The term hypospadias comes from the Greek language. Hypospadias is the most common congenital anomaly of the penis. It usually develops sporadically and has no obvious cause. The external urethral opening is abnormally located, in mild cases on the ventral side of the penis near the normal urethral opening, in severe cases opening in the scrotum or perineum. The ventral foreskin of the penis is lacking while the dorsal side is ample, like a turban. Penile hypospadias is common in severe cases. Cryptorchidism and inguinal hernia are common complicating malformations. As the degree of hypospadias increases, the rate of complicating deformities increases. For simple anterior or mid-segment hypospadias, ancillary testing is usually not required. Patients with posterior hypospadias or complicating malformations of other organ systems should be examined thoroughly. In boys with otherwise healthy hypospadias, the best time to operate is at 6 to 12 months of age. Patients who have surgery even with imperfect results usually have a satisfactory sex life.
Morbidity
The incidence of hypospadias is estimated to be 0.4 to 8.2 per 1000 live male infants. Differences in values may be related to geography, environment, genetics and different statistical methods. The prevalence is high in Caucasians and low in Spaniards. An increase in the incidence of hypospadias has been reported in recent years, but there are no similar reports in less developed countries.
Etiology
The causes are multiple. Most cases have a scattered onset with no clear cause. In general, the more severe the hypospadias, the easier it is to identify the underlying cause. It has been suggested that the cause can be found in 32 to 36% of severe cases of hypospadias by clinical and molecular biological examination.
Insufficient production of testosterone by the testes and adrenal glands, impaired conversion of testosterone to dihydrotestosterone, lack of androgen receptors in the penis, or decreased binding of dihydrotestosterone to androgen receptors can all contribute to hypospadias. Studies have found abnormal testosterone response to human chorionic gonadotropin in some men with hypospadias. Hormones, medications, and diet during pregnancy may be the cause, and genetic factors may also play a role.
Clinical presentation
The external urethral opening is located on the ventral side of the penis, either close to the normal opening or in the scrotum or perineum. In severe cases, the scrotum splits into two halves. There is usually no micropenis, except in severe cases with penile hypospadias.
The ventral foreskin of the penis is lacking while the dorsal side is ample, like a turban.
Hypospadias is a curvature of the penis to the ventral side. The curvature is more pronounced during erection and is more pronounced in cases where the urethral opening is proximal to the penis.
Those with a posterior urethral opening are unable to urinate standing up, while those with an opening near the glans can have normal urination. Penile hypospadias affects sexual life.
Related abnormalities
Cryptorchidism and inguinal hernia are common complicating anomalies. As the degree of hypospadias increases, the rate of complicating deformities increases. The possibility of hermaphroditism should be considered in patients with hypospadias complicated by cryptorchidism. The incidence of urological malformations such as obstruction of the ureteral bladder connection, vesicoureteral reflux, pelvic ectopic kidney, horseshoe kidney, crossed ectopic kidney, and renal dysplasia is 1% in mild cases of hypospadias and 5% in severe cases. There is a direct correlation between the severity of hypospadias and the incidence of complicating malformations.
Examination
Mild hypospadias does not require ancillary testing. Severe hypospadias should be examined by urological ultrasound. Chromosomal analysis should be performed in cases of combined cryptorchidism or unknown sex. Based on the medical history and physical examination, sex hormones can be checked, sex hormone-related genes can be analyzed, and pituitary gland can be examined.
Treatment
The goals of treatment are to straighten the penis to enable sexual intercourse, to position the external urethral opening in front of the glans to enable standing to send urine, to have a new urethra of appropriate and uniform diameter, to have a satisfactory penile appearance, and to minimize complications. For very mild hypospadias surgery should also be performed to improve the psychological impact. For otherwise healthy boys with hypospadias, the best time to operate is between 6 and 12 months of age, and after 6 months, the risk of anesthesia is no greater than when the child is older. Studies have found that early surgery has little effect on the gender aspect.
Penis size is not a limiting factor for early surgery. If the penis is short or undercircumcised, it can be improved with exogenous hormones prior to surgery.
There are many surgical options for hypospadias, none of which is suitable for all cases. Most cases can be repaired with a single surgery. For severe cases, two separate surgeries can be performed. In severe cases and in cases of hypospadias, it is necessary to completely remove the fibrous cords to the hypospadias lesion. Regardless of the severity of the lesion or the complexity of the surgery, a suprapubic urinary diversion is not necessary for hypospadias surgery.
Early complications of hypospadias surgery include bleeding, hematoma, wound infection, wound dehiscence, flap necrosis, urinary tract infection, and urinary retention. Late complications are urethral fistula, urethral stricture, penile curvature, urethral stricture, urethral diverticulum.
Prognosis
Children with hypospadias have normal pubertal development. Most patients have normal testes and secondary sexual characteristics. Sexual life should be normal after successful treatment. Fertility should be normal if there are no other pathologies. Even patients with less than perfect results usually have a satisfactory sexual life.