Original Paper

Urologia
Internationalis

Urol Int 2007;78:214218 DOI: 10.1159/000099340

Received: November 14, 2005 Accepted after revision: September 26, 2006

Sixteen Years of Experience with Stone Management in Horseshoe Kidneys

Domenico Viola Theodore Anagnostou Trevor J. Thompson Gordon Smith Sami A. Moussa David A. Tolley
The Scottish Lithotriptor Centre, Western General Hospital, Edinburgh, UK

Key Words Horseshoe kidney Urolithiasis Extracorporeal shock wave lithotripsy Percutaneous nephrolithotomy

Introduction

Abstract Introduction: Horseshoe kidney is the commonest congenital renal fusion anomaly, and is often complicated by urolithiasis. We focus on our 16 years of experience with stone management in horseshoe kidneys. Materials and Methods: We reviewed the progress of 44 patients treated between 1987 and 2002. Shock wave lithotripsy (SWL) was used in 25 patients; the average stone surface area was 91 (range 101,600) mm2 and average follow-up was 36.5 (range 191) months. 19 patients underwent percutaneous nephrolithotomy (PCNL); the average stone surface area was 197 (range 62,400) mm2. Follow-up data are available for 8 patients and the average follow-up was 42.3 (range 3144) months. Results: In the SWL group the 3-month stone-free rate (SFR) was only 31%. In the PCNL group the SFR was 75% on the postoperative day-1 KUB. Complications occurred in 9 patients. Conclusions: Stone management in horseshoe kidneys is challenging: PCNL produces a higher SFR with minimal major complications and failed access. PCNL thus appears to be the preferred management option in patients with urolithiasis in horseshoe kidneys. Copyright 2007 S. Karger AG, Basel

Horseshoe kidney is the commonest fusion anomaly in the development of the kidney, with a reported incidence of between 1/400 and 1/1,000 births, and a slight male predominance [1]. It was first extensively described by Botallo in 1546, after being recognized at autopsy by Decarpi and its complications were studied and delineated by Morgagni in 1761 [1, 2]. This anomaly is the consequence of abnormal migration and fusion across the midline of the metanephric blastema during weeks 46 of gestation. The two kidneys are generally fused at the lower pole by an isthmus and the presence of the inferior mesenteric artery hampers their ascent, which in turn prevents the normal rotation of the kidneys. As a result the renal pelvis lies anteriorly and the caliceal system lies dorsolaterally and dorsomedially. The insertion of the ureter on the renal pelvis is displaced superiorly and laterally, and the ureter must pass anteriorly over the isthmus before descending to the bladder. These factors are thought to determine impaired drainage of the collecting system, stasis, the higher rate of infection and a predisposition to stone formation [3]. Urolithiasis is the commonest complication of horseshoe kidney, with a reported incidence between 20 and 60% [3]. The main therapeutic options available are shock

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Table 1. Characteristics of patients

SWL Number of patients Mean age, years (range) Average stone surface area, mm2 (range) Single vs. multiple stones Staghorn calculi 25 48 (1071) 91 (101,600) 11 vs. 18

PCNL 19 52.5 (383) 197 (62,400) 14 vs. 5 1

Total 44 50 (383)

wave lithotripsy (SWL) and percutaneous nephrolithotomy (PCNL). There are less than 10 reports on the efficacy and safety of PCNL in horseshoe kidneys in the literature [410]. In this article we present our experience with 44 horseshoe kidney patients, treated either with PCNL, SWL or both, at our institution over a period of 16 years, with special emphasis on stone-free rates (SFRs) and complications.

Materials and Methods

The data of all the patients prospectively entered into our stone database were analyzed and the records of 44 patients treated for stones in 49 horseshoe kidneys between 1987 and 2002 were retrospectively reviewed. The average age was 50 (range 383) years and 81% were male. Presenting symptoms were hematuria, recurrent urinary tract infections, and loin pain. The stones were equally located on the right and left side. All patients underwent a standard assessment with medical history, physical examination, urine culture, renal function tests and excretory urography (IVP). Patients characteristics are summarized in table 1. 25 patients for 29 kidneys underwent SWL: there were 18 single stones and 11 multiple stones, and the average stone surface area was 91 (101,600) mm2. Three different machines have been used throughout the 16 years: the spark-gap generator Dornier MPL 9000, the piezoceramic generator Wolf Piezolith 2300 and, more recently, the electromagnetic generator Dornier Compact Delta. Patients underwent an average of 2.9 (range 113) sessions, performed on an outpatient basis, with no anesthesia. All were treated in the supine position. A successful treatment outcome was defined as a patient being stone-free at the 3-month follow-up. PCNL was used to treat 19 patients, 12 as a primary indication because of a large stone burden, 7 after SWL failure including obesity and inability to focus on the kidney. There were 14 multiple stones, 5 single stones and 1 staghorn calculus. The average stone surface area was 197 (range 62,400) mm2. Previous open stone renal surgery was performed in 2 patients; previous unsuccessful SWL sessions in 7 patients; stent insertion in 2 patients with concomitant UPJ obstruction, and 1 patient had laparoscopically assisted heminephrectomy. We performed 20 PCNL procedures under fluoroscopic guidance, general anesthesia, intravenous antibiotic cover, and prior

placement of a retrograde ureteral catheter. Percutaneous access was established by our interventional radiologist, taking into account the modification needed by ectopia, malrotation, and vascular anomalies typical of horseshoe kidneys [11]. As the horseshoe kidney lies lower in the abdomen, usually a more vertical, lower puncture is required and the upper pole approach may actually be facilitated with the upper pole lying below the costal margin. Sometimes a lower pole entry might be hampered because of the shield offered by pelvic bones. Malrotation of the kidney can make accurate localization of the stone-bearing calices more difficult because the calices are often superimposed on anteroposterior views, and the use of the C-arm and antegrade pyelography may be useful in this regard. The vascular supply of the horseshoe kidney is very variable, but usually all blood vessels, except the ones supplying the isthmus, enter the kidney at its anteromedial aspect, making the risk of arterial bleeding not higher than in a normal kidney. Tract dilation was accomplished with metallic concentric dilators to 30 french, and a 26-french Amplatz sheath was then inserted and the rigid nephroscope used to inspect the collecting system. We did not use an elongated sheath, but when the length of the tract involved was a problem, we would push the sheath beyond the skin margin, after having secured it to the skin with a stitch, so that we could pull it back at the end of the procedure. Ultrasound energy was used in all cases, with the addition of Holmium:YAG laser energy in 1 case. At the end of the procedure a 10.2-french cope nephrostomy was left in all the patients which was usually clamped and removed after 2 days. A kidney, urethra and bladder (KUB) scan was performed on postoperative day 1 to assess residual stone fragments, whereas neither nephrostogram nor chest X-ray were performed on a regular basis. The SFR, need for second intervention, and complications were then assessed. The SFR was defined as the absence of any fragment on the postoperative day-1 KUB. Complications were defined as major if they required additional intervention or prolonged hospitalization, or minor if they could be managed conservatively. Patients were asked to return for follow-up if they lived within acceptable traveling distance of our center or were referred back to the referring travelling physician. The average follow-up was 36.5 (range 191) months in the SWL group, whereas follow-up data are available for 8 patients in the PCNL group and the average follow-up was 42.3 (range 3144) months.

Stone Management in Horseshoe Kidneys

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Multiple tracts (15%)

Upper and middle pole posterior calices (15%)

Single tract (85%)

Upper pole posterior calix (35%)

Middle pole posterior calix (35%)

Lower pole posterior calix (15%)

Fig. 1. Percutaneous access to horseshoe kidney.

Table 2. Results

Results
SWL PCNL 75

Stone free rate, %

31

Table 3. Percutaneous access to horseshoe

kidneys % Single tract Upper pole posterior calix Middle pole posterior calix Lower pole posterior calix Multiple tracts Upper and middle pole calices 85 35 35 15 15 15

In the SWL group the fragmentation rate was 90%, with an average of 3,221 (range 1,4303,975) shock waves at 18.9 (range 1722) kV delivered per session. The 3-month SFR was 31% (table 2). In a subset of 18 patients with a small stone burden (^13 mm), the 3month SFR was 40%. At 12 months the SFR increased to 40% and at 36 months to 50%; 7 patients with unsuccessful SWL subsequently underwent PCNL. No complications were reported. Percutaneous access to the horseshoe kidney was obtained via a single tract through the upper pole posterior calix in 7 patients (35%), the middle pole posterior calix in 7 patients (35%), and the lower pole posterior calix in 3 patients (15%). Three patients (15%) required two tracts involving the upper and middle calices (table 3; fig. 1). Percutaneous access did not appear to be a problem in our patients, although 3 patients required 2 punctures to
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Table 4. Complications

SWL n Major Septicemia Pyelovascular communication Minor Prolonged spell with nephrostomy Moderate blood loss 0 (0%) 0 (0%)

PCNL n 2 (10%) 1 (5%) 1 (5%) 7 (37%) 2 (10%) 5 (27%)

In the 7 patients operated on after previous SWL failure, the average stone burden was 20 (range 831) mm2, and there were 4 multiple stones and 2 single stones. Six patients were rendered stone free after one PCNL, whereas the 7th patient required a second percutaneous procedure. Stone composition was available in 32 patients and revealed calcium oxalate in 10, calcium phosphate in 6, uric acid in 5, and mixed stone composition in 11 patients.

Discussion

clear the stones. Perioperative problems were encountered in a 3-year-old boy in whom, after initial stone clearance, while searching for a residual 4-mm fragment, it became evident that antegrade contrast material was readily outlining the renal vein and inferior vena cava. The procedure was terminated without further incident or need for intervention, other than a short percutaneous procedure to retrieve the fragment when the tract was mature. The SFR, defined as the absence of any fragment on the postoperative day-1 KUB, was 75% (table 2). Among the 5 patients with residual fragments were a patient with staghorn calculus and a patient with the biggest stone burden of 2,400 mm2. The first patient was treated with a second percutaneous procedure and SWL before becoming stone free. The second underwent another percutaneous procedure under CT guidance after a previous attempt to perform the puncture had been unsuccessful; this patient had a 3-mm residual fragment and was managed conservatively. The third patient became stone free after a second percutaneous procedure and the fourth with SWL. The fifth patient was lost to follow-up. The overall SFR was then 18/20 (90%). The overall complication rate was 47% (9 patients); 2 (10%) were major complications, namely a pyelovascular communication between the pelvis and the renal vein, managed with the immediate suspension of the procedure and the positioning of a nephrostomy, which was kept in place for 2 weeks. A subsequent nephrostogram showed the healing of the fistula, and one septicemia, successfully managed with intravenous antibiotic therapy. The minor complications were 5 cases (27%) of moderate blood loss which did not require transfusion, while 2 patients (10%) had a prolonged spell with nephrostomy (3 weeks) because of poor drainage on clamping, but subsequent nephrostogram showed adequate drainage (table 4).
Stone Management in Horseshoe Kidneys

Urolithiasis is the most common complication of horseshoe kidney. Minimally invasive surgery, such as PCNL, and minimally invasive procedures, such as SWL, play a pivotal role in the treatment of stones associated with a horseshoe kidney, while the role of open surgery is considered obsolete. Many authors believe that SWL is the first-choice treatment for small calculi in a horseshoe kidney [7, 12]. However, this is not necessarily the case: disappointing results, with SFR as low as 50 [13] and 60% [14] have been reported, and our series confirms these data, particularly when small multiple stones are involved. These poor results might be explained in part by imaging difficulties, interference of the spine in optimal visualization, although the multiplicity of the stones and poor drainage may impair the spontaneous passage of fragmented stones [6, 13]. Percutaneous surgery is established as an effective and safe treatment modality [410]. The first attempt to achieve percutaneous access to a horseshoe kidney was reported by Fletcher and Kettewell [15] in 1973; since then, PCNL in horseshoe kidneys has been considered the standard of care for stones of 12 cm, or for stones in which SWL has failed. To the best of our knowledge, this study represents the largest single institution series of PCNL in patients with horseshoe kidneys [410]. Our success rate of 75% after one session of PCNL, and up to 90% with a second percutaneous procedure with or without SWL, or with SWL alone, compares well with the results of other series of PCNL in normal kidneys [1618], as well as with other series of PCNL in horseshoe kidneys [410]. Percutaneous access and imaging of stones in our patients has not been a particular problem due to the modifications of techniques used [11] and the presence of a dedicated and experienced uroradiologist. We do not feel the need to perform routine preoperative abdominal CT
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as suggested by Al Otabi and Hosking [4]; we used CTguided tube placement in 1 patient after a conventional fluoroscopy attempt to puncture the kidney had been unsuccessful. Nevertheless, there is no doubt that a preoperative abdominal CT can be very useful in less experienced hands. The lower abdominal position of the horseshoe kidney often necessitates upper or middle pole puncture, performed in 85% of our patients: these are easier and safer than in a normal kidney, as a supracostal puncture is rarely needed. As a result, no case of violation of the pleural cavity was recorded. Moreover, in the horseshoe kidney, most of the calices point either dorsomedially or dorsolaterally and this orientation of the collecting system offers surprisingly good access for PCNL; the calices pointing dorsally are entered by direct puncture, whereas the ones in the isthmus are entered through the pelvis [19]. No major bleeding necessitating transfusion was encountered in our series. The blood supply of horseshoe kidneys, although quite variable, tends to follow an ordered patterns, inasmuch as all blood vessels, except some to the isthmus, enter the kidney from its ventromedial aspect. Furthermore, the dorsal arteries to the isthmus are usually protected by the spine and situated far away

from the nephrostomy tract; the risk of arterial bleeding is therefore not higher than in a normal kidney [19]. In two studies [4, 5], the use of flexible nephroscopy to achieve better stone clearance for caliceal stones has been stressed. We did not routinely use flexible nephroscopy and our success rate and need for a second look procedure were comparable to those in other series. Nonetheless, we recognize the usefulness of this approach, provided the flexible nephroscope is available together with an appropriate fragmentation device.

Conclusions

Stone management in patients with horseshoe kidneys is a challenging procedure. SWL is less effective in the majority of patients due to imaging, focusing, or drainage problems and multiplicity of the stones. PCNL, on the other hand, produces higher SFRs with minimal major complications and failed access. The results of the present study suggest that PCNL is a safe and effective procedure, has a higher success rate than SWL and is therefore the preferred management option in patients with urolithiasis in horseshoe kidneys.

References
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