Unique Roberts syndrome with bilateral congenital glaucoma: A case report

BACKGROUND Congenital glaucoma associated with Roberts syndrome(RS)is an unusual and unique condition.No previous report describes this association.A multidisciplinary approach including molecular studies were conducted to reach the final diagnosis.CASE SUMMARY We present a rare case of a 1-wk-old male with RS associated with bilateral congenital glaucoma,left ectopic kidney,and left-hand rudimentary digits.A comprehensive approach was applied by which bilateral non-penetrating glaucoma surgery was performed with good control of intraocular pressure for more than 6 mo.Cytogenetic and molecular testing were conducted and revealed normal measurements.CONCLUSION This report described a case of a male baby with clinical features of RS but with a negative molecular analysis,presenting with left-hand rudimentary digits,bilateral congenital glaucoma,and left ectopic kidney.To the best of our knowledge,this is the first case reported with phocomelia,bilateral congenital glaucoma,and unilateral ectopic kidney.

Comparison of ocular axis and corneal diameter between entropion and non-entropion eyes in children with congenital glaucoma

BACKGROUND Children with congenital glaucoma are often accompanied by acquired epiblepharon in the lower eyelid,which causes entropion of the lower eyelid and damages the cornea.AIM To infer the possible causes of lower eyelid entropion by comparing the difference of ocular axis and corneal diameter between inverted and non-inverted ciliary eyes in children with congenital glaucoma.METHODS A total of 15 patients(11 males and 4 females)diagnosed with congenital glaucoma between July 2016 and January 2019 at Tongren Hospital were included.Five patients had bilateral glaucoma,and ten had unilateral glaucoma.Each patient had only one eye with lower eyelid entropion which is associated with congenital glaucoma.All the patients had no entropion in another eye.The clinical data were collected.Main outcome measures were the ocular axis and corneal diameter.RESULTS The average age of the 15 patients was 1.85±0.49 years.Paired t-test showed that the average ocular axis of congenital glaucoma eyes with lower eyelid entropion(24.86±3.44 mm)was significantly longer than that of congenital glaucoma eyes without lower eyelid entropion(20.79±1.34 mm;P<0.001).The average corneal diameter of congenital glaucoma eyes with lower eyelid entropion(13.61±0.88 mm)was also significantly greater than that of congenital glaucoma eyes without lower eyelid entropion(11.63±0.48;P<0.001).CONCLUSION The rapid growth of the ocular axis and corneal diameter may be the main cause of congenital glaucoma with acquired lower eyelid entropion.Therefore,children with poor control of intraocular pressure and excessive growth of ocular axis and corneal diameter must be observed for the existence of acquired epiblepharon.

Trabeculectomy with Ologen implant versus mitomycin C in congenital glaucoma secondary to Sturge Weber Syndrome

AIM： To compare the efficacy and safety of collagen matrix implant [Ologen（OLO） implant] versus mitomycin C（MMC） with subscleral trabeculectomy（SST） for the surgical treatment of congenital glaucoma（CG） in SturgeWeber Syndrome（SWS）.METHODS： A prospective comparative randomized study of 20 eyes of 16 patients with CG associated with SWS was divided into two groups. The first group（MMC Group） included 10 eyes that were subjected to SST with MMC. The second group（OLO Group） included 10 eyes that were subjected to trabeculectomy with a collagen matrix implant（OLO implant）. Postoperative evaluation included intraocular pressure（IOP） level, bleb evaluation, complications, and the need for further medication or surgical intervention. RESULTS： The mean preoperative IOP was 29±3.16 mm Hg in MMC and 29.8±3.08 mm Hg in OLO eyes. Mean 12-month percentage reduction in IOP was significant in both groups（57.9% and 56.3%）. At the end of the 12 postoperative follow-up month, in the MMC Group, 80% of eyes achieved the complete success, 20% of eyes had qualified success with no failed surgery in comparison to OLO Group which 70% of eyes achieved the complete success, 20% of eyes had qualified success with 10% failed surgery. In terms of complications, the MMC Group had a higher rate of complications than the OLO Group in the form of thin polycystic bleb in 6 eyes（60%）, blebitis in only one eye（10%） treated with topical antibiotics, shallow anterior chamber in two eyes（20%）.CONCLUSION： This study proves that the use of a collagen matrix implant yields equally effective results as MMC when combined with trabeculectomy for the treatment of CG in SWS. Furthermore, OLO implantation is safe and has low incidences of complications.

Therapeutic effect analysis on the treatment of congenital glaucoma through modified combined trabeculotomy-trabeculectomy

AIM： To evaluate the therapeutic effect and the safety of the treatment of congenital glaucoma through modified combined trabeculotomy-trabeculectomy. METHODS： The clinical data of 27 cases （altogether 42 eyes）, which included 7 cases of infants （10 eyes） and 20 cases of teenagers （32 eyes）, of congenital glaucoma undertook modified combined trabeculotomy trabeculectomy were analyzed retrospectively. The parameters evaluated included the post operation visual acuity, the anterior chamber, the filtering bleb, the intraocular pressure, the C/D ratio, visual field, the retinal nerve fiber layer changes and the complications. RESULTS： The follow-up period was 1 to 29mo, averaging 13.3 ±7.7mo. Upon the last visit after the operation, functional filtering blebs developed in all the involved eyes. The intraocular pressure was controlled under 21 mm Hg, which was decreased by 60% when compared with that before the operation, without using any medication. There were no significant changes in the post operation visual acuity and the retinal nerve fiber layer thickness before and after the operation in teenager group （P〉0.05）, and both the post operation C/D ratio and the visual field mean defect （MD） were reduced compared with those before the operation （P〈0.05）. There were no severe complications in any of the patients. CONCLUSION： The modified combined trabeculotomy- trabeculectomy can effectively reduce the intraocular pressure and control the development of glaucoma in cases of congenital glaucoma. It is a safe and effective operative method for the treatment of congenital glaucoma

Clinical effect of improved viscocanalostomy for the treatment of primary congenital glaucoma

AIM: To evaluate the clinical effect of improved viscocanalostomy in patients with primary congenital glaucoma. METHODS: Retrospective analysis of improved viscocanalostomy was performed on 51 eyes of 42 patients with primary congenital glaucoma. The outcome evaluation included postoperative intraocular pressure (IOP), corneal diameter, cup/disc ratio and complications. All patients were followed up at week 1, month 1, 3, 6 and 12. RESULTS: The results revealed that postoperative IOP was decreased from (38.57 +/- 13.61)mmHg to (10.53 +/- 3.91)mmHg. (14.89 +/- 5.26)mmHg,(15.42 +/- 5.11)mmHg, (13.82 +/- 3.46)mmHg, (13.16 +/- 5.29)mmHg at follow-up time of 1 week,1, 3, 6, 12 months (P<0.001). The postoperative corneal diameter was decreased significantly (P=0.002); The mean cup/dish ratio wasn't significantly different (P=0.148) before and after the surgery, the cup/dish ratio of successful surgery was evidently decreased (preoperative 0.7 +/- 0.2, postoperative 0.6 +/- 0.3, P = 0.007), but the complications like unformed anterior chamber were not observed. The mean follow-up period was 12 months. CONCUJSION: Improved viscocanalostomy improves the clinical effects of the patients with primary congenital glaucoma, such as higher success rates, lower postoperative mean IOP and fewer complications.

Ultrastructural Observations of The Anterior Chamber Angle Tissues in Congenital Glaucoma

Eighteen trabeculectomy specimens of congenital glaucoma were examined by light and transimssion electron microscopy. The results showed that the primary anomalies in congenital glaucoma included the developmental defects of trabecular meshwork, excessive collagen fibrils in the trabecular matrix, shifting forward of ciliary muscle fibres and persistent mesenchymal tissues in the anterior-chamber angle. The authors also pointed out the importance of the secondary lesions of the trabecular meshwork in th...

Seven-year follow-up of spontaneously resolved primary congenital glaucoma:a case report

Background:Spontaneously resolved primary congenital glaucoma is rare and the mechanism remains unknown.Previous literature described the phenomenon of spontaneous resolution of primary congenital glaucoma,with no further reports on follow-up visits and visual development of patients.We report a case of unilateral spontaneously resolved primary congenital glaucoma at a 7-year follow-up visit and describe the differential development of axial length(AL)between affected eye and healthy eye.Case Description:A 6-year-old boy firstly presented at Zhongshan Ophthalmic Center with expanded corneas and ruptures in Descemet’s membranes(Haab’s striae)and apparently thin retinal nerve fiber layer(RNFL)in the left eye(LE),but normal intraocular pressure(IOP)of both eyes without anti-glaucoma medications or surgeries.At 7-year follow-up,the IOPs of bilateral eyes were stable ranging from 8 to 11 mmHg.A cup to disc ratio and the RNFL was stable in the LE at the following visit.The AL increased almost 3 mm in the right eye(RE)but 1.5 mm in the LE.Without anti-glaucoma medications or surgeries,the primary congenital glaucoma was spontaneously resolved.Conclusions:It may figure out the development and influence of the affected eye of the patient,including AL and refraction state with regular ophthalmic examination at periodic follow-up.

Modified trabeculotomy for primary congenital glaucoma

In traditional trabeculotomy,a radial incision is performed at the corneoscleral transition zone to locate the Schlemm＇s canal.However,in some conditions,finding the Schlemm＇s canal is difficult because of the enlargement of the comea,developmental anomalies of the angle,etc.In our study,we performed a modified trabeculotomy,to locate the Schlemm＇s canal easily and accurately and evaluated the outcomes.

A retrospective and consecutive study of viscocanalostomy versus trabeculectomy for primary congenital glaucoma

Background Surgical interventions are the main treatment for primary congenital glaucoma （PCG）. This study aimed to compare the efficacy and safety between viscocanalostomy and mitomycin C （MMC）-trabeculectomy in patients with PCG. Methods A total number of 43 patients with PCG who underwent either viscocanalostomy （group 1） or MMC- trabeculectomy （group 2） between June 2003 and June 2008 were retrospectively reviewed. The patients＇ intraocular pressures （lOPs） were examined before surgery and on day 1, week 1, month 1, month 6, and month 12 post-operative. Mean horizontal corneal diameters, success rates, intra- and post-operative complications were compared between the two groups. Results Pre-operative lOPs were （31.96±3.90） mmHg in group 1 and （32.56±4.00） mmHg in group 2. At the last visit, lOPs were （16.78±2.20） mmHg and （15.77±2.60） mmHg, respectively （P 〈0.001）; the complete success rates of group 1 and group 2 were 45.9% and 67.4%, respectively, and the difference was not statistically significant （P=0.158）. There were no major complications occurred in the two groups. Conclusions Both viscocanalostomy and trabeculectomy can lower lOP in PCG patients effectively. Although there was no major complications occurred in both groups, viscocanlostomy may decrease the probability of postoperative haemorrhage, hypotony, cataract, or choroid effusion.

A case of 9p deletion syndrome with congenital infantile glaucoma,effective method of diagnosis,and treatment

Dear Editor,I am Dr.Jia X from the Department of Ophthalmology,Second Xiangya Hospital,Central South University,Changsha,China.I write to present a rare case report of 9p deletion syndrome with congenital infantile glaucoma in an infant,accompanying with an effective method of both diagnosis and treatment.

Profile of pediatric glaucoma patients in Shanghai Eye, Ear, Nose and Throat Hospital

Background The extent of our knowledge of the epidemiology of pediatric glaucoma in China is limited. To better characterize the epidemiology of pediatric glaucoma in eastern China, we report the clinical profile, etiologies, and treatment modalities in patients 〈18 years of age in Shanghai Eye, Ear, Nose and Throat Hospital. Methods The medical records of patients presenting glaucoma between January 2003 and December 2010 were retrospectively reviewed. The demographic characteristics, the proportion of different glaucoma subtypes and surgical precedures were collected and analyzed. Results A total of 1 142 eyes of 734 pediatric patients （500 males） were included. Congenital glaucoma was the leading subtype, accounting for 47.55% of all patients. The ratio of boys to girls was 2.5：1. Patients with congenital glaucoma affecting both eyes accounted for 72.5% of all patients examined. Patients with primary juvenile glaucoma were the second most common group （n=125, 17.03%）. Traumatic glaucoma was the third most common subtype （n=81, 11.03%）. The type of surgery was related to the subtype of glaucoma. Conclusions Congenital glaucoma, primary juvenile glaucoma, and traumatic glaucoma are the most prevalent subtypes in pediatric glaucoma patients in Shanghai Eye, Ear, Nose and Throat Hospital. The characteristics of congenital glaucoma in China are similar to those in Western countries.