Urachal Anomalies

  • Mohamed Fahmy
Chapter

Abstract

Urachal duct remnant is the most common congenital urachus anomaly; these anomalies are more common than previously thought, with more cases discovered incidentally, because of the increased use of cross-sectional imaging antenatally. Although an abnormal persistence of an embryologic communication between the bladder and the umbilicus is often recognized and managed in childhood, it may persist into adulthood, with a greater risk of morbidity. Patent urachus, a persistent tubular structure connecting the dome of the bladder to the umbilicus, represents 50% of urachal anomalies. It is usually detected neonatally because of leakage of the urine from the umbilicus. Of note, one-third of these cases occur in association with bladder outlet obstruction caused by posterior urethral valves or urethral atresia. The frequency of other urachal anomalies is as follows: urachal cyst (30%), umbilical–urachal sinus (15%) and vesicourachal diverticulum (3–5%). The sinus is a tract with a blind ending at the umbilicus. The diverticulum represents a distal dilation of the bladder directed towards, but not communicating with, the umbilicus; most diverticula are asymptomatic.

Congenital urachal anomalies that are detected early can benefit from an optimized management including surgical approach with a complete resection of the urachal remnant in cases when spontaneous resolution has failed; also laparoscopy is implemented for diagnosis and deals with urachal anomalies at different age group. At imaging, the different types of urachal anomalies have a distinct appearance; a patent urachus is recognized as an elongated patent connection between the bladder and the umbilicus, and an umbilical–urachal sinus is depicted as a blind focal dilation at the umbilical end, whereas a vesicourachal diverticulum is a focal outpouching at the vesical end. Urachal cysts are visualized as midline fluid-filled sacs most frequently located near the bladder dome. Complications of urachal anomalies have nonspecific clinical findings and can mimic other abdominal and pelvic lesions. Complications, such as infection and tumors, should be also recognized early to ensure optimal management. Understanding of the embryonic development of the urachus is necessary for the surgeons to diagnose the wide variety of urachal disease.

Keywords

Urachus Allantois Remnants Prenatal management Urachal Carcinoma 

References

  1. 1.
    Tolaymat LL, Maher JE, Kleinman GE, et al. Persistent patent urachus with allantoic cyst: a case report. Ultrasound Obstet Gynecol. 1997;10(5):366–8. doi: 10.1046/j.1469-0705.1997.10050366.x-.CrossRefPubMedGoogle Scholar
  2. 2.
    Moore KL, Persaud TVN. The developing human: clinically oriented embryology. 5th ed. Philadelphia: Saunders; 1993.Google Scholar
  3. 3.
    Skandalakis JA, Gray SW. Embryology for surgeons. Baltimore: Williams & Wilkins; 1994. p. 675–81.Google Scholar
  4. 4.
    Pazos HMF, Costa WS, Sampaio FJB, Favorito LA. Structural and ontogenetic study of the urachus in human fetuses. Cells Tissues Organs. 2010;191:422–30. doi: 10.1159/000258785.CrossRefPubMedGoogle Scholar
  5. 5.
    Rubin A. A handbook of congenital malformations. Philadelphia: Saunders; 2009.Google Scholar
  6. 6.
    Begg RC. The urachus and umbilical fistulae. Surg Gynecol Obstet. 1927;45:16–78.Google Scholar
  7. 7.
    Bilchert M, Nielsen OV. Congenital patent urachus and acquired variants. Acta Chir Scand. 1971;137:807–14.Google Scholar
  8. 8.
    Yu J, Kim K, Lee H, Lee Y, Yoon C, Kim M. Urachal remnant disease: spectrum of CT and US findings. Radiographics. 2001;21:451–6.CrossRefPubMedGoogle Scholar
  9. 9.
    Berman SM, Berman BM, Laor E, Freed SZ. Urachal remnants in adults. Urology. 1988;31(1):17–21. doi: 10.1016/0090-4295(88)90564-X.CrossRefPubMedGoogle Scholar
  10. 10.
    Herman TE, Siegel MJ. Prune belly syndrome. J Perinatol. 2009;29:69–71.CrossRefPubMedGoogle Scholar
  11. 11.
    Bourdelat D, Husson S, Soisic F, Vrsansky P. Embryological study of the mechanism of antenatal lower urinary tract obstruction. Ann Urol (Paris). 1998;32(4):253–68.Google Scholar
  12. 12.
    Sadler TW. Urogenital system. In: Longman’s medical embryology. 10th ed. Philadelphia: Lippincott, Williams & Wilkins; 2006. p. 236–8.Google Scholar
  13. 13.
    Bureau M, Bolduc S. Allantoic cysts and posterior urethral valves: a case report. Ultrasound Obstet Gynecol. 2011;38:116–8. doi: 10.1002/uog.8910.CrossRefPubMedGoogle Scholar
  14. 14.
    Gobet R, Bleakley J, Craig A. Peterspremature urachal closure induces hydroureteronephrosis in male fetuses. J Urol. 1998;160(4):1463–7. doi: 10.1016/S0022-5347(01)62592-8.CrossRefPubMedGoogle Scholar
  15. 15.
    Fox JA, McGee SM, Routh JC, Granberg CF, Ashley RA, Hutcheson JC, et al. Vesicoureteral reflux in children with urachal anomalies. J Pediatr Urol. 2011;7:632–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Atobatele MO, Olalekan IO, Abdulrasheed AN, John OB. Posterior urethral valve with unilateral vesicoureteral reflux and patent urachus: a rare combination of urinary tract anomalies. Urol Ann. 2015;7(2):240–3. doi: 10.4103/0974-7796.150496.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Razvi S, Murphy R, Shlasko E, Cunningham-Rundles C. Delayed separation of the umbilical cord attributable to urachal anomalie. Pediatrics. 2001;108(2):493–4.CrossRefPubMedGoogle Scholar
  18. 18.
    Kilicdag EB, et al. Large pseudocyst of the umbilical cord associated with patent urachus. J Obstet Gynaecol Res. 2004;30(6):444–7.CrossRefPubMedGoogle Scholar
  19. 19.
    Little DC, Shah SR, St Peter SD, et al. Urachal anomalies in children: the vanishing relevance of the preoperative voiding cystourethrogram. J Pediatr Surg. 2005;40:1874–6.CrossRefPubMedGoogle Scholar
  20. 20.
    Flanagan DA, Mellinger JD. Urachal-sigmoid fistula in an adult male. Am Surg. 1998;64:762–3.PubMedGoogle Scholar
  21. 21.
    Lugo B, McNulty J, Emil S. Bladder prolapse through a patent urachus: fetal and neonatal features. J Pediatr Surg. 2006;41(5):e5–7. doi: 10.1016/j.jpedsurg.2005.12.062.CrossRefPubMedGoogle Scholar
  22. 22.
    Galati V, Donovan B, Ramji F, et al. Management of urachal remnants in early childhood. J Urol. 2008;180(4 Suppl):1824e6. [discussion 1827]Google Scholar
  23. 23.
    Siow SL, Mahendran HA, Hardin M. Laparoscopic management of symptomatic urachal remnants in adulthood. Asian J Surg. 2015;38(2):85–90. doi: 10.1016/j.asjsur.2014.04.009.CrossRefPubMedGoogle Scholar
  24. 24.
    Rivera M, Granberg CF, Tollefson MK. Robotic-assisted laparoscopic surgery of urachal anomalies: a single-center experience. J Laparoendosc Adv Surg Tech A. 2015;25(4):291–4. doi: 10.1089/lap.2014.0551.CrossRefPubMedGoogle Scholar
  25. 25.
    Widni EE, Höllwarth ME, Haxhija QE. The impact of preoperative ultrasound on correct diagnosis of urachal remnants in children. J Pediatric Surg. 2010;45:1433–7.CrossRefGoogle Scholar
  26. 26.
    Pal K, Ashri H, Al-Ghazal FA. Allantoic cyst and urachus. Indian J Pediatr. 2009;76:221–3.CrossRefPubMedGoogle Scholar
  27. 27.
    Ohgaki M, Higuchi A, Chou H, Takashina K, Kawakami S, Fujita Y, Hagiwara A, Yamagishi H. Acute peritonitis caused by intraperitoneal rupture of an infected urachal cyst: report of a case. Surg Today. 2003;33:75–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Mazzucchelli R, Scarpelli M, Montironi R. Mucinous: adenocarcinoma with superficial stromal invasion and villous adenoma of urachal remnants: a case report. J Clin Pathol. 2003;56:465–7.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Blichert-Toft M, Nielson OV. Congenital patent urachus and acquired variants: diagnosis and treatment. Review of the literature and report of 5 cases. Acta Chir Scand. 1971;137:807–14.PubMedGoogle Scholar
  30. 30.
    Pesce C, Costa L, Musi L, Campobasso P, Zimbardo L. Relevance of infection in children with urachal cysts. Eur Urol. 2000;38:457–60.CrossRefPubMedGoogle Scholar
  31. 31.
    Seo IY, Park SC, Oh SJ. Laparoscopic excision of complicated urachal cyst in child. Korean J Urol. 2005;46(3):324–6.Google Scholar
  32. 32.
    Walsh SA, Weiss RM. Case report: persistent dysuria and a suprapubic mass in a 3-year-old boy. Curr Opin Pediatr. 2002;14:647–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Tiao MM, Ko SF, Huang SC, Shieh CS, Chen CL. Urachal inflammatory mass mimicking an intra-abdominal tumor two years after excision of the urachal sinus in a child. Chang Gung Med J. 2003;26(8):598–601.PubMedGoogle Scholar
  34. 34.
    Yiee JH, Garcia N, Baker LA, Barber R, Snodgrass WT, Wilcox DT. A diagnostic algorithm for urachal anomalies. J Pediatr Urol. 2007;3:500–4.CrossRefPubMedGoogle Scholar
  35. 35.
    Wang B, Tashiro J, Pelaez L, Rodriguez MM, Perez EA, Neville HL, Sola JE. A unique presentation and rare pathological finding for urachal sinus. J Pediatric Surg. 2013;48(9):1977–80. doi: 10.1016/j.jpedsurg.2013.07.002.CrossRefGoogle Scholar
  36. 36.
    El Ammari JE, Ahallal Y, El Yazami Adli O, El Fassi MJ, Farih MH. Urachal sinus presenting with abscess formation. ISRN Urol. 2011;2011:820924. doi: 10.5402/2011/820924.CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Mistrya KA, et al. Late presentation of congenital urachal sinus in a middle aged male complicated by an umbilical abscess: a case report. Egypt J Radiol Nuclear Med. 2015;46(3):755–9. doi: 10.1016/j.ejrnm.2015.04.010.CrossRefGoogle Scholar
  38. 38.
    Blichert-Toft M, Nielsen OV. Diseases of the urachus simulating intra-abdominal disorders. Am J Surg. 1971;122:123–8.CrossRefPubMedGoogle Scholar
  39. 39.
    Ozbülbül NI, Dagli M, Akdogan G, Olçer T. CT urography of a vesicourachal diverticulum containing calculi. Diagn Interv Radiol. 2010;16:56–8.PubMedGoogle Scholar
  40. 40.
    Ansari MS, Hemal AK. A rare case of urachovesical calculus: a diagnostic dilemma and endo-laparoscopic management. J Laparoendosc Adv Surg Tech A. 2002;12:281–3.CrossRefPubMedGoogle Scholar
  41. 41.
    Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE. Malignant urachal lesions. J Urol. 1984;131:1–8.CrossRefPubMedGoogle Scholar
  42. 42.
    Ghazizadeh M, Yamamoto S, Kurokawa K. Clinical features of urachal carcinoma in Japan: review of 157 patients. Urol Res. 1983;11(5):235–8.CrossRefPubMedGoogle Scholar
  43. 43.
    Gopalan A, Sharp DS, Fine SW. Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol. 2009;33:659–68.CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Szarvas T, et al. Clinical, prognostic, and therapeutic aspects of urachal carcinoma—a comprehensive review with meta-analysis of 1,010 cases. Urol Oncol: Semin Orig Invest. 2016;34(9):388–98.CrossRefGoogle Scholar
  45. 45.
    Bruins HM, et al. The clinical epidemiology of urachal carcinoma: results of a large, population based study. J Urol. 2012;188(4):1102–7. doi: 10.1016/j.juro.2012.06.020.CrossRefPubMedGoogle Scholar
  46. 46.
    Herr HW, Bochner BH, Sharp D, et al. Urachal carcinoma: contemporary surgical outcomes. J Urol. 2007;178:74.CrossRefPubMedGoogle Scholar
  47. 47.
    Molina JR, Quevedo JF, Furth AF, et al. Predictors of survival from urachal cancer: a mayo clinicstudy of 49 cases. Cancer. 2007;110:2434.CrossRefPubMedGoogle Scholar
  48. 48.
    Ashley RA, Inman BA, Sebo TJ, et al. Urachal carcinoma: clinicopathologic features and long-term outcomes of an aggressive malignancy. Cancer. 2006;107:712.CrossRefPubMedGoogle Scholar
  49. 49.
    Charles HM. Exstrophy of the bladder and its treatment. JAMA. 1917;25:2079–81. doi: 10.1001/jama.1917.02590520001001.CrossRefGoogle Scholar
  50. 50.
    Sakellaris G, Cervellione RM, Dickson AP. An unusual bladder/cloacal exstrophy with urachal exstrophy. Urol Int. 2008;81(1):113–5. doi: 10.1159/000137651.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • Mohamed Fahmy
    • 1
  1. 1.Pediatric SurgeryAl Azher University Pediatric SurgeryCairoEgypt

Personalised recommendations