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Ureteropelvic junction obstructions: Prenatal diagnosis andneonatal surgery in 47 cases
Ureteropelvic Junction Obstructions: Prenatal Diagnosis and Neonatal Surgery in 47 Cases By J.M. Guys, F. Borella, and G. M o n f o r t Marseille, France 9 In a series of 150 patients with congenital urologic disorders diagnosed in utero and managed in the neonatal period from 1980 to 1985, 48 cases w e r e ureteropelvic junction obstructions. One was a false positive, and 47 w e r e documented pyelocaliceal distension and retention; 12 of them w e r e bilateral. Five cases had a spontaneous resolution. Three had a nephrectomy performed (destroyed kidney). Fifty kidneys have been operated on (Andorson-Hynes dysmembered pyeloplasty). Ninety percent of the cases are reported as clinically, biologically, and radiologically fair. Six percent postoperative complications are reported. The authors pointed out the great interest in neonatal repair of this condition, using microsurgical techniques without stent or nephrostomy. A comparison is made of the overall results with an identical series of older patients operated on during the same period. 9 1988 by Grune & Stratton, Inc. INDEX W O R D S : Urinary tract malformations.
I N C E OUR FIRST R E P O R T in 19781 up to the end of December 1985, 153 patients have been observed in our institution with a prenatal diagnosis of obstructive uropathy. Forty-seven of these (31%) were pyelocaliceal retention secondary to pelviureteric junction obstruction confirmed in the postnatal period. During the same time, the number of ureteropelvic obstructions diagnosed and admitted on a more conventional basis has continuously decreased, except in 1985 (Fig 1).
S
MATERIALS AND METHODS Diagnosis of unilateral or bilateral kidney distension of the fetus was obtained in 48 pregnant women, usually around the seventh month of pregnancy. In all cases but one (early delivery) these pregnancies have been managed conservatively and serial ultrasound performed every 2 weeks. After a normal delivery, diagnosis was immediately confirmed by direct scanning, and intravenous pyelography (IVP) and cystogram performed in the first 2 weeks of life. 99-tc Labeled DTPA renogram with lasix injection was obtained in 20 patients. Fourteen patients have had delayed exploration; one presented with a documented urinary tract infection. The false-positive rate was low: one patient (2%). Twenty-five percent of these obstructions were bilateral, usually asymetrical. Associated malformations were reported in nine cases (19%).
From the Department of Pediatric Surgery, School of Medicine of Marseille, France. Address reprint requests to J.M. Guys, Department of Pediatric Surgery, School of Medicine of Marseille, Professor M. Carcassonne, Boulevard Jean Moulin, 13005 Marseille, France. 9 1988 by Grune & Stratton, Inc. 0022-3468/88/2302-0016503.00/0 156
There were seven cases of vesicoureteral reflux: stage 4, one case; stage 3, three cases; stage 2, two cases; and stage 1, one case; one mega ureter; and one controlateral multicystic kidney. Treatment has been initially conservative in 19 (40.5%) patients, but a new evaluation after 3 to 6 months showed spontaneous improvement in only one (2%). Four of the bilateral conditions demonstrated unilateral improvement. Eighteen patients (94.5%) of that group were operated on after a 6-month follow-up. Primary surgical treatment during the first month of life has been performed in the 28 other patients (59.5%: Table 1). A subcostal horizontal approach was used in 32 cases, posterior vertical lombotomy in seven (one bilateral) and transperitoneal in six (bilateral). Fifty dismembered pyeloplasties and one vascular switch were performed, without extensive resection of the redundant pelvis, using microsurgical techniques in all cases, with 7 x 0 PDS or Maxon monofilament suture. Three primary nephrectomies and one secondary were performed due to insufficient renal function (DTPA). Urinary diversion was performed for five kidneys; two for technical reasons (stent for small ureter), two for bilateral lesions (one side was drained by a "security" nephrostomy) and one for concomitant ureteroneocystostomy for vesicoureteral gross reflux. Surgical treatment of reflux was performed during the same operation in two cases in which the possibilities of spontaneous cure were judged poor by endoscopy, and two others after a 6-month period of follow-up. Three (two grade 2 and one grade 1) were followed conservatively and disappeared within a year, as well as did the case of megaureter. RESULTS
Follow-up of these series ranged from 2 to 6 years. In 90% of the patients a significant reduction of pelvicaliceal distension, a good appearance of the ureter, and normal kidney function were obtained. Eight percent have still a delayed secretion and 2% a persistant pyelocaliceal distension. Six percent were complicated by a postoperative leak that was successfully treated by a ureteral catheter inserted at cystoscopy, and left in place between three and five days. The mean hospitalization time was five days; none needed a reoperation. In all cases except one, the urine remained sterile and postoperative antibacterial treatment was discontinued after 2 weeks. DISCUSSION
Early delivery in unilateral cases of pyelocaliceal retention is usually not recommended. 13 In our series, one bilateral case was delivered at 32 weeks. The intrauterine relief of obstruction has not yet been proved to have any effect on further quality of evolution. 46 We, therefore, recommend that no attempt of modification of normal evolution should be Journal of Pediatric Surgery, Vol 23, No 2 (February), 1988: pp 156-158
URETEROPELVIC JUNCTION OBSTRUCTIONS
157
Table 2. C o m p a r a t i v e S t u d y From 1981 to 1 9 8 4
aal
/ f
Antenatal Diagnosis n = 42 (%)*
Postnatal diagnosis n = 38 (%)
Pyelonephritis
1 (2.3)
16 (42.1)
Nephrectomy
4
5
Delayed secretion Persistent dilatation
2 . (7.1)
7 3 (26.3)
ANTE NATAL DIAGNOSIS
1981 to 1984
20 UPO
pn
OK > 3 6 mo
58
(92.8)
(73.7)
IO
19BI
19B2
19B3
1984
1985
Fig 1. U r e t e r o p e l v i c junction obstruction (UPOI. an. a n t e n a t a l diagnosis (-); pn, postnatal diagnosis (-).
made in that period. However, a consultation with the team of pediatric surgeons who are going to be in charge of the neonate has a very great psychologic value. Knowledge of the hospital structures, postnatal program of exploration, and timing of operations, if any, allows one to dedramatize the situation and to tell the parents and the obstetrician that the emergency situation will only exceptionally require an early mother-child separation. A safe and convenient exploration can be done within the first 2 weeks of life. Echography, retrograde or suprapubic cystography, and IVP are the most usual modalities for radiologic evaluation of the cases. Antegrade pyelography is used in poorly functional grossly dilated units. 7'8 The DTPA scan in the neonatal period to determine separate index fixation of both kidneys as well as physiopathology of excretion (with or without Lasix injection) has been used in 50% of our cases. However, interpretation of the results are often difficult in the presence of a widely dilated system. The rapid modification of glomerular filtration rate in the first week of life is to be taken into consideration for interpretation of results and evaluation of the seriousness of the situation. Lasix test was considered positive in 50% of the 19 patients who were primarily treated conservatively. All but one underwent subsequent pyeloplasty after 6 Table 1. T r e a t m e n t
1979 to 1985
in 4 7 P a t i e n t s Unilateral
Bilateral
Lesions
Lesions
Medical treatment first
7
12
Primary surgery (2 mo)
28
O
Surgery after follow-up
10
8
months for a significant increase of the retention and distension of renal cavities. DTPA or DMSA evaluation of separate renal function 6 months to 4 years after surgical correction of the obstruction was done in all the patients and compared with a similar series of children in which the diagnosis was obtained later with more conventional symptoms (urinary tract infection, hematuria, flank mass, and pain) during the same period (Table 2). Ninety-three percent satisfactory results in 42 kidneys operated in the neonatal period were observed. This was only 73.7% in the other group. Delayed secretion and stasis were noted to be 7.1% compared with 26.3%. No postoperative urinary tract infection was documented in either group. In the absence of severe infection or prematurity we do not advocate the use of preoperative percutaneous nephrostomy as do others. 9 Infection, retraction of renal pelvis and more difficult surgery are the usual consequences of preoperative external drainage. We prefer immediate surgical correction, with the help of a stent nephrostomy if the situation is difficult (six kidneys, 12%). Anderson-Hynes dismembered nonstent pyeloplasty has been the method of choice in our hands, always performed under microsurgical conditions. Posterior lombotomy was prefered to the transperitoneal approach in bilateral conditions. Otherwise we still prefer the transverse split incision. CONCLUSION
Neonatal repair of urologic obstructive conditions is advocated now by many authors. 6'91] It seems that early relief of severe obstructions gives a better chance of subsequent significant increase of creatinine clearance in most instances. Antenatal diagnosis is a major advance in pediatric urology in the past 10 years. It allows early recognition before postnatal complications. Precise sutures with an operating microscope may avoid the use of a stent or nephrostomy.
REFERENCES 1. Morisson-Lacombe G, Monfort G, Giaufre E, et al: Zwei falle pranataler diagnostik yon ureterozelen bei doppelbildung. Aktuelle Urologie 10:29-33, 1979
2. Hellstrom WJG, Kogan BA, Jeffrey RB, et al: The natural history of prenatal hydronephrosis with normal amounts of amniotic fluid. J Urol 132:947-950, 1984
158
3. Hobbins JC, Romero R, Grannum P, et al: Antenatal diagnosis of renal anomalies with ultrasound. Am J Obstet Gynecol 148:868877, 1984 4. Kramer SA: Current status for fetal intervention for congenital hydronephrosis. J Urol 130:641-646, 1983 5. Glick PL, Harrisson MR, Golbus MS, et al: Management of the fetus with congenital hydronephrosis. Part II. J Pediatr Surg 20:376-387, 1985 6. Flake W, Harrisson MR, Sauer L, et al: Ureteropelvic junction obstruction in the fetus. J Pediatr Surg 21:1058-1063, 1986 7. Toma P, Lituania MA: Urinary tract malformations: Prenatal diagnosis and neonatal follow-up by sonography. Ann Radiol (Paris) 27:215-222, 1984
GUYS, BORELLA, AND MONFORT
8. Coolsaet BL: Urodynamic approach of the upper urinary tract by percutaneous puncture nephrostomy. Acta Urol Belgica 51:458472, 1983 9. Heloury Y, Schmitt P, Allouch G, et al: Treatment of neonatal hydronephrosis by malformations of the ureteropelvic junction. Eur Urol 12:224-229, 1986 10. King LR, Coughlin PWF, Bloch EC, et al: The case for immediate pyeloplastie in the neonate with ureteropelvic junction obstruction. J Urol 132:725-728, 1984 11. Evans BB: Obstructive uropathy in the neonate. Clin Perinatol 8:273-287, 1981
I N C E OUR FIRST R E P O R T in 19781 up to the end of December 1985, 153 patients have been observed in our institution with a prenatal diagnosis of obstructive uropathy. Forty-seven of these (31%) were pyelocaliceal retention secondary to pelviureteric junction obstruction confirmed in the postnatal period. During the same time, the number of ureteropelvic obstructions diagnosed and admitted on a more conventional basis has continuously decreased, except in 1985 (Fig 1).
S
MATERIALS AND METHODS Diagnosis of unilateral or bilateral kidney distension of the fetus was obtained in 48 pregnant women, usually around the seventh month of pregnancy. In all cases but one (early delivery) these pregnancies have been managed conservatively and serial ultrasound performed every 2 weeks. After a normal delivery, diagnosis was immediately confirmed by direct scanning, and intravenous pyelography (IVP) and cystogram performed in the first 2 weeks of life. 99-tc Labeled DTPA renogram with lasix injection was obtained in 20 patients. Fourteen patients have had delayed exploration; one presented with a documented urinary tract infection. The false-positive rate was low: one patient (2%). Twenty-five percent of these obstructions were bilateral, usually asymetrical. Associated malformations were reported in nine cases (19%).
From the Department of Pediatric Surgery, School of Medicine of Marseille, France. Address reprint requests to J.M. Guys, Department of Pediatric Surgery, School of Medicine of Marseille, Professor M. Carcassonne, Boulevard Jean Moulin, 13005 Marseille, France. 9 1988 by Grune & Stratton, Inc. 0022-3468/88/2302-0016503.00/0 156
There were seven cases of vesicoureteral reflux: stage 4, one case; stage 3, three cases; stage 2, two cases; and stage 1, one case; one mega ureter; and one controlateral multicystic kidney. Treatment has been initially conservative in 19 (40.5%) patients, but a new evaluation after 3 to 6 months showed spontaneous improvement in only one (2%). Four of the bilateral conditions demonstrated unilateral improvement. Eighteen patients (94.5%) of that group were operated on after a 6-month follow-up. Primary surgical treatment during the first month of life has been performed in the 28 other patients (59.5%: Table 1). A subcostal horizontal approach was used in 32 cases, posterior vertical lombotomy in seven (one bilateral) and transperitoneal in six (bilateral). Fifty dismembered pyeloplasties and one vascular switch were performed, without extensive resection of the redundant pelvis, using microsurgical techniques in all cases, with 7 x 0 PDS or Maxon monofilament suture. Three primary nephrectomies and one secondary were performed due to insufficient renal function (DTPA). Urinary diversion was performed for five kidneys; two for technical reasons (stent for small ureter), two for bilateral lesions (one side was drained by a "security" nephrostomy) and one for concomitant ureteroneocystostomy for vesicoureteral gross reflux. Surgical treatment of reflux was performed during the same operation in two cases in which the possibilities of spontaneous cure were judged poor by endoscopy, and two others after a 6-month period of follow-up. Three (two grade 2 and one grade 1) were followed conservatively and disappeared within a year, as well as did the case of megaureter. RESULTS
Follow-up of these series ranged from 2 to 6 years. In 90% of the patients a significant reduction of pelvicaliceal distension, a good appearance of the ureter, and normal kidney function were obtained. Eight percent have still a delayed secretion and 2% a persistant pyelocaliceal distension. Six percent were complicated by a postoperative leak that was successfully treated by a ureteral catheter inserted at cystoscopy, and left in place between three and five days. The mean hospitalization time was five days; none needed a reoperation. In all cases except one, the urine remained sterile and postoperative antibacterial treatment was discontinued after 2 weeks. DISCUSSION
Early delivery in unilateral cases of pyelocaliceal retention is usually not recommended. 13 In our series, one bilateral case was delivered at 32 weeks. The intrauterine relief of obstruction has not yet been proved to have any effect on further quality of evolution. 46 We, therefore, recommend that no attempt of modification of normal evolution should be Journal of Pediatric Surgery, Vol 23, No 2 (February), 1988: pp 156-158
URETEROPELVIC JUNCTION OBSTRUCTIONS
157
Table 2. C o m p a r a t i v e S t u d y From 1981 to 1 9 8 4
aal
/ f
Antenatal Diagnosis n = 42 (%)*
Postnatal diagnosis n = 38 (%)
Pyelonephritis
1 (2.3)
16 (42.1)
Nephrectomy
4
5
Delayed secretion Persistent dilatation
2 . (7.1)
7 3 (26.3)
ANTE NATAL DIAGNOSIS
1981 to 1984
20 UPO
pn
OK > 3 6 mo
58
(92.8)
(73.7)
IO
19BI
19B2
19B3
1984
1985
Fig 1. U r e t e r o p e l v i c junction obstruction (UPOI. an. a n t e n a t a l diagnosis (-); pn, postnatal diagnosis (-).
made in that period. However, a consultation with the team of pediatric surgeons who are going to be in charge of the neonate has a very great psychologic value. Knowledge of the hospital structures, postnatal program of exploration, and timing of operations, if any, allows one to dedramatize the situation and to tell the parents and the obstetrician that the emergency situation will only exceptionally require an early mother-child separation. A safe and convenient exploration can be done within the first 2 weeks of life. Echography, retrograde or suprapubic cystography, and IVP are the most usual modalities for radiologic evaluation of the cases. Antegrade pyelography is used in poorly functional grossly dilated units. 7'8 The DTPA scan in the neonatal period to determine separate index fixation of both kidneys as well as physiopathology of excretion (with or without Lasix injection) has been used in 50% of our cases. However, interpretation of the results are often difficult in the presence of a widely dilated system. The rapid modification of glomerular filtration rate in the first week of life is to be taken into consideration for interpretation of results and evaluation of the seriousness of the situation. Lasix test was considered positive in 50% of the 19 patients who were primarily treated conservatively. All but one underwent subsequent pyeloplasty after 6 Table 1. T r e a t m e n t
1979 to 1985
in 4 7 P a t i e n t s Unilateral
Bilateral
Lesions
Lesions
Medical treatment first
7
12
Primary surgery (2 mo)
28
O
Surgery after follow-up
10
8
months for a significant increase of the retention and distension of renal cavities. DTPA or DMSA evaluation of separate renal function 6 months to 4 years after surgical correction of the obstruction was done in all the patients and compared with a similar series of children in which the diagnosis was obtained later with more conventional symptoms (urinary tract infection, hematuria, flank mass, and pain) during the same period (Table 2). Ninety-three percent satisfactory results in 42 kidneys operated in the neonatal period were observed. This was only 73.7% in the other group. Delayed secretion and stasis were noted to be 7.1% compared with 26.3%. No postoperative urinary tract infection was documented in either group. In the absence of severe infection or prematurity we do not advocate the use of preoperative percutaneous nephrostomy as do others. 9 Infection, retraction of renal pelvis and more difficult surgery are the usual consequences of preoperative external drainage. We prefer immediate surgical correction, with the help of a stent nephrostomy if the situation is difficult (six kidneys, 12%). Anderson-Hynes dismembered nonstent pyeloplasty has been the method of choice in our hands, always performed under microsurgical conditions. Posterior lombotomy was prefered to the transperitoneal approach in bilateral conditions. Otherwise we still prefer the transverse split incision. CONCLUSION
Neonatal repair of urologic obstructive conditions is advocated now by many authors. 6'91] It seems that early relief of severe obstructions gives a better chance of subsequent significant increase of creatinine clearance in most instances. Antenatal diagnosis is a major advance in pediatric urology in the past 10 years. It allows early recognition before postnatal complications. Precise sutures with an operating microscope may avoid the use of a stent or nephrostomy.
REFERENCES 1. Morisson-Lacombe G, Monfort G, Giaufre E, et al: Zwei falle pranataler diagnostik yon ureterozelen bei doppelbildung. Aktuelle Urologie 10:29-33, 1979
2. Hellstrom WJG, Kogan BA, Jeffrey RB, et al: The natural history of prenatal hydronephrosis with normal amounts of amniotic fluid. J Urol 132:947-950, 1984
158
3. Hobbins JC, Romero R, Grannum P, et al: Antenatal diagnosis of renal anomalies with ultrasound. Am J Obstet Gynecol 148:868877, 1984 4. Kramer SA: Current status for fetal intervention for congenital hydronephrosis. J Urol 130:641-646, 1983 5. Glick PL, Harrisson MR, Golbus MS, et al: Management of the fetus with congenital hydronephrosis. Part II. J Pediatr Surg 20:376-387, 1985 6. Flake W, Harrisson MR, Sauer L, et al: Ureteropelvic junction obstruction in the fetus. J Pediatr Surg 21:1058-1063, 1986 7. Toma P, Lituania MA: Urinary tract malformations: Prenatal diagnosis and neonatal follow-up by sonography. Ann Radiol (Paris) 27:215-222, 1984
GUYS, BORELLA, AND MONFORT
8. Coolsaet BL: Urodynamic approach of the upper urinary tract by percutaneous puncture nephrostomy. Acta Urol Belgica 51:458472, 1983 9. Heloury Y, Schmitt P, Allouch G, et al: Treatment of neonatal hydronephrosis by malformations of the ureteropelvic junction. Eur Urol 12:224-229, 1986 10. King LR, Coughlin PWF, Bloch EC, et al: The case for immediate pyeloplastie in the neonate with ureteropelvic junction obstruction. J Urol 132:725-728, 1984 11. Evans BB: Obstructive uropathy in the neonate. Clin Perinatol 8:273-287, 1981