Vertical deviation and A pattern of type Ⅲ Duane retraction syndrome

• Published in: Chinese medical journal Vol. 125; no. 16; pp. 2929 - 2932
• Main Authors: She, Hai-Cheng, Man, Feng-Yuan, Wang, Zhen-Chang, Wang, Jing-Hui, Jiao, Yong-Hong, Zhao, Kan-Xing
• Format: Journal Article
• Language: English
• Published: China Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University 01.08.2012; Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing 100730, China%Beijing Tongren Imaging Centre, Beijing Tongren Hospital,Capital Medical University, Beijing 100730, China%Tianjin Eye Hospital, Clinical College of Ophthalmology, Tianjin 300020, China
• Subjects: Adolescent; Child; DRS; Duane Retraction Syndrome - diagnosis; Electromyography; Female; Humans; Magnetic Resonance Imaging; Male; Young Adult; 动眼神经; 垂直偏差; 类型; 经济衰退; 结构异常; 综合征; 肌电图; vertical deviation; magnetic resonance imaging; type Ⅲ Duane retraction syndrome
• ISSN: 0366-6999; 2542-5641; 2542-5641
• DOI: 10.3760/cma.j.issn.0366-6999.2012.16.024

Background Researches in ocular electromyography （EMG） and Magnetic resonance imaging （MRI） of patients with Duane retraction syndrome （DRS） suggest that there may be additional abnormalities such as paradoxical innervation between horizontal rectus muscles and vertical rectus muscles, hypoplasia of vertical rectus muscle and that oblique muscles may also contribute to the heterogeneity of the clinical manifestation of DRS. This paper reports the results of superior rectus recession for vertical deviation and A pattern in DRS Type III and discusses the pathogenesis of the disease. Methods Superior and lateral rectus recession were performed in 5 cases of Huber type III DRS to treat vertical deviation and A pattern strabismus. Before operation, MRI of the brain, brainstem, cavernous sinus, and orbits were performed. Results All subjects had unilateral limitation of both abduction and adduction, with palpebral fissure narrowing and globe retraction in adduction. Three cases had A pattern of strabismus, three cases had hypertropia. The abducens nerves （CN6） were either absent or hypoplasitic in the brainstem in all patients. Two eyes had larger oculomotor foramen. Two eyes had hypoplasia of the superior rectus and the inferior rectus. There was presumably a branch of the third cranial nerve （CN3） innervating the lateral rectus （LR） in one eye. While in another eye, two branches of CN3 sent into medial rectus were revealed. After surgery, vertical deviation in the primary position was reduced in all patients and A pattern was eliminated in 3 patients. One patient developed 10A consecutive esotropia postoperatively. Conclusion The results suggest that structural abnormalities of vertical muscle and abnormal orbital innervation may be related to vertical deviation and the presence of A pattern in DRS type Ⅲ. Recession of the superior rectus muscle seems to be a safe and effective treatment for vertical deviation and A pattern strabismus in DRS Type Ⅲ.