More than 90% of patients with diffuse intrinsic pontine glioma (DIPG) will die within 2 years of diagnosis. Patients deteriorate rapidly during the disease course, which severely impairs their quality of life. To date, no specific research on this clinically important subject has been conducted. This study aimed to compile an inventory of symptoms experienced, interventions applied, and current service provision in end-of-life care for DIPG. We performed a retrospective cohort study of children with DIPG, aged 0–18 years, who received treatment under the care of 2 London hospitals. Symptoms, interventions, and services applied during the 12 weeks before death were analyzed. In addition, we conducted a global questionnaire-study among health care professionals. In more than 78% of DIPG patients, problems concerning mobility, swallowing, communication, consciousness, and breathing arose during end-stage disease. Supportive drugs were widely prescribed. The use of medical aids was only documented in <15% of patients. Palliative and end-of-life care was mostly based on the health care professional's experience; only 21% of the questionnaire respondents reported to have a disease-specific palliative care guideline available. This research assessed the current state of palliative and end-of-life care for children with DIPG. Our results show the variability and complexity of symptoms at end-stage disease and the current lack of disease-specific guidelines for this vulnerable group of patients. This first descriptive paper is intended to act as a solid basis for developing an international clinical trial and subsequent guideline to support high-quality palliative and end-of-life care.

Juvenile idiopathic arthritis (JIA) is the most common cause of uveitis in children. While symptoms are usually mild, persistent eye inflammation could lead to severe complications and impaired vision. It is essential that JIA patients at risk are diagnosed with uveitis early, receive adequate treatment, and avoid developing complications, such as cataract, glaucoma, and amblyopia. The purpose of this mini-review is to summarize the screening strategies and clinical management for JIA-associated uveitis (JIA-U) as well as the current state of molecular markers linked to this condition. Because glaucoma is one of the most common causes of visual loss in JIA-U, special focus will be put on this serious complication. We conclude by describing the current evidence regarding the long-standing question of whether chronic anterior uveitis without arthritis may be the same disease entity as JIA-U.

Purpose: To compare the outcomes and complications of different surgical interventions for secondary glaucoma in pediatric uveitis. Methods: Systematic review following the PRISMA standards. Main inclusion criteria were surgery for secondary glaucoma in pediatric uveitis at a mean age of 16 years or below, a mean follow-up period of at least 1 year after surgery, and at least 10 eyes per surgical intervention per study. We used the GRADE approach to assess study quality. Primary outcomes were intraocular pressure (IOP) and number of IOP lowering medications before and after surgery. Secondary outcomes were success rate and complications. Results: Fourteen studies fulfilled the inclusion criteria, in which one (n = 11) or more (n = 3) surgical interventions were described, comprising in total six different procedures. According to the GRADE criteria, the quality of the studies was low to very low, in particular because of the small size and the applied study designs. All surgical interventions provided a significant decrease in IOP and number of IOP lowering medications. The success rates during follow-up varied widely, with the lowest rates of success after cyclophotocoagulation. The most frequently reported complications were ocular hypertension, hypotony, and hyphema, with an indication for a reoperation in more than one-third of the cases. Permanent vision loss was infrequently seen and was attributed to prolonged hypotony. Conclusions: The described surgical interventions are able to prevent blindness by lowering a medically uncontrolled IOP to an acceptable level. Therefore, there is a crucial role for surgical intervention in these children. Based on the present studies, no preferences can be made. Given the reported complications, more research with larger sample sizes and direct comparisons is needed to determine the most successful glaucoma treatment in children with uveitis.

Secondary glaucoma is still a blinding complication in childhood uveitis, for which most commonly used surgical interventions (trabeculectomy or glaucoma drainage implant) involve multiple re-interventions and/or complications postoperatively. The goniotomy procedure has never been investigated in the current era, in which patients with pediatric uveitis receive biologics as immunosuppressive therapy for a prolonged period, with potential implications for the outcome. The purpose of the study is to evaluate the efficacy and safety of a goniotomy procedure in pediatric non-infectious uveitis in a retrospective, multicenter case series. The primary outcomes were the postoperative intraocular pressure (IOP), number of IOP-lowering medications, and success rate. Postoperative success was defined as 6 ≤ IOP ≤ 21 mmHg, without major complications or re-interventions. Fifteen eyes of ten children were included. Median age of the included patients at goniotomy was 7 years; median follow-up was 59 months. Median (interquartile range) IOP before surgery was 30 (26–34) mmHg with 4 (3–4) IOP-lowering medications. At 1, 2, and 5 years after goniotomy, median IOP was 15, 14, and 15 mmHg with 2 (0–2), 1 (0–2), and 0 (0–2) medications, respectively (p < 0.001 postoperatively versus preoperatively for all timepoints). Success rate was 100%, 93%, and 80% after 1, 2, and 5 years, respectively. There were no significant changes in visual acuity and uveitis activity or its treatment, and there were no major complications. Our results show that the goniotomy is an effective and safe surgery for children with uveitic glaucoma.

Uveitic glaucoma (UG) is a complex disease with nearly three times higher risk for severe visual loss than uveitis without glaucoma. Monitoring and management vary among ophthalmologists, making it difficult to identify optimal follow-up and treatment approaches. The Uveitic Glaucoma Interest Group (UGIG) proposes guidelines to reduce variation in practice through best practice management guidelines for uveitis-related ocular hypertension (UOHT) and UG. A group of ophthalmologists with expertise in uveitis and glaucoma conducted an extensive literature review of UOHT and UG. Following review and discussion generated recommendations to standardize best management practices. The recommendations cover: 1) glaucoma-related testing and timing, 2) intraocular pressure (IOP) monitoring and goals, 3) follow-up after starting corticosteroids (CS) or IOP-lowering agents, 4) timing of referral to a glaucoma specialist, 5) management considerations and guidelines. Uveitic eyes have a heightened risk of developing ocular hypertension (OHT) and glaucoma. IOP is often very high and highly variable, associated with rapid progression rates from OHT to glaucoma, and with more rapid visual field loss. Glaucoma can also develop at lower IOP as compared to non-uveitic patients. The UGIG proposes recommendations to standardize the care of UG patients, facilitating more effective management and optimizing visual outcomes. A consensus is presented regarding IOP management based on a more stringent IOP threshold of 16 mmHg. When possible, a close collaboration between a glaucoma specialist and the uveitis expert is preferred. Appropriate referral to glaucoma specialist should be made early, ideally at the time IOP-lowering therapy is initiated.