Two stage repair of deceptive distal penile hypospadias with hypoplastic urethra and severe chordee

6-year male from Kerala presented to the Hypospadias Foundation clinic with complaints of passing urine from distal penile region associated with downward curvature of the penis (chordee). On examination, meatus was in distal penile region, but the urethra was thin till the proximal penile region. The stretched penile length (SPL) was 40mm and glans diameter (GD) was 12.5mm. In view of small glans diameter, he received two doses of injection testosterone in the pre surgery period which were given 1 month apart. Following the two doses, the stretched penile length improved to 35mm and glans diameter to 15mm.

He was planned for hypospadias repair and the family was counselled for single or two stage repair based on the degree of chordee.

The surgery was started by complete degloving which means the penile skin was completely taken down preserving its blood supply. After degloving, the chordee was assessed by artificial erection test. Here in this case after complete degloving, artificial erection test showed chordee of more than 45 degrees. This was because of the hypoplastic (underdeveloped) tissues on the underside of the penis. We decided to divide the urethral plate and mobilise the urethra. Spongiosum was raised on the sides first, urethral plate divided at coronal level and urethra mobilised till penoscrotal junction to fully evaluate the chordee. Chordee was reassessed and there was persistent chordee of more than 45 degrees. Site of maximum curvature was marked on the ventral side (underside of the penis) and three corporotomies were made by placing horizontal incisions over the tunica with middle incision at the site of maximum curvature. Chordee was reassessed and there was still significant chordee necessitating a ventral penile lengthening procedure called as dermal graft. The central corporotomy incision was deepened to full divide tunica and then cavernosal tissue was mobilised from tunica on all sides. A skin ellipse was harvested from the inguinal region, de-epithelized, and defatted to create a dermal graft. This dermal graft was sutured at the site of tunical defect with 6-0 PDS interrupted sutures. An artificial erection test following dermal graft revealed no chordee.

Once the chordee was corrected, the proximal urethra was sutured in the proximal penile region with 6-0 PDS. Glans wings were raised. Byars flap was created. Byars flap involves division of the dorsal prepuce in the midline and then rotation to the underside of the penis to cover the whole underside of the penis from glans till all around the meatus.

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Picture 1: On clinical examination, the meatus was in the distal penile region with thin urethra till the proximal penile region. Surgery was started by complete degloving

Picture 2: Artificial erection test showed chordee of more than 45 degrees

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Picture 3: Urethral plate divided, mobilised and chordee reassessed. More than 45 degrees chordee noted

Picture 4: Three ventral corporotomies given and chordee reassessed, persistent chordee of more than 30 degrees noted, hence planned to go ahead with ventral lengthening procedure

Picture 5: Deep corporotomy given at the site of maximum curvature and tunica incised, dermal graft harvested from the right inguinal region

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Picture 6: Dermal graft sutured at the site of tunical defect. No chordee noted after dermal graft.

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Picture 7: Dorsal prepuce (foreskin) divided in the midline and flaps rotated to cover the underside of the penis

Second stage of Hypospadias Repair:

After 6 months of first hypospadias surgery, the child was taken up for second stage urethroplasty. We checked chordee again before starting the urethroplasty (urethral reconstruction) and the penis was completely straight. The prepucial flaps which were done in first stage had healed very well. The flaps were wide and supple. 15mm wide incision was marked on the flaps and, local anaesthesia was infiltrated at the marked site and incisions were deepened till the corpora. Urethroplasty was done over 7Fr Infant feeding tube in two layers with 6-0 PDS absorbable sutures. Dartos flap was harvested from both sides and sutured over the entire urethroplasty. Glans wings were raised. Distal urethroplasty and glansplasty was done, and a new meatus was created on the glans. The dressing in the post operative period was changed on post operative day 7 and catheter was removed on post operative day 12. Child was passing urine through the new opening in a good stream.

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Picture 8: Prepucial flaps 6 months after stage 1 were healed very well. Flaps were wide and supple. Artificial erection test done before urethroplasty done showed no residual chordee

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Picture 9: 15mm wide flaps marked and incised.

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Picture 10: Urethroplasty done over 7Fr Infant feeding tube, dartos flap raised and sutured over the urethroplasty and glansplasty done

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Picture 11: Completion of urethroplasty and follow-up at post operative day 12

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Picture 12: Urine stream following catheter removal

Distal penile hypospadias with severe chordee (Deceptive hypospadias)

In some cases of hypospadias, the urethral opening is not only in an abnormal location but also the urethra can be underdeveloped or hypoplastic. Hypoplastic urethra is characterized by thin, underdeveloped, paperlike quality of urethral plate and the existing distal urethra. While the meatal opening may be near the tip, the presence of a hypoplastic or underdeveloped urethra indicates a more complex form of hypospadias, even if the urethral meatus appears relatively distal. This is often associated with chordee which contributes to the overall complexity of the hypospadias.

The hypospadias repair in hypoplastic urethra is more challenging. This thin urethra does not have enough robust tissue to create a new functional urethra. Using this thin urethra in reconstruction can lead to complications such as fistula formation, stricture, diverticulum formation or dehiscence.

The surgical management in hypoplastic urethra often requires specialized techniques. Degloving in these cases must be done carefully because of higher chance of injury to the urethra due to its paper-thin consistency. The type of surgical repair in cases with hypoplastic urethra is decided based on the degree of chordee. If the chordee is not significant and if single stage repair is planned, then either the thin urethra must be laid open before staring the urethral reconstruction or spongiosa around the thin urethra has to be mobilized and sutured over the thin urethra which is called as Spongioplasty.

For severe cases, like in the above-mentioned case with significant chordee and extensive hypoplastic urethra, two stage repair may be necessary. In the first stage, the penis is straightened and prepucial flaps are created. In the second stage which is 6 months after stage 1, the flaps are tubularized to form the new urethra.

Long term follow in cases of hypoplastic urethra is necessary because complications like stricture can develop years after hypospadias repair surgery. Hence these complex cases of hypoplastic urethra with hypospadias should be performed by an experienced hypospadias surgeon.

At hypospadias foundation, we have operated many cases of hypospadias with hypoplastic urethra. Each case is thoroughly examined in the preoperative period, carefully degloved during the surgery, meticulously assessed and hypospadias repair is done considering factors such as degree of chordee, severity of hypoplasia and quality of urethral plate. This has helped us achieve success rate of more than 95% even in the most complex cases.

The team at Hypospadias Foundation has two experienced hypospadias surgeons. Dr A.K Singal is considered the best hypospadias surgeon in India and the world and has a special expertise in managing complex hypospadias cases. His profound anatomical knowledge, meticulous surgical skill and an unwavering commitment to restoring both form and function in hypospadias has transformed various lives.

Dr Ashwitha Shenoy is an expert hypospadias surgeon who along with Dr Singal has dedicated her life to treating children and adults with hypospadias. Their dedication extends beyond the operating room, offering compassionate guidance and hope to families navigating a challenging diagnosis.

What is urethrocutaneous fistula after hypospadias repair and why does it occur?

Hypospadias is a condition seen in 1 in 150 to 200 newborn boys. Surgical repair is the primary treatment for hypospadias, aiming to reposition the urethral opening to the tip of the penis, straighten any abnormal curvature and to improve the overall appearance of the penis. Like any other surgery, complications can occur even after hypospadias repair. The common complications which occur are urethrocutaneous fistula, meatal stenosis, glans dehiscence and rarely urethral diverticulum or urethral stricture. Among this the most common and notorious complication is the urethral fistula.

Urethrocutaneous fistula refers to the development of an abnormal opening between a newly constructed urethra and the skin on the underside of the penis. When this occurs the child or the adult will have more than one urine stream. There will be one stream from the tip of the penis (the newly created opening) and the other stream or leak of urine from the fistula site. The leaking of urine from the fistula site tends to fall on the legs or can wet the clothing causing inconvenience to the children and the adult. In our centre, we see lot of children coming for urethral fistula treatment after first surgery somewhere else like the case below.

Post hypospadias surgery fistula can occur due to multiple reasons. Some of them are as follows:

1. Intraoperative factors:

a. Poor blood supply: Poor blood supply also called as ischemia is one of the most common factors that can severely impair and delay tissue healing. The process of wound healing is complex and heavily relies on the continuous supply of essential nutrients along with oxygen via the blood stream. During urethroplasty if the blood supply of the tissues is poor then fistula formation can occur post-surgery due to poor healing.

b. Inadequate tissue closure: During urethroplasty, we must take care that there should be a watertight closure of the newly reconstructed passage. And this urethral passage should be reinforced with multiple layers of tissues such as local tissues or dartos flap so that the risk of fistula formation decreases. Gaps in the urethral closure with deficient overlying tissue layers can increase the risk of fistula formation.

c. Tight closure of tissues: The urethroplasty and overlying tissue closure in hypospadias should never be under tension. If the tissue closure is tight then the vascularity is affected and can cause ischaemia of the tissues leading to skin necrosis and fistula formation.

d. Narrow or stenosed urethra: The newly reconstructed urethra in hypospadias should be of good calibre. If the urethra becomes narrow, then there can be increased pressure within the urethra proximal to the narrow segment leading to a weaker point which gives away causing urine leaking from this pointwhich becomes a fistula.

e. Type and severity of hypospadias: Severe hypospadias or complex hypospadias is usually associated with severe penile curvature along with urinary opening situated far from the head of the penis. Longer urethral reconstruction is inherently more complex with higher risk of complications and hence higher chance of fistula formation compared to milder forms of hypospadias.

f. Fibrotic or unhealthy tissues: Fibrotic tissues are associated with poor blood supply and using such tissues for urethral reconstruction can be disastrous with higher possibility of dehiscence and fistula formation.

g. Surgeon experience: The risk of fistula formation will be higher at a centre performing very few hypospadias repairs compared to a centre performing more than 50-60 repairs every year. The technique, tissue handling etc gets better only once the surgeon performs more than 50 hypospadias surgeries every year.

2. Post operative factors:

1.Infection: If the operated hypospadias site gets infected in the post-surgery period, then there is a possibility of dehiscence at the site of infection which in turn can cause fistula formation.

2.Poor nutrition: Optimal nutrition is not just beneficial but fundamental for wound healing. Deficiency in key nutrients can cripple the body’s ability to repair itself leading to weakened tissue, increased infection risk and ultimately wound breakdown and higher chance of fistula formation

3.Presence of constipation: Constipation can indirectly contribute to wound breakdown. Straining to pass stool can put pressure on the penile area and can put undue stress on the delicate tissues and new sutures which can give away and cause fistula formation.

Inspite of the best efforts of the hypospadias surgeon, fistula can occur after hypospadias repair. Even if fistula occurs, there is a possibility that the fistula can close spontaneously. At Hypospadias Foundation, we wait for 6 months to assess the same and if it does not close then surgery in the form of fistula closure may be required. Before urethral fistula closure it is mandatory to check the urinary passage beyond the site of fistula. If the passage beyond the site of fistula is narrow, then simple urethral fistula closure may not suffice, and reconstruction of the entire distal passage (distal urethroplasty) may be necessary.

Hypospadias foundation is a centre located in Kharghar, Navi Mumbai, Maharashtra, India with surgeons having expertise in hypospadias repair in children and adults. We regularly manage both primary hypospadias repairs and complex cases including those with previous multiple failed repairs. A significant focus of our patient base consists of children and adults who have had failed hypospadias surgeries elsewhere, including persistent fistulas. Our approach involves not just good surgical technique but also diligent pre-operative assessment, focussed post-surgery care and critical decision making for good long-term outcomes.

Dr A.K. Singal is the founder and head of hypospadias foundation, India. He is considered the best hypospadias surgeon in India and the world and has successfully treated more than thousands of children and adults with hypospadias with excellent results.

Dr Ashwitha Shenoy is an expert hypospadias surgeon with special interest in the field of hypospadias and pediatric urology. Both Dr Singal and Dr Shenoy strive to achieve excellent outcomes in adults and children with hypospadias. Our success rate at hypospadias foundation for all types of repairs including complex and failed repairs are more than 95%.

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    Enhancing success rates in adult primary hypospadias surgery

    In the last few years, we have been treating a lot of adults coming with unrepaired primary hypospadias wanting to get hypospadias repair surgery done. Adult Hypospadias repair surgery is a very different ballgame from child hypospadias surgery. Traditionally clinical outcomes for adult hypospadias surgery have not been as good with pediatric hypospadias surgeries as:

    1. Chances of infection are high
    2. Due to erections at night, chances of dehiscence are high

    After having done hundreds of adult hypospadias surgeries, we can clearly see differences in adult hypospadias and children with hypospadias. Whether it is the size of penis, overall anatomy of tissues, pre-surgery preparation, intra-operative instruments and stitches or post-op care- everything is very different. Over the years, we have understood various steps which should be done to decrease risk of hypospadias surgery complications in adults. This has led to improved results in adult hypospadias surgeries.

    Our Stepwise Adult Hypospadias Surgery and Care protocol:

    1.Pre- surgery counselling: We make sure that expectations are set right. While hypospadias surgery can correct the curvature, cosmetic appearance and urine flow issues, hypospadias surgery cannot increase the size of penis or improve fertility. In our hypospadias clinic, after we have examined the adult hypospadias patients, we discuss what they want and the propose the outlined treatment plan.

    2. Pre-surgery tests: Before hypospadias surgery is planned, we like to do blood tests for ruling our diabetes, check out any risks for anesthesia or any infection. We also like to do a urine routine test to check for any infection. Chest Xray and ECG test are done to make sure that the person is fit for anesthesia. We also take a consult from a physician to make sure that the
    person is fit to undergo anesthesia for hypospadias surgery.

    3. Pre-surgery preparation: We start a betadine scrub bath for cleaning pubic area twice daily 2 days before surgery. Pubic hairs are not shaved as shaving 1-2 days before surgery can lead to higher risk of infections. Typically, we trim the hair in the OT with a hair trimmer.

    4. Surgery: General anesthesia with epidural block is given. All aseptic precautions are taken to prevent infection. Special microsurgery adult hypospadias instruments are used. Sutures used are also absorbable ones. Care is taken to handle tissues, nerves and blood vessels very gently. Dressing is done to support the penis and catheters are secured properly. Once the patient is out of anesthesia, we shift out to recovery room and thenward.

    5. Post-Surgery care: We give IV antibiotics for 2 days and also open the dressing on day 5 to check for any infection. For some cases, we also advise hyperbaric oxygen therapy for 5-7 days. Catheter in adult urethroplasty is kept for a longer period as healing is slow. We typically keep two catheters – a suprapubic catheter and a urethral catheter. Both are kept for 3 weeks.

    6. Follow-up: After urethral catheter removal, we typically clamp the suprapubic catheter and once the patient is passing urine from urethral well, we remove the suprapubic catheter after 2 days.

    By following this protocol, our results in adult primary hypospadias surgeries have become the best in India and amongst the top centres in the world. Our success rates in Adult Hypospadias Surgery are over 95% in primary one or two stage repair with very less number of people needing further surgeries. At Hypospadias Foundation, a dedicated team of surgeons takes care of adult hypospadias. Dr A.K.Singal is a reputed and top hypospadias surgeon who has been doing hypospadias repair surgeries since 2006 and is rated amongst the best in the world for clinical results. Dr Ashwith Shenoy is a hypospadias surgeon who has deep experience in managing hypospadias. Both of them work closely giving best outcomes to adults with hypospadias. Given their team work and dedication to success rates in hypospadias, it is no surprise that people come from all over India and more than 20 countries to seek hypospadias treatment under their care.

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      Safe and Effective Anesthesia for Hypospadias Repair Surgery in Children

      Hypospadias surgery is a procedure to correct the opening of the urethra on the underside of the penis. It is one of the most common pediatric urological surgeries. Ensuring your child’s safety and comfort throughout the surgery is paramount. Anesthesia plays a crucial role in achieving this goal.

      Types of Anesthesia for Hypospadias Surgery:

       General Anesthesia: The most common approach, general anesthesia renders your child unconscious and pain-free. Medications are typically
      administered intravenously (through an IV) or via inhalation through a breathing mask. The anaesthesia is usually short and there is no significant
      effect on the child.

       Regional Anesthesia: Regional anaesthesia is one which works only in the specific area of the body. The regional anaesthesia used in hypospadias
      repair is a caudal block, an injection near the tailbone for targeted pain control. This caudal block gives good pain relief for six to eight hours and
      sometimes even up to ten hours after hypospadias surgery. Older children may feel tingly and numb in the lower limbs and may find it difficult to walk till it wears off, usually within first 12 hours

       Combined Anesthesia: Combining general and regional anesthesia offers the benefits of both: comprehensive pain management during surgery and a smoother recovery from general anesthesia.

      The anaesthesia medicines chosen in children is such that there are minimal side effects and, we can resume oral intake as early as 1-2 hours after the hypospadias correction surgery.

      Pediatric Anesthesiologists:

      Anesthesia for hypospadias surgery is administered by a trained pediatric anesthesiologist, a medical doctor specializing in anaesthesia for infants and children and takes good care before, during, and after hypospadias repair surgery. They will meticulously assess your child’s health before hypospadias surgery, discuss anesthesia options, and choose the safest and most suitable approach for your child’s individual needs.

      Communicate and Ask Questions:

      Open communication with your child’s hypospadias surgeon & urologist and anesthesiologist is vital. Don’t hesitate to ask questions regarding the anesthesia plan and any concerns you may have. Feeling informed and involved in your child’s care can significantly ease anxieties.

      At Hypospadias foundation, we have trained pediatric anesthesiologists who have managed more than thousands hypospadias correction repairs. From airway management, right medications to post operative pain relief we believe in a complete care of the child from pre surgery to post surgery. Safe and effective anaesthesia is necessary for good post operative recovery after hypospadias surgery.

      Effective Pain Relief After Hypospadias Surgery

      Pain relief is very important especially in a child because a cranky child makes the parents and the hypospadias surgeon anxious. After any procedure, it’s natural for your child to experience some discomfort but the discomfort should be manageable. Here’s a guide to effective pain relief after hypospadias surgery:

      Doctor-Prescribed Medications:

       Pain relievers: After surgery we prescribe pain medications, often starting with acetaminophen(paracetamol) or ibuprofen. The medicines must be given as advised because good pain relief means a comfortable child and in turn leads to good healing after hypospadias repair.

       Antibiotics: To prevent infection, antibiotics are prescribed after hypospadias surgery. They must be continued till the catheter removal and
      sometimes for a few days even after the hypospadias catheter is removed. Infection can destroy the entire hypospadias repair hence we need to be
      vigilant and avoid infection at all costs.

       Bladder antispasmodics: The presence of a urinary tube can cause urinary bladder contractions. The only way to manage this is by prescribing anti spasmodic medicines. Hence after hypospadias surgery till the catheter removal, the child will be on anti-spasmodic medicines.

       Pain due to erection: Painful erections after hypospadias surgery occur in every child and are even more prominent in teenagers and adults. This pain can be quite bad due to swelling of penis and the presence of a urinary tube. Hence we prescribe a stronger painkiller diclofenac in the form of
      suppository during these episodes of severe pain. This medicine gives good pain relief in times of extreme pain. For adults we also add more medicine to decrease painful erections at night.

      At-Home Pain Management Strategies:

       Positioning: Encourage your child to rest comfortably, positioning themselves to minimize tension on the surgical area. This can be lateral or
      supine position with pillow support.

       Distraction: Engaging your child in their favourite activities or games can help take their mind off any discomfort after hypospadias surgery

       Loose Clothing: Tight clothing can apply pressure on the operated site and cause discomfort to the child. Opt for loose-fitting clothing made from
      breathable fabrics to avoid irritation.

       Dietary Adjustments: Prefer healthy nutritious food over processed food. Processed food does not have fibre and cause constipation in a child which in turn can cause bleeding at the operated site during straining. Maintaining hydration is also essential to prevent constipation.

       Hygiene: Maintain proper hygiene around the hypospadias surgical site as instructed by your doctor. Change inner diaper frequently when dirty and be vigilant for soling of dressing. If dressing gets dirty at any point, do visit the hospital and get a hypospadias dressing change.

       Activity Restrictions: Follow the doctor’s recommendations regarding activity levels to promote healing and prevent complications. Any vigorous
      activities are generally avoided typically for 4-6 weeks after hypospadias surgery. Children can play at home and other board games comfortably.

      Remember:

       Consult your Hypospadias Doctor: If pain seems excessive or worsens, consult your child’s doctor for further evaluation and possible medication
      adjustments.

       Be Patient: Healing takes time after a hypospadias surgery. Be patient with your child and offer support throughout the recovery process.

      At Hypospadias Foundation India, we have treated thousands of children and adults with hypospadias. Our approach to hypospadias repair includes
      comprehensive care from preoperative to post operative period. Remember, every child heals at their own pace. By being prepared, informed, and supportive, you can help your child through this journey and ensure a successful recovery. Dr A.K.Singal and Dr Ashwitha Shenoy are rated as the best hypospadias surgeons in India. Their experience in the field of hypospadias makes them the best surgeons for hypospadias repair for both children and adults. If you need an opinion or consult us for your child, please do write to us at hypospadiasfoundationindia@gmail.com or fill up this contact form or visit us at the hospital.

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