Fierz Fabienne C, Landau Klara, Kottke Raimund, Wichmann Werner, Sturm Veit, Weber Konrad P, Gerth-Kahlert Christina
Department of Ophthalmology (FCF, KL, KPW, CG-K), University Hospital Zurich and University of Zurich, Zurich, Switzerland ; Department of Diagnostic Radiology (RK), University Children's Hospital Zurich and University of Zurich, Zurich, Switzerland ; Institute of Neuroradiology (WW), University Hospital Zurich and University of Zurich, Zurich, Switzerland ; Department of Ophthalmology (VS), Cantonal Hospital St. Gallen, St. Gallen, Switzerland ; and Department of Neurology (KPW), University Hospital Zurich and University of Zurich, Zurich, Switzerland .
J Neuroophthalmol. 2022 Mar 1;42(1):115-120. doi: 10.1097/WNO.0000000000001237. Epub 2021 Apr 14.
Brown syndrome is characterized by a restrictive elevation deficit of the affected eye in adduction. Besides the well-known congenital form, different acquired etiologies including inflammation, trauma, and surgery may prevent the superior oblique (SO) tendon from gliding freely through the trochlea on attempted upgaze. We present MRI findings in pediatric and adult patients with inflammatory acquired Brown syndrome.
Retrospective review of clinical and MRI findings of 6 patients (4 children: median age 8.4 years [range 6.1-8.7]; 2 adults: age 46.4 and 51.1 years). Median follow-up was 23 months (range 1-52).
In all 6 patients, orbital MRI demonstrated inflammatory changes of the SO tendon-trochlea complex. A striking feature was circumferential contrast enhancement of the trochlea with central sparing where the tendon passes, reminiscent of an eyelet. In all cases, the motility restriction improved either spontaneously or with systemic anti-inflammatory treatment. Although both adult patients had a history of known seronegative spondyloarthritis, there was no associated systemic condition in the children in our series.
Both in children and in adults, MRI can provide evidence of inflammatory changes located at the trochlea-tendon complex in acquired Brown syndrome here referred to as the "eyelet sign," which may be helpful in confirming the clinical diagnosis and guide appropriate treatment.
布朗综合征的特征是患眼内收时上转受限。除了众所周知的先天性形式外,包括炎症、创伤和手术在内的不同后天病因,在试图上视时可能会阻止上斜肌腱在滑车中自由滑动。我们展示了炎性后天性布朗综合征儿童和成人患者的MRI表现。
回顾性分析6例患者(4例儿童:中位年龄8.4岁[范围6.1 - 8.7岁];2例成人:年龄分别为46.4岁和51.1岁)的临床和MRI表现。中位随访时间为23个月(范围1 - 52个月)。
所有6例患者的眼眶MRI均显示上斜肌腱 - 滑车复合体有炎性改变。一个显著特征是滑车周围有对比增强,而肌腱通过的中央区域无强化,类似小孔。在所有病例中,运动受限情况自发改善或经全身抗炎治疗后改善。虽然2例成年患者有已知的血清阴性脊柱关节炎病史,但我们系列中的儿童患者无相关全身性疾病。
在儿童和成人中,MRI均可提供炎性后天性布朗综合征滑车 - 肌腱复合体炎症改变的证据,此处称为“小孔征”,这可能有助于确诊临床诊断并指导适当治疗。