hypospadias
Introduction
Introduction to hypospadias The urethral ectopic opening in the ventral side of the urethra is called hypospadias, and 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. basic knowledge The proportion of illness: the incidence rate is about 0.03% Susceptible people: no specific population Mode of infection: non-infectious Complications: cryptorchidism inguinal hernia, prostate cyst, urinary tract obstruction
Cause
Cause of hypospadias
Endocrine factors (15%):
In some cases, androgen receptor and 5-reductase deficiency, it is also found that after stimulation with human chorionic gonadotropin (HCG), the androgen increase response in patients with hypospadias is significantly lower than that of the normal control group. The hypothalamic-pituitary-gonadal axis of patients with hypospadias is abnormal.
Environmental factors (15%):
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 estrogen has Antagonize androgen action.
Chromosomal abnormalities and genetic mutations (20%):
The rate of chromosomal aberrations in patients with hypospadias is significantly higher than in the normal population, including autosomal aberrations and sex chromosome aberrations. It was found that patients with hypospadias may have an androgen receptor gene, sex determining gene, 5-reductase gene, anti-Mullerian hormone gene, and mutation of CYP21B gene.
Pathogenesis
During embryonic development, the development and fusion of the ventral genital tract in the penis is affected by the pituitary and androgen. If the androgen is deficient, the fusion of the pleural folds on both sides of the genital tract will cause a defect in the ventral side wall of the urethra. The urethra of the penis is behind the normal urethral opening, forming a hypospadias.
According to the urethral opening, the hypospadias can be divided into penis head type, penis type, penis scrotum type and perineal type, among which penis head type and penis type are dominant.
Prevention
Hypospadiac prevention
After surgical correction of penile curvature and urethra formation, the patient can stand urinating and can ejaculate in the vagina. In general, the appearance and prevention of sputum are still a problem that needs to be highly valued in various shaping operations.
Complication
Hypospadias complications Complications, cryptorchidism, inguinal hernia, prostate cyst, urinary tract obstruction
1. Cryptorchidism and inguinal hernia
The most common concurrent malformation of hypospadias is cryptorchidism and inguinal hernia, the incidence of which is 7% to 13%, and the closer the urethral opening is to the scrotum, the higher the incidence.
2. Urinary tract malformation
The incidence of posterior hypospadias is about 1% to 5%. The incidence of other system malformations is high. The incidence of malformation is 7%, the incidence of malformation is 13%, and the incidence of three system malformations. 37%.
3, prostate capsule
Prostate sac is a manifestation of Müllerian insufficiency or urinary sinus dysplasia during embryonic development. The incidence of posterior hypospadias is 10% to 15%. Prostate sac can cause urinary tract obstruction. Formation and infection of stones in the sac.
4, hermaphroditism
Severe hypospadias such as blurred gender characteristics of the external genitalia such as testicular hypoplasia, small penis, penile scrotum transposition, scrotal division and other manifestations, should pay attention to the situation of hermaphroditism, should be tested for chromosomes and related endocrine function tests, Uncommon accompanying malformations include anal atresia and meningocele.
Symptom
Hypospadias symptoms Common symptoms Male urination tingling persistent pain Urinary tract rupture Penile short giant yin penis urethra
1, penis head type
The urethral opening is located in the ventral side of the coronary sulcus, often in the form of fissures, some may be complicated by urethral stricture, the dorsal foreskin is long, the ventral side is without foreskin and ligament, the penis head is bare, small and slightly flat, spherical, penis to the abdomen Side bending, but to a lesser extent, does not affect sexual intercourse and urination.
2, penis type
The 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 urethral opening is more closely curved to the side, affecting sexual intercourse and urination. It also affects fertility, and the shape of the penis head and foreskin is the same as that of the penis head type.
3, penis scrotum type
The urethral opening is located in the midline of the scrotum. The scrotum is often split like a female labia majora. The distal end of the urethra is formed with a fibrous cord. The penis is severely curved. It needs to be urinated, the penis is short and flat, and some are like female clitoris. The testicles did not fall into the dividing scrotum or form a penile scrotum transposition.
4, perineal type
The urethral opening is located in the perineum, the scrotum is divided, the scrotum is incomplete, and the cryptorchidism can be combined. The penis is small and curved, which is like a hypertrophic clitoris. The whole genital development is like a female vulva, so that many parents mistakenly think that women need to urinate.
Examine
Hypospadias examination
Laboratory, X-ray, endoscopy: penis scrotum type and perineal type of urethra often accompanied by scrotal division, the external reproductive organs sex is difficult to determine, so oral buccal mucosa smear and karyotype should be examined to determine gender, Urethoscopy and cystoscopy can be used to understand the development of a colon in males; excretory urography can be used to understand whether congenital malformations of recurrent kidneys and ureters are combined. Excretory urethrography for patients with hypospadias can be classified as a routine examination. The value of glans type hypospadias is small, because the incidence of upper urinary tract malformation in these patients is not higher than that of ordinary people.
Diagnosis
Diagnosis of hypospadias
diagnosis
The diagnosis of penile head type and penile type hypospadias is relatively easy, and it can be diagnosed by appearance characteristics.
Differential diagnosis
Penile scrotal and perineal hypospadias should be distinguished from female pseudohermaphroditism and true hermaphroditism, especially those with cryptorchidism, regardless of the type of hypospadias, the sex chromatin should be negative, the sex chromosome is XY, urinary 17-ketosteroids are normal, and the gonads are testes.
1. Female pseudohermaphroditism
The cause is due to the congenital defects of certain enzymes in the adrenal cortex, resulting in abnormal synthesis and metabolism of the adrenal cortex, which increases the intermediate metabolites of androgenic properties, male genitalia of the fetus, and the vulva continues to develop in the male direction after birth, clitoris hypertrophy Similar to the penis, the urethral opening is located in the hypertrophic root of the clitoris and resembles the perineal hypospadias. The vagina is narrow. The main points of identification are as follows:
1 Careful examination of the vulva: In addition to the urethral opening, there is still a vaginal opening; there is no testicle in the enlarged labia majora.
2 urine 17-ketosteroid test: the value increased.
3 sex chromatin examination: the oral mucosal epithelium or vaginal mucosal epithelium, skin or white blood cells, the positive rate of check chromatin after special staining, the disease is female, the positive rate should be higher than 10%.
4 sex chromosome examination: should be XX, if necessary, can do adrenal imaging examination, except for adrenal cortical tumors.
2, true hermaphroditism
The genital gland of true amphoteric malformation has both testis and ovary, or egg testis. Therefore, the external genitalia can show the appearance of both sexes at the same time. It can also have a typical appearance of hypospadias, and its chromatin can be positive. Can be negative; sex chromosome 2/3 is XX, 1/3 is XY, if sex chromatin is positive, sex chromosome is XX, can exclude hypospadias, if the gender is not determined or the final diagnosis is true hermaphroditism, then Gonadal biopsy is the basis.
3, need to be differentiated from adrenal gland abnormalities.
