Urology
Hypospadias
What Every Parent Should Know
What is hypospadias?Hypospadias is one of the more common birth abnormalities of the genitalia among male infants. Most families are unaware of its existence since the condition is rarely talked about. The term "hypospadias" comes from Greek (hypo, under and spadias, rent) and refers only to the location of the opening through which the child urinates.
In a normal penis, the urine tube (urethra) travels through the shaft of the penis to an opening (meatus) located in the center of the head of the penis (glans). The foreskin is the loose skin that surrounds the head of the penis and is the part that is removed during circumcision. In boys with hypospadias, the urine tube is short and does not come out to the end of the penis. The opening could be located anywhere along the underside of the shaft of the penis or even in the scrotum. In addition, the foreskin is incompletely formed and there may be a bend at the penis called chordee. Fortunately, almost 90 percent of hypospadias cases are minor with the urethral opening on or just below the head of the penis. The remaining are more severe, producing some degree of functional difficulty with both voiding and sexual activity. When does hypospadias develop?Hypospadias develops during pregnancy. The genitalia of babies are formed by the third month of pregnancy. Prior to that time, both male and female fetuses have genitalia that are indistinguishable from each other. However, starting at about the tenth week of pregnancy, the urethra begins to close and the genital tubercle (which will form the penis) begins to grow. It is at this point that one can, by outward examination, distinguish a male from a female fetus.
What causes hypospadias?The exact causes of hypospadias are unknown, but it would seem to be the result of some breakdown of the normal developmental process, either by virtue of decreased hormonal stimulation or a genetically programmed arrest of genital development. There is no question that hypospadias can be familial although the exact mechanism by which it is inherited is as yet unknown. Eight percent of the fathers of boys with hypospadias have hypospadias themselves. If two people in a family have hypospadias, the chance of recurrence in another pregnancy is just over 20 percent.
How is a diagnosis made?The diagnosis of hypospadias is usually obvious on inspection. At times, hypospadias can be diagnosed from a prenatal ultrasound examination. If it is not identified before birth, it is usually identified during the baby's initial physical examination. The first clue to the problem is often the abnormal foreskin. However, there are some cases of hypospadias in which the foreskin is completely normal and the diagnosis is not made until the foreskin is retracted or until a circumcision has been performed.
Are there any tests needed?Most children with hypospadias have no other abnormalities although inguinal hernias and undescended testes are seen more commonly in children with hypospadias than in the general population. It was once thought that there was an increased incidence of abnormalities of the upper urinary tract (kidneys and ureters) associated with hypospadias, but a number of studies have shown that the incidence of upper urinary tract abnormalities is no greater in boys with hypospadias than in the general population. For this reason, there is no need for upper tract imaging studies (e.g., excretory urograms or ultrasound examination). Rarely, there can be abnormalities of the chromosomes, especially when a combination of hypospadias and undescended testes is present. In these cases, a karyotype (chromosome analysis) is often requested.
When is surgery for hypospadias indicated?While not every child with hypospadias needs surgery, there are certain functions which may not be possible if the problem is not corrected. The opening (meatus) should be far enough out on the shaft for a man to stand to void and to ensure insemination (fertilization). The penis must be straight enough with erection to assure penetration during intercourse and the penis must be cosmetically acceptable to the child. If any of these are not adequately addressed then long term psychological and sexual dissatisfaction will almost certainly follow.
When should surgery be performed?Current practice for the treatment of hypospadias suggests that surgery be performed between 6 and 12 months old. If surgery is performed at an earlier age, there is increased risk to general anesthesia. Since there is no medical need to repair hypospadias prior to six months of age, it seems prudent to wait until this risk is decreased by maturity. After six months, however, there is no advantage to waiting longer. After 18 months of age, children sometimes exhibit temporary emotional upset after repair. during the last six months of the first year of life.
What does the surgery involve?It is important, especially in severe cases of hypospadias, that the child not be circumcised prior to the procedure because the tissue from the foreskin is essential for the repair. The absence of the foreskin can complicate the difficulties faced by the surgeon. Hypospadias surgery is tedious and technically very demanding, and there is no single method of repair that suffices for all patients. The results of hypospadias repairs by and large are good, especially when performed by an experienced surgeon.
There are a number of goals that the surgeon seeks to achieve during the course of the repair. First, the surgeon will correct the chordee (i.e., fix the penis so it will be straight at the time of erection). In many instances, the cause of the chordee is inelastic tissue below the skin on the undersurface of the penis. Removal or incision of this tissue allows the penis to straighten. In other cases, chordee is produced by a disproportion in the length of the corporal bodies (the structures that fill with blood to cause an erection) such that the top surface is longer than the bottom surface, thereby producing a bend. Should this be the problem, it can be addressed by either taking a tuck in the dorsal (top) surface or by lengthening the ventral (bottom) surface, depending on the overall length of the penis. It will rarely be necessary to transect the urethra in order to correct the chordee.
Once the penis is straight, the next goal is to create a urethra that will extend to the tip of the penis. This can be achieved by creating tubes from local skin, forming a tube from flaps from the foreskin, or using free grafts from foreskin, bladder lining, or the lining of the cheek.
After a urethra has been created, the glans penis is remolded around it (the urethra) and skin is brought around the dorsum (top) of the penis to the ventrum to provide coverage of the newly created urethra.
A catheter is often left in the penis for urinary drainage for some period of time after surgery. There are a myriad of dressing techniques that are utilized to cover the penis temporarily while the wounds are healing.
What are the results of surgery?The modern results of hypospadias surgery are good. Overall, greater than 90 percent of patients with hypospadias will have the problem corrected in a single operation. The most common complication of modern hypospadias repair is a fistula (leak), which occurs in 5 to 10 percent of cases and can be easily repaired if it occurs.
These operations are often performed on an outpatient basis and rarely involve an overnight stay in the hospital. This certainly is a far cry from 20 to 25 years ago when hospital stays for hypospadias surgery averaged 10 days, and many patients were subjected to multiple operations.
With good communication between pediatric urologist, parents and pediatricians, most patients with hypospadias can expect a good outcome from repair with minimum of discomfort and a maximum of functional and cosmetic gain.
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