OPTIC DISK COLOBOMA AND CONTRALATERAL OPTIC DISK PIT MACULOPATHY TREATED BY VITRECTOMY IN A PATIENT WITH NOONAN SYNDROME WITH MULTIPLE LENTIGINES.

PURPOSE: To report a case of Noonan syndrome with multiple lentigines with unusual ocular features. METHODS: The authors describe a case of a 7-year-old girl with Noonan syndrome with multiple lentigines and anomalous optic disks. RESULTS: A 7-year-old girl with genetically proven Noonan syndrome with multiple lentigines ( PTPN11 gene mutation) and anomalous optic disks was referred for treatment of persistent macular detachment after 1 year of conservative follow-up. The right eye demonstrated an optic disk coloboma with the best-corrected visual acuity of 20/32, the left eye demonstrated an optic disk pit with serous macular detachment (best-corrected visual acuity 20/50-20/80). Optical coherence tomography demonstrated a neurosensory detachment. Twenty-five gauge pars plana vitrectomy was performed with posterior hyaloid detachment, drainage over disk pit area, and SF6 20% gas tamponade. Surgery resulted in subretinal fluid reduction and improvement of the visual acuity to 20/32. CONCLUSION: A case of Noonan syndrome with multiple lentigines with optic disk coloboma in the right eye and optic disk pit with related maculopathy in the left eye. To the best of the authors' knowledge, this is the first reported case describing the association of Noonan syndrome with multiple lentigines and congenital optic disk anomalies. Optic disk pit maculopathy was managed surgically because of its longstanding nature with the deteriorating visual acuity.

Incidence of rhegmatogenous retinal detachment after bag-in-the-lens IOL implantation: extended follow-up in a larger cohort of patients.

PURPOSE: To report the incidence of rhegmatogenous retinal detachment (RRD) and associated risk factors after cataract surgery using the bag-in-the-lens (BIL) intraocular lens (IOL) implantation technique. SETTING: Department of Ophthalmology, Antwerp University Hospital, Belgium. DESIGN: Prospective cohort study. METHODS: All consecutive BIL IOL surgeries performed between January 2001 and December 2010 were included, with the exclusion of combined procedures and IOL exchanges. The incidence of RRD was reported first in the total cohort, then in a subgroup of patients with 1 year to 5 years of follow-up, and finally in the group remaining after exclusion of all risk factors, except gender. Risk factors associated with RRD were examined using multiple Cox regression analysis with a random intercept. RESULTS: Rhegmatogenous RD was diagnosed in 36 eyes (1.06%) of 3385 BIL cases, with a mean follow-up of 48.28 ± 40.05 months (range 0 to 195 months). The 2-year cumulative RRD incidence rate was 0.66% (17 cases in 1024 eyes; 0.00% in patients without risk factors). The 5-year cumulative RRD incidence rate was 1.17% (26 cases in 931 eyes; 0.15% without risk factors). Five risk factors were confirmed: male sex, age less than 60 years at the time of surgery, axial length 25.0 mm or greater, a history of contralateral RD, and intraoperative surgical complications. CONCLUSIONS: The incidence of RRD after BIL IOL implantation is comparable with that of lens-in-the-bag (LIB) implantation. This larger study provided a longer follow-up and suggested that RRD incidence is even lower than that previously reported. This study also confirmed intraoperative surgical complications as an additional risk factor for RRD development, as already described with LIB implantation.

Incidence of rhegmatogenous retinal detachment after bag-in-the-lens intraocular lens implantation.

PURPOSE: To determine the incidence of rhegmatogenous retinal detachment (RRD) and associated risk factors after phacoemulsification and bag-in-the-lens intraocular lens (IOL) implantation. SETTING: Antwerp University Hospital, Department of Ophthalmology, Antwerp, Belgium. DESIGN: Prospective cohort study. METHODS: All consecutive bag-in-the-lens IOL implantations performed between January 2001 and December 2007 were included, with the exception of combined procedures and IOL exchanges. The retinal detachment (RD) incidence was studied in the total cohort, in a subgroup of patients with 1 to 5 years of follow-up, and finally in the group remaining after exclusion of all risk factors except gender. RESULTS: RD after bag-in-the-lens IOL implantation in 1323 eyes with an average follow-up of 44.75 months (range 0 to 152 months) was found in 19 eyes (1.44%). The 1-year RD incidence was 0.49% (5 RD cases in 1024 eyes) (0.00% in patients without risk factors). The 2-year cumulative RD incidence was 0.84% (9 RD cases in 931 eyes; 0.15% without risk factors). Four clinically significant risk factors were confirmed: male gender, young age at time of surgery (<60 years), axial myopia (axial length ≥25 mm), and history of contralateral RD in the total cohort. CONCLUSION: The RRD incidence following bag-in-the-lens IOL implantation was comparable to that seen after lens-in-the-bag (LIB) implantation. The wide variation in study design in the literature precludes direct comparison, so there is a need for standardization in evaluating RRD incidence after cataract surgery. Future prospective studies should consider patients with and without risk factors (except gender) separately. FINANCIAL DISCLOSURE: Prof. dr. M.J. Tassignon has intellectual property rights to the bag-in-the-lens intraocular lens (U.S. patent 6 027 531; EU patent 009406794; PCT/120268), which is licensed to Morcher GmbH, Stuttgart, Germany.