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Epispadie und Harnröhrenduplikatur

Epispadias and urethral duplication

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Zusammenfassung

Die Epispadie ist die mildeste Form des Blasenekstrophie-Epispadie-Komplexes und tritt nur selten isoliert auf. Charakteristisch ist die am Dorsum penis gelegene offene Urethralplatte. Abhängig vom Ausprägungsgrad dominiert klinisch eine dauernde oder intermittierende Harninkontinenz. Die primäre Peniskorrektur wird um den 12. Lebensmonat empfohlen und sollte in einem Zentrum mit ausgewiesener Expertise erfolgen. Eine kontinenzschaffende Blasenhalsrekonstruktion kann im Verlauf nötig werden. Trotz adäquater Rekonstruktionstechniken kann die psychosoziale und psychosexuelle Entwicklung der Betroffenen beeinträchtigt sein. Die Inzidenz der Urethralduplikatur bleibt aufgrund ihrer Seltenheit unklar. Mehrere Klassifikationen zeigen die anatomische Variationsbreite. Die klinische Symptomatik ist abhängig von Mündung, Verlauf und Schließmuskelbeziehung der akzessorischen Urethra. Neben der klinischen Untersuchung gehört eine Miktionszysturethrographie zur Basisdiagnostik. Nach endoskopischer Diagnostik kann eine individuelle operative Strategie erstellt werden.

Abstract

Epispadias is the mildest form of the exstrophy–epispadias complex and rarely occurs in an isolated form. The characteristic clinical sign is a mucosal strip on the penile dorsum. Clinically, epispadias presents with urinary incontinence. Primary surgical correction is recommended at the age of 12 months and should only be performed at a specialized center. During follow-up, further bladder neck surgery may be necessary. Despite a functional and cosmetically appealing penis, psychosocial and psychosexual development of the affected individuals can severely be affected. The incidence of urethral duplication remains unclear. Several classifications reflect the anatomical variation. The clinical presentation depends on the continuity of the duplicated urethra and its relationship to the sphincteric mechanism. In addition to clinical examination, a voiding cysturethrography is part of the basic diagnostic workup. After endoscopic examination an individual operative approach can be defined.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. A.-K. Ebert und K. Adamczyk geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Ebert, AK., Adamczyk, K. Epispadie und Harnröhrenduplikatur. Urologe 54, 634–640 (2015). https://doi.org/10.1007/s00120-015-3792-5

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