SCIENTIFIC POSTER 2016_Surgical Management of Multiple Anterior Segment Malformations with RD in a 3 Year Old Boy Patrycja Krzyzanowska-Berkowska

Objective:

To present major challenges of complicated congenital bilateral cataract surgery and pediatric pars plana vitrectomy in eyes with anterior proliferative vitreo-retinopathy (PVR).

Case Report:

We present a 3-year-old boy with bilateral congenital cataract, subluxated lens, iris coloboma and ciliary processes displacement, who developed retinal detachment after cataract surgery in both eyes. Patient was operated because of the congenital cataract in April 2014. Standard pediatric cataract surgery included the continuous curvilinear capsulorhexis, bimanual lens aspiration, posterior capsulotomy and anterior vitrectomy. During the surgery ciliary processes displacement and subluxation of the lens in the inferior quadrants were found. Due to the pathology of the anterior segment the PC IOL insertion was abandoned. Second eye surgery was planned in a short time to manage optimal visual rehabilitation, but recurrent infections postponed the execution of the procedure. Mother did not report on the routine controls with a child to the out-patients clinic until the time of the second surgery. The boy was admitted to the hospital in January 2015. A slit lamp examination revealed posterior capsule phimosis and further dislocation of the ciliary processes to the center as well as total retinal detachment with an anterior PVR in the right eye. The 20 gauge pars plana vitrectomy, ILM peeling, peripheral endolaser and 5000 silicon oil endotamponade were performed to obtain retinal attachment. Iris was sutured in order to create Ando pseudo-iridectomy. In February 2015 cataract surgery of the left eye was performed. The procedure included the lens removal and the anterior vitrectomy. Due to the unfavorable course of the previous operation the treatment of the other eye was modified to avoid a PVR and its sequelae. A capsular bag of the left eye was totally removed, ciliary processes were cauterized with endocautery because of bleeding and anty-VEGF was injected at the end of surgery. Four months later we observed a peripheral retinal detachment in the left eye. The boy was operated with 20 gauge pars plana vitrectomy with 5000cs silicone oil endotamponade. In July 2015 we obtained retinal attachment with 5000cs silicone oil tamponade in both eyes. Refraction error in the right eye was +13 Diopters with -1,75 cylinder in 174 axis. In the left eye refraction error was +17 Diopters, with -0,5 cylinder in 160 axis, with normal fundus in postoperative examination. In October 2015 the boy was operated on again because of the retinal redetachment under silicone oil and anterior proliferative vitreo-retinopathy in the right eye. Results: We have obtained incomplete retinal attachment with 5000cs silicone oil tamponade in both eyes. In the left eye we observe a shallow lift of the retina from the bottom with proliferations underneath, and retinal detachment in the nasal quadrant in the right eye. Macula is attached in both eyes. Best corrected visual acuity in the right eye is counting fingers and in the left eye 0,1 on the Snellen chart. The major problem is the passage of oil to the anterior chamber and the risk of corneal decompensation. Another issue is the presence of the retinal detachment under the 5000cs silicone oil and the consequent poor visual prognosis. Genetic tests came out correctly.

Conclusion:

In complicated cases of congenital subluxated cataract with ciliary processes dislocation the complete removal of the posterior bag does not prevent retinal detachment and anterior proliferative vitreo-retinopathy. There is discussion to decide on the treatment of cataract surgery in the left eye, whereas postoperative course in the right eye has been complicated with retinal detachment. Due to the high risk of poor vision in eyes with dense, congenital cataract, it is difficult to determine the appropriate procedure in this case.

Contact Details:

Email: p_krzyzanowska@wp.pl
Cell Phone: +48502612901

Patrycja Krzyżanowska-Berkowska