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ORIGINAL ARTICLE
Year : 2000  |  Volume : 17  |  Issue : 1  |  Page : 32-35
 

Congenital unilateral multicystic dysplastic kidney in children: A clinical study


Department of Paediatric Surgery, Postgraduate Institute of Medical Sciences, Rohtak, India

Correspondence Address:
Arun K Sharma
c/o B.L. Sharma, 5-A, Double Storey, Tansen Nagar, Gwalior - 474 002
India
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Source of Support: None, Conflict of Interest: None


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   Abstract 

Multicystic kidney is one of the most frequently encoun­tered masses in the newborns, ranking either first or sec­ond in occurrence. Totally 15 cases were managed over the last 5 years. The most common age of presentation was within first month of life, the affliction occurring more on the left side. Commonest mode of presentation was ab­dominal mass. Ultrasonography was the diagnostic inves­tigation of choice. Five cases were diagnosed antenatally. Associated anomalies were found in 8 cases with anovesti­bular fistula, PUJO in contralateral kidney, right con­genital lumbar hernia and oesophageal atresia with TOE. Nephrectomy was the surgical treatment performed along with appropriate management of the associated anoma­lies.


Keywords: Multicystic Dysplastic Kidney; Unilateral; Children


How to cite this article:
Sharma AK, Rattan K N, Sharma N. Congenital unilateral multicystic dysplastic kidney in children: A clinical study. Indian J Urol 2000;17:32-5

How to cite this URL:
Sharma AK, Rattan K N, Sharma N. Congenital unilateral multicystic dysplastic kidney in children: A clinical study. Indian J Urol [serial online] 2000 [cited 2019 Jul 18];17:32-5. Available from: http://www.indianjurol.com/text.asp?2000/17/1/32/41010



   Introduction Top


Cystic lesions of the kidney present a variety of forms in infants and children. Multicystic kidney disease is a well-defined group of unilateral and occassionally bila­teral cases. [1] It is a developmental anomaly of the kidney in which renal parenchyma is replaced by tense non-com­municating cysts and the proximal ureter is atretic or non­patent. In 1936, Schwartz [2] first described multicystic kidney disease but the characteristics of the disease was first described by Spence in 1955. [3]

Different theories were advanced as to the etiology of renal cyst formation. The original "failure of union" theory of Hilderbrant, the "persistence of vestigeal structures" thesis of Kampmeier and "the localised urinary obstruc­tion plus vascular impairment" mechanism postulated by Hepler. None seems universally satisfactory or acceptable. Actually all that can be stated definitely in this regard is that muticystic kidney is a congenital defect resulting from foetal maldevelopment not on a hereditary basis.

A review of the patients at our institution in whom a multicystic kidney had been diagnosed was undertaken and the results of this study and those in literature are dis­cussed in relation to the overall management of the asymtomatic multicystic dysplastic kidney (MCDK).


   Patients and Methods Top


During a 5-year-period 15 children with multicystic dys­plastic kidney who underwent surgical treatment were identified. Of the 15 cases, 5 were diagnosed antenatally via ultrasonography while 5 were less than one month of age and 4 were less than 4 years of age. Only one of the case presented at 12 years of age in our series [Table 1]. Male patients were predominant (9:6). A family history concerning the renal disease was absent in all the 15 cases. The left kidney was affected slightly more often than the right kidney (10:5).


   Results Top


5 cases were diagnosed antenatally by ultrasound while rest 10 presented with a flank or abdominal mass which was the commonest mode of presentation. Of the 15 pa­tients with multicystic dysplastic kidney 12 had unilateral disease with normal opposite kidney while 3 had PUJ ob­struction on the contralateral side. On routine abdominal palpation in an otherwise healthy infant a large, unilat­eral, lobulated, non-tender movable mass is found in the flank [Figure 1]. Four patients were admitted primarily for the treatment of congenital anomalies and multicystic kid­ney were diagnosed during work up of the patients [Table 2]. Urine examination was found to be normal in 12 cases while in 3 cases, urine was infected and positive cultures of B.coli or B. proteus were obtained. Blood urea and se­rum creatinine was found to be normal in all the cases.

Ultrasonography is the investigation of choice which is non-invasive and establishes the diagnosis in almost all cases, including antenatal diagnosis. Most significant find­ing on excretory urography were non-opacification of the kidney mass with a normal or hydronephrotic contrala­teral kidney in 3 cases which was associated with PUJO. Associated anomalies found were urologic and non-uro­logic. Urologic anomalies included contralateral uretero­pelvic junction obstruction in 3 cases while hypospadias was present in one of the cases. Non-urological anomalies included anovestibular fistula in 3 cases, oesophageal atre­sia with TOF in 1 case, right congenital lumbar hernia in 1 case [Table 2]. Cystourethroscopy was done showing complete absence of the ureteric orifice in the bladder on the side of lumbar mass and arrest of the catheter in the ureter at varying levels during catheterisation on the side of lumbar mass.

Nephrectomy was performed by transperitoneal ap­proach in all the 15 cases after demonstration of a life­supporting contralateral kidney.

The multicystic kidney removed does not have a reni­form outline on gross examination but consists of a grape­like cluster of cysts held together by connective tissue [Figure 2]. The size of the cysts varies and so also the number. Cut-section showed thin-walled cysts around a small nubbin of solid or spongy tissue near the centre [Figure 3]. Although there was no apparent communication between the cysts [4] but some authors have reported that these cysts may communicate with one another. [5]

The ureter is atretic [Figure 4] in 13 cases and in 2 cases the ureter was found to be entirely absent and was repre­sented by a very thin fibrous strand. All the multicystic kidneys presented with a histological picture of the pres­ence of primitive renal elements often surrounded by loose embryonic mesenchyme. The walls of the cyst were com­posed of fibrous tissue and the epithelial lining was found to be flat or cuboidal.


   Discussion Top


The term "unilateral multicystic kidney" was first proposed in 1936 by Schwartz. [2] All the 15 cases in our series MCDK were unilateral but bilateral cases can also occur. [1] Most common age of presentation was within first month of life and 5 cases were diagnosed antenatally by ultrasono­graphy. It is possible that approximately three-quarters of the unilateral multicystic kidneys remained undiagnosed in the days before pre-natal ultrasound diagnosis. [1] The incidence of unilateral multicystic kidney was 1 in 4300 live births. The incidence of unilateral renal agenesis in the adult population has been reported variably to be be­tween 1:1500 to 1:2000. [6],[7] It is possible that a significant proportion of these cases are in fact the end result of invo­lution of a muticystic kidney.

Male patients were predominant, [5] although some say that there is no predilection for side or sex. [3]

Commonest mode of presentation was lump in the ab­domen. There was no evidence of pain or discomfort due to lump, - it did not cause any disturbance of micturition being excluded from the urinary tract. Ultrasonography was the investigation of choice in our series and 5 of the cases had been diagnosed by pre-natal ultrasonography.

Investigation: Most significant finding on excre­tory urography was non-visualisation of muticystic dys­plastic kidney with a normal-appearing kidney on the opposite side. Classically renal function is absent in MCDK and there should be no detectable concentration of radiographic contrast medium. [8],[9] In recent years the phenomenon of the total-body-opacification phase of high­dose urography has been used in new borns as a diagnos­tic aid. Some multicystic kidneys might, indeed, be capable of minimal function adequate to visualize radio­graphically although insufficient to support life. We did renal isotope scan in 3 cases; no function was seen in MCDK. Cystoscopy and attempted retrograde urography when performed in the reported cases have shown either an absence of the ureteral orifice on the side correspond­ing to the lesion or a blind termination of a small ureter below the ureteropelvic junction.

Treatment: The most appropriate management of the multicystic dysplastic kidney remains controversial. Nephrectomy was done after demonstration of a life-supporting contralateral kidney in all the cases. The trans­peritoneal approach was preferred since it has certain ad­vantages in the early control of blood supply, avoidance of injury to adjacent viscera and abdominal exploration particularly of the opposite kidney is possible.

Long-term finding show that MCDK may take as long as 20-25 years to resolve; families and physicians should be prepared for long-term follow-up (Wacksman 1993) which is not possible in Indian circumstances.

The controversial issue is the long-term risk of the de­velopment of malignancy in the MCDK. Nodular renal blastema is a part of the nephroblastomatosis complex and it bears an association with Wilm's tumor. Such blastemal elements are considered to be precursor lesions to Wilm's tumor according to the "2 hit" theory of Knudson and Strong. [12] The true incidence of nodular renal blastema is increased in multicystic dysplasia and it is in the range of 3-5 percent. [14] One must accept that there is a risk for the development of neoplasia although it appears to be small. From 1966 to 1986 - 6 reports of malignancy associated with MCDK had been reported. Flow cytometric evalua­tion of MCDK demonstrated a diploid pattern of deoxyri­bonucleic acid. But these findings do not support or negate the potential for neoplasm associated with MCDK. Since a diploid deoxyribonucleic acid pattern does not elimi­nate the possibility of the future development of malig­nancy. [15] Hypertension has been reported in 4 children with retained MCDK. [16]

The most pressing of these issues in Indian circum­stances seems to be how to assure life-long surveillance and also that they are not lost to follow-up. It would seem that life-long surveillance will probably be less than suc­cessful in a large number of cases and in that regard op­erative removal of the MCDK would be definitive. Also it would appear that in experienced hands, anaesthetically and surgically the risk of removing a MCDK is small and perhaps even smaller than the incidence of development of Wilm's tumor if the organ is retained. [18] We believe clearly that surgery is the best option in those children in whom the retained mass appears to be growing, hyperten­sion develops, the diagnosis is in question or adequate follow-up can not be assumed. Since the multicystic dys­plastic kidney is unilateral, the prognosis is good.

Follow-Up: All the 15 cases are under follow-up. We did ultrasonography after 3 months to rule out any multicystic changes in the opposite kidney. Routine urine examination and renal function test like blood urea and serum creatinine must be carried out every month. There was no mortality in our series.

 
   References Top

1.Gordon AC, Thomas DFM. Arthur RJ, Irving HC. Multicystic dys­plastic kidney: Is nephrectomy still appropriate? J Urol 1988; 140: 1231-1234.  Back to cited text no. 1    
2.Schwartz J. An unusual unilateral multicystic kidney in an infant. J Urol 1936; 35: 259.  Back to cited text no. 2    
3.Spence HM. Congenital unilateral muticystic kidney: an entity to be distinguished from polycystic kidney disease and other cystic disorders. J Urol 1955; 74: 693.  Back to cited text no. 3  [PUBMED]  
4.Pathak IG, Williams M DI. Multicystic and cystic dysplastic kid­neys. Br J Urol 1964; 36: 318.  Back to cited text no. 4    
5.Greene LF, Feinzaig W, Dahlin DC. Multicystic dysplasia of the kidney with special reference to the contralateral kidney. J Urol 1971:105:482.  Back to cited text no. 5    
6.Campbell MF. Congenital absence of one kidney: Unilateral renal agenesis. Ann Surg 1928; 88: 1039.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Emanuel B, Nachman R, Aronson N, Weiss H. Congenital solitary kidney: a review of 74 cases. J Urol 1974; 111: 394-397.  Back to cited text no. 7  [PUBMED]  
8.Kyaw MM. The radiological diagnosis of congenital multicystic kidney `radiological triad'. Clin Radio! 1974; 25: 45.  Back to cited text no. 8    
9.Leonidas JC, Strauss L. Krasna IH. Roentgen diagnosis of multicystic renal dysplasia in infancy by high dose urography. J Urol 1972; 108: 963-965.  Back to cited text no. 9    
10.Warshawsky AB, Miller KE, Kaplan GW. Urographic visualisa­tion of multicystic kidneys. J Urol 1977; 117: 94-96.  Back to cited text no. 10  [PUBMED]  
11.Wacksman J, Phipps L. Report of the multicystic kidney registry: Preliminary findings. J Urol 1993; 150: 1870-1872.  Back to cited text no. 11  [PUBMED]  
12.Knudson AG Jr, Strong LC. Mutation and Cancer: a model for Wilm's tumor of the kidney. J Nail Cancer Inst 1972: 48: 313.  Back to cited text no. 12    
13.Beckwith JB. Wilm`s tumor and other renal tumors of childhood: an update. J Urol 1986: 136: 320-324.  Back to cited text no. 13    
14.Dimmick JE, Johnson HW, Coleman GU, Carter M. Wilm's tumorlet, nodular renal blastema and multicystic renal dysplasia. J Urol 1942: 48: 198.  Back to cited text no. 14    
15.Jung WH, Peters CA. Mandell J, Vawter GF, Retik AB. Flow cytometric evaluation of multicystic dysplastic kidneys. J Urol 1990; 144: 413-415.  Back to cited text no. 15    
16.Javadpour N, Chelouhy E, Moncada L, Rosenthal IM. Bush IM. Hypertension in a child caused by a multicystic kidney. J Urol 1970; 104: 918-921.  Back to cited text no. 16    
17.Chen YH, Stapleton FB, Roys S III. Noe HN. Neonatal hyperten­sion from a unilateral muticystic dysplastic kidney. J Urol 1985; 133: 664-665.  Back to cited text no. 17    
18.Noe HN, Marshall JH, Edwards OP. Nodular renal blastema in the multicystic kidney. J Urol 1989: 142: 486-488.  Back to cited text no. 18    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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    Abstract
    Introduction
    Patients and Methods
    Results
    Discussion
    References
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