Complete corporeal preservation clitoroplasty: new insights into feminizing genitoplasty

ABSTRACT Introduction: 46,XX Congenital adrenal hyperplasia (CAH) remains the first cause of genital virilization and current surgical techniques aim to restore female aspect of genitalia while preserving dorsal neurovascular bundle but not at the expense of not preserving erectile tissue. We aim to report our experience with a new surgical technique for clitoroplasty, completely preserving corporeal bodies, neurovascular bundles without dismembering the clitoris, in four patients with over a year follow up. Materials and Methods: After IRB approval four patients with 46,XX CAH and Prader 5 and 3 external genitalia, underwent feminizing genitoplasty. Complete preservation of erectile tissue was accomplished without a need to dissect dorsal neurovascular bundle. Glans size allowed no need for glanular reduction and there was no need to dismember the corporeal bodies. Results: Four patients 12 to 24-months-old underwent complete corporeal preservation clitoroplasty (CCPC), mean age was 18.5 months, mean follow up was 10.25 months. Vaginoplasty was performed in all patients with partial urogenital mobilization (PUM) and Urogenital Sinus flap (UF), only one severely virilized patient required a parasagittal pre-rectal approach to mobilize the vagina. We had no complications until last follow up. Conclusion: To our knowledge, we are introducing the concept of CCPC without the need of disassembling the corporeal bodies, neurovascular bundle and glans. It stands as a new alternative for feminizing genitoplasty with complete preservation of erectile tissue and no dissection of neurovascular bundle. Although there is still lacking long-term follow-up, it represents a new step in conservative reconfiguration of the external virilized female genitalia.

Nova classificação para hipertrofia dos pequenos lábios vaginais e correlação com as técnicas cirúrgicas indicadas / New classification of hypertrophy of the labia minora and correlation with indicated surgical techniques

Introdução: Primeira colocada entre as mais procuradas cirurgias plásticas genitais, a ninfoplastia ou labioplastia visa a correção da hipertrofia dos pequenos lábios vaginais e prepúcio, retirando seu excesso, sem interferir na sua função de proteção da vagina e auxílio na lubrificação genital. Diversos tipos de classificações foram propostos para facilitar a compreensão do grau de hipertrofia dos pequenos lábios vaginais e ajudar na escolha da técnica da labioplastia. Após analisar várias classificações, o autor propõe uma nova classificação, no intuito de facilitar a compreensão da hipertrofia das ninfas, capuz do clitóris e prepúcio e ajudar na escolha da técnica apropriada para labioplastia. Métodos: Foi feita uma busca na literatura médica PubMed/Medline com os termos hipertrofia lábios vaginais, labioplastia, labiaplasty, labioplasty, lábia minora hipertrophy, labial protrusion. Foram analisadas todas as classificações descritas nos trabalhos encontrados. Resultados: Uma nova classificação foi proposta. A hipertrofia dos pequenos lábios vaginais foi classificada em 4 graus: Grau 0 (≤ 1 cm), Grau 1 (> 1 cm e ≤ 3 cm), Grau 2 (> 3 cm e ≤ 5 cm) e Grau 3 (> 5 cm). Conclusões: A nova classificação, além de facilitar a compreensão do tamanho e extensão da hipertrofia das ninfas, também auxilia na escolha da técnica a ser escolhida para a labioplastia.

Introduction: Nymphoplasty or labioplasty is the most common genital plastic surgery. The objective of labioplasty is to correct hypertrophy of the labia minora and clitoral prepuce, removing excess tissue without affecting their function of protecting the vagina and aiding in genital lubrication. Several types of classifications have been proposed to facilitate the understanding of the degree of hypertrophy of the labia minora and assist in selecting the most suitable procedure in labioplasty. After analyzing several classifications, the author proposes a new classification to facilitate the understanding of hypertrophy of the labia minora, clitoral hood, and vaginal prepuce and help select the best labioplasty procedure. Methods: A literature search was conducted in PubMed/Medline using the following terms: hipertrofia lábios vaginais, labioplastia, labiaplasty, labioplasty, labia minora hypertrophy, and labial protrusion. All the classifications described in the identified studies were analyzed. Results: A new classification has been proposed. Hypertrophy of the labia minora was classified in four grades: grade 0 (≤ 1 cm), grade 1 (> 1 cm and ≤ 3 cm), grade 2 (> 3 cm and ≤ 5 cm), and grade 3 (> 5 cm). Conclusions: The new classification improves the understanding of the size and extent of hypertrophy of the labia minora and helps select the best procedure in labioplasty.

Nymphoplasty: classification and technical refinements / Ninfoplastia: classificação e refinamentos técnicos

BACKGROUND: Hypertrophy of the labia minora is a functional and esthetic problem that can have a significant impact on quality of life. Current surgical procedures are based on the excision of excess tissue and reconnection of the edges. However, anatomical alterations associated with labial hypertrophy such as hypertrophy of the foreskin of the clitoris are common, and, if not treated properly, may limit the results of surgery and cause esthetic and functional sequelae. The present report proposes a classification of the different types of hypertrophy and recommended treatments, and describes refinements in the labia minora reduction techniques. METHODS: A total of 20 female genital plastic surgery cases from the Center for Plastic Surgery of Brasilia and at Hospital das Forças Armadas from June 1999 to March 2008 were retrospectively reviewed. Patients were classified into three groups according to the degree and location of labia minora hypertrophy. RESULTS: The patients were satisfied with the esthetic results of surgery. No complications were reported, and all patients underwent surgery of the labia minora in accordance with the protocol proposed by the authors and based on hypertrophy type. CONCLUSIONS: The surgical procedures reviewed in this study were based on the classification of labia minora hypertrophy. Satisfactory esthetic and functional results were obtained, thus providing new methods for the surgical reduction of labia minora and foreskin of the clitoris without surgical stigma or reduction of sensitivity and no effects on sexual function.

INTRODUÇÃO: A hipertrofia dos pequenos lábios traz problemas estéticos e de comprometimento do comportamento íntimo e social. As técnicas existentes, via de regra, propõem excisão do excesso de tecido e reaproximação das bordas; entretanto, diferenças anatômicas são comuns e, frequentemente, observa-se hipertrofia do prepúcio do clitóris associada a aumento dos pequenos lábios. Essas alterações, se não tratadas adequadamente, limitam o resultado, podendo produzir sequelas estéticas e funcionais. Este trabalho propõe uma classificação dos tipos de hipertrofia, com tratamento diferenciado para cada um deles, além de refinamentos técnicos na abordagem da hipertrofia dos pequenos lábios. MÉTODO: Estudo retrospectivo de 20 casos de plástica genital feminina, realizada no Centro de Cirurgia Plástica de Brasília e Hospital das Forças Armadas, no período de junho de 1999 a março de 2008. As pacientes foram classificadas em três grupos, de acordo com o grau e a localização da hipertrofia dos pequenos lábios. RESULTADOS: As pacientes mostraram-se muito satisfeitas com o aspecto estético proporcionado pela cirurgia. Não foram verificadas complicações relacionadas aos procedimentos realizados. Todas as pacientes foram submetidas a tratamento cirúrgico dos pequenos lábios de acordo com o protocolo proposto pelos autores, baseado no tipo de hipertrofia. CONCLUSÕES: Os procedimentos cirúrgicos realizados, propostos de acordo com a classificação da hipertrofia dos pequenos lábios, permitiram a obtenção de resultados estéticos e funcionais satisfatórios, proporcionando à paciente oportunidade de redução do excesso dos pequenos lábios e do prepúcio do clitóris, sem criar estigmas cirúrgicos ou diminuição da sensibilidade, não prejudicando, portanto, a função sexual.

Tratamento cirúrgico da hipertrofia clitoriana / Surgical treatment of clitorian hypertrophy

Segundo vários autores, a hipertrofia discreta do clitóris está presente em 25%das mulheres. Quando o volume passa a incomodar no ato sexual, acompanhado ou nãode distúrbios psicológicos, as pacientes buscam inicialmente ajuda de ginecologistas ouendocrinologistas e não raramente são encaminhadas à cirurgia plástica para a resoluçãodos problemas. Método: Nossa experiência é baseada em 9 pacientes, durante 15 anos,portadoras de genitália ambígua, submetidas a tratamento cirúrgico. Foram selecionados3 casos para ilustrar os resultados obtidos com a técnica. Duas pacientes com hipertrofiaclitoriana do tipo II de Prader foram selecionadas; uma de origem adquirida na idade adulta,e outra de origem possivelmente congênita. A terceira paciente apresentava anomaliassexuais múltiplas, de origem congênita: agenesia de vagina, hipertrofia clitoriana, presençade ovários e testículos atrofiados, pseudo-hermafroditismo feminino, catalogada como tipoV de Prader. Todos os três pacientes foram submetidas a redução do clitóris pela dissecçãoda cobertura músculo-cutânea (desenluvamento) e sepultamento do corpo clitoriano porsutura. Resultados: Os procedimentos cirúrgicos realizados preservaram a estética e asensibilidade, com redução das dimensões do clitóris. Conclusão: Os procedimentos apresentamrelativa simplicidade, porém dentro de critérios

Moderate clitoris hypertrophy has been described in 25% of the women.When its shape and volume disturb at the sexual activity, combined or not by psychologicalproblems, initially patients search for help from gynecologists and or by endocrinologistsand not rarely are send to plastic surgeons. Our experience is based on 9 patients withambiguous external genitalia, submitted to these surgeries during the last 15 years. Threecases were selected to demonstrate the obtained results with the technique. Two femalepatients with Prader type II clitoris aspect were selected; one of them presents the problemat adult age and, in the other, the etiology was possible congenital. The third patients presentedhypertrophic clitoris combined with pseudo-hermaphroditism with ovarium, atrophictesticles as described in Prader Type V. Methods: The all three patients were submitted toclitoris reduction by dissection of the mucous-cutaneous covering and its excess buried bysuture. The third patient with pseudo-hermaphroditism had also neo-vagina reconstruction.Results: The surgical procedures preserved esthetic and sensibility, with clitoris dimensionsreduction. Conclusion: The surgical procedures presented moderate simplicity, but withinthe surgical criteria to preserve the functions and the external genitalia anatomy.

Congenital adrenal hyperplasia causing clitoromegaly

Congenital Adrenal Hyperplasia [CAH] is caused by congenital insufficiency of the enzyme 21 - hydroxylase [21-OHD] in the cortisol synthesis pathway. Because of the virilizing effects of androgens over-production, affected girls develop clitoral hypertrophy. Three patients with CAH are discussed below along with their surgical management and follow-up

Anterior sagittal transanorectal approach and passerini-glazel procedure: two surgical techniques combined for severely masculinized adrenogenital syndrome

A 46XX patient raised as boy, affected by severely masculinized female adrenogenital syndrome [congenital adrenogenital hyperplasia] received female sex assignment and underwent a one-stage clitorovulvoplasty and vaginoplasty at the age of 3 years. The patient was never hospitalized, in spite of persistent vomiting from birth and the appearance of pubic hair and facial acne at 1 year and 6 months of age. At the age of 2 years and 10 months, the correct diagnosis of female congenital adrenogenital hyperplasia was finally reached. The father took the final, difficult decision of gender reversal and the patient was prepared for a surgical program of genital reconstruction. At the time of the operation, the uncircumcised penis had an absolute normal male appearance, the stretched penile length was 7 cm and the scrotum was flat and empty. An abdominal ultrasound showed normal ovaries and uterus. The voiding cystourethrography did not show the presence of vaginal opening, therefore the preliminary endoscopic evaluation was mandatory. Inside of a very long urogenital sinus, a high implanted, narrow vaginal introitus was found at about 1.5 cm below the bladder neck. A one-stage clitorovaginovulvoplasty was performed utilizing the anterior sagittal transanorectal approach, without colostomy, and the Passerini-Glazel technique. At nine-month follow-up, the appearance of external genitalia was extremely satisfactory, the urethral meatus and vaginal introitus were definitely separate. In conclusion, a satisfactory restoration of a normal genital anatomy was carried out in a case of delayed diagnosis of severely masculinized female congenital adrenogenital hyperplasia with a remarkably high-ending and small vagina, which represents the extreme form in the spectrum of the diseases. An earlier surgical reconstruction would have allowed enormous psychological and surgical advantages in this case of clearly wrong sex assignment at birth. A small, high implanted vagina presents more of a surgical challenge, especially at the age of 3 years, but the result obtained in this case confirms the feasibility of vaginal reconstruction, together with clitorovulvoplasty, in a one-stage procedure, using the combination of the anterior sagittal transanorectal approach and Passerini-Glazel procedures

Vaginoplastía y clitoroplastía: tratamiento a temprana edad / Vaginoplasty and clitoroplasty: treatment at small age

Se estudiaron 21 pacientes con diagnóstico de hiperplasis suprarrenal congénita, disgenesia gonadal mixta y hermafroditismo verdadero. Fueron sometidos a reconstrución genital mediante vaginoplastía y clitoroplastía. Se recomienda la reconstrucción temprana, es decir, antes de los 18 meses de vida en pacientes que presentan un seno urogenital y desembocadura de la vagina distal al cuello vesical. Se analizan los diferentes métodos de estudio; ultrasonido pélvido, genitograma y endoscopía. La edad promedio de operación fué de 15.1 meses. Se obtuvieron buenos resultados y complicaciones mínimas, por lo que se recomienda este tipo de tratamiento en edades tempranas.

Hiperplasia adrenal congênita: alteraçöes similares da genitália em duas irmäs / Congenital adrenal hyperplasia: similar genitalia changes in 2 sisters

Analisam-se dois casos de hiperplasia adrenal congênita por deficiência de enzima 21-hidroxilase, em duas irmäs portadoras de alteraçöes similares da genitália: clitóris aumentado de volume, orifício urogenital único e ausência de vagina. Tecem consideraçöes sobre a fisiopatologia e descrevem duas formas clínicas clássicas da afecçäo: a virilizante simples e a perdedora de sal. Submetidas a cirurgia, perineotomia e clitoridectomia parcial, tiveram evoluçäo satisfatória