Horseshoe kidney

from Wikipedia, the free encyclopedia
Classification according to ICD-10
Q63.1 Horseshoe kidney
ICD-10 online (WHO version 2019)
Horseshoe kidney (pink), schematic
3D VR representation of a computed tomography of a horseshoe kidney

The horseshoe kidney is a congenital malformation with partial fusion of both kidneys , which are normally separate , so that they form the eponymous shape of a horseshoe . It is not a disease, but an anatomical deviation from the norm (anomaly).

Epidemiology

The incidence of a horseshoe kidney is 1: 400-1: 800. It is found in around 15% of patients with Turner syndrome . There is also an association with trisomy 18 , and more rarely with Williams-Beuren syndrome or Eastman-Bixler syndrome .

Emergence

It is believed that the horseshoe kidney arises in the embryonic stage through the union of the kidneys on both sides, namely at the point in time when the two organs are still very close to each other with five weeks in the pelvis.

Clinical manifestations

The two kidneys are most often connected to each other at the lower pole by a connective tissue or, more often, a parenchymal bridge. As a result, the axial position of the kidneys, which normally runs from top-inside to bottom-outside, is changed, at the same time the rotation of the hollow system inward ( medial ) has not occurred, so that the renal pelvis points forward ( ventral ). As a result of the changed position of the renal pelvis, the ureters usually extend cranially and must run "over the mountain" of the parenchymal bridge.

The abnormal position of the renal pelvis calyx system predisposes to urinary disorders with possible consequences such as nephrolithiasis or pyelonephritis due to unfavorable drainage conditions .

In many cases, the horseshoe kidney is asymptomatic or appears to be a seemingly smaller kidney on ultrasound . In children with a horseshoe kidney, Wilms' tumor is two to eight times more common than the average.

As an unusual peculiarity there is the Rovsing syndrome named after Niels Thorkild Rovsing (lower abdominal pain when a horseshoe kidney is present due to vascular compression)

Investigation methods

Axial horseshoe kidney on computed tomography.

All conventional imaging methods are suitable for diagnosing a horseshoe kidney, above all sonography , computer tomography as well as magnetic resonance tomography , but also conventional X-ray examinations such as urography .

literature

  • E. Richter, W. Lierse: Radiological anatomy of the newborn for x-rays, sonography, CT, MRI , 1990 Urban & Schwarzenberg, ISBN 3-541-13141-1
  • V. Hofmann, KH Deeg, PF Hoyer: Ultrasound diagnostics in pediatrics and pediatric surgery. Textbook and atlas. Thieme 2005, ISBN 3-13-100953-5 .

Individual evidence

  1. a b c horseshoe kidney. In: Orphanet (Rare Disease Database).
  2. ^ R. Kleta, JH Brämswig: Horseshoe kidney and Turner syndrome. In: Nephrology, dialysis, transplantation: official publication of the European Dialysis and Transplant Association - European Renal Association. Vol. 15, No. 7, July 2000, p. 1094, ISSN  0931-0509 . PMID 10862660 .
  3. ^ L. Spitz, AG Coran (Editors) Rob & Smith's Operative Surgery. Pediatric Surgery, 5th Edition 1995, Chapman & Hall, ISBN 0-412-59110-3

Web links