We report on the location, symptoms, and management of plexiform neurofibroma (PN) in children with Neurofibromatosis Type 1 (NF1) attending the 2 National Complex Neurofibromatosis 1 Services at Guy's and St. Thomas' NHS Foundation Trust, London and St Mary's Hospital, Manchester. Retrospective data collection was performed from patient chart reviews from April 2018 to April 2019. There were 127 NF1 patients with PN, age range 0.8-17.0, mean age was 9.9 years (SD ± 4.2 years). The main location of the PN was craniofacial in 35%, and limb in 19%. Disfigurement was present in 57%, pain in 28%, impairment of function in 23%,and threat to function in 9% of children. Fifty-four percent of patients were managed conservatively, 28% surgically, and 19% are either taking or due to start a mitogenactivated protein kinase kinase (MEK) inhibitor (selumetinib or trametinib), either through a clinical trial or compassionate usage scheme. This national study provides a comprehensive overview of the management of children with PN in an era where new therapies (MEK inhibitors) are becoming more widely available. We anticipate that there will be a shift to more patients receiving MEK inhibitor therapy and combination therapy (surgery and MEK inhibitor) in the future.

Developmental dysplasia of the hip (DDH) ranges from a clinically detectable dislocation of the hip to radiologically diagnosed hip abnormalities. It is caused by both antenatal and postnatal factors. The Neonatal and Infant Physical Examination Screening Programme recommends that newborns should undergo a hip ultrasound (USS) when risk factor or clinical features suggestive of DDH are present. The aim of hip ultrasonography is to detect DDH early and prevent late complications through early treatment. Here, we discuss how to use hip examination and USS in newborns.

Results Majority of the children were in preschool/lower primary with 78.6% males and 65.5% Chinese. The most common diagnosis was Autism Spectrum Disorder (ASD; 46.4%). Almost two-thirds of caregivers reported working from home during the Circuit Breaker period. The majority indicated that their children had no difficulties coping with the restrictions in place during this period. Of those who did, the main difficulties were with wearing a mask, being restricted from going out, and having to practice social distancing. 26.2% of children had deterioration in their behavior, and a third of caregivers struggled to manage this. There was no significant difference in proportion of children experiencing a deterioration in behavior by diagnostic group [X 2 (d.f.=6, N=84) =5.7, p=0.47]. Yet some (16.7%) had improvements in behavior. Sleep was minimally affected, but there was a significant increase in screen use in 81% of cases, especially in children with ASD. The majority of children received home-based learning from schools but many encountered challenges with this. About two -third of children continued to receive their early intervention or hospital-based therapy services. 54.7% of caregivers reported feeling more stressed than usual, mainly from juggling work-vs-family commitments. In spite of all the challenges, around two-thirds reported being able to spend more quality time with their child and over a half over a half of them reported having improved relationships with their child Conclusions This survey shows that whilst many coped with the restrictive lockdown measures, there are those families who are more vulnerable, or caregivers who are less adept, and who need to be identified early for future support. Parents have the potential to be effective therapy providers in the home setting, given adequate support, but may struggle to balance work and family commitments. Telehealth has been developed but improvements in telehealth service provision and audits of its efficacy are still required.