Pediatric Surgery for Bladder Anomalies 

  • Chief Editor: Marc Cendron, MD   more...
 
Updated: Jan 25, 2010
 

Background

This article discusses some of the more common bladder anomalies, such as bladder diverticula, and some of the uncommon bladder anomalies, such as bladder ears, congenital hypoplasia of the bladder, megacystis, bladder agenesis, duplication anomalies of the bladder, and finally, bladder septa. A discussion of urachal anomalies (ie, urachal sinus, urachal cyst, urachal diverticulum, patent urachus) is also included.[1, 2]

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Pathophysiology

The timing of altered embryogenesis leading to these conditions has been a matter of speculation, and the embryologic cause of these lesions is unknown. Bladder development occurs during the fifth to seventh week of gestational development. Development depends upon many factors, such as proper mesenchymal differentiation, mesenchymal growth, urine production that stimulates bladder expansion, and detrusor contraction. Bladder cycling, the process of sequential expansion and contraction, is important in the anatomic and physiologic development of the normal bladder. Bladder ears are considered a normal variation, not a congenital anomaly.

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Epidemiology

Frequency

United States

Bladder diverticula are uncommon, but not rare. In a series of over 5000 children studied, the approximate incidence was 1.7%. All of the other entities are uncommon or rare.

Mortality/Morbidity

Most of these conditions have a low mortality rate and little morbidity.

  • Bladder diverticula and vesicourachal diverticula often cause no symptoms, but in symptomatic cases, patients most often present with urinary tract infection. Rarely, bladder diverticula and vesicourachal diverticula may cause bladder outlet obstruction. Death from peritonitis due to a ruptured infected urachal cyst has been reported in a few cases, but most bladder anomalies are incidental findings, and patients are asymptomatic.
  • Patent urachal anomalies do not usually cause significant morbidity or mortality. However, adenocarcinoma has been reported in adults in urachal remnants, presumably from chronic inflammation and infection.
  • Bladder agenesis generally is incompatible with life.
  • Megacystitis, bladder duplication, and bladder septation are rare. Morbidity of these conditions is generally related to associated abnormalities (when present) such as high-grade vesicoureteral reflux or renal dysplasia.
  • Only 50 reported cases of bladder duplication exist.[3, 4]
  • Bladder septations are equally rare.

Sex

All of these anomalies are infrequent or rare, and each condition occurs in both males and females. Data are insufficient to provide reasonably accurate frequency differences in the sexes.

Age

Bladder anomalies are generally diagnosed in infancy or childhood. With the advent of prenatal ultrasonography, such abnormalities are fully evaluated after birth. When not detected by antenatal screening, most are discovered in the evaluation of a urinary tract infection or, in the case of urachal anomalies, periumbilical drainage or redness.

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Contributor Information and Disclosures
Coauthor(s)

Bartley G Cilento, Jr, MD  Instructor, Department of Surgery, Division of Urology, Children's Hospital of Boston and Harvard Medical School

Bartley G Cilento, Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Martin David Bomalaski, MD, FAAP  Pediatric Urologist, Alpine Urology

Martin David Bomalaski, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American Urological Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Harry P Koo, MD  Chairman of Urology Division and Director of Pediatric Urology, Virginia Commonwealth University; Professor of Surgery, VCU School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond

Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Urological Association

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Marc Cendron, MD  Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston

Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, European Society for Paediatric Urology, Johns Hopkins Medical and Surgical Association, New Hampshire Medical Society, Society for Fetal Urology, and Society for Pediatric Urology

Disclosure: Nothing to disclose.

References
  1. Caldamone AA. Anomalies of the bladder and cloaca. In: Gillenwater JY, Grayhack JT, Howards SS, et al, eds. Adult and Pediatric Urology. 2nd ed. St. Louis, Mo: Mosby Year Book; 1987:2023.

  2. Cilento BG, Nguyen HT. Bladder diverticula, urachal anomalies, and other uncommon anomalies of the bladder. In: Gearhart JP, Rink RC, Mouriquand P, eds. Pediatric Urology. Philadelphia, Pa: WB Saunders; 2001.

  3. Tacciuoli M, Laurenti C, Racheli T. Double bladder with complete sagittal septum: diagnosis and treatment. Br J Urol. Dec 1975;47(6):645-9. [Medline].

  4. Coker AM, Allshouse MJ, Koyle MA. Complete duplication of bladder and urethra in a sagittal plane in a male infant: case report and literature review. J Pediatr Urol. Aug 2008;4(4):255-9. [Medline].

  5. Blane CE, Serin JM, Bloom DA. Bladder diverticula in children. Radiology. 1194;190:695. [Medline].

  6. Stephens FD. The vesicoureteral hiatus and paraureteral diverticula. J Urol. Jun 1979;121(6):786-91. [Medline].

  7. Pieretti RV, Pieretti-Vanmarcke RV. Congenital bladder diverticula in children. Journal of Pediatric Surgery. 1999;34:468. [Medline].

  8. Verghese M, Belman AB. Urinary retention secondary to congenital bladder diverticula in infants. J Urol. Dec 1984;132(6):1186-8. [Medline].

  9. Barrett DM, Malek RS. Observations on vesical diverticulum in childhood. Journal of Urology. 1976;116:234. [Medline].

  10. Burbige KA, Lebowitz RL, Colodny AH. The megacystis-megaureter syndrome. J Urol. Jun 1984;131(6):1133-6. [Medline].

  11. Levard G, Aigrain Y, Ferkadji L. Urinary bladder diverticula and the Ehlers-Danlos syndrome in children. J Pediatr Surg. Nov 1989;24(11):1184-6. [Medline].

  12. Burns E, Cummins H, Hyman J. Incomplete reduplication of the bladder. Journal of Urology. 1947;57:257.

  13. Shokeir AA, Ashamallah A, Abol-Enein H. Incomplete bladder duplication. Br J Urol. Jan 1995;75(1):106-7. [Medline].

  14. Cilento BG Jr, Bauer SB, Retik AB. Urachal anomalies: defining the best diagnostic modality. Urology. Jul 1998;52(1):120-2. [Medline].

  15. Powell CR, Kreder KJ. Treatment of bladder diverticula, impaired detrusor contractility, and low bladder compliance. Urol Clin North Am. Nov 2009;36(4):511-25, vii. [Medline].

  16. Yohannes P, Bruno T, Pathan M, Baltaro R. Laparoscopic radical excision of urachal sinus. J Endourol. Sep 2003;17(7):475-9; discussion 479. [Medline].

  17. Castillo OA, Vitagliano G, Olivares R, Sanchez-Salas R. Complete excision of urachal cyst by laparoscopic means: a new approach to an uncommon disorder. Arch Esp Urol. Jun 2007;60(5):607-11. [Medline].

  18. Chiarenza SF, Scarpa MG, D'Agostino S, Fabbro MA, Novek SJ, Musi L. Laparoscopic excision of urachal cyst in pediatric age: report of three cases and review of the literature. J Laparoendosc Adv Surg Tech A. Apr 2009;19 Suppl 1:S183-6. [Medline].

  19. Hutch JA. Saccule formation at the ureterovesical junction in smooth-walled bladders. Journal of Urology. 1961;86:390.

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Voiding cystourethrogram showing a bladder diverticulum arising from the posterior aspect of the bladder.
Voiding cystourethrogram showing 2 posteriorly placed bladder diverticula.
Bladder ultrasound showing 2 posteriorly placed bladder diverticula.
Duplicated bladder with a urethral catheter placed into each bladder.
Voiding cystourethrogram demonstrating a duplicated bladder.
Intravenous pyelogram demonstrating a duplicated bladder. Notice how each ureter drains into the ipsilateral bladder.
Urachal cyst at the level of the umbilicus.
Bladder anomalies. Ultrasound demonstrating urachal cyst. Ultrasound cursors mark the extent of the cyst.
Voiding cystourethrogram showing megacystitis. Bilateral vesicoureteral reflux is also observed (grade 3 on the right, grade 2 on the left).
 
 
 
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