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Findings in this case: 1. Absence of a normal RIGHT kidney. 2. Enlarged LEFT “kidney” with a normal-appearing upper collecting system and an abnormally rotated lower collecting system. 3. Normal entrance of the RIGHT ureter into the bladder.
Diagnosis: Crossed fused ectopia.
File: 5.A
Organ system: 5.0 Kidney, ureter and bladder.
Organ: A. Kidney.
Information:
Renal rotational abnormalities and ectopia; 1) Non-fused renal ectopia: A.k.a. pelvic kidney. A) Failure to ascend and rotate. B) Often maintains fetal blood supply from the iliac vessels and/or the distal aorta. 2) Fused renal ectopia: Horseshoe kidney – non-cross fused ectopia. A)Kidneys joined at poles by parenchymal / fibrous isthmus. B) Occurs if the developing kidneys come into contact during ascent. C) The lower poles of the kidney is fused. Ascension stops when the the isthmus reaches the inferior mesenteric artery. D) Multiple renal arteries and/or veins. E) Increased risk for hydronephrosis, renal stones and renal cell carcinoma. F) Most common fusion anomaly. G) Incidence: 1-4:1,000 births; 0.2-1% (autopsy series); H) M:F = 2-3:1
Cross fused ectopia: 1) Occurs when there is contact between the ascending kidneys and one kidney moves to the opposite side rather than ascending on its native side. 2) The ectopic kidney is always inferior to the native kidney. Malrotation of the ectopic kidney almost always occurs. 3) Incidence is approximately 1 and 7500 autopsies. Approximately 1 in 1000 live births. 4) Occurs in males greater than females and RIGHT greater than LEFT in a ratio of approximately 2:1.
Crossed, non-fused ectopia: 1) Rare anomaly occurring in less than 10% of crossed ectopias. 2) Occurs more frequent on the RIGHT than the LEFT and more commonly in males females.
Rotational abnormality: 1) Nonrotation: Renal pelvis is directed anteriorly. 2) Over rotation: Renal pelvis is directed posteriorly. 3) Inverse rotation: Renal pelvis is directed laterally.
References:
Unfused renal ectopia: a rare form of congenital renal anomaly
Gül Nihal NURSAL and Gülgün BU¨YU¨KDEREL
Annals of Nuclear Medicine Vol. 19, No. 6, 507–510, 2005. http://www.jsnm.org/files/paper/anm/ams196/ANM19-6-11.pdf
Teresa Berrocal, Pedro López-Pereira, Antonia Arjonilla, and Julia Gutiérrez
Anomalies of the Distal Ureter, Bladder, and Urethra in Children: Embryologic, Radiologic, and Pathologic Features Radiographics September 2002 22:1139-1164
Abstract
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Normal Development of the Kidney
David A. Hatch, M.D.
http://www.meddean.luc.edu/lumen/MedEd/urology/nlrendev.htm
Raymond B. Dyer, Michael Y. Chen, and Ronald J. Zagoria
Classic Signs in Uroradiology Radiographics October 2004 24:S247-S280; doi:10.1148/rg.24si045509
Abstract
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PREVALENCE OF RENAL ECTOPIA BY DIAGNOSTIC IMAGING
Muhammad Asghar* and Fidaullah Wazir**
Department of *Radiology and **Anatomy, Gomal Medical College, Dera Ismail Khan, Pakistan. Gomal Journal of Medical Sciences July-December 2008, Vol. 6, No. 2.
www.gjms.com.pk/ojs786/index.php/gjms/article/download/132/131
