Early detection and timely treatment hold the key to cure retinopathy of prematurity (ROP). ROP screening is carried out unevenly in China. Examination equipments and personnel experiences are the major factors to constraint ROP screening. In addition to strengthening personnel training, it is necessary to establish a standard guideline and pipeline for ROP consultation and referral. Laser photocoagulation and cryotherapy are the first options for the threshold ROP and Type 1 prethreshold ROP. Scleral buckling or vitrectomy is needed for advanced ROP when retinal detachment occurs. The clinical efficacy of intravitreal injection of bevacizumab (an anti-vascular endothelial growth factor monoclonal antibody) for severe ROP is encouraging, but needs further verification. Genetic interference and stem cell therapy will be the prosperous futures in the treatment of ROP. The screening and treatment of ROP in China is becoming more common and improved, but it is still a long way to go considering the huge population of China.
“The international classification of retinopathy of prematurity (ROP)(The 3rd edition)”, retains current definitions such as zone, stage, and circumferential extent of disease, however, there are also many updates. Major updates include: (1) increase of the definition of posterior pole Ⅱ; (2) introduction of a new concept "notch"; (3) definition of stage 5's subcategorization; (4) recognition that a continuous spectrum of vascular abnormality exists from normal to plus disease. Updates also include the definition of “aggressive ROP” to replace “aggressive-posterior ROP”. ROP regression and reactivation are described in detail, with additional description of long-term sequelae.
ObjectiveTo observe the fundus fluorescein angiography (FFA) manifestations of pediatric morning glory syndrome (MGS) patients. MethodsFourteen eyes diagnosed as MGS of 14 patients were studied. Among the 14 cases, there were 7 male and 7 female patients. At the time of FFA, the mean age of the patients was (38.75±33.91) months old, ranging from 5.5 to 128.0 months. Among the 14 eyes, four (28.57%) were associated with persistent hyperplastic primary vitreous; four (28.57%) were associated with retinal detachment with no retinal breaks, and one (7.14%) was associated with peripapillary subretinal exudation. All patients underwent peripapillary laser photocoagulation under general anesthesia first and then FFA with the third generation of wide-angle digital retinal imaging system. The arm-retinal circulation time (A-RCT), numbers of blood vessels on the edges of optic disc of the MGS eyes and the contralateral healthy eyes, retinal vascular morphology, the peripheral avascular area, neovascularization, retinal detachment and other abnormalities were documented. The horizontal and vertical diameters of the optic disc of the affected eyes and the contralateral healthy eyes were measured. To compare the A-RCT, 16 children with normal FFA were selected as control group. ResultsThe diameters of the vertical and horizontal axis of the affected eyes were as (2.56±0.58) and (2.73±0.60) times of the contralateral healthy eyes respectively. The average A-RCT of the affected eyes and eyes of the control group were (13.25±4.10) and (9.34±2.20) s respectively. The affected eyes had significantly prolonged A-RCT. At early stage, the optic disc and peripapillary areas showed hypo-fluorescence, while the irregular retinochoroidal atrophy area outside of the optic disk manifested as hyper-fluorescence ring. At late stage, optic disc showed hyper-fluorescence. Numbers of blood vessels on the edge of the optic disc of the affected eyes and contralateral healthy eyes were 30.27±4.86 and 15.83±1.95 respectively, the affected eyes had much more vessels than the contralateral healthy eyes. All affected eyes had peripheral retinal non-perfusion areas. ConclusionFFA examination showed prolonged A-RCT and peripheral retinal non-perfusion areas in the affected MGS eyes.
ObjectiveTo analyze and discuss the clinical features and management of pediatric retinal detachment (RD) associated with morning glory syndrome (MGS). MethodsThe clinical data of 49 patients (51 eyes) with RD associated with MGS were retrospectively analyzed, including 27 males (27 eyes) and 22 females (24 eyes). The age at first diagnosis ranged from 1 week after birth and 13 years old (4.2±3.2) years. There were 33 eyes of exudative RD, 3 eyes of rhegmatogenous RD and 15 eyes of RD with undetermined cause. Twenty eyes of 20 patients had other congenital ocular abnormalities, including persistent hyperplastic primary vitreous, microphthalmia, choroidal coloboma, iris coloboma. Besides retinal detachment, other complications were found, including cataract, secondary glaucoma,corneal leukoma or edema, strabismus and nystagmus. Twenty-two cases (22 eyes) received treatment. Five cases of mild exudative retinal detachment took oral methazolamide tablets. Three eyes with mild and restricted retinal detachments got retinal laser photocoagulation around the optic disc. Fourteen cases of 14 eyes underwent surgery including vitrectomy, lensectomy, and phacoemulsification. Follow-up after treatment were three months or more, with an average of (20.3±11.8) months. The visual acuity, retinal reattachment and intraocular pressure and other complications after treatment were followed up. ResultsFive patients of exudative retinal detachment were remained stable by taking methazolamide tablets. Two of the 3 eyes remained stable after laser therapy; however, the remaining one eye was not controlled after relaser. Cataract and glaucoma were resolved by lensectomy in 7 eyes and phaco-emulsification surgery in 1 eye. Retina was re-attached in 6 eyes after vitrectomy. Among those treated, 10 eyes had records of visual acuity. Visual acuity was improved in 1 eye after laser therapy and remained stable in 9 eyes. There were no drugs and surgery-related complications. ConclusionsRetinal detachment was the main complication of MGS. The high incidence and poor prognosis call for the need of close follow-up and timely treatment, including medicine, laser and surgery.
ObjectiveTo observe the efficacy and safety of combination of intravitreal injection of ranibizumab and laser photocoagulation for the treatment of aggressive posterior retinopathy of prematurity (AP-ROP). MethodsMedical records of 70 eyes of 35 premature infants with a primary diagnosis of AP-ROP in our clinic were reviewed and analyzed retrospectively. All the lesions were located in posterior zone, with 42 eyes in zone 1 and 28 eyes in zone 2. Forty-six eyes had iris neovascularization, while 19 eyes combined with vitreous hemorrhage. All participants underwent intravitreal injection of ranibizumab as the primary treatment within 12 hours after diagnosis of AP-ROP. The systemic and ocular adverse effects were observed. The change of retinal vascular tortuosity and dilatation before and after the intravitreal injection of ranibizumab was observed one week after injection. Laser photocoagulation was used as adjuvant therapy if the plus disease persisted more than two weeks or new-onset ridge occurred after injection. The mean time interval between injection and laser therapy was (5.1±2.6) weeks (range, 1-10 weeks). Follow-up ranged from 6 to 18 months, with a mean of (10.3±3.9) months. The anatomical results and complications were evaluated after treatment. The eyes that progressed to stage 4 or 5 during the follow-ups were underwent lens-sparing vitrectomy or lensectomy combined with vitrectomy. ResultsNo major systemic or ocular complications were observed. Preretinal hemorrhages were found in 12 eyes of 8 patients (17.1%), but they were absorbed spontaneously during the follow-ups. All lens remained transparent and no iatrogenic retinal hole was occurred during the follow-ups. After the injection, the regression of iris neovascularization was observed in 46 eyes within one week, vitreous hemorrhage absorbed significantly in 16 eyes (84.2%), and plus disease disappeared completely within one week in 61 eyes (87.1%). 59 eyes (84.3%) demonstrated vascularization toward the peripheral retina after treatment. 32 out of 42 eyes (76.2%) with zone 1 demonstrated vascularization toward to zone 2, while 24 out of 28 eyes (85.7%) with zone 2 demonstrated vascularization toward to the junction of zone 2 and 3. After intravitreal injection of ranibizumab combined with laser photocoagulation, 62 of 70 eyes (88.6%) had retinal vascular ridge and plus disease regression. However, 8 eyes of 6 patients (11.4%) showed significant fibrovascular proliferation and progressed to retinal detachment after the combination treatment of intravitreal ranibizumab injection and laser photocoagulation. Four eyes underwent lens-sparing vitrectomy, while the other 4 eyes underwent vitrectomy combined with lensectomy. Five eyes achieved totally retinal reattachment after surgery, while 3 eyes achieved partially retinal reattachment. ConclusionThe combination of intravitreal injection of ranibizumab and laser photocoagulation is safe and effective in the treatment of AP-ROP.
ObjectiveTo observe the clinical features of familial exudative vitreoretinopathy (FEVR). Methods202 FEVR patients (404 eyes) from 84 families were included in this retrospective study, all the clinical diagnosis was made by the same expert in our Department. All patients were examined with slit lamp microscope, indirect ophthalmoscopy, children's retinal imaging system or Heidelberg HR2 fundus cameras for fundus photographs. Fluorescein angiography (FFA) was also performed for all patients, for those≤7 years old it was performed under general anesthesia. Data collected from charts included gender, age at presentation and family history. According to the fundus presentation and FFA results, FEVR was classified into 0-5 stages. Stage 0 (normal) is for the health fellow eye in unilateral FEVR; stage 1: retinal peripheral avascular zone with no fluorescence leakage in FFA; stage 2: retinal peripheral avascular zone, with fluorescence leakage in FFA; stage 3: partial retinal detachment not involving the macular; stage 4: partial retinal detachment involving the macular; stage 5: total retinal detachment. ResultsThere were 119 male patients and 83 females. The average age of diagnosis was 16 months, the average gestational age was 39 weeks and the average birth weight was 3223 g in the proband. At the first clinical visit, there was clear family history of FEVR in 4 patients (1.98%); family history of other eye disease (nystagmus, cataract, small eyes, strabismus and others) in 25 patients (12.38%); not family history of eye disease in 173 patients (85.64%). FEVR family history was confirmed by fundus examination and FFA for the family members. In all 404 eyes, 9 eyes (2.23%) was normal, 162 eyes (40.10%) was stage 1, 97 eyes (24.01%) was stage 2, 72 eyes (17.82%) was stage 3, 20 eyes (4.95%) was stage 4, and 44 eyes (10.89%) was stage 5. In 202 patients, 59 cases (29.21%) was asymmetry bilateral disease with different stage for each eye. In 404 eyes, retinal fold was found in 74 eyes (18.32%). ConclusionsFEVR was common in full-term and/or normal birth weight newborns. More than half of patients are in stage 1 and 2 without any clinical features. 29.21% eyes were asymmetry bilateral diseases.