© Association for the Bladder Exstrophy Community Fertility and the Exstrophy Patient Although almost all exstrophy males (including cloacal, classic and isolated epispadias) produce healthy sperm in their testes, many have problems fathering children. The problem lies in the delivery of the sperm to the egg. Some problems relate to retrograde ejaculation while others do not ejaculate at all or have very little ejaculate. Retrograde ejaculation in exstrophy males occurs when the semen travels retrograde (backwards) into the bladder because of a malfunctioning bladder neck muscle that usually closes off during normal ejaculation. Those with little or no ejaculate may have insufficient muscle or misshaped prostates to achieve forceful ejaculation. Most men report that some semen dribbles out with orgasm but others report that there is a regular discharge of semen from the meatus (tip) unrelated to sexual activity. In exstrophy females, the problem with infertility is entirely anatomical. The introitus (the vaginal opening) is narrow and almost always requires surgical enlargement. Thereafter, these females appear to have normal fertility. There is at least 50% incidence of uterine prolapse and few women with exstrophy are able to have more than 2 children before repair of prolapse. As the repair involves a sling around the cervix, further pregnancy may be impossible. Some men with retrograde ejaculation may be taught to collect some semen in a syringe from their bladder to use it themselves for artificial insemination. In most cases however, there are several options for the ‘infertile' exstrophy male to investigate. A clinical treatment of retrograde ejaculation entails the collection of sperm from the urine (in the bladder after ejaculation) that is then ‘washed' and prepared for use in an assisted fertilization procedure. Three of the most commonly used assisted fertilization procedures are:
In complicated cases of infertility where sperm count is low, the sperm condition is poor, or after unsuccessful intrauterine insemination or IVF, the next step may be ICSI (Intracytoplasmic Sperm Injection). ICSI is a procedure that was first developed in 1992 in Belgium to help infertile couples undergo in-vitro fertilization. In cases where no viable sperm can be obtained from the ejaculate or the bladder, sperm can be obtained directly from the testes or the epididymis (coiled tubing outside the testicles which store sperm). A single sperm is then injected directly into an egg to be fertilized (Fig.1). If the egg were fertilized and viable, the next step would be to inseminate the donor. Fertilization occurs in 50% to 80% of injected eggs and approximately 30% of all ICSI cycles performed in the United States in 1998 resulted in a live birth, which is comparable to rates seen with traditional IVF. Because ICSI is a relatively new technique, long-term data concerning future health and fertility of children conceived with ICSI is not available. Some IVF clinics show an increased incidence of hypospadias and neural tube defects in babies conceived through ICSI. Increased incident of hypospadias may reflect inheritance of mutant paternal genes that were the cause for the spermatogenic abnormalities necessitating ICSI. Increased neural tube defects were seen in registries in Australia and New Zealand but registries elsewhere show results comparable to the rest of the population. This is an area of ongoing investigation. Because some causes of male infertility are familial and are related to genetic problems, male offspring might have reproductive problems as adults. Although the cause of bladder exstrophy is unknown, we are given statistics that the bladder exstrophy patient has a higher than normal chance of having a bladder exstrophy child (which seems to imply some genetic cause). Now that many if not all bladder exstrophy males and females can become parents, we may in fact see a rise in bladder exstrophy in the future (and other birth defects). Despite these concerns, ICSI is a still a major advance in the treatment of severe infertility.Given the high costs of fertility drugs, in-vitro fertilization and intracytoplasmic sperm injection, (anywhere between $5,000- $20,000US) these treatments are unfortunately inaccessible to the majority of the infertile population. Some U.S. states have mandated insurance coverage for infertility treatment. Some countries are progressive in dealing with female infertility, and countries such as Australia and Canada (Ontario only) do fund (government) limited cycles of in-vitro for female infertility caused by fallopian tube obstruction. Based on the state of the health care in many countries it may be years before government funding will allow for extra funding for male infertility.If you are considering looking into in-vitro fertilization and/or ICSI, we urge you to talk to your urologist and check out the references at the end of this article, in particular the fact sheet on ICSI that is available through the American Society for Reproductive Medicine and the Human Fertilization and Embryology Authority (HFEA) in the UK. IVF clinic statistics in the United States are listed on the Center for Disease Control website. The HFEA also publishes a “Patient Guide to IVF Clinics” that lists in-vitro success rates in the UK. If you have any further questions or would like to comment on this topic, please do not hesitate to email me at bdward@rogers.com or contact the ABC at (910) 864-4308. Further Reading and Websites:
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