Hypospadias Diagnosis and Repair
by Jessica Jewell
by Jessica Jewell
Our son was born with two congenital birth defects. He had a heart defect, a Ventricular Septal Defect (VSD), and Hypospadias, a defect of the urethra in boys. His heart defect was found at 40 days old and diagnosed by seven weeks. Around age nine weeks he was also given a failure to thrive label and was diagnosed with severe GERD shortly thereafter.
Hypospadias
Hypospadias is a condition where a male child's urethra has not formed correctly while in utero. This condition cannot be diagnosed by regular fetal ultrasounds. Hypsospadias are labeled in grades. My son, Deuce, had the least severe, called a Grade 1, without a chordee, which is a bend in the muscles of the shaft of the penis usually due to tethering of connective tissue. This meant that the actual opening to Deuce's urethra was about 1/4 of an inch off from the center of the head of his penis. His foreskin was also malformed in that it was all located on one side of the penis and the skin on one side was extremely thin as well.
Grade 2 and Grade 3 Hypospadias differ in that the opening is farther down on the shaft and therefore makes for a more difficult multi-stage repair. If a chordee is present it is also repaired so that normal sexual function can be achieved when the child is old enough.
In cases where a Hypospadias is present at birth, boys are not circumcised before going home. This is done so that the foreskin can be used during the repair to make the new urethra and close the old opening. After the repair is done, all boys will be circumcised because the foreskin is used in the surgery.
Deuce's Story
Deuce had a normal delivery but the Pediatrician was called into our room to check him after physical examination shortly after birth revealed some anomalies. The Pediatrician came over to my bedside and told my husband and me that our newly born son had a Hypospadias. At the time we had no idea what that was or any other issues that would go along with it. We were quickly told that his urethra and foreskin were malformed and that around the age of six months he would need to have it repaired in the operating room. We took this all in stride since otherwise Deuce seemed healthy. The pediatrician also told us that his Hypospadias was mild and should be a very easy repair. Kidney function is not affected in Deuce's case by his Hypospadias.
The only concern for care at home would be to watch out when we changed his diaper since the Hypospadias caused Deuce to pee sideways. This was always quite amusing and I got peed on quite often, especially since I was left handed and his opening was facing me.
We brought Deuce to the Urologist around age eight months to see when she would want to do his repair. Because of all of his other health issues with his heart, it was decided at that time that his repair would be put off until his GI tract and caloric intake was more stable. While it might sound odd that caloric intake would be of a concern, in order to give the body the best chance for a successful repair, we would want optimum calories to be available. We were told that having a Hypospadias repair requires a skin graft to close up the old opening, and kind of like a burn victim needs his nourishment to heal better, good nutrition is paramount.
Repair
Deuce had his first repair done in May of 2008. The surgery itself lasted about five and a half hours. Our urologist likes to take her time and do everything as slowly as possible. She is very meticulous.
During the surgery, the first step was to remove his foreskin through circumcision. They then made a small incision on Deuce's shaft and used the foreskin to create the new urethra. After that part of the surgery was done, the urologist used the rest of the foreskin to close up and repair the outside of the penis to ensure that it looked completely normal. This all sounds simple enough, but it is pretty painful.
Deuce was also given a catheter that stayed in until the new urethra had completely healed. The reason the catheter is placed is to ensure that the newly made urethra does not heal onto itself, thereby making the passage of urine impossible. A balloon is filled and the catheter is stitched in place with one stitch onto the head of the penis.
The whole penis is wrapped in a dressing to keep it stable over time. The dressing greatly resembles a larger version of the dressing used when a child is circumcised. Our urologist also had surgical tape from front to back in between his legs. To be honest, he was pretty black and blue from front to back and swollen quite a bit. The surgery causes a lot of trauma to that already delicate area.
In recovery Deuce was given a Caudal Block. He was given one at the start of the surgery as well. The block works like an epidural to keep the pain from being felt. It also made his legs useless for the rest of that day; he could not bear any weight on them. The Caudal Block kept the pain away so there was no need at that time for any stronger medication for pain. That night we were allowed to give him Motrin and regular Tylenol on a four hour rotation for pain. These two meds were enough to keep him pain free.
Practical Concerns during Recovery
We were taught how to do "double diapers" on Deuce to enable him to be swollen and more comfortable and to provide room for the catheter. Please see the pictures and description at the end of this article for more information. This entails taking a diaper of his size and cutting an X in the front of that area where his penis normally would be located. We had to fold back the sides of the hole we created and tape them down to come to a final diaper that had a huge hole in the front of it. We then put that diaper on Deuce allowing the dressing and catheter to hang out the hole. Over top of that we took a diaper two sizes too big and put that over the bottom diaper. We had to make sure that the catheter was not pinched or kinked. We got in the habit of making sure it was pointing up since that made Deuce more comfortable as well.
We had a scare the night we came home when he had a pretty big bowel movement. In doing so, he got feces all over his surgical site. Since we were not allowed to bathe him, we called the on call urologist who told us just to wipe it as clean as we could and it would be just fine. The diaper area is not a sterile environment so Deuce would be fine.
We realized the next morning that we needed to find a solution to the massive quantity of pee created overnight since Deuce received G-tube feeding for 10 hours nightly. The next night we used a feminine sanitary pad (without wings) and stuck it sideways across the tabs on the front to the diaper with the holes in it. We made sure that when we put on the next diaper over top that we laid the catheter onto the pad so that the urine would leak there first. This all ensured that we dealt with less absorbent "crystals" leaking from the insides of the diaper the next morning when we changed him. Each night we had a diaper then a pad then another diaper all on Deuce's bottom.
The next day he was as active as he always liked to be, which was actually quite an issue. No pressure was to be placed in between his legs at all. We had such a hard time keeping him calm, though we tried to treat him as normally as we could during this time. We brought him to the mall and to church to play. We just watched him closely to make sure he didn't get hit or fall on that area of his body. I also informed the church nursery to come and get me and I would change his diapers since they were complicated.
At his second post-op visit the week following surgery, it was noted that he had quite a bit of post-op swelling. He swelled so much that he broke many of the stitches used on the skin graft. The decision was made to keep the catheter in longer than normal because of the swelling. The urologist also did this to help prevent a fistula or passageway from forming. The catheter came out about two weeks later. Normally the catheter comes out at a week after surgery.
Physically, Deuce looked relatively normal at this point. The swelling itself could take up to six months to go completely down. We went on with life as normal. A bit of scar tissue was noticed, and we decided to wait until the swelling had gone down completely before deciding if it would need to be removed.
More Complications
Deuce was showing an interest in peeing on the potty before the repair, so we went back to letting him do so after he was all healed. It was during this time that we noticed he was dripping from the middle of his penis about where the "rim" would be located. I knew right away what had happened. I called the urologist and immediately made an appointment. This was not good news.
From our phone conversation, she was pretty sure that Deuce had developed a fistula, an abnormal passageway leading to a hole in his shaft. She saw us the next day and confirmed that he did indeed have a fistula. Upon physical exam, she could see urine passing out an improper point in his shaft. This, too, would require surgery, but we had to wait until he was at least six months post-op from the first one.
The fistula repair was done in December of 2008, and took about four hours. She took a tiny camera and inserted it into Deuce's urethra. This was done so she could see how well the inside of the new urethra had healed and to look for other fistulas on the inside as well. At this point we received a call that everything looked good and all that would need to be done was the fistula repair. If the urethra did not look good a complete re-repair on the whole site would have been needed. We were very relieved to get this phone call.
She cleaned up quite a bit of scar tissue that had developed and used some of that scar tissue to close up the fistula. She made a zig-zag incision on his shaft to pull together some of the excess scar tissue to give him an even more normal look. She also did it this way because no foreskin was available for this repair.
This second surgery was immensely different from the first. He was in almost no pain and only needed the Caudal Block without any of the extra pain medication afterwards. We came home with no catheter and no double diapering. He had virtually no swelling either. Within a few weeks, he was back to his old self in every way. His recovery was remarkable.
On the surgical table they also preformed an erection test. We asked to have one done because we had not seen a spontaneous erection since prior to his first repair. We, as worried parents, were concerned that he had lost his ability to have one. The erection test was done and was normal, though this only confirms that his penis is straight and not that he can have erections on his own. We were assured that none of the nerves or muscles involved in having an erection were cut during his two surgeries. The area that the surgery had been performed on was not near those nerve endings.
Success!
Today Deuce's genital area looks just like that of any other little boy who has been circumcised. If I look really closely I can see the scars on his shaft, but as he grows, these will become pretty much invisible. At this time, he is still being followed by his urologist since we are less than six months post op. He has no lasting effects from his surgery, and the only people who will remember all the pain will be his loved ones. For this fact, we are eternally grateful.
Double Diaper Procedure
Items needed for Double Diapering after a Hypospadias repair while Catheter is still inserted:
- Two diapers, one regular size and one two sizes too big
- A sanitary napkin without wings
- Serrated scissors
- 3M Transpore tape (non silky kind)
Use serrated scissors to make a cross hatch cut into the front of the diaper.
Fold back cross hairs of cuts and tape with 3M Transpore tape on corners.
Place diaper on child. Make sure packing and catheter is facing up. Also ensure that catheter is not kinked. (Packing and catheter not shown in picture.)
Place non winged sanitary pad across the diaper tabs of diaper making sure that catheter is laying on it. We only used the sanitary pad at night due to nighttime G tube feedings. During the day we made sure to change his diaper about every three hours and this reduced the crystals from forming over his catheter and incision packing.
Secure larger diaper over top.