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CASE REPORT
Year : 2002  |  Volume : 18  |  Issue : 2  |  Page : 157-159
 

Medullary sponge kidney and congeni­tal hemihypertrophy : A case report


Department of Urology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India

Correspondence Address:
L N Dorairajan
Department of Urology, JIPMER, Pondicherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


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Keywords: Medullary Sponge Kidney; Renal Anomalies and Hemihypertrophy.


How to cite this article:
Singh H, Pandey S, Dorairajan L N, Kumar S. Medullary sponge kidney and congeni­tal hemihypertrophy : A case report. Indian J Urol 2002;18:157-9

How to cite this URL:
Singh H, Pandey S, Dorairajan L N, Kumar S. Medullary sponge kidney and congeni­tal hemihypertrophy : A case report. Indian J Urol [serial online] 2002 [cited 2019 Jul 17];18:157-9. Available from: http://www.indianjurol.com/text.asp?2002/18/2/157/37624



   Case Report Top


A 50-year-old male presented with a history of recur­rent graveluria since the age of 38 years. He used to have dysuria and irritative voiding symptoms at the time of graveluria but without flank pain. One month before ad­mission he had acute retention of urine and was catheter­ised. On examination he had asymmetric limbs. Left upper limb was 3 cm and lower limb was 12 cm longer than opposite limb (Figure 1]. Intravenous urography revealed bilateral nephrocalcinosis, multiple renal and vesical cal­culi, papillary blush and precalyceal cystic spaces filled with contrast suggestive of medullary sponge kidney [Figure - 2]. Cystolithotripsy was done. Qualitative stone analy­sis revealed calcium, oxalate and phosphate. Patient was started on hydrochlorthiazide to reduce the risk of stone formation.


   Comments Top


Medullary sponge kidney is a congenital benign disor­der of kidney consisting of diffuse ectasia or cystic dilata­tion of collecting ducts within medullary pyramid of one or both kidneys. Its incidence in general population has been estimated to be between 1 in 5,000 and l in 20,000. [1] Congenital hemihypertrophy is a rare condition of un­known aetiology with an incidence of 1 in 86,000. [2] It has been associated with variety of benign and malignant ab­dominal disorders. [3] The association of medullary sponge kidney with congenital hemihypertrophy was first reported in 1964. Since then only 30 cases have been documented in literature. Harris RE et al [4] reviewed 24 cases of medul­lary sponge kidney and congenital hemihypertrophy re­ported till 1981 and thereafter 6 more cases have been reported. Medullary sponge kidney is also found in asso­ciation with  Beckwith-Wiedemann syndrome More Details particularly if congenital hemihypertrophy is a part of syndrome. Although it has been proposed that the combination of med­ullary sponge kidney and congenital hemihypertrophy is a minor form of Beckwith-Wiedemann syndrome, our case did not have any other feature of Beckwith-Wiedemann syndrome.

 
   References Top

1.Mayall GF. The incidence of medullary sponge kidney. Clin Rad 1970; 21: 171.  Back to cited text no. 1    
2.Parker DA, Skaeko RG. Congenital asymmetry -report of 10 cases with associated developmental abnormalities. Pediatrics 1969; 44: 584.  Back to cited text no. 2    
3.Hennessy WT, Cromie WJ, Duckett JW. Congenital hemihypertrophy and associated abdominal lesions. Urology 1981: 18: 576.  Back to cited text no. 3    
4.Harris RE, Fuchs EF, Kaempf MJ. Medullary sponge kidney and congenital hemihypertrophy: case report and literature review. J Urol 1981; 126: 676-678.  Back to cited text no. 4    


    Figures

  [Figure - 1], [Figure - 2]



 

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