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Bagheri, Kumar, Esmaeilpour, Hassan, and Pytel: Simultaneous bilateral nephrectomy of hugely enlarged polycystic kidneys – a case report

Introduction

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 to 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 1 in 400–1000 live births.3 Polycystic kidney disease presents with high blood pressure, back pain or flank pain, increase in size of the abdomen, haematuria 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 (UAE) 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 the 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

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 oedema 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 haemodialysis 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 insists 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. CT scan confirmed bilateral enlarged kidneys with multiple cystic lesions of varying sizes compressing and dislocating the surrounding organs (Figure 1). On 16 October 2016, bilateral nephrectomy was performed through a bilateral subcostal chevron incision. The operation time was 215 minutes and the blood loss was about 300 ml. There was no need for any blood transfusion. The only intra-operative 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 × 18 cm in diameter and weighed 6 100 g (Figure 2). The right kidney was 34 × 17 cm in diameter and weighed 6,950 grams (Figure 3). Post-operative period was uneventful and he was discharged in good general condition on the seventh post-operative 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 activity alone. He is planned for kidney transplantation.

FIGURE 1
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FIGURE 2
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FIGURE 3
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Discussion

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 ml 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 more than 40 times and weight of both kidneys accounted for 18.5% of the patient 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 haemodialysis 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 polycystic kidney disease. Moreover, the large size of the kidneys mechanically displaces the lungs causing dyspnoea and compressing the great vessels leading to oedema 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 more than 13 kg. After bilateral nephrectomy, our patient has a satisfactory quality of life and is undergoing haemodialysis three times a week and waiting for renal transplant.

Conclusion

In patients with end-stage renal failure due to polycystic kidney disease 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.

References

1. 

Belibi FA, Reif G, Wallace DP, et al. Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells. Kidney Int 2004; 66:964–73. https://doi.org/10.1111/j.1523-1755.2004.00843.x

2. 

Torres VE, Wang X, Qian Q, Somlo S, Harris PC, Gattone VH. Effective treatment of an orthologous model of autosomal dominant polycystic kidney disease. Nat Med 2004; 10:363–4. https://doi.org/10.1038/nm1004

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.

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. https://doi.org/10.2215/CJN.00330705




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