Ophthalmic manifestations and treatments of proteus syndrome: a case report and systematic review.

BACKGROUND: Proteus syndrome (PS) is an extremely rare disorder with ocular manifestations. In this study, we aimed to describe the ophthalmic characteristics and the clinical course of an unusual PS patient to acquire a comprehensive and intensive understanding of ocular PS and highlight the importance of collaborative treatment by ophthalmologists. CASE PRESENTATION: A case of PS with atypical ocular features and syndromes was observed in a Chinese female. Her proptosis and vision impairment were relieved after Endoscope-Navigation system (ENS)-aided optic canal decompression. A 1.5-year follow-up showed that the treatment was temporarily effective, but the disease continued to develop. A review of the literature was conducted: forty-eight patients met the inclusion criteria. Although ocular manifestations play important roles in PS diagnosis, only a limited number of cases have been reported to have ocular abnormalities. And to date, almost none of these reports have described the treatment in detail. Therefore, PS patients with ocular manifestations were reviewed. CONCLUSIONS: PS is a complex disorder with variable characteristics and progressive imbalances. In this paper, the clinical symptoms, molecular characteristics, and differential diagnosis of PS are introduced. More importantly, the ocular manifestations, treatment, and prognosis of PS cases to date are summarized and discussed. This study aimed to acquire a comprehensive and intensive understanding of ocular PS and to reveal the importance of collaborative treatment by ophthalmologists.

[HedgeHog inhibitor targeted therapy for periocular basal cell carcinoma: a case report].

A female patient, 87 years old, presented with an irregular swelling in the left medial canthus for 7 years. Due to the patient's poor general condition, radical surgery was not considered appropriate. Surgical biopsy confirmed periocular basal cell carcinoma of the left medial canthus. The patient was administered oral HedgeHog inhibitor targeted therapy for 6 months, resulting in approximately 90% reduction in tumor size. The patient's condition improved, and the tumor remained stable during the course of follow-up.

[Seventy years of development and prospects of the ocular oncology specialty in China].

Ocular tumors include intraocular tumors and tumors of the eyelid, orbit, conjunctiva and lacrimal apparatus. After seventy years of continued growth, ocular oncology in China, from small to large and from weak to strong, has made great achievements. Especially since the beginning of the new century, there has been all-round and rapid development. The mechanism of ocular tumorigenesis has been elucidated based on the biobank and animal models. New therapeutic techniques and treatment modalities have been established based on multi-center cohort studies. The team competence and the discipline level have been improved based on academic organization constructions and international exchanges. Looking into the future, ocular oncology in China will move on in gene detection and early diagnosis, basic research and drug targeting, interdisciplinary, intelligent diagnosis and treatment, clinical research and translational medicine, with the support of national strategies and scientific and technological innovation, to further improve the survival rate, the ocular salvage rate and the quality of life for patients with ocular tumors. This article is written to congratulate the Chinese Journal of Ophthalmology on the 70th anniversary of its publication. (Chin J Ophthalmol, 2020, 56: 646-652).

[An update on the treatment of orbital venous malformation].

Orbital venous malformation (OVM) ranks as the most common form of vascular lesions in the orbit. OVM may cause functional impairment as well as disfigured appearance in orbital and periorbital regions. A variety of treatment methods has been used to deal with OVM, including sclerotherapy, laser therapy, embolization, surgical resection. The treatment scenario should be made based on hemodynamics, location and extent of lesions in order to achieve encouraging results. With increased understanding on hemodynamics of OVM and application of newly proposed techniques, a combined modality of multiple approaches with defined order has been advocated to be applied in the treatment of OVM. In this paper, the latest advancement in treatment of OVM is reviewed. (Chin J Ophthalmol, 2018, 54: 548-550).

[The progress in the diagnosis and management of orbital lymphoma].

Lymphoma is one of the most common malignant tumor of the orbit in adults. Orbital lymphoma mainly consists of extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue, follicular lymphoma, diffuse large B-cell lymphoma, mantle cell lymphoma, infrequent NK/T cell lymphoma and Burkitt lymphoma. Diagnosis on orbital lymphoma remains challenging in that it is difficult to distinguish it from benign lesions, especially for low degree lymphomas. Combined examinations of histological, cytological and molecular features are required to make final differentiation. There are many treatment options for diagnosis of orbital lymphoma, such as surgical resection, radiotherapy, chemotherapy, monoclonal antibody targeted therapy, immunotherapy. The appropriate scenario of treatment on orbital lymphoma should be made based on the Ann Arbor or TNM staging system to determine the stage of orbital lymphoma at the moment for maximizing the results. The latest concepts on diagnosis and management of orbital lymphoma are reviewed in this paper. (Chin J Ophthalmol, 2017, 53: 632-636).

VEGF-targeted RNA interference suppresses angiogenesis and tumor growth of retinoblastoma.

Vascular endothelial growth factor (VEGF) is one of the most important angiogenic growth factors for tumor angiogenesis which has been verified to be involved in neovascularization of retinoblastoma. Here, we sought to explore whether RNA interference (RNAi) targeting VEGF could inhibit retinoblastoma angiogenesis and tumor growth. Stable transfection of the two human retinoblastoma cell lines SO-RB50 and HXO-RB44 with VEGF-targeted small interfering RNA (siRNA) expression plasmid significantly inhibited VEGF expression determined by real-time polymerase chain reaction (PCR), enzyme-linked immunosorbent assay and Western blot, whereas the control transfection showed no effects. The chemically synthesized VEGF siRNA dramatically suppressed tumor angiogenesis (CD34 immunohistochemistry) and tumor growth in the SO-RB50 subcutaneous xenograft model. Significant downregulation of VEGF expression both on messenger RNA and protein levels in VEGF-siRNA-treated SO-RB50 subcutaneous xenograft was confirmed by real-time PCR and Western blot compared to control. Our data demonstrate the suppression function on angiogenesis and tumor growth of retinoblastoma by VEGF-targeted RNAi. This novel therapeutic strategy promises to play a part in the clinical management of retinoblastoma.

Fluctuation of microcystins in water plant.

This study was undertaken to investigate the distribution and variation of microcystins (MC) from a water resource to a water plant in China including long distance transportation, ClO2 preoxidation, coagulation/precipitation, filtration, and disinfection. Advanced treatment methods including ozonation and granular activated carbon (GAC) adsorption were tested to remove microcystins from drinking water. The results indicated that, when containing high level of algae in raw water, preoxidation at 2 mg/L of ClO2 was not enough to stop algae growth in long distance transportation. In the water plant studied, both coagulation and filtration caused significant increase instead of removal of microcystins in drinking water. Chlorine disinfection removed part of microcystins. However, both ozonation and GAC adsorption were able to remove microcystins completely, thereby improving the quality of drinking water in terms of microcystins.