Type II Duane’s retraction syndrome with severe upshoot with ipsilateral superior oblique muscle palsy: a rare presentation

Type II Duane’s retraction syndrome (DRS) is the least common strabismus characterized by limitation of adduction with the presence of upshoot, downshoot or both. Abduction can be normal or slightly altered. Secondary muscle changes like fibrosis, and anomalous insertions have been associated with DRS. Its associations with dissociated vertical deviations have been reported. We report a rare case of DRS with superior oblique palsy presenting post trauma in a tertiary care centre. Introduction Duane’s retraction syndrome (DRS) is a unusual form of strabismus characterized by limitation of horizontal movements and globe retraction with palpebral fissure narrowing on attempted adduction of the affected eye. In general, 1–4% of strabismic patients have Duane’s syndrome. 3 Type II DRS is least common and presents as limitation of adduction with exotropia of the affected eye. Abduction can be normal or slightly affected. A characteristic upshoot, downshoot or both may occur in adduction. Its association with dissociated vertical deviation has been reported previously. Superior rectus overaction/contracture syndrome (SRSy) was described by Jampolsky in 1964. Superior rectus contracture has been previously reported in a patient with unilateral superior oblique palsy (SO). Here, we report a rare case of type II DRS with ipsilateral SO. Case Report A 50-year-old male presented with complaints of blurring of vision and double vision in downgaze following head trauma 6 days back, which was associated with black eye. There were no preceding systemic illnesses, and patient’s birth history, family history and medical history were not significant. There was no history of surgical intervention in the past. Previous CT scan showed thickening of right superior oblique muscle. On examination, best corrected visual acuity was 6/6 with no significant refractive error. The patient had small left head tilt with left face turn. Ocular motility showed global limitation in adduction with upshoot on adduction with palpebral fissure narrowing in the right eye. The alternate prism cover test showed 20PD right hypertropia (RHT) with 6PD exotropia in primary gaze and 12 PD RHT with 6PD exotropia in downgaze. Deviation of near showed 14RHT with 6PD exotropia. Rest movements in both eyes were full. The patient had diplopia in a distance Worth 4-dot test. Double Maddox rod showed 10° exotorsion in primary gaze and 15° in downgaze (Figs 1 and 2). Diplopia and torsion were noted in diplopia charting and superior oblique underaction on Hess charting. Fundus examination showed mild extorsion. Review of the old photographs prior to trauma did not reveal any face turn. There was a diagnostic dilemma regarding the co-existence of SO with DRS. Although right hypertropia pointed towards the severe upshoot in adduction, the appearance of left face turn after trauma, and Figure 1: Left face turn. Figure 2: Extraocular movements in all gazes showing global limitation in adduction with upshoot on adduction with palpebral fissure narrowing in the right eye. Correspondence: