International Journal of Urology (2007) 14, 388392

doi: 10.1111/j.1442-2042.2006.01726.x

Original Article: Clinical Investigation

Urological emergency in neonates with congenital hydronephrosis

Kenji Shimada, Fumi Matsumoto, Mari Kawagoe and Futoshi Matsui
Department of Urology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan

Objective: It is well described that unilateral pelviureteric junction obstruction (PUJO) is a benign condition, because the dilatation resolves spontaneously and the function does not decrease in most of the kidneys. However, there is exceptional PUJO that requires emergent treatment in neonatal periods. The aim of this article is to report the urological emergency and management in neonates with PUJO. Materials and Methods: Nine children (seven boys and two girls) with PUJO who underwent neonatal emergent treatment during the last 13 years were reviewed. Renal function was evaluated according to decay curve of serum creatinine (SCr) levels corresponding to gestational age (GA) at delivery. Physical examination, ultrasonographic monitoring, and chest and abdominal plain radiographs were repeated in each neonate. Results: Eight patients were detected prenatally. In ve patients, multicystic dysplastic kidney (MCDK) was demonstrated on the contralateral side. Three patients underwent percutaneous puncture of fetal hydronephrosis. Decrease of amniotic uid was evident in three fetuses. Indications for emergent treatment included mass effect from hydronephrosis in three patients, renal dysfunction in ve, and severe urinary tract infection in one. During neonatal periods, a percutaneous nephrostomy tube was placed in seven, and open nephrostomy in one with anorectal malformation. Repeated punctures of the dilated renal pelvis were done in one patient. Renal function after pyeloplasty was stable in eight patients, while it was moderately decreased in one who was associated with oligohydramnios in utero. Conclusion: Indications for emergent treatment in neonates with PUJO included mass effect from giant hydronephrosis, renal dysfunction and severe urinary tract infection. At birth, respiratory and circulatory conditions must rst be stabilized. In neonates with hydronephrosis of the solitary kidney or severe bilateral PUJO, serial SCr should be monitored to evaluate renal function. Decrease of amniotic uid suggested renal functional compromise that would not recover after urological management. Key words: mass effect, neonatal emergency, pelviureteric junction obstruction (PUJO), prenatal diagnosis, renal dysfunction.

Introduction
According to the widespread use of prenatal ultrasonography, more than half of congenital hydronephrosis (pelviureteric junction obstruction, PUJO) are detected before clinical symptoms or signs present themselves.1,2 This dramatic change of clinical presentation has led to confusion as to the evaluation and treatment of this entity. It still remains unsolved how to identify prenatal PUJO that requires surgery.3 It is well described that unilateral PUJO is the benign condition that carries a low risk for renal damage. Even in the most severe grade of PUJO, urgent studies and intervention are deferred for several weeks after birth, because the condition resolves spontaneously and the function does not decrease in most of the kidneys.4,5 There are, however, exceptional PUJO that require emergent treatment in neonatal periods. They include mass effect from compression of huge hydronephrosis, renal dysfunction and severe urinary tract infection.6 Active participation of pediatric urologists, although limited to some institutes associated with maternal units, has led to earlier intervention and improvement of these patients. The aim of this article is to report on urological emergency and management in neonates with PUJO.

Materials and methods

Of the 562 children with PUJO who were treated 19912004, nine children (1.6%), seven boys and two girls, underwent emergent urinary tract drainage during neonatal periods. We retrospectively reviewed the medical records of these children with respect to clinical presentation, prenatal courses, mode of delivery, emergent treatment and follow-up renal function. Renal function during neonatal periods was evaluated according to decay curve of serum creatinine (SCr) levels corresponding to his or her gestational age (GA) at delivery.7 When SCr levels continued to rise, or the fall of Scr in the rst few days of life was slower than that of a control of the same GA, urgent drainage of hydronephrosis was indicated. Physical examination, ultrasonographic monitoring, and chest and abdominal plain radiographs were repeated in each neonate. A voiding cystourethrogram (VCUG) was done in all patients to exclude the association of vesicoureteral reux or infravesical obstruction. The average follow-up period was 6 years and 7 months (range, 3 years and 8 months to 10 years and 6 months).

Results
Clinical presentation
Among the patients, eight were detected by prenatal ultrasonography as having hydronephrosis between GA weeks 20 and 37 (Table 1). The contralateral kidney was normal in three patients, while in another ve multicystic dysplastic kidney (MCDK) was suspected prenatally and conrmed postnatally. Three of them underwent percutaneous puncture of fetal hydronephrosis to decompress the giant mass. Analysis of
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Correspondence: Kenji Shimada MD PhD, Department of Urology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo-cho, Izumi, Osaka, Japan. Email: shimada@mch.pref.osaka.jp Received 15 August 2006; accepted 6 November 2006.

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Neonates with congenital hydronephrosis

AFV, amniotic uid volume; ARM, anorectal malformation; Bil, bilateral; c/s, caesarean section; F, female; GA, gestational age; L, left; M, male; R, right; PNS, percutaneous nephrostomy; Tx, treatment; UTI, urinary tract infection; w, week.

Decreased

punctured urine revealed an electrolyte content equivalent to that of good prognosis.8 Oligohydramnios or decrease of amniotic uid was evident in three fetuses. One neonate who was not detected prenatally showed abdominal distention and presented feeding intolerance from day 4 after birth. This child with Kabuki make-up syndrome died from pneumonia at the age of 3 years.

Kabuki make-up, died

Renal

VACTER syndrome ARM, ectopia Good Good Good Good Good

Others

Good Good

Good

Mode of delivery
Cesarean section was elected in ve patients due to the distended abdomen from huge hydronephrosis inappropriate for vaginal delivery. Two patients were induced for early delivery at GA 37 weeks.

Pyeloplasty (35) Pyeloplasty (1y5m) Pyeloplasty (80) Pyeloplasty (26)

Pyeloplasty (25)

R-PNS (21), L-PNS (33) Pyeloplasty (42)

Denitive Tx function

Emergency drainage
Indications for neonatal emergent treatment included mass effect from giant hydronephrosis in three patients, renal dysfunction in ve, and severe urinary tract infection in one. The rst three patients showed respiratory distress or feeding intolerance by the mass effect of huge hydronephrosis. Among the ve children who presented with renal dysfunction during neonatal periods, four had grade 4 hydronephrosis in the solitary kidney. One female patient had bilateral hydronephrosis associated with oligohydramnios as early as GA 35 weeks. The last male neonate who underwent puncture of the left kidney on the day of delivery showed deterioration of hydronephrosis in association with febrile urinary tract infection on the 21st day after birth. Percutaneous nephrostomy was performed in seven patients during days 125 after birth. Repeated punctures of the dilated renal pelvis was done in one patient. An open nephrostomy was performed in one neonate with anorectal malformation to create a colostomy on the rst day of life.

Bil. Pyeloplasty (51) c/s (36 w)

Nephrostomy (0)

Vaginal (35 w) c/s (34 w) Induced (37 w) Induced (37 w)

c/s (37 w) Vaginal

PNS (25) PNS (2) PNS (6) PNS (1)

Shunt (29 w, 31 w)

Puncture (35 w)

Puncture (2 9w)

Prenatal Tx

c/s (30 w)

c/s (37 w)

Delivery (days)

Puncture (1, 21)

Neonatal Tx (days)

PNS (3) PNS (13)

Pyeloplasty
Pyeloplasty was performed in all children except one (case 7) through simple pelviureteric anastomosis. In two children, the nephrostomy tube was removed approximately 1 month after the placement, because antegrade pyelography showed good passage of contrast medium through the pelviureteric junction. Severe hydronephrosis recurred in both patients 1 month and 5 months after removal of the catheter. Both children underwent pyeloplasty at the ages of 2 months and 6 months, because they showed gradual increase of SCr and electrolyte imbalance. At the time of writing, one female had not yet been operated on because her left ureter was hypoplastic from the PUJ to the lower most portion.

Detection

GA 28 w Day 4

GA 20 w GA 28 w GA 25 w GA 37 w

GA 25 w

GA 23 w Renal dysfunction Oligoamnios

Table 1 Clinical presentation of nine cases of congenital hydronephrosis

Decreased AFV

Mass effect Renal dysfunction Renal dysfunction Renal dysfunction

Renal dysfunction

Oligoamnios

Fever, abd.distension

Signs and symptoms

Respiratory distress Feeding intolerance

Respiratory distress Anuria

GA 36 w

Mass effect Mass effect

UTI and mass

Prognosis of renal function

Renal function after pyeloplasty was stable in eight patients, while it was moderately decreased in one who was associated with oligohydramnios in utero.

Cause of emergency

L R, solitary L, solitary L, solitary

R L, solitary

L, solitary

Laterality

Bilateral

Case presentation
Case 1
A male newborn was temporally intubated from respiratory distress on the rst day of life due to elevation of the diaphragma compressed by a right huge hydronephrosis. Birthweight was 2980 g, and the Apgar scores were 6 and 7. At GA 28 weeks, he was detected as having giant hydronephrosis by prenatal ultrasonography. Although percutaneous puncture of fetal hydronephrosis was done twice at GA 29 weeks and
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Sex

M M

M M M M

Case

Y.M. K.I.

1 2

3 4 5 6

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K.C.

M.I. K.K. J.Y. S.Y.

Y.N.

Y.S.

K SHIMADA ET AL.

Fig. 1 Case 1. Prenatal ultrasonography revealed huge hydronephrosis in a fetus of gestational age (GA) 35 weeks.

GA 35 weeks, distention of the renal pelvis recurred quickly (Fig. 1). Cesarean section was indicated at GA 37 weeks. Chest and abdominal radiographs revealed displacement of the right diaphragma from beneath (Fig. 2). After removal of the intubated tube on the second day, he gradually showed feeding intolerance. Percutaneous nephrostomy was done on the third day of life, and pyeloplasty was performed on the 25th day. Renal scintigraphy at the age of 9 years revealed good function of the right kidney with split uptake equivalent to that of the contralateral normal kidney.

Fig. 2 Case 1. Chest and abdominal plain radiograph showed elevation of right diaphragma and displacement of intestinal shadows to the left.

Case 2
A 4-week-old male was referred to us with a nephrostomy of the right kidney. He was detected by prenatal ultrasonography at GA 28 weeks in the previous hospital as having right hydronephrosis and left multicystic dysplastic kidney (MCDK). Fetal intervention was tried , but was not effective (no precise information). Cesarean section was indicated at GA 34 weeks. Birthweight was 1860 g, and the Apgar scores were 8 and 9. On the second day of life, his SCr rose up to 2.2 mg/dL, and electrolyte imbalance became apparent. After placement of a nephrostomy tube in the right kidney, SCr dropped to 0.4 mg/dL upon referral to us. The nephrostomy tube was removed at 6 weeks of age because antegrade pyelography showed good passage at the PUJ (Fig. 3). He was well with mild pelvicalyceal dilatation, and showed stable renal function until 6 months of age when hydronephrosis aggravated again to grade 4 (Fig. 4). Pyeloplasty was performed , and postoperative course was uneventful. At the age of 8 years, his SCr was 0.48 mg/dL, and his left MCDK regressed spontaneously.

Discussion
Prenatal ultrasonography has impacted on the identication and management of fetuses with urological anomalies. Currently, severe urinary tract obstruction is the most common problem that we, pediatric urologists, are called upon for the consultation of antenatal emergency. Active participation of pediatric urologists, although limited to some
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institutes associated with maternal units, has led to urgent perinatal management resulting in improvement of prognosis.9 Antenatal intervention includes medical treatment, termination of pregnancy, early delivery induction and surgical management. Reports of fetal surgery on urological anomalies are gradually increasing but still remain rare.10 Correct explanation to the expecting parents on the long-term outcome of problems detected antenatally is sometimes difcult because of the inaccuracy of precise diagnosis and of the complexity of the anomaly.11,12 It is well described that unilateral PUJO is a benign condition that carries a low risk for renal damage, and acute clinical consequences associated with PUJO in the neonatal period seem to occur rarely. Even in the most severe grade of PUJO, urgent studies and intervention are deferred for several weeks after birth, because the condition will resolve spontaneously and the function will not decrease in most of the kidneys.4,5 There are, however, exceptional PUJO that require emergent treatment in neonatal periods. They include mass effect from compression of huge hydronephrosis, renal dysfunction and severe urinary tract infection.6 Three neonate cases presented with feeding intolerance and/or respiratory distress, presumably due to compression of the gastric outlet and elevation of the hemidiaphragma by the huge hydronephrotic mass. In addition, gastroesophageal reux may cause apnea and bradycardia.12 Breakout of these symptoms is an apparent indication for emergent drainage of hydronephrosis. Five patients showed compromise of renal function in perinatal periods. Four of them had hydronephrosis of the solitary kidney, and the other one had bilateral hydronephrosis. PUJO usually occurs unilaterally, although in approximately 20% of patients diagnosed in infancy the condition is bilateral.13 Cases of bilateral PUJO or those of the solitary kidney are considered at risk for poor outcome from renal
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Neonates with congenital hydronephrosis

Fig. 3 Case 2. Antegrade pyelography on 40th day after birth showed good passage at pelviureteric junction.

dysfunction.14 Three of our patients revealed decreased amniotic uid or oligohydramnios. None of them required ventilation assistance after birth. In another two patients, monitoring of serial SCr levels and electrolyte balance in the rst few days after birth indicated renal dysfunction requiring emergent drainage. Assessment of fetal renal function includes sonographic appearance of renal parenchyma, fetal urine analysis through serial aspiration, and amniotic uid status during prenatal courses.15 Management of the fetus with hydronephrosis and decreasing amniotic uid depends on gestational age and fetal pulmonary maturity. It is considered that gradual decrease of amniotic uid volume in the third trimester suggests severe compromise of fetal renal function with minimum risks of pulmonary hypoplasia.16 The renal damage depends on the timing and severity of the in utero obstruction. Fetuses with oligohydramnios starting around 30 weeks of gestation are considered to have fetal acute renal failure. In such patients, we choose to induce early delivery followed by emergency urinary drainage, with satisfactory results in respect to renal function.9,17 However, one of our series showed moderate decrease of renal function after the neonatal effective drainage. We have to know the limitation of renal functional recovery in solitary PUJO or bilateral severe PUJO. The diagnosis of neonatal renal dysfunction is generally based upon urine output and SCr levels. It is important to recognize that renal failure can occur with a high urine ow rate like bilateral PUJO of our cases. Renal dysfunction in the newborn is unique in that it occurs after a period of intrauterine life and in a setting of immature renal development. Evaluation of neonatal renal function by means of inulin
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Fig. 4 Case 2. Follow-up ultrasonography revealed aggravation of hydronephrosis at 6 months of age.

clearance or radio-substituted radiopharmaceuticals involves many technical problems and is not available in clinical practice.18 On the contrary, SCr, which reects maternal levels on the day of birth, decreases slowly but does not rise in neonates at the gestational age of 30 weeks or later.19 Typically in neonates with a GA of 36 weeks or later, SCr decreases along the decay curve to half in 2 weeks. When SCr levels continue to rise in neonates with a GA of 30 weeks or later, or the fall of SCr in the rst few days of life is slower than that of the decay curve of a normal control, renal dysfunction is highly suspected and requires urinary drainage.

Conclusion
We reported nine patients with PUJO who required emergent treatment in neonatal periods. Indications for neonatal emergent treatment included mass effect from giant hydronephrosis in three patients, renal dysfunction in ve, and severe urinary tract infection in one. At birth, respiratory and circulatory conditions must rst be stabilized , if necessary, by puncture of the hydronephrosis or placement of a nephrostomy tube. In neonates with hydronephrosis of the solitary kidney or bilateral severe PUJO, serial SCr should be monitored to evaluate renal
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function. Association of oligohydramnios or decrease of amniotic uid suggested renal functional compromise which would not recover after the urological management.

References
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