RESUMO
Clitoral hypertrophy is a rare genital malformation that can be congenital or acquired. In congenital forms, the most common cause is adrenal hyperplasia. The acquired forms are caused by endocrinological diseases, benign tumours or cysts. Idiopathic clitoral hypertrophies can be detected after the elimination of secondary causes. A complete assessment is needed to treat the origin of clitoridomegaly. The hypertrophy is often increased or unmasked during sexual arousal with the appearance of a true vulvar appendage in erection. It is often accompanied by a hypertrophy of the clitoral hood and can cause psychological suffering with an impact on the quality of sexual life. When the cause of clitoral hypertrophy is diagnosed, treated or stabilized, the plastic surgeon may be called upon for surgical correction. Reconstructive surgery in this area has evolved considerably since the historical clitoral amputations which led to the current technique of partial resection with sparing the dorsal neurovascular pedicle of the clitoris as described by Professor Paniel. We propose a modified conservative technique to treat clitoral hypertrophy and the clitoral hood and present two clinical cases: ventral reduction clitoridoplasty with preservation of the neurovascular pedicle associated with a chevron plasty of the clitoral hood and a lipofilling of the labia majora. The postoperative follow-up is simple with reports of great satisfaction from patients regarding their quality of life.
Assuntos
Procedimentos de Cirurgia Plástica , Qualidade de Vida , Clitóris/cirurgia , Feminino , Humanos , Hipertrofia/cirurgia , Vulva/cirurgiaRESUMO
The aim of this study is to review the natural course, clinical features, and reproductive prognosis of ovarian tumors associated with hyperandrogenemia. We retrospect 33 patients of ovarian tumors with hyperandrogenemia. Thirty cases (91%) were sex cord-stromal tumors. Sertoli-Leydig cell tumors, Leydig cell tumors, and steroid cell tumors were the most common types. It is not possible, to predict the pathological subtypes based on androgen levels alone. Most of these tumors were solid masses, with an average diameter of 3.9 cm. These tumors are soft or fragile, no clear boundary with normal tissue, thus excision is superior to exfoliation. The average disease course of the top three tumors was 32.6, 35.4, and 67.7 months, respectively. Among 11 married women with a desire to get pregnant, nine cases resumed menstrual periods after surgery and became pregnant naturally. Hyperandrogenemia might predict a better prognosis. The asynchronism of hyperandrogenemia and undetectable tumor may cause irreversible change and emotional depress, the methods of early diagnosis need further study.
Assuntos
Hiperandrogenismo/complicações , Hiperandrogenismo/diagnóstico , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/diagnóstico , Adolescente , Adulto , Idoso , Androgênios/sangue , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/metabolismo , Criança , Pré-Escolar , Progressão da Doença , Feminino , Preservação da Fertilidade , Humanos , Hiperandrogenismo/patologia , Hiperandrogenismo/cirurgia , Pessoa de Meia-Idade , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Prognóstico , Estudos Retrospectivos , Tumor de Células de Sertoli-Leydig/complicações , Tumor de Células de Sertoli-Leydig/diagnóstico , Tumor de Células de Sertoli-Leydig/patologia , Tumor de Células de Sertoli-Leydig/cirurgia , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/cirurgia , Carga Tumoral , Adulto JovemRESUMO
BACKGROUND: Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity. PURPOSE: Clitoral reduction surgery has evolved over the years. Total clitorectomy, clitoral recession and neuro-vascular pedicle sparing clitoroplasty can be considered as three major milestones in its evolution. In nerve sparing clitoroplasty, earlier dorso-lateral approach was challenged by ventral approach, which is establishing itself as the approach of choice by its own merits. Clitoral reduction surgery being not a very commonly performed surgery, demands refinements in its technique to improve its results and particularly the aesthetic outcome, which is gaining more importance in recent years. METHODS: An adult case of late onset congenital adrenal hyperplasia (CAH) with clitoromegaly underwent nerve sparing reduction clitoroplasty by ventral approach under moderate magnification. We present key points of the preoperative anatomic evaluation. Technical refinements planned and executed step wise during the procedure resulted in satisfactory outcome. RESULT: Patient now has normal appearing genitalia, a small clitoris nicely covered by skin hood and very well preserved sensation to light touch. CONCLUSIONS: Preservation of sensation in the glans clitoris is of paramount importance during clitoral reduction surgery. Knowledge of the anatomy of the neurovascular supply to the glans clitoris gives us freedom from not isolating it in order to preserve it. In fact a deliberate attempt to dissect it can prove detrimental particularly when operating on a smaller organ during childhood. A ventral approach with refined technique proves safe and gives satisfactory result. Moderate degree of magnification eases dissection. A component-based detailed preoperative planning improves the overall aesthetic appearance of the female genitalia.
RESUMO
El hipotiroidismo congénito es la deficiencia de hormonas tiroideas presente al nacimiento, con una frecuencia mundial aproximada de 1:4000; en Cuba es menor que 1 x 4 500 con predominio del sexo femenino como característica interesante. Se presentó una niña nacida a término en el Hospital Vladimir Ilich Lenin, Holguín, la cual fue remitida para evaluación endocrinológica como un caso sospechoso de hiperplasia suprarrenal congénita debido a que presentaba una hipertrofia del clítoris como principal hallazgo clínico. Los valores de 17-OH progesterona resultaron normales y a los seis días del nacimiento el Centro de Pesquisa Activa Integral de Tacajó del municipio Báguanos, determinó que el valor de la hormona estimulante de la tiroides determinado a partir de sangre seca sobre papel de filtro, resultó en el límite superior para esta prueba, lo cual fue confirmado por el Hospital Pediátrico Provincial, que obtuvo un valor similar para la detección serológica de la hormona estimulante de la tiroides, por lo que se llegó a la conclusión que la hipertrofia del clítoris observada en la niña era debida a un hipotiroidismo congénito. Luego de recibir tratamiento sustitutivo con levo-tiroxina, la niña ha llevado un desarrollo normal y se recupera satisfactoriamente.
Congenital Hypothyroidism is the deficiency of Thyroids Hormones at birth, with a global frequency of approximately 1: 4000; in Cuba it is less than 1: 4500. Female predominance is an interesting feature. A newborn baby girl who was born at Vladimir Lenin Hospital, Holguín, was referred to a pediatric endocrine evaluation as suspicious case of congenital adrenal hyperplasia (CAH), because the physical exam showed an evident clitoral hypertrophy. The serum concentration of 17-OH-progesterone was normal, nevertheless when baby girl was six days old underwent a neonatal screening by Municipal Screening Center of Tacajó and the results showed that TSH concentration measured in dry blood on filter paper was in the highest limit for this test. The case was confirmed in the Provincial Pediatric Hospital by serum TSH determination, whose concentration was highest for this test too. The findings showed that clitoris hypertrophy was associated to congenital hypothyroidism. The girl is undergoing medical treatment with levothyroxine at present, and she is recovering satisfactorily.