Orthopaedic management of children with spinal dysraphism.

Aims: Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs. Methods: A PROSPERO-registered systematic literature review was performed following PRISMA guidelines. All relevant studies published until January 2023 were identified. Individual outcomes and outcome measurement tools were extracted verbatim. The measurement tools were assessed for reliability and validity, and all outcomes were grouped according to the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT) filters. Results: From 91 eligible studies, 27 individual outcomes were identified, including those related to clinical assessment (n = 12), mobility (n = 4), adverse events (n = 6), investigations (n = 4), and miscellaneous (n = 1). Ten outcome measurement tools were identified, of which Hoffer's Functional Ambulation Scale was the most commonly used. Several studies used unvalidated measurement tools originally developed for other conditions, and 26 studies developed new measurement tools. On the OMERACT filter, most outcomes reported pathophysiology and/or the impact on life. There were only six patient- or parent-reported outcomes, and none assessed the quality of life. Conclusion: The outcomes that were reported were heterogenous, lack validation and failed to incorporate patient or family perceptions. Until outcomes can be reported unequivocally, research in this area will remain limited. Our findings should guide the development of a core outcome set, which will allow consistency in the reporting of outcomes for this condition.

Investigating the relationship between FRailty And Quality of LIfe in patients with heart faiLure and CKD (FRAIL study).

AIMS: Patients with chronic kidney disease (CKD) or heart failure (HF) are disproportionally affected by frailty, an independent predictor of morbidity. The prevalence of frailty and its impact on quality of life (QoL) in a unique population of patients with both CKD and HF (CKD-HF) is unclear. The aim of this study was to investigate the association between frailty and QoL in patients with CKD-HF. METHODS AND RESULTS: Patients were identified from a tertiary care cardiorenal clinic. Eligible patients had CKD-HF with a stable estimated glomerular filtration rate of <60 mL/min/1.732. Data were collected from each participant at one point in time using surveys delivered by study personnel between 14 July 2022 and 31 March 2023. Frailty was defined as Modified Frailty Phenotype (MFP) score ≥3. The Medical Outcomes Study 36-item Short Form Health Survey (SF-36) was used to assess QoL. Demographic data were retrospectively collected from electronic patient records. Demographics and QoL were compared between frail and non-frail cohorts using Pearson's R and Student's t-test (two-tailed, alpha-priori = 0.05). One hundred five participants consented, and 103 completed the questionnaires in full. Amongst the 103 participants, 49.5% (n = 51) were frail. Frailty was related to sex (P = 0.021) and medication count (P = 0.007), however not to other clinical measures, including estimated glomerular filtration rate (P = 0.437) and ejection fraction (P = 0.911). Frail patients reported poorer QoL across physical functioning (P < 0.001), general health (P < 0.001), bodily pain (P = 0.004), social functioning (P < 0.001), and energy levels (P < 0.001), however not emotional wellbeing (P = 0.058); 51.5% cited 'better quality of life' as their healthcare priority, over longer survival (23.3%) or avoiding hospital admissions (22.3%). This was consistent across frail and non-frail groups. CONCLUSIONS: A large proportion of CKD-HF patients are frail, regardless of disease severity, and more susceptible to significantly poorer QoL across physical and social domains. Improving QoL is the priority of patients across both frail and non-frail cohorts, further emphasizing the need for prompt recognition of frailty as well as possible intervention and prevention.

A 'Hub and Spoke' Shared Care initiative for CTEV Ponseti service.

Aims: The Ponseti method is the gold standard treatment for congenital talipes equinovarus (CTEV), with the British Consensus Statement providing a benchmark for standard of care. Meeting these standards and providing expert care while maintaining geographical accessibility can pose a service delivery challenge. A novel 'Hub and Spoke' Shared Care model was initiated to deliver Ponseti treatment for CTEV, while addressing standard of care and resource allocation. The aim of this study was to assess feasibility and outcomes of the corrective phase of Ponseti service delivery using this model. Methods: Patients with idiopathic CTEV were seen in their local hospitals ('Spokes') for initial diagnosis and casting, followed by referral to the tertiary hospital ('Hub') for tenotomy. Non-idiopathic CTEV was managed solely by the Hub. Primary and secondary outcomes were achieving primary correction, and complication rates resulting in early transfer to the Hub, respectively. Consecutive data were prospectively collected and compared between patients allocated to Hub or Spokes. Mann-Whitney U test, Wilcoxon signed-rank test, or chi-squared tests were used for analysis (alpha-priori = 0.05, two-tailed significance). Results: Between 1 March 2020 and 31 March 2023, 92 patients (139 feet) were treated at the service (Hub 50%, n = 46; Spokes 50%, n = 46), of whom nine were non-idiopathic. All patients (n = 92), regardless of allocation, ultimately achieved primary correction, with idiopathic patients at the Hub requiring fewer casts than the Spokes (mean 4.0 (SD 1.4) vs 6.9 (SD 4.4); p < 0.001). Overall, 60.9% of Spokes' patients (n = 28/46) required transfer to the Hub due to complications (cast slips Hub n = 2; Spokes n = 17; p < 0.001). These patients ultimately achieved full correction at the Hub. Conclusion: The Shared Care model was found to be feasible in terms of providing primary correction to all patients, with results comparable to other published services. Complication rates were higher at the Spokes, although these were correctable. Future research is needed to assess long-term outcomes, parents' satisfaction, and cost-effectiveness.

Scoring systems for the management of oncological hepato-pancreato-biliary patients.

Oncological scoring systems in surgery are used as evidence-based decision aids to best support management through assessing prognosis, effectiveness and recurrence. Currently, the use of scoring systems in the hepato-pancreato-biliary (HPB) field is limited as concerns over precision and applicability prevent their widespread clinical implementation. The aim of this review was to discuss clinically useful oncological scoring systems for surgical management of HPB patients. A narrative review was conducted to appraise oncological HPB scoring systems. Original research articles of established and novel scoring systems were searched using Google Scholar, PubMed, Cochrane, and Ovid Medline. Selected models were determined by authors. This review discusses nine scoring systems in cancers of the liver (CLIP, BCLC, ALBI Grade, RETREAT, Fong's score), pancreas (Genç's score, mGPS), and biliary tract (TMHSS, MEGNA). Eight models used exclusively objective measurements to compute their scores while one used a mixture of both subjective and objective inputs. Seven models evaluated their scoring performance in external populations, with reported discriminatory c-statistic ranging from 0.58 to 0.82. Selection of model variables was most frequently determined using a combination of univariate and multivariate analysis. Calibration, another determinant of model accuracy, was poorly reported amongst nine scoring systems. A diverse range of HPB surgical scoring systems may facilitate evidence-based decisions on patient management and treatment. Future scoring systems need to be developed using heterogenous patient cohorts with improved stratification, with future trends integrating machine learning and genetics to improve outcome prediction.