Other Congenital Penis Conditions Besides Hypospadias
“Chordee Without Hypospadias”
“Chordee” is simply another word for “curvature”. Most boys with hypospadias have only a partial foreskin on the topside of the penis. This partial foreskin can also be found in some other newborns who do not have hypospadias. This is called “chordee without hypospadias”, even though most these boys do not have penis curvature.
In some cases, the foreskin is so close to normal that no surgery is needed, unless the parents want the boy to be circumcised. For most, the partial foreskin looks neither natural nor circumcised, and this difference in appearance may result in body image concerns and fear of ridicule from peers later in life. Either circumcision or foreskin repair is done to avoid these concerns.
A typical example of “chordee without hypospadias”. There is a partial dorsal foreskin with a normal urine opening.
Much less commonly, a boy with this condition has true ventral (forward) curvature due to a short urinary channel or bending of the erection cylinders. Although they have a urine opening at a normal location in the head of the penis, these patients are treated similarly to boys with proximal hypospadias and may need a 2-stage operation to make the penis straight, the first surgery to cut the shortened urinary channel and lengthen the shorter side of the penis, and the second to create a urinary channel that is the proper length.
A boy with “chordee without hypospadias” whose curvature persisted after the skin was released. A 2-stage hypospadias-type repair was needed to make the penis straight and then restore the urine opening to the normal location.
Lateral Penile Curvature
The penis can also be bent to one side, most often to the left. This can occur without other penis abnormalities, or with distal hypospadias. Curvature greater than 30 degrees to the side can cause discomfort to the man or his sexual partner during intercourse.
Many surgeons try to correct this bending using so called “plications”, stitches into the opposite side to shorten it to match the bent side. While this method will straighten the penis during the operation, afterwards the curvature can recur with a high rate if the curvature is more than 30 degrees. An alternative with less risk for recurrence is to lengthen the side that is bent.
Lateral penile curvature
An otherwise normal penis can be twisted to one side, usually towards the left in a counter-clockwise direction. This “torsion” can also occur in boys with distal hypospadias. Unless the penis is un-twisted surgically, the boy will pee to the side. It is thought to be due to abnormal skin attachments which are released during surgery. While patients with mild torsion (45 degrees) may be able to have this corrected while preserving the foreskin, some surgeons may recommend circumcision in boys with severe torsion (90 degrees) and hypospadias because of a high rate of foreskin complications in this rare circumstance.
Congenital Concealed Penis
This condition is caused by abnormal development of the foreskin, resulting in relatively less visible skin on the shaft, and more than usual inner foreskin. This foreskin does not retract. The appearance is that of a tent, or pyramid, from the skin ballooning when the boy pees. The urine exits the urine opening in the penis and collects inside the abnormal foreskin, slowly dribbling out into the diaper. Surgery is needed to remove the excess inside foreskin and cover the outside of the penis to look normal. Circumcision is done, as successful reconstruction of the abnormal foreskin into a functioning normal foreskin has not been reported.
Typical appearance of congenital concealed penis. Touching the ballooned area squirts urine out of the foreskin
The foreskin has been pulled back, showing there is an abnormally large amount of inside foreskin compared to relatively deficient outside foreskin and penis shaft skin
Epispadias refers to a birth defect with the urine opening in an abnormal location on the top of the penis, most often with upward curvature of the penis. It is rare, occurring in about 1 of every 120,000 newborn boys. In contrast, hypospadias is found in 1 of every 200 newborn boys. Based on an estimated 2 million boys born in the US each year, only 16 will have epispadias.
The urine opening most often is at the base of the penis (penopubic epispadias), but can also be on the shaft or the head of the penis.
If the upward curvature is not successfully straightened, teens and adults can have erections with the head of the penis touching the abdomen.
The penis is shorter, and often wider, that normal. Surgery does not correct this short penis length, since it is due to shorter than normal erection cylinders. Many teens and adults with this condition are disturbed by their short penis, despite otherwise successful reconstruction to make the penis straight and move the urine opening to the correct location. There is no surgery to significantly lengthen the penis.
Glanular epispadias, with the opening on the head of the penis
Upward curvature of the penis with glanular epispadias
Penopubic epispadias, with the urine opening at the base of the penis. Upward curvature is not seen in this picture because the penis is being pulled down to show the urine opening
In addition, the pubic bone is separated open (“diastasis” of the pubic bone), and the majority of these boys will have urine incontinence that becomes apparent after toilet training.