Year : 2006 | Volume
: 22 | Issue : 3 | Page : 260--261
Multicystic renal dysplasia of lower moiety in a duplicated system causing hypertension
NK Agarwal, LN Dorairajan, S Kumar, SVR Chandra Murthy
Department of Urology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
L N Dorairajan
Department of Urology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry - 605 006
Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children. Ipsilateral duplication anomalies associated with multicystic kidney have rarely been reported. Hypertension associated with multicystic kidney has been reported previously in a few cases but on Medline search no report was found where hypertension was associated with segmental multicystic dysplasia in a duplicated system. We report a one-year-old girl in whom hypertension was associated with left partial duplicated system and lower moiety multicystic dysplasia. After lower pole heminephrectomy the hypertension resolved and remained so at six months follow-up. In our knowledge this is the first report of its type.
|How to cite this article:|
Agarwal N K, Dorairajan L N, Kumar S, Chandra Murthy S. Multicystic renal dysplasia of lower moiety in a duplicated system causing hypertension.Indian J Urol 2006;22:260-261
|How to cite this URL:|
Agarwal N K, Dorairajan L N, Kumar S, Chandra Murthy S. Multicystic renal dysplasia of lower moiety in a duplicated system causing hypertension. Indian J Urol [serial online] 2006 [cited 2020 Apr 8 ];22:260-261
Available from: http://www.indianjurol.com/text.asp?2006/22/3/260/27636
Multicystic dysplastic kidney (MCDK) results from abnormal induction of metanephros by the ureteric bud, severe ureteral obstruction early in gestation or ureteral atresia. Typically, MCDK is associated with ureteral atresia and single collecting system and represents the most severe form of infundibulopelvic dysgenesis. There are a few reports of hypertension associated with MCDK and few case reports of ipsilateral duplication anomalies associated with segmental multicystic dysplastic kidney. However, a Medline search did not reveal a report of these two rare phenomena occurring together. We treated a one-year-old female child with lower moiety renal dysplasia with hypertension that resolved after lower pole heminephrectomy.
The patient was born at term after an uncomplicated pregnancy. There was no history of antenatal exposure to teratogens. She was incidentally detected to be hypertensive at eight months of age. The blood pressure on multiple occasions ranged from the 90th to 95th percentile for her age (Systolic pressure 100 to 110 and diastolic pressure 70 to 80 mm Hg). Physical examination revealed a small multilobulated mass palpable in the left side of the abdomen. Urine analysis, serum electrolytes and serum creatinine were normal.
USG abdomen [Figure 1] demonstrated a left renal duplex system with normal upper moiety and a lower pole replaced with multiple noncommunicating cysts. The right kidney was normal. Tc 99 m DTPA scan [Figure 1] showed cold areas in the lower pole region. Intravenous pyelography [Figure 2] showed left kidney upper pole displaced upward with nonvisualization of lower pole. Distal 2 cm of the ureter of the lower pole was seen, probably due to reflux from the upper pole ureter. Voiding cystography was normal.
Blood pressure was controlled initially with tab Amlodepine 1.25 mg twice a day. At one year of age she underwent left lower pole heminephrectomy. Intraoperatively the lower pole of the left kidney was replaced with multiple tense cystic areas with a few solid areas in between. An approximately 4 cm long atretic ureter was joining the upper pole ureter. The distal 2 cm of this ureter was having a narrow lumen. Histological examination showed multicystic dysplastic kidney. Postoperatively the blood pressure became normal without antihypertensive drugs. At the last follow-up six months postoperatively she continued to have normal blood pressure.
Multicystic dysplastic kidney is a form of renal dysplasia in which the kidney is replaced with multiple tense noncommunicating cysts of various sizes and the ureter is atretic or nonpatent. Multicystic dysplastic kidney is the most common cause of abdominal mass in the new born. This can be readily diagnosed by USG and radionuclide study. Surgical intervention is rarely required, being confined usually to cases with a very large mass or where there is a suspicion of malignancy. A more conservative approach has been applied. Complete involution has been reported in between 16-27% of cases.
Wilms' tumor and hypertension has been rarely associated with retained MCDK. Although contralateral anomalies such as ureteropelvic junction obstruction, obstructive megaureter and vesicoureteric reflux are well described in 7-43% cases of multicystic kidney, there have been only 14 cases of ipsilateral duplication anomalies associated with segmental dysplasia reported since 1964 including our case. This anomaly is more common in female newborns and 75% of patients have it on the left side as was seen in our patient also. Presumably, aberrant ureteral development or essentially total ureteral obstruction caused abnormal induction of the lower half of metanephric blastema with formation of segmental MCDK in our case.
Nineteen cases of hypertension associated with MCDK have been reported till now., The true prevalence of hypertension associated with MCDK is yet obscure. Narchi reviewed 29 studies on MCDK and found that six cases of hypertension developed in 1115 children with unilateral MCDK managed conservatively. He estimated the mean probability of a child with unilateral MCKD developing hypertension to be 5.4 per 1000. However, it was not established that the dysplatic kidney was the cause of hypertension in all the cases. Of 12 pediatric cases of hypertension associated with MCDK reported up to 2000, the renin level has been measured in only five and was elevated in only three. But hypertension in all five cases resolved after nephrectomy. Peripheral renin activity may be normal in lateralizing hyperreninemic hypertension and it may not be feasible to obtain inferior vena cava samples in infants or young children. Typically, blood pressure elevated due to excess renin secretion rapidly returns to normal, unless it is prolonged due to other reasons. Contralateral renal anomalies and vesicoureteral reflux, which may rarely be the cause of hypertension, were also not present in our case.
After lower pole heminephrectomy hypertension resolved even before the child was discharged. Though we have not measured plasma renin levels, the rapid resolution of hypertension suggests that it may be due to excessive secretion of renin from the dysplastic tissue, as hypertension has resolved early after nephrectomy.
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