Achievement of continence is problematic with exstrophy, as none of the normal structures to control the bladder exist, and they must be created through surgical intervention. Some techniques include vesicostomies, Mitrofanoff diversions, augmentation of bladder volume with sections of sigmoid colon, and so forth. In some cases, normal voiding through the penile urethra can occur, but in others a diversion of the urinary flow through means of clean intermittent catheterization through a stoma, or surgically created port, is necessary. Artificial bladder neck devices to control the bladder are available.
By the way, people with hypospadias can have difficulty with incontinence, too, although it is often more a leaking situation rather than a reduction in bladder voiding control. Lots of other conditions can cause incontinence, such as spinal cord damage or weakness of the pelvic floor muscles. Incontinence issues can be difficult to handle emotionally, but these issues can be “right sized” as the person accepts that incontinence is just another physical problem that we learn to deal with.
Note: Incontinence pads and briefs for men and women are available (you can buy them online if you’re shy) and they work pretty well, as I’ve learned from experience. –Betty
Fertility in men with isolated epispadias is often lower than fertiity in the normal population. Abnormal ejaculation, a lower sperm count, or azoospermia is seen in roughly half to three quarters of men with exstrophy-epispadias complex. Theoretically, if normal sperm are present in the testicles of these men, the potential for fatherhood exists, but assisted fertility techniques may be required to overcome poor sperm quality.
Women with epispadias who are pregnant may have more difficulty carrying their baby to term than other women because of differences in their pelvises and other organs. The gynecologist/obstetrician may decide to monitor pregnant women with epispadias more often and more carefully.
Treatment is surgical in all cases. The main goal of surgery for the exstrophy is closure of the abdominal wall and formation of a bladder, if one can be salvaged. The spacing or width of the pelvis can can be abnormal in cases of exstrophy, called diastasis, and surgery to rotate and join the halves of the pelvis often takes place in infancy.
Epispadias is corrected with techniques like the Cantwell-Ransley approach to close the urethra and the Kelly procedure. The main difference in epispadias is that the penis is very wide and very short. The erectile chambers or cavernosa in the epispadic penis are separated and are not connected to each other, which is not the case in the typical penis or hypospadic penis. The aim of the Kelly procedure is to bring the portions of the penis anchored to the pelvis together to conserve and liberate as much length as possible so the proportions of the penis are more typical. Correction of chordee follows standard techniques practiced in hypospadias repair.
Epispadias is caused by malformation of the embryonic genital tubercle and cloacal membrane sometime in the 5th or 6th week of pregnancy. It is not related to hypospadias, which is thought to be a hormonally moderated defect of penile formation and closure of the urethal plate which takes place about 8 weeks after conception.
Estimates are that epispadias with exstrophy occurs about once every 30,000 births, isolated epispadias without exstrophy once in 120,000, and isolated epispadias in females once in 500,000.
Epispadias is a malformation of the male genitalia in which the urethra ends or opens on the upper surface of the penis. This birth defect is most commonly observed as a part of the exstrophy-epispadias complex, which is a spectrum of failures of midline fusion affecting the pelvis, abdominal wall, and bladder. Characteristics of epispadias include a very short and wide penis often classified as complete epispadias when the entire urethra is exposed from the abdominal wall to the tip of the penis. There is usually upward bending or chordee associated with epispadias.
Epispadias can also occur in females, where it is manifested as a split clitoris and displacement of the urinary meatus.
Epispadias is always a part of the exstrophy complex, but can also occur as an isolated birth difference in a child with an otherwise normal bladder, pelvis, and abdominal wall.