Anorectal malformation with rectobladder neck fistula: A distinct and challenging malformation

  • Inbal Samuk
    Correspondence
    Corresponding author at: Department of Pediatric Surgery, Schneider Children's Medical Center, 14 Kaplan Street, Petah Tikva 4941492, Israel. Tel.: +972 3 9253735; fax: +972 3 9253930.
    Affiliations
    Department of Pediatric and Adolescent Surgery, Schneider Children's Medical center, affiliated to Sackler faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel

    Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA
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  • Andrea Bischoff
    Affiliations
    Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA
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  • Jennifer Hall
    Affiliations
    Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA
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  • Marc Levitt
    Affiliations
    Center for Colorectal and Pelvic reconstruction, Nationwide Children's Hospital, Columbus, OH, USA
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  • Alberto Peña
    Affiliations
    Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA
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      Abstract

      Background

      Rectobladder neck fistula is the highest and most complex anorectal malformation in boys and the only one that requires an abdominal approach, open or laparoscopic, for repair. The aim of this study was to describe the unique characteristics of rectobladder neck fistulas that warrant special attention and to describe the associated anatomic variants in the genitourinary tract.

      Methods

      The database of a tertiary medical center was retrospectively reviewed for all patients treated for rectobladder neck fistula, by our team in 1980–2011. Data on surgical history, associated and functional defects, treatment and outcome were collected by chart review.

      Results

      The study group included 111 patients. The most common anatomic urologic defect was a single kidney in 37 patients (33.3%) and the most common functional urologic defect was vesicoureteral reflux in 40 patients (36%), including 11/37 patients with a single kidney (29.7%). Of the 40 patients who underwent cystoscopy, 16 (40%) had a higher than normal location of the verumontanum. Follow-up ranged from 2 to 290 months (median 59). Urinary continence was achieved in 40 of the 61 patients (65.5%) for whom data were available, and fecal continence was achieved in 9 of the 69 patients (13%) for whom data were available. A sacral ratio of 0.4 or less was associated with lower rates of urinary control (23%) and fecal control (0%), relative to higher ratios. Twenty stomas (18%) were found to be located too distally, limiting the availability of the bowel for a pull through.

      Conclusions

      Rectobladder neck fistula carries a poor prognosis for bowel control and is associated with a high rate of urinary malformations that require long-term care. Pediatric surgeons need to be aware of these complications in order to provide proper treatment and parental counseling. Intra-vesical verumontanum is found in a surprisingly high percentage of patients. The combination of a single kidney with vesicoureteral reflux is common and should be closely followed to avoid renal deterioration. Special attention should be given to colostomy construction to avoid complications and unnecessary procedures. A sacral ratio of 0.4 or less is an indicator of poor fecal and urinary control.

      Abbreviations:

      ASD (atrial septal defect), CIC (clean intermittent catheterization), PSARP (posterior sagittal anorectoplasty), SR (sacral ratio), VBM (voluntary bowel movements), VM (verumontanum), VUR (vesicoureteral reflux)

      Key words

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      References

        • Levitt M
        • Peña A
        Anorectal malformations.
        Orphanet J Rare Dis. 2007; 2: 33
        • Smith ED
        • Stephens FD
        High, intermediate and low anomalies in the male.
        Birth Defects Orig Artic Ser. 1988; 24: 17-72
        • Peña A
        Anorectal malformations.
        Semin Pediatr Surg. 1995; 4: 35-47
        • Rich M
        • Brock W
        • Peña A
        Spectrum of genitourinary malformations in patients with imperforate anus.
        Pediatr Surg Int. 1988; 3: 110-113
        • Fidan K
        • Kandur Y
        • Buyukkaragoz B
        • et al.
        Hypertension in pediatric patients with renal scarring in association with vesicoureteral reflux.
        Urology. 2013; 81: 173-177
        • Goossens WJ
        • de Blaauw I
        • Wijnen MH
        • et al.
        Urological anomalies in anorectal malformations in The Netherlands: effects of screening all patients on long-term outcome.
        Pediatr Surg Int. 2011; 27: 1091-1097
        • Cunningham BK
        • Khromykh A
        • Martinez AF
        • et al.
        Analysis of renal anomalies in VACTERL association.
        Birth Defects Res A Clin Mol Teratol. 2014; 100: 801-805
        • Sanchez S
        • Ricca R
        • Joyner B
        • et al.
        Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: a retrospective review.
        J Pediatr Surg. 2014; 49: 91-94
        • Konuma K
        • Ikawa H
        • Kohno M
        • et al.
        Sexual problems in male patients older than 20 years with anorectal malformations.
        J Pediatr Surg. 2006; 41: 306-309
        • Pena A
        • Migotto-Krieger M
        • Levitt MA
        Colostomy in anorectal malformations: a procedure with serious but preventable complications.
        J Pediatr Surg. 2006; 41: 748-756
        • Bischoff A
        • Levitt MA
        • Peña A
        Bowel management for the treatment of pediatric fecal incontinence.
        Pediatr Surg Int. 2009; 25: 1027-1042
        • Bischoff A
        • Levitt MA
        • Bauer C
        • et al.
        Treatment of fecal incontinence with a comprehensive bowel management program.
        J Pediatr Surg. 2009; 6: 1278-1284
        • Bischoff A
        • Levitt MA
        • Peña A
        Laparoscopy and its use in the Repair of anorectal malformations.
        J Pediatr Surg. 2011; 46: 1609-1617