Hellenic Urology

CASE REPORT
Year
: 2020  |  Volume : 32  |  Issue : 2  |  Page : 97--98

Case report: Polycystic horseshoe kidney incidentally detected in a male geriatric patient – Review of the literature


Sophia Voidila1, Panagiotis Sideris2,  
1 Department of Internal Medicine of General Hospital of Aigio, Achaia; Department of Radiology, National and Kapodistrian University of Athens, Athens; Department of Radiology, Patras University, Patras, Greece
2 Department of Internal Medicine of General Hospital of Aigio, Achaia; Department of Radiology, National and Kapodistrian University of Athens, Athens, Greece

Correspondence Address:
Sophia Voidila
Stratigon 11 Str., Poulitsa Korinthias - 20006
Greece

Abstract

The case concerns a geriatric male patient who was hospitalized for gastrointestinal symptoms. Polycystic horseshoe kidney was incidentally detected during imaging. The spectrum of clinical manifestations is broad extending from asymptomatic patients (e.g., the presented case) to patients with acute deterioration demanding surgical treatment. Concomitant presence of renal polycystic disease and horseshoe anomaly is likely to bring closer the age of the presentation of renal failure, due to polycystic disease, and complicates the surgical technique for nephrectomy. Due to the rarity of the anomaly, there is lack of clinical experience, so is of clinical importance to report such cases.



How to cite this article:
Voidila S, Sideris P. Case report: Polycystic horseshoe kidney incidentally detected in a male geriatric patient – Review of the literature.Hellenic Urology 2020;32:97-98


How to cite this URL:
Voidila S, Sideris P. Case report: Polycystic horseshoe kidney incidentally detected in a male geriatric patient – Review of the literature. Hellenic Urology [serial online] 2020 [cited 2022 Dec 8 ];32:97-98
Available from: http://www.hellenicurologyjournal.com/text.asp?2020/32/2/97/301821


Full Text



 Introduction



Polycystic horseshoe kidney is a rare clinical condition. About twenty cases of polycystic horseshoe kidney have been reported in literature.[1],[2] It is thought to represent two separate forms of renal disease, without genetic association involved. Horseshoe kidney is the most common renal fusion anomaly, occurring in about 0.25% of the population.[1] Polycystic kidney disease (autosomal-dominant polycystic kidney disease [ADPKD]) is a hereditary disorder due to mutations in the genes responsible for the expression of the proteins polycystin 1 (ADPKD1) and polycystin 2 (ADPKD2).[9] Horseshoe kidney occurs in 1/400–800 live births and ADPKD in 1 in 400–1000 live births.[2] It is usually asymptomatic but can be related with renovascular hypertension, renal stones, and ureteropelvic junction syndrome.[5] Concomitant polycystic disease is extremely rare, with an incidence of 1 in 134,000 to 1 in 8,000,000 live births.[2] The concomitant presence of renal polycystic disease and horseshoe anomaly is likely to bring closer the age of the presentation of renal failure, due to polycystic disease, and complicates the surgical technique for nephrectomy.[3] Ultrasound and magnetic resonance imaging (MRI) are the appropriate diagnostic tools.

 Materials and Methods



The present case concerns of an 85-year-old male patient who reached the emergency department, with a history of vomiting for 4 days, without other concomitant symptoms. Physical examination revealed jaundice of the skin and white of the eyes, without edema. The initial clinical examination revealed blood pressure of 90/50 mmHg and pulse rate of 83 beats/min, with an average temperature. Cardiovascular and respiratory system examination had no remarkable findings. No signs of acute abdomen were detected, with a negative Murphy sign, while the right hypochondrium area was tender. The patient had a history of dementia, heart failure, coronary disease, and atrial fibrillation, receiving proper medication. The 2nd day, an increase of serum creatinine (2.2 mg/dl) and urea (137 mg/dl) levels were detected with concomitant acute renal failure. The initial abdominal ultrasound revealed the presence of polycystic kidneys, with imprecision of the lower poles, a sign indicative of horseshoe kidney. The MRI performed confirmed the condition showing a horseshoe kidney, with an isthmus of renal tissue linking the lower renal poles, and multiple renal cysts of varying sizes in the cortical as well as medullary regions [Figure 1]. No definite obstructive phenomena, lithiasis, or renal mass lesion was identified. No definite cysts were seen in the liver, pancreas, or seminal vesicles.{Figure 1}

 Results



The diagnosis of polycystic horseshoe kidney was made, which was, in fact, an incidental detection, as the abdominal pain was due to concomitant cholelithiasis, with transient secondary deterioration of renal function. The patient was given conservative therapy with proper doses of antibiotics and hydration and maintained normal renal function until today. This patient presents an exception of this rare anomaly, which is likely to bring closer the age of renal failure presentation due to polycystic disease.[7]

 Discussion/Conclusions



Polycystic horseshoe kidney is an infrequent clinical condition, which consists of a combination of two separate renal anomalies: one hereditary and one fusion anomaly. ADPKD is a hereditary disorder due to gene mutations and the third most common cause of end-stage renal disease.[3] Horseshoe kidney is the most common of all renal fusion anomalies and is often asymptomatic.[8],[4] Polycystic horseshoe kidney is, however, extremely rare, with only twenty cases reported in literature,[1] with incidence ranging from 1 in 134,000 to 1 in 8 million cases.[10] Early recognition is of great importance for prevention of renal failure due to polycystic disease. The appropriate diagnostic tools are ultrasound and MRI.[10] Coexistence of renal polycystic disease and horseshoe anomaly is likely to shorten the age of renal deterioration and failure and therefore demands closer surveillance of these patients, despite the incidental diagnosis.[3] Patients with adult polycystic kidney disease usually develop renal failure after 40 years of age.[3] The clinical presentation includes abdominal pain, headache, and hematuria. Treatment for such patients consists of individually handling hypertension and serious complications including pain, hematuria, infection, nephrolithiasis, diverticulosis, and arterial aneurysms.[9] Isthmus undivided bilateral nephrectomy has been reported in polycystic horseshoe kidney patients.[6] This case, probably consisting an exception to the rule, tells us to keep in mind that the age of renal failure for these patients differs from individual to individual, so close surveillance of renal function is needed. Formulation of guidelines for management of these cases should be based on the clinical manifestations of the disease, and this is the reason to report this case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Brumm FA, Becker M, Uglione A, Da Ros CT. Polycystic horseshoe kidney. J Urol 1997;158:229-30.
2Fekak H, Mezzour MH, Rabii R, Joual A, Bennani S, El Mrini M. Management of horseshoe kidney based on a series of 36 cases. Prog Urol 2004;14:485-8.
3Hammontree LN, Passman CM. Case report: Bilateral handassisted laparoscopic nephrectomy in a patient with polycystic horseshoe kidney. J Endourol 2006;20:397-8.
4Batista Peres LA, Leonel Ferreira JR, Alvares Delfino VD. Polycystic horseshoe kidney. Nephrol Dial Transplant 2007;22:652-3.
5Hosseni SR, Gooran S, Alizadeh F, Dadgari S. Polycystic horseshoe kidney anomaly. Appl Radiol 2009;38:38-9.
6Prasad AK, Ram R, Swarnalatha G, Shetty M, Naidu GD, Dakshinamurty KV. Polycystic horseshoe kidney. Indian J Nephrol 2013;23:387.
7Ghonge N, Jasuja S. Polycystic horseshoe kidney: Dealing with double trouble. Clin Kidney J 2014;7:413.
8Chikkannaiah P, Kangle R, Bali A, Honawad MN. Polycystic horseshoe kidney. Med J Armed Forces India 2015;71:S181-3.
9Yildiz G, Hür E, Maǧden K, Tanju Şen S, Yilmaz A, Gürkan A. Polycystic Horseshoe Kidney and polycystic liver disease. World J Surg Surgical Res 2019;2:1115.
10Batista Peres LA, Leonel Ferreira JR, Alvares Delfino VD. Polycystic horseshoe kidney. Nephrol Dial Transplant 2007;22:652-3.