Search

Purpose: To assess the management of patients with congenital nasolacrimal duct obstruction (CNLDO) in a paediatric population and review the long-term outcomes over a 5-year interval, with particular emphasis on the difference between patients with trisomy 21 and those without trisomy 21., Methods: This single-centre, retrospective, cross-sectional, case review study included patients suffering from CNLDO at Alder Hey Children's Hospital NHS foundation Trust. Patients were divided into two groups: Non-trisomy 21 and trisomy 21. Patients were followed-up for a 60-month interval. Patients aged <12 months at the time of surgery, patients with <60 months of follow-up data and patients with acquired nasolacrimal duct obstruction were excluded. The main outcome measures were discharge rates in patients undertaking primary intervention with syringe and probe (S&P), number of patients requiring further treatment with lacrimal intubation or dacryocystorhinostomy (DCR) and overall symptom-free periods post-treatment., Results: Ninety-three patients (142 eyes) were included. The mean number of surgical interventions was 1.53 ± 0.65. The mean interval between the 1st and 2nd intervention was 15.54 ± 16.33 months. There was a trend towards greater success rates non-trisomy 21 patients versus patients with trisomy 21 ( p  = 0.1352). The average symptom-free period after the final intervention was 44.31 ± 20.68 months, significantly longer in the non-trisomy 21 group compared to the trisomy 21 group ( p  = 0.0074)., Conclusions: The overall success rate after primary S&P was 55.9%. Our results suggest that in trisomy 21 patients suffering from CNLDO, a one-stage intervention with primary monocanalicular intubation should be considered instead of sequential approach.

A male patient in his early childhood presented to rheumatology with a hoarse voice and recurrent oral and cutaneous ulceration. Serological investigation revealed persistently elevated inflammatory markers. Despite compliance to treatment, flare-ups persisted, prompting the use of further treatment. An airway endoscopy revealed cystic changes to the left vocal cord. Referral to ophthalmology revealed multiple, waxy, skin-coloured, beaded papules on thickened, irregular eyelid margins with distichiasis, in keeping with moniliform blepharosis. Enrolment into the 100 000-genome project helped clinch the diagnosis of lipoid proteinosis. Although this case highlights the diagnostic power of genetics, it also sheds light on the importance of targeted clinical referral. When one considers the typical symptoms and signs of lipoid proteinosis, referral to a centre of rare diseases would have proven effective in not only avoiding polypharmacy but also reducing the psychological burden of several years of uncertainty must have had on our patient., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Aim: To describe the changes in corneal graft thickness following ultrathin Descemet's Stripping Automated Endothelial Keratoplasty (UT-DSAEK) comparing pre- and postoperative values over a 24-month period., Methods: In this retrospective single-center case series, patients who received eye bank-prepared tissues for UT-DSAEK surgery were included. Preoperative and postoperative graft thickness measurements were determined in the eye bank and in clinic using anterior segment optical coherence tomography (AS-OCT) images. Graft thickness measurements and their percentage change between preoperative values and values at 1 day, 1 week and 1, 6, 12, 24 months were calculated., Results: In total, 47 eyes of 47 patients with a mean age of 69 ± 11 years (29 males) were included. Twnty-three patients had Fuchs' endothelial dystrophy (49%) and the remaining 24 had pseudophakic bullous keratopathy (51%). In total, 29/47 eyes underwent UT-DSAEK alone (62%) and 18/47 received combined cataract surgery as a triple procedure (38%). Preoperative donor graft thickness was 92 ± 28 μm. Compared to preoperative values, where graft thickness increased to 194 ± 101.3 μm at 1 day, 151.1 ± 71.4 μm at 1 week, and 108.4 ± 52.5 μm at 1 month. Graft thickness continued to gradually decrease over time until 6 months (91.7 ± 33.6 μm), and then plateaued at 12 months (83.9 ± 25.0 μm), showing minimal changes at 2 years (101.4 ± 37.5 μm)., Conclusion: Preoperative DSAEK graft thickness measurements as reported by the eye bank are a valid approximation of DSAEK graft thickness at 6 months after surgery and these measurements tend to stabilize over time up to 2 years after surgery., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Objective: To compare clinical outcomes and complications between pre-loaded ultra-thin Descemet stripping automated endothelialkeratoplasty (pl-UT-DSAEK) and pre-loaded Descemet membrane endothelial keratoplasty (pl-DMEK)., Methods and Analysis: Comparative study in patients with endothelial dysfunction associated with Fuchs endothelial corneal dystrophy and pseudophakic bullous keratopathy who underwent pl-UT-DSAEK or pl-DMEK transplants. For both groups, the tissues were pre-loaded at the Fondazione Banca degli Occhi del Veneto (Venice, Italy) and shipped to The Royal Liverpool University Hospital (Liverpool, UK). Best corrected visual acuity (BCVA) and re-bubbling rates were the main outcome measures., Results: 56 eyes of 56 patients were included. 31 received pl-UT-DSAEK and 25 received pl-DMEK. At 12 months, BCVA (LogMAR) was significantly better for pl-DMEK (0.17±0.20 LogMAR) compared with pl-UT-DSAEK (0.37±0.37 LogMAR, p<0.01). The percentage of people that achieved ≥20/30 was significantly higher in the pl-DMEK group. The rate of re-bubbling, however, was significantly higher for pl-DMEK (44.0%) than for Pl-UT-DSAEK (12.9%), p<0.01., Conclusion: Pl-DMEK offers better BCVA than pl-UT-DSAEK. The higher re-bubbling rate associated with pre-loaded DMEK is of concern., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ.)