The hypospadias-specific health-related quality of life conceptual framework: a scoping review of the literature.

PURPOSE: Health-related quality of life (HRQoL) is an important yet understudied construct for individuals with hypospadias (HS). An important barrier towards understanding HRQoL and integrating it into research and clinical care for people with HS is the absence of an underlying conceptual framework to illustrate this construct. We propose a conceptual framework for HS-specific HRQoL based upon a scoping review of the HS literature. METHODS/MATERIALS: We conducted a literature review of articles published between 1989 and 2019. Our search in Embase and Pubmed used the keyword "hypospadias" in combination with "quality of life" and "patient-reported outcomes." We used thematic analysis of the resulting publications to identify core HRQOL domains. From these results and review of HRQoL literature in other pediatric populations, we developed a conceptual framework representing HS-specific HRQoL. RESULTS: We identified five domains of HRQOL previously studied in research with youth and adults with HS: penile appearance, voiding, social interaction, sexual health, and psychological or behavioral function. We propose a model of HS-specific HRQoL comprised of these domains and their areas of overlap, based upon the findings and conceptual mapping of our literature review. CONCLUSION: This novel conceptual framework provides a foundation for understanding disease-specific HRQoL in individuals with HS and may serve as a guide for the conduct of future qualitative studies of the HS population. The overlapping biopsychosocial domains illustrate the possible effects of HS on day-to-day life. This framework may guide future surgical, clinical, and behavioral interventions that aim to improve medical care and quality of life outcomes for HS patients.

External validation of a low fidelity dry-lab platform to enhance loupes surgical skills techniques for hypospadias repair.

INTRODUCTION: Hypospadias repair is an index pediatric urology procedure that requires trainee familiarity with surgical loupes. A previous low-fidelity, 6-step curriculum was proposed that deconstructed the most important steps of loupe surgery. We expanded on this curriculum with an intermediate-fidelity silicone hypospadias model and designed an abbreviated version of the 6-step curriculum to precede the hypospadias repair simulation. OBJECTIVE: To assess the validity of our prior, low-fidelity conceptual model using the metric of improved performance on the intermediate-fidelity silicone hypospadias model. STUDY DESIGN: A silicone model was first prototyped with the design software Solidworks™, and then fabricated using a cast made of a mixture of silicone rubbers designed to function like skin and soft tissue (Mold Star 20T, Dragon skin FX-pro and Slacker). Casts were used to create the penile shaft model and the dorsal hooded foreskin model. The urethral plate was cast separately on a flat surface. The model was then assembled by hand. The model used for simulation included the penile shaft and urethral plate, while the dorsal-hooded foreskin was prepared to simulate the penile anatomy separately. Trainees were then divided into two groups. Group 1 practiced the low-fidelity curriculum (3 tasks) and then performed dissection of the urethral plate and suturing using the intermediate-fidelity hypospadias model. Group 2 practiced hypospadias repair prior to the low-fidelity curriculum. Both groups' models were scored by 3 blinded urologists. Trainees were then asked to complete a post simulation satisfaction survey. Data analysis was performed in IBM SPSS Statistics for Macintosh (Version 28.0 Armonk, NY: IBM Corp). RESULTS: Twenty-two candidates across Wisconsin, USA, and Dublin, Ireland participated in the study. This included 7 s-year residents, 9 third-year residents, 2 fourth-year residents, and 3 fifth-year residents. Both Groups 1 and 2 had a similar distribution of trainees (p = 0.60). Group 1 outperformed group 2 in all tasks (p < 0.05, Table 1). Trainees reported that the platform was very useful (91%). DISCUSSION: Our curriculum showed improvement in trainee ability and comfort to perform hypospadias repair. Advantages of such a simulated curriculum include improving current resident training in microsurgery, improving surgical ergonomics for trainees prior to real-time experience, and decreasing the learning curve for trainees pursuing pediatric urology. CONCLUSION: An intermediate-fidelity hypospadias platform externally validates the conceptual model implemented in the low-fidelity loupes curriculum. This appears to lead to improvement in loupe surgical skills regardless of trainee level.

Evaluating quality of patient-reported outcome measures in patients with hypospadias.

INTRODUCTION: There is growing recognition of the importance of patient-reported outcomes (PROs) in pediatric hypospadias. We have previously presented a conceptual framework for Hypospadias-Specific Health-Related Quality of Life (HRQoL), which posited 5 domains of HRQoL in this population. The framework components (domains) included penile appearance, voiding function, social function, psychological/behavioral function, and pubertal/sexual health. In this work, we investigated the established validity and relevance of PROs within each of these domains for patients with hypospadias. MATERIALS AND METHODS: We evaluated existing measures with published psychometric data, including validation data, in the hypospadias population. We also assessed the available data on each measure according to the guidelines of the Scientific Advisory Committee of the Medical Outcomes Trust (Table) in order to establish measure quality. We also examined the power of existing validation studies according to suggested guidelines for psychometric validation and factor analysis. DISCUSSION: Available validated measures in the hypospadias population have focused primarily on penile appearance and to a lesser degree on pubertal development/sexual health. There were no validated disease-specific measures with dedicated evaluations of other key HRQoL domains including voiding-related sequelae, social function, or psychological function. In addition, no single measure addressed all of the quality guidelines posed by the Scientific Advisory Committee. CONCLUSIONS: Current generalized measures for PROs lack relevance to the experience of hypospadias patients, and disease-specific assessments are often focused on penile appearance. Improving measure quality is necessary to optimize the value of our assessments and better help our patients with hypospadias.

Effect of intrauterine nutritional deprivation on neuromotor behaviour of the newborn.

The neurological maturation in 25 newborn babies born to severely undernourished mothers was studied by evaluating muscle tone and excitability status. These mothers had weight below the 25th percentile expected for height, haemoglobin less than 80.0 g/l and serum albumin less than 25.0 g/l. Twenty-three babies born to healthy mothers were studied as control. The babies of undernourished mothers demonstrated gross intrauterine growth retardation. There occurred parallel reduction in placental weight and its protein content. The neuromotor behaviour of these newborns showed significant alteration in the performance of most reflexes, resembling normal motor behaviour of preterm infants. As many as 72% newborns could be classified as hypotonic and 56% hypoexcitable. However, no newborn demonstrated hypertonia or hyperexcitability. The parameters of neuromotor assessment were found to have no correlation with the birth weight in both undernourished as well as control group. These observations suggest that muscle tone and excitability are better indices of maturation of central nervous system than the birth weight. It seems that the neurological evaluation becomes unreliable in babies who suffer from intrauterine nutritional deprivation.

Sleep cycle studies in babies of undernourished mothers.

Polygraphic sleep cycle studies--comprising simultaneous recording of electroencephalogram (EEG), electrocardiogram (ECG), electro-oculogram (ECOG), phasic body activity and respiration--were performed on 19 term newborn babies born to severely undernourished mothers, and results compared with 19 healthy newborn babies. The sleep cycle in babies of undernourished mothers showed disorganisation during active rapid eye movement (REM) sleep and quiet nonrapid eye movement (NREM) sleep. The sleep disorganisation was more pronounced during quiet NREM sleep when frequent phasic activity and ocular movements were registered, thereby leading to a significant increase in the content of intermediate sleep. Furthermore, the duration of total sleep cycle and its components, active REM and quiet NREM sleep, were significantly shortened.