Megacystis-Megaureter Syndrome

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Classification according to ICD-10
Q62.7 Congenital vesico-uretero-renal reflux
ICD-10 online (WHO version 2019)

The megacystis-megaureter syndrome (MCMUS) is a rare disease with a combination of solid VUR in a significantly expanded ureter and a large volume, thin-walled bladder due to a constant shuttling of urine from the bladder and ureter. There is no urinary obstruction . Often there is an accompanying urinary tract infection .

The clinical picture is often associated with bilateral kidney dysplasia and impaired kidney function . Synonyms are: Megaureter-Megacystis Syndrome

The name was coined in 1975 by DI Williams.

It is not a syndrome in the strict sense of the word, as only the key findings of radiological examinations are described. The name includes various clinical pictures with pendulous reflux.

After the urinary bladder has been relieved, the dilatation of the ureters and upper urinary tract normalizes. Evidence of renal dysplasia are imaged in the X-ray the hand swore like upturned goblets of the kidney.

The diagnosis is made or suspected prenatally today .

literature

  • W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, p. 701, 5.3.3, ISBN 3-540-60224-0 .
  • KA Burbige, RL Lebowitz, AH Colodny, SB Bauer, AB Retik: The megacystis-megaureter syndrome. In: The Journal of urology. Volume 131, Number 6, June 1984, pp. 1133-1136, doi: 10.1016 / s0022-5347 (17) 50843-5 , PMID 6726913 .
  • Marcel Bettex (ed.), Max Grob (introductory), D. Berger (editing), N. Genton, M. Stockmann: Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd, revised edition, pp. 8.116, 8.126, Thieme, Stuttgart / New York 1982, ISBN 3-13-338102-4

Individual evidence

  1. ↑ Megacystis-Megaureter Syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ emedicine
  3. ↑ https://www.pschyrembel.de/Megazystis-Megaureter-Syndrom/K0DW6 Psyrembel online
  4. ↑ R. Stein, J. Thüroff: Urnleiteranomalien. In: HU Schmelz, C. Sparwasser, W. Weidner (Ed.): Specialist knowledge of urology. Springer, Berlin, Heidelberg 2014, doi : 10.1007 / 978-3-642-44942-0_28 , print ISBN 978-3-642-44941-3, online ISBN 978-3-642-44942-0
  5. ^ DI Williams: Surgery of the uretero-vesical junction. In. AW Wilkinson: Recent advances in Pediatric Surgery. Vol. 3. Livingstone 1975
  6. ↑ A. Sigel: Urinary obstruction in childhood. In: A. Sigel, R.-H. Ringert (Ed.): Pediatric urology. 2nd edition, p. 103 8.5.6., Springer, Berlin 2001, ISBN 3-662-08081-8 (print) / ISBN 978-3-662-08080-1 (e-book), p. 118.
  7. ^ UV Willi, RL Lebowitz: The so-called megaureter-megacystis syndrome. In: American Journal of Roentgenology . Volume 133, Number 3, September 1979, pp. 409-416, doi: 10.2214 / ajr.133.3.409 , PMID 111498 .
  8. ↑ JW Thüroff, H. Schulte-Wissermann (editor): Pediatric urology in clinic and practice. , 2nd edition, pp. 225ff, 2000, Thieme, ISBN 3-13-674802-6
  9. ^ J. Mandell, RL Lebowitz, CA Peters, JA Estroff, AB Retik, BR Benacerraf: Prenatal diagnosis of the megacystis-megaureter association. In: The Journal of urology. Volume 148, Number 5, November 1992, pp. 1487-1489, doi: 10.1016 / s0022-5347 (17) 36945-8 , PMID 1433553 .

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