The Management of Congenital Microphthalmia With Orbital Cyst: A Case Series.

PURPOSE: To describe a series of patients treated for congenital microphthalmia associated with orbital cyst and recommend a management protocol. METHODS: This retrospective case series comprised 6 patients (7 eyes) who attended an oculoplastic tertiary medical center from 2001 to 2018. Clinical, treatment, and outcome data were collected from the electronic files. Main outcome measures were preservation of vision and cosmetic appearance. RESULTS: Four patients were diagnosed at birth. Six cysts were located inferiorly and one superiorly. Two patients had a visual potential of light perception or better in the affected eye. In 4 eyes, the cyst was initially retained and the eye was fitted with a custom-made conformer. In 1 eye, the fornices were too shallow for a conformer, warranting fornix reconstruction and cyst excision. Early surgery was required in 1 eye for an expanded cyst and large orbit volume, and in another eye the cyst had overgrown the orbit, causing bone erosion and remodeling. Cosmetic results were good in 3 of the eyes in which the cyst was retained in early childhood, stimulating orbital growth. CONCLUSIONS: Congenital microphthalmia with orbital cyst is rare. Management should focus on preserving visual potential, especially in unilateral cyst cases when the other eye is also microphthalmic. Otherwise cosmetic symmetry is the main concern; cyst retention combined with ocular conformers may stimulate socket expansion. The authors found that, in most cases, if treated early, enucleation was avoidable during cyst excision. Early assessment, meticulous follow-up, and individually tailored treatment are warranted. [J Pediatr Ophthalmol Strabismus. 2022;59(3):192-199.].

Effect of Subretinal Fluid Drainage Using Perfluorocarbon During Retinal Detachment Repair on Postoperative Metamorphopsia.

BACKGROUND AND OBJECTIVE: To determine whether subretinal fluid drainage (SRF) using perfluorocarbon (PFC) during rhegmatogenous retinal detachment (RRD) repair reduces postoperative metamorphopsia. PATIENTS AND METHODS: Consecutive patients after RRD were evaluated for best-corrected visual acuity (BCVA), funduscopy, and metamorphopsia using M-CHARTS. Fundus autofluorescence and optical coherence tomography were performed. Clinical and operative data were collected. RESULTS: The cohort included 30 patients, of whom 11 (36.7%) underwent intraoperative PFC injection. Rates of macula-off RRD were similar in the two groups (54.5% and 47.4%, P = .705). No association was found between PFC injection and metamorphopsia score. Preoperative macula-off RRD was associated with significantly higher vertical and horizontal metamorphopsia scores than preoperative macula-on. BCVA was significantly worse in the patients with metamorphopsia (logMAR 0.15 vs. logMAR 0.04; P = .042) CONCLUSION: Intraoperative SRF drainage with PFC appears to have no beneficial effect on postoperative metamorphopsia. Metamorphopsia is associated with macular status. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e263-e270.].

Nuclear factor-kappaB protects the liver against genotoxic stress and functions independently of p53.

p53 and NF-kappaB are two key effectors in the chemotherapy-induced genotoxic response. Although p53 is a universal inducer of apoptotosis in many stress responses, including the genotoxic response, the role of nuclear factor (NF)-kappaB is not consistent and was reported to both counteract and mediate apoptosis. Although the reason for the apparent contradictory effects of NF-kappaB is not understood, it may partly be related to the reported cross-regulation of NF-kappaB and p53. Thus far, all studies exploring the cross-talk between p53 and NF-kappaB in conjunction with apoptosis have been performed in tissue-cultured cells and may therefore not faithfully represent conditions that prevail within a chemotherapy-subjected organism. To address this concern, we examined the respective roles of NF-kappaB and p53 in a liver model of doxorubicin-induced DNA damage. Using this animal model, we report that NF-kappaB is activated in response to doxorubicin-induced genotoxic stress and exerts a pronounced protective effect in opposing chemotherapy-induced tissue damage. Importantly, the activation of NF-kappaB occurs independently of p53 status. Furthermore, although p53 is also induced in this in vivo system, its induction is independent of NF-kappaB and does not contribute to the extent of tissue damage. These findings may have important implications with respect to the potential use of NF-kappaB modulators in cancer therapy.