Megacalicosis

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Classification according to ICD-10
Q63.8 Other specified congenital malformations of the kidney
ICD-10 online (WHO version 2019)

The Megakalikose , from ancient Greek μέγας megas , German , tall ' and ancient Greek κύλιξ KYLIX , German , chalice' , Latin calix , is a very rare congenital malformation with the main feature of great cups in one or both kidneys. There is no drainage disturbance . Often the number of calyxes is increased to 12–25, the renal pelvis and the lower urinary tract are normal.

Synonyms are: congenital megacalicosis; Megapolicalicosis; Megacalycosis; English Puigvert Disease; Puigvert's disease

The first description comes from 1963 by the Spanish urologist Antonio Puigvert Gorro (1905–1990).

distribution

The frequency is given as less than 1 in 1,000,000, so far only a few affected people have been reported. The male sex is affected about 6 times as often as the female. There is an association with the ( congenital ) megaureter .

root cause

The cause is assumed to be a disturbance in embryogenesis , the connection between the ureteral bud and the metanephrone, hypoplasia of the muscles in the renal pelvis or hypoplasia of the juxtamedullary glomeruli with hypoplasia of the medullary pyramids.

Clinical manifestations

Clinical criteria are:

Often it is an incidental finding .

diagnosis

The diagnosis is based on imaging by sonography , excretory urogram or magnetic resonance tomography . The kidneys are of normal size, the calyxes are enlarged at the expense of the medulla, and the renal cortex is normal. The cortex / medulla ratio has changed from normal 1: 2 to 1: 1. The number of cups can be increased considerably (megapolicalicosis). The vascular network is also inconspicuous.

Differential diagnosis

The following are to be distinguished:

therapy

Treatment is usually not required.

literature

  • C. Kalaitzis, E. Patris, E. Deligeorgiou, P. Sountoulides, A. Bantis, S. Giannakopoulos, S. Touloupidis: Radiological findings and the clinical importance of megacalycosis. In: Research and reports in urology. Volume 7, 2015, pp. 153–155, doi: 10.2147 / RRU.S81519 , PMID 26528455 , PMC 4621186 (free full text).
  • H. Kalaitzis, M. Fedel, R. Nagel: Megacalicosis: a rare “disease picture”. In: Current Urology. 26, 1995, p. 285, doi: 10.1055 / s-2008-1057815 .
  • HPW Kozakewich, RL Lebowitz: Congenital megacalyces. In: Pediatric Radiology. 2, 1974, p. 251, doi: 10.1007 / BF00972699 .

Individual evidence

  1. a b c d e f Pschyrembel online
  2. a b c Megacalicosis, congenital. In: Orphanet (Rare Disease Database).
  3. Urotoday
  4. A. Puigvert: Megacaliosis: Diagnostico con la hidrocaliectasia. In: Medicina clinica. Volume 41, October 1963, pp. 294-302, PMID 14125258 .
  5. Orphanet_prevalences
  6. Radiopaedia
  7. D. Manski, online textbook of urology
  8. a b c d Marcel Bettex (ed.), Max Grob (introduction), D. Berger (arrangement), N. Genton, M. Stockmann: Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd, revised edition, p. 8.35, Thieme, Stuttgart / New York 1982, ISBN 3-13-338102-4
  9. W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. 2nd edition, Springer 1996, ISBN 3-540-60224-0 , p. 694