Duane retraction syndrome (DRS) is a congenital eye movement anomaly characterized by variable horizontal duction deficits, with narrowing of the palpebral fissure and globe retraction on attempted adduction, occasionally accompanied by upshoot or down-shoot. The etiopathogenesis of this condition can be explained by a spectrum of mechanical, innervational, neurologic and genetic abnormalities occurring independently or which influence each other giving rise to patterns of clinical presentations along with a complex set of ocular and systemic anomalies. Huber type I DRS is the most common form of DRS with an earlier presentation, while Huber type II is the least common presentation. Usually, patients with unilateral type I Duane syndrome have esotropia more frequently than exotropia, those with type II have exotropia and those with type III have esotropia and exotropia occurring equally common. Cases of bilateral DRS may have variable presentation depending upon the type of presentation in each eye. As regards its management, DRS classification based on primary position deviation as esotropic, exotropic or orthotropic is more relevant than Huber’s classification before planning surgery. Surgical approach to these patients is challenging and must be individualized based on the amount of ocular deviation, abnormal head position, associated globe retraction and overshoots.
Purpose:To study the long-term safety profile and visual outcomes of primary intraocular lens (IOL) implantation in infants <6 months of age.Methods:This was a retrospective observational study conducted at a tertiary eye care center in South India. Infants under 6 months meeting the selection criteria who underwent cataract surgery (lens aspiration, primary posterior capsulorhexis, and anterior vitrectomy) with primary IOL implantation between January 2008 and December 2011 and minimum 3-year follow-up were included. Patient demographics, serial refractions, visual acuity, complications, and associated amblyopia/strabismus were reviewed. Visual acuity, myopic shift, and complications were the outcome measures.Results:Sixty-nine eyes of 38 infants (31 bilateral; mean age: 4.6 months) were reviewed. Mean follow-up was 51 months (range: 36–84). Median logMAR best-corrected visual acuity at the final visit was 0.74 (interquartile range [IQR]: 0.50–0.98) in eyes with bilateral cataracts and 0.87 (IQR: 0.60–1.14) in eyes with unilateral cataracts with an average myopic shift of 6.7 diopters over 4.2 years. Most common postoperative complication was visual axis opacification (VAO) (13 eyes, 18%), necessitating membranectomy followed by pigmentary IOL deposits (11 eyes, 15%), and IOL decentration and glaucoma in four eyes each (5.6%). Mixed linear effect model found no significant association of age, gender, laterality, and postoperative complications with final visual acuity (P ≥ 0.05). Eyes with unilateral cataracts had a greater myopic shift than bilateral cases (P = 0.03).Conclusion:Primary IOL implantation in infants <6 months is reasonably safe in appropriately selected infants. VAO was the most common postoperative complication, and a large myopic shift was observed.
Various surgical approaches have been described for the management of Duane retraction syndrome (DRS), a type of congenital cranial dysinnervation disorder (CCDD), the goals of which include correcting the primary position deviation and abnormal head posture (AHP), minimizing globe retraction and overshoots and improving the ocular rotations. Vertical rectus transposition (VRT) is one such technique, found more effective in improving abduction and thereby expanding the field of binocular vision, as compared to horizontal muscle surgery. VRT, however, is associated with the risk of inducing vertical deviations and also poses a risk for development of anterior segment ischemia. To overcome these concerns, transposition of only the superior rectus to the lateral rectus was proposed and evaluated to reveal improvement in alignment, AHP and motility comparable to VRT but with lesser surgical time and fewer post-operative complications. With promising results in the management of DRS, superior rectus transposition (SRT) has been extensively studied and has evolved over the last decade with several modifications to further increase the efficiency and reduce the risk of post-operative complications. This article focusses on the pre-operative considerations while planning SRT in DRS, various approaches and surgical techniques described, and the outcomes and complications of SRT in DRS. The role of SRT in the management of other CCDDs may be explored with further studies.
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