|Year : 2002 | Volume
| 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
L N Dorairajan
Department of Urology, JIPMER, Pondicherry - 605 006
Source of Support: None, Conflict of Interest: None
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
| 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.  Congenital hemihypertrophy is a rare condition of unknown aetiology with an incidence of 1 in 86,000.  It has been associated with variety of benign and malignant abdominal disorders.  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  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|| |
|1.||Mayall GF. The incidence of medullary sponge kidney. Clin Rad 1970; 21: 171. |
|2.||Parker DA, Skaeko RG. Congenital asymmetry -report of 10 cases with associated developmental abnormalities. Pediatrics 1969; 44: 584. |
|3.||Hennessy WT, Cromie WJ, Duckett JW. Congenital hemihypertrophy and associated abdominal lesions. Urology 1981: 18: 576. |
|4.||Harris RE, Fuchs EF, Kaempf MJ. Medullary sponge kidney and congenital hemihypertrophy: case report and literature review. J Urol 1981; 126: 676-678. |
[Figure - 1], [Figure - 2]