Department of Pediatric Ophthalmology, Strabismus and Neuro-ophthalmology, Dr Shroff's Charity Eye Hospital, New Delhi, India.
Department of Ophthalmology, Massachusetts Eye and Ear, Boston, MA, USA.
Strabismus. 2024 Sep;32(3):195-201. doi: 10.1080/09273972.2024.2344538. Epub 2024 Jul 29.
Congenital enophthalmos is a rare condition characterized by posterior displacement of the globe, often associated with bony orbital anomalies or whole globe development defects. The purpose of this report is to present two unrelated cases of congenital enophthalmos secondary to anomalous accessory orbital bands and to describe characteristics of orbital imaging that differentiate this condition from the other causes.
The case records of two patients who presented with congenital enophthalmos and were discovered to have anomalous accessory orbital extraocular muscle bands were reviewed. The clinical features, initial diagnosis, high resolution magnetic resonance imaging (MRI) findings, and surgical outcomes were noted. A 3-dimensional reconstruction model was used to understand the approach and surgical management in one of the cases.
Both patients presented with unilateral severe enophthalmos, globe retraction, and restricted ocular motility in all directions since birth. High-resolution MRI of the orbits revealed a short anomalous band, isointense to the muscle, arising from a rectus muscle belly and attaching to the posteroinferior part of the globe adjacent to the optic nerve. The caliber of the extraocular muscles and ocular motor nerves was normal. In one patient, surgery was not pursued due to the extreme posterior location of the band with proximity to the optic nerve. In the other patient, the deviation did not improve, despite successfully severing the accessory band, due to extensive scarring.
Anomalous accessory orbital extraocular muscle bands are a rare and often overlooked cause of congenital enophthalmos when associated with limited ocular motility. Imaging the orbit can aid in diagnosis and help differentiate it from other causes. Safe surgical approaches to address the problem are limited, and available approaches may not be effective. These two cases highlight that the management of accessory extraocular muscle bands causing enophthalmos can be extremely challenging and difficult to improve even with intensive surgical intervention.
先天性眼球内陷是一种罕见的疾病，其特征为眼球后移位，常伴有骨眶畸形或整个眼球发育缺陷。本报告旨在介绍两例因异常副眶眼外肌带引起的先天性眼球内陷病例，并描述区分这种情况与其他病因的眼眶影像学特征。
回顾了两名表现为先天性眼球内陷并发现异常副眶眼外肌带的患者的病例记录。记录了临床特征、初始诊断、高分辨率磁共振成像（MRI）结果和手术结果。使用三维重建模型来理解其中一个病例的手术方法和处理。
两名患者均表现为单侧严重眼球内陷、眼球退缩和自出生以来各向运动受限。眼眶高分辨率 MRI 显示一条短的异常带，与肌肉等信号，从直肌腹发出，附着在视神经附近的眼球后下部分。眼外肌和眼运动神经的口径正常。在一名患者中，由于带的极度后向位置靠近视神经，手术未进行。在另一名患者中，尽管成功切断了副带，但由于广泛的疤痕形成，斜视没有改善。
当伴有有限的眼球运动时，异常副眶眼外肌带是先天性眼球内陷的一种罕见且常被忽视的原因。眼眶成像有助于诊断，并有助于与其他病因区分。解决问题的安全手术方法有限，现有的方法可能无效。这两个病例表明，即使进行密集的手术干预，异常副眼外肌带引起的眼球内陷的处理也极具挑战性且难以改善。
