenetic disease, CHARGE syndrome, or association with endocrine and central nervous system anomalies are necessary.
The author recommends occlusion therapy for children with Morning Glory syndrome or other organic asymmetric optic nerve anomalies, during the period of amblyopia reversibility. Most patients' vision improves after treatment. This case is an illustration.
牵牛花综合征的特征是视神经盘发育异常且增大,伴有神经胶质组织,是视神经的先天性异常之一。该综合征较为罕见,多见于女孩,通常为单侧。可表现为眼球震颤、斜视或弱视。
我们报告了一名2.5岁女孩的临床病例,因左眼牵牛花综合征伴严重弱视(罗萨诺视力表1/10,国际标准视力表1/20)和内斜视前来就诊。该综合征伴有中枢神经系统异常及基底脑膨出。经功能性弱视治疗后,1年后视力提高到罗萨诺视力表7/10,国际标准视力表1/2。
对于器质性视神经异常导致的功能性弱视进行康复治疗至关重要。在感觉发育成熟阶段发现患有牵牛花综合征的儿童,必须进行遮盖治疗,随后进行维持治疗。这部分治疗可预防严重弱视。此外,定期眼科随访以检测视网膜脱离并发症,以及多学科随访以检测细胞遗传学疾病、CHARGE综合征或与内分泌及中枢神经系统异常的关联是必要的。
作者建议在弱视可逆期,对患有牵牛花综合征或其他器质性不对称视神经异常的儿童进行遮盖治疗。大多数患者治疗后视力会有所改善。本病例就是一个例证。