Congenital ocular counter-roll: a review of cases treated exclusively by ophthalmologists

Abstract

Aim: To review the demographics, clinical manifestations, and surgical experiences of patients with congenital ocular counter-roll, whose treatments were performed exclusively by ophthalmologists.

Methods: A retrospective review was conducted consisting of patients who received strabismus surgery between 2017 to 2019. Patients with obvious ocular counter-roll were included.

Results: A total of 7008 patients who received strabismus surgery, 28 (12 males, 16 females) were diagnosed as congenital ocular counter-roll, accounting for 0.40%. All patients were initially misdiagnosed: 21 patients were misdiagnosed as superior oblique palsy (SOP), 3 as inferior oblique overaction, 2 as dissociated vertical deviation (DVD), 1 as superior oblique overaction with A-pattern exotropia, and 1 as medial rectus palsy. The mean±SD age was 12.4±9.4y (range 2.5-36y). The most common clinical findings included ocular counter-roll, vertical deviation or vertical deviation combined with outward deviation and head tilt. At follow-up, an excellent surgical result was achieved in 20 patients. Preoperative horizontal deviation of 26±24 prism diopter (PD) and vertical deviation of 18±12 PD were reduced to 0±12 PD (P=0.0001) and 3±4 PD (P=0.001), respectively.

Conclusion: Congenital ocular counter-roll is a rare supranuclear vertical strabismus caused by congenital abnormalities involving vestibule-ocular reflex pathways. In addition to ocular counter-roll, the most salient clinical features included, but are not limited to, hyperdeviation, outward deviation, overelevation in adduction and head tilt.

Keywords: congenital ocular counter-roll; cyclovertical strabismus; skew deviation.

Figure 1. Ocular cyclotorsion as measured with use of the disc-foveal method (DFA).

Figure 2. Preoperative clinical features of congenital ocular counter-roll (Case 3)

A: Fundus photography shows an incyclotorsion of the right eye and excyclotorsion of the left eye; B-E: The patient showed a left exotropia and hypertropia, head tilt to left, a scale of +2 overelevation in adduction of the left eye and a positive Bielschowsky head tilt test response.

Figure 3. Postoperative clinical features of Case 3

A: The torsion was incyclotorsion in the right eye; B-D: The patient showed a residual exotropia of 6 PD and normal ocular motility; E: The head tilt dissipated and the Bielschowsky head tilt test response was negative.

Figure 4. Anatomical structures of VOR and mechanisms of ocular tilt reaction

Lower brain stem lesions produce an inhibition of the ipsilateral graviceptive-ocular pathway (depression and excyclotorsion) and activation of the contralateral graviceptive-ocular pathway (elevation and incyclotorsion). INC: Interstitial nucleus of Cajal; III: Oculomotor nuclei; IV: Trochlear nuclei; VI: Abducens nuclei; VN: Vestibular nuclei; SO: Superior oblique; SR: Superior rectus; IO: Inferior oblique; IR: Inferior rectus.