A comparison of 467 uroflowmetry results in repaired hypospadias vs. normal male flows.

INTRODUCTION: There are currently no clinical criteria for obstructed urinary flow after hypospadias repair surgery. Previous studies have utilized adult and pediatric nomograms and flow shapes to define obstruction, however these methods are limited by a lack of standardization and lack of interrater reliability when determining flow shapes, respectively. The idealized voider derived flow indexes offer a way to track uroflowmetry results in a volume and age agnostic manner. OBJECTIVE: We sought to evaluate all our hypospadias patients over a 10-year period and identify patients without complications and those with complications and determine their respective flow parameters. Our secondary objective is to identify which uroflowmetry parameters are the most significant predictors of urethral stricture and meatal stenosis at the time of the uroflowmetry study. STUDY DESIGN: Retrospective chart review was used to compile demographic information, details of hypospadias repair surgeries, and uroflowmetry results from pediatric hypospadias repair patients. Subjects were divided into distal, midshaft, and proximal groups based on the initial location of their urethral meatus. Flows from the hypospadias repair groups were compared to flows from normal age matched controls from a previous study. We compared flows from hypospadias repair patients with no complications present with those who had urethral stricture or meatal stenosis present at the time of uroflowmetry. Binary logistic regression and ROC analysis was used to assess different uroflowmetry parameters' ability to detect the presence of obstructed urine flow. RESULTS: 467 uroflowmetry studies from 200 hypospadias repair patients were included in the database. Compared to controls, the hypospadias repair groups tended to have significantly lower Qmax, Qavg, Qmax FI, Qavg FI, and longer ttQmax. Significant differences in flow parameters were observed when comparing hypospadias repair patients with and without flow obstructing complications at the time of uroflowmetry. Binary logistic regression including various uroflowmetry parameters showed Qmax FI had a significant effect on the odds of observing the absence of a stricture in proximal and distal hypospadias cases. DISCUSSION: Of the uroflowmetry parameters analyzed, binary logistic regression and the likelihood ratio of a positive result all point to Qmax FI as the better parameter to use to detect the presence of complications in patients who have undergone distal or proximal hypospadias repair surgery. CONCLUSION: We have established normal parameters for post-operative hypospadias repair patients which can be used to follow patients over time and allow for the identification of complications by keeping track of flow indexes which are volume and age agnostic.

What should be next in lifelong posterior hypospadias: Conclusions from the 2023 ERN eUROGEN and EJP-RD networking meeting.

BACKGROUND: A congenital disease is for life. Posterior hypospadias, the severe form of hypospadias with a penoscrotal, scrotal, or perineal meatus, is a challenging condition with a major impact on lifelong quality of life. AIM: Our network meeting is aimed to identify what is currently missing in the lifelong treatment of posterior hypospadias, to improve care, quality of life, and awareness for these patients. METHODS: The network meeting "Lifelong Posterior Hypospadias" in Utrecht, The Netherlands was granted by the European Joint Programme on Rare Diseases-Networking Support Scheme. There was a combination of interactive sessions (hackathons) and lectures. This paper can be regarded as the last phase of the hackathon. RESULTS: Surgery for hypospadias remains challenging and complications may occur until adulthood. Posterior hypospadias affects sexual function, fertility, and hormonal status. Transitional care from childhood into adulthood is currently insufficiently established. Patients should be more involved in defining desired treatment approach and outcome measures. For optimal outcome evaluation standardization of data collection and registration at European level is necessary. Tissue engineering may provide a solution to the shortage of healthy tissue in posterior hypospadias. For optimal results, cooperation between basic researchers from different centers, as well as involving clinicians and patients is necessary. CONCLUSIONS: To improve outcomes for patients with posterior hypospadias, patient voices should be included and lifelong care by dedicated healthcare professionals guaranteed. Other requirements are joining forces at European level in uniform registration of outcome data and cooperation in basic research.

ECLAMC Study: prevalence patterns of hypospadias in South America: multi-national analysis over a 24-year period

ABSTRACT Objective To evaluate prevalence trends of hypospadias in South-America it is essential to perform multicenter and multinational studies with the same methodology. Herein we present systematic data as part of an international multicenter initiative evaluating congenital malformations in South America over a 24-year period. Materials and Methods A nested case-control study was conducted using the Latin American Collaborative Study of Congenital Malformations (ECLAMC), between January 1989 and December 2012. Cases were stratified as isolated (IH) and non-isolated hypospadias (NIH). Global prevalence was calculated and discriminated by country. Associations between birth weight and gestational age, and NIH distribution by associated abnormality and severity of hypospadias, were analyzed. Results A total of 159 hospitals from six countries participated, reporting surveillance on 4.020.384 newborns. A total of 4.537 hypospadias cases were detected, with a global prevalence of 11.3/10.000 newborns. Trend analyses showed in Chile, Brazil and Uruguay a statistically significant increase in prevalence. Analysis of severity and associated anomalies did not to find an association for distal cases, but did for proximal (RR=1.64 [95% CI=1.33-2.03]). Conclusion This is one of only a few Latin American multicenter studies reporting on the epidemiology of hypospadias in South America in the last two decades. Our data adds to evidence suggesting an increase in some countries in the region at different times. There were also variations in prevalence according to severity. This study adds to literature describing associated anomalies at a hospital-based level.

Achados audiológicos e eletrofisiológicos de indivíduos com a síndrome G/BBB / Auditory findings and electrophysiologics in individuals with G/BBB syndrome

A síndrome G/BBB é uma condição rara, caracterizada por hipertelorismo, fissura de lábio e palato e hipospádia. Não foram encontrados trabalhos sobre a audição em indivíduos com esta síndrome. OBJETIVO: Investigar a função auditiva em pacientes com síndrome G/BBB quanto à ocorrência ou não de perda auditiva e a condução nervosa auditiva periférica e central. MATERIAL E MÉTODO: Catorze pacientes de 7 a 34 anos, do gênero masculino, com a síndrome G/BBB, foram avaliados por meio de otoscopia, audiometria, timpanometria e potenciais evocados auditivos de tronco encefálico (PEATE). Forma de Estudo: Estudo de série clínico prospectivo. RESULTADOS: Limiares audiométricos normais em 12 (66,7 por cento) pacientes da amostra e alterados em dois (33,3 por cento), sendo um com perda condutiva e um neurossensorial. Quanto ao PEATE, foram encontrados: latências absolutas da onda I normais em todos os pacientes, aumento das latências absolutas da onda III e V em dois e seis pacientes respectivamente; latências interpicos I-III, III-V e I-V aumentadas em quatro, três e oito pacientes, respectivamente. CONCLUSÃO: Perdas auditivas periféricas podem ocorrer na síndrome G/BBB. Há evidências de comprometimento das vias auditivas centrais em nível do tronco encefálico. Estudos com exames de imagem são necessários para maior clareza dos achados clínicos.

The G/BBB syndrome is a rare condition characterized by hypertelorism, cleft lip and palate, and hypospadias. No studies were found on the hearing of individuals with this syndrome. AIM: To investigate the auditory function in patients with G/BBB syndrome, such as the occurrence of hearing loss, and central and peripheral auditory nerve conduction. METHODS: Fourteen male patients aged 7-34 years with the G/BBB syndrome were assessed by otoscopy, audiometry, tympanometry and evoked auditory brainstem response (ABR). Model: A retrospective clinical series study. RESULTS: Audiometric thresholds were normal in 12 (66.7 percent) of the sample and altered in two (33.3 percent), one with conductive and one with sensorineural loss. ABR resuts were: all patients had normal absolute wave I latencies; absolute wave III and V latencies were increased in two and six patients, respectively; interpeak latencies I-III, IV and V interpeak latencies were increased in four, three and eight patients respectively. CONCLUSION: Hearing loss can occur in the G/BBB syndrome. There is evidence of central auditory pathway involvement in the brainstem. Imaging studies are needed to clarify the clinical findings.