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Supernumerary Kidneys: a Clinical and Radiological Analysis of Nine Cases Peter Rehder1†, Rafael Rehwald2,3†, Julia M

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Supernumerary Kidneys: a Clinical and Radiological Analysis of Nine Cases Peter Rehder1†, Rafael Rehwald2,3†, Julia M Rehder et al. BMC Urology (2019) 19:93 https://doi.org/10.1186/s12894-019-0522-0 RESEARCH ARTICLE Open Access Supernumerary kidneys: a clinical and radiological analysis of nine cases Peter Rehder1†, Rafael Rehwald2,3†, Julia M. Böhm4, Astrid E. Grams5, Alexander Loizides4, Marco Pedrini1, Jannik Stühmeier1 and Bernhard Glodny4* Abstract Background: A supernumerary kidney (SK) is an additional kidney with its own capsule and blood supply that is not fused with the ipsilateral kidney (IK). Because individual case reports indicate a high morbidity rate, the aim of this retrospective study was a detailed analysis of this rare anatomical variant. Methods: Our systematic imaging-based search for SKs, conducted in the period from 2000 and to 2017, yielded 9 cases in total (5 men, 4 women; mean age: 51.8 ± 22.8 years). Results: The SKs were observed on the right in six and on the left side in three cases. In six subjects (66%) they were located caudal and in three cases (33%) cranial to the ipsilateral kidney. Calculi were found in three (33%) of the renal collecting systems. Five (56%) of the SKs had hydronephrosis grade IV and one SK had recurrent pyelonephritis (11%). Two of the ureters opened into the ipsilateral seminal vesicle (22%). Two (22%) SKs were functional but atrophic. Clinically relevant findings were made in 33% of the IKs: atrophy (n = 2), calculi (n = 1), and reflux with recurrent pyelonephritis (n = 1); another 33% had anatomical anomalies without functional impairment. The correct diagnosis of a SK is possible using CT imaging in all subjects. The prevalence of SK based on CT imaging can be estimated to be 1:26750. Conclusions: CT is the method of choice for visualizing SKs. The correct diagnosis is crucial in preventing dispensable surgical procedures and for providing optimal patient treatment and outcome. Keywords: Supernumerary kidneys, Computed tomography, Renal variants, Diagnostic imaging, Duplex kidneys, Ureter fissus Background vulva [10], the prostatic urethra, or into vesical or vaginal Supernumerary kidneys (SKs) are usually one [1], rarely pouches [1] have also been described. two [2–5] additional kidneys, distinguished from the signifi- Supernumerary kidneys have no inherent clinical rele- cantly more common duplex kidneys by the fact that they vance [1]. They are ̶ however ̶ often accompanied by are not fused with the other kidney and have their own cap- urolithiasis, pyonephrosis, infections or hydronephrosis sule and blood supply [1]. Most SKs are smaller than nor- [1, 6, 11]. Individual cases have also been reported in mal kidneys [6], but they can also be larger [1, 6, 7]. SKs are which an associated adenocarcinoma [12], a Wilms usually located caudal, less frequently cranial to the ipsilat- tumor [13] or a cyst [7] were observed. Furthermore, eral kidney [1]. While there is as yet no comprehensive de- SKs can be subject to traumatic injuries [14]. scription of the blood supply of SKs because of its high Formerly, most cases of SKs were detected during variability [1], the ureters of a SK tend to join with those of surgery or autopsy [6, 15]. Diagnostic methods such as the ipsilateral kidney (IK) [1]. However, completely separate retrograde pyelography [16], urography [12, 17–19], or ureters draining into the bladder [1, 8], the vagina [9], the angiography [12, 18–20] were less likely to lead to such a finding. Today, supernumerary kidneys are more fre- * Correspondence: [email protected] † quently diagnosed using computed tomography (CT) Peter Rehder and Rafael Rehwald contributed equally to this work. m 4Department of Radiology, Medical University of Innsbruck, Anichstraße 35, [21], ultrasound, Tc-99 MAG3 scintigraphy [22], Tc- 6020 Innsbruck, Austria Full list of author information is available at the end of the article © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Rehder et al. BMC Urology (2019) 19:93 Page 2 of 8 99m DTPA scintigraphy [23] or magnetic resonance im- iodinated contrast medium during a parenchymatous aging (MRI) [24]. phase in 5 mm slice-thickness, while a urographic phase One notable reason for the increased morbidity ap- was almost always missing. All MRI examinations in pears to arise from misinterpretations in the assessment which a supernumerary kidney was identified consisted of diagnostic procedures, in which SKs are not recog- of at least a T2-weighted sequence. nized as such. There may be no consequences [25], but A significant part of the abdominal CT and MRI exami- this can at worst also lead to unnecessary interventions, nations was evaluated in the context of daily clinical rou- such as biopsies [26] or surgery [27], including the risk tine. Additionally, the radiological reports of all included of further complications [28]. modalities were systematically screened for incidental The prevalence of SKs is unknown. There are only findings of the urogenital tract, and the respectively identi- case reports of incidental findings of supernumerary kid- fied imaging material carefully assessed. In a second step, neys, with a few exceptions of reports including two [1] every detected anomaly was independently re-evaluated or three cases [29, 30]. and classified by both a senior board-certified radiologist This study represents the yet largest case series, de- (BG) and urologist (PR), resulting in nine cases of a SK scribing nine patients with supernumerary kidneys–the beeing identified based on the inclusion criteria described result of a systematic imaging-based assessment between (an organ with an asymmetric renal collecting system with 2000 and 2017. The aim of the present study was to esti- an arising ureter, own capsule and blood supply and with- mate the frequency of SKs and to precisely describe and out fusion with the other kidney). A histopathological summarize both the anatomy and the clinical features of examination of the SK was available for four cases. these patients for the first time. The renal anatomy of each identified SK has been comprehensively illustrated based on the imaging mater- ial available (Fig. 1), and additional coronal and sagittal Methods CT and MRI reconstructions are presented for some pa- A SK was defined as organ with a typically asymmetric tients in Fig. 2a-c, as these planes are considered par- collecting system to which a ureter is connected, having ticularly well suited to demonstrate supernumerary its own capsule and blood supply and not being fused kidneys [36]. with the other ipsilateral kidney, distinguishing it from Descriptive statistics were performed using IBM SPSS duplex kidneys [1]. The ureter of the SK may not open Statistics for Windows, version 21 (IBM Corp., Armonk, into the vagina in order to rule out confusion with N.Y., USA), with mean values and standard deviations Gartner’s duct cysts [31, 32], and no ureter may open given. For the statistical analysis, Fisher’s exact test was into the presumed supernumerary kidney [32, 33]. The used for categorical and the Mann–Whitney U test for SK must be connected to the lower urinary tract [32, continuous variables. The sensitivity of the modalities 34] and it must not be filled with blood or mucus at was estimated under the assumption that kidneys can be theonsetofpuberty[32, 35]. reliably diagnosed using computed tomography [37]. A total of 461,500 radiological examinations of the abdo- The prevalence was determined based on the total num- men (without duplicate examinations), performed between ber of patients examined between 2000 and 2017 with 2000 and 2017 at our institution were systematically each imaging modality, respectively. A p < 0.05 was con- reviewed for anomalies of the kidneys: 214,000 contrast- sidered to be statistically significant. enhanced CT scans acquired on spiral CT machines from different vendors (Genesis HiSpeed RP, QXi LightSpeed 4 Results Slice, LightSpeed 16, LightSpeed VCT 64, General Electric, Nine patients with a SK were identified; five men and W.I.,USA;SomatomSensation4,16and64series,Somatom four women (mean age 51.8 ± 22.8 years). The SKs were Definition Flash, Siemens AG, Erlangen, Germany), 31,500 identified on the right in six cases and on the left in MRI scans, performed on various systems (1.5 T Sonata, three cases (p = 0.132, Fisher’s exact test), caudal to the Symphony and Avanto, 3 T Magnetom Skyra, Siemens AG, IK in six cases (cases 1, 4, 5, 6, 7, and 9) and cranial in Erlangen, Germany), 208,000 abdominal ultrasound exami- three cases (cases 2, 3, and 8). The supernumerary kid- nations (HDI 5000, iU22, CX50 and EPIQ 7 ultrasound sys- neys are illustrated in Fig. 1a-I. Figure 2a-c shows sec- tems, Koninklijke Philips N.V., Best, the Netherlands; Xario tional images, as well as ostensive images of selected 200, Aplio a550 and i800 series, Canon Medical Europe B.V., cases. Zoetermeer, the Netherlands), as well as 8000 angiographies Three out of nine patients (cases 4, 6, and 9; 33%) and urographies (Integris angiography unit, Koninklijke Phi- were clinically symptomatic due to the SK (infection and lips N.V., Best, the Netherlands). pain in case 4, pain in cases 6 and 9). The other cases Most CT scans in patients with supernumerary kid- were incidental findings (66%). None of the patients had neys were acquired after the intravenous application of initially had a urological examination; in three cases, Rehder et al.
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