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.

A novel "six stitches" procedures for pediatric and adult buried penis

ABSTRACT Introduction: The buried penis, if not treated before adolescence, will lead to psychological and physical disorders in adulthood. Therefore, early surgical intervention is necessary. At present, the common surgical methods include the penile corpus fixation, the Johnson's operation, the Devine's method, the modified Devine's method, Shiraki's method, etc. However, we found that these traditional surgeries showed various postoperative complications, such as long-term prepuce edema, avascular necrosis of skin flaps, stenotic prepuce, scarring, and poor appearance. This video shows the main technical steps of our innovative surgical procedure "Six Stitch" (SS) method for the buried penis. Materials and Methods: The designation of the so-called SS method was based on the total knots made (six knots were made for the SS procedure). After the crura penis was fully exposed via a longitudinal incision at the penoscrotal junction, at the 2 o'clock position (around the penis), the superficial layer of albuginea of the crura penis was sutured to the prepubic ligament with 2-0 non-absorbable sutures to prevent the retraction of the penis (the 1st knot). The same procedure was used for the 10 o'clock position (the 2nd knot); At the 2 o'clock position, the skin and subcutaneous tissue at the pubic mound were sutured to the prepubic ligament to reconstruct the appearance of dorsum penis (the 3rd knot). The same procedures were used for the 10 o'clock position (the 4th knot). At the 5 o'clock position, the ventral albuginea was sutured to the tunica dartos and subcutaneous tissue at the penoscrotal junction to reconstruct the penoscrotal angle (the 5th knot). The same procedures were used for the 4 o'clock position (the 6th knot). Finally, the gloved prepuce was reset and circumcision was conducted if the redundant prepuce existed. Results: We have done a total of 64 cases of SS procedures for concealed penis; mean length improvement was 3.8 ± 0.5 cm, with a satisfying 95 percent (61 / 64), which was much longer than the outcome of the above-mentioned methods. Mean operative time was 62.3 ± 12.1 minutes, and there was no serious intraoperative or postoperative complication (only 2 presented scar hyperplasia at the incision site). Conclusions: In conclusion, after the SS procedure, patients with buried penis can acquire an almost 4 cm improvement of penile length and covert incision at the midline of the scrotum, with an acceptable and low incidence of adverse events. This safe and effective procedure may be a viable option for the surgical management of pediatric and adult buried penis.

Objective measurements of the penile angulation are significantly different than self-estimated magnitude among patients with penile curvature

ABSTRACT Introduction: The study was aimed to assess the presence of actual differences between the objective and the perceived magnitude of a curvature between patients affected by Peyronie's disease (PD) and congenital penile curvature (CPC). Materials and Methods: Wee analysed a cohort of 88 consecutive patients seeking medi- cal help for either CPC or PD. All patients were invited to provide a self-made drawing of their penis in erection in order to obtain self-provided description of the deformity. An objective measurement of the deformity was also performed drawing two intersecting lines through the center of the distal and proximal straight section of the penile shaft. Results: Our findings showed significant differences between patient self-estimation and the objective measurements of the penile angulation performed by trained experts, with only 32% of patients correctly assessing their own curvature. Overall, patients tended to overestimate (56%) their degree of curvature, but the results are different in patients with PD than those with CPC. In the 60 men (68%) who did not accurately assess their curvature, PD patients generally overestimated their curvature versus CPC patients (67% vs 16%). On the contrary CPC patients underestimated their curvature compared to PD (42% vs. 4%). Conclusion: In order to improve patients' satisfaction rates, the surgeon needs to take into consideration the patient's perception of the deformity when planning the type of surgical correction.

An unanswered question in pediatric urology: the post pubertal persistence of prepubertal congenital penile curvature correction by tunical plication

ABSTRACT Objective: The aim of this study is to analyze post pubertal results of pre pubertal tunica albuginea plication with non-absorbable sutures in the correction of CPC. Materials and Methods: The files of patients who underwent tunica albuginea plication without incision (dorsal/lateral) were retrospectively reviewed. Patients younger than 13 years of age at the time of operation and older than 14 years of age in November 2015 were included. Patients with a penile curvature of less than 30 degrees & more than 45 degrees and penile/urethral anomalies were excluded. All of the patients underwent surgery followed by circumcision. Results: The mean age of patients at the time of the operation was 9.7 years (range, 6-13 years). The mean degree of ventral penile curvature measured during the operation was 39 degrees while it was 41 degrees in the lateral curvatures. All of the patients were curvature-free at the end of the operation. At the time of the follow-up examination, the mean age was 16.7 years (range, 14-25 years). Six patients had a straight (0-10 degrees) penis during erection and seven patients had recurrent penile curvatures ranging from 30 to 50 degrees. Conclusion: Pre pubertal tunica albuginea plication of congenital penile curvature (30-45 degrees) with non-absorbable sutures performed without incision is a minimal invasive method especially when performed during circumcision. However, recurrence might be observed in half of the patients after puberty.

Surgical correction of ectopic penis and scrotum associated with bilateral orchidopexy / Correção cirúrgica de pênis e escroto ectópicos associada à orquidopexia bilateral

ABSTRACT Ectopic penis is usually associated with penoscrotal transposition, and it is rarely observed in isolation. We report a surgical approach for an extremely rare case. A 10-year-old male patient with bilateral cryptorchidism and ectopic penis and scrotum in perineal area, with no penoscrotal transposition, representing an association not yet described in literature. A previous orchiopexy failed due to ectopic scrotum. By means of an inverted Y incision, the penis was mobilized and a perineal skin flap in form of a testicular sac was prepared. Finally orchiopexy was performed. The surgery was essential to treat cryptorchidism and to improve the self-image of the patient.

RESUMO O pênis ectópico geralmente ocorre associado à transposição peno-escrotal, sendo raro isoladamente. Relatamos uma abordagem cirúrgica para um caso extremamente raro. Tratava-se de paciente do sexo masculino, 10 anos, com criptorquidia bilateral e pênis e escroto ectópicos, na região perineal, sem transposição peno-escrotal, representando uma associação ainda não descrita na literatura. Orquidopexia prévia sem sucesso, devido à ectopia do escroto. Por meio de uma incisão em Y invertido, mobilizou-se o pênis e preparou-se um retalho da pele perineal em forma de bolsa testicular. Por fim, realizou-se a orquidopexia. A cirurgia foi fundamental para tratar a criptorquidia e promover ganho na autoimagem do paciente.

Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

Hipospadia perineal em um cão: relato de caso / Perineal hypospadias in a dog: case report

A hipospadia é uma anomalia congênita da genitália externa, na qual a uretra peniana termina ventral e caudalmente à sua abertura normal. Pode ser classificada, com base na localização da abertura uretral, como glandular, peniana, escrotal, perineal e anal. O objetivo deste trabalho é relatar um caso raro de hipospadia perineal, em que são abordados seus aspectos clínicos e terapêuticos, em um cão macho, sem raça definida, com seis meses de idade. Um canino com histórico de suspeita de hermafroditismo e presença de um orifício, logo abaixo do ânus, pelo qual urinava foi atendido no Hospital de Clínicas Veterinárias da Universidade Federal do Rio Grande do Sul (UFRGS). Durante o exame físico, foi observado o meato uretral localizado ventralmente ao ânus, além de alterações no prepúcio, pênis e escroto. Foram realizados exames de triagem, tais como: hemograma completo, urinálise, ecografia abdominal e uretrocistografia. O cão foi encaminhado para a cirurgia, quando foi submetido à amputação peniana e prepucial total e também à orquiectomia. O canino recebeu antibioticoterapia, analgésicos, anti-inflamatório e limpezas diárias dos pontos e da região perineal e apresentou excelente recuperação pós-operatória. Após 30 dias da cirurgia, o paciente retornou ao hospital, e foi verificada completa cicatrização da ferida cirúrgica, sem sinais de infecção urinária e inflamação ou assaduras na região perineal. A hipospadia perineal é um caso raro de anomalia uretral congênita, ainda pouco documentada em medicina veterinária. O procedimento cirúrgico realizado demonstrou ser importante, uma vez que removeu toda a genitália externa defeituosa, onde, futuramente, poderiam desenvolver-se infecções e/ou inflamações. Com isso, minimizou as complicações dessa doença e melhorou a qualidade de vida do paciente...

Hypospadias is a congenital anomaly of the external genitalia, where the penile urethra finishes its normal opening ventrally and caudally. It can be classified based on the location of the urethral opening as glandular, penile, scrotal, perineal and anal. The aim of this work was report an uncommon case of perineal hypospadias, working on the clinical and therapeutic aspects in a six-month-old male dog of undefined race. A dog with a history of suspected hermaphroditism and presenting a hole below the anus by which it urinated, was rescued at the Veterinary Teaching Hospital of the Federal University of Rio Grande do Sul (UFRGS). During the physical exam, it was observed that the urethral meatus was situated ventrally to the anus. Foreskin, penis and scrotum alterations were also seen on the dog. Screening exams such as complete blood count, urinalysis, abdominal ultrasound and cystourethrography were done. The dog went straight to surgery, where it had total penile and total preputial amputation and also orchiectomy. The canine showed excellent postoperative recovery, receiving antibiotic therapy, analgesics, anti-inflammatories and daily cleaning on stitches and the perineal region. Thirty days after surgery the patient went back to the Hospital presenting a complete healing of the surgical wound, without reporting signs of urinary infection and inflammation or rashes on the perineal region. The perineal hypospadias is a rare case of urethral congenital anomaly, still not well documented by veterinary medicine. The surgical procedure done demonstrated to be important, since there was the removal of all of the defective external genitalia where, in the future, inflammation and infection could develop, decreasing the complications of this disease and improving this patient's quality of life...

Application of Human Amniotic Membrane in Canine Penile Tunica Albuginea Defect: First Step toward an Innovating New Method for Treatment of Peyronie?s Disease

Purposes To evaluate the efficacy of human amniotic membrane (AM) grafting in the canine penile tunica albuginea defect; we developed an animal model as the first step toward an innovating new method for the treatment of Peyronie’s disease, penile cancers, and congenital deformities of the penis. Material and Methods From August to September 2011, ten healthy male dogs were selected. A rhomboid incision about 3x2cm over the tunica albuginea and its overlying squamous epithelium was made and then excised. The amniotic membrane was folded twice on itself and grafted on the defect. After 8 weeks, artificial erection was made for 5 dogs and for the other 5 dogs after 12 weeks. After artificial erection, partial penectomy was done and histopathological evaluation was performed on the grafts. Results Artificial erection performed successfully in all of the dogs. No infection or any other complication was seen. Histopathological examination showed complete re-epithelialization with squamous epithelium and collagen fiber deposition. Also, no dysplasia was seen. Conclusions The amniotic membrane can be used as a suitable substitution for tunica albuginea. It is safe, inexpensive, biodegradable, and available and may be used for the treatment of Peyronie’s disease, penile cancers, congenital penile deformities, and penile reconstructive surgery. .

Prevalence and risk factors for penile lesions/anomalies in a cohort of Brazilian men ≥ 40 years of age

Purpose To report the prevalence and risk factors of penile lesions/anomalies in a Metropolitan Brazilian city. Materials and Methods All participants undergoing prostate cancer screening in the city of Curitiba were systematically examined to identify penile lesions including cutaneous mycosis, sexually transmitted diseases, penile cancer, meatal stenosis, hypospadias, and Peyronie's disease. Outcomes of interest included the prevalence and the relative risk and 95% confidence intervals of the lesions/anomalies according to age, school level, race, personal history of diabetes, arterial hypertension, nonspecific urethritis, and vasectomy. Results Balanoposthitis occurred in 11.8% of all participants, with an increased risk in those with diabetes (RR = 1.73), or past history of nonspecific urethritis (RR = 1.58); tinea of the penis was present in 0.2%; condyloma acuminata in 0.5%; herpes virus infection in 0.4%; urethral discharge in 0.2%; genital vitiligo in 0.7%, with an increased prevalence in non-white men (RR = 4.43), and in subjects with lower school level (RR = 7.24); phimosis in 0.5%, with a nearly 7-fold increased risk in diabetics; lichen sclerosus in 0.3%; stenosis of the external urethral meatus in 0.7%, with a higher prevalence in subjects with lichen sclerosus (RR = 214.9), and in those older than 60 years of age (RR = 3.57); hypospadia in 0.6%; fibrosis suggestive of Peyronie's disease in 0.9%, especially in men older than 60 years (RR = 4.59) and with diabetes (RR = 3.91); and penile cancer in 0.06%. Conclusion We estimated the prevalence and risk factors of commonly seen penile diseases in an adult cohort of Brazilian men. .

One case of triphallia in the marine snail Echinolittorina peruviana (Caenogastropoda: Littorinidae) / Un caso de trifalia en el caracol marino Echinolittorina peruviana (Caenogastropoda: Littorinidae)

Gonochoric representatives of Littorinidae have a reproductive system organized with transit organs that connects the testicle with a penis specialized for internal fertilization. However, malformations at this level can produce, like triphallia (presence of triple penis). This description corresponds to one case of triphallia found in Echinolittorina peruviana (Lamarck, 1822) inhabitant of the South Pacific coasts. The macro and microscopic analysis reveals that every penis is an independent appendix whose individual morphology is similar to that in normal individuals, characterized by a non pigmented conic penis associated with a mammilliform gland located at the base of the right ocular tentacle. The cervical sperm groove lined by a simple ciliated and secretory columnar epithelium, runs along the neck of the animal, is subdivided into the base of each penis forming the penile seminal groove in each of them, and ascends dorsally from the base to the tip. The other organs that make up the reproductive system have a normal organization (no duplication or triplets), and the testicle organized in acini contains all the cell types of the male germ line. Although the cause of this condition is still unknown, might be associated with genetic causes and not of environmental kind...

Los representantes gonocóricos de Littorinidae presentan un sistema reproductor organizado con órganos de tránsito que conectan al testículo con un pene especializado para la fecundación interna. Sin embargo, pueden ocurrir malformaciones a este nivel, como la trifalia (presencia de pene triple). Esta descripción corresponde a un caso de trifalia encontrado en el representante gonocórico Echinolittorina peruviana (Lamarck, 1822), habitante de las costas del Pacífico Sur. El análisis macro y microscópico revela que cada pene es un apéndice independiente cuya morfología individual es semejante a la de individuos normales, caracterizada por un pene de forma cónica no pigmentado asociado a una glándula de tipo mamiliforme ubicada en la base del tentáculo ocular derecho. El surco espermático cervical revestido por un epitelio simple cilíndrico ciliado y secretor, recorre el cuello del animal, se subdivide en la base de cada pene conformando el surco espermático peneano en cada uno de ellos, y asciende dorsalmente desde la base hasta el ápice. Los otros órganos que conforman el sistema reproductor poseen una organización normal (sin duplicaciones o triplicaciones), y el testículo organizado en acinos contiene todos los tipos celulares de la línea germinal masculina. Aunque aún la causa de esta patología es desconocida podría estar asociada a causas genéticas y no de tipo ambiental...

Long-term follow-up of penile curvature correction utilizing autologous albugineal crural graft

PURPOSE: Peyronie's disease is an acquired connective tissue disorder of the penile tunica albuginea with fibrosis and inflammation. The disease produces palpable plaques, penile curvature and pain during erections. Usually it results in impairment of the quality of life. Our objective is to review the long-term results of the albugineal grafting harvested from the penile crura for the treatment of severe penile curvature. MATERIALS AND METHODS: Thirty-three patients with Peyronie's disease were submitted to a grafting with tunica albuginea from the penile crura for the correction of penile curvature. The results were evaluated after 6 months of the procedure. Variables studied were overall satisfaction with the procedure, correction of the penile curvature, erectile capacity, penile shortening and the presence of surgical complications. RESULTS: Mean follow-up after surgery was 41 months. Complete correction of the curvature was achieved in 30 patients (90%). The mean preoperative curvature was 91.8 degrees and median plaque length was 2 cm (ranged from 1 to 5 cm). Three patients (9%) experienced recurrence of the penile curvature and required a new procedure. In 30 men (90%) the procedure fulfilled their expectations and in 31 patients (93.9%) their opinions were that sexual partners were satisfied with the penile correction. Penile shortening or augmentation was referred in 6 (18.1%) and 1 (3%) patient, respectively. CONCLUSION: Our series demonstrated that grafting the albugineal defect after incision of the tunica albuginea with tunica from the crus for the correction of penile curvature is safe and results in satisfactory straight erections duringa long-term follow-up.

Índice de pene brazo en el diagnóstico de la disfunción sexual eréctil / Penile brachial index in the diagnosis of sexual erectile dysfunction

Se efectuó un estudio descriptivo y prospectivo de 58 pacientes a los cuales se les indicó la realización del índice de presiones de pene brazo para el diagnóstico de disfunción sexual eréctil en el Hospital Provincial Docente Dr Joaquín Castillo Duany de Santiago de Cuba, en el periodo de un año (2010-2011), con vistas a identificar los parámetros hemodinámicos y otras condiciones asociadas para evaluar la participación vascular en la enfermedad. El mayor número de hombres con resultados positivos tenía más de 50 años y los índices inferiores a las cifras normales se presentaron en 46,5 por ciento de ellos. La edad, el tabaquismo y la hipertensión arterial proporcionaron los valores más bajos de ese indicador en la casuística.

A descriptive and prospective study was conducted in 58 patients in whom the penile brachial pressure index was measured for the diagnosis of sexual erectile dysfunction in Dr Joaquín Castillo Duany Provincial Teaching Hospital of Santiago de Cuba during a year (2010-2011) in order to identify the hemodynamic parameters and other associated conditions and evaluate vascular involvement in the disease. The greater number of men with positive results were over 50 years and the indexes below normal range were observed in 46,5 percent of them. Age, smoking habit and hypertension provided the lowest values of this indicator in our cases.

Transposição penescrotal / Penoscrotal transposition

Transposição de pênis e escroto no ser humano é uma rara anomalia da genitália externa, caracterizada pela má posição do pênis em relação ao escroto, podendo ser na forma incompleta (parcial) ou completa. Frequentemente a transposição de pênis está associada a anomalias do genital (hipospadia, uretra curta), sistema renal, esquelético, gastrointestinal, cardiovascular e, ocasionalmente, a síndrome de regressão caudal. A reconstrução da transposição penescrotal, com prioridade a correção do ?chordee?, deverá produzir resultados satisfatórios, em termos estético e funcional, desde que o escroto e o pênis sejam colocados em suas posições anatômicas verdadeiras, evitando assim efeitos psicogênicos adversos.

Long-term Follow-up after Feminizing Genital Reconstruction in Patients with Ambiguous Genitalia and High Vaginal Confluence

We evaluated the long-term results of feminizing genital reconstruction in patients with genital ambiguity with high vaginal confluence. The medical records of 10 consecutive patients with ambiguous genitalia and high vaginal confluence who underwent feminizing genital reconstruction from 1996 to 2007 were reviewed. Seven patients had congenital adrenal hyperplasia, one had mixed gonadal dysgenesis, one had partial androgen insensitivity, and one had 5-alpha reductase deficiency syndrome. Median age at operation was 21 months (range, 2-47 months). Median follow up was 7.7 yr. Of the six patients who underwent feminizing genital reconstruction with the Gonzalez method, three had good results. Of the other three patients, one had a urethrovaginal fistula and underwent fistula repair 9 yr after, one had distal vaginal stenosis and underwent revision vaginoplasty 9 yr after, and one had a urethrovaginal fistula and urethral stricture. The patient with urogenital mobilization had persistent urogenital sinus. Feminizing genitoplasty using the Gonzalez method showed good long-term results in patients with ambiguous genitalia and Congenital adrenal hyperplasia. The procedure is less invasive than other approaches and results in excellent cosmetic outcomes; and complications can be corrected by relatively simple procedures.

Caracterización clinicoepidemiológica y terapéutica de pacientes con hipospadias / Clinical epidemiologic and therapeutic characterization of patients with hypospadias

La hipospadia es una de las malformaciones congénitas más frecuentemente observadas en la práctica de la cirugía pediátrica urológica a escala mundial y una de las causas de consultas iteradas en este territorio. A los efectos se realizó un estudio descriptivo, observacional y retrospectivo de 108 niños con el diagnóstico de hipospadia, atendidos en el Servicio de Urología del Hospital Pediátrico Sur de Santiago de Cuba, durante el período comprendido desde enero del 2000 hasta diciembre del 2006. El análisis efectuado reveló un predominio de los pacientes de 1 a 4 años y de la técnica quirúrgica de Snodgrass para la ejecución de la uretroplastia. La evolución clínica general resultó satisfactoria en 52,8 por ciento de los integrantes de la serie, pues los restantes requirieron nuevas intervenciones.

Hypospadias is one of the more frequently observed congenital malformations in the worldwide urologic pediatric practice and one of the reasons for repeated visits in this territory. In this respect a descriptive, observational and retrospective study was carried out in 108 children with diagnosis of hypospadias, who were attended in the Service of Urology of the Southern Children Hospital of Santiago de Cuba, from January, 2000 to December, 2006. The analysis revealed prevalence of patients aged between 1 and 4 years and of the Snodgrass's surgical technique for the urethroplasty. The general clinical course was satisfactory in 52,8 per cent of the series patients, and the remainder required new interventions.

Congenital segmental dilatation of sigmoid colon, penile agenesis and umblical cord hernia: a new association

Congenital segmental dilatation of the colon, penile agenesis and umbilical cord hernia are individually rare congenital malformations. An association between all three malformations has not been previously reported. We report a neonate with congenital segmental dilatation of the colon, penile agenesis and umbilical cord hernia all coexistent in a new born

Distal hypospadias; Barcat-Redman urethroplasty

To assess the efficacy, safety and complication of Barcat-Redman urethroplasty technique in the management of distal hypospadias. Prospective study was completed at Urology Department, Services Hospital Lahore and Larkana from 1998 to 2007. Fifty patients of distal hypospadias were selected from the Urology Department of Services Hospital Lahore and Larkana for Barcat-Redman urethroplasty technique. Age of the patients range from 5 to 25 years. The mean age was 11 years. Forty [80%] patients belonged to urban areas while 10 [20%] were of rural areas. All the patients presented with dystopia of external urethral meatus and chordee [ventral curvature] while 25 [50%] presented with misdirected stream, 3 [5%] spraying of urine and 3 [5%] narrow stream. The subcoronal type of distal hypospadias was commonly seen in our study. Forty [80%] patients had subcoronal and 10 [20%] distal penile urethral opening. Following barcat-redman technique 35 [70%] patients had good result and 8 [16%] patients were declared as fair result while 4 [14%] declared failure. The overall morbidity rate was 30% while hospital stay was 3 days. Barcat-Redman urethroplasty technique is simple, less time consuming and has success rate for the management of Distal hypospadias

Long-term patient satisfaction after surgical correction of penile curvature via tunical plication

OBJECTIVE: To assess patient satisfaction and functional results at long term follow-up after surgical correction for Peyronie's disease (PD) and congenital penile curvature (CPC) with the technique of tunical plication. MATERIALS AND METHODS: One hundred and two men operated for PD (n = 76) or CPC (n = 26) in four different departments of urology in public hospitals agreed to answer a six-question telephone questionnaire about treatment satisfaction. Tunica albuginea plication procedures represented the standard surgical approach. Subjects under investigation were correction of the deformity, feeling of bumps under the skin, pain during erection, penile sensory changes, development of erectile dysfunction (ED) and postoperative ability for complete vaginal intromission. Subjective response rates were compared using the chi square test on the basis of the etiology of the disease (CPC or PD). RESULTS: Significant differences (p < 0.05) between patients with CPC and PD were noticed in the prevalence of postoperative penile deformity, sensory changes, ED and ability to complete vaginal intromission, PD patients always showing a more pessimistic view. No significant differences (p = ns) were detected in terms of unpleasant nodes under the penile skin or pain during erection. CONCLUSIONS: Long-term outcome after surgical correction for PD and CPC with the technique of tunical plication can be poor. Probably patient expectations are above the real performance of surgical techniques. Preoperative information should be more exhaustive.

Duplicación del pene / Penis duplication

Recién nacido, producto eutócico del primer embarazo, con controles prenatales normales y sin antecedentes perinatales de importancia. Peso de nacimiento 2,150 g, tamaño 48 cm y perímetro cefálico 34 cm. Al nacimiento se observó un gran defecto en pared abdominal.

Hypospadias repair

The Departments of Urology, Pediatric Surgery and Plastic Surgery, Quaid-e-Azam Medical College, Bahawal Victoria Hospital, Bahawalpur. Jan 1999 to Dec 2004. Prospective. Patients admitted with hypospadias in these departments were included in this study except patients with multiple failed repairs previously. Standard procedures were practiced for every type of defect i.e. MAGPI and Mathieu's repair for coronal hypospadias, Snodgrass urethroplasty for proximal and distal penile hypospadias. The age range observed during this study was 1.5 to 25 years while 64% of patients were less than 10 years of age. The type of defect was coronal in 25%, penile in 60%, penoscrotal and perineal in 15% of the patients. Initial success rate was 78% and overall success rate was 92%. Complications observed were fistula formation 7%, stenosis of anastomotic site 7% and dehiscence of repair 3%. Thorough evaluation of urethral and penile malformation brings best outcome of surgery for hypospadias. Hypospadias repair should be offered to the child before school going age so as to prevent psychological impacts of genital malformations