RESUMO
Sengers syndrome is a rare autosomal recessive mitochondrial disorder characterized by congenital cataract, hypertrophic cardiomyopathy, and mitochondrial myopathy. We report two siblings with known mutation for Sengers Syndrome (AGK gene mutation) who presented to us with cataract and hypertrophic cardiomyopathy. They have a deceased elder sibling who was operated for cataract earlier.
Assuntos
Cardiomiopatias , Catarata , Idoso , Catarata/diagnóstico , Catarata/genética , Criança , Humanos , Mutação , Linhagem , Fosfotransferases (Aceptor do Grupo Álcool)/genéticaRESUMO
PURPOSE: Phacoemulsification is the surgical procedure of choice for cataract, providing excellent visual and safety outcomes. Femtosecond laser-assisted cataract surgery (FLACS) is an addition to the surgical armamentarium. The study aims to compare the outcomes of FLACS using LenSx™ (Alcon Inc., USA) to standard 2.2 mm clear corneal phacoemulsification. Prospective case-control, comparative, interventional study was conducted in a tertiary care center. METHODS: In each group, 55 eyes of 55 patients underwent cataract surgery using either FLACS or conventional phacoemulsification (control group). The primary outcome variables, uncorrected visual acuity (UCVA), best-corrected visual acuity (BCVA), specular microscopy, pachymetry, mean absolute error (MAE), and anterior chamber depth (ACD) were compared between two groups at 4 weeks postoperatively. Intraoperative effective phaco time (EPT), postoperative circularity of the rhexis, capsular overlap over the edge of the intraocular lens (IOL), and decentration of the IOL were the secondary variables which were compared. RESULTS: No significant difference was found between the groups for UCVA, pachymetry, MAE, and ACD at 4 weeks postoperatively. The FLACS group had better BCVA (P = 0.0294). Circularity of capsulorrhexis (P = 0), circular overlap over the edge of IOL (P = 0), and centration of IOL (P = 0.002) at 4 weeks postoperatively were better in the FLACS group. EPT was lower in FLACS for similar grade of cataract (P = 0). Endothelial cell loss in FLACS group was 4.2% more (P = 0.032). CONCLUSIONS: FLACS is superior to conventional phaco in the circularity of rhexis, capsular overlap, and centration of the IOL and uses less EPT. However, conventional phacoemulsification is equivalent to FLACS in most other parameters.