Expert classification of hypospadias: an external validation and evaluation of agreement for Glans-Urethral Meatus-Shaft (GMS) and Hypospadias Objective Penile Evaluation (HOPE) scores.

PURPOSE: This study evaluates the inter-rater agreements of both the Glans-Urethral Meatus-Shaft (GMS) hypospadias score and Hypospadias Objective Penile Evaluation (HOPE) score, aiming to standardize disease classification for consistent agreement in clinically relevant characteristics of hypospadias. METHODS: Photos of hypospadias in children were collected from two separate institutions. Three raters scored the photos using GMS and HOPE, excluding penile torsion and curvature assessment in HOPE due to photo limitations. RESULTS: A total of 528 photos were included. With GMS, Fleiss' multi-rater kappa showed an agreement of 0.745 for glans-urethral plate, 0.869 for meatus, and 0.745 for shaft. For HOPE scores, the agreements were 0.888 for position of meatus, 0.669 for shape of meatus, 0.730 for shape of glans, and 0.708 for the shape of the skin. The lower agreement in the shape of the meatus evaluation may be attributed to the lack of a quantitative classification method in HOPE. Experts rely on their subjective judgment based on the provided example photos and their index patient. CONCLUSIONS: While there is high agreement among experts when evaluating hypospadias using the GMS and HOPE scoring criteria, only the position of the meatus achieved nearly perfect agreement highlighting that the current scoring systems entail a subjective element in disease classification.

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.

Analysis of the social and clinical factors affecting the age of children when receiving surgery for hypospadias: a retrospective study of 1611 cases in a single center.

We aimed to explore the associations between the age at which children undergo surgery for hypospadias and a range of social and clinical factors in a single center. Our aim was to promote the early surgical treatment of children with hypospadias. For a 6-year period, social and clinical data were collected from all children undergoing surgery to repair hypospadias in Children's Hospital of Chongqing Medical University (Chongqing, China), located in southwest of China. We analyzed the correlations between age at surgery and a range of social and clinical factors. A total of 1611 eligible cases were recruited, with a mean age of 54.3 months and a median age of 42 months: 234 cases (14.5%) were classified into a "timely operation" group, 419 (26.0%) cases into a "subtimely operation" group, and 958 (59.5%) cases into a "delayed operation" group. According to multivariate regression analyses, the higher the regional economic level, the closer the urethral opening to the perineum, and the higher the educational level of the guardians was, the younger the children were when they underwent the initial surgery for hypospadias; this was also the case for families without other children. Our subgroup analysis showed that the primary educational level of the guardians was a risk factor for subtimely surgery in their children (odds ratio [OR] = 1.52, 95% confidence interval [CI]: 1.08-2.15, P < 0.05). A lower regional economic level (OR = 1.87, 95% CI: 1.26-2.78, P < 0.01), a lower educational level of the guardians (OR = 3.84, 95% CI: 2.31-6.41, P < 0.01), and an anterior-segment urethral opening (OR1 [vs middle hypospadias] = 2.07, 95% CI: 1.42-3.03; OR2 [vs posterior hypospadias] = 2.63, 95% CI: 1.75-3.95; P < 0.01) were all risk factors for delayed surgery in children.

Spongiosum-combined glanuloplasty reduces glans complications after proximal hypospadias repair.

We aim to design a new glanuloplasty procedure combined with spongiosum to reduce the incidence of glans dehiscence and coronal fistula after proximal hypospadias repair. Patients who underwent urethroplasty by dorsal preputial island flap for proximal hypospadias between January 2014 and December 2016 were reviewed in this retrospective cohort study. Those who underwent spongiosum-combined glanuloplasty comprised the new-maneuver group, whereas those who underwent conventional glanuloplasty comprised the control group. The incidence of complications was then compared. In the new-maneuver group, dysplastic corpus spongiosum alongside lateral Buck's fascia (0.3-0.4 cm wide) on both sides of the urethral plate was separated from the proximal normal spongy tissue, joining into the glans wings to increase tissue volume and covering the neourethra in the glans penis. In the control group, the neourethra was covered with superficial fascia under the coronal sulcus. As a result, the new-maneuver and control groups comprised 47 and 28 patients, respectively. In the new-maneuver group, no glans dehiscence was detected; however, two (4.3%) patients had coronal fistula, two (4.3%) had urethral stricture, and four (8.5%) had diverticulum. In the control group, two (7.1%) patients had glans dehiscence, eight (28.6%) had coronal fistula, four (14.3%) had urethral stricture, one (3.6%) had diverticulum, and one (3.6%) had penile curvature recurrence. The new-maneuver group had less incidences of coronal fistula (P < 0.001), glans dehiscence (P = 0.033), and urethral stricture (P = 0.008) but had a higher incidence of diverticulum than the control group (P = 0.040). It clearly demonstrates that spongiosum-combined glanuloplasty can significantly reduce the incidences of coronal fistula and glans dehisce.

Digital Pattern Recognition for the Identification and Classification of Hypospadias Using Artificial Intelligence vs Experienced Pediatric Urologist.

OBJECTIVE: To improve hypospadias classification system, we hereby, show the use of machine learning/image recognition to increase objectivity of hypospadias recognition and classification. Hypospadias anatomical variables such as meatal location, quality of urethral plate, glans size, and ventral curvature have been identified as predictors for postoperative outcomes but there is still significant subjectivity between evaluators. MATERIALS AND METHODS: A hypospadias image database with 1169 anonymized images (837 distal and 332 proximal) was used. Images were standardized (ventral aspect of the penis including the glans, shaft, and scrotum) and classified into distal or proximal and uploaded for training with TensorFlow. Data from the training were outputted to TensorBoard, to assess for the loss function. The model was then run on a set of 29 "Test" images randomly selected. Same set of images were distributed among expert clinicians in pediatric urology. Inter- and intrarater analyses were performed using Fleiss Kappa statistical analysis using the same 29 images shown to the algorithm. RESULTS: After training with 627 images, detection accuracy was 60%. With1169 images, accuracy increased to 90%. Inter-rater analysis among expert pediatric urologists was k= 0.86 and intrarater 0.74. Image recognition model emulates the almost perfect inter-rater agreement between experts. CONCLUSION: Our model emulates expert human classification of patients with distal/proximal hypospadias. Future applicability will be on standardizing the use of these technologies and their clinical applicability. The ability of using variables different than only anatomical will feed deep learning algorithms and possibly better assessments and predictions for surgical outcomes.

Development of a prediction model for postoperative complications after primary hypospadias correction.

PURPOSE: To develop a prediction model for postoperative complications after primary one-stage hypospadias correction to improve preoperative parental counseling. MATERIALS AND METHODS: In this retrospective cohort study, data were collected from 356 patients with anterior or middle hypospadias who had a one-stage hypospadias correction from 2003 onwards. Potential treatment- and patient-related factors were selected and used to develop a prediction model for postoperative complications within one year (wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications). Multivariable logistic regression analysis with stepwise backward selection and a p-value of 0.20 was used to select the final model, which was internally validated using the bootstrap procedure. RESULTS: Complications within one year postoperatively occurred in 66 patients (19%), of which 13% and 37% were seen in anterior and middle type of hypospadias, respectively. Hypospadias phenotype, surgical technique, chordectomy, and surgeon's experience were included in the final prediction model, whereas none of the patient-related factors were. The final model had a good discriminative ability (bias corrected C statistic 0.70) and calibration. CONCLUSION: Using easily obtainable information, this model showed good accuracy in predicting complications within one year after hypospadias surgery. It is a first step towards individualized risk prediction of postoperative complications for anterior and middle hypospadias and can assist in preoperative parental counseling. TYPE OF STUDY: Prognostic study. LEVEL OF EVIDENCE: Level II.

The current state of tissue engineering in the management of hypospadias.

Hypospadias is a congenital malformation resulting from the disruption of normal urethral formation with varying global prevalence. Hypospadias repair, especially that of proximal hypospadias (in which reconstruction of a long urethra is necessary), remains a surgical challenge despite more than two decades of surgical technique development and refinement. The lack of tissue substitutes with mechanical and biological properties similar to those of native urethra is a challenge for which the field of tissue engineering might offer promising solutions. However, the use of tissue-engineered constructs in preclinical studies is still hindered by complications such as strictures or fistulae, which have slowed progression to clinical application. Furthermore, the generation of uniform tubular constructs remains a challenge. Exciting advances in the application of nanotechnology and 3D bioprinting to urethral tissue engineering might present solutions to these issues.

Distal Urethroplasty and Glanuloplasty Procedure Can be Suitable for All Types of Glanular/Subcoronal Hypospadias.

OBJECTIVE: To correct all types of glanular/subcoronal hypospadias, we performed surgery named the distal urethroplasty and glanuloplasty procedure (DUG procedure). We analyzed cases that we have experienced. METHODS: A vertical incision with the meatal margin was made in the 12 o'clock direction, with the margin of the external urethral meatus as the center. By this meatoplasty according to Heineke-Mikulicz principle, we changed all type of glanular/subcoronal hypospadias to the hypospadias with wide meatus and wide glans. And then Thiersh-Duplay procedure was performed. RESULTS: Consecutive 24 underwent modified DUG procedure. The mean age at the time of surgery was 19.0 ± 11.9 months and the mean preoperative glans width was 13 ± 1.5 mm. The external urethral meatus was located glanular in 13 and subcoronal in 11. Hypoplastic urethra (HU) was observed in 7. Skin chordee in 10, penile torsion in 6 and meatal stenosis in 10 were observed. The mean surgical duration was 106 ± 25.4 minutes and the mean postoperative observation period was 40.5 ± 26.2 months. All patients with preoperative skin chordee, penile torsion, and meatal stenosis were improved postoperatively, and in all cases, apart from the patient with meatal regression with longest HU from glanular to distal penile, the slit-like shape of the external urethral meatus was achieved. CONCLUSION: DUG procedure can be used for any type of glanular/subcoronal hypospadias but care should be taken not to indicate too aggressively for glanular/subcoronal hypospadias accompanying long HU to distal penile shaft.

[Hypospadias : Insights and challenges]. / Hypospadie : Erkenntnisse und Herausforderungen.

Disorders of the ventral tubularization of the urethra, such as the hypospadias, are among the second most frequent congenital childhood malformations. An increasing incidence has been observed suggesting a doubling in the US, which could not be documented for the European area. The underlying causes of this congenital defect remain unidentified. Genetic risk constellations or environmental influences, in particular by so-called endocrine disrupting chemicals (EDCs), are discussed as triggering factors. Boys after in vitro fertilization are more likely to have hypospadias than in nonreproductive-assisted pregnancies. Animal models (especially mice) elicited causal relationships between prenatal hormonal exposure (estrogens, progesterone) and antiandrogens such as flutamide, finasteride, antiandrogenic fungicides (vinclozolin) and phthalates and the formation of hypospadias. An aesthetic and/or functional deficit are indications for surgical correction. The indications and the complications of hypospadias surgery must be in detail and realistically discussed with the parents and patients. Recent publications demonstrated that the risk of complications increases with the increase of the follow-up time. High-volume centers with extensive experience have a positive effect on the complication rate. Competent follow-up to adult age should be ensured.

[Risk factors for different types of hypospadias].

OBJECTIVE: To explore the risk factors for different types of hypospadias. METHODS: According to the 1∶1 ratio, we included hypospadias children in the case group and those without urinary abnormality as controls, all from the Third Affiliated Hospital of Zhengzhou University between October 2015 to October 2016. Using univariate and multivariate logistic regression analyses, we investigated the risk factors for hypospadias as well as for four different types of the disease. RESULTS: Among the 440 subjects, the risk factors for hypospadias included preterm birth, fetal growth restriction, rural residence of the mother, pregnancy age <20 or >35 years, primipara, maternal smoking (including passive smoking), oral progesterone, cold or fever during pregnancy, and exposure to high temperature in early pregnancy, while the protective factors included protein supplement in early pregnancy. The pregnancy age <20 or >35 years was the main risk factor for type I hypospadias; preterm birth, fetal growth restriction, rural residence of the mother, primipara, and maternal smoking (including passive smoking) during pregnancy were the risk factors for type Ⅱ; preterm birth, fetal growth restriction, rural residence of the mother, and exposure to high temperature in early pregnancy were those for type Ⅲ; and exposure to high temperature in early pregnancy and oral progesterone during pregnancy were those for type Ⅳ. CONCLUSIONS: The risk factors for hypospadias vary for different types, and therefore hypospadias-related clinical studies should be conducted and preventive measures should be taken accordingly. However, a larger sample size is needed to get more scientific and reliable results concerning the risk factors for different types of hypospadias.

[Expression of ZEB1 in the prepuce of hypospadias children and its implication].

OBJECTIVE: To investigate the expression of zinc finger E-box binding homebox 1 (ZEB1) in the prepuce of hypospadias children and its relationship to the incidence of hypospadias. METHODS: Prepuce tissues were collected from 37 children aged 6-15 months undergoing hypospadias repair and 11 age-matched controls receiving circumcision. Based on the position of the urethral meatus, the hypospadias cases were classified as severe (n = 13) and mild-moderate (n = 24). The mRNA and protein expressions of ZEB1 were determined by immunohistochemistry and RT-PCR. RESULTS: The expression of the ZEB1 protein was remarkably higher in the severe (100% [13/13]) and mild-moderate hypospadias patients (75.0% [18/24]) than in the controls (9.1% [1/11]), with statistically significant differences between any two groups (P < 0.05). RT-PCR showed the integrated density value (IDV) of the ZEB1 mRNA expression to be (0.67 ± 0.21), (0.81 ± 0.24), and (1.55 ± 0.29) in the control, mild-moderate, and severe hypospadias patients, respectively, significantly higher in the severe hypospadias than in the control and mild-moderate hypospadias groups (P < 0.05), but with no significant difference between the latter two (P = 0.64). CONCLUSION: The expression of ZEB1 is significantly increased in hypospadias patients, and its upregulation is positively correlated with the severity of hypospadias, which suggests that the overexpression of ZEB1 may contribute to the development of hypospadias.

Repair of megameatus: a modified approach.

OBJECTIVE: To demonstrate surgical reconstruction of a megameatus variant of hypospadias by using a modified, patient-specific approach. METHODS: An 8-month-old male had a megameatus, which was discovered after newborn circumcision. In repair of megameatus, it is important to recognize the full extent to which the distal urethra is widened, in order to avoid inadvertent urethrotomy during mobilization of the glans wings and skin immediately proximal to the glans. In this case, a small wedge of tissue was excised from the ventral aspect of the urethra, and a midline relaxing incision of the urethral plate was made. Standard principles of hypospadias repair should be followed, with tension-free urethral tubularization, coverage of the neourethra with well-vascularized tissue, and adequate mobilization of the glans wings to allow midline reconstruction without compression of the underlying neourethra. RESULTS: There were no perioperative or postoperative complications in this case. The urethral meatus was positioned near the tip of the glans penis, and there was no evidence of meatal stenosis or urethrocutaneous fistula at 5-months follow-up. CONCLUSION: Each case of megameatus is unique, and a variety of surgical techniques can be used in formulating an approach that achieves optimal cosmetic and functional outcomes.

Further analysis of the Glans-Urethral Meatus-Shaft (GMS) hypospadias score: correlation with postoperative complications.

INTRODUCTION AND OBJECTIVE: The Glans-Urethral Meatus-Shaft (GMS) score is a concise and reproducible way to describe hypospadias severity. We classified boys undergoing primary hypospadias repair to determine the correlation between GMS score and postoperative complications. STUDY DESIGN: Between February 2011 and August 2013, patients undergoing primary hypospadias repair were prospectively scored using the GMS classification. GMS scoring included a 1-4 scale for each component: G - glans size/urethral plate quality, M - meatal location, and S - degree of shaft curvature, with more unfavorable characteristics assigned higher scores [Figure]. Demographics, repair type, and complications (urethrocutaneous fistula, meatal stenosis, glans dehiscence, phimosis, recurrent chordee and stricture) were assessed. Total and individual component scores were tested in uni- and multivariate analysis. RESULTS: Two-hundred and sixty-two boys (mean age 12.3 ± 13.7 months) undergoing primary hypospadias repair had a GMS score assigned. Mean GMS score was 7 ± 2.5 (G 2.1 ± 0.9, M 2.4 ± 1, S 2.4 ± 1). Mean clinical follow-up was 17.7 ± 9.3 months. Thirty-seven children (14.1%) had 45 complications. A significant relationship between the total GMS score and presence of any complication (p < 0.001) was observed; for every unit increase in GMS score the odds of any postoperative complication increased 1.44 times (95% CI, 1.24-1.68). Urethrocutaneuous fistula was the most common complication, occurring in 21 of 239 (8.8%) of single-stage repairs. Patients with mild hypospadias (GMS 3-6) had a 2.4% fistula rate vs. 11.1% for moderate (GMS 7-9) and 22.6% for severe (GMS 10-12) hypospadias (p < 0.001). Degree of chordee was an independent predictor of fistula on multivariate analysis; S4 (>60° ventral curvature) patients were 27 times more likely to develop a fistula than S1 (no curvature) boys (95% CI, 3.2-229). DISCUSSION: The GMS score is based on anatomic features (i.e. glans size/urethral plate quality, location of meatus, and degree of chordee) felt to most likely impact functional and cosmetic outcomes following hypospadias repair. We demonstrated a statistically significant increase in the likelihood of any postoperative complication with every unit increase in total GMS score. The concept that factors aside from meatal location affect hypospadias repair and outcomes is not novel, and degree of ventral curvature and urethral plate quality are often cited as important factors. In our series, boys with greater than 60° of ventral curvature undergoing a single-stage repair were 27 times more likely to develop a fistula than those without chordee on multivariate analysis, making severe curvature an independent predictor of urethrocutaneous fistula formation. That meatal location did not retain significance on multivariate analysis highlights the importance of considering the entire hypospadias complex when determining severity, rather than just evaluating the position of the meatus. Our study has several limitations that warrant consideration. While GMS scores were assigned prospectively, the data was collected retrospectively, subjecting it to flaws inherent with such study design. Furthermore, type of repair is influenced by surgeon preference and subjective assessment of hypospadias characteristics not incorporated in our scoring system (i.e. tissue quality, urethral hypoplasia, penoscrotal transposition). Despite these limitations, our study demonstrates a strong correlation between the GMS classification and surgical complications, furthering supporting its potential as a tool to standardize hypospadias severity and gauge postoperative complications. CONCLUSION: The Glans-Urethral Meatus-Shaft (GMS) classification provides a means by which hypospadias severity and reporting can be standardized, which may improve inter-study comparison of reconstructive outcomes. There is a strong correlation between complication risk and total GMS score. Degree of chordee (S score) is independently predictive of fistula rate.

[Surgical treatment of hypospadias and complications in relation to the child's age]. / Tratamiento quirúrgico del hipospadias y sus complicaciones en relación con la edad del niño.

BACKGROUND: Hypospadias is one of the most common congenital malformations. Few studies have explored the association of age at time of surgery and the occurrence of complications after hypospadias repair. OBJECTIVE: Determine whether age at hypospadias repair is a risk factor for development of complications. METHODS: Retrospective cohort of patients with hypospadias repair in one surgical time. The data included: age, type of hypospadias, surgical technique and complications presented. The monitoring was conducted for six months after the surgery. Patients were classified according to age group A less than 24 months, group B from 24 to 48 months and group C over 48 months. We calculated the relative risk and confidence intervals of 95%. RESULTS: 170 patients were included in the analysis. The incidence of complications was 24.1%, the most frequent complication was urethrocutaneous fistula (52.2%). The median age in months of the complication group was 40.8 (6-196), whereas in the group without complications was 37.5 (6-196). Age was not associated with an increased risk for complications, group B (RR= 0.975 [95% CI 0.374-2.547]), and group C (RR= 0.966 [95% CI 0.386-2.416]) when compared with group A. CONCLUSIONS: Age at time of surgery for hypospadias correction in one phase is not associated with complications.

Antecedentes: el hipospadias es una de las malformaciones congénitas más comunes. Pocos estudios han explorado la asociación de la edad al momento de la cirugía y las complicaciones. Objetivo: determinar si la edad al momento de la corrección del hipospadias es un factor de riesgo de complicaciones. Material y métodos: estudio de cohorte retrospectiva de pacientes con reparación del hipospadias en un tiempo quirúrgico. Los datos incluyeron: edad, tipo de hipospadias, técnica quirúrgica y complicaciones. El seguimiento se efectuó incluso seis meses después de la cirugía. Los pacientes se clasificaron según su edad: el grupo A menores de 24 meses, grupo B de 24 a 48 meses y el grupo C mayores de 48 meses. Se calculó el riesgo relativo y los intervalos de confianza de 95%. Resultados: se analizaron los expedientes de 170 pacientes. La incidencia de complicaciones fue de 24.1%, la más frecuente fue la fistula uretrocutánea (52.2%). La mediana de edad en meses del grupo con complicación fue: 40.8 (6-196), mientras que en el grupo sin complicación fue: 37.5 (6-196). La edad no se asoció con mayor riesgo de complicaciones, grupo B (RR= 0.975 [IC 95% 0.374-2.547]); y el grupo C (RR= 0.966 [IC 95% 0.386-2.416]) al compararla con el grupo A. Conclusiones: la edad al momento de la corrección del hipospadias en un tiempo quirúrgico no se asocia con complicaciones quirúrgicas.

Differences in risk factors for second and third degree hypospadias in the national birth defects prevention study.

BACKGROUND: Hypospadias is a frequent birth defect with three phenotypic subtypes. With data from the National Birth Defects Prevention Study, a large, multi-state, population-based, case-control study, we compared risk factors for second and third degree hypospadias. METHODS: A wide variety of data on maternal and pregnancy-related risk factors for isolated second and third degree hypospadias was collected by means of computer-assisted telephone interviews to identify potential etiological differences between the two phenotypes. Logistic regression was used to calculate odds ratios including a random effect by study center. RESULTS: In total, 1547 second degree cases, 389 third degree cases, and 5183 male controls were included in our study. Third degree cases were more likely to have a non-Hispanic black or Asian/Pacific Islander mother, be delivered preterm, have a low birth weight, be small for gestational age, and be conceived with fertility treatments than second degree cases and controls. Associations with both second and third degree hypospadias were observed for maternal age, family history, parity, plurality, and hypertension during pregnancy. Risk estimates were generally higher for third degree hypospadias except for family history. CONCLUSION: Most risk factors were associated with both or neither phenotype. Therefore, it is likely that the underlying mechanism is at least partly similar for both phenotypes. However, some associations were different between second and third degree hypospadias, and went in opposite directions for second and third degree hypospadias for Asian/Pacific Islander mothers. Effect estimates for subtypes of hypospadias may be over- or underestimated in studies without stratification by phenotype.

[Questions and dilemmas in the management of hypospadias]. / A hypospadiasis ellátásában felmerülo kérdések, dilemmák.

Hypospadias is the second most common congenital malformation in males. Etiology remains unknown in about 70% of the cases. Distal hypospadias is considered not only developmental abnormality of the urethra in males, but it may also constitute a mild form of sexual development disorder in 46,XY males. Most urologists and endocrinologists consider that it is necessary to perform a detailed investigation of children presenting with proximal hypospadias associated with a small phallus or poorly developed scrotum and undescended testes. Currently, there is no generally accepted recommendation for the preoperative evaluation of hypospadias and, therefore, masculinizing surgery without preoperative evaluation is performed in these children. The authors summarize the international literature data and their own experience for the assessment and management of hypospadias concerning questions and problems related to preoperative investigation, masculinizing surgery and additional surgery. A detailed algorithm is presented for preoperative evaluation of both proximal and distal hypospadias.