Abstract
The most frequent anomaly of the urogenital tract is a simple renal ectopia with one organ lying in the pelvis. Crossed renal ectopia is a less common condition in which the ectopic kidney is located on the opposite side of the midline from the ureteral insertion in the urinary bladder. The cause of both types of renal ectopia is the arrest or failure of the kidney ascent from the pelvic to the lumbar position. Whereas an accelerated ascent leads to a subdiaphragmal or intrathoracic ectopic position, an ectopic ureter can be defined as one that does not drain into the trigonum vesicae. The ectopic orificium can be located situated in the bladder neck and urethra as well as somewhere in the genital area.
Exstrophy of the urinary bladder is not a complete ectopia. Because the abdominal wall and the anterior part of the bladder wall are lacking, the bladder mucosa grows directly into the skin. The complex exstrophy of the bladder and intestine corresponds to a cloacal exstrophy, in which the bladder is split in two halves on either side of the gut portion. Testicular ectopia refers to the location of the testis in a position outside of its normal course of descent.
Prostatic ectopia does not refer to the wrong location of the entire organ, but to a scattered group of prostate glands, which are mostly found in the submucosal part of the urinary bladder or proximal urethra. Other described locations are the intestinal wall, anus, pericolic fat tissue, spleen, seminal vesicle, testis, and cervix uteri.
The associated ectopic penis, scrotum, and penoscrotal transposition are the least common and probably the absolutely most unknown malformations of the male genitalia. The ectopic penis and scrotum are located in the perineum, whereas in the transposition the penis lies above the scrotum.
Zusammenfassung
Eine der häufigsten Missbildungen der Harnwege ist die einfache Ektopie der Niere, bei der das Organ im kleinen Becken liegt. Bei der etwas selteneren gekreuzten Ektopie liegt die Niere auf der kontralateralen Seite in Bezug zur Uretermündung in die Harnblase, d. h., ihr Ureter kreuzt die Mittellinie des Körpers. Die Ursache dafür ist das Ausbleiben des Aszensus, des Aufstiegs der ursprünglichen Niere aus dem Becken in die orthotope Lage. Ein beschleunigter Aszensus führt hingegen zur intrathorakalen Nierenektopie. Ektope Ureteren münden außerhalb des Trigonum vesicae. Bei Störungen in der Organogenese kann deren Orificium sowohl in Blasenhals und Urethra als auch im Bereich der Geschlechtsorgane liegen.
Die Blasenekstrophie ist ein fehlender Verschluss der vorderen Bauchwand, sodass die Blasenschleimhaut in die umgebende Haut übergeht. Bei der kloakalen Ekstrophie ist die Harnblase gespalten, dazwischen liegt der ileozökale Anteil des Darms. Als Hodenektopie bezeichnet man die Lage des Organs außerhalb der Deszensuswege.
Bei der Ektopie der Prostata handelt sich nicht um eine Fehllagerung des gesamten Organs, sondern um versprengte Prostatadrüsen, die vor allem in der Blasenwand und in der Urethra zu finden sind. Einige Fälle wurden aber auch in der Darmwand, im Anus, im perikolischen Fettgewebe, Milz, Samenblase, Hoden sowie in der Cervix uteri und Vagina beschrieben.
Die Penis- und Skrotumektopie und die penoskrotale Transposition sind die seltensten Missbildungen der männlichen Geschlechtsorgane überhaupt. Die seltenste Form ist die gemeinsame Verlagerung von Penis und Skrotum ins Perineum. Bei der Transposition liegt hingegen der Penis unter dem Skrotum oder zwischen beiden Hoden in einer Furche des gespaltenen Hodensacks.
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Notes
An abnormal elevation of the diaphragm.
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G. Mikuz declares that he has no competing interests.
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K.W. Schmid, Essen
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Mikuz, G. Ectopias of the kidney, urinary tract organs, and male genitalia. Pathologe 40 (Suppl 1), 1–8 (2019). https://doi.org/10.1007/s00292-018-0505-z
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DOI: https://doi.org/10.1007/s00292-018-0505-z