This is a term male infant who is noted to have a ventral penile chordee with mid-penile shaft hypospadias. His testes are descended bilaterally. No circumcision is performed. He voids normally, and at 6 months of age undergoes repair of the hypospadias and chordee using the foreskin as a vascularized graft. Postoperatively he develops a urethrocutaneous fistula along the suture line. This is repaired 6 months later, and he subsequently has no problems.
Hypospadias occurs in 1 of 300 males in the United States, and is the most common congenital anomaly of the penis (1). "Hypospadias" refers to an abnormal penile configuration in which the urethral meatus is located on the ventral surface of the penis, proximal to the end of the glans, and anywhere from the ventral gland to the perineum. Epispadias refers to the condition in which the meatus is located on the dorsal surface of the penis.
Penile chordee (ventral bending of the penile shaft) is often associated with hypospadias, and may be due to tethering or dysplasia of the ventral penile shaft skin (2). A dorsal hood of incomplete prepuce may also be present.
There is no single known cause of hypospadias. Genetic factors exist, most likely based on a multifactorial mode of inheritance (3). Hypospadias is more common in first degree male relatives. Fathers of affected boys have an 8 percent incidence of hypospadias; and male siblings, 14 percent. Undescended testes and inguinal hernia occur in about 9 percent of children with hypospadias (1,3). Other anomalies do not occur with any significance in isolated hypospadias. This is related to the fact that both are under androgenic hormonal control during development. There is a significantly increased incidence of intersexuality when both conditions coexist (4), and a karyotype should be considered (5).
Since urethral development occurs under the influence of dihydrotestosterone (which is converted in peripheral tissue from testosterone by 5-alpha-reductase), the development of hypospadias can be related either to a reduction in 5-alpha-reductase activity, to a lack of testosterone production, or to failure of the local receptors to recognize the hormone (2).
Hypospadias should be classified based on the anatomical location of the urethral meatus after the chordee has been released: glanular (meatus is located on the glans), coronal, distal shaft, midshaft, penoscrotal, scrotal, or perineal. Associated chordee should be described in terms of severity (mild, moderate, or severe). This provides the most practical classification of hypospadias.
Anterior hypospadias (glanular and coronal types) account for 50% of all hypospadias. Middle hypospadias (distal, midshaft, and proximal penile types) account for 30% of hypospadias cases. Posterior hypospadias (penoscrotal, scrotal, and perineal types) account for 20% of cases (1).
An older classification system, not used by urologists anymore, but which you may encounter, describes hypospadias by degrees. First degree with the meatus between the glans and the distal shaft; second degree with the meatus between the midshaft and the proximal shaft; and the third degree with the meatus being penoscrotal, scrotal or perineal. The severity of chordee is not considered in this system (2).
The pediatrician will be the first physician to exam the genitalia after birth. The foreskin should be examined for completeness circumferentially. Thinned ventral foreskin (a "hooded" penis) is associated commonly with hypospadias. The meatal position should be noted if abnormal (glanular, penile, penoscrotal, scrotal, or perineal), as well as the presence or absence of penile chordee (mild, moderate or severe). The stretched penile length in the newborn is 3.5 cm normally (range 2.8 cm to 4.2 cm) (3), and should be noted if abnormal.
The gonads should be palpated and any cryptorchidism (undescended testes) noted. Any scrotal abnormalities should also be noted, such as a bifid scrotum (a deep cleft between the scrotal sacs) or penoscrotal transposition (the penis lying in or beneath the scrotum). There is an increased incidence of an intersex state (the expression of male and female physical and sexual characteristics within the same individual) in unilateral and bilateral cases of cryptorchidism with hypospadias, especially if the hypospadias is severe (4). Any inguinal hernia should be noted.
If hypospadias is present, a family history of hypospadias should be noted. Any history of maternal ingestion of hormonal medication during pregnancy should be noted. Other congenital anomalies should also be noted (e.g., anorectal anomalies), if present.
Upper urinary tract abnormalities have been reported to be more frequent in boys with hypospadias (3,5). However, routine screening with ultrasound, IVP, or cystograms is not justified because the incidence of defective upper tract anomalies is low. If other associated anomalies are present, with a known higher incidence of upper urinary tract abnormalities (e.g., anorectal malformation), then imaging screening studies are justified (5).
No circumcision should be done in the newborn with hypospadias or any other penile anomaly, as the foreskin may be necessary to create a neourethra, and/or provide penile shaft skin coverage.
If the gonads are nonpalpable and the hypospadias is proximal (penoscrotal or scrotal), then the risk of having an intersex state is high, and emergent urologic consultation is indicated, as well as observation for salt wasting congenital adrenal hyperplasia conditions (the most common cause of intersex states). For hypospadias, urological consultation or referral should be obtained during or shortly after the neonatal period.
The goals of corrective surgery for hypospadias are to provide the child with a normally appearing circumcised penis with the urethral meatus well placed at the tip of the glans. The child should be able to stand to void and have a straight penis when erect (2). This will allow both normal voiding as well as reproductive functionality of the penis after repair.
The hypospadias repair is best performed when the patient is between 6 and 18 months of age. At this age, babies are amnestic of the procedure, post operative management while the patients are still in diapers is easier and allows the procedure to be performed as outpatient surgery (1). The child's anesthetic risk is lower after 6 months of age if a good pediatric anesthesiologist is used.
There are over 200 named surgical procedures to correct hypospadias (1), but there are general concepts in the approach to hypospadias repair common to all. Ventral penile chordee must be corrected first, as the urethral meatus may move proximally as the penis is straightened. Next, the urethroplasty (urethral advancement) is performed to allow the placement of the neourethra well into the glans (to the glans tip). The neourethra is formed from either local skin flaps, or from foreskin flaps (the reason circumcision is not performed). A glanuloplasty to create a normal appearing rounded glans penis may also be performed, if necessary. Penile shaft skin coverage is then accomplished by bringing penile shaft skin, or foreskin flaps ventrally. A short, small caliber silastic urethral catheter that drains directly into the diaper may be used to direct the urine away from the repair, which is removed 7 to 14 days later.
Most hypospadias repairs can be done with a single stage repair. Sometimes a 2-stage repair is necessary, especially for very long urethral defects. The chordee is corrected first, and the prepuce spread along the ventral shaft. Six months later, the neourethra is completed in a second stage repair.
The most common complication seen after hypospadias surgery are fistulae, strictures and recurrent chordee, occurring approximately 10 percent of the time (1,2,5). A wait of at least 6 months is necessary to allow complete healing of the tissue, before the secondary surgery is performed.
Although parents are usually quite distraught when their child is born with hypospadias, the technique for hypospadias repair used by pediatric urologists today are very successful in transforming the hypospadiac penis to a normally appearing and functioning penis, and can be done while the child is still in infancy.
1. What is the incidence of hypospadias in newborn males in the United States?
2. Why is the presence of non-palpable gonads and hypospadias worrisome?
3. What are the anomalies most commonly associated with hypospadias?
4. What are the goals of hypospadias repair?
5. What are some common complications of hypospadias repairs?
6. Describe the possible locations for the hypospadiac urethral meatus.
7. How should chordee be described?
1. Duckett JW Jr. Successful Hypospadias Repair. Contemp Urol 1992;4(4):42-55.
2. Roth DP. Hypospadias. In: Gonzales ET, Bauer SB (eds). Pediatric Urology Practice. 1999, Baltimore: Lippincott, Williams & Wilkins, pp. 487-497.
3. Belman AB. Hypospadias and other urethral abnormalities. In: Kelalis P, King L, Belman AB (eds). Clinical Pediatric Urology. 1992, Philadelphia: WB Saunders, pp. 619-663.
4. Kaefer M, Diamond D, Hendren H, et al. The Incidence of Intersexuality in Children with Cryptorchidism and Hypospadias: Stratification Based on Gonadal Palpability and Meatal Position. J Urology 1999:162(3-II):1003-1006.
5. Stock JA, Scherz, HC, Kaplan GW. Distal Hypospadias. Urol Clin North Am 1995;22(1):131-137.
Answers to questions
1. 1 in 300 newborn males.
2. Nonpalpable gonads and hypospadias (especially severe proximal hypospadias) is associated with an increased risk of the presence of an intersex state (about 27%) (4).
3. Cryptorchidism and inguinal hernias.
4. A normal appearing circumcised penis with the meatus at the glans tip. The erect penis should be straight.
5. Urethral fistula, urethral stricture and recurrent penile chordee.
6. Glanular, coronal, distal penile shaft, midshaft, penoscrotal, scrotal, perineal.
7. Mild, moderate, severe.