Cure of Hypertension in a Child with Renal Artery Stenosis and Hydronephrosis in a Solitary Kidney

Vol. 109, January Printed in U.S.A.

THE JOURNAL OF UROLOGY

Copyright © 1973 by The Williams & Wilkins Co.

CURE OF HYPERTENSION IK A CHILD WITH RENAL ARTERY STENOSIS A:"JD HYDRONEPHROSIS IN A SOLITARY KIDNEY ANDHEW J. PRESTO, III

,\ND

RICHARD G. MIDDLETON

Frorn the Division of Urology, Department of Surgery, University of Utah Co/lege of Medicine, Salt Lake City, Utah

Hypertension is uncommon in children and its cause can usually be found. 1- 4 Renal disease accounts for most cases of hypertension in children. Primary glomerular disease is responsible for 60 to 70 per cent of the cases attributed to renal disease. 5 Congenital stenosis of the renal artery with renovascular hypertension has been reported. In 1938 Leadbetter and Burkland reported on the first case of hypertension in a child to be cured by removal of a kidney with a steno tic artery. 6 Since then other investigators have reported cases of renovascular hypertension in children cured by nephrectomy. 4 • 1 - 10 Repair of the renal arterial lesion is preferable to nephrcctomy if the kidney seems worth salvaging. Smith, DcCamp and Oliver and their associates have reported on renal arterioplasty accomplished in children without sacrificing the involved kidney. 4 • 11 • 12 DeCamp performed a successful splenorenal arterial shunt in a 10-year-old girl with severe hypertension and a solitary left kidney. Occasionally hydronephrosis is a cause of hyper-

tension. Delman reported on a patient with hypertension and elevated plasma renin associated with urcteral obstruction.13 Relief of the obstruction resulted in cure of the hypertension and return of the plasma renin level to normal. Palmer described a case of ureteropelvic junction obstruction in a solitary kidney associated with hypertension and a normal plasma renin. 14 In this patient hypertension was relieved by pyeloplasty. Herein we describe a child with severe hypertension and elevated plasma renin associated with a solitary kidney in which there was congenital arterial stenosis and ureteropelvic junction obstruction. CAi:i-'" REPORT

An 11-year-old white boy, 27-19-37, was admitted to the hospital in August 1971 because of hypertension 7 years in duration. At age 4 he had been admitted to another hospital for evaluation of hypertension and a heart murmur. He was found to have a patent ductus arteriosus and a small ventricular septal defect. Excretory urography (IVP) revealed right hydronephrosis, resulting from ureteropelvic junction obstruction, and a normal left upper urinary tract. Following an uneventful recovery from repair of the patent ductus arteriosus, the patient underwent right renal exploration. At this time right nephrectomy was carried out in order to relieve the hypertension. However, the operation was not successful. The patient continued to have severe hypertension unresponsive to various antihypertensive medications. On admission to the hospital in August 1971 the patient's blood pressure was 170/125. It reached a maximum of 200/150. Hypertensive changes were present in the optic fundi with arteriolar narrowing and mild arteriovenous nicking. There were well healed left thoracotomy and right flank scars. The heart was enlarged with a point of maximal impulse located at the anterior axillary line. A grade III/VI holosystolic, apical murmur was heard with radiation to the posterior axillary line. Strong and equal pulses were palpated in all extremities. Laboratory data included hematocrit 45 per cent and white blood count 5,300 with a normal differential. Urinalysis revealed specific gravity 1.026, pH

Accepted for publication June 2, 1972. Loggie, J. M.: Hypertension in children and adolescents. I. Causes and diagnostic studies. J. Pediat., 74: 331, 1969. 2 Singh, S. P. and Page, L.B.: Hypertension in early life. Amer. J. Med. Sci., 253: 2.5/':i, 1967. 3 Ooi, B. S., Chen, B. T. M., Toh, C. C. S. and Khoo, 0. T.: Causes of hypertension in the young. Brit. Med. J., 3: 744, 1970. 4 Smith, J.P. and Saylor, M. L.: Henal vascular hypertension in children. J. Urol., 84: 17, 1960. 5 McCrory, W.W. and Nash, F. W.: Hypertension in children: a review. Amer. J. Med. Sci., 223: 671, 19.52. 6 Leadbetter, W. F. and Burkland, C. E.: Hypertension in unilateral renal disease. J. Urol., 39: 611, 1938. 7 Cook, G. T., Marshall, V. F. and Todd, J.E.: Malignant renovascular hypertension in a newborn. J. Urol., 96: 863, 1966. 8 Ullmann, T. D., Shapiro, T., Barzilay, B. and Schwartz, A.: Vascular abnormalities in a well functioning kidney as the cause of long-standing severe juvenile hypertension, cured by unilateral nephrectomy. Amer. J. Med., 26: 960, 19.59. 9 Snyder, C. H., Bost, R. B. and Platov, R. V.: Hypertension in infancy with anomalous renal artery; diagnosis by renal arteriography, apparent cure after nephrectomy. Pedia tries, 15: 88, 1955. 10 DeCamp, P. T. and Birchall, H.: Recognition and treatment of renal arterial stenosis associated with hypertension. Surgery, 43: 134, 1958. 11 DeCamp, P. T., Snyder, C.H. and Bost, R. B.: Severe hypertension due to congenital stenosis of artery to solitary kidney: correction by splenorenal arterial anastomosis. Arch. Surg., 75: 1023, 1957. 12 Oliver, W. J., Talner, N. S. and Stern, A. M.: Hypertension in infancy and childhood. J. Michigan State Med. Soc., 59: 82, 1960. 1

13 Belman, A. B., Kropp, K. A. and Simon, N. M.: Renal-pressor hypertension secondary to unilateral hydronephrosis. New Engl. J. Med., 278: 1133, 1968. 14 Palmer, J. M., Zweiman, F. G. and Assaykeen, T. A.: Renal hypertension due to hydronephrosis with normal plasma renin activity. New Engl. J. Med., 283: 1032, 1970.

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5.0, protein O and occasional white and red blood cells per high power field. Urine culture was sterile. Other laboratory data included normal electrolytes, blood urea nitrogen 21 mg. per cent, creatinine 0.9 mg. per cent and fasting blood sugar 90 mg. per cent. The 24-hour urine collection yielded the following values: sodium 177 mEq., potassium 48 mEq., protein 14.4 mg., aldosterone 5 µg., vanillyl mandelic acid 2.4 units, metanephrins 0.2 mg. and creatinine clearance 48 cc per minute (uncoffected). Peripheral tension unresponsive to various antihypertensive

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vein renin was 2.5 ng. per mL per hour of incubation (normal in this laboratory is O to 1.5). An electrocardiogram .demonstrated left ventricular hypertrophy, which was confirmed by chest x-ray. IVP showed a slightly enlarged left kidney with mild to moderate ureteropclvic junction obstruction (fig. 1, A). The ureteropelvic junction obstruction was confirmed by occlusive retrograde pyelography (fig. 1, B). An abdominal aortogram demonstrated marked left renal arterial stenosis with post-stenotic dilatation and abundant collateral circulation (fig. 2).

Frn. 1. A, preoperative IVP demonstrates slightly hypertrophied left kidney with mild to moderate ureteropelvic junction obstruction. B, occlusive left retrograde pyelogram confirms ureteropelvic junction obstruction.

Frn. 2. Aortogram demonstrates short, stenotic segment of left renal artery with post-stenotic dilatation

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blood pressure of 105/65. Urinalysis is normal. A recent IVP is shown in figure 3. DISCUSSION

FIG. 3. IVP 6 months after arterioplasty and pyeloplasty shows prompt function and well tapered ureteropelvic junction.

The left kidney was explored through a subcostal, transabdominal incision. A short, narrowed segment was found in the renal artery about 2 cm. distal to its origin in the aorta. Post-stenotic dilatation was noted and a thrill was palpated in the renal artery. As anticipated, there was considerable collateral circulation around the hilus of the kidney. With temporary arterial occlusion the artery was opened longitudinally through the stenotic section and an autogenous, elliptical segment of saphenous vein patch was sutured in place to enlarge the caliber of the artery. Upon release of the arterial occlusion, there was prompt return of color to the kidney. The pulse in the renal artery was strong and the thrill had been eliminated. At the time of the exploration a dismembered Foley Y-V pyeloplasty was carried out using a nephrostomy tube and splinting qatheter. A renal biopsy obtained at operation showed normal renal parenchyma. Convalescence was satisfactory and by 9 days postoperatively the blood pressure ranged between 120 to 130/75 to 80. At 3 weeks postoperatively the ureteral splinting catheter and nephrostomy tube were removed after a satisfactory nephrostomy tube pyelogram had been obtained. At 12 months postoperatively this patient remains normotensive with a

The etiology of hypertension in a child must be investigated. A cause can usually be found and uncorrected high blood pressure in childhood can be life-threatening.7 • 15- 17 After the onset of left ventricular hypertrophy the average survival may be as short as 14 months, with death owing to uremia, cardiac failure and hypertensive encephalopathy. 1 Still reported that 9 per cent of 55 hypertensive children had renal artery stenosis.18 Other reported causes of renal hypertension in children are renal arterial aneurysm, arteriovenous fistula, renal vein thrombosis, trauma, arteritis and Wilms tumor, in addition to glomerular disease. With unilateral renal ischemia nephrectomy may be desirable particularly in an infant or in an older child if the involved kidney is small. Since the kidney was solitary and the hypertension was severe and unresponsive to medication in our case, renal arterial repair was the only choice. However, arterial repair in the solitary kidney is risky because of the possibility of early technical failure and late recurrent stenosis. In our patient hydronephrosis and ureteropelvic junction obstruction developed in a previously normal left upper urinary tract within 7 years of right nephrectomy. Although the wisdom of performing simultaneous arterial repair and pyeloplasty in a solitary kidney may be debatable, we believed the arterioplasty to be satisfactory and preferred simultaneous pyeloplasty to later operation in the same site. The hypertension can be attributed to the arterial stenosis alone because it pre-dated the development of hydronephrosis in the left kidney. SUMMARY

An 11-year-old boy with severe hypertension unresponsive to antihypertensive medication was found to have a solitary left kidney, stenosis of the left renal artery, ureteropelvic junction obstruction and an elevated plasma renin level. An arterioplasty with a saphenous vein patch and a pyeloplasty were performed. At 12 months postoperatively this child is normotensive. 1 5 Ljungqvist, A. and Wallgren, G.: Unilateral renal artery stenosis and fatal arterial hypertension in a newborn infant. Acta Paediat., 61: 575, 1962. 16 Dawson, I. M. P. and Nabarro, S.: A case of intimal hyperplasia of arteries with hypertension in a male infant. J. Path. Bact., 66: 493, 1953. 17 Schmidt, D. M. and Rambo, 0. N., Jr.: Segmental intimal hyperplasia of the abdominal aorta and renal arteries producing hypertension in an infant. Amer. J. Olin. Path., 44: 546, 1965. 18 Still, J. L. and Cottom, D.: Severe hypertension in childhood. Arch. Dis. Child., 42: 34, 1967.