Hypospadias repair





Definition

Hypospadias repair refers to a group of surgical approaches used to correct or reconstruct parts of the external genitalia and urinary tract related to a displaced meatus, or opening of the urethra. The urethra is the passageway that carries urine from the bladder to the outside of the body. Hypospadias is the medical term for a birth defect in which the urethra opens on the underside of the penis (in boys) or into the vagina (in girls). The word hypospadias comes from two Greek words that mean underneath and rip or tear, because severe forms of hypospadias in boys look like large tears in the skin of the penis.

Hypospadias is one of the most common congenital abnormalities in males. It was described in the first and second centuries A . D . by Celsus, a Roman historian of medicine, and Galen, a Greek physician. The first attempt to correct hypospadias by surgery was made in 1874 by Duplay, a French surgeon; as of 2003, more than 200 different procedures for the condition have been reported in the medical literature.

Hypospadias repair is, however, controversial because it is genital surgery. Some people regard it as unnecessary interference with a child's body and a traumatic experience with psychological consequences extending into adult life. Others maintain that boys with untreated hypospadias are far more likely than those who have had surgery to develop fears about intimate relationships and sexuality. There is little information about the emotional aftereffects of hypospadias repair on girls.


Purpose

Although there are several different surgical procedures used at present to correct hypospadias depending on its severity, all have the following purposes:

  • To permit emptying of the bladder standing up. The abnormal location of the urethral meatus on the underside of the penis forces many boys to void urine sitting down, which leads to anxiety about using public restrooms or otherwise being seen undressed by other males.
  • To correct a condition associated with hypospadias known as chordee. Chordee, which comes from the French cordée , which means tied or corded, is a condition in which the penis bends downward during an erection. This curving or bending makes it difficult to have normal sexual intercourse as an adult.
  • To prevent urinary tract infections (UTIs). It is common in hypospadias for the urethral meatus to be stenotic, or abnormally narrowed. A stenotic urethra increases the risk of frequent UTIs.
  • To lower the risk of developing testicular cancer. Hypospadias has been identified as a risk factor for developing testicular cancer after adolescence.
  • To confirm the boy's sexual identity by improving the outward appearance of the penis. The external genitals of babies with severe hypospadias may look ambiguous at birth, causing stress for the parents about their child's gender identity.

Demographics

Hypospadias is much more common in males than in females. In Canada and the United States, the incidence of hypospadias in boys is estimated to be 1:250 or 1:300 live births. In girls, the condition is very rare, estimated at 1:500,000 live births. One troubling phenomenon is the reported doubling of cases of hypospadias in both Europe and North America since the 1970s without any obvious explanation. According to a recent press release from the U.S. Centers for Disease Control and Prevention (CDC), data from two surveillance systems monitoring birth defects in the United States show that the rate of hypospadias rose from 36 per 10,000 male births in 1968 to 80 per 10,000 male births in 1993. In addition to the increase in the number of cases reported, the proportion of severe cases has also risen, which means that the numerical increase cannot be explained as the result of better reporting.

The severity of hypospadias is defined according to the distance of the urethral opening from its normal location at the tip of the penis. In mild hypospadias, which is sometimes called coronal/glandular hypospadias, the urethral opening is located on the shaft of the penis just below the glans. In mild to moderate hypospadias, the opening is located further down the shaft of the penis toward the scrotum. In severe hypospadias, which is also called penoscrotal hypospadias, the urethral opening is located on the scrotum. About 80–85% of hypospadias are classified as mild; 10–15% as mild to moderate; and 3–6% as severe.

In hypospadias, the urethral opening is at the base of the penis, instead of the tip (A). Tissue grafts are used to create an extension for the urethra (C) and alleviate the tight skin, or chordee, on the underside of the penis. (Illustration by GGS Inc.)
In hypospadias, the urethral opening is at the base of the penis, instead of the tip (A). Tissue grafts are used to create an extension for the urethra (C) and alleviate the tight skin, or chordee, on the underside of the penis. (
Illustration by GGS Inc.
)

Although the causes of hypospadias are not yet fully understood, the condition is thought to be the end result of a combination of factors. The following have been associated with an increased risk of hypospadias:

  • Genetic inheritance. Hypospadias is known to run in families; a boy with hypospadias has a 28% chance of having a male relative with the condition.
  • Genetic disorders. Hypospadias is found in boys with a deletion on human chromosome 4p, also known as Wolf-Hirschhorn syndrome; and in persons with a variety of intersex conditions related to chromosomal abnormalities. Several different genetic mutations responsible for a deficiency in 5-alpha reductase, an enzyme needed to convert testosterone to a stronger androgen needed for urethral development, have been found in boys with hypospadias.
  • Low birth weight. Several studies in the United Kingdom as well as in the United States have shown that male infants with hypospadias weigh less and are smaller at birth than controls. It is thought that these low measurements are markers of fetal androgen dysfunction.
  • Drugs taken by the mother during pregnancy. Diethylstilbestrol (DES), a synthetic hormone that was prescribed for many women between 1938 and 1971 to prevent miscarriage, has been associated with an increased risk of stenosis of the urethral meatus as well as hypospadias in the sons of women who took the medication. Boys born to mothers addicted to cocaine also have an abnormally high rate of hypospadias.
  • Environmental contamination. One proposal for explaining the rising rate of hypospadias and other birth defects in males is the so-called endocrine disruptor hypothesis. Many pesticides, fungicides, and other environmental pollutants contain estrogenic or anti-androgenic substances that interfere with the normal androgen pathways in embryonic tissue development—in birds and other animals as well as in humans.
  • Assisted reproduction. A study done in Baltimore of children who were conceived through in vitro fertilization (IVF) between 1988 and 1992 found that the incidence of hypospadias among the males was five times that of male infants in a control group.

With regard to ethnic and racial differences in the American population, the CDC reports that Caucasians have the highest rates of hypospadias, Hispanics have the lowest, and African Americans have intermediate rates. Other studies have found that hypospadias is more common in males of Jewish or Italian descent than in other ethnic groups.


Description

Correction of hypospadias in boys

The specific surgical procedure used depends on the severity of the hypospadias. The objectives of surgery always include widening the urethral meatus; correcting chordee, if present; reconstructing the missing part of the urethra; and making the external genitalia look as normal as possible. Most repair procedures take between one-anda-half and three hours, and are performed under general anesthesia. Mild hypospadias can be corrected in a one-step procedure known as a meatal advancement and glanduloplasty, or MAGPI. In a MAGPI procedure, the opening of the urethra is moved forward and the head of the penis is reshaped. More severe hypospadias can also be corrected in one operation, which involves degloving the penis (separating the skin from the shaft) in order to cut the bands of tissue that cause chordee, and constructing a new urethra that will reach to the tip of the penis. The specific technique of reconstruction is usually decided in the operating room , when the surgeon can determine how much tissue will be needed to make the new urethra. In some cases, tissue must be taken from the inner arm or the lining of the mouth. In a few cases, the repair may require two or three stages spaced several months apart.

There is some remaining disagreement among professionals regarding the best age for hypospadias repair in boys. Most surgeons think the surgery should be done between 12 and 18 months of age, on the ground that gender identity is not fully established prior to toilet training and the child is less likely to remember the operation. Some doctors, however, prefer to wait until the child is about three years old, particularly if the repair involves extensive reconstruction of the urethra.

Recent advances in hypospadias repair include the use of tissue glues and other new surgical adhesives that speed healing and reduce the risk of fistula formation. In addition, various synthetic materials are being tested for their suitability in constructing artificial urethras, which would reduce the risk of complications related to skin grafting .


Correction of hypospadias in girls

The most common surgical technique for correcting hypospadias in girls is construction of a new urethra that opens to the outside of the body rather than emptying into the vagina. Tissue is taken from the front wall of the vagina for this purpose.

Diagnosis/Preparation

Diagnosis

The diagnosis of hypospadias in boys is often made at the time of delivery during the newborn examination. The condition may also be diagnosed before birth by ultrasound; according to a group of Israeli researchers, ultrasound images of severe hypospadias resemble the outline of a tulip flower. Ultrasound is also used prior to surgical repair to check for other abnormalities, as about 18% of boys with hypospadias also have cryptorchidism (undescended testicles), inguinal hernia, or defects of the upper urinary tract.

Hypospadias in girls may not be discovered for several months after birth because of the difficulty of examining the vagina in newborn females.


Preparation

Male infants with hypospadias should not be circumcised as the foreskin may be needed for tissue grafting during repair of the hypospadias.

Some surgeons prescribe small doses of male hormones to be given to the child in advance to increase the size of the penis and improve blood supply to the area. The child may also be given a mild sedative immediately before surgery to minimize memories of the procedure.


Aftercare

Short-term aftercare

Many anesthesiologists provide a penile nerve block to minimize the child's postoperative discomfort. Dressings are left in place for about four days. The surgeon places a stent, which is a short plastic tube held in place with temporary stitches, or a catheter to keep the urethra open. The patient is usually given a course of antibiotics to reduce the risk of infection until the dressings and the stent or catheter are removed, usually 10–14 days after surgery.

The child should be encouraged to drink plenty of fluids after returning home in order to maintain an adequate urinary output. Periodic follow-up tests of adequate urinary flow are typically scheduled for three weeks, three months, and 12 months after surgery.


Long-term aftercare

Boys who have had any type of hypospadias repair should be followed through adolescence to exclude the possibility of chronic inflammation or scarring of the urethra. In some cases, psychological counseling may also be necessary.


Risks

In addition to the risks of bleeding and infection that are common to all operations under general anesthesia, there are some risks specific to hypospadias repair:

  • Wound dehiscence. Dehiscence means that the incision splits apart or reopens. It is treated by a follow-up operation.
  • Bladder spasms. These are a reaction to the presence of a urinary catheter, and are treated by giving medications to relax the bladder muscles.
  • Fistula formation. A fistula is an abnormal opening that forms between the reconstructed urethra and the skin. Most fistulae that form after hypospadias surgery close by themselves within a few months. The remainder can be closed surgically.
  • Recurrent chordee. This complication requires another operation to remove excess fibrous tissue.
  • Urethral stenosis. Narrowing of the urethral opening after surgery is treated by dilating the meatus with urethral probes.

Normal results

Hypospadias repair in both boys and girls has a high rate of long-term success. In almost all cases, the affected children are able to have normal sexual intercourse as adults, and almost all are able to have children.


Morbidity and mortality rates

Surgical repair of hypospadias has a fairly high short-term complication rate:

  • leakage of urine from the area around the urethral meatus: 3–9%
  • formation of a fistula: 0.6–23% for one-stage procedures; 2–37% for two-stage procedures
  • urethral stenosis: 8.5%
  • persistent chordee: less than 1%

Alternatives

There are no medical treatments for hypospadias as of 2003. The only alternative to surgery in childhood is postponement until the child is old enough to decide for himself (or herself) about genital surgery.

See also Orchiectomy .


Resources

BOOKS

"Congenital Anomalies: Chromosomal Abnormalities." Section 19, Chapter 261 in The Merck Manual of Diagnosis and Therapy , edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

"Congenital Anomalies: Renal and Genitourinary Defects." Section 19, Chapter 261 in The Merck Manual of Diagnosis and Therapy , edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

"Drugs in Pregnancy." Section 18, Chapter 249 in The Merck Manual of Diagnosis and Therapy , edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

"Infertility: Sperm Disorders." Section 18, Chapter 245 in The Merck Manual of Diagnosis and Therapy , edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.


PERIODICALS

Baskin, Laurence S. "Hypospadias, Anatomy, Embryology, and Reconstructive Techniques." Brazilian Journal of Urology 26 (November-December 2000): 621–629.

Fredell, L., et al. "Complex Segregation Analysis of Hypospadias." Human Genetics 111 (September 2002): 231–234.

Greenfield, S. P. "Two-Stage Repair for Proximal Hypospadias: A Reappraisal." Current Urology Reports 4 (April 2003): 151-155.

Hendren, W. H. "Construction of a Female Urethra Using the Vaginal Wall and a Buttock Flap: Experience with 40 Cases." Journal of Pediatric Surgery 33 (February 1998): 180–187.

Hughes, I. A., et al. "Reduced Birth Weight in Boys with Hypospadias: An Index of Androgen Dysfunction?" Archives of Disease in Childhood: Fetal and Neonatal Edition 87 (September 2002): F150–F151.

Klip, H., et al. "Hypospadias in Sons of Women Exposed to Diethylstilbestrol in Utero: A Cohort Study." Lancet 359 (March 30, 2002): 1102–1107.

Meizner, I., et al. "The 'Tulip Sign': A Sonographic Clue for In-Utero Diagnosis of Severe Hypospadias." Ultrasound in Obstetrics and Gynecology 19 (March 2002): 250–253.


ORGANIZATIONS

American Academy of Pediatrics (AAP). 141 Northwest Point Boulevard, Elk Grove Village, IL 60007. (847) 434-4000. http://www.aap.org .

American Board of Urology (ABU). 2216 Ivy Road, Suite 210, Charlottesville, VA 22903. (434) 979-0059. http://www.abu.org .

American Urological Association (AUA). 1120 North Charles Street, Baltimore, MD 21201. (410) 727-1100. http://www.auanet.org .

Society for Pediatric Urology (SPU). C/o HealthInfo, 870 East Higgins Road, Suite 142, Schaumburg, IL 60173. http://www.spuonline.org .


OTHER

Centers for Disease Control Press Release. Hypospadias Trends in Two U.S. Surveillance Systems [cited April 24, 2003]. http://www.cdc.gov/od/oc/media/pressrel/hypospad.htm .

Gatti, John M., Andrew Kirsch, and Howard M. Snyder III. "Hypospadias." eMedicine. January 31, 2003 [cited April 25, 2003]. http://www.emedicine.com/PED/topic1136.htm .

Santanelli, Fabio and Francesca R. Grippaudo. "Urogenital Reconstruction, Penile Hypospadias." eMedicine. November 6, 2002 [cited April 24, 2003]. http://www.emedicine.com/plastic/topic495.htm .

Silver, Richard I. "Recent Research Topics in Hypospadias." Society for Pediatric Urology Newsletter 1 (October 1999). http://www.kids-urology.com/HypospadiasResearch.html .


Rebecca Frey, Ph.D.

WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?


Surgery to correct hypospadias is done by a pediatric urologist, a surgeon with advanced training in urology as well as in treating disorders affecting children. According to the Society for Pediatric Urology (SPU), pediatric urologists educated in the United States have completed two years in a general surgery residency after medical school, followed by four years in a urologic surgery residency and an additional two years in a pediatric urology fellowship program.

Surgical procedures to correct mild or mild to moderate hypospadias with little chordee may be done on an outpatient basis. Correction of moderate or severe hypospadias with some chordee, however, involves hospitalizing the child for 1–2 days. Parents can usually arrange to stay overnight with their child.

QUESTIONS TO ASK THE DOCTOR


  • How often do you perform hypospadias repair, and what is your success rate?
  • How severe is my child's hypospadias, and what procedure do you recommend to correct it?
  • What do you consider the best age for corrective genital surgery and why?


User Contributions:

Helyn Joseph
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Jun 3, 2009 @ 11:11 am
This should help out some!!!!! I love you very much!!!
Leonard J. Douglas
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Jul 18, 2009 @ 11:23 pm
Thanks for your information on Hypospadias as my wife and I will be sponsoring a child here in the Philippines, and We needed to understand this child's medical Problem.
riyam
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Jul 20, 2009 @ 9:09 am
in case of hypospadia not operated at childhood,he is married one and half year ago;his wife had no pregnancy during this period;his wife investigations were normal ;but he had less than12.5%rapid progressive sperm ;what is the cause of infirtility....hypospadia or low active rapid sperm level??!!
prathap kumar
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Oct 14, 2009 @ 6:06 am
thank you very much for this information.my son underwent an hypospadiasis operation in 2008 in india . now a sack is formed under his penis. urine got collected in that sack and stone has formed. the stone was removed after surgery.the cathiter was blcked after 3 days they changed the dressing and removed the cathiter.now there is a tear on his stitch. will you be able to help me out in this.
xxx
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Jan 5, 2010 @ 6:06 am
I have been in the end of 2008 & suffering from penoscrotal hypospadias. All is well but formed fistulae and semen is not coming out of the constructed meatus. Urine is
Coming always but sometimes after few seconds late. Guide me Plz?
melissa
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Jan 15, 2010 @ 3:03 am
gud day, i have a son with this kind of illness, how can i seek assisitance . janakis3@yahoo.com
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Apr 1, 2010 @ 12:00 am
gud day..im have a hypospadia..severe one..im already 20 years old now..im wondering if theres a way to fix my defect...my parents here in the Philippines doesnt know about my situation..perhaps thats the reason why they just let my unusual penis be as it is..

please im seeking an assistance...
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Apr 14, 2010 @ 10:10 am
I have the same problem but i am 14 ...

Tell me what to do.
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May 12, 2010 @ 11:11 am
my son is already 13 and is about to have his circumcision when we found out about this defect, his doctor did not proceed with it instead we are reffered to a urologist, now we have to prepare for a major operation,it will be very expensive since it was found already in the late stage already.thank you for the information.atleast now it has opened my mind how to cope with situation.
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Jul 21, 2010 @ 10:22 pm
I'm 43 y o & was woundring if hypospadias at this age can be repaired ?
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Oct 9, 2010 @ 5:17 pm
Im 14 and i have hypospadias and my parents still dont know. Is it possible to repair it at my age?
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Nov 3, 2010 @ 6:06 am
Kindly my son has Hypospidas case and he did the operation from one and half month and he has a fistula could you please advice after how many month we can say that its a mature fistula that needs an operation to be fixed by.
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Nov 3, 2010 @ 7:07 am
Kindly my son has Hypospidas case and he did the operation from one and half month and he has a fistula could you please advice after how many month we can say that its a mature fistula that needs an operation to be fixed by.
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Jan 13, 2011 @ 4:16 pm
I am 42 and have been thinking of having the hypospadias repaired. Can you tell me more about this? When I urinerate it splatters everywhere so I usually sit to do this. I would love to be able to stand and urinerate. It is about a half an inch opening. I have never talked about this to my parents and they me either. There has been some sugery at birth, half of my foreskin has been removed and stitch marks all the way down the shaft of my penis. Can you give me any answers and advise?

Thanks: Chad
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Apr 22, 2011 @ 3:03 am
I need the list of very good hypospadias surgeons in Europe.
Please send me a list.
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Oct 16, 2011 @ 4:16 pm
YA I HAD A HYPO SURGERY WHEN I WAS BORN IT HAS AFFECTED ME IN MANY WAYS. IT WAS A VERY SERIOUS PROBLEM. I WOULD LIKE TO STAND AND SAY I HAVE ACHIEVED MANY RESULTS IN MY LIFE. I WAS IN THE 82ND AIRBORNE INFANTRY PARACHUTE AND I DID ALMOST 20 YRS OF MACHINING WORK. NOW IM A COMMERCIAL DIVER. I WISH I CAN HELP. I HAVE SO MANY STORY TO TELL. IF I COULD ELABORATE ITS HARD TO BELIEVE MY FAMILY AND CIRCUMSTANCES
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Nov 3, 2011 @ 10:10 am
I Have twine baby , both are boy, one of them hypospadias and at present he 18 month old . My question is it is quire able by the surgical treatment?
srinivas gorityala
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Nov 6, 2011 @ 10:22 pm
iam 29 year getting marriage...so please give me the solution for my problem, in my childhood i got hypospadisis surgery in MUMBAI...now i have the same problem,so is it possible to operation in this age..
Pradeep bhadauriya
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Dec 19, 2011 @ 3:03 am
Are hypospadias effected boys able to became a father
greg
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Jan 20, 2012 @ 2:14 pm
i had hypospadiis surgery 10 years ago,still having issues with restrictions,,recently saw a urologist and took x-rays,found restrictions in my urinary tract ,is saying i need reconstuctive surgery to take care of concern, said he would need to take tissue from my mouth and possible bladder to complete, does this sound common,any advice would be appreicated
Don
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May 11, 2012 @ 1:01 am
I had 5 repair surgeries from age 5 to 13. Everything works but it has caused severe psychological problems throughout my life. I am now 56 years old. I have an ugly penis which has kept me from searching out sexual partners for the past 14 years although I have had several partners throughout the years and no one has ever kicked me out of bed because of it. I don't like the way it looks and I would like to consider surgery for cosmetic reasons. I am also gay as if that has anything to do with it.
Where do I begin searching for the right surgeon? Should I contact a urologist and go from there?
Any replies will be appreciated.
Thank you, Don
hussain
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May 18, 2012 @ 8:08 am
SIR i want know, basically what is hypospadis is it Deficiency OR Disease OR any thing else?
jose
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Oct 31, 2012 @ 8:08 am
nw i am going 2 30yr old.maried 2 yrs before.last week i found one drs talk in tv about hypospadias.then i found my hypospadias.its just below the glands.aftr erection its curved down.i have difficulty in sexual intercourse..can get corrected in this age group.its difficult or not.aftr correction i can maintain the normal sex.pls give me the advise.
dmounsdon
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Nov 14, 2012 @ 11:11 am
I have a 15 month old grandson with severe hypospadius. His urethra emerges at the base of his penis and his penis is held in the downward position by a hood of skin. They have said that it will require two surgeries to correct it. His testicles are positioned on either side right next to his penis and he lacks a scrotum. His parents have been counseled that he should have that repaired as well but they are thinking that it is just cosmetic. Will he be sterile if that is not repaired due to the fact that a scrotum is reactive to heat and cold to keep the sperm at the right temperature ? I have heard that some men have trouble concieving if they wear tight briefs because the sperm die as the testicles are too hot. Also wouldn't his testicles be less protected from injury where they are now ? If they do have to repair that what skin do they graft that would be reactive to temperature ?
Brandi
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Apr 13, 2013 @ 11:23 pm
As I was reading this I realized that the reason the corrective surgery hasn't been performed on children since 2003, is because its considered cosmetic and or the laws regarding children needing corrective surgery due to un distinguished gender of the new born. My husband has this and underwent surgery as a baby. Has the world really become that complex that doctors can't tell if a child doesn't have a penis or is born with a birth abnormality? There is a woman worried about her grandson who was born without a scrotem, I'm not a doctor but I would have to say that that isn't cosmetic! We are talking about quality of life here! I understand the issue behind children born with no gender and the issues behind deciding a gender for a child through surgery. But that's not what this is! This is a child born with a known gender, with a birth defect that can be as severe as the tip of the penise split in two. Though the child isn't in pain and can use the bathroom, I don't think sex will be an option for that particular child as an adult. Here is my issue, I once read that this wasn't genetic but have since learned that it is. So if our son is born with hypospadios my insurance will not cover corrective surgery. Kinda makes a women have second thoughts about children, there for effecting the mans quality of life extreamly! Especially since my husband look forward to being a father.
khurram
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Jun 25, 2013 @ 8:08 am
i am 38yr old..last week i found one drs talk in tv about hypospadias.then i found my hypospadias.its just below the glands.aftr erection its curved down..can get corrected in this age group.its difficult or not.aftr correction i can maintain the normal sex.pls give me the advise.
Thibinyane R
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Jul 17, 2013 @ 3:03 am
I'm 43 yrs with hypo condition and have 3 children, is necessary to correct at this age?
Wispers
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Aug 4, 2013 @ 3:15 pm
I am a woman with this condition. Where can I go to get help for me and my family and coping, surgery. Where can I go! I am from NY and really need information about women with this condition. I understand it is quite rare, and would like to get help as I have symptoms to correct.
King
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May 6, 2014 @ 10:22 pm
Im 26 yrs old having a hypospadias.. Is there a chance to be repaired through surgery at this age??
David
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Jun 15, 2014 @ 4:16 pm
i'm 21 yrs old with hypo condition i don't know if i will recovery my illness bcz i don't if my country has qualified drs to correct my condition
martin
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Oct 19, 2014 @ 5:05 am
it is possible to have an operation i live in the uk i am 51 years old and am about to have an operation i did have operations as a baby but were not put right

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