Posterior Urethral Valve : Its Clinical, Biochemical and Imaging Patterns

Posterior Urethral Valve : Its Clinical, Biochemical and Imaging Patterns K BHAUMIK 1 I CHATTERJEE 2 KS BASU 3 N SAMANTA 4 S DAS 5 Purpose : To observe the clinical, biochemical and imaging patterns of Posterior Urethral Valve (PUV) patients and how they change after initial treatment and during follow-up. Methods : 79 patients of posterior urethral valves were studied between January, 2000 and June, 2002. Their age ranged from 1 day to 11 years. Presenting features of these patients were studied clinically. Biochemical studies as blood urea, serum creatinine, electrolytes and imaging studies as Micturating Cystourethrogram (MCU), Ultrasonography (USG) and renal isotope scan were performed. Result : 55% of the patients presented under one year of age. The most common presentation was poor urinary stream followed by dribbling of urine. 37% of the patients presented with palpable kidneys, 62% with recurrent Urinary tract infection (UTI). Blood urea and Serum creatinine were raised in 91% of patients. Electrolyte abnormalities were present in 22 cases. USG showed hydronephrosis in 87% of cases. Micturating Cystourethrogram (MCU) showed reflux in 45% of cases. Renal scan showed bilateral hydronephrosis, reduced Glomerular Filtration Rate (GFR) and delayed excretory pattern. Key Words : PUV, Urinary diversion, Vesicostomy. Posterior urethral valve is the commonest Dewan have identified it as a single structural cause of urinary outflow obstructing membrane with a small defect or obstruction in boys. 1 It is also the most opening in the posterior midline of the common type of obstructive uropathy urethra and prefers to call the entity leading to childhood renal failure. 2 Young congenital obstructing posterior urethral classified the morphological variation of the membrane (COPUM). 3 Though there is valvular obstruction into 3 types. Recently controversy regarding the development and Department of Pediatric Surgery NRS Medical College & Hospital Kolkata, India 1 & 2 MCh (Post Doctoral Trainee) 3 RMO cum Clinical Tutor 4&5 Professors Dr K Bhaumik 22, Iswar Gupta Road Kolkata 700 028 Phone-(033) 5515006 E-mail : kuntalbhaumik@vsnl.net

154 J INDIAN ASSOC PEDIATR SURG VOL 8 (JUL-SEP 2003) nature of the valve (3, 4), the entire urinary tract from the level of obstruction upwards is affected by the lesion to a varying degree. 5 The incidence of PUV ranges from 1 in 8000 6 to 1 in 25000 1 live male births and it constitutes about 10% of prenatally diagnosed hydronephrosis. 7 Patients with the most severe form of the disease often die immediately after birth and do not present for treatment. 8 In addition, up to 26% of patients treated in the neonatal period subsequently develop end stage renal failure. 9 There is an intermediate group that does not require renal transplantation but also does not have enough renal function to permit growth and development. 10 Materials and Methods In a period of two & half years from Jan 2000 to June 2002, 79 patients, age ranging from 1 day to 11 years, were admitted in our department with PUV. We have studied the clinical, biochemical and imaging patterns of all these patients at presentation and after initial treatment. At presentation, all the urinary and non-urinary symptoms were noted (Table I & II). Blood was collected for estimation of blood urea, serum creatinine, complete hemogram and electrolytes, at presentation and also after initial treatment. USG of abdomen were done at presentation and also during follow up. Renal scan was performed initially, usually after one month of initial treatment and then during follow up period to ascertain any improvement or deterioration in function. Results In our study, 21 out of 79 patients (27%) presented within one month, 22 patients (28%) between the age of one month and one year. 29 patients (36%) were in the age group of 1 to 6 years and 7 (9%) were between 6 and 11 years of age (Fig. 1). Antenatal USG was performed in 14 cases. In 3 cases, PUV was suspected antenatally due to presence of bilateral hydronephrosis, thick walled distended bladder and less amniotic fluid.

PUV : CLINICAL, BIOCHEMICAL & IMAGING PATTERNS 155 At presentation, blood urea & serum creatinine were raised in 72 patients (91%). Creatinine level was above 1.5 mg% in 42 patients (53%) and between 1 & 1.5 mg% in 30 patients (38%). Hemoglobin level was 90% & below in 27 cases (34.2%). Hyponatremia and hyperkalemia were noted in 22 cases. On examination of urine, plenty of pus cells & positive urine culture were noted in 51 patients (65.5%). Most common organism grown was E. Coli. USG revealed dilated posterior urethra & distended thick walled bladder in all cases. Hydronephrosis was seen in 69 cases (87.3%) and hydroureter in 46 cases (58.2%). Corticomedullary junction could be identified in 30 cases. Of the 49 patients who had non-visualisation of Corticomedullary junction, 42 had impaired renal function. MCU was performed in all cases and all of them showed dilated and elongated posterior

156 J INDIAN ASSOC PEDIATR SURG VOL 8 (JUL-SEP 2003) urethra. Margin of the bladder was irregular and multiple pseudo-diverticulae were noted in 49 patients. Vesicoureteric reflux was seen in 36 cases (46%) at presentation of which bilateral reflux was present in 19 (24%). Among the 17 cases of unilateral reflux, right side was affected in 7 cases and left side in 10 cases. Nine cases showed severe reflux (Gr. IV & V). DTPA renal scan was performed in 63 cases, all of which showed bilateral hydronephrosis of mild to moderate degree. Perfususion was low and there was grossly delayed excretory pattern. GFR was reduced in the affected kidneys. As a routine first scan was performed one month after initial treatment. At presentation, 62 patients were treated by bladder catheterisation and intravenous antibiotics. After proper resuscitation with intravenous fluid, correction of electrolyte imbalance and acidosis either vesicostomy or valve fulguration was done. As we do not have a small sized cystoscope or resectoscope in our institute, we could not perform primary valve fulguration in infants below one year of age. Thus in all the patients who were very sick at presentation, and all the patients below one year of age, we performed vesicostomy (in 51 cases). Primary valve fulguration was done in 27 cases. In 8 cases, after primary valve fulguration, the patient's condition did not improve and the blood urea and serum creatinine level did not come down. Vesicostomy was performed as a secondary procedure. In 4 cases the urinary drainage after primary vesicostomy was not adequate and the biochemical parameters did not improve. High diversion in the form of bilateral ureterostomies were performed in these patients. Complications of vesicostomy were wound infection, stomal stenosis, and mild prolapse. In 4 cases vesicostomy was revised to treat stenosis. In our series, 4 patients expired. One patient who presented with severe respiratory distress, edema and renal failure died on first day. One patient died due to severe urosepsis on the fourth day after the vesicostomy; 2 patients died of renal failure even after performing bilateral ureterostomies, one at 5 months of age and another at 7 months of age. Two patients presented with acute renal failure and were treated with peritoneal dialysis one of them expired. After vesicostomy, valve fulguration was performed in 24 cases when the infants were older and had gained in weight. So far in this series, vesicostomy closure was performed in 7 cases. In the follow-up, at one month, serum creatinine level came down in 43 out of 72 patients. Creatinine level was improved in 31 cases out of 51 vesicostomies & in 12 cases out of 21 cases of primary valve fulguration. Creatinine level at presentation was below 1 mg% in 7 patients, between 1 & 1.5 mg% in 30 patients, between 1.5 & 3 mg% in 25 patients and above 3 mg% in 17 patients. At one year follow-up, out of 71 patients only 18 patients had serum creatinine level above 1, in rest of the patients it was below 1 mg%. At 2 years follow-up, 7 patients were lost to follow-up. Out of 68 patients seen at two years, 7 patients had creatinine level above 1 mg%. Follow-up MCU in 3 cases who had Grade I & II reflux showed resolution within 2 years, and the grade of reflux improved in 11 other cases. Subsequent renal scan in 9 cases

PUV : CLINICAL, BIOCHEMICAL & IMAGING PATTERNS 157 showed improvement of excretory pattern. Discussion Approximately one third of PUV patients present during the neonatal period, onethird between 1 month and 1 year and one third over one year of age. 9,11,12 More recent reviews have demonstrated increased presentation during the neonatal period and this early presentation is atributed to greater awareness about the condition, and better antenatal diagnosis. 1 PUV is increasingly recognised by routine prenatal USG. 1,13, 14 The characteristic findings are bilateral hydronephrosis and a distended thickened bladder. A dilated posterior urethra is seen occasionally. Low amniotic fluid and bright renal parenchyma (suggestive of dysplasia) provide important clues about the severity of renal damage. In our series, antenatal USG was performed in 14 patients. In 3 of these patients, PUV was suspected because of bilateral hydronephrosis. Out of these 3 patients, one patient was very sick at birth and died on the first day before any surgical intervention could be done. Whether prenatal diagnosis has any impact on long term outcome of these patients is still controversial. 9 In neonates, obstructive symptoms (straining, poor stream, dribbling, palpable bladder) may be seen in 77% of cases and many of them present with features of renal failure. 1,11 Infants present with features of infection (78%) like septicemic shock, poor feeding, abdominal pain, and positive urine culture. 11 They also present with abdominal distention, dribbling of urine (40%), failure to thrive (30%) and palpable kidneys (25%). 13 In toddlers, renal failure becomes less common but voiding symptoms and infection predominate, 50% of patients over 5 years of age present with enuresis and dribbling. 1 These later presentations is indicative of milder forms of valvular obstruction and their presentation may be delayed into adulthood. 5 In our series, obstructive symptoms in the form of poor stream and dribbling were present in all patients. In 11 patients who were of older age group the symptoms were present for a short period, 37% of patients presented with a palpable kidney and 62% presented with recurrent UTI. Renal failure was more common in those who presented at an earlier age. Failure to thrive was observed in 43% of patients. At presentation, 91% patients had raised blood urea & serum creatinine levels and at one month follow-up, the scrum creatinine level remained high in 72% of cases. At one year follow-up, 23% of patients and at 2 years follow-up, 10% of patients had serum creatinine level above 1 mg%. In contrast to an incidence of 40% in published literature. 25% of neonates in our series presented with urinary ascites. 15 USG demonstrates hydrouretcrone-phrosis, thick walled bladder, dilated posterior urethra and occasionaly bright kidneys with loss of cortico-medullary differentiation suggestive of renal dysplasia. 16 In our series cortico-medullary junction was well demarcated in 38% of cases while the demarcation was lost in 49 patients (62%). MCU is the gold standard in diagnosis of a case of posterior urethral valve. It permits assessment of vesico-ureteric reflux in almost 50% patients. 17 Of these, 20% will

158 J INDIAN ASSOC PEDIATR SURG VOL 8 (JUL-SEP 2003) have renal dysplasia. 9,18 In 15%, there is spontaneous resolution of reflux and in the remaining 15% reflux persists and needs surgical correction. 18 In our series, 36 patients (46%) presented with reflux and during a short follow-up, 3 patients showed resolution of reflux. Since the introduction of neonatal resectoscope, management of PUV cases have changed but a group of patients still require diversion. High rate of diversion in our series is due to non-availability of a neonatal resectoscope. We have found that vesiscostomy is a very useful method of diversion, as functioning vesicostomy provides an adequate drainage of the lower urinary tract as proposed by Duckett. 19,20 It is a minimally invasive surgery and is an easily reversible technique. It allows cycling of the bladder to take place at a low pressures. 21 We performed secondary vesicostomy in 8 patients and we believe that it is an excellent choice to treat otherwise refractory urinary sepsis and to stabilise renal function. Bilateral ureterostomy was advocated by some authors as a better alternative method of diversion in developing countries, for patients with persistently high serum creatinine level. 22 However in our series ureterostomies had to be done only in 4 cases as a secondary procedure after vesicostomy had failed to drain the upper urinary tract adequately. This is an ongoing study. Clinical parameters and investigation parameters of 79 patients over a period of two and half years are being presented for a disease like PUV which has varied manifestations. Long-term follow up is necessary to see the effect of treatment in these patients. Aggressive management of vesicoureteric reflux with early surgical intervention is rarely justified as upper urinary tract complications resolve spontaneously in some cases. In our short follow up we have observed resolution of reflux in 3 cases. References 1 Atwell JD posterior urethral valves in the British Isles a multicenter BAPS review. J Pediatr Surg. 1983; 18:70-77. 2 Warshaw BL, Edelbrock HH, Ettenger RB, et al. Renal transplantation in children with obstructive uropathy. J Urol 1980; 123:737-742. 3 Dewan PA, GoH DG Variable expression of the congenital obstructive posterior urethral membrane. Urol 1995; 45:507-509. 4 Dewan PA (Guest Ed) Congenital obstruction in the Male Urethra Dialogues. Pediatr Urol 1995; 18:1-8. 5 Hendren WH Posterior urethral valve in boys. A broad clinical spectrum. J Urol 1971;106:298-307. 6 Casale AJ. Early Urethral surgery for posterior urethral valves. Urol Clin North Am. 1990; 17:361-368. 7 Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. Am J Roentgenol. 1987; 148:959-962. 8 Nakayama DK, Harrison MR, delorimier AA. Prognosis of posterior urethral valves presenting at birth. J Pediatr Surg. 1986; 21:43-45. 9 Parkhouse HF, Barratt TM, Dillon MJ, et al. Long-term outcome of boys with posterior urethral valves, Br J Urol. 1988; 62:59-62.

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