National UK guidelines for the management of paediatric craniopharyngioma.

Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic-pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidity against a background of high overall survival. To date, the optimal management strategy for these tumours remains undefined, with practice varying between centres. In light of these discrepancies, as part of a national endeavour to create evidence-based and consensus-based guidance for the management of rare paediatric endocrine tumours in the UK, we aimed to develop guidelines, which are presented in this Review. These guidelines were developed under the auspices of the UK Children's Cancer and Leukaemia Group and the British Society for Paediatric Endocrinology and Diabetes, with the oversight and endorsement of the Royal College of Paediatrics and Child Health using Appraisal of Guidelines for Research & Evaluation II methodology to standardise care for children and young people with craniopharyngiomas.

Bilateral, Unaugmented, Loop Myopexy Performed for a Severe Case of Heavy Eye Syndrome.

AIM: To report the clinical features and surgical outcomes of one patient with heavy eye syndrome who underwent bilateral, unaugmented, full loop myopexy. METHODS: A 47-year-old lady with high myopia, high axial length, progressive esotropia, slippage of the lateral rectus (LR) inferiorly and superior rectus (SR) medially on magnetic resonance imaging (MRI) was diagnosed with heavy eye syndrome. Unaugmented loop myopexy without medial rectus (MR) recession was offered. RESULTS: On follow-up at 30 months, a small residual esotropia of 6 prism diopters (PD) at near and 10 PD at distance was achieved. Both abduction and elevation were improved in both eyes. CONCLUSIONS: The high angle of esodeviation can be challenging to correct adequately with surgery, with many options available: resection-recession, hemitranspositions (Yamada's procedure), partial loop myopexy (modified Jensen's procedure) and full loop myopexy (Yokoyama's procedure). It remains unclear which procedure is optimal for severe disease. In this case, we present bilateral, unaugmented, full loop myopexy as our preferred choice for high esotropia.

Visual outcomes after chemotherapy for optic pathway glioma in children with and without neurofibromatosis type 1: results of the International Society of Paediatric Oncology (SIOP) Low-Grade Glioma 2004 trial UK cohort.

AIMS: To report visual acuity (VA) outcomes following chemotherapy for optic pathway glioma (OPG) in children with or without neurofibromatosis type-1 (NF1) and to analyse associated risk factors. METHODS: A prospective, multicentre, cohort study involving 155 children treated between September 2004 and December 2012. Initial and final VA was used for per-eye and per-subject analysis. Correlation tests were performed to determine whether initial VA predicted final VA. Logistic regression was used to determine whether age and tumour location were associated risk factors. RESULTS: 90 children had complete ophthalmological data. At initiation of chemotherapy, 26% and 49% of eyes with NF1-OPG and sporadic OPG, respectively, had VA of ≥0.7 log of the minimum angle of resolution (logMAR). At final visit, per eye, 49% had ≤0.2, 23% had 0.30-0.60 and 28% had VA≥0.70 logMAR in the NF1-OPG group. In the sporadic OPG group, per eye, 32% had ≤0.2, 11% had VA 0.30-0.60 and 57% had ≥0.70 logMAR. Children with sporadic OPG, per eye, were significantly less likely to have VA outcomes ≤0.60 logMAR compared with children with NF1-OPG (OR=0.30; 95% CI 0.16 to 0.56; P<0.0001). Per subject, VA improved in 24%, remained stable in 35% and worsened in 41% of children with NF1-OPG and improved in 18%, remained stable in 43% and worsened in 39% of children with sporadic OPG. CONCLUSIONS: Children with and without NF1 demonstrated the same rate of VA improvement, stabilisation or worsening; however, children with sporadic OPG had a poorer VA outcome. Better initial VA, older age, absence of postchiasm tumour and presence of NF1 were associated with improved or stable VA outcomes.

INTRAVITREAL RANIBIZUMAB AS AN ADJUNCTIVE TREATMENT FOR COATS DISEASE (6-YEAR FOLLOW-UP).

PURPOSE: To evaluate the effectiveness of intravitreal ranibizumab in combination with laser photocoagulation in the management of Coats disease. METHOD: Six intravitreal injections of 0.5 mg (0.05 mL) ranibizumab were performed, each 4 weeks apart. Correspondence to therapy was evaluated using visual acuity measurements and optical coherence tomography images. Six months after the initiation of treatment, laser photocoagulation was applied on the telangiectasias and on the surrounding ischemic areas, followed by a single intravitreal ranibizumab injection. Three months later, laser photocoagulation was repeated in purpose to prevent recurrence. RESULTS: In the sixth month, no improvement in visual acuity was recorded, as thick circinate hard exudates still remained in the submacular space. Nevertheless, retinal detachment had completely subsided, and fluorescein angiography showed a significant decrease of leakage from the telangiectatic vessels. At the 12-month follow-up visit, an impressive improvement was recorded, with total absorption of the submacular exudate and visual acuity being 20/30. At the 20-month follow-up visit, his visual acuity was 20/20 and the retina was flat with no signs of macular edema or exudates. At the 6-year follow-up visit, the patient was absolutely stable. DISCUSSION: Elevated vascular endothelial growth factor levels have been demonstrated in Coats disease. Anti-vascular endothelial growth factor agents assist to the decrease of vascular permeability of the capillary endothelial cells, thus increasing the efficiency of laser application.

The ophthalmic natural history of paediatric craniopharyngioma: a long-term review.

We present our experience over the long-term of monitoring of visual function in children with craniopharyngioma. Our study involves an analysis of all paediatric patients with craniopharyngioma younger than 16 at the time of diagnosis and represents a series of predominantly sub-totally resected tumours. Visual data, of multiple modality, of the paediatric patients was collected. Twenty patients were surveyed. Poor prognostic indicators of the visual outcome and rate of recurrence were assessed. Severe visual loss and papilledema at the time of diagnosis were more common in children under the age of 6. In our study visual signs, tumour calcification and optic disc atrophy at presentation are predictors of poor visual outcome with the first two applying only in children younger than 6. In contrast with previous reports, preoperative visual field (VF) defects and type of surgery were not documented as prognostic indicators of poor postoperative visual acuity (VA) and VF. Contrary to previous reports calcification at diagnosis, type of surgery and preoperative VF defects were not found to be associated with tumour recurrence. Local recurrence is common. Younger age at presentation is associated with a tendency to recur. Magnetic resonance imaging (MRI) remains the recommended means of follow-up in patients with craniopharyngioma.

Experimental investigation on mechanism of hydrophilic acrylic intraocular lens calcification.

PURPOSE: To construct a model simulating intraocular lens (IOL) opacification attributable to the formation of calcium phosphate deposits and to investigate the kinetics of deposit formation. DESIGN: Prospective laboratory investigation. SETTING: Department of Ophthalmology, Medical School and Department of Chemical Engineering, Laboratory of Inorganic and Analytical Chemistry, University of Patras, Greece. STUDY POPULATION: Three hydrophilic acrylic IOLs (26% water content) were placed inside a 10-mL double-walled thermostated reactor simulating the anterior chamber. Simulated aqueous humor was injected continuously into the reactor using a pump with variable speed. OBSERVATION PROCEDURES: The observation of IOLs was carried out in situ daily by optical microscopy. Scanning electron microscopy and energy-dispersive radiographic spectroscopy were used for the identification of the morphologic features and the composition of the deposits. RESULTS: The lenses were removed and inspected 5, 9, and 12 months after the initiation of the experiment. Investigation showed deposits of calcium phosphate crystallites in the interior of opacified IOLs. However, these deposits were not observed on the surface of the IOLs. CONCLUSIONS: In agreement with earlier reports by our group and in the literature, IOL opacification is the result of calcification. It is suggested that the surface hydroxyl groups of the polyacrylic polymeric components of the IOLs are capable of inducing surface nucleation and crystal growth of calcium phosphates. However, most important is the finding that the calcification of IOLs is initiated from their interior through the development of sufficiently high local supersaturation, realized through the diffusion of calcium and phosphate ions.