Department of Radiology, Mayo Clinic, 200 1st St SW, MN 55901, Rochester, MN, USA.
University of Colorado Hospital, Aurora, CO, USA.
Clin Neuroradiol. 2023 Sep;33(3):645-651. doi: 10.1007/s00062-022-01251-1. Epub 2023 Jan 2.
Congenital absence of the stapedial tendon is a rare entity with characteristic imaging findings, which can go unrecognized due the scarcity of the diagnosis and limited previous description in the imaging literature. We aim to characterize the imaging features of this entity.
A series of 9 cases with surgical confirmation of stapedial tendon absence were retrospectively reviewed and the most common abnormalities on high resolution computed tomography (CT) of the temporal bone described.
Congenital fixation of the stapes footplate was present in nearly all cases of stapedial tendon absence (n = 8, 89%), a clinically important association because the stapes footplate abnormality was not detectable on preoperative CT. Absence or hypoplasia of the pyramidal eminence and aperture was identified in almost all cases (n = 8, 89%), which may be the sole imaging finding to suggest stapedial tendon absence and associated stapes footplate fixation prior to surgery.
The most reliable indicator of stapedial muscle absence on temporal bone CT is the absence or hypoplasia of the pyramidal eminence and aperture. Importantly, most patients had congenital stapes footplate fixation confirmed intraoperatively with a normal stapes footplate on CT, meaning the pyramidal eminence/aperture abnormality was the only preoperative imaging finding that could have suggested the footplate fixation.
先天性镫骨肌腱缺失是一种罕见的疾病,具有特征性的影像学表现,但由于诊断罕见,且影像学文献中对此病的描述有限,可能会被漏诊。我们旨在描述该疾病的影像学特征。
回顾性分析了经手术证实为先天性镫骨肌腱缺失的 9 例患者的资料,并对颞骨高分辨率 CT 最常见的异常表现进行了描述。
几乎所有先天性镫骨肌腱缺失患者(8 例,89%)都存在镫骨足板固定,这是一种重要的临床相关异常,因为术前 CT 无法检测到镫骨足板异常。几乎所有患者(8 例,89%)都存在锥状隆起和孔的缺失或发育不良,这可能是唯一的影像学表现,可在术前提示存在先天性镫骨肌腱缺失和相关的镫骨足板固定。
在颞骨 CT 上,提示先天性镫骨肌缺失最可靠的指标是锥状隆起和孔的缺失或发育不良。重要的是,大多数患者术中证实存在先天性镫骨足板固定,而 CT 上的镫骨足板正常,这意味着锥状隆起/孔的异常是唯一可以提示足板固定的术前影像学表现。