Vol. 113, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
A NEW DIMENSION IN THE DIAGNOSIS OF POSTERIOR URETHRAL VALVES IN CHILDREN JAMES K. MOONEY, WALTER E. BERDON
AND
JOHN K. LATTIMER
From the Pediatric Urology and Radiology Services, Babies Hospital, Columbia-Presbyterian Medical Center, New York, New York
The classical signs and symptoms of posterior urethral valves in children are well known-bladder distension, dribbling, urinary infection, poor stream and failure to thrive. Unfortunately, these are not always early signs in infants and, if one waited for them, it would lead to serious delays in diagnosis. The signs and symptoms which should lead to the early suspicion of posterior urethral valves in infants based on less well known clinical findings include: 1) newborns with unexplained respiratory distress complicated by pneumomediastinum and pneumothorax, 2) infants who first present with flank masses caused by urinomas or urinary ascites and 3) critically ill, dehydrated infants when rapid loss of concentrating ability leads to a large output of dilute urine thought to be indicative of diabetes insipidus. The cases herein reported illustrate these new dimensions in the clinical presentation of valves. Recognition of these clues will lead to early diagnosis and, hopefully, improved survival of these infants. CASE REPORTS
Case 1. A 6 pound 14 ounce white male infant, 208-71-63, was born elsewhere to a 27-year-old prima gravida, after 36 weeks of gestation. There was no mention of the Apgar score nor the presence or quantity of amniotic fluid. Meconium aspiration was not noted and resuscitation was not needed. Mild respiratory distress was noted and a chest x-ray revealed pneumomediastinum and bilateral pneumothoraces of moderate size, which later resolved spontaneously (fig. 1, A). When the patient was 5 days old generalized edema and a palpable bladder were noted. The infant was hyponatremic, acidotic and azotemic, with a blood urea nitrogen (BUN) of 35 mg. per cent. An excretory urogram (IVP) was interpreted only as non-visualization. An indwelling catheter was placed and later a voiding cystourethrogram was done, which revealed posterior urethral valves without obvious reflux (fig. 1, B). The patient was transferred to our hospital when he was 12 days old. He was febrile, lethargic, acidotic and azotemic. The urine was infected and Accepted for publication June 21, 1974. Read at annual meeting of Western Section, American Urological Association, San Francisco, California, March 31-April 5, 1974. Supported in part by the Irene Heinz and John La Porte Given Foundation for Pediatric Urology at Columbia University.
the child had an inguinal hernia. A repeat IVP the next day revealed improved visualization with bilateral, tortuous, moderately dilated ureters. The valves were resected suprapubically. When the child was 10 months old bilateral massive reflux was found and bilateral ureteroneocystostomy was done, although the left kidney had not grown and showed poor function. The left kidney was removed when the child was 2 years old and, subsequently, he has done well. Case 2. A full-term white male infant 206-34-13, was born elsewhere after a reportedly normal pregnancy. Mild respiratory distress was noted and a chest x-ray revealed a right pneumothorax and pneumomediastinum (fig. 2), which resolved spontaneously. When he was 2½ weeks old the patient underwent exploration for torsion of the left testicle elsewhere, which was complicated by prolonged postoperative drainage of clear fluid. When he was 3 ½ weeks old he underwent right inguinal hernia repair but, postoperatively, the patient did poorly with failure to thrive, hyponatremia and acidosis. The infant was transferred to our hospital when he was 7 weeks old. He was acidotic and azotemic. An IVP showed poor visualization of what seemed to be hydronephrotic kidneys and, although not recognized as such, a right perirenal extravasation (urinoma) was present (fig. 3, A). The patient became distended and febrile. A voiding cystourethrogram revealed posterior urethral valves, bilateral reflux, right perirenal extravasation and ascites, the latter having developed 3 days after the IVP (fig. 3, B). The patient underwent right cutaneous loop ureterostomy, during which urine was noted in the retroperitoneum and the peritoneum. When he was 12 weeks the patient was less ill and underwent left loop cutaneous ureterostomy and fixation of the right (contralateral) testis. A bilateral ureteral reimplantation with suprapubic excision of the posterior urethral valves was done when the child was 22 months old and he is currently undergoing further surgical revision. Case 3. A 7 pound 9 ounce male newborn, 212-43-75, was the product ofa full-term uncomplicated pregnancy. Family history revealed that the father had undergone Y-V plasty of the bladder neck when he was 20 years old. There was no history of respiratory distress. The child dribbled urine and was colicky. At 5 weeks of age the patient was hospitalized elsewhere. He had protruding abdomen and flanks, and a BUN of 96 mg. per 272
DIAGNOSIS OF POSTERIOR URETHRAL VALVES IN CHILDREN
FIG. 1. Case 1. A, frontal chest film in newborn shows moderate sized right µueuu1e1cuunu1. tinum. B, voiding cystogram shows posterior urethral dilatation owing to valves.
FIG. 2. Case 2. Frontal chest film in newborn shows right pneumothorax and small pneumomediastinum, although not well seen.
cent. An IVP revealed poor visualization of moddilated ureters with bilateral perirenal extravasation and ascites 4). The was transferred to our hospital and, within 5 minutes of he had a cardiac and respiratory arrest. He survived and evaluation revealed that he was uremic 90 mg. per acidotic, hyponatremic and had infected urine. A cystourethrogram revealed posterior urethral valves without reflux 5). A percutaneous bilateral was done as an emergency and 2 days later a bilateral loop cutaneous ureterostomy was done. A transurethral resection of the urethral valves via urethrostorny and closure of the cutaneous ureterostomies were done when the child was 14 months old. The is now 4 years old and has sterile urine, normal BUN and prompt
excretion of contrast material 'Nith no evidence obstruction on IVP. Case 4. The patient, 198-03-70, full-term infant with an The mother did use diuretics the month of pregnancy because of excessive gain. Immediate neonatal was unrema.rkable. When the infant was 3 weeks diarrhea and and illness. Dehydration and output was noted. The patient was admitted to another H~•u..,cv,u for evaluation. He was remic and acidotic. He was treated with intravenous fluids and continued to have high outputs with low between and 1.010. Medical evaluation at that included renal concentration tests that strated an to concentrate urine. The patient was transferred to our he was 5 weeks old and He was
unm
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
A NEW DIMENSION IN THE DIAGNOSIS OF POSTERIOR URETHRAL VALVES IN CHILDREN JAMES K. MOONEY, WALTER E. BERDON
AND
JOHN K. LATTIMER
From the Pediatric Urology and Radiology Services, Babies Hospital, Columbia-Presbyterian Medical Center, New York, New York
The classical signs and symptoms of posterior urethral valves in children are well known-bladder distension, dribbling, urinary infection, poor stream and failure to thrive. Unfortunately, these are not always early signs in infants and, if one waited for them, it would lead to serious delays in diagnosis. The signs and symptoms which should lead to the early suspicion of posterior urethral valves in infants based on less well known clinical findings include: 1) newborns with unexplained respiratory distress complicated by pneumomediastinum and pneumothorax, 2) infants who first present with flank masses caused by urinomas or urinary ascites and 3) critically ill, dehydrated infants when rapid loss of concentrating ability leads to a large output of dilute urine thought to be indicative of diabetes insipidus. The cases herein reported illustrate these new dimensions in the clinical presentation of valves. Recognition of these clues will lead to early diagnosis and, hopefully, improved survival of these infants. CASE REPORTS
Case 1. A 6 pound 14 ounce white male infant, 208-71-63, was born elsewhere to a 27-year-old prima gravida, after 36 weeks of gestation. There was no mention of the Apgar score nor the presence or quantity of amniotic fluid. Meconium aspiration was not noted and resuscitation was not needed. Mild respiratory distress was noted and a chest x-ray revealed pneumomediastinum and bilateral pneumothoraces of moderate size, which later resolved spontaneously (fig. 1, A). When the patient was 5 days old generalized edema and a palpable bladder were noted. The infant was hyponatremic, acidotic and azotemic, with a blood urea nitrogen (BUN) of 35 mg. per cent. An excretory urogram (IVP) was interpreted only as non-visualization. An indwelling catheter was placed and later a voiding cystourethrogram was done, which revealed posterior urethral valves without obvious reflux (fig. 1, B). The patient was transferred to our hospital when he was 12 days old. He was febrile, lethargic, acidotic and azotemic. The urine was infected and Accepted for publication June 21, 1974. Read at annual meeting of Western Section, American Urological Association, San Francisco, California, March 31-April 5, 1974. Supported in part by the Irene Heinz and John La Porte Given Foundation for Pediatric Urology at Columbia University.
the child had an inguinal hernia. A repeat IVP the next day revealed improved visualization with bilateral, tortuous, moderately dilated ureters. The valves were resected suprapubically. When the child was 10 months old bilateral massive reflux was found and bilateral ureteroneocystostomy was done, although the left kidney had not grown and showed poor function. The left kidney was removed when the child was 2 years old and, subsequently, he has done well. Case 2. A full-term white male infant 206-34-13, was born elsewhere after a reportedly normal pregnancy. Mild respiratory distress was noted and a chest x-ray revealed a right pneumothorax and pneumomediastinum (fig. 2), which resolved spontaneously. When he was 2½ weeks old the patient underwent exploration for torsion of the left testicle elsewhere, which was complicated by prolonged postoperative drainage of clear fluid. When he was 3 ½ weeks old he underwent right inguinal hernia repair but, postoperatively, the patient did poorly with failure to thrive, hyponatremia and acidosis. The infant was transferred to our hospital when he was 7 weeks old. He was acidotic and azotemic. An IVP showed poor visualization of what seemed to be hydronephrotic kidneys and, although not recognized as such, a right perirenal extravasation (urinoma) was present (fig. 3, A). The patient became distended and febrile. A voiding cystourethrogram revealed posterior urethral valves, bilateral reflux, right perirenal extravasation and ascites, the latter having developed 3 days after the IVP (fig. 3, B). The patient underwent right cutaneous loop ureterostomy, during which urine was noted in the retroperitoneum and the peritoneum. When he was 12 weeks the patient was less ill and underwent left loop cutaneous ureterostomy and fixation of the right (contralateral) testis. A bilateral ureteral reimplantation with suprapubic excision of the posterior urethral valves was done when the child was 22 months old and he is currently undergoing further surgical revision. Case 3. A 7 pound 9 ounce male newborn, 212-43-75, was the product ofa full-term uncomplicated pregnancy. Family history revealed that the father had undergone Y-V plasty of the bladder neck when he was 20 years old. There was no history of respiratory distress. The child dribbled urine and was colicky. At 5 weeks of age the patient was hospitalized elsewhere. He had protruding abdomen and flanks, and a BUN of 96 mg. per 272
DIAGNOSIS OF POSTERIOR URETHRAL VALVES IN CHILDREN
FIG. 1. Case 1. A, frontal chest film in newborn shows moderate sized right µueuu1e1cuunu1. tinum. B, voiding cystogram shows posterior urethral dilatation owing to valves.
FIG. 2. Case 2. Frontal chest film in newborn shows right pneumothorax and small pneumomediastinum, although not well seen.
cent. An IVP revealed poor visualization of moddilated ureters with bilateral perirenal extravasation and ascites 4). The was transferred to our hospital and, within 5 minutes of he had a cardiac and respiratory arrest. He survived and evaluation revealed that he was uremic 90 mg. per acidotic, hyponatremic and had infected urine. A cystourethrogram revealed posterior urethral valves without reflux 5). A percutaneous bilateral was done as an emergency and 2 days later a bilateral loop cutaneous ureterostomy was done. A transurethral resection of the urethral valves via urethrostorny and closure of the cutaneous ureterostomies were done when the child was 14 months old. The is now 4 years old and has sterile urine, normal BUN and prompt
excretion of contrast material 'Nith no evidence obstruction on IVP. Case 4. The patient, 198-03-70, full-term infant with an The mother did use diuretics the month of pregnancy because of excessive gain. Immediate neonatal was unrema.rkable. When the infant was 3 weeks diarrhea and and illness. Dehydration and output was noted. The patient was admitted to another H~•u..,cv,u for evaluation. He was remic and acidotic. He was treated with intravenous fluids and continued to have high outputs with low between and 1.010. Medical evaluation at that included renal concentration tests that strated an to concentrate urine. The patient was transferred to our he was 5 weeks old and He was
unm
