Hypospadias
| Classification according to ICD-10 | |
|---|---|
| Q54 | Hypospadias |
| ICD-10 online (WHO version 2019) | |
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The hypospadias ( hypospadias penis ) is a congenital disorder of the urethra (urethral) of the man. The mouth of the urethra (Meatus urethrae externus) is more ventral / proximal (i.e. on the underside) than in healthy people. The hypospadias often occurs in connection with a curvature of the penile shaft (chorda), meat stenosis (narrowing of the outer urethral opening), a split prepuce (long foreskin on one side, absence of the foreskin on the other side) or an undescended testicle (Maldescensus testis) on.
The opposite, the opening of the urethra on the top of the penis , is known as epispadias .
Clinical picture
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In men, the urethral opening ends, depending on its severity, from the underside of the glans down to the perineum. The micturition (urination) at the boy standing up is difficult because the urine stream to the rear or bottom runs ( "urination typically sitting"). In girls, on the other hand, one speaks of hypospadias when the urethra ends not in the vestibule but on the inside of the vaginal wall, so that the urine then flows out of the vagina during micturition .
- Disease value
The severity of the disease depends on the severity of the hypospadias and accompanying factors such as curvature of the penile shaft (chorda), meatal stenosis (narrowing of the outer urethral orifice), a split prepuce (long foreskin on one side, lack of foreskin on the other) or an undescended testicle (Maldescensus testis) dependent. In addition to cosmetic and psychological issues, functional aspects such as difficult micturition or immissio are often complaints.
Since previously - and possibly still today - the hypospadias were not corrected in all children, one must expect to see adult patients with this malformation as well. This surprise is usually experienced when a transurethral urinary catheter is inserted. Patients feel healthy and often have healthy offspring. The extent to which a disease value then exists in each individual case must be decided individually.
In individual cases (proximal forms) an endocrinological and chromosomal evaluation is indicated . These tests should be done immediately after the birth , especially if primary sex characteristics are missing, in order to provide security for naming and other important decisions.
Emergence
In men, the urethra is formed in the first step when the urogenital gap closes towards the end of the third month of pregnancy. At this point it does not yet reach the tip of the penis. The distal portion of the urethra is formed in the fourth month of pregnancy (SSM) by cells of the ectoderm , which first form an epithelial cord that grows onto the cavity that the urethra forms at this time. In a second step, this cord channels itself and then, under physiological conditions, forms the meatus externus, which opens into the glans penis. In women, the urogenital gap does not completely fuse.
If there is an interruption in the development of the external meatus during formation, hypospadias occurs. The severity of the hypospadias is determined depending on the stage at which the development stop occurs. An early interruption of development leads to a proximal , a later interruption to a more distal form of hypospadias.
A certain form of hypospadias (PPSH) is the result of a genetic defect in which an enzyme , steroid 5α-reductase , is missing in the body.
frequency
Hypospadias occurs in about 1: 300 to 1: 1000 newborn boys and is therefore classified as "frequent". Many lighter forms may not be diagnosed because there are no visual or functional abnormalities associated with them. The causes of hypospadias are not fully understood. However, it is assumed that genetic , endocrinological and teratogenic influences can play a role. The intake of progesterone ( steroid hormone or sex hormone ) by the mother during pregnancy could also have an influence. According to one study, an increased incidence of hypospadias can be seen in underweight premature babies. The developmental disorder is likely caused by the same factors that later lead to underweight and / or premature birth .
treatment
The therapy consists primarily of a surgical correction in the 1st or 2nd year of life. In individual cases, hormonal stimulation may be necessary. A relevant meatal stenosis (narrowing of the urethral orifice) must be eliminated in the infant for functional reasons.
The procedure itself takes place under general anesthesia and takes between one and four hours, depending on the severity. More severe cases may require multiple operations, which are performed using microsurgical techniques. Optionally, a mucosal transplant can be performed e.g. B. be necessary from the oral cavity .
There are many different surgical procedures. The criteria for an operation method depend on the curvature of the penis, the location of the urethra, the length and texture of the penis, the size of the preputial apron and also on the surgeon's personal experience.
Goals of a surgical procedure:
- Extension of the limb - erection of the penis; It is important to ensure that the simple Nesbit duplication is not carried out for straightening. This shortens the penis and often leads to pain when erecting.
- Reconstruction of the urethra and urethral ending at the tip of the glans
- Possibility of normal urination and erection
- Achieve a cosmetically normal aspect of the penis; Especially for this it is important to turn to proven specialists. It should be borne in mind that hypospadias, as it is usually recognized and treated early, is a minor burden for children, but a penis that has become aesthetically unsightly following a standard procedure can mean decades of shame and withdrawal for the man concerned.
Apart from the medical aspects, a correction should also be made for later psychological reasons for the boy, as these should not be underestimated. The complication rate after a correction is relatively high and ranges from 5 to 30 percent. It depends on the severity of the hypospadias, the experience of the surgeon, the chosen approach and the medical and nursing follow-up care. Possible early complications after the operation are excessive swelling, secondary bleeding, graft loss, urethral constriction, renewed penile curvature, penile shortening and wound healing disorders . The most common late complications are fistula formation , urethral stricture and residual curvature.
In the context of syndromes
In some syndromes, hypospadias can appear as a feature, such as McKusick-Kaufman syndrome , Naguib-Richieri-Costa syndrome or N syndrome .
Web links
Individual evidence
- ↑ a b J. Steffens, among others: Frequent urological diseases in childhood . Birkhäuser, 2000, ISBN 3-7985-1245-0 , p. 63 ( limited preview in the Google book search).
- ↑ Immissio = inserting the erect member into the vagina during vaginal intercourse . Due to the unphysiological location of the urethral outlet, hypospadias in men can also lead to insufficiently deep penetration of the sperm into the vagina during sexual intercourse and thus to impaired fertility.
- ↑ a b A. G. Hofstetter et al.: Urology for the practice. Bergmann Verlag, ISBN 3-8070-0351-7 , p. 183.
- ^ Forum Health: Hypospadias .
- ↑ J. Langmann: Medical Embryology. Thieme, ISBN 3-13-446606-6 , pp. 176-203.
- ↑ Pseudovaginal Perineoscrotal Hypospadias. In: Online Mendelian Inheritance in Man . (English).
