Hope-Ross M, Johnston S S
Department of Ophthalmology, Royal Victorial Hospital, Belfast, Northern Ireland.
Ophthalmic Paediatr Genet. 1990 Jun;11(2):147-53. doi: 10.3109/13816819009012962.
A 10-month-old infant was referred to the ophthalmic department of the Royal Victoria Hospital for assessment. Pregnancy and delivery had been normal. At birth, cleft lip, cleft palate, hypertelorism and a soft tissue mass in the mouth were noted. At two days of age he developed meningitis, axial computerized tomography showed a sphenoethmoidal encephalocele, and agenesis of the corpus callosum. The basal encephalocele was surgically repaired. On examination in the ophthalmic department he was able to follow a light with the left eye. He perceived light with the right eye but did not follow. There was a manifest right divergent squint, measuring -10 degrees, and a right afferent nerve defect. Examination under anaesthetic was performed. The right fundus showed a Morning Glory syndrome. The disc was pink and deeply excavated, and surrounded by a ring of chorioretinal pigmentary disturbance. There was a central tuft of glial tissue. The left fundus was normal. The association of Morning Glory syndrome and basal encephalocele is rare; four previous cases have been reported. In all patients there were associated mid-facial congenital anomalies such as cleft lip and cleft palate. The presence of a basal encephalocele should be suspected if the Morning Glory syndrome occurs in association with mid-facial congenital anomalies.
一名10个月大的婴儿被转诊至皇家维多利亚医院眼科进行评估。妊娠和分娩过程正常。出生时，发现有唇裂、腭裂、眼距过宽以及口腔内有一软组织肿块。出生两天时，他患上脑膜炎，轴向计算机断层扫描显示有蝶筛部脑膨出和胼胝体发育不全。基底脑膨出已通过手术修复。在眼科检查时，他能用左眼追随光线。右眼能感知光线，但不能追随。有明显的右眼外斜视，度数为 -10度，且有右侧传入神经缺陷。进行了麻醉下检查。右眼眼底显示为牵牛花综合征。视盘呈粉红色且深陷，周围有一圈脉络膜视网膜色素紊乱。有一束中央神经胶质组织。左眼眼底正常。牵牛花综合征与基底脑膨出的关联罕见；此前已报道过4例。所有患者均伴有面中部先天性异常，如唇裂和腭裂。如果牵牛花综合征与面中部先天性异常同时出现，应怀疑存在基底脑膨出。
