Epispadias | |
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Specialty | Medical genetics |
Frequency | Rare [1] |
This article needs additional citations for verification .(March 2013) |
Epispadias is a birth defect in which the urethra fails to fully develop, resulting in urine leaving the body from an abnormal site. [1] In males, this may be an opening on the upper aspect of the penis, [2] and in females when the urethra develops too far anteriorly. It occurs in around 1 in 120,000 male and 1 in 500,000 female births.[ citation needed ]
Most cases involve a small and bifid penis, which requires surgical closure soon after birth, often including a reconstruction of the urethra. Where it is part of a larger exstrophy, not only the urethra but also the bladder (bladder exstrophy) or the entire perineum (cloacal exstrophy) are open and exposed on birth, requiring closure.[ citation needed ]
Despite the similarity of name, an epispadias is not a type of hypospadias, and involves a problem with a different set of embryologic processes. [1]
Women can also have this type of congenital malformation. Epispadias of the female may occur when the urethra develops too far anteriorly, exiting in the clitoris or even more forward. For females, this may not cause difficulty in urination but may cause problems with sexual satisfaction. Frequently, the clitoris is bifurcated at the site of urethral exit, and therefore clitoral sensation is less intense during sexual intercourse due to frequent stimulation during urination. However, with proper stimulation, using either manual or positional techniques, clitoral orgasm is definitely possible.[ citation needed ]
Epispadias is an uncommon and partial form of a spectrum of failures of abdominal and pelvic fusion in the first months of embryogenesis known as the exstrophy - epispadias complex. While epispadias is inherent in all cases of exstrophy it can also, much less frequently, appear in isolation as the least severe form of the complex spectrum. It occurs as a result of defective migration of the genital tubercle primordii to the cloacal membrane, and so malformation of the genital tubercle, at about the 5th week of gestation.[ citation needed ]
The main treatment for isolated epispadias is a comprehensive surgical repair of the genito-urinary area usually during the first 7 years of life, including reconstruction of the urethra, closure of the penile shaft and mobilisation of the corpora. The most popular and successful technique is known as the modified Cantwell-Ransley approach. In recent decades however increasing success has been achieved with the complete penile disassembly technique despite its association with greater and more serious risk of damage.[ citation needed ]
Even with successful surgery, patients may have long-term problems with: [ citation needed ]
In amniotes, the clitoris is a female sex organ. In humans, it is the vulva's most erogenous area and generally the primary anatomical source of female sexual pleasure. The clitoris is a complex structure, and its size and sensitivity can vary. The visible portion, the glans, of the clitoris is typically roughly the size and shape of a pea and is estimated to have at least 8,000 nerve endings.
The labia minora, also known as the inner labia, inner lips, or nymphae, are two flaps of skin that are part of the primate vulva, extending outwards from the inner vaginal and urethral openings to encompass the vestibule. At the glans clitoris, each labium splits, above forming the clitoral hood, and below the frenulum of the clitoris. At the bottom, the labia meet at the labial commissure. The labia minora vary widely in size, color and shape from individual to individual.
The urethra is the tube that connects the mammalian urinary bladder to the urinary meatus. In placental mammals, the urethra transports urine through the penis or vulva during urination and semen through the penis during ejaculation.
In female humans and other mammals, the clitoral hood is a fold of skin that surrounds and protects the glans of the clitoris; it also covers the external clitoral shaft, develops as part of the labia minora and is homologous with the foreskin in the male reproductive system. The clitoral hood is composed of mucocutaneous tissues; these tissues are between the mucous membrane and the skin, and they may have immunological importance because they may be a point of entry of mucosal vaccines.
The glans is a vascular structure located at the tip of the penis in male mammals or a homologous genital structure of the clitoris in female mammals.
Hypospadias is a common malformation in fetal development of the penis in which the urethra does not open from its usual location on the head of the penis. It is the second-most common birth defect of the male reproductive system, affecting about one of every 250 males at birth, although when including milder cases, is found in up to 4% of newborn males. Roughly 90% of cases are the less serious distal hypospadias, in which the urethral opening is on or near the head of the penis (glans). The remainder have proximal hypospadias, in which the meatus is all the way back on the shaft of the penis, near or within the scrotum. Shiny tissue or anything that typically forms the urethra instead extends from the meatus to the tip of the glans; this tissue is called the urethral plate.
Metoidioplasty, metaoidioplasty, or metaidoioplasty is a female-to-male gender-affirming surgery.
Vaginoplasty is any surgical procedure that results in the construction or reconstruction of the vagina. It is a type of genitoplasty. Pelvic organ prolapse is often treated with one or more surgeries to repair the vagina. Sometimes a vaginoplasty is needed following the treatment or removal of malignant growths or abscesses to restore a normal vaginal structure and function. Surgery to the vagina is done to correct congenital defects to the vagina, urethra and rectum. It may correct protrusion of the urinary bladder into the vagina (cystocele) and protrusion of the rectum (rectocele) into the vagina. Often, a vaginoplasty is performed to repair the vagina and its attached structures due to trauma or injury.
Pediatric urology is a surgical subspecialty of medicine dealing with the disorders of children's genitourinary systems. Pediatric urologists provide care for both boys and girls ranging from birth to early adult age. The most common problems are those involving disorders of urination, reproductive organs and testes.
Intersex medical interventions (IMI), sometimes known as intersex genital mutilations (IGM), are surgical, hormonal and other medical interventions performed to modify atypical or ambiguous genitalia and other sex characteristics, primarily for the purposes of making a person's appearance more typical and to reduce the likelihood of future problems. The history of intersex surgery has been characterized by controversy due to reports that surgery can compromise sexual function and sensation, and create lifelong health issues. The medical interventions can be for a variety of reasons, due to the enormous variety of the disorders of sex development. Some disorders, such as salt-wasting disorder, can be life-threatening if left untreated.
A genital tubercle, phallic tubercle, or clitorophallic structure is a body of tissue present in the development of the reproductive system of amniotes. It forms in the ventral, caudal region of mammalian embryos of both sexes, and eventually develops into a primordial phallus. In the human fetus, the genital tubercle develops around week four of gestation, and by week nine, becomes recognizably either a clitoris or penis. This should not be confused with the sinus tubercle which is a proliferation of endoderm induced by paramesonephric ducts. Even after the phallus is developed, the term genital tubercle remains, but only as the terminal end of it, which develops into either the glans penis or the glans clitoridis.
Bladder exstrophy is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm. Exstrophy means the inversion of a hollow organ.
The cloaca is a structure in the development of the urinary and reproductive organs.
The urinary meatus, also known as the external urethral orifice, is the opening of the urethra where urine exits the penis or vulva during urination. It is where semen exits the penis during ejaculation. The meatus has varying degrees of sensitivity to touch.
The development of the reproductive system is the part of embryonic growth that results in the sex organs and contributes to sexual differentiation. Due to its large overlap with development of the urinary system, the two systems are typically described together as the genitourinary system.
Diphallia, penile duplication (PD), diphallic terata, or diphallasparatus is an extremely rare developmental abnormality in which a male is born with two penises. The first reported case was by Johannes Jacob Wecker in 1609. Its occurrence is 1 in 5.5 million boys in the United States.
The mechanics of human sexuality or mechanics of sex, or more formally the biomechanics of human sexuality, is the study of the mechanics related to human sexual activity. Examples of topics include the biomechanical study of the strength of vaginal tissues and the biomechanics of male erectile function. The mechanics of sex under limit circumstances, such as sexual activity at zero-gravity in outer space, are also being studied.
Male genital examination is a physical examination of the genital in males to detect ailments and to assess sexual development, and is normally a component of an annual physical examination. The examination includes checking the penis, scrotum, and urethral meatus. A comprehensive assessment of the male genitals assesses the pubic hair based on Sexual Maturity Rating and the size of the testicles and penis. The exam can also be conducted to verify a person's age and biological sex. The genitourinary system can also be assessed as part of the male genital examination. During a genital examination, the doctor can detect any of the following: structural abnormalities, urethral opening abnormalities, problems related to not being circumcised, lumps, tumors, redness, excoriation, edema, lesions, swelling, cancer, hair-related issues, and many others. In some instances where a physical examination of the male genitals is not sufficient to diagnose an individual, then an internal genital examination using imaging or ultrasounds will be needed for further evaluation.
Clitoroplasty is a type of plastic surgery involving the clitoris. It encompasses several procedures, including clitoral reduction, clitoral reconstruction, and the creation of a neoclitoris in male-to-female gender-affirming surgery. These surgeries aim to retain or restore sensation and function in the clitoris, often employing nerve-sparing techniques.