Indian Journal of Urology
CASE REPORT
Year
: 2002  |  Volume : 18  |  Issue : 2  |  Page : 157--159

Medullary sponge kidney and congeni­tal hemihypertrophy : A case report


H Singh, S Pandey, LN Dorairajan, S Kumar 
 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




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;18:157-159


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 2021 Sep 20 ];18:157-159
Available from: https://www.indianjurol.com/text.asp?2002/18/2/157/37624


Full Text

 Case Report



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



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 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

1Mayall GF. The incidence of medullary sponge kidney. Clin Rad 1970; 21: 171.
2Parker DA, Skaeko RG. Congenital asymmetry -report of 10 cases with associated developmental abnormalities. Pediatrics 1969; 44: 584.
3Hennessy WT, Cromie WJ, Duckett JW. Congenital hemihypertrophy and associated abdominal lesions. Urology 1981: 18: 576.
4Harris RE, Fuchs EF, Kaempf MJ. Medullary sponge kidney and congenital hemihypertrophy: case report and literature review. J Urol 1981; 126: 676-678.