Abstract
The spectrum of manifestations of duplex kidneys on 99mTc-dimercaptosuccinic acid (DMSA) renal cortical scans and correlating findings on other imaging modalities are presented. Relevant embryology of the duplex systems and technical aspects of DMSA scintigraphy are reviewed.
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Introduction
DMSA (99mTc-dimercaptosuccinic acid), a renal cortical imaging radiopharmaceutical, is commonly used in the evaluation of children with urinary tract infections. Approximately 60% of the intravenously administered dose is gradually taken up and retained by the proximal tubular cells. The remaining 40% is filtered and excreted at low concentration. Therefore, unlike in other functional imaging modalities such as intravenous urogram (IVU), CT, MRI and renal scans with MAG-3 or DTPA, direct visualization of the duplex pelvicalyceal systems is not possible on DMSA cortical scans. However, there are often changes in the renal cortex that are diagnostic or highly suggestive of duplication as demonstrated in this pictorial essay.
Types of duplication
Duplication of the ureters is the most common congenital urinary tract abnormality. It was present in 0.7% in two large autopsy studies [1–4]. It is seen more frequently in children being evaluated for urinary tract infections (8%) [1]. It is 2–4 times more common in girls compared with boys [4]. The reported incidence of bilateral duplication has varied from 17% to 33% [4].
The most widely accepted nomenclature for kidneys drained by two ureters is the one proposed by the Committee on Terminology, Nomenclature and Classification of the American Academy of Pediatrics Section on Urology. A duplex or duplicated system is defined as a kidney that has two pelvicalyceal systems. In incomplete duplication, the two ureters join before entering the bladder. A bifid pelvis is the mildest form. In complete duplication, two ureters enter the bladder separately [4, 5].
Incomplete duplication is significantly more common than complete duplication by a ratio of 3:1 [2]. Incomplete duplication can be associated with vesicoureteral reflux into both moieties, ureteropelvic junction (UPJ) obstruction of the lower moiety and rarely with ureteroureteral or yo-yo reflux [2].
Complete duplication is present in 1 in 500 individuals [2]. The two ureters have separate ureteral orifices in the bladder. The upper moiety typically consists of only a few calices or a compound calyx drained by a single infundibulum. The most common abnormalities associated with complete duplication are vesicoureteral reflux (lower moiety) and, ectopic ureterocele or ectopic ureteral insertion (upper moiety). A less common associated abnormality is UPJ obstruction of the lower moiety [2].
A ureterocele is the dilated submucosal terminal segment of the upper moiety ureter; it is 4–8 times more common in girls than boys [2]. More commonly, ureteroceles are associated with hydronephrosis and ureterectasis of the upper moiety. Rarely, the ureterocele may be large with a small dysplastic upper moiety, described as ureterocele disproportion [6, 7].
Embryology
Incomplete duplication develops from early bifurcation of a single ureteral bud before it meets the metanephric blastema at approximately 5–6 weeks [2, 4, 7]. Occasionally, one of the bifid ureters is blind ending [4, 6, 7].
Complete duplication results from two separate ureteral buds arising from the mesonephric duct. As the distal portion of the mesonephric duct is being absorbed into the developing bladder, the ureter to the lower segment of the metanephros separates earlier from the mesonephric duct and is absorbed into the bladder. The ureter to the upper part of the metanephros separates later after it has been carried medially and inferiorly by the migrating mesonephric duct and after the two ureters have crossed. This embryological relationship, wherein the upper moiety ureteral orifice is always medial and caudad to that of the lower moiety ureter, is known as the Weigert-Meyer law (Fig. 1) [4, 6]. Mackie and Stephens [8] hypothesized in 1975 that metanephric blastema is an elongated structure, only the mid-portion of which can form normal renal parenchyma. If a ureteral bud meets the more cephalad or the more caudal ends of the metanephric blastema, renal parenchymal abnormality results. The final location of the ureteral orifice correlates with the degree of renal abnormality [8].
DMSA renal cortical scintigraphy
At our center, approximately 1.5 h after intravenous injection of DMSA, a posterior image of both kidneys using high-resolution parallel hole collimator, as well as posterior and posterior oblique images of each kidney using a pinhole collimator (4-mm aperture insert) are obtained (Fig. 2a and b). The administered dose of 99mTc-DMSA is 0.05 mCi/kg (minimum 0.5 mCi and maximum 3 mCi). Imaging may be done with or without sedation depending on patient age. Differential renal function is calculated using the posterior parallel-hole image, except in cases of spinal deformities where the kidneys may be at different positions. In such cases, simultaneous anterior and posterior images are obtained and the geometric mean of the counts obtained in both projections is used to obtain the differential function. In some cases of complicated duplication, relative function of the individual moieties of the duplex kidney is also calculated. This can often guide in choosing the best surgical option (Fig. 2c and d).
Radiation considerations
The administered dose should be optimized toward answering the clinical question and at the same time decreasing the radiation risk to the patient. As per the North American consensus guidelines, the recommended administered activity of DMSA in children is 0.05 mCi/kg (1.85 MBq/kg) with a minimum of 0.5 mCi (18.5 MBq) [9]. The estimated radiation dose to the kidneys (target organ) of a 1-year-old child from 99mTc-DMSA is 0.78 mGy/MBq administered dose. Similarly, for a 5-year old, the target organ dose is 0.45 mGy/MBq [10]. The effective doses for 1- and 5-year-old children are 0.037 and 0.021 mSv/MBq, respectively [11].
These estimates are averages over a wide range of children at each age and may not take into consideration anatomical and physiological differences. A given child’s body type may vary from the standard model with respect to size, weight, shape, organ orientation and distances from other organs. Hence, the absorbed fraction and organ mass vary among children. The existing methods were developed for estimating the average dose to a population and should not be used to estimate the dose to a specific child [12].
Normal DMSA renal scan
In the absence of duplication, a normal kidney on a high-resolution DMSA scan shows tracer uptake in the renal cortex with relative central photopenia corresponding to the complex of the medullary portion of the kidney and the pelvicalyceal system. Prominent cortical columns are often seen. However, they are partial and do not traverse the full thickness of the kidney and do not cause division of the normal central photopenic region into two separate compartments (Fig. 2a and b).
Scintigraphic findings in uncomplicated duplications
In uncomplicated duplications, the renal cortical uptake is normal. Occasionally, the only clue to unilateral renal duplication is asymmetry in renal sizes; the duplex kidney appears longer than the contralateral side. The cortical bar separating the upper and lower moieties may be sometimes hard to appreciate on the parallel-hole images, but is well depicted on the pinhole images (Figs. 3 and 4). In cases of bilateral uncomplicated duplication, the renal sizes may be symmetrical, but cortical bands separating the upper and lower moieties are seen. The relative position of the moieties of duplex kidneys is usually superior and inferior, separated by a transverse cortical band (Fig. 3). Occasionally, the upper moiety is small with normal cortex, located superomedially and separated by an oblique cortical band from the larger lower moiety, which is located inferolaterally (Fig. 4).
Scintigraphic findings in complicated duplications
The scintigraphic findings in complicated duplications are dependent on the nature and severity of the associated developmental or acquired abnormalities such as dysplasia, vesicoureteral reflux, ectopic ureteral insertion, UPJ or ureterovesical junction (UVJ) obstruction, acute pyelonephritis and post-pyelonephritic scars.
The scintigraphic pattern of duplex kidneys with abnormal function of one of the moieties and normal function of the other moiety may vary, depending on the relative size of the two moieties.
A duplex system may appear as a small kidney located either at the level of the upper pole of the contralateral normal kidney, indicating a nonfunctioning lower moiety, or at the level of the lower pole of the contralateral kidney in case of a nonfunctioning upper moiety. High-resolution pinhole image may show minimal tracer uptake in the abnormal moiety (Figs. 5 and 6).
A small dysplastic upper moiety, which is usually associated with ectopic ureteral insertion or vesicoureteral reflux, may be seen as an indentation or flattening of the medial aspect of the upper pole if it is nonfunctioning (Fig. 7) or as focal decreased uptake if it functions poorly (Fig. 8). These scintigraphic findings may mimic focal cortical scar or acute pyelonephritis. The only clue to duplication is normal uptake and thickness of the cortex at the site of indentation or underneath the area of focal decreased uptake. This normal cortex underneath corresponds to the upper pole of the normal lower moiety. Ectopic insertion of the ureter of a small nonfunctioning or poorly functioning upper moiety into the vagina clinically presents as diurnal enuresis [7]. Occasionally, the abnormality on the DMSA scan of the small upper moiety with ectopic ureteral insertion is subtle such as mild cortical thinning and/or prominent central photopenia (Fig. 9). Therefore, in interpretation of DMSA scans particularly in girls with diurnal enuresis, careful attention should be made to the medial aspects of the upper poles of the kidneys. Correlation with renal sonogram carefully directed to finding a suspected duplex collecting system is often helpful (Figs. 8 and 9).
Sometimes the lower moiety may be developmentally small related to high-grade reflux (Fig. 10).
In hydronephrotic kidneys without duplication, cortical thickness of the upper and lower poles is usually similar. But a duplex kidney with hydronephrosis of the upper or lower moiety may be manifested as preserved normal cortical thickness in the lower or upper pole, respectively, with reduced cortical thickness in the affected pole (Fig. 11). In addition, the hydronephrosis will be eccentric in the kidney, related to the dilated upper or lower moiety, rather than central as is seen with a single collecting system. Hydronephrosis can be caused by reflux and/or obstruction in either of the upper and lower moieties (Figs. 11 and 12).
Conclusion
Diagnosis of duplex kidney on a DMSA scan requires a careful systematic review of the images. The findings can be subtle and it is important for the radiologist to recognize them. Correlation with clinical history and other imaging modalities such as US and voiding cystogram can be complementary.
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Kwatra, N., Shalaby-Rana, E. & Majd, M. Scintigraphic features of duplex kidneys on DMSA renal cortical scans. Pediatr Radiol 43, 1204–1212 (2013). https://doi.org/10.1007/s00247-013-2619-z
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DOI: https://doi.org/10.1007/s00247-013-2619-z