Heritable Disorders of Connective Tissue (HDCTs) are syndromes that disrupt connective tissue integrity. They include Osteogenesis Imperfecta (OI), Ehlers Danlos Syndrome (EDS), Marfan Syndrome (MFS), Loeys-Dietz Syndrome (LDS), Epidermolysis Bullosa (EB), Stickler Syndrome (STL), Wagner Syndrome, and Pseudoxanthoma Elasticum (PXE). Because many patients with HDCTs have ocular symptoms, commonly myopia, they will often present to the clinic seeking refractive surgery. Currently, corrective measures are limited, as the FDA contraindicates laser-assisted in-situ keratomileusis (LASIK) in EDS and discourages the procedure in OI and MFS due to a theoretically increased risk of post-LASIK ectasia, poor wound healing, poor refractive predictability, underlying keratoconus, and globe rupture. While these disorders present with a wide range of ocular manifestations that are associated with an increased risk of post-LASIK complications (e.g., thinned corneas, ocular fragility, keratoconus, glaucoma, ectopia lentis, retinal detachment, angioid streaks, and ocular surface disease), their occurrence and severity are highly variable among patients. Therefore, an HDCT diagnosis should not warrant an immediate disqualification for refractive surgery. Patients with minimal ocular manifestations can consider LASIK. In contrast, those with preoperative signs of corneal thinning and ocular fragility may find the combination of collagen cross-linking (CXL) with either photorefractive keratotomy (PRK), small incision lenticule extraction (SMILE) or a phakic intraocular lens (pIOL) implant to be more suitable options. However, evidence of refractive surgery performed on patients with HDCTs is limited, and surgeons must fully inform patients of the unknown risks and complications before proceeding. This paper serves as a guideline for future studies to evaluate refractive surgery outcomes in patients with HDCTs.
Purpose To assess visual outcomes over time of femtosecond laser-assisted cataract surgery compared to standard phacoemulsification cataract surgery. Design A retrospective, single-center comparative study. Methods Patient data including demographics, ocular biometry, pre- and postoperative visual acuity, postoperative complications, primary (uncorrected distance visual acuity over time) and secondary visual outcomes (uncorrected near visual acuity, best distance visual acuity, patient complaints, satisfaction, and postoperative surgery) were gathered and statistically analyzed. Demographic differences between patients receiving femtosecond-laser assisted cataract surgery (FLACS) versus standard phacoemulsification cataract surgery (PCS) were corrected for outcome comparison. Safety, efficacy, predictability, and stability were analyzed for each procedure and compared. Results A total of 155 eyes in PCS and 143 eyes in FLACS were analyzed at 1 week, 3 months, and 1 year using odds ratio. The odds ratio of being 20/20 or better and 20/40 or better at the specified time periods were similar and statistically insignificant at all time periods analyzed except 20/20 or better for uncorrected distance visual acuity (UDVA) at 1 year (p=0.0001) and uncorrected near visual acuity (UNVA) at 1 week (p=0.02). In both cases, the odds of being 20/20 or better favored FLACS. Mean UDVA, UNVA, and best distance visual acuity (BDVA) were all similar and statistically insignificant between the two groups. Postoperative patient complaints, safety, efficacy, predictability, and stability between the two groups showed no statistical significance. Conclusion Despite the odds ratio of being 20/20 or better favoring FLACS for UDVA at 1 year and UNVA at 1 week, the mean logMAR UDVA, BDVA, and UNVA were similar and statistically insignificant between the FLACS and PCS groups at 1 week, 3 months, and 1 year. Differences in visual acuity were likely due to differences other than surgical approaches. While both FLACS and PCS are appropriate approaches to cataract surgery, one does not appear to be superior when assessing longitudinal markers for visual acuity, safety, efficacy, predictability and stability.
This case report discusses the follow-up of a woman with HR+/ERBB2 invasive ductal breast carcinoma metastatic to the lungs, brain, and spine.
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