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Ailmemts & Remedies Pediatric

Hypospadias

Definition:
Hypospadias is a birth defect found in boys in which the penile meatus is not at the tip of the penis. The meatus is the term for the opening of the penis through which urine normally exits the bladder. The incidence is reported to be 1 in 300 live male births. There is some family risk of hypospadias, as familial tendencies have been noted. Up to 14% of male siblings are affected.

Hypospadias is usually classified according to the location of the opening. As the defect increases in severity, the opening to the penis will be found further back on the penis. The most severe types can have openings at the region of the scrotum and even in the perineum (the region between the anus and scrotum).

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In some men with hypospadias, there’s another abnormality called chordee, in which the penis curves downwards and the foreskin only covers the front of it.In the most severe forms of hypospadias, the urethral opening is so far back it’s almost in the scrotum. The scrotum itself may be small and the testes may not have descended (that is, they’re still deep in the abdomen). When babies are born like this, it can be difficult to work out which sex they are without further tests.

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Both hypospadias and chordee must be repaired so that a child can have normal urinary and reproductive health.

Symptoms:
Hypospadias is a structural abnormality that doesn’t progress or put the man at risk of any other serious illness. However, as with any abnormalities of the urinary system, there may be an increased risk of urinary infection in more severe cases.

Hypospadias may cause emotional turmoil when a boy realises he’s different from his friends. It can also cause practical problems with passing urine (those with the condition usually have to sit down to pee) and later with sexual intercourse, which may be embarrassing or difficult to cope with. Hypospadias may cause general worries about sexuality and fertility.

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

Causes:
Hypospadias is present at birth (congenital). The exact reason this defect occurs is unknown. Sometimes hypospadias is inherited.

As the penis develops in a male fetus, certain hormones stimulate the formation of the urethra and foreskin. Hypospadias results when a malfunction occurs in the action of these hormones, causing the urethra to develop abnormally.

As a boy is developing in utero, the penis begins to form in the sixth week of fetal life. Two folds of tissue join each other in the middle and a hollow tube is formed in the middle of the future penis. This tube is the urethra and its opening is called the penile meatus. As the skin folds develop to form the penis, any interruption in this process leads to the meatus being located in a location further from the end of the penis. The exact etiology for this premature cessation of urethral formation is poorly understood. In addition, the etiology of the often-associated abnormal downward curvature (chordee) is also poorly understood.

Risk Factors:
This condition is more common in infants with a family history of hypospadias.

Some research suggests that there may be an increased risk of hypospadias in infant males born to women of an advanced age or those who used in vitro fertilization (IVF) to conceive. The connection to IVF may be due to the mother’s exposure to progesterone, a natural hormone, or to progestin, a synthetic form of progesterone, administered during the IVF process. Other research, however, hasn’t confirmed a link between IVF and hypospadias, but did find an association between a mother’s exposure to pesticides and hypospadias.

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

Treatment
The treatment of hypospadias is always surgical. Initially when the child is born and hypospadias is identified, it is important to delay any thoughts of circumcision until seen by a urologist. This is because the foreskin can provide essential additional skin needed to reconstruct the urethra.

Hypospadias is often repaired  before a child is one year of age. This way, the boy is in diapers and management of dressings are made easier. However, the exact age of repair can vary according to the size of the penis and severity of the defect. It can be repaired in most of the  cases with a single operation, but on occasion, a second operation may be needed. The operation is performed under general anesthesia with the child completely asleep. Most of the boys will have a small tube exiting the tip of their new meatus. This “stent” will protect the new urethra and allow for adequate healing. Most patients leave the hospital the same day or the following day. However, more complex repairs for the more severe types of hypospadias can require longer hospital stays due to the need for bedrest and immobilization in the immediate post-operative setting.


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The exact type of operation employed varies according to the severity of the defect. For the more distal defects that have openings closer to the normal position at the end of the penis, a new tube can be created from the surrounding skin. This creation of a tube is known as a Thiersch-Duplay repair. For more severe defects, the options range. Additional hairless skin is often needed to recreate the urethral tube when longer defects are seen. Here, the subdermal skin of the foreskin can be used. For the most severe defects, we can remove mucosal skin from the inside of the cheek or use subdermal skin from other hairless parts of the body. It is important to use hairless skin as future hair growth in the neourethra can present multiple problems.

Complications:
The usual risks of surgery are present at the time of performing  hypospadias repairs. Risk of infection is controlled with use of antibiotics with the surgery and in the post-operative setting. Bleeding is well controlled by using a penile tourniquet during the operation. This limits the blood loss to a very minimal amount, while allowing for good visualization of the tissues for the surgeon.

By using good surgical techniques   the longer-term complications of the surgery are minimised. The most common problems that present are fistula and stricture. A fistula occurs if a hole develops along the pathway of the repair proximal to the tip of the penis. In other words, a hole can develop along the underside of the penis allowing for leakage of urine. Additionally, a stricture is a scar that can form causing a narrowing in the urethra. If either of these complications occur, an additional repair will be needed usually 6 months later

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.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/hypospadias.shtml
http://www.nlm.nih.gov/medlineplus/ency/article/001286.htm
http://www.mayoclinic.com/health/hypospadias/DS00884
http://www.cornellurology.com/pediatrics/hypospadias.shtml

http://www.medindia.net/patients/paediatrics/Hypospadias.htm

http://www.surgeryencyclopedia.com/Fi-La/Hypospadias-Repair.html

http://www.adhb.govt.nz/newborn/Guidelines/Anomalies/Hypospadias.htm

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