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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 12  |  Issue : 1  |  Page : 34-36

Simultaneous bilateral nephrectomy of hugely enlarged polycystic kidneys


Department of Urology, Dubai Hospital, Dubai, United Arab Emirates

Date of Web Publication27-Feb-2019

Correspondence Address:
Fariborz Bagheri
Department of Urology, Dubai Hospital, Dubai
United Arab Emirates
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HMJ.HMJ_36_18

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  Abstract 


This case is the first of its kind of simultaneous bilateral nephrectomy of the largest size of autosomal dominant polycystic kidneys in the United Arab Emirates. A 57-year-old man referred to our department with abdominal pain and distension, difficulty in walking and breathing, and edema of both legs caused by hugely enlarged bilateral polycystic kidneys. He was on hemodialysis for the past 3 years. Any type of operation was refused by him. In October 2016, he was admitted to the hospital due to deterioration of his general condition and intolerable quality of life caused by severe compression to the abdominal organs. After obtaining written consent, simultaneous open bilateral nephrectomy was done successfully. Mass of both kidneys removed was 13,050 g which is, to our knowledge, the largest polycystic kidneys removed simultaneously so far. Early bilateral nephrectomy is advisable in patients with end-stage renal failure with the tendency of progressive enlargement of the kidney size.

Keywords: Kidney diseases, nephrectomy, renal failure


How to cite this article:
Bagheri F, Kumar S, Esmaeilpour H, Hassan M, Pytel A. Simultaneous bilateral nephrectomy of hugely enlarged polycystic kidneys. Hamdan Med J 2019;12:34-6

How to cite this URL:
Bagheri F, Kumar S, Esmaeilpour H, Hassan M, Pytel A. Simultaneous bilateral nephrectomy of hugely enlarged polycystic kidneys. Hamdan Med J [serial online] 2019 [cited 2019 May 20];12:34-6. Available from: http://www.hamdanjournal.org/text.asp?2019/12/1/34/238565




  Introduction Top


Polycystic kidney disease (PKD) is the most common cause of renal failure in adults and children. PKD is characterized by progressive cystic dilatation of the renal tubules. Cyst growth displaces and destroys normal kidney tissues, resulting in fibrosis, renal architectural derangement, and ultimately kidney failure.[1],[2] PKD can be inherited as either an autosomal dominant trait (ADPKD) or autosomal recessive trait (ARPKD). The kidney appears to be composed solely of a cluster of cysts, up to 3–4 cm in diameter, with no intervening parenchyma. Kidneys are enlarged and the numerous small cysts in the cortex and medulla give the kidney a sponge-like appearance. The disease is invariably bilateral.

ADPKD occurs in 1 in 400–1000 live births.[3] PKD presents with high blood pressure, back pain, or flank pain; increase in size of the abdomen; hematuria; and frequent urinary tract infection or occasionally may be asymptomatic.

Total kidney volume associates with the development of end-stage renal disease and significantly affects patients' quality of life.[4] Women have smaller kidneys and develop renal failure later than men.

Kidneys weighing up to 7–10 kg have also been reported, which is rare. We present our case because of its rarity and it is the first case in Dubai (the United Arab Emirates) with such huge enlargement of both kidneys that underwent simultaneous bilateral nephrectomy. Very few such cases have been published in the literature worldwide. The rate of enlargement of the kidneys and the accompanying hypertension in ADPKD patients are of crucial importance for the fatal outcome. Hence, timely intervention by doing nephrectomy also plays an important role in decreasing the morbidity in ADPKD.

In this report, we present a patient with end-stage renal failure due to extraordinarily enlarged bilateral polycystic kidneys who refused any surgical intervention until he developed extremely distended abdomen and suffered with significantly deteriorated quality of life.


  Case Report Top


A 57-year-old male, weighing 70 kg, was referred to our hospital in October 2016 with abdominal pain and distension, associated with difficulty in breathing, inability of walking without aids, and edema of lower extremities. He was a known case of ADPKD with positive family history and recent history of hypertension. Due to progressive enlargement of bilateral polycystic kidneys and consequently end-stage renal failure, he was on hemodialysis since 2014. Previously, bilateral nephrectomy was recommended several times, but he refused any surgical intervention due to its potential complications. In October 2016, he accepted admission and further management due to intolerable quality of life and incision of his family members.

On physical examination, an extremely enlarged and tense abdomen was seen resembling that of a pregnant woman at term. He had difficulty in breathing and could not walk without aid.

We evaluated him with blood and radiological investigations and prepared him for simultaneous bilateral open nephrectomy. Computed tomography scan confirmed bilateral enlarged kidneys with multiple cystic lesions of varying sizes compressing and dislocating the surrounding organs [Figure 1]. On October 16, 2016, bilateral nephrectomy was performed through a bilateral subcostal chevron incision. The operation time was 215 min and the blood loss was about 300 ml. There was no need for any blood transfusion. The only intraoperative complication was a tear in the angle of the right renal vein and the inferior vena cava. This was controlled by Satinsky clamps and secured by Prolene sutures. Both kidneys could be easily separated intact from the surrounding organs without aspiration or any destruction of the cysts. The left kidney was 31 cm × 18 cm in diameter and weighed 6100 g [Figure 2]. The right kidney was 34 cm × 17 cm in diameter and weighed 6950 g [Figure 3]. The postoperative period was uneventful and he was discharged in good general condition on the 7th postoperative day. The histopathology result confirmed ADPKD. About 1 month after the operation, the patient was very happy with his quality of life and he was able to perform all daily activities alone. He is planned for kidney transplantation.
Figure 1: Computed tomography scan confirmed bilateral enlarged kidneys with multiple cystic lesions of varying sizes compressing and dislocating the surrounding organs

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Figure 2: The left kidney was 31 cm × 18 cm in diameter and weighed 6100 g

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Figure 3: The right kidney was 34 cm × 17 cm in diameter and weighed 6950 g

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  Discussion Top


The normal weight of an adult man's kidney is about 150 g. The average volume of polycystic kidneys in autopsied specimen is from 500 to 650 ml. Rapid enlargement of the polycystic kidneys can be observed in very rare cases. The exact cause is still not clear. In the case under discussion, the weight of each kidney had increased > 40 times and weight of both kidneys accounted for 18.5% of the patient's total weight. We cannot predict what exactly initiated the enlargement of the kidneys during the last 3 years of the patient's life, but it is hardly determined by genetic factors, as none of his other family members with ADPKD showed such a tendency. The conventional therapy with hemodialysis which the patient received also cannot be assumed as a direct relation to the increase in size of the kidneys. The size of the cysts and the development of hypertension are bad prognostic signs in adult PKD. Moreover, the large size of the kidneys mechanically displaces the lungs causing dyspnea and compressing the great vessels leading to edema of the legs. Early bilateral nephrectomy is indicated for patients with large kidneys causing pressure symptoms, pain, infection, bleeding, hypertension, erythrocytosis, and suspicion of malignancy. Nephrectomy is also indicated in these patients to create space for renal allograft. Nephrectomy in ADPKD can be done either by open surgery or laparoscopically. We proceeded with open surgery by bilateral subcostal chevron incision due to the huge size of the kidneys. It is worth reporting our case as a unique case of hugely enlarged polycystic kidneys of >13 kg. After bilateral nephrectomy, our patient has a satisfactory quality of life and is undergoing hemodialysis three times a week and waiting for renal transplant.


  Conclusion Top


In patients with end-stage renal failure due to PKD with progressive enlargement of the kidney size, early bilateral nephrectomy is advisable in order to avoid compressive consequences to the adjacent organs and decrease the potential risks of surgical complications.

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.
Belibi FA, Reif G, Wallace DP, Yamaguchi T, Olsen L, Li H, et al. Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells. Kidney Int 2004;66:964-73.  Back to cited text no. 1
    
2.
Torres VE, Wang X, Qian Q, Somlo S, Harris PC, Gattone VH 2nd, et al. Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Nat Med 2004;10:363-4.  Back to cited text no. 2
    
3.
Dalgaard OZ. Bilateral polycystic disease of the kidneys; a follow-up of two hundred and eighty-four patients and their families. Acta Med Scand Suppl 1957;328:1-255.  Back to cited text no. 3
    
4.
Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: The major factor determining clinical outcomes. Clin J Am Soc Nephrol 2006;1:148-57.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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Abstract
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Case Report
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