Double dartos flap layer in tubularized incised plate urethroplasty to prevent urethrocutaneous fistula in uncircumcised patients with distal hypospadias / 亚洲男科学杂志(英文版)

Urethrocutaneous fistula may complicate hypospadias repair. We noticed that double-layered preputial dartos flaps added to tubularized incised plate urethroplasty can reduce the risk of urethrocutaneous fistula. The aim of this study was to compare the outcomes of tubularized incised plate urethroplasty with double-layered preputial dartos flaps to with single-layered local fascial flaps in preventing urethrocutaneous fistula. A retrospective cohort study was conducted between January 2017 and December 2020 at Jordan University Hospital (Amman, Jordan). Boys who were aged between 6 months and 5 years, diagnosed with distal hypospadias, and not circumcised were included. The primary outcome was the occurrence of urethrocutaneous fistula in patients who underwent tubularized incised plate urethroplasty with a double-layered fascial flap. The results showed a total of 163 boys with distal hypospadias; among them, 116 patients underwent tubularized incised plate urethroplasty with a single-layered fascial flap, and 47 underwent tubularized incised plate urethroplasty with a double-layered fascial flap. The development of urethrocutaneous fistula was higher in the group receiving tubularized incised plate urethroplasty with a single-layered fascial flap than in the group receiving tubularized incised plate urethroplasty with a double-layered fascial flap after 1 month, 6 months, and 12 months (6.9% vs 0, 10.3% vs 0, and 5.2% vs 0, respectively), and the difference after 6 months was statistically significant (P = 0.02).

Hipospadias: del diagnóstico ecográfico prenatal al manejo posnatal / Hypospadias: from prenatal ultrasound diagnosis to postnatal management

El hipospadias es la localización anormal del meato urinario y es la malformación de genitales externos más frecuentemente diagnosticada. El diagnóstico prenatal es posible mediante ecografía sistemática desde la semana 20 de gestación, siendo más fácil su diagnóstico en el tercer trimestre. Las formas leves suelen ser aisladas, familiares o asociadas a disfunción placentaria o restricción de crecimiento intrauterino, mientras que las formas más graves presentan hasta un 30% de asociación a defectos fetales, anomalías cromosómicas/genéticas o anomalías del desarrollo sexual. La tríada para el diagnóstico ecográfico prenatal consiste en curvatura ventral del pene, anomalía del prepucio dorsal y punta del pene roma. La valoración de la uretra durante la micción y el aspecto del chorro miccional son de gran utilidad para clasificar el defecto. Cuando se diagnostica hipospadias peneano o escrotal es aconsejable realizar una amniocentesis para estudio genético fetal y valorar otros signos de adecuada virilización, como el descenso testicular a partir de la semana 27. El seguimiento tras el parto debe ser multidisciplinario, incluyendo urólogo y endocrinólogo infantil. En hipospadias leves el pronóstico es bueno con reparación quirúrgica en el primer año de vida, pero las formas graves pueden presentar un reto mayor para su corrección funcional y estética.

Hypospadias refers to the abnormal location of the meatus; it is the most common genital malformation detected in the fetus and newborn. Prenatal diagnosis is feasible from 20 weeks onwards with routine ultrasound; however, it is easier to diagnose during the third trimester of pregnancy. Mild defects are usually isolated, familiar o related to placental disfunction or intrauterine growth restriction, while the severe hypospadias are associated to other fetal defects, genetic or chromosomal abnormalities or disorders of sex development. In about 30% of cases. The triad of ultrasound findings prenatally is ventral curvature of the penis, redundant dorsal foreskin and blunt distal penis. The identification of the urethra during the micturition and the direction of the urinary stream help in the classification of the defect. When severe hypospadias is detected, the recommendation is to perform genetic amniocentesis and search for other ultrasound findings related to poor virilization in the fetus, as testicular descent after 27 weeks of gestation. Postnatal follow up should be multidisciplinary including infantile urologist and endocrinologist. The prognosis in distal hypospadias is usually good following surgical repair, however in severe cases surgical interventions may be more challenging in order to obtain satisfactory outcome in terms of function and esthetic.

High grade persistent ventral curvature after multiple hypospadias surgery: how to correct?

ABSTRACT Introduction: A challenging situation in proximal hypospadias is the presentation of patients with successful urethroplasty but with persistent or recurrent ventral curvature (VC) after multiple hypospadias repair. Materials and Methods: We present a 13 year-old boy with 7 previous surgeries (long TIP, Duplay, meatoplasty) to treat hypospadias presenting with 60 degrees of VC, in spite of a well-accepted coronally neomeatus. We degloved the penis and artificial erection clearly appointed corporal disproportion causing curvature. We disconnected urethra from corpora. After excision of remnant fibrotic tissue, there was a residual curvature so a lenghtening corporoplasty with dermal graft from groin was performed. We have adjusted the urethral meatus position into a proximal penile shaft. We used a buccal mucosa graft placed in an inverted U-shape position planning a second stage urethroplasty (1). An indwelling silicone Foley tube was left for one week. The patient was discharged the day after surgery. Results: The aspect after corporoplasty proved satisfactory curvature correction. Patient had an excellent outcome and is scheduled for a second-stage after 6 months. Discussion: Snodgrass and Bush (2) reported that on 73 patients with an average of 2.7 operations for proximal shaft to perineal hypospadias; of which, 83% had VC at re-operation averaging 50°. We do believe that some good results with minimal dorsal plicature may recur in adolescence and therefore when these procedures may be considered, they should be performed by classic Nesbit technique (3). Otherwise, the choice for primary ventral lengthening should be taken. Conclusion: Severe curvature associated with hypospadias should undergo a major procedure at early stage to avoid decompensation after dorsal plicature in adolescence. We had a very satisfactory result, the patient awaits the second stage procedure (Figure-1). Figure 1 End aspect after complete healing of buccal mucosa. Notice that penis is straight and second stage will be performed after 6 months of interval.

Bilateral En-Block Horseshoe Kidney Laparoscopic Nephrectomy / Nefrectomía bilateral en bloque en riñón en herradura

Horseshoe kidney (HSK) has a prevalence of 1 in every 500 individuals. The management of patients with HSK is usually conservative, except in the presence of symptoms such as obstruction, stones, glomerulopathies, and tumors. In the following case report, we describe how a bilateral en-block transmesenteric laparoscopic nephrectomy in supine position was performed. A 5-year-old boy, with proximal hypospadias and early onset of chronic kidney disease due to focal segmental glomerulosclerosis on biopsy, underwent a genetic molecular evaluation that confirmed a pathogenic mutation at the WT-1 gene. Due to the increased risk of developing Wilms tumor, he underwent a bilateral transmesenteric nephrectomy. In a five-minute video, we describe how we performed an en-block transperitoneal and transmesenteric laparoscopic nephrectomy with special attention to patient positioning, including the feasibility of performing the dissection of the left renal hilum and isthmus with the patient in supine with no need for repositioning, and then moving to the dissection of the right renal hilum and completion of the procedure. The case herein reported enables us to describe the technical key-points to perform a bilateral en-block laparoscopic nephrectomy with shorter operative time and reduction of blood loss by preserving the entire specimen, without the need for an isthmus transection.

El riñón en herradura (RH) tiene una prevalencia de 1 en cada 500 individuos. El manejo del RH es usualmente conservador, excepto cuando genera síntomas como obstrucción, litiasis, glomerulopatías o tumores. Con este reporte de caso, describimos como se realizó una nefrectomia bilateral en bloque transmesentérica en un paciente con riñón en herradura. Un paciente de 5 años de edad, con hypospadias proximal y desarrollo temprano de enfermedad renal crónica por glomeruloesclerosis focal segmentaria, fue sometido a un estudio molecular que confirmó la presencia de una mutación en el gen WT-1. Dado el alto riesgo de desarrollar tumor de Wilms, se decidió realizar una nefrectomía transperitoneal laparoscópica bilateral. En un video de cinco minutos, describimos como se realizó una nefrectomía transperitoneal y transmesentérica en bloque, con especial atención al posicionamiento del paciente, incluso la viabilidad de realizar la disección del hilio renal izquierdo y el istmo con el paciente en supino, sin necesidad de reposicionarlo, y, después, la disección del hilio renal derecho y el fin del procedimiento. El caso reportado nos permite describir los puntos clave técnicos para realizar una nefrectomía laparoscópica bilateral en bloque con un tiempo operativo más corto y reducción del sangrado al preservar todo el espécimen, sin la necesidad de realizar la transección del istmo.

Creation of a Pilot Surgical Program for the Comprehensive Management of Patients with Congenital Urological Malformations / Creación de un programa quirúrgico piloto para el manejo integral de pacientes con malformaciones urológicas congénitas

Objectives Congenital malformations constitute the first cause of morbidity and mortality in childhood in Latin America. That is why, since 2001, a surveillance system for congenital malformations has been implemented in Bogota - Colombia. However, despite the increase in detection, an impact on treatment has not been achieved. The present study describes our experience with a novel social program focused on congenital urologic disorders. Methods The present manuscript is a retrospective observational study. We reviewed two national databases containing patients with congenital malformations. Patients were actively contacted to verify the status of the malformations. Children in whom the malformation was confirmed were offered a free consultation with a multidisciplinary group. After screening for surgical indications, patients were scheduled for surgery. Results Between November 2018 and December 2019, 60 patients were identified. In total 44, attended the consultation; the remaining did not attend due to financial or travel limitations. The most common condition assessed was hypospadias. In total, 29 patients underwent surgery. The total cost of care was of US$ 5,800. Conclusions Active search improves attention times and reduces the burden of disease. The limitations to be resolved include optimizing the transportation of patients and their families, which is a frequent limitation to access health care.

Objetivos Las malformaciones congénitas corresponden a la principal causa de morbimortalidad en la infancia en América Latina, motivo por el cual desde el 2001 se viene implementando un sistema de vigilancia epidemiológica de malformaciones congénitas en Bogotá, Colombia. Sin embargo, a pesar del aumento en la cobertura del reporte obligatorio, no se ha logrado un impacto sobre su tratamiento. Este estudio busca mostrar nuestra experiencia con un programa integral de pacientes con malformaciones urológicas congénitas. Métodos El presente es un estudio observacional retrospectivo. Los menores con malformaciones congénitas fueron identificados en dos bases de datos nacionales que incluyen pacientes con malformaciones congénitas. Los pacientes reportados fueron contactados telefónicamente para verificar el estado actual de la malformación. A aquellos niños en quienes se les confirmó la malformación, se les ofreció de manera gratuita una consulta con un grupo multidisciplinario. Una vez confirmadas las indicaciones quirúrgicas, fueron llevados a cirugía. Resultados Se identificaron 60 pacientes entre noviembre del 2018 y diciembre de 2019. De los pacientes identificados, 44 acudieron a consulta. Los demás no asistieron por limitaciones económicas. La principal condición valorada fue hipospadias. En total, 29 pacientes fueron llevados a cirugía. El costo total de la atención de estos pacientes fue de 22 millones de pesos colombianos. Conclusiones La búsqueda activa mejora los tiempos de atención y reduce la carga de la enfermedad. Una de las limitaciones aun por resolver es optimizar el transporte de los pacientes y sus familias, que resulta una limitación frecuente para el acceso a la salud.

Analysis of clinical phenotype and genotype of Chinese children with disorders of sex development / 中华儿科杂志

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.

Application of the Mathieu combined tunnel technique for repairing glans dehiscence after failed hypospadias repair / 亚洲男科学杂志(英文版)

Repairing glans dehiscence after failed hypospadias repair is challenging for pediatric surgeons. Here, we introduced and evaluated a newly modified Mathieu technique, Mathieu combined tunnel (MCT), which involves multiple custom-designed flaps for the shortage of flap source material after repeated operations; we also constructed a tunnel to avoid the glans incision that may carry new risks of dehiscence. This retrospective study included 26 patients who were consecutively admitted to the First Affiliated Hospital of Sun Yat-Sen University (Guangzhou, China) for glans dehiscence repair after failed hypospadias repair from October 2014 to October 2020; sixteen patients underwent surgery using the MCT (MCT group) and ten patients underwent surgery using the tubularized incised plate (TIP) technique (TIP group). The operative time, blood loss, postoperative complications, normal urethral meatus rate, success rate, and Hypospadias Objective Penile Evaluation (HOPE) score were compared between the two groups. The MCT group achieved an overall satisfactory penile appearance and voiding function, with a higher rate of normal urethral meatus (15/16, 93.8%) and a lower rate of glans dehiscence (1/16, 6.2%), compared with the TIP group (70.0% and 30.0%, respectively). However, these differences were not statistically significant, possibly because of the limited number of patients (all P > 0.05). Mean postoperative HOPE scores were similar in the MCT group (mean ± standard deviation: 8.83 ± 0. 89) and TIP group (8.94 ± 0.57) (P > 0.05). No significant differences were found between the two groups in terms of blood loss and success rate, nor in the rates of various complications (e.g., fistula, urethral stricture, and glans dehiscence). In conclusion, the MCT technique appears to be feasible and reliable for repairing glans dehiscence after failed hypospadias repair.

Geographical Distribution of Access to Healthcare in Patients Diagnosed with Hypospadias / Distribución geográfica del acceso a la asistencia sanitaria en pacientes con diagnóstico de hipospadias

Objective Hypospadias is a congenital disease of unknown etiology involving multiple epigenetic, genetic, and endocrinological factors. It is a highly incapacitating condition. Its surgical management is one of the most frequent surgical procedures done by pediatric urologists. Furthermore, the geographical distribution and healthcare access is limited in Colombia. The Colombian Ministry of Health has consolidated a nationwide registry called Integrated Social Protection Information System (SISPRO, in the Spanish acronym) to collect comprehensive information on the use and frequency of resources associated with health care in Colombia. The aim of the present study was to analyze the number of cases reported between 2014 and 2018 and the geographical distribution of access to healthcare of patients with hypospadias in Colombia. Methods An observational, retrospective study of hypospadias in Colombia, 2014­ 2018, was performed using data extracted from the Individual Health Records System (RIPS) in SISPRO. Satscan, version 9.6 was used to perform a distribution analysis of the georeferenced population using a Poisson model. To visualize the results, the software projected the result onto a Google Earth map. Results Between January 2014 and December 2018, a total of 8,990 cases of hypospadias were evaluated in Colombia. The geographical distribution in the national territory has areas with high evaluation rates. On average, the departments in which the majority of cases were evaluated during the study period were Bogotá, D.C., Antioquia, and Valle del Cauca (2,196, 1,818 and 1,151 cases, respectively). The statistical analysis of the space exploration (►Fig. 1) identified the area with the highest concentration of cases (red) and the areas in which the lowest number of patients was evaluated (blue). The geographical distribution showed increasing trends in areas near the center of the country, especially in the cities of Bogotá, Cali, Ibagué, and Pereira. Conclusion There is a greater concentration of cases evaluated in the center of the country, where the cities with better access to subspecialized medical care are located. This highlights inequalities in health services and the opportunity for surgical care among regions of the country. If we consider that the prevalence rates of hypospadias remain stable, 87% of the patients with hypospadias will not be evaluated by a subspecialist.

Objetivo Hipospadias es una enfermedad congénita de etiología desconocida que involucra múltiples factores epigenéticos, genéticos y endocrinológicos. Es una condición sumamente incapacitante. Su manejo quirúrgico es uno de los procedimientos quirúrgicos más frecuentes realizados por urólogos pediátricos. Además, la distribución geográfica y el acceso a la atención médica son limitados en Colombia. El Ministerio de Salud de Colombia ha consolidado un registro a nivel nacional denominado Sistema Integrado de Información de Protección Social (SISPRO) para recopilar información integral sobre el uso y frecuencia de los recursos asociados a la atención de la salud en Colombia. El objetivo del presente estudio fue analizar el número de casos notificados entre 2014 y 2018 y la distribución geográfica del acceso a la atención médica por los pacientes con hipospadias en Colombia. Métodos Se realizó un estudio observacional y retrospectivo de hipospadias en Colombia, 2014-2018, utilizando datos extraídos del Sistema de Registros Sanitarios Individuales (RIPS) en SISPRO. Se usó Satscan, versión 9.6 para realizar un análisis de distribución de la población georreferenciada usando un modelo de Poisson. Para visualizar los resultados, el software proyectó el resultado en un mapa de Google Earth. Resultados Entre enero de 2014 y diciembre de 2018, se evaluaron un total de 8.990 casos de hipospadias en Colombia. La distribución geográfica en el territorio nacional tiene áreas con mayor concentración de la atención de pacientes con hipospadias, al igual que áreas sin atención de esta condicion. En promedio, los departamentos donde se evaluaron la mayoría de los casos durante el período de estudio fueron Bogotá, D.C., Antioquia, y Valle del Cauca (439.2, 363.6, y 230.2, respectivamente). El análisis estadístico de la exploración espacial ([Figura 1]) identificó el área con la mayor concentración de casos (rojo) y las áreas donde se evaluó el menor número de pacientes (azul). La distribución geográfica mostró tendencias crecientes en áreas cercanas al centro del país, especialmente en las ciudades de Bogotá, Cali, Ibagué y Pereira. Conclusiones Existe una mayor concentración de casos evaluados en el centro del país, donde se encuentran las ciudades con un mejor acceso a atención médica subespecializada. Esto pone de relieve las desigualdades en el acceso a los servicios de salud y la oportunidad de atención quirúrgica entre las regiones del país. Si consideramos que las tasas de prevalencia de hipospadias permanecen estables, aproximadamente el 87% de los pacientes con hipospadias no serán evaluados por un subespecialista.

A new option to prevent fistulas in anterior urethroplasty in patients with kippered urethra: the tunica vaginalis flap

ABSTRACT The objective of this study is describing a technique with the use of a tunica vaginalis flap (TVF) to cover the suture line during anterior urethroplasty in patients with kippered urethra due to chronic indwelling catheterization (CIC). We studied 5 patients (mean age=50.2) with a neurogenic bladder that developed urethral erosion after a long period of CIC. Foley catheter was removed on the 14th postoperative day. One patient developed wound infection and utethrocutaneous fistula, which was conservatively managed and after 12 months of follow-up all the patients didn't report difficulties in intermittent self-catheterization. In conclusion, a urethroplasty with TVF technique may be a viable method for repairing penile urethral erosions, but further studies are required with a bigger sample to confirm our results.

Does parental opinion differ from the health care team regarding cosmesis after hypospadias repair?

SUMMARY OBJECTIVE: Hypospadias is the most common malformation of the male genitalia. Surgical correction has traditionally focused on anatomic and functional outcomes, with less attention being paid to cosmetic results. Our purpose is to compare the cosmetic results of hypospadias repair among different groups of observers, namely the patient's family and the health care team, using photography and a simple rating scale. METHODS: Prospective observational study included 9 boys undergoing Snodgrass hypospadias repair. Photographs of the penis taken before, immediately after, and six months after surgery were assessed by a panel of 15 observers (parents and health care team) and a scale including three questions with diagrams for comparison with the pictures was used. Observers also assigned an overall postoperative score for the cosmetic result. RESULTS: Interobserver agreement was noted for the group of parents of other children with hypospadias regarding the shape of the glans (k=0.404; p=0.008) and for the group of pediatric surgeons regarding the degree of residual curvature (k=0.467; p=0.005). Two observers in the pediatrician group have indicated good performance in the assessment of residual curvature (k=0.609; P=0.024). In the overall assessment of cosmetic outcomes, the highest scores were assigned by observers in the parents group and in the pediatrician group, while the pediatric surgeons group has one of the lowest scores (p<0.001). CONCLUSIONS: Photography appears to be suitable for documenting corrections of hypospadias regarding penile curvature, and postoperative cosmetic result. Surgeons seem more concerned about cosmesis than parents.

Clinical evaluation of Compound Chamomile and Lidocaine Hydrochloride Gel for postoperative hypospadias in children / 中华男科学杂志

Objective@#To evaluate the clinical efficacy of Compound Chamomile and Lidocaine Hydrochloride Gel for postoperative hypospadias in children.@*METHODS@#From January to December 2020, we treated 116 children with distal hypospadias in the Department of Urology, Department of Pediatrics and the Seventh Medical Center of the PLA General Hospital, 58 by primary Snodgrass urethroplasty only (the control group) and the other 58 with Compound Chamomile and Lidocaine Hydrochloride Gel smeared on the penis postoperatively in addition (the trial group). We compared the operation time and postoperative pain score, edema regression and incidence of infection between the two groups, followed by statistical analysis using T test and Chi-square test.@*RESULTS@#All the operations were successfully completed by the same surgeon under general anesthesia. There were no statistically significant differences between the trial and control groups in age (［2.5 ± 0.8］ vs ［2.4 ± 0.6］ yr, P > 0.05) or operation time (［95.6 ± 14.5］ vs ［97.1 ± 15.2］ min, P > 0.05). No incision infection occurred in any of the cases. The pain scores at dressing removal were remarkably lower in the trial than in the control group at 2 hours (1.4 ± 1.0 vs 2.6 ± 1.3, P < 0.05), 24 hours (2.2 ± 1.3 vs 3.9 ± 1.6, P < 0.05), 48 hours (1.2 ± 0.7 vs 1.6 ± 0.9, P < 0.05) and 72 hours after surgery (2.5 ± 0.8 vs 3.7 ± 1.8, P < 0.05). Significantly more cases of edema regression were achieved in the trial than in the control group at 2 weeks postoperatively (35 vs 19, P < 0.05).@*CONCLUSIONS@#Compound Chamomile and Lidocaine Hydrochloride Gel can effectively relieve pain, reduce edema and accelerate edema regression after surgery in children with hypospadias, and therefore deserves wide clinical application.、.

Identification of a novel variant of SRD5A2 gene in a child featuring steroid 5α-reductase type 2 deficiency / 中华医学遗传学杂志

OBJECTIVE@#To explore the clinical characteristics and genetic basis of a child with 5α-reductase type 2 deficiency.@*METHODS@#Clinical data of the child was retrospectively analyzed. Targeted capture-next generation sequencing and Sanger sequencing were carried out to detect potential variants.@*RESULTS@#The patient's main features included micropenis and hypospadia. He was found to harbor compound heterozygous c.680G>A (p.R227Q) and c.3G>T (p.M1I) variants of the SRD5A2 gene. Among these, c.680G>A (p.R227Q) was inherited from his father and was a known pathogenic mutation, while c.3G>T (p.M1I) was inherited from his mother and was unreported previously.@*CONCLUSION@#The compound heterozygous variants of the SRD5A2 gene probably underlay the disease in this child, who was eventually diagnosed with 5α-reductase 2 deficiency.

Laringoespasmo reflejo en cirugía urológica pediátrica / Reflex laryngospasm in pediatric urologic surgery

Introducción: El laringoespasmo es una complicación temida por los anestesiólogos. Se asocia a broncoespasmo, hipoxia, arritmias, aspiración del contenido gástrico y paro cardiaco. Es más frecuente en neonatos y lactantes. Está estrechamente vinculado al tipo de cirugía y consiste en un cierre intenso y prolongado de la glotis en respuesta a la estimulación glótica directa o refleja. Objetivo: Describir el tratamiento del laringoespasmo parcial reflejo en un paciente pediátrico durante una intervención urológica. Presentación del caso: Paciente de 18 meses de edad al cual se le realiza meatotomía. Durante la cirugía presenta laringoespasmo parcial reflejo tratado con maniobras físicas. Sin la necesidad de usar medicamento ni vía área mecánica. Conclusiones: Las maniobras utilizadas son seguras y efectivas en el tratamiento del laringoespasmo reflejo sin la necesidad de abordar la vía aérea ni uso de medicamentos, se lograron resultados satisfactorios con una relación riesgo beneficio a favor del paciente(AU)

Introduction: Laryngospasm is a complication feared by anesthesiologists. It is associated with bronchospasm, hypoxia, arrhythmias, aspiration of gastric contents, and cardiac arrest. It is more common among neonates and infants. It is closely related to the type of surgery and consists in an intense and prolonged closure of the glottis in response to direct or reflex glottic stimulation. Objective: To describe the management of reflex partial laryngospasm in a pediatric patient during a urological intervention. Case presentation: 18-month-old patient who received meatotomy. During surgery, he presented reflex partial laryngospasm managed with physical maneuvers, without the need to use medication or the mechanic airways ventilation. Conclusions: The maneuvers used are safe and effective for the management of reflex laryngospasm without the need to address the airway or using of medications. Satisfactory outcomes were achieved with a risk-benefit ratio in favor of the patient(AU)

Vacuum physiotherapy after first stage buccal mucosa graft (BMG) urethroplasty in children with proximal hypospadias

ABSTRACT Introduction To assess the feasibility of vacuum physiotherapy meant to decrease graft contraction and recurrent penile curvature (PC), hence successful tubularization and a straight penis in patients underwent two-stage buccal mucosa graft (BMG) urethroplasty, in proximal hypospadias repair. Material and methods Between January 2014 and July 2018, 59 two-stage BMG urethroplasties performed at our referral center, were included in the study. The parents were counseled to use the vacuum device between the two stages. An internal, self-administered, semiquantitative, non-validated questionnaire was designed to record parent and patient adherence to the vacuum physiotherapy and parent satisfaction. Success rate of graft tubularization, curvature correction rates, and status of early (4 months) postoperative urinary stream were evaluated. Results Of 45/59 (76.3%) who returned the questionnaire, 77.8% followed the recommended physiotherapy protocol using the vacuum device. 93.3% of parents replied that the use of the vacuum was easy or moderately easy. None of the parents interrupted the physiotherapy because of perceived difficulty or intolerability. 100% of parents would have repeated the physiotherapy, if they had to. Overall, success rate of tubularization was 98.3% (58/59), complete curvature correction was achieved in 88.2% (52/59) of patients, and 79.7% (47/59) of patients showed a straight and powerful early post-operative urinary stream. Conclusions Physiotherapy with the vacuum device is safe, easy and practically feasible. Our vacuum physiotherapy protocol had high compliance rate. Vacuum physiotherapy should be considered for further assessment in patients undergoing two stage hypospadias repair using buccal mucosa.

The GUD technique: glandar urethral disassembly. An alternative for distal hypospadias repair

ABSTRACT Introduction We present an alternative procedure for distal hypospadias consisting of urethral mobilization and partial glandar disassembly, namely GUD (glandar urethral disassembly) technique. Materials and Methods A subcoronal circumcision exposes distal dysplastic urethra. We incise the Buck´s fascia on both sides of urethra releasing it partially from the corpora. We keep a thin bridge of urethral plate to the glans and disassembly almost completely the glans from the corpora, except for the bridge. The glans is incised creating two wide wings that are extremely mobile. The urethra is mobilized, advanced and sutured to the tip of the glans. The glans wings embrace the distal urethra producing a conical glans. Discussion The concept of urethral mobilization has been reported and popularized by Koff in the literature to correct distal hypospadias. One of the limitations of this procedure is the risk of urethral retraction due to extensive proximal dissection. We got inspiration from Mitchell and Bagli' s work of penile disassembly in epispadias to develop the GUD concept. We adopt minimal urethral mobilization mainly in glandar/proximal penile shaft and complete deconstruction of the glans, detaching the corpora from the glans and rotating the wide glans wings to embrace the urethra. Therefore we avoid suture urethroplasty and refurbish the glans to a better conical shape. Conclusion We are convinced that this operation can be regarded as a genuine alternative to distal hypospadias (coronal and subcoronal) but should not be addressed to midshaft forms.

Comparison of continuous eversion and inverting subepithelial suture in transverse preputial island flap urethroplasty in proximal hypospadias repair: A retrospective study

ABSTRACT Introduction: Transverse preputial island flap urethroplasty (TPIFU) is one of the most frequently performed technique for single-stage repair in proximal hypospadias. It was reported that the subepithelial urethroplasty would obviously decrease urethrocutaneous fistula (UF) complication after proximal TIP. But in the process of TPIFU, it had not been reported yet. Objective: We reviewed our experience to evaluate and compare the effect of continuous eversion suture (CES) versus continuous inversion subepithelial suture (CIS) on complication rates in the TPIFU. Material and methods: A retrospective review of all patients operated with CES and CIS in our institution between January 2017 and Jun 2017 was performed. Results: A total of 161 patients were enrolled in the research. Patients were followed up for 12~17 months. Total success rate was 73.9% (119/161). No statistically difference was found between the two groups with regard to age of patients (P=0.097), catheter size (P=0.52), time of catheterization (P=0.47), length of neourethra (P=0.20), non-urethral comorbidity (P=0.44) and post-operative infection (P=1.0). The overall postoperative complications had no statistically difference between the two groups (P=0.067). There were no statistically significant differences in the incidence of urethra-cutaneous fistula (UF) (OR=0.07, 95% CI: -0.24~0.037, P=0.22), urethral diverticulum (UD) (OR=0.026, 95% CI: -0.16~-0.056, P=0.323), urethral stricture (US) (OR=0.081, 95% CI: -0.15~0.15, P=1.0) and breakdown of urethral repair (BU) (OR=0.02, 95% CI: -0.118~-0.044, P=1.0). Discussion: The comparison of two group's postoperative complications was feasible because there were no statistically differences among perioperative variables. It seemed as if continuous inversion subepithelial suture would promote healing. However, it indicated that the overall success rate and the incidences of UF, UD, US and BU complications had no statistically difference between groups. It might be accounted for the subtle differences of techniques changing the process of establishing prime and side branches vascularization. Conclusions: The CIS technique had no significantly different effect on the four complications rates when compared with CES in TPIFU. Thus, CES and CIS could be randomly adopted in TPIFU as personal preference.

Effects of spongioplasty on neourethral function following hypospadias repair: an experimental study in rabbits

ABSTRACT Purpose: Spongioplasty (mobilization and midline approximation of the two branches of the bifid dysplastic distal corpus spongiosum) can form a covering layer for the neourethra to prevent urethrocutaneous fistula in hypospadias repair surgery. However, it remains unclear whether spongioplasty affects neourethral function. The objective of this study was to compare neourethral function after hypospadias repair with and without spongioplasty. Materials and Methods: Fourteen congenital hypospadiac New Zealand male rabbits were randomly allocated into two groups, seven animals underwent Duplay hypospadias repair and spongioplasty (experimental group), while seven underwent Duplay surgery alone (control group). Functional differences between groups were assessed by comparing neourethral compliance and flow rate. Two months after surgery, in vivo neourethral compliance was assessed by measuring intraluminal pressure with a digital pressure meter of an isolated neourethral segment, following progressive distension with 1, 2, and 3mL of air. Penises were harvested for uroflowmetry test using a simple device. Results: Postoperatively, fistula developed in one and zero rabbits in the control and experimental groups, respectively. Mean pressures tended to be higher in the experimental group than in the control group (82.14 vs. 69.57, 188.43 vs. 143.26, and 244.71 vs. 186.29mmHg for 1, 2, and 3mL of air, respectively), but the difference was not statistically significant. Mean flow rates also did not significantly differ between the experimental and control groups (2.93mL/s vs. 3.31mL/s). Conclusion: In this congenital rabbit model, no obvious functional differences were found between reconstructed urethras after hypospadias repair with and without spongioplasty.

Variant analysis on steroid 5-reductase type 2 deficiency caused by a novel SRD5A2 mutation / 中国当代儿科杂志

This article reported the clinical characteristics and SRD5A2 gene mutation pattern of a child with steroid 5-α reductase type 2 deficiency. The 2-month-old boy showed hypospadias and short penis shortly after birth. DNA was extracted from the peripheral blood of the child and his parents. The endocrine disease-related genes were captured and sequenced by high-throughput sequencing technology, and the family DNA samples were verified by Sanger sequencing. The results showed that c.680G>A(p.R227Q) and c.608G>A(p.G203D) compound heterozygous mutations existed in the SRD5A2 gene of the child. The c.680G>A mutation inherited from his father, which was a known pathogenic mutation. The c.608G>A mutation originated from his mother, which was a novel mutation discovered in this study. These results provide molecular evidence for the etiological diagnosis of the child and genetic counseling for the family, as well as extend the mutation spectrum of SRD5A2 gene.

Our tubularized incised plate urethroplasty Repair. Results for Hypospadias Surgery

Background: The Tubularized Incised Urethral Plate (TIP) urethroplasty is a widely accepted technique among urologists and heavily used in their centers. This technique was developed by Snodgrass in 1994 for repairing varied degrees of hypospadias. Aim: This paper aims at analyzing and evaluating our early experience and the outcomes of using TIP urethroplasty technique. Therefore, the results have to be assessed in a large series. Methods: In this prospective study, 95 patients with varying hypospadiac meatus levels who experienced the procedure of TIP. During the entire sampling period, patients had varying degrees of hypospadias ranging from glanular to penoscrotal (glanular 2, coronal/subcoronal 37, distal shaft 30, mid-shaft 10, proximal shaft 5 and penoscrotal 11). Results: The overall success rate of TIP procedure repair of hypospadias was 87.4%; whereas the overall reoperation rate was 12.6%. The highest percentage was for those with failed previous repair (secondary). The average duration of the procedure was 58.6±18.4 minutes. Nearly a third of the patients developed one or more postoperative major complication. The total rate of fistula was 12.6%, with "10.6% in primary distal, 15.9% in primary proximal and 20% in secondary repair". Sixteen cases had a mild degree of "meatal stenosis" (16.8%), but all were managed by simple dilatation at the office or at home using 8 F feedingtube. Conclusion: The outcomes demonstrated that the TIP procedure is a quick, safe and reliable technique. Additionally, it can provide excellent cosmoses and function with few complications and acceptable reoperation rate.