In the course of last few years of dedicated practice in Pediatric Urology and Hypospadiology, We realized that anxious parents have a lot of questions and they forget to ask many of them when they are consulting us in the clinic. Here I have put down some of these question/ answers and I hope it helps the parents in participating better in the care of their little ones.All in all it’s a team effort.
New molecular research, better understanding, long term follow-up data and better surgical techniques have allowed us to offer a more comprehensive approach including counseling, planned surgical correction and appropriate gender assignment in these babies.
1. Learnings about hypospadias treatment
Hypospadias Foundation at MITR Hospital at Kharghar, Navi Mumbai, India remains World’s First & only organization dedicated towards hypospadias and Disorders of Sex Development (Intersex). Every year, we treat more than 200 patients with hypospadias and these patients travel from all over India and 20 countries to get treated at Hypospadias foundation. In total, since November 2008, more than 2500 kids and adults with Hypospadias and DSD have benefitted from care at Hypospadias Foundation.
Our learnings from last 14 years can be summarized briefly here:
- Personalized Care & Surgery: No child with hypospadias is alike- even simple hypospadias can be deceptive and full effort should be made to treat every case with utmost care.
- Don’t miss DSD: Any child with hypospadias and undescended testis should be investigated for Disorder of Sex Development. This includes hormonal workup, Karyotype, Radiology imaging and participation of a pediatric endocrinologist
- Right age for surgery: We like to operate on children with hypospadias around between 9 months to 15 months of age. If the child is born preterm, is underweight, has any coexisting anomaly like cardiac issues- we like to postpone the surgery to 3 or 4 years of age. A close coordination with a pediatrician is important to assess fitness for surgery, general clinical condition and any significant medical history.
- Safe Pediatric Anesthesia: Children require specialized pediatric anesthesia by trained teams of pediatric anesthetists. Safety first, everything else later.
- Basics of Surgery: Chordee correction (correction of penile ventral curvature) remains the most important step in hypospadias surgery. If there is significant curvature, it is better to do a staged repair in view of better long term results. Single stage surgery with residual chordee is going to lead to long term issues.
- Counseling & supporting the parents: When a child is born with hypospadias parents have a lot of questions and a significant number are worried about future fertility and urination. A significant number also blame themselves. Making sure that you answer all their questions and support them, before and after surgery is very important. Here the parents are making a decision for their child, if something goes bad then they will leave with parental regret.
2. What is hypospadias?
This is a condition where the urinary opening (pee hole) is not in the correct place but located on the underneath surface of the penis. The type of hypospadias is described by where the opening is. The mildest form (glanular) is where the opening is on the glans (see diagram). In moderate hypospadias the opening comes where the glans meets the body of the penis (coronal and sub-coronal). Openings farther back (on the penis itself or at the base of the penis) are the severe varieties. In addition to the hole being in the wrong place, the foreskin is often incompletely formed on the undersurface and looks like a hood on the top. Sometimes the penis is bent downwards (chordee) usually due to tight skin but sometimes due to abnormality of the body of the penis.
3. Is it common?
Yes. It occurs to some degree in 1 in 150 to 200 boys. In India every year more than 80,000 babies are born with hypospadias.
4. Is it associated with any other abnormalities?
Mild to moderate hypospadias are rarely associated with other abnormalities, so no further tests are required. If the hypospadias is very severe or there is also an undescended testis or penile size is very small then further testing is required.
5. Why does it happen?
The urinary pipe called urethra normally forms from a strip of special skin forming itself into a tube on the under side of the penis. It closes up rather like a zip fastener pulling closed from the back end to the tip of the penis. For some reason the end part of the tube fails to form and remains as a flat plate. The underlying cause is unknown in most cases. There are several theories including increased female hormone like substances in the environment causing mild forms of hypospadias. Very occasionally, it seems to run in families.
6. Is surgery required?
Surgery may be required for 2 reasons:
- To make everything work properly, i.e. to make the urine comes out from the tip and to make the penis straight.
- For cosmetic reasons.
If the boy can pass urine forwards then the operation is purely cosmetic. However, if it is not operated then there is a risk of your child becoming upset by the appearance as he gets older. Surgical results are best in the first year of life.
7. When should the surgery be done in ideal circumstances?
Ideal age for surgery in most of the hypospadias is between 6-12 months of age. If there are any co-existing problems, then surgery may be delayed till they are sorted out.
8. Why are the babies operated at such a young age? Why not wait till they are older?
Anesthesia is relatively safe after 6 months of age, that’s why we wait till that age. In infancy the babies are still in diapers, so managing them post surgery at home is very easy for the parents. The catheter can just drip into the diapers and the baby can be sent home, the evening of surgery or maximum the next day. Further the skin and tissues are very pliable and heal very well at this age. Most importantly, erections and infections are also less of an issue at such a young age. Older kids have painful erections after surgery and also the post operative care may be difficult.
9. Will there be any problem in the future regarding married life and children?
Most of isolated hypospadias once corrected do not have long-term sequences. Only cases where further investigation is warranted to look for fertility issues are the cases where the hypospadias is very severe, there is associated undescended testis or the gender itself is in question. This can be ascertained by a set of certain investigations which your doctor will explain to you if required.
10. How are failed hypospadias managed?
We at hypospadias foundation specialize in managing children with previously failed surgeries done elsewhere. If a part of the urethra is salvageable and the penis is straight – then local flaps with good blood supply are used for giving a predictable good result. Dr Singal & Dr Dubey have performed more than 25 such surgeries for kids with failed surgeries elsewhere. If the previous urethra is totally unusable or the penis is still bent (chordee) – an extensive 2-3 stage procedure like buccal mucosa grafting and then urethroplasty may be required. Buccal mucosa refers to the lining of the inside of cheeks or lips, which is biologically closest to urethral tissues and adapts well to use in urinary system. This procedure is often the last resort typically in children with previously failed 3-4 surgeries. With growing experience, the requirement for such procedures has become less.
11. What are the usual precautions before surgery?
Babies are checked for fitness for anesthesia by a pediatrician/ anesthetist. A couple of blood tests may be required. Any history of bleeding/blood disorder in the family should be asked. The baby should be free from any infection elsewhere (cold, cough, diarrhea, skin infections etc.). Generally babies are kept empty stomach for 4-6 hours before surgery, as it is a requirement for anesthesia. Bathing is advisable in the morning of surgery.
12. How long does the surgery take?
Surgery time depends upon the severity of the hypospadias. Generally total time spent in the operation room (inclusive of anesthesia time) for a penile hypospadias is between 2-3 hours and more for severe hypospadias sometimes even 4-5 hours.
13. What does surgery involve?
There are many types of operations designed to repair hypospadias. Essentially the operations we use, try to bring the hole up to the correct position on the ‘head’ of the penis (glans), make sure that the penis is straight and repair or remove the foreskin all in one operation. Most of the hypospadias repair operations can be done as day care procedures (in and out of hospital the same day). Sometimes the child may need to stay in hospital overnight and have a tube (stent) draining the urine for a few days. Our doctor will explain the type of surgery planned for your child.
14. What is a stent?
The surgeon may decide to leave a tube (stent) into the bladder to drain the urine. This is left in place for 5 to 14 days depending on the type of the operation, and usually simply drains urine into the nappy. A bag can be attached for older children who no longer use nappies. If a stent is used for more than 2 days, antibiotics are prescribed to prevent an infection in the urine. The catheter is used to prevent urine running over the internal stitches, so that in the first 24 hours there is not so much stinging. When it is removed, the child may still find passing urine slightly painful but this gets better in 24 hours. For bigger operations, a catheter is used to keep urine from bursting through the stitches for a longer period to help healing. This catheter may irritate the bladder causing spasms in about 10% of cases. The baby cries out about every 30 minutes. If this happens it is easily treated by giving a medicine to stop the spasm.
15.What is the usual post-operative course?
Babies are usually allowed feeds within 3-4 hours after surgery, once they are fully awake and asking for feeds. Initially water and juices are started. If there is no vomiting, gradually milk and solids are introduced. Generally, babies are on their usual diet the morning after surgery. Diaper care is taught to the parents by the doctors and the nurses. Medications (syrups) are explained well and the discharge back home happens by evening or morning after.
16. When do we have to come again to the hospital after discharge?
First follow-up visit is generally arranged within 5-7 days after surgery, for removal of dressing. After removal of dressing, an antibacterial ointment is applied 4-5 times a day and at each diaper change. Depending on the type of surgery done, a second visit is arranged at 10-14 days for removal of catheter. A further checkup is done after 3-4 weeks, 3 months and at one year.
17. What are common problems after surgery that we should know about?
- Pain: Most of the time the babies are slightly cranky but manageable. They feel better at home, that is why we try to send them home as soon as possible. It is also easier for the parents to manage them at home. Further an analgesic (pain-killer) syrup is prescribed to help in pain relief.
- Spasms: Babies may have intermittent spasms due to irritation by the catheter. For this reason a small dose of bladder relaxant is usually prescribed. The dose may need to be adjusted if cramps still happen.
- Blood spotting in the diaper/ catheter may occur in the first few days. A few drops of the blood are acceptable. In case of continuing ooze, a hospital visit may be required, but this is very infrequent.
- Dressing issues: Dressing loosening up may occur in some babies. If it happens during first 2-3 days, then a new dressing is placed. After that, the dressing is just removed.
- Infection may happen and is the most common cause of the failure of surgery. To prevent this, broad spectrum antibiotic syrup is usually prescribed for 7-10 days. It is vital to prevent stool smearing up the dressing in immediate post-operative period.
- Fistula: Fistula is a small area of breakdown in the operated area, leading to sideways leakage of urine. Small, off centre fistulas may heal in due course of time, if the tip of the new urethra is not tight. Others may require repair 6-9 months later. The rate of fistula in experienced hands, in penile hypospadias is typically 5%.
- Stenosis/ Stricture: During healing period, the new urinary tube may become tight. Mostly this happens at the tip of penis and can be easily managed by daily calibration at home with a small feeding tube. Sometimes, if the repair becomes too tight, then a second surgery may be required.