Siepmann K, Herzau V
Abteilung für Erkrankungen des vorderen und hinteren Augenabschnitts, Universitäts-Augenklinik Tübingen.
Klin Monbl Augenheilkd. 2005 May;222(5):413-8. doi: 10.1055/s-2005-858094.
Recent reports postulate that the concomitant vertical deviation found in congenital superior oblique palsy is due to mechanical abnormalities rather than a congenitally paretic muscle, and is overcome in most patients by fusion. On the basis of the clinical characteristics alone a primary paresis is indeed unlikely. Although intraoperatively a different elasticity of the superior oblique tendon exists in congenital versus acquired cases of superior oblique palsy, preoperatively performed MR imaging shows that the clinical findings in congenital superior oblique muscle malfunction could nevertheless be of paretic origin.
Seventeen consecutive patients (males: n = 13; females: n = 4) were examined. The vertical deviation in adduction was concomitant in vertical versions, the excyclotropia was small and concomitant in all directions of gaze and was less than 10 degrees even after diagnostic occlusion. All patients showed a positive Bielschowsky head tilt phenomenon and large fusional ability. We performed preoperative MR imaging of both orbits in high resolution 3 mm sections in coronal and axial orientations with and without contrast enhancement.
In sixteen patients we found a significant reduction in muscle volume or even total aplasia of the superior oblique muscle of the affected side in comparison to the sound muscle on the other side. In contrast, two patients had a full blown clinical picture of a congenital superior oblique palsy but showed symmetrical muscle volumes on both sides in all coronal sections.
Hypoplasia or aplasia of the superior oblique muscle on magnetic resonance imaging provides evidence for a primary paretic cause for the vertical squint found with congenital superior oblique dysfunction. It is not clear, however, whether this is caused by a primary hypoplasia or is of neurogenic origin. Our data together with the consistent difference in tendon morphology of the congenital and acquired forms of superior oblique palsy seem to exclude a purely neurogenic cause for the affection.
最近的报告推测,先天性上斜肌麻痹中伴随的垂直偏斜是由于机械异常而非先天性麻痹性肌肉所致,并且在大多数患者中可通过融合克服。仅根据临床特征,原发性麻痹确实不太可能。尽管在手术中先天性与后天性上斜肌麻痹病例中,上斜肌腱的弹性不同,但术前进行的磁共振成像显示,先天性上斜肌功能障碍的临床发现仍可能源于麻痹。
连续检查了17例患者(男性13例,女性4例)。内收时的垂直偏斜在垂直运动中是伴随性的,外旋转斜视较小,在所有注视方向均为伴随性,即使在诊断性遮盖后也小于10度。所有患者均表现出阳性的Bielschowsky头位倾斜现象和较大的融合能力。我们对双侧眼眶进行了术前磁共振成像,采用3毫米高分辨率冠状位和轴位切片,有或无对比增强。
与另一侧正常肌肉相比,16例患者患侧上斜肌的肌肉体积明显减小,甚至完全缺如。相比之下,2例患者有典型的先天性上斜肌麻痹临床表现,但在所有冠状切片中双侧肌肉体积对称。
磁共振成像显示上斜肌发育不全或缺如为先天性上斜肌功能障碍所致垂直斜视的原发性麻痹原因提供了证据。然而,尚不清楚这是由原发性发育不全还是神经源性原因引起的。我们的数据以及先天性和后天性上斜肌麻痹在肌腱形态上的持续差异似乎排除了该病纯粹由神经源性原因引起的可能性。