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A 5 years old boy was referred to Hypospadias Foundation after two previous failed surgeries. The child had complications of previous hypospadias surgeries such as distal open area due to dehiscence, distal meatus, chordee (bend of penis) and a thin glans bridge. A 5 years old child failed hypospadias repair.

As a first step, the penis was degloved and chordee checked. There was still significant bending of the head of the penis (pic 2). Chordee correction: Chordee was corrected by Tunical Albuginea plication at 12 o’clock on the upper side of penis and there was no chordee on rechecking the penis (pic3). An onlay flap was harvested from dorsal penile skin with very good dartos blood supply for urethroplasty (pic4). The flap was rotated around the side of penis and brought to underside of penis for urethroplasty (pic5). The urethroplasty was finished uneventfully but there was severe skin shortage on the underside of penis due to two previous surgeries. Multiple z plasties were done to mobilize and stretch the skin for covering the underside of penis (pic6). A 5 years old child failed hypospadias repair.

Catheter was left in place for ten days for healing. After removal of catheter, the child passed urine in good stream from the tip and is doing very well without any need for further surgery after 6 months of checkup.

N.S., a one year old child was brought to Hypospadias Foundation with one failed primary hypospadias surgery which done at a hospital in Mulund, Mumbai. The child had undergone a Snodgrass urethroplasty with preputioplasty (prepuce preserving hypospadias repair) for a distal penile hypospadias. One year boy failed distal hypospadias operated. After removal of the catheter, the parents had noticed this stream and leakage of urine from the underside of penis at the site of previous opening.

They had rushed to us 2 weeks after surgery as the child was having a lot of pain while passing urine. On examination the child has swelling of the penis with local infection. We started the child on oral antibiotics and pain killers along with warm tub baths. Once the infection subsided, we did a check cystoscopy to make sure that the distal passage was ok. Parents were taught calibration with a feeding tube twice a week to check distal urethral passage. After 6 months, the child was taken up for reoperative hypospadias repair with correction of chordee which was still present (pic 1 & 2)

At examination on the table, there was a large fistula in the distal penisleregion and due to misalignment of new urethra because of previous surgery- we could not pass catheter easily into the bladder (pic3).

Incisions for the first step- degloving of the penis were marked (pic4). The penis was degloved (skin was taken down) and all the fibrous scarred bands were released to straighten the penis (chordee correction)- pic5. An artificial erection test was done to confirmed that the penis was absolutely straight without any chordee (pic 6). The urethral fistula closure was performed in multiple layers with 6-0 PDS stitch (pic7). Finally the excess prepuce was excised and a good cosmetic outcome and look like a circumcised penis was ensured. The catheter was left inside for 3 days.

6 months later, the child is passing urine well in a nice thick stream and the penis looks like just a circumcised penis.

Master S.K, a 3-month-old baby, was brought to our clinic with abnormal sexual organs of the baby. The referring doctor could not say whether it was a boy or a girl. A child with Disorder of Mixed Gonadal Dygenesis.

On clinical examination, the child had a severe hypospadias and right sided undescended testis which could not be felt on clinical examination.

A Disorder of Sex Development (DSD) was suspected and detailed testing was done. Ultrasound showed uterus behind the vagina. A special X-ray test called Retrograde Genitogram confirmed the presence of vagina, uterus and right fallopian tube.

Karyotype was done to study the chromosomal sex of the baby and it was 46XY/45XO instead of normal 46XY. 46XY/45XO karyotype is called a mosaic type whereby in 50% cell lines of the body Y chromosome is missing. This leads to faulty development of the reproductive organs. Based on this a diagnosis of Sex Chromosome DSD was made (also called Mixed Gonadal dysgenesis).

After a detailed discussion with the family and considering that the penis development was adequate, a male sex assignment was considered. Dr A.K.Singal along with his team discussed the case in detail.

Surgery was done to remove female internal organs and at the same time Single Stage Urethroplasty was performed using Modified Koyanagi Repair. Dr V.R.Jayanthi from Nationwide Childrens Hospital & Ohio State University, USA was the Chief Surgeon during the Surgery. A second minor repair for a urethral fistula was performed 6 months later; now the baby is 5 years old and is doing well.

Later at 12-13 years of age, he will need counseling and some hormonal replacement to help him with sexual function. He will be infertile but a functional individual having a proper meaningful role in the society, taking care of his parents and family.

Discussion on Disorders of Sex Development (DSD)

DSD or Intersex is a disorder where the sexual differentiation has not been completed. During the development of a normal child inside mother’s womb- till 2 months of pregnancy, it is impossible to distinguish whether it is a boy or a girl. Only after that depending on the chromosomes and hormones, sex is determined.

Sexual differentiation into a male or a female is a very complex and well orchestrated process and sometimes things may go wrong.

A baby may be born with incomplete sexual differentiation into a male or a female. Such condition is called ambiguous genitalia or Intersex.

Commonly these children may present with severe hypospadias, hypospadias with undescended testis or absent testis, ambiguous genitalia, clitoral hypertrophy etc.

It is important to realize that these kids except for the sexual organs are absolutely normal and can function as valuable and meaningful citizens. Just like a heart or brain disease, it is a medical disorder which is treatable.

With the advances in medicine most of these kids can be offered a good hormonal and surgical therapy which will remove the ambiguity and give them a functional male or a female status.

The problems these people face are from three fronts:

• Social
• Personal low self esteem
• Inadequate health care

As a result these kids are taken away by eunuch societies in their young age and denied a meaningful role in the society.

Team Effort is important in managing these children:

• Role of the family

o To seek proper medical help
o To emotionally support the child

• Role of the primary care doctors

o To refer these kids to a specialist as a early as possible
o Not to make hasty sex assignment as it is not possible in all cases to continue with the sex of rearing.
o Not to be judgmental and pass casual statements
o To maintain patient dignity and privacy.

• Role of the society

o To treat such kids as just another medical disorder
o To understand and offer equal opportunities in life
o To formulate legislation and create awareness about such disorders, that they are treatable like any other disorder.