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Prolonged increase in micturition threshold volume by anogenital afferent stimulation in the rat.

OBJECTIVE: To investigate whether anogenital afferent stimulation induces a prolonged increase in the micturition threshold volume of anaesthetized rats. MATERIALS AND METHODS: Thirteen female rats, anaesthetized by alpha-chloralose and paralysed by pancuronium bromide were used for the experiments. The micturition threshold volume was determined by repeated cystometry. In two experiments, afferent activity was recorded from the exposed pudendal nerve; vaginal and anal afferents were stimulated electrically by ring electrodes. In one experiment, the dorsal clitoris nerves were exposed bilaterally and mounted for electrical stimulation. The afferents were stimulated continuously for 5 min at 10 Hz, using unipolar cathodic pulses of 0.5 ms duration with an amplitude of 10 mA (or 0.8 mA for the dorsal clitoral nerves). RESULTS: Anogenital stimulation for 5 min induced a significant and prolonged increase in the micturition threshold volume (from a median value of 0.35 mL before to 0.45 mL after stimulation; P < 0.01). The increase in threshold volume was maintained for about 40 min after the end of stimulation. There was no obvious difference in effect between the stimulation sites nor with direct dorsal clitoral nerve stimulation. Neither the micturition threshold pressure nor the maximal contraction pressure were altered by stimulation. No tonic afferent after-discharge could be detected in the pudendal nerve recordings. CONCLUSIONS: Artificial electrical stimulation of anogenital afferents induced a prolonged increase in the micturition threshold volume of anaesthetized rats. The change presumably involved the modulation of the synaptic transmission in the central micturition reflex pathway. It is proposed that the observed change represents the first step in the curative 're-education' process induced in patients with urge incontinence by electrical stimulation of anovaginal afferents.

Anal Canal↗

A posterior-based omega-shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency.

OBJECTIVE: To describe a technical modification for constructing a vagina in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency, using a narrower skin base but rich subcutaneous tissue, aiming to obtain both longer and larger vaginal segments with better cosmesis of the external genitalia. PATIENTS AND METHODS: From August 1997 to February 2001, 28 girls (aged 5 months to 17 years) had a neovagina constructed using a posterior-based omega-shaped flap. Twenty-six patients had a low vagina entering into the urogenital sinus and two had a high vagina that entered the urogenital sinus. In those with a high vagina the flap procedure was combined with the Passerini-Glazel technique. All the patients were scheduled for vaginal "calibrations" during the first year after surgery and, according to the result, would then undergo vaginal dilatation. RESULTS: Six children were re-operated; five had plastic surgery to correct genital folds that had regained a scrotal aspect, whereas one with a high vagina developed a urethral stricture, with urinary dribbling and infection, and had the urethra reconstructed. These six children are currently well. No hormone therapy was given to one child for 1 year who is scheduled for further surgery for a re-virilized clitoris. Two patients were lost to follow-up. Up to the last visit, 19 girls had not developed a vaginal stricture and the cosmesis of their external genitalia was deemed good. CONCLUSION: The posterior-based omega-shaped flap enabled both the construction of wider vaginal segments with a low risk of developing stenosis in those with a low vagina, and increased vaginal dimension when associated with the Passerini-Glazel technique for those with a high vagina. However, despite good cosmesis of the external genitalia, the follow-up is too short to confirm whether this technique will meet all the expectations.

Adolescent↗

Female circumcision.

Female circumcision, more accurately known as female genital mutilation, is still a common practice in parts of Africa. This ritual genital operation can involve partial or complete excision of the clitoris and labia minora as well as infibulation (labial fusion). The case reported here involves a 16-year-old African girl with a complication of this mutilating procedure.

Adolescent↗

Female pseudohermaphroditism associated with cloacal anomalies: faulty differentiation in the caudal developmental field.

A 1(1/2)-year-old girl with a urogenital sinus defect associated with an enlarged phalluslike clitoris is described. The child had an anteriorly located anus, a single kidney with grade III vesicoureteric reflux, a 3.5-cm-long common urethrovaginal channel, a large vesical calculus, and a hamartoma in the pubic region. After a preliminary colostomy, definitive surgery consisted of urethral reconstruction, vaginal reconstruction using a colonic graft, and posterior relocation of the anus. The discussion centers on a review of the previously reported cases of female pseudohermaphroditism associated with cloacal or urogenital sinus defects (FPHCD). The likely embryogenesis of this condition is discussed, and the similarity of the features with those seen in some patients with complete penoscrotal transposition (CPST) is highlighted.

Anal Canal↗

[Female sexual dysfunction: a systematic overview of classification, pathophysiology, diagnosis and treatment].

Sexual dysfunction is defined as "disturbances in sexual desire and in the psychophysiological changes that characterize the sexual response cycle and cause marked distress and interpersonal difficulty". The female sexual response cycle consists of three phases: desire, arousal, and orgasm. Various organs of the external and internal genitalia, e.g. vagina, clitoris, labia minora, vestibular bulbs, pelvic floor muscles and uterus, contribute to female sexual function. During sexual arousal, genital blood flow and sensation are increased. The vaginal canal is moistened (lubrication). During orgasm, there is rhythmical contraction of the uterus and pelvic floor muscles. Within the central nervous system, hypothalamic, limbic-hippocampal structures play a central role for sexual arousal. Sexual arousal largely depends on the sympathetic nervous system. Moreover, nonadrenergic/noncholinergic neurotransmitters (NANC), e.g. vasoactive intestinal polypeptide (VIP) and nitric oxide (NO), are involved in smooth muscle relaxation and enhancement of genital blood flow. Furthermore, various hormones may influence female sexual function. Estrogen has a significant role in maintaining vaginal mucosal epithelium as well as sensory thresholds and genital blood flow. Androgens primarily affect sexual desire, arousal, orgasm and the overall sense of well-being. The internationally accepted classification of female sexual dysfunction consists of hypoactive sexual desire disorders, sexual aversion disorders, sexual arousal disorders, orgasmic disorders and sexual pain disorders. Vascular insufficiency, e.g. due to atherosclerosis, and neurologic diseases, e.g. diabetic neuropathy, are major causes of sexual dysfunction. Additionally, sexual dysfunction may be due to changes in hormonal levels, medications with sexual side effects or of psychological origin. For the diagnosis of female sexual dysfunction, a detailed history should be taken initially, followed by a physical examination and laboratory studies. Physiologic monitoring of parameters of arousal potentially allows to diagnose organic diseases. Recordings at baseline and following sexual stimulation are recommended to determine pathologic changes that occur with arousal. Duplex Doppler sonography, photoplethysmography or the measurement of vaginal and minor labial oxygen tension may help to evaluate genital blood flow. Moreover, measurements of vaginal pH and compliance should be performed. Neurophysiological examination, e.g. measurement of the bulbocavernosus reflex and pudendal evoked potentials, genital sympathetic skin response (SSR), warm, cold and vibratory perception thresholds as well as testing of the pressure and touch sensitivity of the external genitalia, should be performed to evaluate neurogenic etiologies. Medical management of female sexual dysfunction so far is primarily based on hormone replacement therapy. Application of estrogen results in decreased pain and burning during intercourse. The efficacy of various other medications, e.g. sildenafil, L-arginine, yohimbine, phentolamine, apomorphine and prostaglandin E1, in the treatment of female sexual dysfunction is still under investigation.

Adult↗

Congenital adrenal hyperplasia - how to improve the transition from adolescence to adult life.

Congenital adrenal hyperplasia (CAH) is caused by a defect in the biosynthesis of cortisol that results in maximal activity of the hypothalamic-pituitary adrenal axis with hyperplasia of the adrenals and hyperandrogenism due to the accumulation of androgen precursors. In the salt-wasting subtype of the disorder, which accounts for appr. 75 % of patients with classical CAH, patients are unable to synthesise sufficient amounts of aldosterone and are prone to life-threatening salt-losing crises, whereas the simple virilising form is predominantly characterized by clitoris hypertrophy and posterior labial fusion. In addition, a non-classical variant can be discerned which in most cases is diagnosed at the time of puberty or early adolescence when hirsutism and menstrual irregularities may occur. The vast majority of CAH patients have 21-hydroxylase deficiency (90 - 95 %). Less common forms, such as 11beta-hydroxylase deficiency, will not be discussed in this review. Unfortunately, a considerable number of CAH patients is lost to regular and competent follow-up once they move out of paediatric care. This is most probably the result of insufficient co-operation between paediatric and adult endocrinologists at the time of transition from adolescence to adulthood. Furthermore, there is a lack of clinical guidance regarding psychosexual development in these patients. In this overview we will focus on special aspects of CAH treatment in adolescence and adulthood, and report on our 10-year experience with a transfer system for endocrine patients from paediatric to internal medical care, known as the "Kieler Modell". For practical purposes, we here provide charts for follow-up of CAH patients that can be adapted for use in any endocrine outpatient clinic.

Adolescent↗

[Results of surgical treatment of vulvar cancer].

129 patients with carcinoma of the vulva were treated at the Dept. of Obstetrics and Gynaecology from 1966-1985. FIGO stage I was observed in 21% of the cases, FIGO stage II in 50%, FIGO stage III in 10% and FIGO stage IV in 8% of the patients. In 10% of the patients, definite classification was not possible. Mean age at the onset of the disease was 66.2 years, the mean time of observation 63 months. Kraurosis of the vulva or leukoplakia were simultaneous phenomena recorded locally in 67% of the patients. Histological investigation showed squamous cell carcinoma in 93% of the cases. The tumours were most frequently observed on the labia and the clitoris. 98% of the patients underwent surgery, 64% radical vulvectomy with inguinal lymph node disection. 56% of the patients of this group had a 5-year survival rate, which was 47% for the entire group of patients. Wound healing disorders were the most frequently observed postoperative complications. Metastasis to the inguinal lymph nodes at the time of diagnosis is the critical point of the prognosis. In the absence of lymph node involvement, 68% of the patients achieved a 5-year survival, in the presence of lymph node involvement, the 5-year survival rate was only 13%. Results obtained by this study support the view, that radical surgery at the earliest possible time is the treatment of choice for carcinoma of the vulva.

Adult↗

[Microsurgical therapy of lymphedema of the external female genitalia].

Lymphedema of female external genitalia is an uncommon disorder, for which ablative treatment procedures have been used in the past. Such an operation is most traumatic and achieved unsatisfactory results. On the basis of our experiences in treating lymphedema of the extremities by lymphaticovenous anastomosis, the author developed a microlymphaticovenous operation for treating this condition. From March 1980 to November 1984, a total of 6 patients with lymphedema of female external genitalia were operated and good results were achieved. The edema of the vulva, clitoris, mons veneris and of the lower part of the abdominal wall has been reduced, the chylorrhea disappeared and the blisters on the edematous vulva vanished. On the basis of our clinical experiences in treating lymphedema of female external genitalia by microlymphaticovenous anastomoses, we believe, that the anastomoses should be performed early and their number should be sufficiently high. The indication, types of procedures and advantages of the operation are presented.

Adolescent↗

[Operative correction of a urogenital malformation with persistent cloaca].

A case of malformation of the urogenital system with a persistent urogenital sinus and a cloaca is reported. There was unilateral agenesis of the kidney, megaureter and a double ureter with an ureteral orifice into the vagina and the clitoris. There was a double uterus and vagina with an orifice into the terminal gut. The patient had chronic recurrent urinary tract infections which required a colostomy during childhood. The operation largely restored a normal urogenital area and a functional anus.

Adolescent↗

[Incomplete testicular feminisation].

We report a case of incomplete testicular feminisation in a 53 years old, third time married patient. Prominent symptoms were a slightly masculine structure of the body, masculine features and a hypertrophy of the clitoris. The reason for hospitalization was a lower abdominal tumour. Histologically it was a hydrocele with a testicle including a Leydig's cell-tumour without any signs of malignancy. On the opposite side was on atrophic testicle without spermiogenesis too. As usual the syndrome of incomplete testicular feminisation was discovered very late. Therefore, the necessary psychological guidance, especially from the beginning of puberty and the oncological control to exclude a tumour of the testicle had been left undone. It is the purpose of this report to draw attention to the aspect of this disease despite its rare occurrence in a ratio of 1:2,000 to 1:20,000.

Age Factors↗

A Turner-like phenotype in a girl with an isodicentric fluorescent Y chromosome mosaicism.

BACKGROUND: The Ullrich-Turner syndrome (UTS) demonstrates a great clinical variability according to the cytogenetic and molecular genetic findings in various tissues. In few cases the karyotype reveals the presence of an additional Y-bearing cell line which is referred to as a borderline case of mixed gonadal dysgenesis. In this condition, Turner specific stigmata occur in about half of the cases. PATIENT: A 10 year-old girl with short stature and only a few other signs of Turner syndrome and hypertrophic clitoris revealed 45,X/46,X,idic(Yq) mosaicism with 41% 46,X,idic(Yq) cells in a blood lymphocyte culture. METHODS AND RESULTS: Fluorescence in situ hybridisation (FISH) technique, using alpha-satellite Y-chromosome specific probe for locus DYZ3, confirmed the isodicentric character of this structurally abnormal Y chromosome. Polymerase chain reaction (PCR) analysis using primers for eight loci along the Y chromosome including SRY (Sex determining Region, Y gene) were positive for all loci tested, indicating that sequences from the long arm, centromere and most of the short arm of the Y chromosome are present. CONCLUSIONS: As patients with normal or rearranged Y chromosome have an increased risk of developing gonadal neoplasia prophylactic gonadectomy was performed in our patient. No evidence for gonadoblastoma was found on her streak-like gonads, but they showed some evidence of tubular formation. This paper points out the impact of cytogenetic and molecular genetic investigations in the definition of mosaicism in Turner's syndrome.

Child↗

[An unusual form of bladder exstrophy].

A rare case of bladder exstrophy is presented. It is described as being similar to a superior vesical fissure, but it differs from it by the abnormal course of the urethra which is separated from the bladder and runs along the clitoris.

Bladder Exstrophy↗

[Malignant melanomas of the vulva].

In 13 patients, mean age 57 (31-78) years, with malignant melanoma of the vulva, 7 of the tumors occurred on the Labia majora, 4 on the Labia minora and 2 on the clitoris. 8 cases involved nodular malignant melanomas, 2 cases each involved a superficial-spreading form and an acrolentiginous form, and one case involved a non-classifiable malignant melanoma. According to histological criteria 10 of 13 tumors showed a high risk of metastasising. At the time of diagnosis one of the patients was in stage II and one in stage III of the disease. Of the 13 patients, 6 died, as a result of the tumor, 5 within the first 2 years. The mean survival time was 15 (6-28) months. In three other cases the disease has reached an advanced stage: stage Ib, II or III. Up to now only 4 patients have remained symptom free, the symptom free interval averaging 62 (33-84) months. This investigation confirms the special characteristics of malignant melanomas of the vulva, which are the predominance of tumors having a bad prognosis and the rapidly progressing course of the disease.

Adult↗

Innervation of the female levator ani muscles.

OBJECTIVE: The objective of this study was to characterize the innervation of the human female levator ani muscles. STUDY DESIGN: Detailed dissections of the peripheral innervation of the iliococcygeal, pubococcygeal, puborectal, and coccygeal muscles were performed in 12 fresh-frozen female cadavers (aged, 32-100 years) with the use of transabdominal, gluteal, and perineal approaches. Both the pudendal nerve and the sacral nerve roots that enter the pelvis from the cephalic side were followed from their origin at the sacral foramina to their termination. Pelvic floor innervation was described with reference to fixed bony landmarks, particularly the coccyx, the ischial spine and the inferior pubis. Photographs were taken, and nerve biopsies were performed to confirm the gross findings histologically. Biopsy specimens were stained with Masson's trichrome. RESULTS: In each dissection, a nerve originated from the S3 to S5 foramina (S4 alone, 30%; from S3 and S4, 40%; from S4 and S5, 30%), crossed the superior surface of the coccygeal muscle (3.0 +/- 1.4 cm medial to the ischial spine [range, 1.0-4.2 cm]), traveled on the superior surface of the iliococcygeal muscle innervating it at its approximate midpoint, and continued on to innervate both the pubococcygeal and puborectal muscles at their approximate midpoint. The pudendal nerve originated from the S2 to S4 foramina, exited the pelvis through the greater sciatic foramen, traversed Alcock's canal, and branched to innervate the external anal sphincter, the external urethral sphincter, the perineal musculature, the clitoris, and the skin. Despite specific attempts to locate pudendal branches to the levator ani, none could be demonstrated. Nerve biopsy specimens that were obtained at gross dissection were confirmed histologically. CONCLUSION: Gross dissections suggest that the female levator ani muscle is not innervated by the pudendal nerve but rather by innervation that originates the sacral nerve roots (S3-S5) that travels on the superior surface of the pelvic floor (levator ani nerve). Because definitive studies (eg, nerve transection or neurotracer studies) cannot be performed in humans, further studies that will use appropriate animal models are necessary to confirm and extend our findings.

Adult↗

Innervation of the levator ani muscles in the female squirrel monkey.

OBJECTIVE: The purpose of this study was to characterize the innervation of the levator ani muscles in the female squirrel monkey and to investigate its usefulness as an animal model of pelvic organ prolapse. STUDY DESIGN: Eleven nulliparous female squirrel monkeys with no pelvic organ prolapse were used in this study. Detailed pelvic dissections were conducted (n = 3), and the Koelle stain for acetylcholinesterase was used to identify the motor endplate zone in the levator ani muscles (n = 2). Unilateral levator ani (n = 4) and pudendal (n = 2) neurectomies were performed; changes in levator ani muscle mass and myocyte diameter were examined 14 days after neurectomy. Nerve biopsy specimens from each animal were processed for microscopy. RESULTS: The levator ani nerve originated from the S2 spinal root and entered the pelvic cavity adjacent to the pelvic nerve between the flexor caudalis brevis and iliocaudalis muscles. The levator ani nerve then projected caudally and bifurcated to penetrate the iliocaudalis and pubocaudalis. A single motor endplate zone in each muscle correlated with the point of levator ani nerve penetration. The pudendal nerve originated from the S1-S2 spinal roots to innervate the urethral and anal sphincters, clitoris, and perineum, but not the iliocaudalis or pubocaudalis. Significant atrophy and myocyte shrinkage occurred in the iliocaudalis and pubocaudalis ipsilateral to the levator ani nerve transection (P <.05). Pudendal neurectomy produced no levator ani muscle changes. CONCLUSION: Intrapelvic skeletal muscles in the female squirrel monkey are similar to humans and have distinct innervation with no contribution from the pudendal nerve. The squirrel monkey is likely to be a useful model of pelvic organ prolapse and warrants further study.

Animals↗

Male pseudohermaphroditism in a Labrador Retriever, and a review of mammalian sexual differentiation.

CASE HISTORY: An 8-month-old Labrador Retriever was referred with a history of ambiguous external genitalia. CLINICAL FINDINGS AND TREATMENT: Clitoromegaly within apparent vulval folds, and an adjacent subcutaneous mass were noticed on external examination. An intra-abdominal testicle, with epididymis and suspected vas deferens ducts, was found during exploratory celiotomy. Incision over the subcutaneous mass revealed the accompanying testicle. Clitoridectomy was performed and an os clitoris removed. Normal juvenile testes were diagnosed on histology of the gonads. Chromosomal studies revealed a normal 78, XY male chromosomal constitution. Due to the combination of a male karyotype (78, XY), the presence of testicular tissue in the gonads, and the appearance of the external genitalia, a diagnosis of male pseudohermaphroditism (MPH) was made. CLINICAL RELEVANCE: This case presents the first report of MPH in a Labrador Retriever, and highlights the diagnostic steps recommended when confronted with a dog with ambiguous external genitalia.

Animals↗

Androgens in female genital sexual arousal function: a biochemical perspective.

Female sexual dysfunction is a highly prevalent, multicausal, and multidimensional medical problem that has a major impact on quality of life and interpersonal relationships. It is hypothesized that genital arousal disorder is caused by diminished arterial blood flow within the hypogastric arterial bed. This hypothesis may explain the pathophysiologic mechanisms in older patients with vascular risk factors, but it does not account for either the prevalence or the mechanisms of arousal disorders seen in young women with no vascular risk factors. Hormonal imbalances may be important contributing factors to the pathophysiologic mechanisms involved in female arousal disorders. Androgens modulate the growth and function of female genital sexual organs included the labia, vagina, and clitoris. This article discusses the potential role for androgens in facilitating female genital sexual arousal.

Androgens↗

The prevalence and practice of female genital mutilation in Nnewi, Nigeria: the impact of female education.

Three hundred and twenty-five consecutive live female deliveries at Nnamdi Azikiwe University Teaching Hospital, Nnewi were followed up for 9 months for evidence of any genital mutilation. Their mothers were examined for genital mutilation and a questionnaire based on face-to-face interview of the mothers was also administered. There was no genital mutilation observed among the 200 female babies whose mothers completed the 9 months follow up, The prevalence of genital mutilation among the mothers was 48%. The prevalence of female genital mutilation among the mothers increased with age. The circumcision index C.I. was zero and 3.0 at 16-20 years and 31-35 years age groups, respectively. Also the prevalence decreased with increasing level of education. The circumcision index was 1.0 for mothers at primary level education and least 0.33 at tertiary level of education. There was no relationship with parity. None of the mothers was willing to allow genital mutilation to be performed on her baby but 36% applied local treatment to the clitoris especially powder (28%). Dystocia was the commonest complication in the mothers and the knowledge about female genital mutilation was acquired informally from fellow women. Female education is paramount in the campaign and advocacy against female genital mutilation.

Journal Article↗