ABSTRACT
Literature pertaining to surgical disclosure to the pediatric patient is lacking. We hypothesized parents would find it difficult to disclose urologic surgery to a child.
Parents of patients <5 years old undergoing urologic surgery were contacted for telephone survey. Parents were asked about future plans of surgical disclosure, comfort with disclosure, and any support received.
98 parents consented to study participation. 87% of surgeries were on the genitalia with 62% being minor genitalia surgery (i.e. circumcision). 70% of parents would tell their child about minor genital surgery while 84% would tell about major genital surgery (p=0.07). 4 of 20 parents of children undergoing hypospadias repair (major genital surgery) did not plan to tell their child about surgery. All parents of children undergoing non-genital surgery would tell. Of all parents planning to tell their children about surgery, only 14% were nervous. 34% of parents would find guidance in talking to their child helpful despite the majority (90%) stating no guidance had ever been provided.
Parents seem comfortable discussing urologic surgeries with a child but about 1/3 would appreciate further counseling. 20% of parents of children undergoing hypospadias repair hope to avoid telling their child.
Subject(s)
Adult , Child , Child, Preschool , Female , Humans , Male , Disclosure/statistics & numerical data , Genitalia/surgery , Parent-Child Relations , Urologic Surgical Procedures/psychology , Age Factors , Decision Making , Interviews as Topic , Sex Factors , Surveys and QuestionnairesABSTRACT
To evaluate the etiology, clinical presentations, investigations and surgical management of patients presenting with atypical genitalia in Pediatric surgery department. Disorders of Sexual development are one of the most complex congenital disorders encountered by the treating physicians. In recent years, the diagnostic ability and surgical techniques of gender reconstructions have improved remarkably. The spectrum includes chromosomal, genotype and phenotype abnormalities. The birth of a baby with atypical genitalia poses diagnostic and treatment challenges. This article focuses on etiology, clinical presentations, investigations and surgical management of patients presenting with ambiguous genitalia. A descriptive, prospective case series. Department of Pediatric Surgery, Military Hospital, Rawalpindi, Pakistan. October 2010 to June 2014. All patients with atypical genitalia, who underwent any kind of genital reconstruction surgery were included in the study. A total of 28 patients were operated upon during the study period. Age ranged from 11months to 12 years. Patients were divided into two groups on the basis of their karyotyping. Seventeen patients had 46 XY and 11 had 46 XX. No mosaic pattern was seen. Male genital reconstructive surgery was done in 16 patients while 12 underwent female genital reconstructive surgery. Disorders of sexual development present as a complex spectrum of psychosocial, sexual, endocrine and surgical implications, thus management demands a multidisciplinary team approach. Appropriately timed surgical intervention after thorough work up and detailed counseling, produce excellent cosmetic and functional results
Subject(s)
Humans , Female , Male , Infant , Child, Preschool , Child , Genitalia/pathology , Genitalia/surgery , Chromosome Disorders/pathology , Genotype , Phenotype , Karyotype , Prospective StudiesABSTRACT
Tissue transfer techniques are an essential part of the reconstructive urologist's armamentarium. Flaps and graft techniques are widely used in genital and urethral reconstruction. A graft is tissue that is moved from a donor site to a recipient site without its native blood supply. The main types of grafts used in urology are full thickness grafts, split thickness skin grafts and buccal mucosa grafts. Flaps are transferred from the donor site to the recipient site on a pedicle containing its native blood supply. Flaps can be classified based on blood supply, elevation methods or the method of transfer. The most used flaps in urology include penile, preputial, and scrotal skin. We review the various techniques used in reconstructive urology and the outcomes of these techniques.
Subject(s)
Humans , Genitalia/surgery , Mouth Mucosa/transplantation , Plastic Surgery Procedures/methods , Skin Transplantation/methods , Surgical Flaps , Tissue Transplantation/methods , Ureter/surgery , Urethra/surgery , Urologic Surgical Procedures/methodsABSTRACT
The morphology of the accessory genital glands of the male agouti was studied in twenty-three animals that were raised in captivity. Twenty animals had their genital glands dissected in situ for macroscopic description. The samples of each gland were recovered, embedded in paraffin, sliced and stained by Hematoxylin-Eosin technique. It was founded four pairs of glands: the vesicular glands, the coagulating glands, the prostate and the bulbourethral glands. Histological characteristics of the vesicular, coagulating and prostate glands showed similar morphology, within the pseudostratified columnar epithelium. The tubulo-alveolar type of the bulbourethral glands showed a lack of connective tissue among the tubules, a small amount of red stained presented it the cytoplasm, and the presence of vacuoles in the tissue. This study concluded that the agouti showed to have similar morphological aspect described in the others species of rodents.
A morfologia das glândulas genitais acessórias de cutias foram estudados em 23 animais criados em cativeiros. Vinte animais tiveram suas glândulas genitais dissecadas in situ para as descrições macroscópicas. Para o estudo microscópico foram utilizados três animais. Os fragmentos de cada glândula foram embebidos em parafina, seccionados e corados em hematoxilina e eosina. Foram encontrados quatro pares de glândulas: vesiculares, coaguladoras, próstata e bulbouretrais. As características histológicas da glândula vesicular, coaguladora e próstata mostraram morfologia similar, com epitélio colunar pseudoestratificado. O tipo tuboalveolar da glândula bulbouretral mostrou uma deficiência de tecido conjuntivo, citoplasma pouco corado e presença de vacúolos. Este estudo concluiu que a cutia apresenta as mesmas características morfológicas das glândulas genitais acessórias encontradas em roedores.
Subject(s)
Animals , Genitalia/anatomy & histology , Genitalia/surgery , Genitalia/physiology , Coloring Agents/analysis , Paraffin , RodentiaABSTRACT
Objetivo: Avaliar os resultados da genitoplastia masculinizante, com a técnica de Denis Browne, realizada em um grande grupo de pacientes com distúrbios do desenvolvimento sexual (DDS) tratados em um único hospital de referência. Pacientes e Métodos: Avaliamos 65 pacientes (57 com DDS 46,XY e 8 com DDS 46,XX) com hipospádia proximal e genitália ambígua. Os resultados cosméticos e sintomas urinários foram avaliados objetivamente e os pacientes responderam a um questionário sobre sintomas urinários, atividade sexual e satisfação pessoal após o tratamento cirúrgico. A idade dos pacientes na primeira cirurgia foi em média de 9 ± 10 anos e o segundo tempo cirúrgico foi realizado em média 14,5 ± 16,3 meses após a primeira cirurgia. O seguimento destes pacientes foi em média de 15,1 ± 10 anos e a idade dos pacientes na avaliação final foi em média de 25,9 ± 14,1 anos. Resultados: O aspecto cosmético foi considerado bom em 44%, regular em 53% e ruim em 3% dos pacientes. Houve diferença estatisticamente significante na média do tamanho peniano antes do tratamento entre os grupos com deficiência de 5-RD2 e com DDS de etiologia indeterminada (p<0,05). A média do tamanho peniano na avaliação final dos pacientes póspúberes foi de 7,8 ±2,4 cm, variando de 4 to 12 cm correspondendo a -4,4 ± 1,3 DP (-6,5 a -1,5 DP). Houve diferença estatisticamente significante no tamanho peniano entre os grupos com deficiência na produção de testosterona e com deficiência de 5-RD2 e entre os grupos com DDS de etiologia indeterminada e deficiência de 5-RD2 (p<0,05). O grupo com deficiência de 5-RD2 apresentou o menor tamanho peniano na avaliação final (-5,4±1 DP). As complicações mais freqüentes foram a fistula uretral encontradas em 50% dos pacientes seguida de estenose, presente em 20% dos pacientes. O sintoma urinário mais freqüente foi a perda urinária pós miccional. A atividade sexual foi referida por 86% dos pacientes adultos sendo definida como adequada em 60%, satisfatória...
Purpose: To evaluate the results of masculinizing genitoplasty with the Denis Browne technique performed in a large cohort of patients with disorders of sex development (DSD) treated at a single tertiary centre. Patients and Methods: We evaluated 65 patients (57 with 46,XY DSD and 8 with 46,XX DSD) with proximal hypospadias and genital ambiguity. Cosmetic results and the urinary stream were evaluated objectively, and the patients responded questionnaires regarding satisfaction with the surgical results, as well as urinary and sexual symptoms. The age at first surgery was 9±10 years and the second stage was performed after 14.5±16.3 months. The mean followup was 15.1±10 years and the average patients age at the last examination was 25.9±14.1 years. Results: Cosmetic results were considered good in 44%, regular in 53% and poor in 3% of the cases. The comparison of the mean penile length among 46,XY DSD groups identified a significant statistically difference between 5-RD2 deficiency and undetermined DSD groups at diagnosis (p<0.05). The mean penile length at last clinical evaluation in post-pubertal patients was 7.8 ±2.4 cm, ranging from 4 to 12 cm corresponding to -4,4 ± 1,3 SD (-6.5 to -1.5 SD) and there was a significant statistically difference in the mean penile length amongst testosterone production deficiency and undetermined DSD groups with 5-RD2 deficiency group (p<0.05). The 5RD2 deficiency group presented the smallest penile length at the last evaluation (-5.4±1 SD). The most common complications were urethral fistula (50%) and stenosis (20%) and the most frequent urinary symptom was dribbling after micturition. Sexual activity was reported by 86% of adult patients and was adequate in 60%, satisfactory in 29% and unsatisfactory in 11% of them. Overall, 84% referred satisfaction with surgical results, but 11% complained about penile length and 5% about urethral stenosis. Conclusion: Most of the DSD patients were satisfied with the...
Subject(s)
Humans , Male , Female , Child , Adolescent , Disorders of Sex Development , Genitalia/surgery , Sex Determination AnalysisABSTRACT
Penile agenesis [PA] is an extremely rare anomaly with profound urological and psychological consequences. The opening of the urethra could be either over the pubis or at any point on perineum or most frequently in anterior wall of the rectum. The aim of treatment is an early female gender assignment and feminizing reconstruction of the perineum. We report 8 cases of penile agenesis with urination and defecation through the rectum, apparently normal scrotum, bilateral descended testis, normally located anus, urethral opening in anus, 46xy karyotype and associated anomalies. In 2 cases parents refused any surgical interventions, but in 6 cases we did perform different operations [transforming five cases to females and one case to male gender]. We recommend feminizing operations in newborns or infants, but in older patients, regarding the child's psychology, it is advised to perform masculinizing operations, and finally, no surgical intervention should be undertaken before counseling the parents
Subject(s)
Humans , Review Literature as Topic , Disorders of Sex Development , Karyotyping , Genitalia/surgery , Plastic Surgery ProceduresABSTRACT
El enfoque clínico de manejo de los estados intersexuales, las consecuencias de las intervenciones a largo plazo, la política y la evidencia que los sustenta, han sido recientemente sometidos a una intensa revisión y detallado escrutinio, desde varias perspectivas. Existe hoy una pérdida de un consenso profesional en la cirugía feminizante de los genitales ambiguos y numerosos son los temas que han emergido (médicos, éticos, legales) que retan la práctica actual de tratamiento y manejo de la intersexualidad. Es por eso que nos propusimos en este trabajo describir los hallazgos principales en este campo y exponer los resultados de nuestra experiencia, derivados de la atención psicológica a personas que han recibido cirugía genital. Se concluye que es importante y necesario revisar las ideas que sustentan los tratamientos vigentes, así como desarrollar un contexto de trabajo que propicie la integración y colaboración interdisciplinaria(AU)
Subject(s)
Humans , Male , Female , Sexuality/psychology , Genitalia/surgery , Disorders of Sex Development/etiologyABSTRACT
Los pacientes con genitales ambiguos presentan dificultades diagnósticas y constituyen una emergencia médica. Su estudio requiere de un grupo interdisciplinario para la elección del sexo posible y planear la mejor estrategia quirúrgica. El estudio apropiado y precoz minimiza las compicaciones médicas, psicológicas y sociales del niño y su familia. El objetivo de este estudio es mostrar la experiencia en la utilización de la genitrografía como método de evaluación del seño urogenital en pacientes con ambigüedad genital, luego de la evaluación clínica y ultrasionografica. Mostramos la práctica en 93 pacientes seguidos en nuestra institución durante 17 años. Los diagnósticos fueron: Hiperplasia Suprarrenal Congénita, Hermafroditismo Verdadero. Feminización Testicular y Disgenesia Gonadal. La genitografía detecta con seguridad el nivel de implantación de la cavidad vaginal en la uretra siendo esencial para elegir la estrategia terapéutica. Utilizamos una nueva clasificación de seno urogenital en 3 tipos, de acuerdo a la genitografía.
Subject(s)
Infant, Newborn , Ultrasonography , Gonadal Dysgenesis , Genitalia/abnormalities , Genitalia/surgery , Ovotesticular Disorders of Sex Development/diagnosis , Adrenal Hyperplasia, Congenital/diagnosisABSTRACT
OBJETIVOS: Análise de pacientes com pseudohermafroditismo masculino com objetivo de avaliar as formas de apresentaçäo, diagnóstico, manejo clínico e cirúrgico. CASUíSTICA E MÉTODOS: Foram avaliadas retrospectrivamente 43 pacientes do Setor de Endocrinologia Ginecológica do Departamento de Toxoginecologia da UFPR de 1970 a 1996, 35 tinham idade entre 11 a 31 anos (81,4 por cento). RESULTADOS: O principal motivo para consulta ginecológica foi ausência de menstruaçäo em 24 pacientes (55,8 por cento). O exame complementar mais utilizado para diagnóstico foi cromatina sexual em 36 pacientes (83,7 por cento). Classificaram-se 18 casos (41,8 por cento) como forma completa da síndrome da insensibilidade androgênica e 25 casos (58,2 por cento) como outras formas. A cirurgia mais frequente nas outras formas para adequaçäo sexual feminina foi clitoridectomia em 9 casos (56,2 por cento) e para adequaçäo sexual ao sexo masculino foi ortofaloplasia em 5 (55,5 por cento). neovagina foi realizada em 4 casos (22,2 por cento) na forma completa. A maior parte das pacientes foi seguida com terapia de reposiçäo hormonal (65,2 por cento).
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Amenorrhea , Disorders of Sex Development/diagnosis , Disorders of Sex Development/surgery , Karyotyping , Sex Chromatin , Ultrasonography , Genitalia/abnormalities , Genitalia/surgery , Estrogen Replacement TherapyABSTRACT
Thirty five children with ambiguous genitalia admitted to our centre between January 1986 to December 1991, were followed up and their clinical, laboratory and management strategies were analyzed. Most of them presented between 1 month and 2 years of age and only 2 presented in the newborn period. Sixteen were female pseudohermaphrodites. Eighteen out of 31 children were assigned female sex. One genetic female with congenital adrenal hyperplasia was assigned male sex. We practised more than one type of clitoroplasty in our centre. Parents prefer the intersex children to be reared as male possibly because of the less social stigma attached to an impotent male than to sterile female, and because males are socially independent.
Subject(s)
Adolescent , Adrenal Hyperplasia, Congenital/complications , Child , Child, Preschool , Female , Gender Identity , Genitalia/surgery , Gonadal Dysgenesis, Mixed/genetics , Disorders of Sex Development/genetics , Humans , India , Infant , Infant, Newborn , Male , Disorders of Sex Development , Retrospective StudiesABSTRACT
Com o intuito de chamar a atençäo para a importância das questöes envolvidas no ato cirúrgico da genitália ambígua, pretende mostrar como isso afetará, de forma muito específica, a IDENTIDADE SEXUAL da criança. Säo feitas consideraçöes sobre o processo de alienaçäo que acompanha tais casos, propiciador de consequências drásticas para a estruturaçäo do sujeito, tornando-se imprescindível, em todo o seu curso, o acompanhamento psicológico.
Subject(s)
Humans , Child , Gender Identity , Genitalia/surgery , Sex Characteristics , ChildABSTRACT
A 17-year-old phenotypic female with ambiguous genitalia is presented. The patient complained of progressive dysmenorrhea, passing urine and menstrual blood through the same opening since menarche. Pelvic examination revealed and enlarged clitoris with prominent phallus, an enlarged right labio-scrotal fold with palpable gonad and a 3 mm diameter opening of both the urethral meatus and vaginal orifice at the vestibule. Chromosome analysis showed 46,XX/46XY karyotype. Laparotomy via right inguinal incision confirmed true hermaphroditism because of finding a right unilateral ovotestis. Factors leading to delay in presentation are discussed and the need for early diagnosis and management is emphasized. The clinical, cytogenetic features, gonadal histology and management are described and discussed.
Subject(s)
Adolescent , Female , Genitalia/surgery , Disorders of Sex Development/genetics , Humans , KaryotypingABSTRACT
La hiperplasia adrenal congénita es la causa más frecuente de seudohermafroditismo femenino; de todos los trastornos de la diferenciación sexual, es el único que pone en peligro la vida. Se estudiaron 12 pacientes con este diagnóstico, a los cuales se les trató durante el periodo comprendido entre junio de 1985 y febrero de 1991. Se encontraron antecedentes heredofamiliares positivos en dos y sugerentes en cuatro, por muertes neonatales y genitales ambiguos, dos de los cuales se operaron con diagnóstico erróneo de hipertrofía pilórica. La edad promedio en el momento de la primera valoración fue de 26.4 meses, y sólo cuatro se valoraron durante los dos primeros meses de vida. Todos se enviaron sin diagnóstico, 50 por cineto tenían asignación sexual psicosocial errónea. Todos tuvieron cromatina positiva y cariotipo 46 xx. El perfil endocrinológico reveló elevación de andrógenos y disminución de cortisol en todos los casos, siete con elevación de hormona adrenocorticotrópica. Cinco presentaron la variedad perdedora de sal y 7 (58 por ciento) la variedad simple. La edad ósea fue avanzada en siete por inicio tardío del tratamiento. El tratamiento médico consistió en prednisona más enantato de desoxicorticosterona para los perdedores de sal y prednisona exclusivamente para los de la variedad simple. Se operaron 10 pacientes a una edad promedio de 4.7 años, con límites de 1.6 a 8.9 años. Se realizaron genitoplastia femnizante en un tiempo quirúrgico en ocho pacientes e histerectomía con salpingoooforectomía más colocación de prótesis testiculares y corrección de hipospadias en dos por edad avanzada y negativa de los padres a la reasignación. Quedó pendiente la operación de dos lactantes. Los resultados estéticos fueron buenos, y sólo un paciente manifestó conducta agresiva después del tratamiento quirúrgico. En este estudio se hace patente la necesidad de un diagnóstico oportuno para evitar muertes neonatales, establcer una asignación sexual psicosocial correcta, y aplicar un tratamiento medicoquirúrgico temprano que resulte en la plena expresión gonadal, endocrina y de fertilidad de estos pacientes. PALABRAS CLAVES: Hiperplasia adrenal congénita, seudohermafroditismo femenino, genitales ambiguos.
Subject(s)
Humans , Female , Infant, Newborn , Infant , Child, Preschool , Disorders of Sex Development , Genitalia , Hyperplasia , Adaptation, Psychological , Disorders of Sex Development/etiology , Disorders of Sex Development/surgery , Disorders of Sex Development/therapy , Genitalia/abnormalities , Genitalia/surgery , Hyperplasia/complications , Hyperplasia/surgeryABSTRACT
Transplantation of reproductive organs have been unsuccessful to date because transplantation models utilised have never taken into consideration the vascular models of the reproductive system especially that of the female. This article examines the future of large organ transplantation in reproductive system and also the transplantation of cellular and tissue components as in embryo transfers. It also examines the future of such research procedures as embryo splicing, nuclear gene injections and transplantation in reproductive technologies.
Subject(s)
Female , Forecasting , Genitalia/surgery , Humans , Male , Reproductive Techniques/trendsABSTRACT
Durante un periodo de 15 años se identificaron y sometieron a tratamiento 21 casos de extrofia vesical en el Hospital de Pediatría, Centro Médico de Occidente, IMSS. De ellos 16 se sometieron a cierre primario y los restantes a diversos tipos de derivación urinaria. La colocoloplastía indirecta es un método de derivación urinaria que se realiza por etapas, y que satisface las finalidades del tratamiento de la estrofia vesical al lograr un sistema continente contra el reflujo a expensas del esfínter anal; el resultado de una imagen corporal esterna adecuada con reconstrucción genital aceptable y, por ende, se favorece el desarrollo sexual y psicosocial satisfactorio. En este artículo se presenta la experiencia obtenida en este Hospital con cinco pacientes, cuatro mujeres y un varón, que se trataron con el método mencionado. Tres de los casos eran de cierre primario fallido durante el período neonatal, y en dos la indicación primaria era derivación urinaria. El procedimiento consiste en una primera etapa en la que se realiza cistectomía con reconstrucción de pared abdominal y alargamiento del pene, y una tercera consistente en anastomosis del conducto de colon con el colon rectosigmoideo previa valoración de la continencia anal y de la ausencia de reflujo o estenosis ureterointestinales. El tratamiento finaliza, en el varón, con la corrección del epispadias en una cuarta etapa. En todos los casos mencionados fueron satisfactorios la continencia anal y los resultados estéticos con la pared abdominal. La reconstrucción genital se terminó con buenos resultados en las cuatro mujeres, y el paciente varón esperaba la cuarta etapa del tratamiento en el momento de terminarse la redacción de este artículo, pero el alargamiento del pene en él havía sido satisfactorio. Este procedimiento es una alternativa excelente para los pacientes de edad escolar que no han recibido tratamiento previo, los que tienen a la derivación urinaria como primera indicación y a los que no han reaccionado a las medidas terapéuticas previas. El procedimiento satisface los requisitos estéticos y funcionales deseables en estos casos.
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Male , Female , Bladder Exstrophy/therapy , Genitalia/surgery , Urinary Bladder/abnormalities , Urinary Bladder/surgery , Urinary Diversion , Psychosexual DevelopmentABSTRACT
En las hernias escrotales de gran tamaño es uno de las técnicas de Banks, Mckay y la de Potts resultan muy laboriosas y en el post operatorio presentan grandes edemas escrotales e incluso hematomas. Con la técnica de Duhamel se evitan estos problemas, siendo motivo de este trabajo. Se estudiaron 50 pacientes tratados con la técnica de Duhamel, en el Departamento de Cirugía Pediátrica de Hospital San Bartolomé. Entre los años 1975 y 1979, de las cuales 26 fueron derechas y 24 izquierdas. Todas eran hernias inguino escrotales de gran tamaño; un paciente era portador de hernia recidivada. El acto operatorio se realizó según la técnica descrita por Duhamel, el tiempo operatorio no fue mayor de 30 minutos. La evolución post*operatoria fue satisfactoria en 48 pacientes, en 2 presentaron infección de la herida operatoria, las cuales cedieron al tratamiento