Clinical characteristics and treatment of 22 eyes of morning glory syndrome associated with persistent hyperplastic primary vitreous
Clinical characteristics and treatment of 22 eyes of morning glory syndrome associated with persistent hyperplastic primary vitreous
复制标题
牵牛花综合征伴持续性原发性玻璃体增生症22眼临床特点及治疗
DOI：
10.1136/bjophthalmol-2013-303565
复制
发表时间：
2013-10-01
影响因子：
4.1
通讯作者：
Zhao, Peiquan
中科院分区：
文献类型：
--
作者：
Fei, Ping;Zhang, Qi;Zhao, Peiquan
目的回顾分析牵牛花综合征(MGS)合并持续性玻璃体增生症(PHPV)的临床表现和治疗结果。方法回顾分析本院眼科就诊的MGS患者74例(85眼)的临床资料。所有患者均接受了彻底的眼科检查。诊断为MGS合并PHPV的患者19例22眼，占全部MGS的25.88%。记录了这些患者的临床表现和治疗。结果初诊年龄<1岁者15例，占78.95%。6只眼合并小眼球。术后并发症19只眼(86.36%)，其中白内障10只眼，继发性青光眼8只眼，角膜白斑或水肿8只眼，视网膜脱离8只眼，斜视3只眼，眼震2只眼。治疗方法根据并发症的严重程度而有所不同。9眼继发性青光眼或白内障行晶状体切除术，3眼联合玻璃体切除和晶状体切除术。8例行头颅MRI/MR血管造影或CT检查。3例分别表现为双侧颞叶大脑裂增宽，左侧大脑中动脉分支异常扩张，额叶、枕叶灰质信号异常。结论我们的研究揭示了在相当大比例的MGS患者中存在PHPV，这表明潜在的共同遗传联系。与单纯MGS和PHPV相比，两种情况的联合应用在年轻患者中表现出更高的发生率和更严重的并发症。建议进行密切随访。晶状体切开术和玻璃体切割术是治疗并发症的有效方法。
Purpose To describe the clinical manifestations and treatment outcomes in a retrospective case series of morning glory syndrome (MGS) associated with persistent hyperplastic primary vitreous (PHPV). Methods The medical records of 85 eyes/74 patients referred for ophthalmology consultation diagnosed as MGS in our clinic were reviewed retrospectively. All patients underwent thorough ophthalmological examinations. 22 eyes of 19 patients diagnosed as having MGS associated with PHPV were included, accounting for 25.88% of all the MGS eyes. Clinical manifestations and management of these patients were documented. Results 15 patients (78.95%) were younger than 1 year old at the first diagnosis. Six eyes were associated with microphthalmia. 19 of 22 eyes (86.36%) had complications, including cataract (10 eyes), secondary glaucoma (8 eyes), corneal leucoma or oedema (8 eyes), retinal detachment (8 eyes), strabismus (3 eyes) and nystagmus (2 eyes). Treatment methods varied depending on the severity of the complications. Nine eyes with secondary glaucoma or cataract got lensectomy; three eyes underwent combined vitrectomy and lensectomy. Eight patients underwent cranial MRI/MR angiography or CT examination. Widened cerebral fissures of bilateral temporal lobes, abnormal dilated branch of middle cerebral artery in the left hemisphere and abnormal signal in the grey matter of frontal and occipital lobes were revealed respectively in three patients. Conclusions Our study revealed the coexistence of PHPV in a significant percentage of patients with MGS, suggesting a potential common genetic link. Compared with MGS and PHPV alone, the combination of the two conditions manifested with higher incidence and more severe complications in younger patients. Close follow-up was recommended. Lensectomy and vitrectomy were beneficial in the management of the complications.