Denis D, Cousin M, Zanin E, Toesca E, Girard N
Département d'ophtalmologie pédiatrique, service d'ophtalmologie, hôpital Nord, CHU de Marseille, chemin des Bourrely, 13015 Marseille, France.
J Fr Ophtalmol. 2011 Sep;34(7):476-81. doi: 10.1016/j.jfo.2011.03.006. Epub 2011 May 12.
Duane retraction syndrome (DRS) is a congenital ocular motility disorder with innervational dysgenesis. MRI improves our understanding of this disease by providing in vivo access to nerves and oculomotor muscles. The goal of this prospective study (2000-2008) was to analyze DRS clinically and neuroradiologically.
Twenty-four patients (27 eyes) received a complete ophthalmologic evaluation and a brain-orbital MRI. The average age was 6.1 years. MRI was performed with 3D T2 CISS-weighted images through the brainstem to visualize the cisternal segments of the cranial nerves and the orbit (lateral and medial recti muscles). MRI anomalies were classified according to type I, II, and III and depending on their condition in the posterior fossa (absence, hypoplasia) and in the orbit (muscle anomalies).
Of 27 eyes, 70% were type I, 19% type II, and 11% type III. MRI showed abducens nerve abnormalities in 93% of the cases (78% absence) and muscle abnormalities in 57.5% of the cases. A detailed description showed 100% abducens nerve abnormalities and 58% abnormal lateral rectus muscle in type I, 60% abducens nerve abnormalities and 60% abnormal lateral rectus muscle in type II, and 100% abducens nerve abnormalities and 66% abnormal lateral rectus in type III.
DISCUSSION-CONCLUSION: This study presents two major findings: detection of abducens nerve abnormalities in most cases of DRS whatever the type, associated with muscle abnormalities, and the confirmation that this absence may exist in type II (2/5). Thus MRI proved to be a valuable tool for investigating these patients, improving the comprehension of the physiopathogenics of this disease.
杜安眼球后退综合征（DRS）是一种伴有神经发育不全的先天性眼球运动障碍。磁共振成像（MRI）通过提供对神经和眼球运动肌肉的活体观察，增进了我们对这种疾病的理解。这项前瞻性研究（2000 - 2008年）的目的是从临床和神经放射学角度分析DRS。
24例患者（27只眼）接受了全面的眼科评估和脑部 - 眼眶MRI检查。平均年龄为6.1岁。通过3D T2 CISS加权图像对脑干进行MRI检查，以观察颅神经的脑池段和眼眶（外直肌和内直肌）。MRI异常根据I型、II型和III型进行分类，并依据其在后颅窝（缺如、发育不全）和眼眶（肌肉异常）的情况进行判断。
27只眼中，70%为I型，19%为II型，11%为III型。MRI显示93%的病例存在展神经异常（78%缺如），57.5%的病例存在肌肉异常。详细描述显示，I型中展神经异常率为100%，外直肌异常率为58%；II型中展神经异常率为60%，外直肌异常率为60%；III型中展神经异常率为100%，外直肌异常率为66%。
讨论 - 结论：本研究有两个主要发现：无论何种类型，大多数DRS病例均检测到展神经异常，并伴有肌肉异常；同时证实II型（2/5）可能存在展神经缺如。因此，MRI被证明是研究这些患者的有价值工具，有助于提高对该疾病病理生理学的理解。
