Hypospadie

Erkenntnisse und Herausforderungen

Hypospadias

Insights and challenges

Zusammenfassung

Ventrale Tubularisationsstörungen der Harnröhre (Hypospadien) gehören zu den zweithäufigsten angeborenen kinderurologischen Fehlbildungen. Seit Jahren wird eine steigende Inzidenz dieser Anomalie beobachtet, die in den USA eine Verdopplung nahelegt. Für den europäischen Raum fehlen bisher eindeutige epidemiologische Daten. Die Frage nach den Ursachen für diese Störung bleibt unbefriedigend offen. Genetische Risikokonstellationen oder auch Umwelteinflüsse, insbesondere durch sog. „endocrine-disrupting chemicals“ (EDC) werden als auslösende Faktoren diskutiert. Knaben weisen nach In-vitro-Fertilisation häufiger eine Hypospadie auf als in nicht reproduktiv assistierten Schwangerschaften. In Tiermodellen (v. a. Mäusen) konnten Kausalzusammenhänge zwischen einer pränatalen hormonalen Exposition (Östrogene, Progesteron) und Antiandrogenen wie Flutamid, Finasterid aber auch antiandrogen wirkenden Fungiziden (Vinclozolin) und Phthalaten und der Ausbildung einer Hypospadie belegt werden. Indikationen zur operativen Korrektur betreffen das ästhetische und funktionelle Defizit. Die Indikationen und die Komplikationen der Korrekturoperationen müssen ausführlich und realistisch besprochen werden. Neuere Publikationen zeigen, dass mit zunehmender Nachbeobachtungszeit das Risiko für Komplikationen steigt. Eine große Erfahrung der Zentren mit ausreichend hoher Fallzahl wirken sich positiv auf die Komplikationen aus. Eine kompetente Nachsorge bis ins Erwachsenenalter sollte gewährleistet sein.

Abstract

Disorders of the ventral tubularization of the urethra, such as the hypospadias, are among the second most frequent congenital childhood malformations. An increasing incidence has been observed suggesting a doubling in the US, which could not be documented for the European area. The underlying causes of this congenital defect remain unidentified. Genetic risk constellations or environmental influences, in particular by so-called endocrine disrupting chemicals (EDCs), are discussed as triggering factors. Boys after in vitro fertilization are more likely to have hypospadias than in nonreproductive-assisted pregnancies. Animal models (especially mice) elicited causal relationships between prenatal hormonal exposure (estrogens, progesterone) and antiandrogens such as flutamide, finasteride, antiandrogenic fungicides (vinclozolin) and phthalates and the formation of hypospadias. An aesthetic and/or functional deficit are indications for surgical correction. The indications and the complications of hypospadias surgery must be in detail and realistically discussed with the parents and patients. Recent publications demonstrated that the risk of complications increases with the increase of the follow-up time. High-volume centers with extensive experience have a positive effect on the complication rate. Competent follow-up to adult age should be ensured.

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Correspondence to I. Rübben.

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I. Rübben und R. Stein geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Rübben, I., Stein, R. Hypospadie. Urologe 56, 1256–1265 (2017). https://doi.org/10.1007/s00120-017-0498-x

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Schlüsselwörter

  • Androgene
  • Embryologie
  • Vorhaut
  • Penis
  • Urethra
  • Hypospadie

Keywords

  • Androgen
  • Embryology
  • Foreskin
  • Penis
  • Urethra
  • Hypospadias