HYPOSPADIAS

The term hypospadias comes from the Greek terms hypo meaning under and spadon meaning "a tear". Hypospadias is considered a common "birth defect" that occurs in males where the hole through which urine and semen leave the body (the urethral meatus) is not located at the tip of the head of the penis. Instead, the opening through which urine and semen leave the body is found on the underside of the head of the penis (the glans) or on the underside of the shaft of the penis. The opening can even be located well down the shaft, right in front of the scrotum; or between the balls (testicles) and the anus (rectum). There are several other anatomical variations that often occur in men and boys with hypospadias. These include: y An incomplete foreskin which is attached to the head or the sides of the penis but does not completely surround the head of the penis. This is often called a "dorsal hood". y A mushroom or helmet shaped head of the penis (the glans) rather than a cone-shaped one. This variation is quite common with hypospadias. y A downward tilt of the penis head and shaft known as "chordee".

CAUSES
Why does it happen? 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: y The anti-epilepsy drugs, Valproic acid and Phenytoin; y the hormone Progesterone, often given to mothers as part of IVF treatment; y DES (Diethylstilbestrol) prescribed up until the 1970s to prevent miscarriage y Clomiphene, a drug used to induce ovulation during IVF treatment; y 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 boys penis while he is in his mothers 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 babys 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 childs penis not developing properly in response to the testosterone produced by his testicles during pregnancy. How does it develop? The tube inside the penis through which urine and semen pass is formed during the later stages of pregnancy. It forms by the fusion of two parts: one becomes the shaft of the penis and another forms the head of the penis. As the two parts fold and fuse together, the process may stop too early, at any part of the tube, leaving the opening somewhere on the underside of the penis rather than at the tip of the head of the penis.

What is a chordee?
Chordee is downward bend of the head of the penis at the point where it meets the shaft, sometimes more noticeable on one side of the penis than the other. Sometimes this curve is mild and sometimes it can almost be a 90 degree downward bend. Chordee can occur in men who do not have hypospadias. However, if chordee is present, hypospadias is often also present.

EPIDEMIOLOGY
Studies done recently at the Center for Disease Control in the United States and research in other western countries suggest between 1 in 125 and 1 in 300 male babies have hypospadias, so it is quite common. In fact, this places hypospadias alongside cleft lip and palate as the leading birth defect in boys. Hypospadias occurs most often in white males, less frequently in black males and even less often in Hispanic males. It seems to be getting more common, though: for example, a 1993 study in the United States suggested that hypospadias was twice as common as it was in 1968. If a male family member has hypospadias, there is an increased risk that his male children will be born with hypospadias.

MANIFESTATIONS
The condition varies in severity. In most cases, the opening of the urethra is located near the tip of the penis on the underside. More severe forms of hypospadias occur when the opening is at the midshaft or base of the penis. Occasionally, the opening is located in or behind the scrotum. Males with this condition often have a downward curve (ventral curvature or chordee) of the penis during an erection. (Erections are common with infant boys.) Other symptoms include: y Abnormal spraying of urine y Having to sit down to urinate y Malformed foreskin that makes the penis look "hooded"

EXAMS AND TESTS

A physical examination can diagnose this condition. Imaging tests may be needed to look for other congenital defects.

THERAPEUTIC MANAGEMENT
Surgical Management Infants with hypospadias should not be circumcised. The foreskin should be preserved for use in later surgical repair. In the newborn, a meatotomy a surgical procedure in which the urethra is extended to a normal position may initially be performed to establish better urinary function. When the child is older (age 12 to 18 months), adherent chordee may be released. If the repair is extensive, all surgery may be delayed until the child is 3 to 4 years of age. To encourage penis growth and make the procedure easier, the child may have testosterone cream applied to the penis or receive daily injections of testosterone. It is important that hypospadias be correctetd before school age so the child looks and feels normal. If left uncorrected, in later years, a metal opening at an inferior penile site may interfere with fertility, because it does not allow sperm to be deposited close to the female cervix during coitus. Repair must be made before this time to prevent infertility. After surgical repair, a urethral urinary drainage catheter will be inserted to allow urine output without putting tension against the urethral sutures. The child may notice painful bladder spasms as long as the catheter is in place (3-7 days). An analgesic such as acetaminophen (Tylenol) and an antispasmodic medication such as oxybutynin (Ditropan) may be prescribed for pain relief. After hypospadias repair, children can be expected to be normal in both urinary and reproductive function unless accompanying anomalies of the penis were present (Stokoski, 2004). Nursing Management It is important to address parents concerns at the time of birth. Preoperative teaching can relieve some of their anxiety about the future appearance and functioning of the penis. Postoperative care focuses on protecting the surgical site from injury. The infant or child returns from surgery with the penis wrapped in a simple dressing, and sometimes a urethral stent (a device used to maintain patency of the urethral canal) is placed to keep the new urethral canal open. Plan care to ensure that the stent does not get removed. Refer to the hospitals policy for the appropriate use of physical restraints in this situation.

PROGNOSIS
Results after surgery are typically good. In some cases, more surgery is needed to correct fistulas or a return of the abnormal penis curve.

POSSIBLE COMPLICATIONS
If hypospadias is untreated, a boy may have difficulty with toilet training and problems with sexual intercourse in adulthood. Urethral strictures and fistulas may form throughout the boy's life, requiring surgery. The extent of complications following hypospadias surgery is obviously related to how complex the surgery was. However there are some commonly occurring problems:
y In most operations the surgeon is attempting to move the opening on the penis (the urethral meatus) from its original position on the shaft to the tip of the penis. However, the original opening sometimes reopens and may then leak urine and semen. This is referred to as a fistula, and will probably need further surgery. y In some men, a narrowing of the tube that carries urine and semen (the urethra) occurs after surgery. This narrowing or stricture (stenosis) can cause a decrease in the force of the urine stream and/or ejaculation. Mild strictures can usually be eased by using an instrument that will gradually widen the tube. y Sometimes the new opening becomes smaller than it was after surgery (this is known as a meatal stenosis). This can often be dealt with by inserting an instrument that is larger than the opening, and gradually stretching it. y In the older methods of hypospadias repair, a skin graft using skin with hair was used to repair the hypospadias, and these hairs could form blockages or infections in the urethra. In modern repairs, non-hair bearing skin is used. y A man may develop urinary tract infections if he has problems urinating after the operation.

Reference: Pillitteri, Adele (2007). Maternal and Child Health Nursing: Care of the Childbearing and Childrearing
Family, Volume 2. 5th edition. Lippincott Williams and Wilkins. http://www.hypospadiasuk.co.uk/problems-related-to-surgery