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Type II Duaneis Retraction Syndrome with Severe Case Report Type II Duane’s retraction syndrome with severe upshoot with ipsilateral superior oblique muscle palsy: a rare presentation Dr. Nimisha Sharma, Dr. Manideepa Banerjee, and Dr. S Meenakshi Correspondence: Abstract prism cover test showed 20PD right hypertropia Dr. Nimisha Sharma, Type II Duane’s retraction syndrome (DRS) is the (RHT) with 6PD exotropia in primary gaze and 12 Medical Research Foundation, least common strabismus characterized by limita- PD RHT with 6PD exotropia in downgaze. Sankara Nethralaya, 18 College Road, tion of adduction with the presence of upshoot, Deviation of near showed 14RHT with 6PD exo- Nungambakkam, Chennai, India. downshoot or both. Abduction can be normal or tropia. Rest movements in both eyes were full. The Email: id:nimisha0310@gmail. slightly altered. Secondary muscle changes like patient had diplopia in a distance Worth 4-dot com fibrosis, and anomalous insertions have been test. Double Maddox rod showed 10° exotorsion in associated with DRS. Its associations with disso- primary gaze and 15° in downgaze (Figs 1 and 2). ciated vertical deviations have been reported. We Diplopia and torsion were noted in diplopia report a rare case of DRS with superior oblique charting and superior oblique underaction on Hess palsy presenting post trauma in a tertiary care charting. Fundus examination showed mild extor- centre. sion. Review of the old photographs prior to trauma did not reveal any face turn. There was a diagnostic dilemma regarding the co-existence of Introduction SO with DRS. Although right hypertropia pointed ’ Duane s retraction syndrome (DRS) is a unusual towards the severe upshoot in adduction, the form of strabismus characterized by limitation of appearance of left face turn after trauma, and horizontal movements and globe retraction with palpebral fissure narrowing on attempted adduc- tion of the affected eye.1 In general, 1–4% of stra- bismic patients have Duane’s syndrome.2, 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 devi- ation has been reported previously.4 Superior rectus overaction/contracture syndrome (SRSy) was described by Jampolsky in 1964. Superior rectus contracture has been previously reported in Figure 1: Left face turn. a patient with unilateral superior oblique palsy (SO).5 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 sig- nificant. There was no history of surgical interven- tion 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. Figure 2: Extraocular movements in all gazes Ocular motility showed global limitation in adduc- showing global limitation in adduction with tion with upshoot on adduction with palpebral upshoot on adduction with palpebral fissure fissure narrowing in the right eye. The alternate narrowing in the right eye. 64 Sci J Med & Vis Res Foun October 2016 | volume XXXIV | number 3 | Case Report large vertical and torsional diplopia in the field of under close follow-up and the next plan of action action of superior oblique muscle and underaction is to take care of the exotropia and the residual of the same in Hess charting, suggested the pres- upshoot by lateral rectus recession with or without ence of right SO. The presence of exotropia, a Y-split (Figs 3 and 4). adduction limitation with upshoot and palpebral fissure changes, on the other hand, confirmed Discussion diagnosis of Type II DRS. Subsequently, surgery Heuck described patients with severe limitation of was planned to correct his face turn and vertical ocular motility and retraction of the globe in nine- squint. On table, inferior oblique muscle was teenth century.6 found to have an abnormal insertion. Forced trac- Type II DRS is the least common type (<10%) tion test showed laxity in the right superior characterized by a marked limitation of adduction oblique muscle. A superior oblique tuck of 6 mm with exotropia of the affected eye, abduction with inferior oblique recession in the right eye was normal or slightly limited, retraction of the globe performed. A repeat traction test at the conclusion and narrowing of the fissure on attempted adduc- showed no evidence of iatrogenic Brown’s. On the tion. On electromyography, the lateral rectus first postoperative day, his face turn and small showed peak impulses on abduction and a second head tilt improved significantly with collapse of paradoxical peak on attempted adduction. There the vertical squint to a large extent. He was kept was normal behaviour of the medial rectus. Figure 3: Diplopia chart showing increased separation of the vertical images in the field of action of superior oblique muscle and the presence of torsional diplopia more in downgaze. Figure 4: HESS chart showing severe underaction of superior oblique muscle and mild overaction of inferior oblique muscle in the right eye and overaction of superior oblique and inferior rectus along with underaction of superior rectus in the left eye. Sci J Med & Vis Res Foun October 2016 | volume XXXIV | number 3 | 65 Case Report The pathogenesis of DRS has a wide spectrum, unravelled. Identification of each of these compo- which can be classified into mechanical, anatom- nents requires a meticulous history taking, review ical and innervational disorder. Anatomical of old photographs and thorough clinical examin- changes most commonly seen are fibrotic changes ation and supportive investigations. The plan of and anamolous insertions.7 Mechanical distur- surgery in such cases is a staged procedure: first, bances can occur due to the presence of facial correcting the SO by superior oblique tucking with bands found in some cases of DRS. These band inferior oblique recession and, second, DRS causes the limitation of eye movement. surgery, as recession of lateral rectus muscle on An upshoot may occur due to co-innervation the involved side in proportion to the size of exo- of superior rectus muscle with the lateral rectus or tropia9 with or without Y split for upshoot. could be because of the mechanical factors as the bridle or leash effect due to tight lateral rectus.3 The innervational type of upshoot is character- ized by the presence of hypertropia in the primary References position.8 Another characteristic feature is that, in 1. DeRespinis PA, Caputo AR, Wanger RS, Guo S. Duane’s retraction syndrome. Surv Ophthalmol 1993;38:257–88. innervational type, there is a gradually increasing 2. Ahluwalia BK, Gupta NC, Goel SR, Khurana AK. Study of upshoot of the eye as it moves in adduction. Our Duane’s retraction syndrome. Acta Ophthalmol (Copenh) 1988;66 case had the obvious features of exotropic DRS (6):728–30. with a limitation of adduction, upshoot and palpe- 3. Kirkham TH. Inheritance of Duane’s syndrome. Br J Ophthalmol bral fissure narrowing in adduction. 1970;54(5):323–9. In our case, the exact pathogenesis of SO is not 4. Khawam E, Ghazi N, Salti H. “Jampolsky Syndrome”. Superior known. The laxity of the superior oblique tendon rectus overaction-contracture syndrome: prevalence, characterstics, etiology and management. Binocul Vis Strabismus on forced traction testing hint towards a possibil- Q 2000;15:331–42. fi ity of congenital SO. But the absence of signi - 5. Scott AB, Wong GY. Duane’s syndrome. An electromyographic cant head tilt even prior to trauma and sudden study. Arch Ophthalmol 1972;87:140–7. onset vertical and torsional diplopia in the field of 6. Hueck G. Über Angeborenenvererbten Beweglichkeitsdefect der action of superior oblique muscle following Augen. Klin Monatsbl Augenheilkd 1879;17:253. trauma points more towards an acquired SO with 7. Gobin MH. Surgical management of Duane’s syndrome. Br J 58 – DRS. Ophthalmol 1974; :301 6. 8. Kraft SP. Surgical approach to Duane syndrome. J Pediatr Ophthalmol Strabismus 1988;25:119–30. Conclusion 9. Rosenbaum AL. Costenbader lecture. The efficacy of rectus DRS and SO have been shown to co-exist in our muscle transposition surgery in Duane syndrome and VI nerve case although the exact pathogenesis still remains palsy. J AAPOS 2004;8(5):409–19. How to cite this article Sharma N., Banerjee M, and Meenakshi S. Type II Duane’s retraction syndrome with severe upshoot with ipsilateral superior oblique muscle palsy: a rare presentation, Sci J Med & Vis Res Foun 2016;XXXIV: 64–66. 66 Sci J Med & Vis Res Foun October 2016 | volume XXXIV | number 3 |.
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