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NDT Advance Access originally published online on December 2, 2005
Nephrology Dialysis Transplantation 2006 21(3):799-801; doi:10.1093/ndt/gfi301
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© 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 duplication—a 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



   Introduction
 Top
 Introduction
 Case
 Discussion
 References
 
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.



   Case
 Top
 Introduction
 Case
 Discussion
 References
 
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.


Figure 1
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Fig. 1. Plain chest X-ray revealed an ovoid opacity posterior to the cardiac silhouette.

 
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.


Figure 2
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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.

 

Figure 3
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Fig. 3. Computer tomography disclosed typical appearance of left thoracic kidney with contrast enhancement.

 

Figure 4
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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.

 


   Discussion
 Top
 Introduction
 Case
 Discussion
 References
 
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 [1,3–6].

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 [3–6], 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.


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Table 1. Associated anomalies or complications in patients with thoracic kidney

 
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.



   Acknowledgments
 
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.



   References
 Top
 Introduction
 Case
 Discussion
 References
 

  1. Donat SM, Donat PE. Intrathoracic kidney: a case report with a review of the world literature. J Urol 1988; 140: 131–133[Medline]
  2. Yalcinbas YK, Sasmaz H, Canbaz S. Thoracic left kidney: a differential diagnosis dilemma for thoracic surgeons. Ann Thorac Surg 2001; 72: 281–283[Abstract/Free Full Text]
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  4. Sözübir S, Demir H, Ekingen G, Güvens BH. Ectopic thoracic kidney in a child with congenital diaphragmatic hernia. Eur J Pediatr Surg 2005; 15: 206–209[CrossRef][Medline]
  5. Pelizzo G, Lembo MA, Franchella A, Giombi A, D'Agostino F, Sala S. Gastric volvulus associated with congenital diaphragmatic hernia, wandering spleen, and intrathoracic left kidney: CT findings. Abdom Imaging 2001; 26: 306–308[Medline]
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  14. Kubricht III WS, Henderson RJ, Bundrick WS, Venable DD, Eastham JA. Renal cell carcinoma in an intrathoracic kidney: radiographic findings and surgical considerations. South Med J 1999; 92: 628–629[Medline]
  15. Pickney LE, Moskowitz PS, Lebowitz RL, Prilzsche P. Renal malposition associated with omphalocele. Radiology 1978; 129: 677–682[Abstract]
  16. Cooper CS, Snyder III HM. Ureteral anomalies. In: Gillenwater JY, Grayhack JT, Howards SS, Mitchell ME, eds. Adult and Pediatric Urology, 4th edn. Lippincott Williams & Wiklins, Philadelphia, PA: 2002; 2155–2187
  17. Stephens FD, Smith ED, Hutson JM. Congenital Anomalies of the Urinary and Genital Tracts. Isis Medical Media, Oxford, UK: 1996; 243–262
Received for publication: 10. 9.05
Accepted in revised form: 8.11.05


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This Article
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