Clinical and diagnostic approach of male pseudo hermaphroditism with os-clitoris in French bulldog: A case report.

INTRODUCTION: Hermaphroditism is less frequently reported in dogs and is often associated with infertility. CASE REPORT: An 8-month-old French bulldog weighing 5 kg with an enlarged structure protruding from the vulva was clinically diagnosed with hermaphroditism. Physical, hormonal assay, computed tomography, and gross and histological studies were done in addition to successfully detailed surgical correction. On physical examination, the dog showed the presence of an os-clitoris protruded from the vulvar labia. Hormonal levels of estradiol, testosterone, and progesterone were 6.39 pg/ml, 0.4 ng/ml, and 8.67ng/ml, respectively. Surgical removal of internal gonadal tissues and os clirectomy operations were conducted after the exploratory laparotomy. The removed gonadal tissues were identical to that of a female with testicles instead of ovaries, according to a gross examination. Histological examination confirmed gonads as testis, with inactive seminiferous tubules and epididymis attached to uterine horns. CONCLUSION: The congenital anomalies in the present case were diagnosed as male pseudohermaphroditism (MPH). Surgical correction was performed, and the owner's satisfaction was achieved.

Primary amenorrhoea and clitoromegaly in a nulliparous woman: successful medical and surgical management.

Clitoromegaly is an important sign of virilisation and poses difficulty in sex determination, when present since birth. The diagnosis and treatment in an adult is a major challenge to the treating gynaecologist. The primary reason for its development is androgen excess due to congenital adrenal hyperplasia, polycystic ovarian syndrome, ovarian virilising tumours, neurofibromas, adrenal neoplasm and prolonged intake of anabolic steroids. A case of young nulliparous married woman who presented with primary amenorrhoea and clitoromegaly and was managed successfully has been reported.

Restoring normal anatomy in female patients with atypical genitalia.

Female patients with congenital adrenal hyperplasia (CAH) have varying degrees of atypical genitalia secondary to prenatal and postnatal androgen exposure. Surgical treatment is focused on restoring normal genitalia anatomy by bringing the vagina to the normal position on the perineum, separating the distal vagina from the urethra, forming a normal introitus and preserving sexual function of the clitoris by accepting moderate degrees of hypertrophy as normal and strategically reducing clitoral size only in the most severely virilized patients. There remains a need for continued monitoring of patients as they go through puberty with the possibility of additional surgery for vaginal stenosis. Anatomically based surgery and refinement in surgical techniques with acceptance of moderate degrees of clitoral hypertrophy as normal should improve long-term outcomes.

Male and female aphallia associated with severe urinary tract dysplasia.

INTRODUCTION: Aphallia is exceedingly rare (1/30 million births). Previous reports have provided limited detail on associated urinary tract findings. OBJECTIVE: We reviewed urinary tract anomalies in two boys with aphallia (patients 1 and 2) and a girl with urinary tract dysplasia, a similar external appearance and lack of corporal tissue (patient 3), also consistent with aphallia. CASE REPORTS (FIGURE): Patients 1 and 2 both had a 46XY karyotype, bilateral descended testes in well-formed scrotums, and posterior skin tags containing rudimentary urethras. Patient 1 had a focal area of urethral narrowing; a posterior bladder diverticulum, which drained a ureter; bilateral grade 5 vesicoureteral reflux, with a right partial renal duplication; and hydronephrosis of all moieties. Patient 2 had posterior urethral valves and a bladder diverticulum. Right ureterovesical junction obstruction required a tapered reimplant and later conversion to right-to-left transureteroureterostomy. Patient 3 had a 46XX karyotype and fused, well-formed labia majora. A posterior skin tag was associated with a stenotic urogenital sinus, beyond which were a vagina posteriorly and a right refluxing ureter anteriorly. The left ureter was absent, and a miniscule pouch represented a maldeveloped or absent bladder. Laparoscopy revealed ovaries and normal Müllerian structures. Bilateral renal dysplasia necessitated renal transplant and the creation of an ileocecal neobladder and Mitrofanoff channel. Corporal tissue was diminutive or absent in all. DISCUSSION: We see from these three patients that corporal tissue absence can occur in both male and female patients. We propose that the term aphallia can apply to both sexes, as it is the absence of corporal tissue that defines this condition. This is the only report to include and characterize findings in both male and female aphallia patients. Labioscrotal folds develop with a smooth appearance, and, posteriorly, a urethral orifice or Urogenital (UG) sinus with skin tag may be seen. Obstruction at the level of the urethra was common. Severe urinary tract dysplasia was seen in all, a finding not consistently seen or characterized in previous reports. CONCLUSION: In girls with severe urinary tract dysplasia and characteristic genital ambiguity, aphallia should be considered. Co-occurrence of aphallia and severe urinary tract dysplasia warrants further urinary tract imaging in all aphallia patients, including voiding cystourethrography, renal bladder ultrasound, and serum creatinine level. Urinary tract reconstruction may be performed without hampering future penile reconstruction, due to modern phallic reconstructive techniques.

Clitoral Abnormalities in the Absence of Virilization: Etiology and Treatment Strategies.

OBJECTIVE: To report 3 cases of pseudoclitoromegaly and to describe the strategies used for differential diagnosis and treatment. METHODS: Three cases and a literature review were described in this study. RESULTS: Three cases referred for treatment of clitoromegaly were in fact cases of pseudoclitoromegaly caused by a hamartoma, a neurofibroma, and a hemangioma of the clitoral prepuce. Abnormal virilization was excluded and the tumors were successfully treated by surgical resection and genital esthetic reconstruction. CONCLUSION: Clitoral abnormalities not associated with virilization syndromes are rare. Isolated abnormalities may affect the clitoris and the clitoral prepuce (pseudoclitoromegaly) and have multiple causes. The most frequent are inclusion cysts secondary to female circumcision, but a multiplicity of tumors, usually benign, may be found. Treatment is surgical, except for most infantile hemangiomas. Before deciding to offer surgical treatment, one should consider the degree of genital deformity and the risks of sensitivity loss and/or clitoral ischemia.

Clitoral prepucioplasty with anterior commissure transposition: a case report and description of a new, minimally invasive surgical technique.

BACKGROUND: Clitoral prepucioplasty is a new surgical intervention for clitoral prepuce reconstruction. This procedure has not been described previously in the literature. CASE: A 21-year-old, Caucasian woman, G0P0, presented with aesthetic concerns about her external genital anatomy, particularly the area surrounding the clitoral prepuce, which had significant excess folds of tissue on both sides. She underwent a new surgical procedure: clitoral prepucioplasty with anterior commissure transposition. The redundant clitoral prepuce reconstruction with anterior commissure was performed under local anesthesia. Postoperatively, she gave an account of no feelings of regret about having the operation, and her negative emotional-social feelings about her anatomic aberrations, clitoral area unattractiveness, sexual undesirability, embarrassment during sexual contacts, very low self-perceived body image, and decreased quality of intimate and social life had subsided. No intraoperative or postoperative complications were observed. CONCLUSION: The redundant clitoral prepuce reconstruction was easy to perform and resulted in aesthetically pleasing outcomes, improved quality of life, improved self-perceived body image, and increased sexual desire and frequency.

Aesthetic surgery of female external genitalia.

Aesthetic surgery of the external genitalia in women encompasses many procedures and may address the labia minora, clitoral hood, labia majora, mons pubis, or vaginal opening. During the initial evaluation, the surgeon should consider all aspects of the external genitalia to develop an appropriate surgical plan. It may be necessary to perform 2 or more procedures during the same surgical session to achieve the desired aesthetic result. In this continuing medical education (CME) article, we review the literature and summarize the available cosmetic techniques for female external genitalia. Resection of the labia minora has been described in several peer-reviewed reports. We also discuss the procedures and modifications to direct resection, wedge resection, and deepithelialization of the labia minora. Aesthetic surgery of the clitoral hood may involve straight-line resection, extended wedge resection, or inverted V hoodoplasty. The mons pubis may be treated with mons pubis pexy, wedge resection, or lipomodeling. The labia majora can be managed with direct resection or lipomodeling, and hymenoplasty may be performed to correct a wide vaginal opening.

Small bowel surgery for the gynecologist specializing in benign disease.

INTRODUCTION: Adhesions are fibrous bands of scar tissue that are often a result of surgery. Adhesions of the bowel are a common finding during gynecologic procedures, and their presence can lead to injury. METHOD: This video article demonstrates enterolysis and small-bowel surgery in women undergoing surgery for a benign gynecologic condition and found to have severe adhesive disease. CONCLUSION: Small bowel surgery is best carried out using a team approach. Surgeons should be especially vigilant about injury to the bowel in patients undergoing extensive adhesiolysis or enterolysis.

[Blood cell chimerism in dizygotic twins conceived by in vitro fertilization]. / Quimerismo hemático en gemelos dicigóticos concebidos por fertilización in vitro.

We present a case of hematopoietic chimerism in dizygotic twins (male and female) conceived by in vitro fertilization (IVF). At 8 years of age a blood karyotype was performed on the female due to the presence of clitoromegaly. Two different lines: 46,XX (53%) and 46,XY (47%) were found. FISH studies confirmed the presence of the SRY gene in 46,XY cells. Karyotyping of the male showed two different lines: 46,XY (58%) and 46,XX (42%). SRY gene was present in 46,XY cells. Microsatellite analyses of blood DNA revealed tetra-allelic contribution at some autosomal loci with similar proportions of maternal and paternal alleles and X/Y chromosome dose. FISH in buccal mucous showed that all cells from the female were 46,XX and those from the male 46,XY. The gonadal karyotype in the female was 46,XX without SRY. Hence, we report 46,XX/46,XY chimerism in dizygotic twins. Blood chimerism was confirmed by performing FISH on the buccal cells of the patients.

Persistent unexplained congenital clitoromegaly in females born extremely prematurely.

OBJECTIVE: Unexplained clitoromegaly is a rare but well recognised feature in girls born premature. Although detected at birth, girls may re-present during childhood to paediatric urologists and gynaecologists who should be aware of this condition. The aim of the study was to describe the clinical findings and management of a series of girls presenting with persistent congenital clitoromegaly associated with prematurity. MATERIALS AND METHODS: This was a retrospective notes review set in a tertiary referral centre for Paediatric and Adolescent Gynaecology (PAG). RESULTS: Eight girls with a mean age of 6 years were seen over an eight year period. In all cases a Disorder of Sex Development (DSD) had been previously excluded. The main symptoms were discomfort or concern about appearance. On examination five girls had excess skin over the clitoris and three had enlarged corporal tissue. Management included reassurance and simple measures to ease discomfort. In two cases the parents requested referral to a paediatric urologist to consider clitoral surgery. CONCLUSION: As survival rates for extreme prematurity improve, paediatric urologists and gynaecologists are likely to see more of these cases. Clinicians must be familiar with this condition to ensure children are managed appropriately.

[Clitoromegaly--treatment with the harmonic knife (case description)]. / Przerost lechtaczki--leczenie z zastosowaniem noza harmonicznego (opis przypadku).

Clitoromegaly, state when clitoris is non-physiologically large, most frequently occurs among patients with endocrine disorders (adrenal-genital syndrome, hormonally active tumors producing androgens, the use of anabolic steroids) or genetic. Less often the clitoromegaly is idiopathic of unknown etiology. There are no fixed standards for the size of organ, but it is assumed that not being in erect clitoris due to sexual arousal has a diameter of no more than 1/2 cm. To assess the size and shape of the clitoris can be used five Prader scale. However, it is most commonly used to assess the genitals of intersex. To the Clinic of Gynecology Oncology and Gynecology reported a 33 year old female patient because of escalating discomfort in sexual life. Patient associated her discomfort with her overly enlarged and painful clitoris preventing its proper coexistence. Gynecological survey found the correct size uterus and appendages. Vestibule of the vagina and the vagina of normal length and width. Larger and smaller labia developed properly. Clitoris increased to approximately 4x2 cm, not swollen, moving, with a uniform consistency. Except clitoromegaly in initial examination no abnormalities were found. Prior to surgery the patient has identified karyotype (46 XX) and has made designation of the levels of sex hormones and sonographic examination. In conducting these studies there was no answer found for the reason of clitoromegaly, there was the idiopathic overgrowth stated. Because of the pain during cohabitation associated with clitoromegaly, the patient was classified for reducing operation of this organ. For fear of the possibility of organ dysfunction, excessive bleeding and the appearance of postoperative adhesions abandoned classical surgical technique. It was decided to use in this operation, the harmonic knife. The use of harmonic knife allows avoiding introperative bleeding and precise, a partial reduction of the clitoris to the considered correct size without damaging of the organ structures. A month after surgery, the patient started having satisfying sex without feeling any pain and preserving proper function of the clitoris.

Unusual duplicate bladder exstrophy in a female newborn: a case report.

The authors report a rare variant of exstrophy-epispadias complex, a duplicate bladder with normal bladder communicating with an exstrophic bladder by a fistula, in a girl with no genital malformation except for a duplicated clitoris. This variant could be a hybrid form of duplicate bladder exstrophy and superior vesical fistula. It seems easier to repair and has a better prognosis than classic bladder exstrophy.

Female covered urethral duplication with urogenital sinus.

We report a covered urethral duplication in a girl presenting prenatally with an enlarged fluid-filled vulvar cyst, genital duplication, and urogenital sinus revealed by fetal magnetic resonance imaging (MRI) and serial ultrasounds. Physical examination revealed an enlarged vulvar mass covering the clitoris, a single orifice, and normally sited anus. Congenital adrenal hyperplasia was ruled out at birth. MRI in addition showed an accessory duct between the sinus and the urine-filled vulvar pouch with a bifid clitoris. A total urogenital sinus mobilization with resection of the accessory urethra and vulvoplasty was performed with uneventful follow-up.

Female epispadias.

Isolated female epispadias without bladder exstrophy is an extremely rare congenital anomaly. The symptoms of female epispadias are primary urinary incontinence and abnormal anatomical features. A 7-year-old girl presented with partial incontinence of urine. On physical examination, bifid clitoris and labia minora were seen. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux. With the diagnosis of isolated female epispadias, single stage reconstruction of the urethra, labia minora and clitoris was performed.

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.