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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 16  |  Issue : 1  |  Page : 192-195

A rare presentation in horseshoe kidney


1 Department of Urosurgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India
2 Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India

Date of Submission07-Oct-2020
Date of Decision26-Jan-2021
Date of Acceptance18-Feb-2021
Date of Web Publication29-Jul-2021

Correspondence Address:
Dr. Shyamal Shah
Flat No. 3, Karmabhumi Apartment, Malpur Road, Modasa, Arravalli - 383 315, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_349_20

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  Abstract 


Horseshoe kidney is the most common congenital fusion anomaly which occurs in 1 in 400–800 live births. Pelvi uretric junction obstruction is the most common abnormality associated with horseshoe kidney. Bilateral PUJ obstruction with renal stones with obstructive uropathy with one nonfunctional moiety is a rare occurrence. We here report such a case, and to the best of our knowledge, there is no such case reported in the literature. We managed the case by initial stabilization followed by left pyeloplasty with pyelolithotomy and partial isthmusectomy. In all cases of horseshoe kidney with renal calculi, there should be high index of suspicion for PUJ obstruction. Thoughtful management in such cases results in prompt recovery and decreases the morbidity of patient

Keywords: Bilateral ureteropelvic junction obstruction, horseshoe kidney, open pyelolithotomy, pyeloplasty with isthmusectomy, renal calculus


How to cite this article:
Guru N, Shah S. A rare presentation in horseshoe kidney. J Datta Meghe Inst Med Sci Univ 2021;16:192-5

How to cite this URL:
Guru N, Shah S. A rare presentation in horseshoe kidney. J Datta Meghe Inst Med Sci Univ [serial online] 2021 [cited 2021 Sep 23];16:192-5. Available from: http://www.journaldmims.com/text.asp?2021/16/1/192/322631




  Introduction Top


Horseshoe kidney is the most common congenital fusion anomaly occurring in about 1 in 400–800 live births.[1] Horseshoe kidney embryologically occurs due to fusion of renal poles, usually inferior poles, and failure to ascent above the inferior mesenteric artery. Most of the patients with horseshoe kidney are asymptomatic. The patients may present to the treating clinician with symptoms of abdominal pain, fever, vomiting due to infection, sepsis, or calculus. These symptoms arise owing to obstruction at the level of ureteropelvic junction (PUJ), which may occur due to congenital stricture, high ureter insertion, abnormal ureter coursing over the isthmus, crossing vessels supplying the isthmus, and abnormal moiety of ureter.[2] The incidence of PUJ obstruction in horseshoe kidney is 15%–33% with an incidence of urolithiasis being 20%–60%.[3]

Computed tomography (CT) scan and magnetic resonance imaging (MRI) are diagnostic modalities of choice for the evaluation of renal anatomy, vasculature, and surrounding structures in a case of horseshoe kidney.[4],[5]

PUJ obstruction is the most common abnormality associated with horseshoe kidney. Bilateral PUJ obstruction with renal stones with obstructive uropathy with one nonfunctional moiety is a rare occurrence. We here report such a case, and to the best of our knowledge, there is no such case reported in the literature.

Management of such patients requires stabilization of patient with antibiotics and relieving obstruction by diversion. The definitive management includes pyeloplasty with stone removal and isthmusectomy and nephropexy of the ipsilateral kidney.[6] Endopyelotomy is associated with inferior outcome.[7] Pyeloplasty can be done by open method, laparoscopically,[8],[9],[10] or robotic[8],[9],[10] method. There are contradictory reports for isthmusectomy with recent literature advocating against the practice of isthmusectomy, due to risk of infection, fistula, leakage, and bleeding.[11] Hence, nowadays, isthmusectomy is rarely done and done only if additional procedure is required for correction of associated anomaly.


  Case Report Top


A 21-year-old male patient presented in casualty with complaints of fever and pain in lower abdomen for the past 3 days. On examination, the patient was pale and febrile with tachycardia, perurethral catheter was present with tubing containing pus flakes, and marked tenderness was present over lower abdomen and around umbilicus. The patient was admitted and immediately started on broad-spectrum antibiotics and investigations sent. Investigations revealed anemia with Hb: 8.5 g/dl, total leukocyte count (TLC): 16,700/mm3, and serum creatinine: 2.4 mg/dl. Urine routine microscopy was suggestive of infection. Urine culture was sent and antibiotics started as per sensitivity. Ultrasonography of the abdomen revealed bilateral pelvic ectopic kidneys with bilateral multiple renal calculi and gross hydronephrosis and cortical thinning on the right side; however, isthmus could not be demonstrated. Noncontrast computed tomography (NCCT kidney-ureter-bladder [KUB]) revealed bilateral pelvic ectopic kidney with bilateral hydronephrosis with multiple renal calculi [Figure 1]; with informed consent, bilateral double-J (DJ) stenting was done [Figure 2]. Postoperative hospital stay was uneventful. In the follow-up, the patient was afebrile, and investigations revealed TLC: 4000/mm3 and serum creatinine: 1.3 mg/dl; X-ray KUB showed multiple residual calculi with bilateral DJ stent in situ [Figure 2]. Bilateral DJ stents were removed and CT urography was planned to assess the renal function and contrast excretion. CT [Figure 3] revealed horseshoe kidney with bilateral gross hydronephrosis and bilateral multiple renal calculi, with good contrast uptake and delayed excretion in the left kidney and minimal contrast uptake and no excretion in the right kidney on delayed films, suggestive of bilateral pelviureteric junction obstruction and minimally or nonfunctional right kidney. To confirm the findings, to get a baseline investigation, and to assist the surgical decision-making, the patient was subjected to diethylene triamine pentaacetic acid (DTPA) [Figure 4] which revealed total GFR: 57.2 ml/min, with split renal function - right kidney: 7.4% and left kidney: 92.6%, and insignificant tracer excretion on the right side and delayed tracer transit on the left side (t1/2 >20 min).
Figure 1: Noncontrast computed tomography kidney-ureter-bladder coronal section showing bilateral ectopic kidneys (horseshoe kidney) with hydronephrosis and left renal calculi

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Figure 2: X-ray kidney-ureter-bladder suggestive of bilateral double-J stent in situ and multiple calculi

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Figure 3: Computed tomography urography coronal section showing good uptake of contrast and sluggish excretion on the left side while poor uptake and no excretion on the right side

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Figure 4: Diethylene triamine pentaacetic acid scan suggestive of poorly functioning ectopic right kidney and ectopic left kidney showing moderate parenchymal dysfunction and very sluggish drainage

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After analyzing all investigations, and with informed consent, left-sided Anderson's Hynes pyeloplasty with stone removal with partial isthmusectomy was done [Figure 5].However right sided nephrectomy was also planned during the above procedure but the patient refused to give consent .Hence to avoid adverse consequences stenting of the right kidney was done along with left sided surgery [Figure 5]. The patient tolerated the perioperative and postoperative period well and discharged. During follow-up, the patient was asymptomatic and serum creatinine was 0.2 mg/dl. In spite of insisting, the patient denied nephrectomy and got bilateral DJ stents removed [Figure 6].
Figure 5: Intraoperative picture

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Figure 6: X-ray kidney-ureter-bladder suggestive of bilateral double-J stent in situ and complete clearance of left renal calculi

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  Discussion Top


Horseshoe kidney is the most commonly encountered renal anomaly in clinical practice. The management of horseshoe kidney causing symptoms differs from case to case. PUJ obstruction is by far the most common abnormality associated with horseshoe kidney, leading to hydronephrosis, calculi, and infection.

PUJ obstruction occurring unilaterally is common, but bilateral occurrence is rare. Shadpour et al. in their study reported 15 patients with unilateral PUJ obstruction undergoing pyeloplasty of which 12 had involvement of left side.[12] Our case had bilateral PUJ obstruction with renal calculi with pyonephrosis, septicemia, and one nonfunctional moiety; this combination is rarely found. Singhania et al. reported a similar case of bilateral PUJ obstruction with bilateral renal calculi.[13] However, both moieties were functional.

Initial diagnosis is indicated by ultrasound. Empty renal fossa bilaterally, ectopic inferior and medial location of kidneys with the demonstration of isthmus joining the two moieties, and hydronephrosis are signs indicative of horseshoe kidney with PUJ obstruction on ultrasound.[14] CT scan and MRI scan are the best investigations for demonstrating renal anatomy, vasculature, and surrounding structures in case of horseshoe kidney.[4],[5] Singhania et al. in their case did intravenous pyelogram for establishing the diagnosis.[13] In our case, we initially did NCCT KUB because of raised creatinine followed by CT urography after diversion. DTPA scan is done as a baseline scan to determine the functional status of kidneys and to establish the obstruction to excretion. In our case, DTPA showed bilateral PUJ obstruction with right nonfunctional kidney.

Conventional management of horseshoe kidney with PUJ obstruction was open dismembered pyeloplasty with isthmusectomy and nephropexy of the ipsilateral side.[6] However, recent reports suggest that isthmusectomy and nephropexy are not required. Singhania et al. in their case did open pyeloplasty with pyelolithotomy on both sides followed by percutaneous nephrolithotomy on the one side and ureteroscopic lithotripsy on the other for residual calculus.[13] In our case, we did left pyeloplasty with pyelolithotomy and partial isthmusectomy. Since the cause of PUJ obstruction in our case was apparently abnormal ureteric course over the isthmus, we did partial isthmusectomy following pyeloplasty.[15],[16],[17],[18],[19]


  Conclusion Top


Bilateral PUJ obstruction in case of horseshoe kidney with renal calculi causing septicemia and one nonfunctional kidney is a rare occurrence. In all cases of horseshoe kidney with renal calculi, there should be high index of suspicion for PUJ obstruction. Thoughtful management in such cases results in prompt recovery and decreases the morbidity of patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kaufman E. Textbook of Special Pathological Anatomy. Vol. 2. Berlin; de Gryter; 1957. p. 427-36.  Back to cited text no. 1
    
2.
Lallas CD, Pak RW, Pagnani C, Hubosky SG, Yanke BV, Keeley FX, et al. The minimally invasive management of ureteropelvic junction obstruction in horseshoe kidneys. World J Urol 2011;29:91-5.  Back to cited text no. 2
    
3.
Viola D, Anagnostou T, Thompson TJ, Smith G, Moussa SA, Tolley DA. Sixteen years of experience with stone management in horseshoe kidneys. Urol Int 2007;78:214-8.  Back to cited text no. 3
    
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O'Brien J, Buckley O, Doody O, Ward E, Persaud T, Torreggiani W. Imaging of horseshoe kidneys and their complications. J Med Imaging Radiat Oncol 2008;52:216-26.  Back to cited text no. 4
    
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Lee CT, Hilton S, Russo P. Renal mass within a horseshoe kidney: Preoperative evaluation with three-dimensional helical computed tomography. Urology 2001;57:168.  Back to cited text no. 5
    
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7.
Pe ML, Sterious SN, Liu JB, Lallas CD. Robotic dismembered pyeloplasty in a horseshoe kidney after failed endopyelotomy. JSLS 2008;12:210-2.  Back to cited text no. 7
    
8.
Wang P, Xia D, Ma Q, Wang S. Retroperitoneal laparoscopic management of ureteropelvic junction obstruction in patients with horseshoe kidney. Urology 2014;84:1351-4.  Back to cited text no. 8
    
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Blanc T, Koulouris E, Botto N, Paye-Jaouen A, El-Ghoneimi A. Laparoscopic pyeloplasty in children with horseshoe kidney. J Urol 2014;191:1097-103.  Back to cited text no. 9
    
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12.
Shadpour P, Akhyari HH, Maghsoudi R, Etemadian M. Management of ureteropelvic junction obstruction in horseshoe kidneys by an assortment of laparoscopic options. Can Urol Assoc J 2015;9:E775-9.  Back to cited text no. 12
    
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Singhania PP, Raut NR, Shringarpure SS, Tiwari N, Sathe S. Horseshoe kidney with bilateral ureteropelvic junction obstruction with multiple renal calculi: A case report. Int J Sci Stud 2015;3:233-5.  Back to cited text no. 13
    
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Banerjee B, Brett I. Ultrasound diagnosis of horseshoe kidney. Br J Radiol 1991;64:898-900.  Back to cited text no. 14
    
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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