Hypospadias

Hypospadias is a condition where the meatus isn't at the tip of the penis. Instead, the hole may be any place along the underside of the penis.

The meatus (hole) is most often found near the end of the penis ("distal" position). But it may also be found from the middle of the penile shaft to the base of the penis, or even within the scrotum ("proximal" positions).

Over 80% of boys with this health issue have distal hypospadias. In 15% of those cases, the penis also curves downward slightly, a condition called "chordee." When the meatus opens further down the shaft, curvature occurs in more than 50% of patients.

Hypospadias is a common birth defect found in up to 1 in every 200 boys.

In most cases, hypospadias is the only developmental problem in these infants and doesn't imply there are other flaws in the urinary system or other organs.

The classification of hypospadias depends on the position of the urethral meatus:

• Glanular
• Coronal
• Penile (distal-middle-proximal)
• Scrotal
• Perineal

Signs and symptoms of hypospadias may include:

• Opening of the urethra at a location other than the tip of the penis
• Downward curve of the penis (chordee)
• Hooded appearance of the penis because only the top half of the penis is covered by foreskin
• Abnormal spraying during urination

Problems are likely to occur if hypospadias is left untreated. The further back the opening of the urethra is, the more severe the problems are likely to be.

• Passing urine is different to normal. A baby in nappies will have no problem. However, when older, the urine stream may not be able to be directed forward into a urinal. When going to the toilet the urine is likely to 'spray' backwards. Sitting on a toilet may be needed to pass urine without mess.

• Chordee which causes bending of the penis. This is more noticeable when the penis is erect. Sexual intercourse may be difficult or impossible in severe cases.

• Psychological problems about being 'different' to normal are common.

• Mild erection difficulties and premature ejaculation have been reported in adulthood in patients who have had surgery for hypospadias in childhood.

The exact cause of hypospadias is unknown.

There are thought to be several reasons why hypospadias occurs. In some cases it is passed down from a male relative (often the father) to the son, so it is classified as hereditary. In other families, an uncle or a grandfather may have hypospadias. However, this family occurrence is seldom known or openly discussed so the genetic inheritance cannot be easily established.

A second, but not very common, cause appears to be the side-effects of drugs (that includes illegal drugs, and medicines or pills) which the mother has taken while she is pregnant or trying to become pregnant. Drugs that have been linked to a higher occurrence of hypospadias include:

• The anti-epilepsy drugs, Valproic acid and Phenytoin;
• the hormone Progesterone, often given to mothers as part of IVF treatment;
• DES (Diethylstilbestrol) prescribed up until the 1970s to prevent miscarriage;
• Clomiphene, a drug used to induce ovulation during IVF treatment;
• Loratidine, an anti-allergy drug, though the evidence now seems to suggest that Loratidine is not a risk factor in hypospadias.

Also, the frequency of hypospadias is higher in boys born to mothers addicted to cocaine.

There may of course be other drugs which can affect the development of a boy’s penis while he is in his mother’s uterus, but which have not yet been shown to cause hypospadias.

A third cause of hypospadias may be the increase in levels of man-made chemical pollutants in the world around us. This increase may also explain why hypospadias is becoming more common in our society. Some chemicals like pesticides, fungicides and industrial pollutants are suspected of causing an increased level of hypospadias as well as other birth defects.

A fourth cause of hypospadias is hormonal. If either mother or children are exposed to high levels of female hormones, or if there is something wrong with the baby’s own hormone system, the baby may develop hypospadias. Doctors have known for a long time that a small proportion of cases of hypospadias are caused by a male child’s penis not developing properly in response to the testosterone produced by his testicles during pregnancy.

In addition to a careful physical examination, ultrasound imaging of the urinary organs is necessary to diagnose hypospadias.

If anomalies are identified, an intravenous urography or micturition cystourethrogram may be necessary.

Investigations in Scrotal and Perineal Hypospadias

The risk of disorders of sex development is increased in proximal hypospadias, the following evaluations are necessary:

• Family history
• Karyotyping
• Hormone analysis
• Fibroblast culture with evaluation of androgen receptors and 5α-reductase enzyme activity.
• Pelvic ultrasound imaging, MRI and/or retrograde genitography
• Micturition cystourethrogram
• Cystoscopy

If the hypospadias is mild, with the opening of the urethra just a little down from normal and with no bending of the penis, no treatment may be needed. However, in most cases an operation is required to correct the hypospadias. This can usually be done in one operation. However, if the hypospadias is more complicated, two operations may be necessary. The operation is usually done when the child is around 4-18 months old.

Orthoplasty

Assessment and management of penile curvature is done after artificial erection of the penis. In the majority of cases, the curvature of the penis can be corrected using the technique of Nesbit. In severe cases, grafting of the tunica albuginea helps in straightening the penis. Resection of the chordee is only rarely performed. For tubularized incised plate (TIP) urethroplasty, conservation of the urethral plate is mandatory.

Urethroplasty

Urethroplasty is reconstruction of the missing distal urethra. The below described surgical techniques differ primarily in the technique of urethroplasty: application of flaps, incision of the urethral plate or free oral mucosa transplants.

Neourethral coverage

A second layer of tissue covers the neourethra and prevents the formation of fistulas. Most often, a pedicled subcutaneous (dartos) flap is raised from preputial, penile or scrotal skin.
Meatoplasty and glanuloplasty:

• Reconstruction of the meatus and the glans to achieve meatus at the tip of the penis with a vertical slit.

Skin closure

Skin coverage of the penile shaft is achieved with various techniques (e.g. transfer of penile skin).

MAGPI Hypospadia Operation

MAGPI is Meatal advancement and glanuloplasty. The MAGPI-technique is only suitable for distal hypospadias.

Tubularized incised plate (TIP) Urethroplasty

TIP urethroplasty is suitable for distal and proximal penile hypospadias. The TIP urethroplasty is considered technically simple and has a low complication rate, the cosmetic result of the glans and the meatus is good. Furthermore, the TIP urethroplasty is a useful option for re-operations with preserved urethral plate. The urethral plate is not removed but deeply and longitudinally incised. After mobilization and tubularization, the urethral plate is closed around a catheter. A ventral curvature is corrected using the Nesbit technique.

Mathieu Hypospadia Repair

The Mathieu hypospadia repair is a good option for distal penile hypospadias. A rectangle of skin over the proximal urethra is raised and folded distally. To avoid a horizontal meatus, a modification of the original technique with V-incision of the flap exists (MAVIS = Mathieu and V incision sutured). The most common complications are unfavorable meatal cosmetics, skin flap necrosis with fistula or stricture of the urethral meatus.

Island Flaps Hypospadia Repair

Island flap hypospadia repair is suitable for distal and middle penile hypospadias. The island flap is raised from the prepuce: the pedicled flap consists of the inner leaf of the prepuce with Tunica dartos. The flap is rotated around the penis and used in onlay technique with a preserved urethral plate. If a resection of a chordee has been necessary, a tubular island flap is necessary.

Two-stage Hypospadia Repair

Indications for a two-stage hypospadia repair are severe proximal hypospadias and situations after failed hypospadia surgery.

Hypospadia Repair with a Free Oral Mucosa Graft

Indications for the use of a free oral mucosa graft are situations after failed hypospadias surgery.

Lifestyle modifications are necessary in order to cope with hypospadias.

Various types of support maybe very useful for boys and men with hypospadias and their families. Connecting with others who are living with hypospadias or with other affected parents through the internet can be highly effective, providing a source of information and knowledge which can help overcome difficulties and challenges related to the condition.

Local, regional or national support groups can be another way to lessen the negative effects of hypospadias. Meeting other men with hypospadias in person is one of the most powerful experiences for people affected by hypospadias, helping to break down the loneliness that some men have experienced in keeping their hypospadias a secret all their lives.

Another real source of help is support or discussion groups where you can share experiences and express your feelings and emotions about hypospadias. You can also get emotional support and discuss different ways in which you can cope with hypospadias, and find practical information on how to live positively with hypospadias.

What Do We Know About The Long Term Effects?

Fatherhood

Men with hypospadias have just as much chance of becoming a father as any other man, so long as the opening of their penis is not located at the base of the penis, when it may be difficult to ejaculate semen directly into their partner’s vagina.

There is a slightly greater chance, compared with other men, that men with hypospadias will pass the condition on to their sons.

Urinary infections

These do appear to be more common with men with hypospadias. These infections may be related to a narrowing of the urine tube (a stricture) so that the urine does not completely empty from the bladder, or they may be the result of surgery where a skin graft bearing hair was used in the operation. Surgery disrupts the body’s normal protective arrangements and that can lead to higher susceptibility to an infection.

Sexual functioning

Some men with hypospadias complain that they dribble semen instead of spurting it out when they have an orgasm.

It’s possible this has nothing to do with hypospadias, since the ability to ejaculate forcefully is mostly related to the power of the muscles around the sexual organs rather than the location or size of the urethral opening.

Chordee

Some men with prominent chordee may experience discomfort during sexual intercourse in certain positions. However, other men say that chordee actually enhances their sexual pleasure – and that of their partner also.

Sexual orientation

Some men have expressed a concern that they are gay or bisexual because they have hypospadias. This may lead to actual physical contact with other men, sexual or otherwise, but this desire to look at or experience “normal” penises does not necessarily mean a man is either gay or bisexual.

The complication rate of of hypospadias correction is considerable, from 30 to 50%.

Early complications

Infection (1-2%), bleeding, bruising, wound dehiscence, flap or graft necrosis, urinary tract infections, urinary tract obstruction.

Late complications

Urethral fistula (5 – 10%), meatal stenosis (7 – 15%), recurrent urethral stricture or residual chordee (up to 20%), balanitis xerotica obliterans (BXO), urethral diverticula (4 – 7%).

The complications rate is essentially dependent on the severity of hypospadias, the patient's age at the time of surgical correction, the reconstruction technique used, the quality of the surrounding tissue for the reconstruction, the medical equipment and the surgeon's skills and experience and the number of previous interventions.