The Role of Vesicostomy in the Management of Anterior Urethral Valves in Neonates and Infants

The Role of Vesicostomy in the Management of Anterior Urethral Valves in Neonates and Infants

0022-534 7/87 /1381-0107$02.00/0 THE JOURNAL OF UROLOGY Vol. 138, July Printed in U.S.A. Copyright© 1987 by The Williams & Wilkins Co. Pediatric Ar...

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0022-534 7/87 /1381-0107$02.00/0 THE JOURNAL OF UROLOGY

Vol. 138, July Printed in U.S.A.

Copyright© 1987 by The Williams & Wilkins Co.

Pediatric Articles THE ROLE OF VESICOSTOMY IN THE MANAGEMENT OF ANTERIOR URETHRAL VALVES IN NEONATES AND INFANTS H. GIL RUSHTON,* THOMAS S. PARROTT, JOHN R. WOODARD AND McCLELLAN WALTHER From the Henrietta Egleston Hospital for Children and Scottish Rite Chiklren's Hospital, Division of Urology, Emory University School of Medicine, Atlanta, Georgia

ABSTRACT

We performed vesicostomy as the initial form of management in 2 male newborns and 1 infant with anterior urethral valves associated with proximal urethral diverticula and bilateral hydronephrosis. Prompt improvement in the degree of hydronephrosis was noted after vesicostomy in all 3 patients along with improvement in renal function in 2 who had presented with renal insufficiency. Subsequently, 2 patients have undergone vesicostomy closure and transurethral valve fulguration, and 1 also required ureteral reimplantation for persistent vesicoureteral reflux. The use of vesicostomy in the newborn period rather than transurethral fulguration prevented the potential complications of urethral stricture and inadequate valve resection that can occur owing to the small size of the neonatal urethra. Furthermore, a period of vesicostomy drainage before eventual ureteral reimplantation for severe vesicoureteral reflux obviated the need for ureteral tailoring. J. Ural., 138: 107-109, 1987 Anterior urethral valves well known but rare types of infravesical obstruction in male subjects, are reported to occur approximately 7 times less commonly than posterior urethral valves. 1 This lesion can be located anywhere along the urethra distal to the membranous urethra, although most have been reported proximal to the penoscrotal junction. 2 The etiology of anterior urethral valves remains unclear. Faulty union of the glanular and penile urethra, incomplete formation of the ventral corpus spongiosum of the urethra, congenital cystic dilatation of the periurethral glands and an abortive attempt at urethral duplication have been proposed as possible etiological mechanisms. 3 Anterior urethral valves frequently are associated with proximal urethral diverticula. Some have attempted to differentiate anterior urethral valves from obstructing diverticula of the anterior urethra, while others believe that these entities represent a spectrum disorder. 1 Those favoring anterior urethral diverticula as the correct nomenclature contend that the diverticulum is the primary pathological factor with the distal lip of the diverticulum serving as a pseudovalve creating obstruction during voiding.'1• 4 Others have supported the presence of a primary obstructing valve with secondary diverticular formation. 1 • 2 In some cases the anatomical distortion may be so severe that the question of whether a valve was present initially which then resulted in diverticular formation may be unresolvable. The diagnosis of anterior urethral valves is made best by voiding cystourethrography, although it is essential to visualize the entire distal urethra during micturition to avoid misdiagnosis. Screening of the upper tracts with ultrasound, excretory urography (IVP) and/or appropriate radionuclide renal scans will reveal some degree of upper tract deterioration in as many as 4 7 per cent of these patients, and vesicoureteral reflux has been reported in approximately a third of the cases. 2 Firlit Accepted for publication January 13, 1987. * Current address: Department of Urology, Children's Hospital National Medical Center, 111 Michigan Ave., N. W., Washington, D. C. 20010. 107

and associates have classified the radiographic findings with anterior urethral valves into 4 types based on the severity of the obstruction: type 1-minimal proximal urethral distension, type 2-a proximal urethral diverticulum with a normal bladder and normal upper tracts, type 3-similar to type 2 but associated with bladder distension, trabeculation and minimal ureterectasis, and type 4-obstruction associated with severe hydroureteronephrosis.1 Reported methods of treatment have involved either open surgical excision and reconstruction or transurethral ablation of the anterior urethral valves. We present our experience in 2 male newborns and 1 infant using cutaneous vesicostomy as the initial management of anterior urethral valves associated with proximal urethral diverticula and hydronephrosis. CASE REPORTS

Case 1. A 2-day-old male newborn presented with dribbling urination associated with a lower abdominal midline mass secondary to bladder distension. Birth weight was 5 pounds 4 ounces. Laboratory examination revealed normal serum electrolytes and blood urea nitrogen (BUN) of 16 mg./dl. (normal 7 to 21 rng./dl.). Urinalysis and urine culture were unremarkable. An IVP demonstrated bilateral hydronephrosis, worse on the right side, with delayed excretion (fig. 1, A). Voiding cystourethrography revealed an anterior urethral valve with a proximal urethral diverticulum and grade I left vesicoureteral reflux (fig. 1, B). Because of the small size of the patient and the presence of hydronephrosis a cutaneous vesicostomy was performed when he was 5 days old. An IVP 3 weeks postoperatively demonstrated complete resolution of the hydronephrosis with normalappearing upper tracts (fig. 1, C). Creatinine at the time the patient was discharged from the hospital was 0.8 mg./dl. (normal 0.5 to 1.2 mg./dl.). Distal urethroscopy when he was 22 months old revealed an anterior urethral valve treated by transurethral fulguration and closure of the vesicostomy. This

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RUSHTON AND ASSOCIATES

Fm. 1. Case 1. A, IVP shows bilateral hydronephrosis and bladder distension. B, voiding cystogram demonstrates anterior urethral valve associated with diverticulum formation and narrow distal stream. C, IVP after vesicostomy reveals complete resolution of hydronephrosis.

Fm. 2. Case 2. A, voiding cystourethrogram demonstrates bilateral reflux into massively dilated and tortuous ureters. B, anterior urethral valve with proximal urethral diverticulum. C, IVP after 2 months of vesicostomy drainage shows considerable improvement in degree of hydronephrosis.

Fm. 3. Case 3. A, voiding cystogram demonstrates severe bilateral reflux into markedly dilated, tortuous ureters. B, anterior urethral obstruction associated with diverticulum formation. C, IVP 6 months after vesicostomy shows decreased tortuosity and hydronephrosis of right collecting system, and persistent nonvisualization of left kidney.

patient has since remained asymptomatic and free of urinary infection. Subsequent IVPs have demonstrated normal upper tracts and he voids with a normal urinary stream despite the persistence of a mildly abnormal configuration of the distal urethra. Case 2. A 6½-month-old white infant presented with a 1week history of vomiting and diarrhea associated with weight loss of approximately 1 kg. Medical history was significant for an episode of viral meningitis when he was 3 months old, at which time urinalysis revealed 3+ bacteria and 30 to 40 white blood cells. Admission physical examination was unremarkable except for the absence of a fourth digit from the left hand. Laboratory studies revealed a serum sodium of 122 mEq./1. (normal 135 to 148 mEq./1.), carbon dioxide content 8.2 mEq./1. (normal 22 to

32 mEq./1.), BUN 59 mg./dl., hemoglobin 10.4 gm. and white blood count 26,500 with 22 bands and 50 segmented neutrophils. Urinalysis showed 3+ bacteria, and white and red blood cells too numerous to count. Urine and blood cultures yielded Enterococcus cloacae. Renal ultrasound demonstrated bilateral hydronephrosis associated with moderate renal parenchyma. Voiding cystourethrography showed bilateral severe vesicoureteral reflux in the presence of an anterior urethral valve associated with a proximal urethral diverticulum (fig. 2, A and B). A dimercapto-succinic acid (DMSA) renal scan demonstrated diminished uptake of the radionuclide by the left kidney and patchy uptake by the right kidney. Initial management of urosepsis with intravenous antibiotics was successful. Cystoscopy confirmed the presence of an anterior urethral valve approximately 3.5 cm. proximal to the mea-

VESICOSTOMY FOR ANTERIOR URETHRAL VALVES IN NEONATES AND INFANTS

tus. Vesicostomy was performed to maximize drainage of the upper tracts in view of the recent episode of urosepsis and the coexistence of severe bilateral vesicoureteral reflux with azotemia. BUN when the patient was discharged from the hospital was 7 mg.jdL and serum electrolytes were within normal limits. A followup IVP after 2 months of vesicostomy drainage demonstrated bilateral improvement in the degree of hydronephrosis (fig. 2, C). The patient underwent vesicostomy closure and transurethral fulguration of the anterior urethral valve 6 months later. A subsequent furosemide-enhanced diethylenetriaminepentaacetic acid renal scan demonstrated adequate drainage of both collecting systems with improvement in differential left renal function compared to the earlier renal scan. Because of persistent bilateral vesicoureteral reflux demonstrated by voiding cystography, the patient later underwent bilateral transtrigonal reimplantation. Tapering of the ureters was not required. Case 3. A 2-day-old white male newborn presented with constant urinary dribbling and a palpable mid scrotal mass. Physical examination was otherwise unremarkable. Birth weight was 6 pounds 1 ounce. Laboratory examination revealed a serum creatinine of 2.8 mg./dl. Serum electrolytes were normal except for a carbon dioxide content of 21 mEq. Urinalysis showed 5 to 10 red and 3 to 5 white blood cells. Hemoglobin was 22.7 gm.jdl. (normal 18.5 to 21.5 gm./dl.) and white blood count was 17,300. Total bilirubin was 9.2 mg./dl. (normal 0.2 to 1.0 mg./dl.) with a direct fraction of 3.2 mg./dl. (normal Oto 0.15 mg./dl.). Voiding cystourethrography confirmed the clinical impression of anterior urethral obstruction associated with a large urethral diverticulum and massive bilateral vesicoureteral reflux (fig. 3, A and B). A DMSA renal scan demonstrated diminished, patchy uptake of radionuclide within the right kidney and nonfunction of the left kidney. Cutaneous vesicostomy was performed for management of the urethral obstruction associated with severe bilateral vesicoureteral reflux and renal insufficiency. Followup laboratory examination when the patient was 6 months old revealed a serum creatinine of 1.0 mg./dl., BUN 14 mg./dl. and normal serum electrolytes except for a carbon dioxide content of 20.5 mEq. An IVP 6 months later demonstrated improvement in the degree of tortuosity and hydronephrosis of the right collecting system. The left kidney was not visualized (fig. 3, C). A subsequent DMSA renal scan demonstrated improved uptake of radionuclide in a reniform right kidney and persistent nonfunction of the left kidney. DISCUSSION

Various methods of treatment of anterior urethral valves have been advocated, and most reported cases have been managed with initial open urethrotomy and excision of the anterior urethral valves. 2 This often has included resection of the redundant diverticulum wall with either primary closure or a first stage marsupialization. 3 - 5 Others have performed segmental urethrectomy of the valve-bearing area with a primary end-toend anastomosis. 2 Complications reported with open excision include stricture formation, urethrocutaneous fistula and extravasation of urine. 1 More recently, there has been a trend

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toward initial transurethral management. 1 • 6 • 7 Transurethral treatment has ranged from simple dilation with "fracturing" of the valves to electrofulguration. In 1 infant with anterior urethral valves and bilateral hydronephrosis managed initially by electrofulguration of the valves urosepsis developed subsequently and required high urinary diversion with bilateral loop cutaneous ureterostomy. 8 Firlit and King described 2 other infants with bilateral hydronephrosis and azotemia who were managed initially with high loop cutaneous ureterostomy. 5 Our 3 patients presented during infancy with bilateral hydroureteronephrosis and a proximal urethral diverticulum. In 2 patients the hydroureteronephrosis was associated with severe bilateral vesicoureteral reflux and renal insufficiency. Our patients were managed initially with cutaneous vesicostomy and improvement in the degree of hydronephrosis was noted in all 3. Subsequently, 2 have undergone vesicostomy closure and transurethral fulguration of the valves. The upper tracts have remained stable and followup voiding cystourethrography has confirmed adequate relief of urethral obstruction in both patients. The third and most recent patient currently is awaiting further treatment. There are several advantages of vesicostomy for the management of neonates and infants with anterior urethral valves and hydronephrosis. Vesicostomy provides prompt and effective decompression of the upper tracts, which is particularly important when hydronephrosis is complicated by the presence of severe reflux. The obstruction in neonates or infants with anterior urethral valves often is more severe than that seen in older children and it may be associated with varying degrees of renal insufficiency. By providing more optimal drainage, vesicostomy should maximize the potential for recoverability of renal function in these more severely affected infants. The use of vesicostomy in the newborn period, rather than transurethral resection, prevents the potential complications of urethral stricture and inadequate valve resection that can occur owing to the small size of the neonatal urethra. A period of vesicostomy drainage before eventual ureteral reimplantation for associated severe vesicoureteral reflux may result in significant reduction in the caliber of the ureters and, thus, obviate the need for ureteral tailoring at the time of reimplantation. REFERENCES

1. Firlit, R. S., Firlit, C. F. and King, L. R.: Obstructing anterior

urethral valves in children. J. Urol., 119: 819, 1978. 2. Golimbu, M., Orea, M., Al-Askari, S., Morales, P. and Golimbu, C.: Anterior urethral valves. Urology, 12: 343, 1978. 3. Williams, D. I. and Retik, A. B.: Congenital valves and diverticula of the anterior urethra. Brit. J. Urol., 41: 228, 1969. 1. Graham, S. D., Jr., Krueger, R. P. and Glenn, J. F.: Anterior urethral diverticulum associated with posterior urethral valves. J. Urol., 128: 376, 1982. 5. Firlit, C. F. and King, L. R.: Anterior urethral valves in children. J. Urol., 108: 972, 1972. 6. Malhoski, W. E. and Frank, I. N.: Anterior urethral valves. Urology, 2: 382, 1973. 7. Baum, N., Kaminsky, R. and Wright, J. D.: Anterior urethral valves. Urology, 6: 723, 1975. 8. Small, M. P. and Schoenfeld, L.: Anterior urethral valves. Urology, 11: 262, 1978.