This paper reports a retrospective consecutive case series investigating the efficacy of Mitomycin C-augmented trabeculectomy in the treatment of primary and secondary pediatric glaucoma in a mixed ...etiology, multiethnic cohort of patients.
To evaluate the long-term efficacy and safety of Mitomycin C-augmented trabeculectomy in a mixed, tertiary-referral, pediatric glaucoma cohort.
Retrospective consecutive review of all children (37 eyes) undergoing Mitomycin C-augmented trabeculectomy by a single surgeon between 2008 and 2016. Seventeen eyes (45.9%) had primary congenital glaucoma, and 20 eyes (54.1%) had secondary glaucoma. The median age at surgery was 11 months (range, 2 to 146). The mean follow-up was 69.2±4.7 months (range, 3.5 to 107.9).
Overall, trabeculectomy was successful in 80.6% of eyes at 12 months, 60.5% at 3 years, and 57.5% at 5 years. 45.9% cases (17 eyes) required further laser or surgery for uncontrolled intraocular pressure (IOP) and were therefore deemed as failures. The time to failure ranged from 0.4 to 65.1 months (mean, 22.2±5.1 mo). The proportion of children achieving visual acuity of 1.0 LogMAR equivalent or better increased from 43.2% preoperatively to 63.6% at 1 year and 68% at 5 years. The mean IOP reduced from 24.85±0.88 mm Hg preoperatively to 15.14±0.94 mm Hg at 3 months (39% reduction) and 17.42±1.08 mm Hg at 5 years (30% reduction). IOP-lowering medication requirement reduced from 4.14±0.20 agents preoperatively to 0.84±0.22 at 3 months (80% reduction) and 1.78±0.36 at 5 years (57% reduction). There were no sight-threatening complications such as hyphaema, bleb leak, chronic hypotony, endophthalmitis, retinal detachment, or loss of light perception.
This study provides valuable evidence that Mitomycin C-augmented trabeculectomy is safe and effective as a treatment of primary or secondary pediatric glaucoma, with particularly encouraging results in cases of secondary glaucoma. Trabeculectomy offers the potential for delaying or avoiding glaucoma drainage device surgery in a significant proportion of children.
PURPOSEToxic epidermal necrolysis (TEN) is a devastating form of Stevens-Johnson syndrome (SJS) with acute and chronic ocular complications. We present a novel aggressive combination strategy, termed ...“Triple-TEN”, for the management of acute ocular TEN designed to minimize the risk of chronic, blinding sequelae.
METHODSTwo patients with life-threatening TEN accompanied by severe ocular surface defects and fulminant symblephara formation underwent “Triple-TEN” management of their acute ocular disease under aseptic techniques in the critical care setting, after failed treatment with intensive topical therapy and surgical division of symblephara. The Triple-TEN protocol comprises (1) subconjunctival triamcinolone (Kenalog 20 mg) administered into each of the fornices to curb the local inflammatory response without compromising systemic immunity, (2) placement of amniotic membrane tissue mounted on a polycarbonate skirt (ProKera) over the corneal and limbal regions to facilitate reepithelialization of the ocular surface, and (3) insertion of a steeply curved acrylic scleral shell spacer (Technovent, SC21) to vault the lids away from the globe providing a barrier to symblephara formation.
RESULTSIn both cases, ocular surface inflammation resolved within 4 weeks with no progression of conjunctival cicatrization or evidence of limbal epithelial stem cell failure at 1 year follow-up. There were no long-term complications of the Triple-TEN regimen.
CONCLUSIONSAggressive treatment with the Triple-TEN protocol for acute ocular TEN resistant to first-line therapy, may help prevent long-term blinding sequelae.
Abstract Retinoblastoma is the commonest primary ocular malignancy of childhood. There are two forms — heritable and non heritable. Heritable retinoblastoma is a cancer susceptibility syndrome. ...Presentation is in the first few years of life, sometimes in the neonatal period. Early detection and prompt treatment can give cure rates up to 95% for intraocular tumours, but extraocular disease carries a very high mortality. The diagnosis is essentially clinical and biopsy is contraindicated due to the risk of extraocular spread. Treatment requires significant multidisciplinary input, with local ophthalmic treatment, systemic chemotherapy and external beam or plaque radiotherapy, or surgery to remove the affected eye. Screening of family members is essential for early detection. Lifelong surveillance of mutation carriers is needed due to the risk of second cancers. Newer treatment modalities including intra-arterial chemotherapy have been added to the therapeutic armamentarium in recent years.
The identification of somatic
variation is crucial to confirm the heritability of retinoblastoma. We and others have previously shown that, when tumour DNA is unavailable, cell-free DNA (cfDNA) ...derived from aqueous humour (AH) can be used to identify somatic
pathogenic variation. Here we report
pathogenic variant detection, as well as cfDNA concentration in an extended cohort of 75 AH samples from 68 patients. We show cfDNA concentration is highly variable and significantly correlated with the collection point of the AH. Cell-free DNA concentrations above 5 pg/µL enabled the detection of 93% of known or expected
pathogenic variants. In AH samples collected during intravitreal chemotherapy treatment (Tx), the yield of cfDNA above 5 pg/µL and subsequent variant detection was low (≤46%). However, AH collected by an anterior chamber tap after one to three cycles of primary chemotherapy (Dx1+) enabled the detection of 75% of expected pathogenic variants. Further limiting our analysis to Dx1+ samples taken after ≤2 cycles (Dx ≤ 2) provided measurable levels of cfDNA in all cases, and a subsequent variant detection rate of 95%. Early AH sampling is therefore likely to be important in maximising cfDNA concentration and the subsequent detection of somatic
pathogenic variants in retinoblastoma patients undergoing conservative treatment.
Secondary enucleation (SE) puts an irreversible end to eye-preserving therapies, whenever their prolongation is expected to violate the presumed state of metastatic grace. At present, it must be ...acknowledged that clear criteria for SE are missing, leading to empiric and subjective indications commonly related to disease progression or relapse, disease persistence masking the optic nerve head or treatment-related complications obscuring the fundus view. This absence of evidence-based consensus regarding SE is explained by the continuously moving frontiers of the conservative management as a result of diagnostic and therapeutic advances, as well as by the lack of studies sufficiently powered to accurately stratify the risk of metastasis in conservatively treated patients. In this position paper of the European Retinoblastoma Group (EURbG), we give an overview of the progressive shift in the indications for SE over the past decades and propose guidelines to assist decision-making with respect to when SE becomes imperative or recommended, with corresponding absolute and relative SE indications. Further studies and validation of biologic markers correlated with the risk of metastasis are expected to set more precisely the frontiers of conservative management and thus consensual criteria for SE in the future.
Dilated fundus examinations are a vital, yet time-consuming and sometimes distressing part of paediatric ophthalmology examinations. Limited resources, personnel and time can result in prolonged ...waiting time and increase risk from delayed diagnosis and treatment. Using a Nikon D80 TopCon TRC-NW6S non-mydriatic fundus camera (TopCon (GB) Ltd, Newbury), we aimed to demonstrate the safety and efficacy of orthoptic-led fundus digital imaging and the potential time and cost benefits to the healthcare system.
We conducted a retrospective review of all digital fundus images taken over a six month period in 2012 (n = 616, age range 2.1-16.5 years, mean age 8.7 years).
Overall success rate for paediatric fundus digital imaging was 97%. Successful images were achieved in 87% of patients without the need for pupil dilation. Images were taken for a variety of clinical reasons. 45% of patients were discharged immediately, many with copies of photographs to facilitate follow-up with community optometrists.
Orthoptic-led fundus digital imaging is an innovative, speedy, safe and efficient method of documenting fundal appearance, enabling serial documentation of stability/progression of ocular disease. It allows adequate examination of routine patients, freeing up time within busy clinics. Paediatric fundus digital imaging brings a potential positive cost benefit to healthcare systems under pressure, and facilitates skill development for allied health professionals.
PDF
