NDT Advance Access originally published online on December 2, 2005
Nephrology Dialysis Transplantation 2006 21(3):799-801; doi:10.1093/ndt/gfi301
© The Author [2005]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Case Report
Thoracic kidney and contralateral ureteral duplicationa case report and review of the literature
Chun-Fu Lai1,
Wen-Chih Chiang2,
Ju-Yeh Yang3,
Horng-Chin Yan4,
Shuei-Liong Lin2,
Yung-Ming Chen2,
Kwan-Dun Wu2 and
Bor-Shen Hsieh2
1 Department of Internal Medicine, Far Eastern Memorial Hospital, Taipei, 2 Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, 3 Department of Internal Medicine, National Taiwan University Hospital, Yun-Lin branch, Yunlin County and 4 Division of Pulmonary and Critical Care, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan
Correspondence and offprint requests to: Dr Kwan-Dun Wu, Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 7, Chung-Shan South Rd, Taipei, Taiwan, 100. Email: kdw{at}ha.mc.ntu.edu.tw
Keywords: abnormalities; complications; thoracic kidney; ureteral duplication
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Introduction
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The thoracic location of an aberrant kidney is the least encountered,
accounting for less than 5% of renal ectopy. It occurs more
frequently in males and usually on the left side [
1]. Most of
the patients with thoracic kidneys are asymptomatic, in contrast
to pelvic kidneys, and such kidneys always function normally.
A patient with this anomaly is usually disclosed incidentally
by routine radiological examination. On the other hand, ureteral
duplication is the most common congenital anomaly of the urinary
tract. We report an adult female with left thoracic kidney and
right ureteral duplication, a combination that is extremely
rare. Concurrent anomalies or complications accompanying with
thoracic kidneys reported in the literature are reviewed, along
with a discussion of the role of embryology in these anomalies.
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Case
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A 31-year-old Chinese female teacher was referred to a medical
clinic due to an abnormal chest lesion found incidentally. She
underwent a health examination, and homogeneous opacity superimposed
on the cardiac silhouette was found above the left hemidiaphragm
(
Figure 1). She was completely asymptomatic. There was no history
of trauma or operation. The breathing sounds were clear over
bilateral lungs, and the remainder of the physical examination
was unremarkable. All blood and urine test results were normal.
An ultrasonography revealed the spleen in the normal position,
and the left kidney was in a cephalic direction to it (
Figure 2).
A computer tomography demonstrated the left kidney within
the left thoracic cage (
Figure 3). The subsequent intravenous
pyelography confirmed that the functioning left kidney was cephalic
malpositioned, located between the levels of the 9th and 12th
thoracic vertebrae, along with a long ureter. It also revealed
complete ureteral duplication of the right kidney (
Figure 4).
The insertion site of the right upper moiety ureter was in position
without obstruction. Since there were no respiratory or urinary
complications, the patient was followed in an out-patient clinic.

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Fig. 2. Renosonogram revealed that the left kidney was in the cephalic direction to the spleen, which was in normal position.
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Fig. 3. Computer tomography disclosed typical appearance of left thoracic kidney with contrast enhancement.
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Fig. 4. Intravenous pyelogram demonstrated left thoracic kidney with normal-appearing caliceal system and a long ureter. Ureteral duplication was found in the right kidney.
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Discussion
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In the embryogenesis of the kidneys, cranial migration of the
embryonic metanephrons occurs during the second month of gestation.
It may become arrested at any site above its original point
in the pelvis. In contrast, the ascending developing kidney
may rarely over-shoot and ascend to a higher location
than normal, resulting in thoracic ectopia.
Delayed ingrowths of the ureter bud into the metanephrons, in turn, delaying the differentiation of the metanephrogenic tissue, possibly resulting in the prolongation of the ascending process and high renal ectopia [1]. Diaphragmatic leaflets are formed at the end of the eighth week of gestational age when normal kidneys reach their adult position. Delay or failure of closure of the pleuroperitoneal membrane allowing excessive renal ascent may be another cause of thoracic kidneys [2]. However, these explanations are controversial, and the exact mechanism is not clear [1,3,4]. In addition, traumatic holes in the diaphragm, acquired or congenital hernia through Bochdalek foramen, and eventration of the diaphragm may account for the aberrance [1,36].
The associated anomalies in other organ systems with thoracic kidney are extremely rare and not consistent. Most patients have their adrenal gland and spleen in the normal position, which is the case in our patient. Nevertheless, concurrent thoracic kidneys with high ectopic adrenal glands or spleens have been demonstrated [3,7]. Cases have been reported with accompanying ureteropelvic junction (UPJ) obstruction [8,9], ureteral duplication [10], renal calculi [11], renal cyst [12], diaphragmatic hernia [36], wandering spleen [5], dextocardia [13], and patent ductus arteriosus [10]. Renal cell carcinoma in a thoracic kidney has also been reported [14]. Thoracic kidneys have been found in multiple, severe congenital defects with early death [3,13,15] (Table 1). These cases remind us that, when evaluating a patient with a thoracic kidney, searching for possible complications or concurrent anomalies, albeit infrequent, is very important.
Abnormal division of ureteral bud(s) originating from the mesonephric
duct may spread to the ureter-pelvicalyceal system [
16]. A different
degree of duplication is the most commonly seen congenital urinary
tract anomaly, which may be associated with vesicoureteral (VU)
reflux, ureterocele, or ectopic ureteral insertion [
17].
Our patient had a left thoracic kidney and contralateral ureteral duplication. The combination of anomalies is extremely rare and only one case has been reported in the English language literature since 1966 [10]. A presumptive mechanism for our case is abnormal or unbalanced stimulations to the bilateral mesonephric duct during embryogenesis. A delayed ingrowth of the left uretera bud into the metanephrons resulted in left thoracic kidney. On the other hand, two independent buds arising from the right mesonephric duct led to right ureteral duplication.
In conclusion, thoracic kidney should be kept in mind in the differential diagnosis of thoracic tumours. Early diagnosis of the condition will avert unnecessary interventional examinations. Complications of such aberrance may not be frequent; however, searching for concurrent anomalies, such as UPJ obstruction, ureteral duplication with ureterocele or VU reflux, and neoplasm, is important.
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Acknowledgments
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This study was supported by grants from the Ta-Tung Kidney Foundation
and Mrs. Hsiu-Chin Lee Kidney Research Foundation, Taipei, Taiwan.
Conflict of interest statement. The authors declare that there is no potential conflict of interest.
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Received for publication: 10. 9.05
Accepted in revised form: 8.11.05

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