Lesley Cavalli’s research while affiliated with Great Ormond Street Hospital for Children NHS Foundation Trust and other places

Communication problems (eg, dysphonia, dysfluency and language and articulation disorders), swallowing disorders (dysphagia and globus), cough and upper airway symptoms, resulting from functional neurological disorder (FND), are commonly encountered by speech and language professionals. However, there are few descriptions in the literature of the most effective practical management approaches. This consensus document aims to provide recommendations for assessment and intervention that are relevant to both adults and young people. An international panel of speech and language professionals with expertise in FND were approached to take part. Participants responded individually by email to a set of key questions regarding best practice for assessment and interventions. Next, a video conference was held in which participants discussed and debated the answers to these key questions, aiming to achieve consensus on each issue. Drafts of the collated consensus recommendations were circulated until consensus was achieved. FND should be diagnosed on the basis of positive clinical features. Speech and language therapy for FND should address illness beliefs, self-directed attention and abnormal movement patterns through a process of education, symptomatic treatment and cognitive behavioural therapy within a supportive therapeutic environment. We provide specific examples of these strategies for different symptoms. Speech and language professionals have a key role in the management of people with communication and related symptoms of FND. It is intended that these expert recommendations serve as both a practical toolkit and a starting point for further research into evidence-based treatments.

Importance Paediatric inflammatory multisystem syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) is a novel disease first identified in 2020. Recent cohort studies have described the complex presentation and symptomatology. This paper provides detailed description of the dysphagia and dysphonia symptoms, management, and outcome. Objective To describe dysphagia and dysphonia in PIMS-TS. Design Retrospective cohort study. Setting Single tertiary and quaternary children's hospital. Participants All 50 children treated for paediatric multisystem inflammatory disease between April and June 2020 were included in this study. Main Outcome(s) and Measure(s) Dysphonia: GRBAS Perceptual Severity Scores, Vocal Handicap Index scores and the Vocal Tract Discomfort Scale. Dysphagia: Functional Oral Intake Scale. Results Fifty children met the diagnostic criteria for PIMS-TS. 33 (66%) were male. Median age was 10 years (range: 1–17). 36 (72%) were of Black, Asian or minority ethnic background. Nine (18%) required specialist assessment and management of dysphagia and/or dysphonia. Five (55%) were male with a median age of 9 years 7 months (range: 1–15 years). Symptoms typically resolved within three months. Two children presented with persisting dysphonia three months post-presentation. Neurological, inflammatory, and iatrogenic causes of dysphagia and dysphonia were identified. Conclusions and Relevance Dysphonia and dysphagia are present in children with PIMS-TS. Further data is required to understand pathophysiology, estimate incidence, and determine prognostic factors. This preliminary data highlights the need for dysphagia and dysphonia screening and timely referral for specialist, multidisciplinary assessment and treatment to ensure short-term aspiration risk is managed and long-term, functional outcomes are optimised.

Background Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a new, rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to describe the 6-month outcomes of PIMS-TS. Methods This retrospective cohort study comprised children (aged <18 years) who fulfilled the UK Royal College of Paediatrics and Child Health (RCPCH) diagnostic criteria for PIMS-TS and were admitted to Great Ormond Street Hospital (London, UK) between April 4 and Sept 1, 2020. Patients were followed up by a multidisciplinary team of specialists at 6 weeks and 6 months after admission. Biochemical and functional outcomes were analysed. Findings 46 children were included in this study. The median age at presentation was 10·2 years (IQR 8·8–13·3), 30 (65%) patients were male and 16 (35%) were female, 37 (80%) were from minority ethnic groups, and eight (17%) had pre-existing comorbidities. All patients had elevated markers of systemic inflammation at baseline. None of the patients died. By 6 months, systemic inflammation was resolved in all but one patient. 38 (90%) of 42 patients who had positive SARS-CoV-2 IgG antibodies within 6 weeks of admission remained seropositive at 6 months. Echocardiograms were normal in 44 (96%) of 46 patients by 6 months, and gastrointestinal symptoms that were reported in 45 (98%) of 46 patients at onset were present in six (13%) of 46 patients at 6 months. Renal, haematological, and otolaryngological findings largely resolved by 6 months. Although minor abnormalities were identified on neurological examination in 24 (52%) of 46 patients at 6 weeks and in 18 (39%) of 46 at 6 months, we found minimal functional impairment at 6 months (median Expanded Disability Status Scale score 0 [IQR 0–1]). Median manual muscle test-8 scores improved from 53 (IQR 43–64) during hospital admission to 80 (IQR 68–80) at 6 months, but 18 (45%) of 40 patients showed 6-min walk test results below the third centile for their age or sex at 6 months. PedsQL responses revealed severe emotional difficulties at 6 months (seven [18%] of 38 by parental report and eight [22%] of 38 by self report). 45 (98%) of 46 patients were back in full-time education (virtually or face to face) by 6 months. Interpretation Despite initial severe illness, few organ-specific sequelae were observed at 6 months. Ongoing concerns requiring physical re-conditioning and mental health support remained, and physiotherapy assessments revealed persisting poor exercise tolerance. Longer-term follow-up will help define the extended natural history of PIMS-TS. Funding None.

This cohort study describes the various otolaryngologic manifestations in and rates among patients 18 years or younger with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus 2.

Clinical voice disorders in children arise from, or are associated with, both congenital and acquired laryngeal conditions and whilst many can be managed to a point of resolution some disorders may be expected to havea life-long impact on vocal function. Research indicating the intractable nature of some laryngeal disorders is relatively recent (Reynolds, Meldrum, Simmer, Vijayasekaran, & French, 2017), and studies exploring the nature of impact arising from chronic dysphonia in young people are sparse.There is currently no clear profile of the chronically voice disordered adolescent population nor reported evidence as to those factors that may increase the risk of negative impact arising from a chronic voice disorder. Our study evaluated whether the perceived impact of chronic organic dysphonia, primarily of structural and neurological aetiology, changes across adolescence and aimed to identify other factors such as gender, vocal aetiology, vocal tract discomfort, perceptual voice features, and voice severity, that might impact trends. Data was collected retrospectively from the health records of 67 subjects from the historic voice caseload of a Speech and Language Therapy Service within a tertiary children’s hospital. Subjects ranged from 10 - 18 years and their data extracted from the first recorded contact. Regression analyses were used to evaluate the impact of age and other factors on self-perceived psychosocial impact, measured by the Voice Handicap Index. Results indicate that age can predict self-perceived psychosocial impact of chronic organic dysphonia for the majority of adolescents. The factors of gender, vocal tract discomfort and asthenic voice quality further impact self-perceived psychosocial impact. Interestingly, overall perceptual voice severity was not a predictor of perceived psychosocial impact and in itself should therefore not be used to guide decisions around the need for intervention. Further research is much needed in this field.

Transferring from children’s to adult health and social care services can provide both risk and opportunity (Northumbria, 2017). Effective programmes that offer a period of preparation and integration (transition) should be centred on children and young people (CYP) and placed in the context of CYP’s lives and should include ‘the training of healthcare professionals to utilise effective interpersonal and communication skills’ that meet the needs of each CYP (Kime et al 2013, NICE 2016). In 2018 the CQC identified shared decision-making within the top 4 areas requiring improvement in CYP care.We captured insights from adolescents and their parents with lived experience of transition services both within and external to GOSH. Twenty-nine adolescents participated in an all-day focus group delivered remotely. The group considered 3 key questions. All contributions were transcribed. A separate parent group, focused on the same questions. Themes of Time, Information, Personalisation, Communication and Integration were common to both YP and parents. In addition, YP referenced the impact of mental health on decision making and the impact of health on their education and were aware of diversity and language barriers and the importance of including siblings in communications. Parents had additional concerns around the impact of learning disabilities on their child’s transition and of moves to adult learning institutes. Results provide preliminary information about what ‘good should look like’ in the design and delivery of our transition services with particular reference to communication. Focus group data, systematic review of the literature and engagement with additional stakeholders including health and social care professionals themselves will be triangulated to develop a co-produced training.

Background We review the implementation of a screening tool for suspected vocal cord palsy using ultrasound (US). This is used at our institution to evaluate children with changes to their voice or cry who are not under the primary care of otolaryngology services. Methods A retrospective review (1 st Jan 2015 – 1 st June 2017) of patients referred for US screening of vocal cord function at a single specialist paediatric centre. Results In thirty months, 36 children underwent US assessment of vocal cord function (aged 8 days – 16 years). It was possible to assess vocal cord mobility in all patients. Cases included 22 following cardiothoracic surgery. 21 of 36 had normal US and no subsequent endoscopy. 15 had abnormal function detected (11 left vocal cord palsy, 2 right, 2 bilateral). 12 of the 15 abnormal scans had subsequent endoscopic laryngeal assessment as per the screening protocol. 11 of 12 endoscopic assessments were concordant with US findings. One suspected left VC palsy was not evident at endoscopy. 3 cases lacked sufficient documentation of endoscopy to evaluate concordance. No cases with a negative US scan had a subsequent diagnosis of vocal palsy. Discussion Ultrasonography has been considered an adjunct for evaluation of vocal cord function. The largest cohort of patients in our screening process presented following cardiothoracic surgery. The implementation of this ultrasound screening protocol allowed 58% of cases to avoid endoscopic laryngeal evaluation with no false negatives. Concordance between positive US and endoscopic findings was high (92%). We suggest this is a reliable screening tool to identify vocal cord palsy and avoid unnecessary endoscopic assessment of the larynx.

Introduction Unilateral vocal fold paresis may significantly impair the vocal quality and laryngeal competence of a child. Relatively little literature relates to injection medialization laryngoplasty (IML) in children and previous reports have involved small numbers of heterogenous cases. Methods A retrospective review was conducted of paediatric patients managed by our multidisciplinary specialist voice clinic undergoing IML for unilateral vocal fold paresis. Cases of bilateral paresis, those characterised by vocal fold fixation, and patients without formal preand post-operative voice evaluation were excluded. Results Eighteen IML procedures were performed in 12 children eligible for inclusion between 2005 and 2015. The average age at time of procedure was 12 years (range 9-15 years). Autologous fat was used in 5 procedures, succeeded by calcium hydroxylapatite (Radiesse® Voice) from 2011 (n=13). A significant improvement in median GRBAS score components was observed after calcium hydroxylapatite injection in terms of grade (p=0.008), breathiness (p=0.002) and aesthenia (p=0.016). A pre- and post-procedural Voice Handicap Index was self-completed by 6 patients receiving calcium hydroxylapatite injection; the median change in score was an improvement of 19 points (interquartile range 36.5). Conclusion We describe the outcomes of a comparatively large paediatric series and have found IML using calcium hydroxylapatite to be a reliable technique associated with improved subjective outcome measures. Management of UVCP in the child is a challenge with particular investigative and interventional considerations. Further study supported by high quality subjective and, where possible, objective outcome measures, are required to better inform patient selection, timing of intervention and choice of injection material.

Airway compromise due to paediatric intubation injuries is well documented; however, intubation injuries may also cause severe voice disorders. We report our experience and review the world literature on the voice effects of traumatic paediatric intubation. We report five cases of children referred to Great Ormond Street Hospital for Children who suffered traumatic avulsion of the vocal fold at the time of, or secondary to, endotracheal intubation. All children had significant dysphonia and underwent specialist voice therapy. The mechanisms of injury, risk factors and management of the condition are discussed. Children suffering traumatic intubation require follow up throughout childhood and beyond puberty as their vocal needs and abilities change. At the time of writing, none of the reported patients had yet undergone reconstructive or medialisation surgery. However, regular specialist voice therapy evaluation is recommended for such patients, with consideration of phonosurgical techniques including injection laryngoplasty or thyroplasty.