OBJECTIVE: To identify patient-reported outcome measures (PROMs) that have been used in children and adolescents with glaucoma and to evaluate their methodologic quality.
CONCLUSIONS: Childhood glaucoma impairs vision and quality of life (QoL) throughout all stages of life. Thus, a PROM needs to cover many different age groups and topics. Various instruments have been used to evaluate patient-reported outcomes (PROs) in patients with childhood glaucoma, however, it is unclear which PROM has the highest methodologic quality and complies best with the needs of patients with childhood glaucoma.
METHODS: A systematic literature review was performed searching MEDLINE (PubMed), the Cochrane Library, Web of Science, and PsycINFO (EBSCO). We included peer-reviewed full-text articles of the past 10 years in English, German, or Spanish language that reported PROMs in children with glaucoma. The study selection and methodologic quality assessment of the identified PROMs was performed by 2 independent reviewers using a 7-point checklist. The content was mapped onto the World Health Organization International Classification of Functioning, Disability and Health. The systematic review was prospectively registered in PROSPERO (ID CRD42022353936).
RESULTS: The search strategy retrieved 3295 matches. A total of 2901 studies were screened, and 11 relevant articles were identified using 10 different instruments. The instruments addressed functional visual ability, vision-related QoL, health-related QoL, and life satisfaction. Six instruments were applicable for the use in children. Seven of the questionnaires received the highest number of positive ratings (5/7). None of the instruments considered the views of patients with childhood glaucoma during their development.
CONCLUSIONS: This systematic review provides a descriptive catalog of vision-specific and generic health PRO instruments that have been used in childhood glaucoma cohorts. An instrument specifically developed for childhood glaucoma is lacking which might result in missing important factors, such as permanent treatment with eye drops, repeated surgeries, and heritability of the disease, when investigating the QoL in children with glaucoma.
BACKGROUND: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

UNASSIGNED: This study assesses the effectiveness and safety of using Ologen implants (Aeon Astron Europe BV, Leiden, The Netherlands) as an adjunctive therapy in childhood glaucoma surgeries.
UNASSIGNED: We systematically reviewed the existing literature across various electronic databases to examine the effectiveness and safety of Ologen implants in childhood glaucoma surgeries.
UNASSIGNED: Our analysis encompassed 14 studies on the use of Ologen implants in childhood glaucoma. Among these, seven were prospective, five were retrospective, and two did not specify their study design. Success rates varied depending on the type of surgery and the included childhood glaucoma subtype. The success rates for Ologen implants-augmented surgeries were as follows: 33.3-70% for trabeculectomy, 50-81% for combined trabeculotomy-trabeculectomy procedure, 33%-87% for glaucoma drainage device, and 60% in deep sclerectomy.
UNASSIGNED: Ologen implant has a potential role in mitigating postoperative fibrosis and enhancing success rates in various childhood glaucoma surgeries. However, the existing literature is limited. Future comparative prospective studies with larger cohorts are needed.

Glaucoma drainage devices (GDD) are increasingly utilized in the management of childhood glaucoma. This systematic review and meta-analysis assesses the efficacy of first-time Ahmed or Baerveldt implantation in children. PubMed, Embase, and Cochrane Library were searched for relevant English-language, peer-reviewed literature. Postoperative outcomes were pooled using random effects regression models with restricted maximum likelihood estimation. Thirty-two studies (1,221 eyes, 885 children) were included. Mean ± standard deviation preoperative IOP was 31.8 ± 3.4 mm Hg. Pooled mean IOP at 12 and 24 months postoperatively were 16.5 mm Hg (95% CI, 15.5-17.6) and 17.6 mm Hg (95% CI, 16.4-18.7), respectively. Pooled proportions of success were 0.87 (95% CI, 0.83-0.91) at 12 months, 0.77 (95% CI, 0.71-0.83) at 24 months, 0.54 (95% CI, 0.44-0.65) at 48 months, 0.60 (95% CI, 0.48-0.71) at 60 months, and 0.37 (95% CI, 0.32-0.42) at 120 months. There were no differences in proportion of success at 12 and 24 months among eyes that received Ahmed and Baerveldt tube shunts, nor between eyes with primary glaucoma, glaucoma following cataract surgery, or other secondary glaucoma. Our findings show that Ahmed and Baerveldt shunts substantially reduced IOP for at least 24 months in childhood glaucoma, with similar findings among device types and glaucoma etiologies.

Childhood glaucoma represents a heterogenous group of rare ocular conditions that may result in significant sight threatening complications related to elevated intraocular pressure (IOP). It can be classified as either primary or secondary and the latter may have systemic associations. This review will be based on the work of the childhood glaucoma research network (CGRN) and will focus on the diagnosis and management of the most common types of childhood glaucoma. These include primary congenital glaucoma (PCG) and juvenile open angle glaucoma (JOAG) as well as secondary causes of glaucoma associated with non-acquired ocular anomalies (Axenfeld-Rieger anomaly; Peters anomaly and Aniridia), glaucoma associated with systemic disease (Sturge Weber syndrome and Neurofibromatosis), those due to acquired conditions (Uveitic glaucoma, trauma and tumours) and importantly glaucoma following cataract surgery.

OBJECTIVE: Primary congenital glaucoma (PCG) is a form of childhood glaucoma caused by maldevelopment of the anterior chamber. Disease severity differs greatly amongst patients. Ultrasound biomicroscopy (UBM) is a non-invasive technique that can visualize the anterior segment in infants in vivo. The purpose of this narrative review is to make an overview of the UBM data in PCG and study the applicability of UBM in characterizing the disease.
METHODS: An online search was performed on PubMed in December 2020. After a critical appraisal of the included articles, study and patient characteristics were summarized. The UBM measurements of the anterior segment in PCG of the different studies were analysed.
RESULTS: Six studies were included in this review. All were cross-sectional prospective studies. A total of 221 PCG eyes were examined. PCG eyes showed a larger trabecular iris angle, decreased iris thickness, narrower or absent Schlemm\'s canal and an increased zonular length compared to controls. Abnormal tissue membrane covering the trabecular meshwork and abnormal insertion of the iris and ciliary process were frequently found. The success rate of glaucoma surgery depended on the severity of anterior segment malformations found with UBM.
CONCLUSIONS: Malformations of the anterior segment in PCG can be demonstrated by UBM in vivo. This imaging can help to characterize disease severity and might support surgical treatment decisions.

Objectives: Retinal nerve fiber layer (RNFL) thickness has been detected by numerous studies about alterations and abnormalities in childhood glaucoma, but these studies have yielded inconsistent results about the RNFL thinning region. The investigation of characteristics of RNFL in pediatric patients would contribute to the deep understanding of the neuropathic mechanisms of childhood glaucoma. Thus, the degree of thinning in different quadrants deserves further discussion and exploration. Method: A systematic literature search was conducted using the Cochrane Central Register of Controlled Trials, Medline, Embase, and PubMed databases to identify clinical studies published from inception to April 1, 2021. Results: Ten studies were included in this review with a total of 311 children with glaucoma and 444 in nonglaucomatous controls. The results revealed that average peripapillary RNFL (pRNFL) thickness was attenuated in pediatric patients with glaucoma [weighted mean difference (WMD) = -20.75; 95% CI -27.49 to -14.01; p < 0.00001]. Additionally, pRNFL thickness in eight quadrants (superior, inferior, temporal, nasal, superotemporal, inferotemporal, superonasal, and inferonasal) had different levels of reduction in the pediatric group of glaucoma. Conclusion: This study indicates that eight regions of RNFL thickness show various degrees of thinning in childhood glaucoma. However, caution is required in the interpretation of results due to marked heterogeneity. Future studies, especially larger samples and multicenter, need to confirm our results.

UNASSIGNED: The dexamethasone (DEX) implant is an FDA approved treatment for diabetic macular edema, non-infectious posterior uveitis, and macular edema secondary to branch or central retinal vein occlusions. We describe a case of anterior chamber (AC) migration of a DEX implant in a patient with a history of congenital glaucoma and perform a review of the literature on this particular complication, summarizing the common risk factors, subsequent complications, and management options.
UNASSIGNED: A 46-year-old female with a history of congenital glaucoma, status post cataract extraction with insertion of intraocular lens, pars plana vitrectomy, and Baerveldt tube implant in the left eye was referred for post-operative cystoid macular edema (CME). The patient underwent insertion of a DEX implant, resulting in improvement in her CME. After the fourth implant was injected, the patient noticed a white line in her eye while looking in the mirror after doing jumping jacks. Slit lamp examination confirmed migration of the implant into the AC. Ultimately, the patient was taken to the operating room, where her implant was removed via bimanual vitrectomy through an anterior approach.
UNASSIGNED: This case report and literature review explores the ophthalmic structural changes specific to congenital glaucoma which may have predisposed this eye to anterior migration of the DEX implant. The purpose of this review is to detail the anatomic changes that may increase the risk of anterior chamber implant migration in patients with congenital glaucoma so that physicians may be aware of these risks when selecting patients for this implant.

Despite being documented in medical history from over 2400 years ago, primary congenital glaucoma (PCG), being a disease with low incidence rate, remains a challenge to ophthalmologists. The article provides a broad overview on the pathophysiology and diagnostic approach to PCG with major emphasis on the treatment options of PCG. While reviewing on the well-established treatment options, namely goniotomy, trabeculo-tomy and combined trabeculotomy-trabeculectomy, emphasis has also been made to recent updates on secondary treatments: trabeculectomy, antimetabolites, glaucoma-drainage devices and cyclodestructive procedures. It is, however, important to note that the rarity of PCG places limitations on study design, most studies are, thus, retrospective, nonrandomized and have different definitions of surgical success. Ophthalmologists need to interpret the results with critical thinking and formulate individual treatment plans for each patient. How to cite this article: Yu Chan JY, Choy BNK, Alex LK Ng, Shum JWH. Review on the Management of Primary Congenital Glaucoma. J Curr Glaucoma Pract 2015;9(3):92-99.