Consensus guideline for the diagnosis and management of pituitary adenomas in childhood and adolescence: Part 2, specific diseases.

Pituitary adenomas are rare in children and young people under the age of 19 (hereafter referred to as CYP) but they pose some different diagnostic and management challenges in this age group than in adults. These rare neoplasms can disrupt maturational, visual, intellectual and developmental processes and, in CYP, they tend to have more occult presentation, aggressive behaviour and are more likely to have a genetic basis than in adults. Through standardized AGREE II methodology, literature review and Delphi consensus, a multidisciplinary expert group developed 74 pragmatic management recommendations aimed at optimizing care for CYP in the first-ever comprehensive consensus guideline to cover the care of CYP with pituitary adenoma.

Consensus guideline for the diagnosis and management of pituitary adenomas in childhood and adolescence: Part 1, general recommendations.

Tumours of the anterior part of the pituitary gland represent just 1% of all childhood (aged <15 years) intracranial neoplasms, yet they can confer high morbidity and little evidence and guidance is in place for their management. Between 2014 and 2022, a multidisciplinary expert group systematically developed the first comprehensive clinical practice consensus guideline for children and young people under the age 19 years (hereafter referred to as CYP) presenting with a suspected pituitary adenoma to inform specialist care and improve health outcomes. Through robust literature searches and a Delphi consensus exercise with an international Delphi consensus panel of experts, the available scientific evidence and expert opinions were consolidated into 74 recommendations.

Diclofenac Versus Corticosteroids Following Strabismus Surgery: Systematic Review and Meta-analysis.

The purpose of the current study was to compare outcomes of diclofenac versus corticosteroids following strabismus surgery. A systematic review and meta-analysis were performed in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. An electronic search was performed to include comparative studies of diclofenac versus corticosteroids following strabismus surgery.

NF1 optic pathway glioma: analyzing risk factors for visual outcome and indications to treat.

Background: The aim of the project was to identify risk factors associated with visual progression and treatment indications in pediatric patients with neurofibromatosis type 1 associated optic pathway glioma (NF1-OPG).

Methods: A multidisciplinary expert group consisting of ophthalmologists, pediatric neuro-oncologists, neurofibromatosis specialists, and neuro-radiologists involved in therapy trials assembled a cohort of children with NF1-OPG from 6 European countries with complete clinical, imaging, and visual outcome datasets. Using methods developed during a consensus workshop, visual and imaging data were reviewed by the expert team and analyzed to identify associations between factors at diagnosis with visual and imaging outcomes.

Fifteen-minute consultation: Managing a child with a new-onset squint.

Objective: To provide a systematic approach to the child with a new squint.

Method: Review of the current available literature.

Conclusion: Squint is a common presentation in the paediatric population.

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.

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.

Improving survival of retinoblastoma in Uganda.

Background: Diagnostic delay results in relatively high mortality among children with retinoblastoma in Uganda, where treatment was limited to surgery and, for some, radiotherapy. In order to improve outcomes, a simple programme of neoadjuvant and adjuvant chemotherapy was introduced. Here we report survival before and after this change to medical practice.

Clinical features and survival among children with retinoblastoma in Uganda.

Aims: To characterise the clinical features, treatment and outcome of children diagnosed with retinoblastoma in Uganda.

Methods: The study comprised a 6-year nationwide enrolment with follow-up.

Results: In total, 282 cases were enrolled, 26% (72) were bilateral; 6% were lost to follow-up.

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.

Whole-exome sequencing identifies mutations in GPR179 leading to autosomal-recessive complete congenital stationary night blindness.

Congenital stationary night blindness (CSNB) is a heterogeneous retinal disorder characterized by visual impairment under low light conditions. This disorder is due to a signal transmission defect from rod photoreceptors to adjacent bipolar cells in the retina. Two forms can be distinguished clinically, complete CSNB (cCSNB) or incomplete CSNB; the two forms are distinguished on the basis of the affected signaling pathway.

Replication of the recessive STBMS1 locus but with dominant inheritance.

Purpose: Strabismus is a common eye disorder with a prevalence of 1% to 4%. Comitant strabismus accounts for approximately 75% of all strabismus, yet more is known about the less common incomitant disorders. Comitant strabismus is at least partly inherited, but only one recessive genetic susceptibility locus, on chromosome 7p, has been identified in one family.

Retinopathy of prematurity treatment and vitreous hemorrhage.

Two cases of hemorrhagic retinopathy of prematurity associated with thrombocytopenia are presented. The negative prognosis of vitreous hemorrhage in retinopathy of prematurity and the option of platelet transfusions are discussed.

Genetic, ophthalmic, morphometric and histopathological analysis of the Retinopathy Globe Enlarged (rge) chicken.

Purpose: To identify the locus responsible for rge (retinopathy globe enlarged) in chickens and further characterise the rge phenotype.

Methods: A colony of chickens carrying the rge mutation was rederived from a single heterozygous animal of the original line. The eyes of blind, heterozygous and normal birds were subjected to ophthalmic, morphometric and histopathological examination to confirm and extend published observations.