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Leber congenital amaurosis due to CEP290 ciliopathy is being explored by treatment with an antisense oligonucleotide (AON) sepofarsen. One individual who was part of a larger cohort (ClinicalTrials.gov no. NCT03140969) was studied for 15 months after a single intravitreal sepofarsen injection. Concordant measures of visual function and retinal structure reached a substantial efficacy peak near 3 months post-injection. At 15 months, there was sustained efficacy even though there was evidence of reduction from peak response. Efficacy kinetics can be explained by the balance of AON-driven new CEP290 protein synthesis and a slow natural rate of CEP290 protein degradation in human foveal cone photoreceptors.

Photoreceptor ciliopathies constitute the most common molecular mechanism of the childhood blindness Leber congenital amaurosis. Ten patients with Leber congenital amaurosis carrying the c.2991+1655A>G allele in the ciliopathy gene centrosomal protein 290 (CEP290) were treated (ClinicalTrials.gov no. NCT03140969 ) with intravitreal injections of an antisense oligonucleotide to restore correct splicing. There were no serious adverse events, and vision improved at 3 months. The visual acuity of one exceptional responder improved from light perception to 20/400. RNA antisense oligonucleotide therapy to restore normal splicing of a ciliopathy gene shows promising safety and efficacy results in a clinical trial to treat a form of childhood blindness.

In 1985 this cleft team, dissatisfied with the treatment and results from cleft lip and palate repair, began a longitudinal long-term study using dynamic maxillary orthopedics and periosteoplasty as was originally described by Drs Millard and Latham. All cases were carefully documented through adolescence, including clinical assessments, orthodontic, radiographic, and cephalometric analyses. In 1998, in this journal, we published our data on 35 complete unilateral and 10 complete bilateral cleft patients. At that time facial growth was following normal cephalometric patterns. Crossbites were dental and treated with orthodontics. There was radiologic evidence of bone within the alveolus with elimination of the oronasal fistula, and facial aesthetics revealed soft faded scars and balanced noses.That publication was a preliminary study with the intent to provide long-term results when full facial growth was achieved. This article reports on 25 patients from the initial cohort (20 unilateral and 5 bilateral) that we were able to closely follow up for 25 years, with the same clinical team, making it the longest study of its kind.At this stage, data revealed continued growth of the midface both vertically and horizontally. Secondary alveolar cleft bone grafting when required was in small aliquots placed into well-healed tissue, and orthodontic movement of teeth was through a consolidated alveolus. Orthognathic procedures were performed in 2 of 5 bilateral and 0 of 20 unilateral cases.We concluded that in this cohort, dynamic maxillary orthopedics and periosteoplasty, despite controversy in the literature, did not negatively impact facial growth and provided the benefit of early structural normalization and social integration by consolidation of the maxilla, closure of the oronasal fistula, tension free closure of the lip, and by balancing the nose.

Background Board-certified plastic surgeons utilizing the Sientra 207 Highly Cohesive Gel breast implants provided their initial satisfaction with usage and postoperative results on primary and revision augmentation patients, and study participants provided their satisfaction with surgical result. Methods Up to 15 participating investigators at institutional review board-approved sites enrolled up to 180 women who received Sientra 207 implants for primary augmentation or revision augmentation procedures. Investigator and participant assessments were performed preoperatively and postoperatively at 3, 6, and 12 months. Results All participating surgeons found the implants to be easy or very easy to implant, and 92 percentage reported satisfaction with surgical results. Patients receiving these implants reported similarly high rates of satisfaction (94%). Conclusion With this combination of high satisfaction and low complication rates, the Sientra 207 silicone breast implants now provide a welcome addition to plastic surgeons' breast implant options.

In this review, the authors provide a 20-year review and comparison of implant options and describe the evolution of breast implant surface textures; compare available implant surfaces; present long-term safety data from the 10-year US-based Core clinical studies; list the key benefits and risks associated with smooth and textured implants; and provide perspectives on breast implant-associated anaplastic large cell lymphoma (BIA-ALCL). The authors explore the key benefits and risks associated with all available devices so that optimal and safe patient outcomes can be achieved.

After reading this article, the participant should be able to: 1. Understand the key decisions in patient evaluation for cosmetic breast augmentation. 2. Cite key decisions in preoperative planning. 3. Discuss the risks and complications, and key patient education points in breast augmentation.Breast augmentation remains one of the most popular procedures in plastic surgery. The integral information necessary for proper patient selection, preoperative assessment, and surgical approaches are discussed. Current data regarding long term safety and complications are presented to guide the plastic surgeon to an evidence-based approach to the patient seeking breast enhancement to obtain optimal results.