Hypospadias

Introduction The urethral ectopic opening in the ventral side of the urethra is called hypospadias. The hypospadias opening can occur anywhere from the perineum to the penis head. The distal end of the external urethra, the urethra and surrounding tissues are underdeveloped, and the formation of a fiber cord involves the penis, causing the penis to bend to the ventral side. Congenital penile curvature is not all of the hypospadias, but the hypospadias have different degrees of penile curvature. Hypospadias is a congenital defect in the abnormal position of the male urethra. The urethral orifice can be distributed in the line from the normal urethra to the perineum.

Cause

(1) Causes of the disease

In recent years, the etiology of hypospadias has been summarized in several aspects.

1. Endocrine factors Some cases of androgen receptor and 5-reductase deficiency. It has also been found that after stimulation with human chorionic gonadotropin (HCG), the androgen increase response in patients with hypospadias is significantly lower than that in the normal control group, suggesting that the hypothalamic-pituitary-gonadal axis of patients with hypospadias is not normal.

2. Environmental factors Studies have found that the incidence of hypospadias in neonates who have used progesterone in the early pregnancy is higher, and studies have shown that the levels of estradiol and estrone in patients with hypospadias increase. These studies suggest that estrogens antagonize androgen action.

3. The chromosomal aberration rate of chromosomal abnormalities in patients with hypospadias is significantly higher than that of normal people, including autosomal aberrations and sex chromosome aberrations.

4. Gene mutations In patients with hypospadias, there may be an androgen receptor gene, sex determining gene, 5-reductase gene, anti-Mullerian hormone gene, and mutation of CYP21B gene.

(two) pathogenesis

During embryonic development, the development and fusion of the ventral genital tract in the penis is affected by pituitary and androgen. If the androgen is deficient, the fusion of the wrinkles on both sides of the genital tract will cause a defect in the ventral side wall of the urethra, and the urethra will open behind the normal urethral opening of the ventral side of the penis to form a hypospadias.

According to the location of the urethral opening, the hypospadias can be divided into four types: penis head type, penis type, penis scrotum type and perineal type. Among them, the penis head type and the penis type account for the majority.

an examination

Related inspection

Amniocentesis cell culture chromosome examination laparoscopy

1. Physical examination: observe the patient's body shape, physical development, secondary sexual characteristics, external genital examination for the presence or absence of vagina, touch the texture and volume of the testis surface.

2. Abdominal ultrasound.

3. Chromosome examination.

4. Determination of urinary 17 ketosteroids.

5. Laparoscopy and gonadal biopsy.

Differential diagnosis

Differential diagnosis

Urinary tract rupture: male urethra is a muscle mucosal tube, about 20cm long, can be divided into two sections, with urogenital sputum as the boundary. The anterior urethra is a cavernous body, including the penis head, penis and ball, with a total length of 15 cm. The posterior urethra includes a membrane portion and a prostate portion, and is about 5 cm long. The male urethra has two curves of pubic bone and pubic bone. The subpubic curvature is basically fixed, and the pubic anterior curvature disappears when the penis is attached to the lower abdomen. The back of the urethra is shorter than the ventral surface and is fixed. When the penis is in a relaxed state, there are many wrinkles in the ventral surface of the urethra. The urethral mucosa is rich in glands, soft in nature, and rich in blood supply to the submucosal tissue. Male urethra is susceptible to injury due to its anatomical features. Male urethral injury is a common emergency in urology, which can cause complications such as extravasation of urine, infection, urethral stricture and fistula. Female urethra is short and rarely damaged. However, in the case of dystocia, fetal head compression or application of forceps can cause damage and produce urethral fistula. Urethral injury can only damage the mucous membrane or contusion of the urethral wall, but most of them hurt the whole layer and cause rupture of the urethra. This rupture can be longitudinal or transverse, and can be partially broken or completely cut off. Retracted, there is a gap and a misalignment between the ends. Hematuria may be present after full-thickness urethra laceration. The extent of hematuria extravasation varies depending on the location and extent of urethral injury. Familiarity with the anatomy of the perineum can be of great help in understanding the extent of hematuria.

Urethral nodules: Urethral nodules are one of the symptoms of paraurethral adenocarcinoma. Early symptoms of paraurethral adenocarcinoma are dysuria, urethral bleeding, frequent urination, and dysuria. A nodular or red hemorrhagic mass appears in the distal urethra or urethra, and local swelling of the urethra can reach the mass. When the tumor is enlarged, it can block the urethra or expand into the vestibular vestibule and vaginal opening, and there are obvious ulcers and hemorrhagic masses, accompanied by pain and possible metastasis of the groin and pelvic lymph nodes.

Pain in urination: In men, in addition to painful urination, the flow of urine appears to be small, the strength of urine is also weak, and sometimes even scattered, it may be that the urine is in the process of outflow This kind of obstacle is mostly caused by the enlarged prostate. However, it may also be one of the causes of urinary pain caused by the numbness of the muscles that control the bladder function.

Penile-like urethra: Penile-like hypospadias is the most common congenital malformation in the male genitourinary system, with an incidence of 1 in 300. The urethral ectopic opening in the ventral side of the urethra is called hypospadias. The hypospadias opening can occur anywhere from the perineum to the penis head. The distal end of the external urethra, the urethra and surrounding tissues are underdeveloped, and the formation of a fiber cord involves the penis, causing the penis to bend to the ventral side. Congenital penile curvature is not all of the hypospadias, but the hypospadias have different degrees of penile curvature.

diagnosis

I. Symptoms

1. The penis head type urethral opening is located in the ventral side of the coronary sulcus, often with fissures, and some may have urethral stricture, the dorsal foreskin is long, and the ventral side has no foreskin and ligament. The penis head is bare, small and slightly flat and spherical. The penis bends to the ventral side, but to a lesser extent, it does not affect sexual intercourse and urination.

2. The penis-type urethral opening is located at the ventral side of any part of the junction of the coronary sulcus to the penis scrotum. The urethral plate at the distal end of the urethral opening is separated, and the penis is not formed into a tubular shape. The penis is bent to the ventral side, and the more the urethral opening is curved, the more severe it is affected. Sexual intercourse and urination also affect fertility. The shape of the penis head and foreskin is the same as that of the penis head type.

3. The penis scrotum-type urethral opening is located in the midline of the scrotum, and the scrotum is often split like a female labia majora. A fiber cord is formed at the distal end of the urethral opening. The penis is severely bent and needs to be urinated. The penis is short and flat, and some resemble female clitoris. Some testicles do not fall into the split scrotum or form penile scrotum transposition.

4. Perineal urethral opening is located in the perineum, scrotal division, hypoplasia, can be combined with cryptorchidism, penis small and curved, very similar to the fat clitoris. The entire genital development is like a female vulva, which is mistaken for women by many parents. Need to urinate.

Second, diagnosis

The diagnosis of penile head type and penile type hypospadias is relatively easy, and it can be diagnosed by appearance characteristics.

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