Paediatric retinal disease is the most important part of paediatric ophthalmology. It usually manifests as leukocoria or yellow pupil, typically in retinopathy of prematurity, familial exudative vitreoretinopathy, persistent hyperplastic primary vitreous, Coats disease and retinoblastoma. It also can be manifested as nystamus poor visual fixation or progressive worsening of visual function, typically in Leber congenital amaurosis, Stargardt disease, Best disease and cone and rod dystrophy. Paediatric retinal diseases can be roughly divided into acquired, hereditary and congenital developmental abnormalities. With the development of gene and stem cell technologies, the advent of new medicine, equipments and new techniques, the concept of diagnosis and treatment in paediatric retinal diseases is also changing. In China, the level is improving progressively in both clinical and research areas of paediatric retinal diseases.
ObjectiveTo investigate the effects of limbal vitrectomy for persistent hyperplastic primary vitreous (PHPV) with disappeared anterior chamber and corneal opacity. MethodsSixteen eyes of 16 children with unilateral PHPV were included in this retrospective study. All the 16 eyes had both anterior and posterior disease, including opaque cornea, disappeared anterior chamber, pupil occlusion and extensive synechia, and lens opacity. The visual acuity was hand movement in 1 eye, and light perception in 15 eyes. There were 7 eyes with microphthalmia, 4 eyes with total retinal detachment, 3 eyes with retinal dysplasia, 1 eye with retinal folds, and 4 eyes with high intraocular pressure preoperatively. All the eyes underwent limbal vitrectomy, including dissection of synechia, pupil formation, lensectomy, posterior lens membrane peeling and vitrectomy. Four eyes with retinal detachment received long-acting gas tamponade following vitrectomy. The mean follow-up was 15.8 months (range from 9 to 21 months). ResultsNo eye had intraoperative complications. After surgery, all the 16 eyes had normal anterior chamber, round pupil and improved corneal transparency. Two eyes achieved a final visual acuity of 20/940 or better. Of the 4 eyes with retinal detachment, 3 eyes achieved retinal reattachment. Of the 4 eyes with preoperative high intraocular pressure, 3 eyes had controlled intraocular pressure postoperatively and the remaining 1 eye underwent glaucoma procedure at 6 months after the primary surgery. ConclusionPHPV eyes with disappeared anterior chamber and corneal opacity have a potential for developing better vision with improved cosmetic outcome and controlled intraocular pressure after vitrectomy by limbal approach.
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 evaluate the outcomes of laser photocoagulation of congenital X-linked retinoschisis (XLRS) at progressive stage. MethodsTwenty-seven cases (36 eyes) of XLRS sick kids were enrolled in this study. All patients were followed up for more than 1 year, retinoschisis has developed slowly but complications occurred during the follow-up. They are all boys from 3 to 12 years old; the average age was 6.47 years old. There were 18 unilateral cases, 9 bilateral cases. The affected eyes were randomly divided into treatment group and control group (n=18 eyes). The treatment group eyes received multi-wavelength krypton yellow laser photocoagulation around the retinoschisis, but no laser spots were laid in a optic-disk area surrounding the macular and optic disc. Children in the control group were followed up every six months without treatment. Both groups of children were followed up for 3 years. The best corrected visual acuity (BCVA, logMAR), complications (vitreous hemorrhage, retinal detachment) were measured at the last follow up. ResultsAt the last follow-up, the treatment group mean logMAR BCVA was 0.73±0.41, which is the same as pre-treatment BCVA (t=1.187, P=0.201). The control group mean logMAR BCVA 0.88 ±0.60, which is the same as pre-treatment BCVA (t=-2.093, P=0.033). The changes of the BCVA in these two groups was statistically different (t=-2.093, P=0.033). For the treated 18 eyes, visual acuity improved in four eyes (22.2%); not changed in 10 eyes (55.6%) and decreased in four eyes (22.2%). For the 18 eyes in the control group, visual acuity improved in three eyes (16.7%); not changed in four eyes (22.2%) and decreased in 11 eyes (61.1%). The vision reduction rate in treatment group was statistically less than the control group (χ2=5.600, P<0.01). There were 2 eyes (11.1%) and 7 eyes (38.9%) with serious complications in the treated and control eyes respectively. The complication rate treatment group was statistically less than the control group (χ2=3.710,P<0.05). ConclusionLaser photocoagulation can stabilize or improve vision of advanced XLRS patients, and prevent the occurrence of serious complications.
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.
ObjectiveTo observe the optical coherence tomography (OCT) features of X-linked juvenile retinoschisis (XLRS) and its relationship and visual acuity. Methods Twenty-three XLRS patients (38 eyes) were enrolled in this study.All patients underwent best corrected visual acuity (BCVA) and frequency-domain optical coherence tomography (SD-OCT) examination. The international standard visual acuity chart was used for BCVA test and the results were then converted into logarithm of the minimum angle of resolution (logMAR) visual acuity. The mean logMAR BCVA of all patients was 0.41±0.28. Topcon 3D-OCT 1000 and Topcon DRI-OCT were applied to detect the retinal layers of retinoschisis. Based on the OCT features, retinoschisis was classified into 3 types, including splitting between outer nuclear layer (ONL) and outer plexiform layer (OPL), inner nuclear layer morphology (INL) splitting, splitting between ganglion cell layer (GCL) and nerve fiber layer (NFL). Based on the OCT features of fovea, there were photoreceptor inner segment (IS)/outer segment (OS) presence group and IS/OS absence group. ResultsThere were 17 eyes (44.7%) with splitting between ONL/OPL, 38 eyes (100%) with INL splitting, 13 eyes (34.2%) with splitting between GCL/NFL. INL splitting can coexist with splitting in other retinal layers. There were 25 eyes (65.8%) with splitting in 2 or more retinal layers. The mean logMAR BCVA were 0.32±0.17 and 0.44±0.22 respectively in the IS/OS absence group and presence group, the difference was statistically significant (t=6.531, P=0.008).The mean fovea thickness (FT) were(517.10±96.92)and(523.08±72.84) μm respectively in the IS/OS absence group and presence group, the difference was not statistically significant (t=2.282, P=0.061). There was no correlation between logMAR BCVA and FT (r=0.200, P=0.929). ConclusionsIn patients with XLRS, the foveomacular schisis involved in multiple retinal layers and most frequently in the INL. The defect of IS/OS is the important reason of the low visual acuity.
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 observe the clinical outcome of minimally invasive vitrectomy for ocular toxocariasis (OT). MethodsThe clinical data of 37 eyes in 37 patients with OT who underwent 23-gauge vitrectomy were retrospectively reviewed. There were 27 male patients and 10 female patients. The age was from 9 months to 22 years, with the mean age of (7.90±4.47) years. Patients who can cooperate underwent non-contact tonometer examination, slit lamp examination, fundus photography, ultrasound examination, optical coherence tomography and ultrasound biomicroscopic examination. The visual acuity was from no light perception to 0.6. According to the clinical type of OT, 18 eyes had posterior granuloma, 13 eyes had peripheral granuloma, 4 eyes had endophthalmitis and 2 eyes had atypical presentation. Seventeen eyes had vitrectomy for tractional retinal detachment (TRD), 13 eyes for epiretinal membrane (ERM), 6 eyes for TRD combined with ERM and 1 eye for endophthalmitis. Twenty-five eyes had lens-sparing vitrectomy, 12 eyes had lensectomy with vitrectomy; five eyes had limbus incision and 32 eyes had pars plana incision. Five eyes (13.51%) had reoperation. Followed up from 6 months to 39 months, with the mean of (13.90±10.25) months. ResultsVision improved in 25 of 35 eyes (71.43%), was unchanged in 9 eyes (25.71%), and was declined in 1 eye (2.86%). Retina was reattached in 22 of 23 eyes that had TRD before surgery (95.65%); the rest one eye had redetachment (4.35%). Three eyes had cataract after surgery, which accounted for 12% in lens-sparing eyes. Among them, 2 eyes had lensectomy therefore. ERM was removed and had no recurrence in 18 of 19 eyes (94.74%). ConclusionMinimally invasive vitrectomy for OT patients can improve their vision and achieve retinal reattachment.
Recently, the new term "lamellar hole-associated epiretinal proliferation" was introduced to describe a specific epiretinal proliferation. Different from conventional epimacular retinal membrane, lamellar hole-associated epiretinal proliferation (LHEP) can be found around the edge of lamellar macular holes and part of full thickness macular holes. It is defined as a thick homogenous layer of yellowish material without any contractive properties on the epiretinal surface with medium reflectivity on optical coherence tomography images. Immunocytochemical analysis showed the presence of glial cells, fibroblasts, hyalocytes and collagen type Ⅱ. Electron microscopy revealed fibroblasts and hyalocytes as predominant cell types, densely packed in cell agglomerations. LHEP is a secondary event in lamellar macular hole formation and may represent a repair process after large and deep retinal defect. Further studies on its clinical features and clinical significance are still required.