Twin babies with Hypospadias- Dilemmas in management!

Last Monday was like any other Monday. My pediatric urology clinic was full with appointments and I was getting into the rhythm of seeing children with complicated urinary problems and hypospadias. A young couple walked in with twin male babies, each about 7 months old. The boys were naughty and attentive and soon were soaking in the yellow and pink and greens of my outpatient clinic. I looked at the file and asked them the pediatric urological issues for which they were seeing me that day. They said their kid had hypospadias and both of them had hypospadias infact and they wanted to see a Pediatric urologist. Now that was a shocker!! Though this was not the first time that I was seeing twin boys with Hypospadias, but it was that once in two years phenomenon. And more striking since these boys had exactly the same type of hypospadias- which was distal penile hypospadias with mild chordee. Last year I had treated two brothers with hypospadias but the age was 3 and 5 years and thus difference in age was 2 years. The elder one has a mid penile hypospadias while the younger one had a distal penile hypospadias. I suddenly remembered the dilemmas which face a Pediatric Urologist & a Hypospadiologist while treating siblings with hypospadias.

• Which boy do you treat first – plan according to age – elder first or severity wise and severe hypospadias first and minor later?

• Whether you do them the same day or one after the other or with some gap of days in between?

• How will the parents manage two babies getting operated for the same thing at the same time?

• And if you operate one baby with hypospadias first and second one a few days later- what is the effect on their minds? How do they cope up with a situation where one has undergone a hypospadias surgery and the other one is waiting. Does the second kid get scared seeing the first one in pain after surgery and undergoing hospital visits and checkups? It was a tough situation to be in – for me as a hypospadias surgeon and even more for the parents who will have to care for the children 24×7 after surgery.

After a lot of deliberation, we decided that we do the hypospadias repair one after the other but on the consecutive days at MITR Hospital & Hypospadias Foundation. We admitted the elder sibling (with more severe hypospadias) for surgery on Tuesday morning and surgery was conducted as the first case in the morning. The younger kid was admitted on Wednesday morning and underwent uneventful repair on Wednesday itself. We arranged for two beds in one room so that both of them can share their side of the story. At least here there was no scope of having a feeling of being left out. Both of them for a fact got a HYPOSPADIAS SURGERY. Recovery period after surgery was smooth and both of them were discharged on Wednesday evening. The kids came back for follow-up on the same day after a week and their catheters were removed the same day. The children pee’d and actually compared their streams and for me luckily- both had good thick straight urine streams without any complications. Deep inside, throughout the recovery period, I was a bit worried about a complication happening. Luckily, everything went well and I had two happy kids with me. Now coming back to present case at hand- where I have a set of twin babies with hypospadias. So going by my previous experience, I think we will stick to the same plan. The only advantage here is that the kids are still less than one year of age and they won’t think much about surgery. I am planning to do them on a Tuesday and a Wednesday and then send them home on Wednesday itself.

Medicine lends itself to unusual off the script events every day and as we say every patient has a story. For us at Hypospadias Foundation – we believe every child hypospadias has a story with happy ending.

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    My child is very young! Is it a safe age to do hypospadias surgery?

    One of those questions which parents often ask and which is probably one of the most difficult to answer. But none the less the Question requires its due answer! To start from the basics, Hypospadias is a condition which is generally diagnosed immediately after birth by the pediatrician / neonatologist. The anomaly is very evident by an incomplete prepuce (foreskin), bare glans (head of penis), often a bent penis (called chordee) and misplaced urinary opening. Mostly it is an isolated anomaly but sometimes there may be an associated undescended testis with hypospadias. Since hypospadias is diagnosed at birth, ideally the child should be referred for a surgical opinion from a pediatric urologist or a surgeon trained in doing hypospadias surgery in the first few months of life. Early referral helps as the parents can then plan their lives around treatment of hypospadias. Hypospadias is a problem that always requires surgery called urethroplasty except in very minor varieties such as glanular hypospadias without chordee. It has been shown in multiple clinical studies that children do better when they are operated for hypospadias in infancy itself, ideally around 6-12 months of age. The reasons for these recommendations are multiple:

    1. At this young age the tissues are very soft and tend to heal very well after surgery.

    2. Emotional and psychological issues around getting operated in genital area are non- existent at this age.

    3. Once the surgical result has been good – children at a later age don’t even notice or come to know that they ever had hypospadias as there is hardly anything to differentiate these children from other children. For most of the distal and moderate hypospadias, penis after surgery just looks like a circumcised penis. There is also a debate whether these children should actually be told that they had hypospadias earlier on in their life.

    4. Incidence of wound infection, post surgery bleeding etc are much less at younger age as bacterial colonization is much less.

    5. Post- operative pain and erections are less common at this age.

    6. Management of a small child in diapers and urinary catheter is much easier in infancy. Typically we use a double diaper care where the catheter is brought out through a small hole in the inside diaper to drain into the outer diaper. The inner diaper remains dry and thus prevents rashes and outer diaper is changed whenever it gets wet with urine. In an older child, diapers are very difficult and bulky to use.

    Considering all this, the general consensus of American Academy of Pediatrics is to recommend surgery between the ages of 6 months to one year. At hypospadias foundation and MITR Hospital, we have been operating children around the age of 6-9 months for the last few years with excellent surgical results and happy parents. Once the surgery and the post operative recovery period is over, parents often come and tell us that it was a good decision that they got their kid operated at this early age and now all their worries are over. Some of the parents still worry about getting their kids operated in infancy and come at a later age for surgery. We and the staff can then see the difference in the recovery and post operative issues when compared to younger kids undergoing hypospadias repair. Another way to look at this is that a child with hypospadias will always need surgery, this is a condition which will never get better on its own. The longer we wait, more the parents will think about it and feel anxious. So earlier dealt with, the better it is for everyone. It is definitely better for the kid but it is in a way better for the family and parents as well. And surely it is better for the doctor as his job is to give the best results and post operative comfort.

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      Should a distal hypospadias be repaired!!

      I did not know what was in store for me – there was 6 new patients in clinic and only 4 follow-ups and by the nature of it new patients take more time and follow-ups finish very fast. Anyway, the first patient who walked in was a 3 year old accompanied by young smart looking parents. The boy flashed a big smile and I said hello and he was eager to shake hands. That was a positive sign – Acceptance of me as a friend. Good so far.It was a nice Saturday evening, the watch clicked 7pm and I was in my Pediatric Urology clinic at Vashi with the last 10 urology appointments and was slated to be back home by 8pm.

      Invariably on Saturdays I get late but today I was all energized to finish consultations fast and head back home for a quiet dinner and then catch up on much neglected sleep.

      Then we got down to the business of consultation- the parents had looked up about hypospadias on internet and had found me and then travelled all the way to Navi Mumbai on a Saturday evening for a consultation for their little one who had hypospadias. I examined the eager and cooperative kid- he has a coronal hypospadias with mild tilt of glans (the head of penis). There was no chordee (actual bend of the body of penis) and testes were in normal location. Overall, it looked like a milder distal hypospadias which should be fairly easy to repair with a predictably good result.

      I explained everything about hypospadias – how it happens and how it is repaired and then the QA began. The parents asked – “Is it necessary to repair it? We have heard that it can lead to long term urinary problems and sexual dysfunctions as sometimes the nerves get cut during sensation leading to a numb penis. Also, there are blogs which talk about no need to do surgery in distal hypospadias as there may be long term complications like hair growth, tight passage, fistulas etc.” They had visited an eminent Urologist who had advised against surgery.

      Well, they had done their due research and it was good but sometimes the truth out there may not be whole truth. I had to counsel them and make sure that they know the whole truth while at the same time sounding detached from the whole process. It was after all their kid and their responsibility- my job was to give full, fair and medically sound information and then their job was to make the right decision for their kid.

      So here are the facts as I discussed with them:

      1. As the boy grows and finally the penis size become adult like, the deformity becomes more and more obvious. The glans (penis head) which was tilted will look more and more abnormal.
      2. The prepuce will only be on the top of the head and not on the underside leading to an incomplete prepuce. It looks rather like the head of a cobra in adulthood
      3. The urinary opening will be on the underside of penis and the urine stream will always be downward instead of forward as in a normal child.
      4. The problems as stated above will be more cosmetic than functional but they affect the psyche as he will be able to see that the shape and configuration of his penis is quite different from that of other children. This can lead to deep seated emotional and social withdrawal issues in teenage and adulthood.
      5. Repairs are easier with predictable and better results in childhood. Later on in teenage and adulthood repairs have higher failure rates and pain. Further erections can cause pain and instability in the repaired area after surgery.
      6. The blogs out there contain stories of people who underwent surgery for hypospadias in childhood and now are adults. Thus the care which they received was 20-25 years back. The art & science of Hypospadiology has moved at much faster pace in last two decades and now- the understanding of anatomy of penis & hypospadias is much better, the surgical techniques have evolved and most of the repairs are done in single stage, new instruments for finer surgeries are available, new fine absorbable & non-reactive stitches are available and above all there are doctors who are well trained in managing the children with hypospadias.
      7. Earlier surgeries using skin from scrotum or other body tissues are not done any more so currently any repair causing hair growth in urethra is outdated and not done by surgeons.
      8. Complications like fistula, tight passage (stricture) are less than 5% in experienced, trained surgeons hands for milder distal hypospadias.

      To sum up, it is worthwhile to repair distal hypospadias considering all the above. The science of hypospadias surgery has made sure that the children get the best results for surgery in their early childhood and then live their lives normal without worrying about their reproductive organs.

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        Safe Surgery for Children with Hypospadias

        One of the important concerns for parents when their little ones are advised a surgery for hypospadias (urethroplasty), are risks associated with surgery. These concerns are true for a kid who is undergoing surgery for hypospadias as well. Safety is the foremost factor for parents while deciding a surgery for their kid.

        Same concern is valid for doctors (Pediatric Urologist, Anesthetist) and hospital team also. In some sense the safety checklist and precautions are important for any surgery which we plan to do in a child.

        The risks mainly stem from anesthesia which is needed for performing a surgery in a child. There are other risks associated with Hypospadias surgery itself but none of them are life threatening.

        To make surgery and anesthesia safe, the following precautions are undertaken:

        1. Pre-operative checkup for fitness: Blood and urine tests are done to make sure that the child is fit enough to undergo surgery and does not pose an increased risk of anesthesia. This also includes a thorough clinical examination to make sure that there is no infection lurking anywhere. In presence of any obvious infection such as cough, running nose, fever, diarrhea, skin infection etc it is better to postpone the surgery till the child recovers fully from infection. Infection leads to higher chances of infection at operated site and also slows down healing as body’s energy is spent in fighting the infection and thus healing takes a back seat.
        2. Sterile precautions: Instruments are properly sterilized the day before surgery and packed properly. The operation room is disinfected the evening before and locked. All sterile aseptic precautions are followed during scrubbing, draping etc.
        3. Availability of proper pediatric anesthesia equipments: Appropriate sized anesthesia equipment such as breathing tubes, masks, connectors, tapes, syringes, intravenous canula, intravenous infusion sets, needles, well calculated & checked anesthetic medicine doses etc are kept ready and labeled. Every single dose should be cross checked and labeled. Emergency medical equipment is cross checked before every single case and kept ready.
        4. Prevention of low body temperatures during surgery: Small children tend to lose a lot of body heat under cold environs of the operation theatre and thus get cold during surgery. The low body temperatures are called hypothermia. This can lead to multiple complications such as prolonged recovery from anesthesia, higher risk of infections and shivering. Hypothermia is best prevented – the steps taken are blanket to cover legs and upper body leaving only the lower tummy till mid thigh exposed for surgery, head cap and warm bandage rolls on arms and legs, electronic warming blanket underneath the baby set at 37-38 degrees, using warmed disinfectants, setting the AC temperatures to 25-26 degrees and not keeping them too low and then keep less operative area exposed and covering the rest of it with surgical drapes. For Hypospadias anyway a small area is exposed after preparing the middle part of the body with disinfectants.
        5. Dedicated Surgical equipment for kids: These include miniaturized microsurgery urethroplasty instruments, small retractors, small sutures (almost as fine as hair), fine clips, suction apparatus and smaller drapes. Larger instruments can crush the delicate tissues of kids and lead to poor surgical outcomes.
        6. Trained professionals: Doctors and nurses handling the kids undergoing surgery should be well trained in understanding that the kids are not small adults, their body behaves differently, the drug does should be perfect . The doses have lesser error margin and thus need to be calculated, cross checked and properly labeled. Putting an intravenous line requires training for handling kids and most importantly the Pediatric Urologist surgeon and the anesthetist should be very well trained in managing kids. Even a small excess of intravenous fluids during surgery can lead to major complications, that is the reason why pediatric infusion sets and connectors are very different from those in adults.
        7. Baby friendly environs of hospital: At MITR Hospital & Hypospadias foundation, we ensure that the kids are kept happy and engaged by the staff and the doctors during their hospital stay.

        Happy kids have a faster & better healing and this also decreases the complications of surgery.

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