Hoehn Mary Ellen, Calderwood Julie, O'Donnell Thomas, Armstrong Gregory T, Gajjar Amar
Hamilton Eye Institute, Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, Tennessee; Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee.
The Eye Center Surgeons and Associates, Huntsville, Alabama.
J AAPOS. 2017 Feb;21(1):34-38. doi: 10.1016/j.jaapos.2016.09.024. Epub 2017 Jan 7.
Dorsal midbrain syndrome (also known as Parinaud syndrome and pretectal syndrome) is a well-known complication of tumors of the pineal region. However, there are few reports regarding outcomes, especially in children. The purpose of this study was to report the ophthalmic outcomes in a group of children with pineal tumors treated at a single institution.
The medical records of pediatric patients diagnosed with pineal region tumors and evaluated at our ophthalmology clinic were studied retrospectively. Descriptive statistics were used to assess rate of dorsal midbrain syndrome, defined as one or more of the following: limitation of upgaze, pupillary light-near dissociation, and convergence retraction nystagmus. Treatment outcomes were recorded.
A total of 35 subjects (age range, 5 months to 20 years) were included, 18 (51%) of whom were found to have dorsal midbrain syndrome. Of those 18, 16 patients (89%) had limitation of upgaze, 15 (83%) had pupillary light-near dissociation, and 9 (50%) had convergence-retraction nystagmus. Convergence insufficiency was noted in 5 patients (28%); exotropia (either intermittent or constant), in 9 (50%). Improvement in dorsal midbrain syndrome findings following treatment was seen in 7 of 17 patients (41%), but only 2 (12%) experienced complete resolution. Treatment consisted of surgery, radiation, and/or chemotherapy.
In our study cohort of children with pineal tumors have a high incidence of dorsal midbrain syndrome. Most cases had residual findings after treatment.
中脑背侧综合征（也称为帕里诺德综合征和顶盖前区综合征）是松果体区肿瘤的一种常见并发症。然而，关于其预后的报道较少，尤其是在儿童中。本研究的目的是报告在单一机构接受治疗的一组松果体肿瘤患儿的眼科预后情况。
回顾性研究在我们眼科诊所诊断为松果体区肿瘤并接受评估的儿科患者的病历。采用描述性统计方法评估中脑背侧综合征的发生率，其定义为以下一种或多种情况：上视受限、瞳孔对光 - 近反射分离以及集合退缩性眼球震颤。记录治疗结果。
共纳入35名受试者（年龄范围为5个月至20岁），其中18名（51%）被发现患有中脑背侧综合征。在这18名患者中，16例（89%）存在上视受限，15例（83%）有瞳孔对光 - 近反射分离，9例（50%）有集合退缩性眼球震颤。5例患者（28%）存在集合不足；9例（50%）有外斜视（间歇性或持续性）。17例患者中有7例（41%）在治疗后中脑背侧综合征的表现有所改善，但只有2例（12%）完全缓解。治疗包括手术、放疗和/或化疗。
在我们的研究队列中，松果体肿瘤患儿中脑背侧综合征的发生率较高。大多数病例在治疗后仍有残留表现。
