Journal of Pediatric Surgery (2011) 46, E27–E29
www.elsevier.com/locate/jpedsurg
Management of a hypoplastic lower urinary tract: a case report Swethan Alagaratnam ⁎, Manoharan Sengamalai, Imran Mushtaq Department of Paediatric Urology, Great Ormond Street Hospital for Children NHS Trust, WC1N 3JH London, UK Received 29 November 2010; revised 11 March 2011; accepted 11 March 2011
Key words: Ureteric hypoplasia; Lower urinary tract reconstruction; Colonic ureteric substitute; Ileal ureteric substitute
Abstract This case relates to a child with an antenatal diagnosis of severe bilateral hydronephrosis with congenital anomalies of the ureters, bladder, and urethra. We describe the presentation and surgical management, highlighting the complexity of this anomaly and the surgical technique used to reconstruct the lower urinary tract. To our knowledge, there has not been a similar case in the literature and the use of a segment of colon for ureteric substitution in children has not been previously reported in the literature. © 2011 Elsevier Inc. All rights reserved.
1. Case report The combination of ureteric, bladder, and urethral anomalies with normal functioning kidneys is an unusual and challenging clinical scenario. We describe our management of such a case of lower urinary tract abnormalities from its antenatal presentation to early childhood. A healthy pregnant mother had an antenatal scan at 23 weeks of gestation which revealed bilateral hydronephrosis. At 31 weeks, a further ultrasound scan showed worsening hydronephrosis with associated anhydramnios. After an urgent review at a fetal medicine unit, she successfully underwent amnioinfusion and insertion of bilateral renal amniotic shunts into the fetus. One week later, she developed premature labor and delivered a 1.94-kg female neonate. The baby did not require resuscitation and with the exception of the shunts in both flanks, examination was normal. In ⁎ Corresponding author. Tel.: +44 07789514646; fax: +44 0207 813 8260. E-mail addresses:
[email protected] (S. Alagaratnam),
[email protected] (M. Sengamalai),
[email protected] (I. Mushtaq). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.03.076
particular, there were no external abnormalities of the perineum and external genitalia. On day 3, one of the shunts dislodged, but urine continued to drain through the drain site. A postnatal ultrasound scan on day 5 revealed kidneys with poor corticomedullary differentiation, pelvicalyceal dilatation with pelviureteric junction configuration, a nonvisualized bladder, and no dilated ureters. Her serum creatinine at that stage was 186 μmol/L. Owing to further deterioration of the renal function, she underwent insertion of bilateral nephrostomies on day 9. Nephrostograms performed during this procedure failed to show passage of contrast through either pelviureteric junction (Fig. 1). She produced good volumes of urine through the nephrostomies, and with improving renal function she was allowed home for a few weeks. A repeat serum creatinine 4 weeks later demonstrated an improvement to 64 μmol/L. A dimercaptosuccinic acid scan performed on day 29 demonstrated bilateral abnormal kidneys with a differential function of 61% on the left and 39% on the right. To investigate the anatomy further, she underwent a cystoscopy which revealed a stenotic urethral passage and an extremely small bladder with pinhole ureteric orifices. A 3-Fr
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Fig. 2 Fig. 1
Nephrostograms performed on day 9 of life.
ureteric stent could not be negotiated beyond 2 cm from the ureteric orifice, and attempts to inject contrast were unsuccessful. We therefore proceeded to fashion bilateral pyelostomies during the same procedure, with the aim of performing major reconstruction of her lower urinary tract after a period of satisfactory growth. She thrived well during infancy with a stable creatinine of 41 μmol/L. At the age of 13 months, she underwent a repeat cystoscopy, cystogram, and attempted retrograde studies. These confirmed the findings of an abnormal lower urinary tract with a stenotic urethra, an extremely small bladder with a capacity of less than 5 mL, bilateral vesicoureteric reflux with a hairline of contrast filling both distal ureters (Fig. 2), and the need for a radical reconstruction of the ureters and the bladder. After discussions with her family, she underwent her reconstructive procedure at around 4 years of age. Her stenotic ureters and hypoplastic bladder were confirmed intraoperatively (Fig. 3). The pyelostomies were closed, and a colo-ileocystoplasty was performed using a 10-cm segment of the sigmoid colon and a 30-cm segment of the ileum. The right ureteric substitute was fashioned using a spiral ileal Monti tube and the left with a 2-cm spiral sigmoid colonic Monti tube (Fig. 4) over a 5.2- and a 6.0-Fr J-J stent, respectively. The appendix was used for the Mitrofanoff channel and this was implanted into the colonic patch of the neobladder. Bilateral nephrostomies were also placed. Her postoperative recovery was interrupted by a left-sided urine leak after clamping the left nephrostomy on the 12th postoperative day. An urgent nephrostogram confirmed the urine leak, and she underwent an exchange of the left nephrostomy which resolved the problem. Repeat nephrostograms on day 30 demonstrated good drainage bilaterally
Intraoperative cystogram.
with no evidence of urine leak. On the same day, the nephrostomy tubes were removed and she was discharged home the following day. Both J-J stents were removed 6 weeks later. At 12 months' follow up, she has made excellent progress. She is successfully managing clean intermittent catheterization every 4 hours during the day without overnight drainage and has maintained a normal serum creatinine. There have been no episodes of urinary tract infection and she has remained completely continent. Ultrasound of her renal tract 12 months after removal of the JJ stents shows very mild pelvicalyceal dilatation bilaterally with no ureteric dilatation. Our long-term follow-up plan includes annual surveillance with renal ultrasound, routine blood tests, dimercaptosuccinic acid scan, and endoscopic surveillance from 10 years after the reconstructive surgery.
Fig. 3 Intraoperative picture showing the stenotic ureter (3-Fr stent visible through the ureteric wall).
Management of a hypoplastic lower urinary tract: a case report
Fig. 4
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Colonic ureteric substitute.
2. Discussion Several cases of bladder agenesis and an extensive array of ureteric abnormalities have been reported in the literature [1-3]. In bladder hypoplasia, it is believed that inadequate filling with urine results in underdevelopment of the fetal bladder. However, this rare constellation of bladder, urethral, and ureteric anomalies with well-functioning kidneys has not been reported and it is difficult to explain such a combination embryologically. It is possible that these abnormalities are the consequence of severe bilateral pelviureteric junction obstructions, leading to the subsequent hypoplasia of the lower urinary tract. The operative management involved a complete reconstruction of the lower urinary tract. The use of colon and ileum for bladder augmentation is well established in pediatric urology. Similarly, the use of the appendix or a segment of the reconfigured ileum (Monti tube) as a conduit for clean intermittent catheterization is extensively practiced. The use of ureteric substitutes is not common in pediatric urology, owing to the rarity of conditions necessitating this practice [4]. Much of the evidence available is related to adult ureteric substitutes. The YangMonti principle limits the length of bowel required for ureteric substitutes, thereby reducing the metabolic, infective, and mucous production complications [5]. Initially reported by Yang [6] and later verified by Monti et al [7], recent reports by Castellan et al [8] and Steffens et al [9] demonstrate good long-term outcomes with low complication rates and good functional results. The use of colonic segments as ureteric substitutes is less widely reported and we have not been able to identify any reports in children. Colonic ureteric substitutes reported in the literature include an ascending colon substitute [10], a transverse colon substitute [11], a sigmoid colon substitute [8], and interposition short-segment colonic grafts [12]. No postoperative complications and good functional outcomes have
been reported in these substitutes, with the longest follow-up of 3.25 years [8]. In our case, the ileal ureteric substitute after 12 months of follow-up has not been associated with any complications. The colonic substitute transiently leaked at the proximal anastomosis, but this settled with conservative management. After 12 months of follow-up, no further issues have arisen from the colonic ureteric substitute. This case illustrates a rare constellation of abnormalities which necessitated a radical reconstruction of the whole lower urinary tract with a very successful initial outcome.
References [1] Aragona F, Glazel GP, Zaramella P, et al. Agenesis of the bladder; a case report and review of the literature. Urol Radiol 1988;10:207-9. [2] Akdaş A, Işeri C, Ozgür S, et al. Bladder agenesis. Int Urol Nephrol 1988;20:261-3. [3] Krull CL, Heyns CF, de Klerk DP. Agenesis of the bladder and urethra: a case report. J Urol 1988;140:793-4. [4] Obaidah A, Mane SB, Dhende NP, et al. Our experience of ureteral substitution in pediatric age group. Urology 2010;75:1476-80. [5] Ghoneim MA. Replacement of ureter by ileum. Curr Opin Urol 2005;15:391-2. [6] Yang WH. Yang needle tunneling technique in creating antireflux and continent mechanisms. J Urol 1993;150:830-4. [7] Monti PR, Lara RC, Dutra MA, et al. New techniques for construction of efferent conduits based on the Mitrofanoff principle. Urology 1997;49:112-5. [8] Castellan M, Gosalbez R. Ureteral replacement using the Yang-Monti principle: long term follow up. Urology 2006;67:476-9. [9] Steffens JA, Anheuser P, Reisch B, et al. Ureteric reconstruction with reconfigured ileal segments according to Yang-Monti: a 4 year prospective report. Urologe A 2010;49:262-7. [10] Pope J, Koch MO. Ureteral replacement with reconfigured colon substitute. J Urol 1996;155:1693-5. [11] Kato H, Igawa Y, Nishizawa O. Transverse colon pouch with total replacement of the ureter by reconfigured colon segment. J Urol 1999;161:1902-3. [12] Ubrig B, Roth S. Reconfigured colon segments as a ureteral substitute. World J Urol 2003;21:119-22.
