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As with all reconstructive surgery for patients clitoris bilder Disorders of Sex Development DSDthree specific reasons for intervening are typically considered: providing anatomy suitable for penile—vaginal intercourse, achieving a manner for urination commensurate with gender identity i. Long-acting glucocorticoids are preferable because they are effective given once or twice daily The specific regiment varies between institutions worldwide.

Regression of the glans clitoris, with creation of a labia minora and clitoral hood to properly conceal the glans, requires a good understanding of these later structures. A of enzymatic defects in the production of clitoris bilder can cause a shunting of cortisol precursors to an alternate metabolic pathway, which in an excess production of adrenal androgens i. In ovotesticular DSD ly labeled true hermaphroditismfunctional ovarian and testicular tissues are both identifiable.

With the ability to abrogate ongoing hormonal stimulation of the phallus, it was no longer deemed necessary to reconstruct these children as males. Most important to the discussion of clitoral surgery is clitoris bilder anatomic course of the nerves that provide sensation to the glans.

In childhood, treatment is geared toward optimizing growth and pubertal development. In a survey in the United Kingdom of 30 teaching centers, a variety of different regimens were utilized; hydrocortisone was the most common, followed by dexamethasone and then prednisolone Mineralocorticoid is given in classic cases of CAH. Fludrocortisone is provided to maintain normal electrolyte and plasma renin activity. Most clitoral surgery clitoris bilder performed in conjunction with a vaginoplasty.

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The primary concern in performing surgeries that address clitoral enlargement is that the procedure may reduce innervation to the clitoris. These changes have been made in an effort to preserve clitoral sensation and preserve orgasmic potential. Treatment of the classic or severe form clitoris bilder CAH requires suppression of adrenal androgen overproduction and replacement of cortisol and aldosterone. In considering the anatomy of the clitoris, one clitoris bilder also be versed in the anatomy of neighboring structures.

The physiologic stress that surgery induces requires thoughtful increases in clitoris bilder steroid dosing. Prior to embarking on major surgery such as clitoroplasty, the patient must be in optimal physiologic condition. InLawson Wilkins demonstrated that it was possible to suppress adrenal androgen production by providing these individuals with cortisone. Innervation governing tumescence and sensation are also similar to that observed in the penis. With a better understanding of anatomy and function, procedures have evolved to preserve clitoral tissue, especially with respect to the neurovascular bundles.

To this point, it is important to note that when compared to controls, sexually mature females who have undergone surgery in childhood frequently report reduced sensation and decreased ability to achieve orgasm 15 — In cases of moderate and severe virilization, both guidelines recommended that clitoroplasty be considered as long as an experienced surgeon performs it.

Although the same gene, CYP21A2, is involved in both the severe and mild clitoris bilder, genetic mutations typically associated with non-classic CAH result in substantially less impairment of hydroxylase activity. Non-classic CAH is a milder form of the disease. The role that clitoral surgery plays in clitoromegally remains strongly debated. A of Disorders of Sex Development DSD possessing a cell line with a Y chromosome can result in with an enlarged phallus who may be ased the female gender role. In addition, the emotional impact of having with clitoromegally will be extremely challenging for most families.

Longer-acting glucocorticoids have typically been avoided in children due to their potential for growth suppression No consensus exists for glucocorticoid dosing in adults. Clitoris bilder overall worldwide incidence of classic CAH is one in 15, live births of which two-thirds are salt wasting 5.

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Levels of hydroxyprogesterone, clitoris bilder, androstenedione, and plasma renin activity are used to evaluate adequacy of therapy and patient compliance. It is for this reason that it is imperative that a multidisciplinary care model be utilized to provide comprehensive care of the child and family. This contention was later brought into question as reports of patients with sexual inhibition and ambivalence toward sexual activity began to surface Any surgical procedure involving the clitoris carries the risk of disruption of the nerve supply 1633 Techniques to reduce clitoral size have attempted to minimize this risk by preserving the dorsal neurovascular bundles 34 — There is no unanimous consensus as to the best technique for achieving this goal.

Females with classic CAH, whether salt-losing or non-salt-losing, present at birth with an enlarged phallus due to in utero exposure clitoris bilder excess fetal adrenal androgens. Since the only known function of the clitoris itself is to provide sexual pleasure, the later goal is the only one that is relevant to the discussion of clitoroplasty.

Clinicians may use hydrocortisone, prednisone, prednisolone, dexamethasone, or a combination of treatments. Prior tomany prominent surgeons supported the surgical removal of the entire clitoris 28 — clitoris bilder This was largely based on the misunderstanding that the clitoris was unnecessary for sexual function The procedure began by placing a Foley catheter in the urethra to avoid injury.

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The target goal for androstenedione, testosterone, and plasma renin values are to have them within the normal range for age. A recent study of non-CAH females revealed that the labia minora converge under the clitoral glans, separate to the clitoral hood. If clitoroplasty surgery is undertaken, it is done so with the intent of providing clitoris bilder appearance that aims to closely resemble the typical female phenotype.

The use of fludrocortisone is also recommended in simple-virilizing CAH and allows management with lower doses of glucocorticoid Some clinicians feel that the same daily dose of fludrocortisone given in split doses twice daily is more effective than once daily therapy Overtreatment should be avoided and may result in hypertension. Surgical techniques for management of clitoromegaly can broadly be divided into three : clitorectomy, reduction clitoroplasty, and corporal-sparing techniques. The advantage of performing both procedures simultaneously is that the common urogenital sinus can be used for a host of reconstructive purposes If vaginoplasty is not undertaken simultaneously, one should always use the sinus tissue to create a more female-like vulva.

The clitoral neurovascular bundles ascend along the ischiopubic rami clitoris bilder meet as paired bundles that course along the dorsal surface and then pass largely clitoris bilder into the glans. Thus, patients with non-classic CAH do not have cortisol deficiency but instead may have manifestations of hyperandrogenism, later in childhood or in early adulthood. Management of the enlarged clitoris, because of its import for sexual function, has been and remains one of the most controversial topics in pediatric urology. As we will see, despite its importance, there has been a dearth of data in this regard.

A vulvoplasty would be subsequently performed with the urogenital sinus tissue. Variability exists with regard to the morphology of the clitoral hood. This view apparently stems from the belief that the clitoris is necessary for normal sexual function. Unlike authors who had advocated partial clitoral resection, Gross emphasized the complete removal of all tissues clitoris bilder order to eliminate the painful neuromata, which were reported to occasionally occur in the stump after amputation. Early controversy surrounding clitoroplasty resulted from many factors including an incomplete understanding of clitoral anatomy and incorrect assumptions of the role of the clitoris in sexual function.

Prior to complete removal, a hemostat was carefully placed across the attenuated tip of the corpus to control the clitoral artery. Future work in the arena of patient satisfaction will undoubtedly play a major role in directing our surgical approach.

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Specific evidenced-based models for multidisciplinary care have been published Evidence supports that laboratory monitoring for appropriate steroid dosing and the provision of mental health care occurs more consistently when these multidisciplinary models are in place. In that the majority of patients who present with clitoromegally are patients with CAH, an understanding of proper endocrine management in these patients is imperative.

The crura were dissected back to the bifurcation clitoris bilder the crura. The three clinical phenotypes are typically classified as classic salt-losing most severeclassic non-salt-losing simple-virilizingor non-classic mild or late-onset. There are a limited of clinical entities that result in clitoromegally. While not having a defined corpora spongiosum, the clitoris does have the male clitoris bilder of the glans, which consists of spongiosal tissue.

As a general rule, there is high concordance between genotypic CYP21A2 mutations and phenotype In the most severe form, concomitant aldosterone deficiency le to salt loss.

It is the goal of this manuscript to describe the different procedures that have been developed for the surgical management of clitoromegally, with emphasis on the risks and benefits of each. While it is beyond the scope of this manuscript to describe the process of asing gender to these individuals, if a female gender role is selected then surgery to render the phallus more clitoral in appearance may be entertained Rare clitoris bilder of females with clitoromegally secondary to pelvic plexiform neurofibroma have also been reported This is felt most likely due to fetal programming causing a surge in LH and the overactivation of the pituitary—gonadal axis At present, the decision to perform clitoris bilder surgery in children with clitoromegally is intensely debated.

In both conditions, the phallus will undergo some degree of enlargement due to endogenous testosterone production.

Once adult height is achieved, treatment should be focused on optimizing fertility and quality of life and minimizing the side effects of glucocorticoid therapy. Bone age clitoris bilder somatic growth data are also used to determine the efficacy of the chosen steroid replacement regimen. Equally important to any discussion of such a sensitive topic is an understanding of long-term patient outcomes. These arteries course ventrally and are on the medial aspect of the bifurcated corpora, where they then course dorsally along the phallic shaft 1.

The dorsal vein and suspensory ligaments were then divided. Treatment of the mild or non-classic form clitoris bilder targeted at controlling excess androgen symptoms and may or may not involve glucocorticoid therapy. This can either be due to the pregnant mother suffering from masculinizing tumors such as arrhenoblastoma [Ovarian Sertoli-Leydig cell tumors SLCTs ] of the ovary or exogenous intake of androgenic hormones 6 — 8.

The neurovascular bundles were then dissected off of the shaft and the crura were separately mobilized, ligated, and divided.

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A far less common condition that can cause enlargement of the phallus in the genetic female is exogenous in utero androgen exposure. The four recognized morphologies include horseshoe, trumpet, coffee bean, and tent 4. The clitoris, like the penis, consists of two corpora cavernosa. Adrenal androgen concentrations later in the day and after medication will be lower, and target levels for hormones measured in this manner are unknown; thus, hormones are best measured early in the morning and before medication.

The corpora cavernosa were clitoris bilder dissected proximally to the bifurcation of the crura and each of the crura was then separated from its attachment to the inferior aspect of the ischial rami. This choice should only be undertaken after extensive multidisciplinary assessment and counseling of the parents. Excision of the corporal bodies with preservation of the glans clitoris and clitoris bilder ventral mucosa was initially described by Goodwin and later expounded upon by others 3238 — In the earliest version of this operation, an incision was made at the dorsal base of the clitoris.