Vol. 116, December Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
Pediatric Articles UNILATERAL RENAL AGENESIS PRESENTING AS ANDRIA JOSEPH GIANGIACOMO* From the Department of Pediatrics, Cardinal Glennon Memorial Hospital for Children, St. Louis University Medical School, St. Louis, Missouri ABSTRACT
The most common cause of sudden and total cessation of urine output is obstructive uropathy, usually at the bladder outlet. Bilateral ureteral obstruction is a much less common cause of anuria. In addition, unilateral obstruction in the presence of a solitary kidney must be considered in the differential diagnosis. Primary renal parenchymal disorders and pre-renal azotemia occasionally may be anuric but more commonly are oliguric. A case of unilateral renal agenesis presenting as anuria and obstruction of the solitary kidney is described. A 7-week-old male infant weighed 6 pounds, 14 ounces at birth and had been well until he became irritable with vomiting and diarrhea. A mass was palpable in the left upper quadrant. There was no evidence of dehydration. Initially, an excretory urogram (IVP) showed non-visualization and an x-ray of the abdomen 18 hours later showed a dilated left pelvis with non-visualization of the right kidney and no contrast material in the bladder (part A of figure). The renal scan showed an enlarged left kidney with dilatation and obstruction at the level of the pelvis, and the right kidney was not demonstrated. There was no urine obtained on catheterization of the
resolved but he became acutely anuric and hyperkalemic, and was managed with sodium polystyrene sulfonate. Although an IVP initially showed non-visualization, 10 hours later there was contrast material filling a dilated left pelvis. Under fluoroscopic visualization a No. 16 venous intracatheter was introduced percutaneously through the back with subsequent drainage of the pelvis. An IVP 1 month later showed decreased pelviocaliectasis, narrowing at the ureteropelvic junction and contrast material in the bladder, with the nephrostomy tube in the pelvis (part B of figure). The tube was removed 2 months later. A recent IVP, 3 months after removal of the nephrostomy
bladder. Serum sodium was 117 mEq. per 1., chloride 82 mEq. per 1., carbon dioxide 17.1 mEq. per 1., potassium 8.3 mEq. per 1., blood urea nitrogen (BUN) 38 mg. per cent and serum creatinine 5.8 gm. per cent. The hyperkalemia had decreased with oral sodium polystyrene sulfonate. Surgical exploration of the left kidney showed a ureteropelvic obstruction, which was removed. Postoperatively, the serum creatinine decreased to 1.1 mg. per cent and the BUN to 7.7 mg. per cent. The infant was hospitalized 3 weeks later for vomiting, diarrhea and dehydration. Subsequently, the gastroenteritis
tube, showed mild pelviocaliectasis (part C offigure). The child is 2 years old and doing well.
Accepted for publication June 18, 1976. * Requests for reprints: Cardinal Glennon Memorial Hospital for Children, 1465 S. Grand Blvd., St. Louis, Missouri 63104. 790
DISCUSSION
Anuria is defined as total cessation of urine output or less than 50 cc of urine production per day. 1 The most common cause is urinary tract obstruction at the level of the bladder outlet and this is seen in older men with prostatic disease. In these cases there is usually a history of gradual increase in difficulty in initiating and terminating the urinary stream. At times there are large and rapid swings in urinary output and this should increase the suspicion that bladder outlet obstruction is present. Bilateral ureteral obstruction is a much less
UNILATERAL RENAL AGENESIS PRESENTING AS ANDRIA
common cause of anuria and a complication of infiltrating malignancies. Acute tubular necrosis, bilateral cortical necrosis, acute glomerulonephritis and rapid progressive glomerulonephritis usually cause oliguria. Of these acute tubular necrosis is the most common cause of acute renal failure. The major precipitating events associated with acute tubular necrosis would be profound shock and extensive trauma, intravascular hemolysis, generalized sepsis, severe dehydration and a host of exogeneous toxins. The incidence of unilateral renal agenesis is estimated as 1.5 in 1,000 newborns, 2 while the autopsy occurrence is 1 in 1,824. 3 In a recent report of 74 cases a significant number had associated congenital anomalies involving vertebrae, cardiac anomalies, and long bones, hands and genitalia.• None presented with anuria. Most of the cases were discovered during evaluation for recurring urinary tract infections, as a result of a comprehensive investigation of multiple malformations or during investigation of congenital heart disease. Major complications occur frequently and these patients are at risk for calculus formation, recurrent infection, chronic pyelonephritis and renal failure, and may ultimately require
transplantation. Early recognition, definitive repair and close followup postoperatively will hopefully decrease the complication significantly. On 2 occasions 1 patient presented with a unique constellation of anuria, hyperkalemia and obstruction of the solitary kidney. A percutaneous pyelostomy (with a venous intracatheter) was performed acutely, thus avoiding the need for a second surgical intervention. Unilateral agenesis with obstruction should be considered in the differential diagnosis of acute anuria, especially when occurring as an isolated incident. REFERENCES
1. Harrington, J. T. and Cohen, J. J.: Acute oliguria. New Engl. J. Med., 292: 89, 1975. 2. Museles, M., Gaudry, C. L., Jr. and Bason, W. M.: Renal anomalies in the newborn found by deep palpation. Pediatrics, 47: 97, 1971. 3. Campbell, M. F .: Congenital absence of one kidney; unilateral renal agenesis. Ann. Surg., 88: 1039, 1928. 4. Emanuel, B., Nachman, R., Aronson, N. and Weiss, H.: Congenital solitary kidney. A review of74 cases. Amer. J. Dis. Child., 127: 17, 1974.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
Pediatric Articles UNILATERAL RENAL AGENESIS PRESENTING AS ANDRIA JOSEPH GIANGIACOMO* From the Department of Pediatrics, Cardinal Glennon Memorial Hospital for Children, St. Louis University Medical School, St. Louis, Missouri ABSTRACT
The most common cause of sudden and total cessation of urine output is obstructive uropathy, usually at the bladder outlet. Bilateral ureteral obstruction is a much less common cause of anuria. In addition, unilateral obstruction in the presence of a solitary kidney must be considered in the differential diagnosis. Primary renal parenchymal disorders and pre-renal azotemia occasionally may be anuric but more commonly are oliguric. A case of unilateral renal agenesis presenting as anuria and obstruction of the solitary kidney is described. A 7-week-old male infant weighed 6 pounds, 14 ounces at birth and had been well until he became irritable with vomiting and diarrhea. A mass was palpable in the left upper quadrant. There was no evidence of dehydration. Initially, an excretory urogram (IVP) showed non-visualization and an x-ray of the abdomen 18 hours later showed a dilated left pelvis with non-visualization of the right kidney and no contrast material in the bladder (part A of figure). The renal scan showed an enlarged left kidney with dilatation and obstruction at the level of the pelvis, and the right kidney was not demonstrated. There was no urine obtained on catheterization of the
resolved but he became acutely anuric and hyperkalemic, and was managed with sodium polystyrene sulfonate. Although an IVP initially showed non-visualization, 10 hours later there was contrast material filling a dilated left pelvis. Under fluoroscopic visualization a No. 16 venous intracatheter was introduced percutaneously through the back with subsequent drainage of the pelvis. An IVP 1 month later showed decreased pelviocaliectasis, narrowing at the ureteropelvic junction and contrast material in the bladder, with the nephrostomy tube in the pelvis (part B of figure). The tube was removed 2 months later. A recent IVP, 3 months after removal of the nephrostomy
bladder. Serum sodium was 117 mEq. per 1., chloride 82 mEq. per 1., carbon dioxide 17.1 mEq. per 1., potassium 8.3 mEq. per 1., blood urea nitrogen (BUN) 38 mg. per cent and serum creatinine 5.8 gm. per cent. The hyperkalemia had decreased with oral sodium polystyrene sulfonate. Surgical exploration of the left kidney showed a ureteropelvic obstruction, which was removed. Postoperatively, the serum creatinine decreased to 1.1 mg. per cent and the BUN to 7.7 mg. per cent. The infant was hospitalized 3 weeks later for vomiting, diarrhea and dehydration. Subsequently, the gastroenteritis
tube, showed mild pelviocaliectasis (part C offigure). The child is 2 years old and doing well.
Accepted for publication June 18, 1976. * Requests for reprints: Cardinal Glennon Memorial Hospital for Children, 1465 S. Grand Blvd., St. Louis, Missouri 63104. 790
DISCUSSION
Anuria is defined as total cessation of urine output or less than 50 cc of urine production per day. 1 The most common cause is urinary tract obstruction at the level of the bladder outlet and this is seen in older men with prostatic disease. In these cases there is usually a history of gradual increase in difficulty in initiating and terminating the urinary stream. At times there are large and rapid swings in urinary output and this should increase the suspicion that bladder outlet obstruction is present. Bilateral ureteral obstruction is a much less
UNILATERAL RENAL AGENESIS PRESENTING AS ANDRIA
common cause of anuria and a complication of infiltrating malignancies. Acute tubular necrosis, bilateral cortical necrosis, acute glomerulonephritis and rapid progressive glomerulonephritis usually cause oliguria. Of these acute tubular necrosis is the most common cause of acute renal failure. The major precipitating events associated with acute tubular necrosis would be profound shock and extensive trauma, intravascular hemolysis, generalized sepsis, severe dehydration and a host of exogeneous toxins. The incidence of unilateral renal agenesis is estimated as 1.5 in 1,000 newborns, 2 while the autopsy occurrence is 1 in 1,824. 3 In a recent report of 74 cases a significant number had associated congenital anomalies involving vertebrae, cardiac anomalies, and long bones, hands and genitalia.• None presented with anuria. Most of the cases were discovered during evaluation for recurring urinary tract infections, as a result of a comprehensive investigation of multiple malformations or during investigation of congenital heart disease. Major complications occur frequently and these patients are at risk for calculus formation, recurrent infection, chronic pyelonephritis and renal failure, and may ultimately require
transplantation. Early recognition, definitive repair and close followup postoperatively will hopefully decrease the complication significantly. On 2 occasions 1 patient presented with a unique constellation of anuria, hyperkalemia and obstruction of the solitary kidney. A percutaneous pyelostomy (with a venous intracatheter) was performed acutely, thus avoiding the need for a second surgical intervention. Unilateral agenesis with obstruction should be considered in the differential diagnosis of acute anuria, especially when occurring as an isolated incident. REFERENCES
1. Harrington, J. T. and Cohen, J. J.: Acute oliguria. New Engl. J. Med., 292: 89, 1975. 2. Museles, M., Gaudry, C. L., Jr. and Bason, W. M.: Renal anomalies in the newborn found by deep palpation. Pediatrics, 47: 97, 1971. 3. Campbell, M. F .: Congenital absence of one kidney; unilateral renal agenesis. Ann. Surg., 88: 1039, 1928. 4. Emanuel, B., Nachman, R., Aronson, N. and Weiss, H.: Congenital solitary kidney. A review of74 cases. Amer. J. Dis. Child., 127: 17, 1974.
