Persistent fetal vasculature syndrome (PFVS) is a rare congenital vitreous dysplasia, which is classified as anterior, posterior and combined types according to the location of the vascular abnormalities. The clinical manifestations of PFVS are diverse, and early surgical intervention is very important. The main objective of surgical treatment is to remove the anterior and posterior traction between fibrovascular membranes and retina as well as lens, and to reconstruct clear visual axis. Surgical treatments include pupilloplasty, lensectomy with or without intraocular lens implantation and vitrectomy via limbal or scleral approach. For new technologies, the applications of ophthalmic viscosurgical device and femtosecond lasers have desirable results . In addition to focusing on improving the success rate of surgery, it is also necessary to systematically and comprehensively assess the overall preoperative condition and postoperative visual function of the patients. PFVS eyes have limited improvement in postoperative vision, which is related to the extent of lesion involvement and the occurrence of complications. Eyes with macular dysplasia and tractional retinal detachment, as well as elongated ciliary process, have a poor prognosis of vision after surgery. How to improve postoperative vision in the eye affecting the posterior segment of the eye with PFVS from the microscopic anatomical relationship between the fibrous vascular pedicle and the retina is worth further study. On the other hand, reducing surgical trauma and optimizing surgical procedures in order to improve postoperative visual acuity and reduce postoperative complications are also the key research directions of future PFVS treatments.
ObjectiveTo evaluate the efficacy and safety of comprehensive treatment for retinoblastoma (RB). MethodsA retrospective clinical study. From January to December in 2019, 157 cases (203 eyes) of RB who were diagnosed by the Department of Ophthalmology of Xinhua Hospital and received comprehensive treatment were included in this study. Of cases, 76 were male, and 81 were female; 111 were unilateral, and 46 were bilateral. The medium of age at diagnosis was 20.1 months. All patients received treatment for the first time. Patients with intraocular tumors were divided into A-E stages, extraocular stage and distant metastasis according to international intraocular RB classification standard. The median follow-up time was 37.4 months. Clinical features, treatment, prognosis and ocular complications of all cases were recorded. ResultsAmong 157 cases (203 eyes), 137 cases (180 eyes) were in intraocular stage; 6, 14, 10, 98, and 52 of eyes were in A-E stages, respectively. Twelve cases (12 eyes) were in extraocular stage; 8 cases (11 eyes) were in distant metastasis stag; 8 cases died due to distant metastasis; 149 cases (94.9%, 149/157) survived; 48 eyes were enucleated, 34 of which underwent initial enucleation, and 14 eyes underwent enucleation after eye-preserving treatment. The overall global salvage rate was 155 eyes (76.4%,155/203), and that after eye-preserving treatment was 91.7% (155/169). Severer eye for bilateral cases was taken into account for statistic; 120 cases (120 eyes) received initial eye-preserving treatment. Among them, 36 and 84 eyes underwent initial intravenous chemotherapy (IVC) and initial intra-arterial chemotherapy (IAC), respectively. The enucleation of the two groups was 7(19.4%, 7/36), 7(8.3%, 7/84); 33 (91.7%, 33/36) and 33 (39.3%, 33/84) eyes received the second treatment, respectively. There was no significant difference in the rate of enucleation between the two treatments (χ2=2.037, P=0.154). There was significant difference in the percentage of secondary treatment (χ2=27.937, P＜0.001). Fifty-four eyes (45.0%, 54/120) stabilized after initial treatment, and 66 eyes (55.0%, 66/120) underwent secondary treatments due to poor response or tumor recurrence. For 66 eyes receiving secondary treatments, enucleation, IAC, intravitreous chemotherapy (IVitC), IAC combined with IVitC, and laser and/or cryotherapy was performed in 6, 18, 12, 13, and 17 eyes, respectively. The number of eyes of enucleation among the IAC, IVitC, and IAC combined with IVitC group was 5 (27.8%, 5/18), 1 (9.3%, 1/12), and 2 (15.4%, 2/13) eyes, which was no significantly different (χ2=2.001, P=0.368). Until the last follow-up, visual acuity outcomes were acquired in 148 eyes (72.9%, 148/203). Among them, 41, 53, 16 and 38 eyes had no light perception, light perception to finger counting, 20/400, and ≥20/200, respectively. In total, among 203 eyes, 121 eyes received IAC, of which 2, 4, and 1 eyes had optic disc atrophy, vitreous hemorrhage, and severe retinal-choroidal atrophy, respectively; 60 eyes received IVitC, of which one and one eye had vitreous hemorrhage and macular hemorrhagic necrosis, respectively. ConclusionsIn this study, the overall survival rate was 94.9% after comprehensive treatment and the rate of global salvage after eye-preserving treatment was 91.7%. The comprehensive treatment of retinoblastoma had a relatively high efficacy and safety.