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.
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
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
Picture 6: Dermal graft sutured at the site of tunical defect. No chordee noted after dermal graft.
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.
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
Picture 9: 15mm wide flaps marked and incised.
Picture 10: Urethroplasty done over 7Fr Infant feeding tube, dartos flap raised and sutured over the urethroplasty and glansplasty done
Picture 11: Completion of urethroplasty and follow-up at post operative day 12
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.
