Dr. A.K.Singal, Author at Hypospadias Foundation

April 04 2024

Hypospadias and small penis size: diagnosis and treatment

Hypospadias is a congenital malformation affecting about 1 in 250 male births, this amounts to more than 50,000 children born in India every year with hypospadias.in this condition, the urethral opening lies on the underside of the penis instead of its tip. While severity varies, hypospadias is sometimes associated with small penile size. Let’s delve into the facts about hypospadias, its effect on size and treatment options.

Understanding Hypospadias:

During development of penis between 8-12 weeks of pregnancy, the urethra (urinary pipe) starts forming from the base of the scrotum to penis, finally proceeding forwards towards the tip. Hypospadias occurs when the tissue forming the urethra doesn’t fully close during fetal development. This results in the urethral opening appearing anywhere on the underside of the penis, from the glans (mildest form) to the scrotum (severest form).

Hypospadias and Penile Size:

The relationship between isolated hypospadias and penile size is complex. Studies show the following:

No significant difference in length: Research suggests that overall penile length in infants and prepubescent boys with isolated distal or minor hypospadias is comparable to unaffected boys in most boys. While the penis may be small in proximal or severe hypospadias.
Chordee causes the penis to look small: Hypospadias can cause the penis to curve downwards, a condition called chordee.This can make the penis appear shorter.
Surgical considerations: Reconstructive surgery for hypospadias aims to correct the urethral opening’s position and straighten the penis. While some techniques may involve using tissue flaps, these typically don’t significantly impact penile length.

Some people with hypospadias may have an actual short penis size. For this to be understood, lets first understand how hormones work to lead to penis growth:

Penis formation is complete by 12 weeks of pregnancy. During the rest of the pregnancy, under the influence of hCG hormone, which is released from placenta of the mother, the testes of developing baby produce a hormone called testosterone. Testosterone is the main male hormone, which is responsible for male pattern of facial hair growth, beard formation, muscle development, thickening of voice etc. which are male external features at puberty. However, testosterone is the not main hormone responsible for penis growth. Testosterone gets converted into dihydrotestosterone (DHT) which is much stronger variant of testosterone and is the main hormone responsible for penis size increase. Collectively, testosterone and dihydrotestosterone are called androgen hormones. Both testosterone and dihydrotestosterone act via androgen hormone receptors. For a moment think of a lock and key mechanism. Hormones are the keys which go and bind to receptors which are the locks. Working together, the lock and key, lead to penis development. So, inadequate androgen action either due to

Less production of testosterone
Testosterone not getting converted to dihydrotestosterone.
or receptors are not working,

Then the penis size will remain small.

Hormonal Reasons which may cause short penis length in hypospadias:

Hormonal disorders: Some children with hypospadias may have hormonal issues like testosterone production defect, androgen insensitivity or 5 alpha reductase deficiency. Whenever we find penis size to be very small along with hypospadias, we typically do a detailed hormonal evaluation to find out the real cause and then treat it.
Low birth weight or premature babies: Our experience shows that babies which are born ahead of their full-term date of birth usually have a smaller penis. This happens as the main penile growth in a baby in pregnancy happens in the last few weeks. If the baby does not receive hCG in the last weeks of pregnancy, then testosterone and DHT is also low, leading to smaller size of penis. Same thing happens in babies who have IUGR or low birth weight, their body produces smaller quantities of hormones for penile growth in the last few weeks of pregnancy.

Addressing small size of penis with hypospadias:

First, penile size should be measured in all children with hypospadias. Two measurements are important – penile length (stretched penile length) and glans diameter. Normally in a 6-month-old child, the SPL should be atleast 35mm and glans diameter should be atleast 14 mm.

If the child was born preterm or was low birth weight, and the glans diameter is atleast 11-12 mm, then as a first choice we give testosterone injections. These injections are given 1 month apart in 2 doses. Typically, it takes 3 months for the penis size to become normal. And then we plan the hypospadias repair surgery. Testosterone injection response also tells us how the penis will respond to natural testosterone at time of puberty and is a good marker to assure the parents and the doctor.

If the penile size is very small in a full-term baby or the baby has severe hypospadias, then we do a full hormonal test battery consisting of LH, FSH, AMH, Testosterone and then give 3 doses of hCG hormone to stimulate the testis. Post hCG stimulation, we test for testosterone and dihydrotestosterone again. There are three possibilities at this stage:

Testosterone does not rise: That means there is testosterone biosynthetic defect. This can be solved by giving external testosterone injections.
Testosterone rises normally but DHT does not rise as evidenced by T/DHT ratio: This is suggestive of 5 alpha reductase enzyme defects. Such children will require DHT gel to help them achieve normal penile size.
Both Testosterone and DHT rise normally: There is a possibility of androgen insensitivity in such cases. We give testosterone injections, but they may not work in all the cases.

All these tests and their interpretation should be done by an experienced pediatric urologist or a hypospadias surgeon or a pediatric endocrinologist.

Summary:

At Hypospadias foundation India, we have been taking care of children and adults with hypospadias for last 15 years with best-in-class results. Children with hypospadias and small penis should be well evaluated whenever needed by a hypospadias expert. This evaluation is important in childhood itself to lay a future healthy path for the child. There is detailed measurement and counselling done before any hypospadias surgery at our centre. Dr A.K.Singal is currently rated as the best hypospadias surgeon expert in India for such cases. Dr Singal works along with Dr Ashwitha Shenoy in MITR Hospital Navi Mumbai to help children and adults get good results for treatment of hypospadias. If you need a second opinion for hypospadias, please do write to us at hypospadiasfoundationindia@gmail.com or fill up this contact form.

References:

American Urological Association: https://www.hypospadias-society.org/
National Institutes of Health: https://www.ncbi.nlm.nih.gov/books/NBK564407/
Mayo Clinic: https://www.mayoclinic.org/diseases-conditions/hypospadias/symptoms-causes/syc-20355148

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March 03 2024

Why does hypospadias occur?

Why does hypospadias occur?

Hypospadias is a congenital abnormality of the penis with an incidence of 1 in 150 live births. In hypospadias, the urinary opening (peehole) is in an abnormal position on the underside of the penis. As a parent of a child with hypospadias, you may wonder what causes hypospadias. Infact this is the most common question which parents ask us in the clinic- Why did my child develop hypospadias?

The exact cause for hypospadias is not known but there are various factors which can increase the risk of hypospadias in your baby. This blog is to help you understand the probable factors which may lead to hypospadias in your baby boy.

Genetic factors:

Family history: While 90-95% of hypospadias are isolated cases, 5-10% of them do run in families. If the father or anyone in first degree family has hypospadias, then the risk of having hypospadias in subsequent male children increases.

Environmental factors:

Maternal exposure to pesticides or herbicides in pregnancy may cause hypospadias.

These pesticides are known to be endocrine disruptors, they interfere with the androgen and oestrogen signalling pathways during genital development, hence causing hypospadias. This is a hypothesis and studies are still under process to clearly link exposure of pesticides to occurrence of hypospadias.

There have been studies showing higher risk of hypospadias due to plasticisers in the single use plastic bottles. The chemicals here act as male hormone blockers and interrupt development of penis, hence causing hypospadias.

Hormonal exposure in pregnancy:

During pregnancy, certain hormones may be advised for the mother to decrease the chance of preterm labour or miscarriage. This is common after invitro fertilization (IVF) conception, twin pregnancy and in precious pregnancies. Carmichael reported that the use of progesterone to prevent early pregnancy loss was associated with risk of developing moderate to severe hypospadias.

Maternal factors:

Maternal pre pregnancy obesity with a BMI>30 has been associated with 1.3-to-2-fold increased risk of hypospadias compared to other women who had normal weight in the pre pregnancy period.

Maternal nutrition is very important for the development of fetus. Folic acid supplementation in the pre conceptional period is necessary to prevent various congenital anomalies. Study conducted by Mavrogenis et al showed that folic acid supplementation early in pregnancy was associated with low risk of hypospadias.

Hypertension in pregnancy and usage of anti-hypertensive medicines are associated with development of hypospadias in the fetus. Untreated hypertension has an odds ratio of is 2.1 and anti-hypertensive usage in pregnancy has an odds ratio of 1.6 for development of hypospadias. The cause for this is most likely because of reduced placental perfusion in hypertension.

It has been hypothesized that the maternal use of estrogen and progestin may cause hypospadias because of interference with fetal androgen production or action. Several other medications have been associated with moderate to severe hypospadias. These include ibuprofen (odds ratio 1.2), Venlafexine(odd ratio 2.4), proton pump inhibitors(odds ratio 4.4) and clomiphene citrate(ovulation induction agent with odds ratio 1.9)

Birth factors:

Infants with hypospadias are most likely to be born preterm, low birth weight or small for gestational age. These are more strongly associated with severe hypospadias than the milder forms of hypospadias. Placental dysfunction may fail to stimulate fetal androgen production which is necessary for urethral closure. It also causes reduced transfer of nutrients to the fetus causing growth restriction. Placental dysfunction and androgen deficiency in early pregnancy are known to cause intrauterine growth restriction and hypospadias.

The most important message here is that it is not the fault of the mother or father which can cause hypospadias in their child. The above-mentioned factors may increase the risk of hypospadias but that is not always true. A pregnant woman should take care of her health and avoid exposure to chemicals as much as possible. She should maintain a healthy diet, get good sleep, and avoid stress. Even if your child has hypospadias inspite of all the extra care, do not worry. Hypospadias is a condition that can be easily corrected by surgery by an expert pediatric urologist or a surgeon who specializes in hypospadias repairs.

At hypospadias foundation India, we have been blessed to treated families having twins or two brothers with hypospadias. We also have cases where father had hypospadias and then their child had hypospadias. Some of these parents themselves did not achieve good results in their day and age but now with latest techniques which we use at Hypospadias Foundation, the results which their children get after hypospadias repair surgery are very good.

In current era, success rate of hypospadias in first surgery itself is more than 95%. At hypospadias foundation, we treat hundreds of children and adults with hypospadias every year, both the ones which are primary or the ones where the previous repair has failed. If your child has hypospadias, do visit us, or consult us online to get the best hypospadias treatment for your child. Every child deserves the best and we are here to support you to give the best care to your child.

Fill up contact form: https://www.hypospadiasfoundation.com/contact/

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February 02 2024

Hypospadias and undescended testis: Diagnosis, surgery and treatment plan

Hypospadias and undescended testis (also called cryptorchidism) are both very common clinical problems in young children. While hypospadias occurs in 1/150 boys, undescended testis is even more common. But the treatment for both of them is different.

Undescended testis: What is the usual treatment plan?

When we see a newborn child with undescended testis, we often wait before advising surgery as nearly 60% of the testis which are undescended at birth may come down to a normal scrotal location by 6 months of age. This is even more true if the testis has already come out of the tummy and can be felt by the doctor during the examination in the OPD (palpable undescended testis). For the testis which are higher up in abdomen (intra-abdominal undescended testis) chances of coming down spontaneously are quite less but possible.

Hypospadias: What is the usual treatment?

Hypospadias on the other side, almost always requires surgical correction. And the right age for doing the hypospadias surgery is between 6-18 months of age. We typically advise the hypospadias repair surgery to be scheduled around 9-10 months of age. For hypospadias without chordee, it is usually a single stage surgery while for severe hypospadias or severe chordee, we choose to do a two-stage hypospadias repair procedure.

Both hypospadias and undescended testis occurring together- what does it mean?

When both hypospadias and undescended testis occur together, the diagnosis and treatment is much more complex. The most important thing to be done for these children is to investigate them for disorder of sex development or DSD. DSD is present in upto 15% of children who have both hypospadias and undescended testis. If the hypospadias is severe like scrotal or perineal hypospadias and the child has both sided undescended testis, the chances of a DSD condition go upto 50%.

So, what tests we do when we see a child with both hypospadias and undescended testis?

Any child who has hypospadias and undescended testis, should be investigated for a disorder of sex development. We are worried about conditions like mixed gonadal dysgenesis or true hermaphroditism or even severe variety of congenital adrenal hyperplasia.

Typical tests to rule out such conditions include:

Ultrasonogram to check for any abnormal organs inside, status of gonads.
Karyotype – to check for chromosomal abnormalities.
Hormonal evaluation – Genitogram or cystoscopy: some of these children if they have DSD also may have remnants of vagina or utricular diverticulum which can be seen only on a genitogram or a cystoscopy. MRI scan: may be needed in selected cases to rule out hemiuterus or fallopian tube.

Treatment plan for hypospadias and undescended testis

As outlined above, the first thing to do is to find out if the child has a DSD. If there is DSD, then we involve a pediatric endocrinologist and then consider assigning the gender and surgical plan starts from there.

If there is no DSD, then we prefer to do the hypospadias repair and undescended testis surgery together at around 9-10 months of age. If the undescended testis is palpable, it can be brought down in one stage via a short open surgery. If the testis is intra-abdominal then child will need a laparoscopic single or staged orchiopexy. For hypospadias, if there is no chordee or mild chordee, then we can do the orchiopexy and single stage hypospadias repair together. If hypospadias is very severe or there is severe chordee then we would need to plan for a two-stage hypospadias repair.

Summary: If your child has hypospadias and undescended testis, you must visit a qualified pediatric urologist and a hypospadias specialist. Before any surgery, a full evaluation has to be done as it has repercussions for future sexual function and fertility. But the good thing is that hypospadias and undescended testis repairs can be combined to help child get good result from both in one hospital stay whenever possible.

At Hypospadias Foundation India, we have treated thousands of children with hypospadias and some of them also had a undescended testis. A thorough evaluation and a plan for management for both was given and it worked very well for these children. Dr A.K.Singal and Dr Ashwitha Shenoy are rated as the best hypospadias surgeons in India. 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.

Keywords: undescended testis, cryptorchidism, hypospadias surgery, hypospadias and undescended testis, right age for hypospadias surgery, right age for undescended testis surgery, undescended testis surgery, disorder of sex development, surgery for DSD, hypospadias repair, hypospadias specialist, best hypospadias surgeon in india, hypospadias treatment in india

Fill up contact form: https://www.hypospadiasfoundation.com/contact/

January 01 2024

Does Hypospadias cause male infertility?

For some men with hypospadias, a congenital condition where the urethral opening lies somewhere on the underside of the penis, questions about fertility can loom large. While hypospadias itself doesn’t directly impact sperm production, its associated complications can sometimes pose challenges. Hypospadias cause male infertility in certain cases due to these complications. But remember, this doesn’t mean that all men with hypospadias will necessarily be infertile. Let’s delve into the link between hypospadias and fertility, exploring potential concerns, available solutions, and fostering hope for fatherhood.

Understanding Hypospadias:

During fetal development, normally the urethra forms by folding of tissues from scrotum to the base of the penis to the tip of the penis. The failure of this urethral closure leads to hypospadias. The severity of hypospadias varies, with the opening appearing anywhere from the scrotum (scrotal hypospadias) to the base of the penis (penoscrotal hypospadias) to near the tip of the penis (distal hypospadias). Besides the misplaced opening, most men with hypospadias also have a downward bending of the penis during erection – called chordee. Hypospadias can cause male infertility as 5% of cases may also have an associated undescended or absent testis, which can lead to lower sperm production. Such men need to be investigated for a disorder of sex development (DSD) or intersex.

Hypospadias and Fertility: the Possible Connections:

In isolated hypospadias with both normal testes, the sperm production usually remains unaffected, yet certain hypospadias-related factors can influence fertility:
 Urethral location: In severe unrepaired hypospadias cases such as scrotal or perineal or penoscrotal hypospadias, ejaculation might not be able to reach the vaginal introitus, hindering fertilization.
 Penile curvature: Significant curvature can make intercourse physically difficult. Sometimes erection can also be painful when there is significant chordee
 Meatal stenosis: abnormal hypospadias opening may be very small leading to urinary and sperm flow obstruction.
 Prostatic utricular diverticulum: Some men with severe hypospadias may also have a large sac near their prostate gland where sperm tubes (vas deferens) open. In such cases sperms may not flow out easily for fertilisation.
 Post hypospadias surgery issues: At Hypospadias foundation, we see lot of men who have had repairs done earlier and have poor fertility though they have a normal sperm production. The common reasons for this are complications of hypospadias surgery such as: residual curvature or chordee, stricture in new passage, diverticulum or baggy new urethra or fistulae in the urethra.

 Psychological considerations: Emotional concerns about body image or sexual function can sometimes affect intimacy and overall fertility.

Hypospadias Treatment Options: Paving the Way for having children
Fortunately, advancements in hypospadias surgery and infertility treatment offer effective solutions for addressing hypospadias-related fertility concerns:
 Hypospadias repair surgery: This procedure reconstructs the urethra and corrects penile curvature, often restoring normal ejaculation and improving sexual function. If there are complications from previous hypospadias surgeries such as stricture, residual chordee, fistula or a diverticulum- these can be repaired by an expert hypospadias surgeon leading to cure for infertility.
 Assisted reproductive technologies (ART): In cases where natural conception proves challenging, sperm retrieval techniques like testicular biopsy or micro epididymal sperm aspiration (MESA) can be combined with IUI, IVF, or ICSI to achieve pregnancy.

Maintaining Hope: Fatherhood is Within Reach for people with Hypospadias:
Do consult an expert and best hypospadias surgeon first. The hypospadias surgeon will check and confirm that the hypospadias repair is proper and there is no structural issue in repair. If there is some residual issue in hypospadias surgery, then that needs to be corrected first. If the infertility persists, and you suspect hypospadias cause male infertility, then you must visit an andrologist for assisted reproductive techniques.

It’s crucial to remember:
 The majority of men with hypospadias have normal fertility.
 Early surgical intervention significantly improves the chances of successful fatherhood.
 Advanced treatments like ART offer alternative paths to parenthood. Open communication with your hypospadias doctor is key to understanding your unique
situation and exploring suitable fertility options. Remember, hypospadias does not define your potential as a father. With proper support and available treatments, you can navigate this journey and realize your dreams of having babies and building a family. At Hypospadias foundation, we treat hundreds of children and adults with hypospadias every year. Since 2008, Dr A.K.Singal, rated as the best pediatric urologist and hypospadias surgeon in India and the world, has devoted his life to helping people with hypospadias get best results and normal life. Along with Dr Ashwitha Shenoy, pediatric surgeon and hypospadias surgeon and a partner at Hypospadias foundation, the team at Hypospadias foundation, has deep expertise in diagnosing and treating complex hypospadias.

References:
 American Urological
Association: https://university.auanet.org/core/pediatric/hypospadias/index.cfm
 National Institutes of Health: https://www.ncbi.nlm.nih.gov/books/NBK482122/
 Mayo Clinic: https://www.mayoclinic.org/diseases-
conditions/hypospadias/diagnosis-treatment/drc-20355153

If you wish to have a teleconsult or a second opinion from Dr Singal/ Dr Shenoy, please write to us hypospadiasfoundationindia@gmail.com or fill up this contact form: https://www.hypospadiasfoundation.com/contact/

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