Excess urethra

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Classification according to ICD-10
Q64.7 Other congenital malformations of the bladder and urethra
ICD-10 online (WHO version 2019)

An excess urethra ( Latin urethral duplicature ) is a very rare malformation of the urethra (urethra) with the presence of a complete - or more often - partial installation of a second urethra ( accessory ductus paraurethralis ).

There are usually several openings in the urethra ( ostium urethrae externum ), with doubling being the most common.

distribution

It is a very rare malformation that usually occurs in males. Up to the year 2000 about 200 affected persons were reported. Occurrence in the female urethra has also been described.

root cause

The cause of the embryological developmental disorder is not yet known.

Classification

Urethral duplication type 1 with blind-ended urethra (a), epispade localization of the accessory urethra (b). The accessory urethra joins the loco typico, the functional urethra is hypospad.
Urethral duplication type IIa. Complete duplication with hypospadic orifice of the urethra (b) Hypospadic urethra appears briefly.

In addition to a simple classification according to the number of existing systems into “duplication”, “trifurcation” or “quatrupication”, several typifications have been proposed.

The figures are based on the following typification:

  • Type 1 blind ending supernumerary urethra
    • (a) epispade localization of the accessory urethra
    • (b) the accessory urethra opens at the normal point (loco typico), the functional urethra hypospad
  • Type 2 complete duplication, both urethras run within the penis shaft
    • (a) of normal length
    • (b) of short length
  • Type 3 complete duplication with functional urethra and urethra opening outside the penile shaft
  • Type 4 duplication with partial or complete caudal duplication with a second urinary bladder and its own lead (epispad)

Sometimes the described types 2 + 3 are combined into one.

Probably the most common classification is that of Effmann from 1976.

  • Type I blind ending, incomplete gait formation
    • (a) distal opening ventrally or dorsally without connection to the urethra (most common form)
    • (b) Starting proximally from the urethra, ending blind
  • Type II Complete duplication throughout
    • (a-1) two separate urethras emerging from the urinary bladder without any connection to one another
    • (a-2) Second canal arises from the urethra and runs to its own meatus (parallel) or in a Y-shape from hypospadic to rectal opening
    • (b) a meatus, 2 urethras unite to form an end piece
  • Type III bladder duplication with double drainage

Clinical manifestations

Depending on the form (type) present, this urethral anomaly can either remain completely free of symptoms, but can also lead to urinary tract infections , incontinence and distraction or splitting of the urinary stream.

Excess epispade urethra

If the urethra is completely superfluous, there is urinary incontinence due to the lack of sphincter muscle (lack of the urethralis muscle ) . If there is only one incomplete double urethra (also called a blind canal), both urethra meet and a double urine stream with continence is created. A blind proximal urethra is also common.

Excess hypospad urethra

This is a double ventral urethra that is either completely or incompletely created. There is a wide range of possible shapes, for example a blind canal or an additional urethra opening into the anus.

Superfluous lateral urethra

Lateral enlargement of the urethra is possible in both boys and girls, and the genitals may also be doubled. If this is the case, it often occurs in combination with an abnormal widening (diastasis) of the pubic symphysis and with anal astresia .

Excess sagittal urethra

Sagittal multiplication of the urethra occurs only in male individuals. Additional epispadias or hypospadias are also possible.

diagnosis

The diagnosis is sometimes incidental , more often on the basis of clinical evidence.

An imaging technique, is performed by the diagnosis voiding cystourethrogram or retrograde Urethrography , urethrocystoscopy or intraoperative administration of contrast agent .

Differential diagnosis

Congenital or acquired urethral fistulas are to be differentiated.

therapy

If necessary, surgery can be performed. The functionally better urethra is preserved and any urethral defects are corrected.

literature

  • AK Ebert, K. Adamczyk: Epispadia and urethral duplication. In: The Urologist. Ed. A. Volume 54, number 5, May 2015, pp. 634-640, doi: 10.1007 / s00120-015-3792-5 , PMID 25987327 (review).
  • V. Zugor, M. Schreiber, AP Labanaris, J. Weissmüller, B. Wullich, GE Schott: Urethral duplication. Long-term results of a rare urethral malformation. In: The Urologist. Ed. A. Volume 47, number 12, December 2008, pp. 1603-1606, doi: 10.1007 / s00120-008-1859-2 , PMID 18806990 .

Individual evidence

  1. ↑ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0 , keyword urethral malformations
  2. ↑ a b c d e M. Cendron: The surplus urethra. In: JW Thüroff, H. Schulte-Wissermann (editor): Pediatric urology in clinic and practice, 2nd edition, 2000, pp. 347-351, 3rd edition, pp. 470-420
  3. ↑ a b E. Stojkova Gafner: Urethral strictures and anomalies. In: SIGUP - Swiss Interest Group for Urological Care. Retrieved April 17, 2018 .
  4. ↑ M. Baid, A. Dutta: Urethral duplication in a 15-year-old: case report with review of the literature. In: Reviews in urology. Volume 16, number 3, 2014, pp. 149-151, PMID 25337048 , PMC 4191638 (free full text) (review).
  5. ^ S. Solanki, MN Babu, V. Jadhav ,. Gowrishankar, S. Ra: Female Urethral Duplication: Rare Anomaly with Unusual Presentation. In: Journal of surgical technique and case report. Volume 7, number 1, 2015 Jan-Jun, pp. 1–3, doi: 10.4103 / 2006-8808.184939 , PMID 27512541 , PMC 4959403 (free full text).
  6. ↑ H. Erdil, A. Mavi, S. Erdil, E. Gumusburun: Urethral duplication. In: Acta medica Okayama. Volume 57, Number 2, April 2003, pp. 91-93, doi: 10.18926 / AMO / 32817 , PMID 12866749 .
  7. ^ Rare Diseases
  8. ↑ EL Effmann, RL Lebowitz, AH Colodny: Duplication of the urethra. In: Radiology. Volume 119, Number 1, April 1976, pp. 179-185, doi: 10.1148 / 119.1.179 , PMID 943804 .
  9. ^ JL Salle, H. Sibai, D. Rosenstein, AE Brzezinski, J. Corcos: Urethral duplication in the male: review of 16 cases. In: The Journal of urology. Volume 163, Number 6, June 2000, pp. 1936-1940, PMID 10799233 .

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