OMIA:001891-9615 : Ectopic ureter in Canis lupus familiaris (dog)

In other species: horse

Categories: Renal / urinary system phene

Links to MONDO diseases: No links.

Mendelian trait/disorder: no

Mode of inheritance: Multifactorial

Considered a defect: yes

Key variant known: no

Inheritance: From records of 1611 Entlebucher Mountain Dogs, Fritsche et al. (2013) estimated heritability of this disorder to be 65%, with some evidence of involvement of a gene of large effect.

Genetic engineering: Unknown
Have human generated variants been created, e.g. through genetic engineering and gene editing

Clinical features: Ectopic ureter (EU) is a congenital malformation where one or both ureters enter the bladder in an abnormal anatomical position, outside of the trigone area. The ureters enter on the bladder neck, urethra, uterus or vagina (Reichler et al., 2012). Ectopic ureters can be classified in intramural and extramural, depending on if they bypass the bladder or not. The extramural EU bypasses the bladder completely and opens into the possible abnormal anatomical structures, while the intramural enters the bladder wall in the correct anatomic position (trigone area) but extends within the bladder to then open into an abnormal position. Urinary incontinence is the most common clinical sign of EU and affected dogs often present at a very young age (Koie et al., 2000), with males also commonly showing signs of urinary incontinence at a more advanced age (Holt and Moore, 1995). Diagnosis of EU is best achieved by different imaging methods such as intravenous urography and ultrasonography (Lamb and Gregory, 1998; Koie et al., 2000; Taylor et al., 2022). Treatment involves surgical reposition of ectopic ureter into the bladder and medication for incontinence when needed (Koie et al., 2000). [IT thanks DVM student Ginady Martinez Casiano, who provided the basis of this contribution in April 2022]

Pathology: Urinary tract infections related to urinary incontinence are common in presentation of dogs with EU (Fritsche et al., 2013). These result in inflammation throughout the urinary tract (e.g. urethritis, cystitis, ureteritis, pyelonephritis). In a large portion of cases, EU has also been associated with other urogenital conformational or functional abnormalities such as hydronephrosis, dilated ureters, vestibulo-vaginal malformations, hypoplastic bladder or kidney, congenital urethral sphincter mechanism incompetence (USMI), ureterocele, renal dysgenesis, pelvic bladder and others (Reichler et al., 2012; Dekerle et al., 2022). [IT thanks DVM student Ginady Martinez Casiano, who provided the basis of this contribution in April 2022]

Breed: Entlebucher Mountain Dog (Dog) (VBO_0200503).
Breeds in which the phene has been documented. For breeds in which a likely causal variant has been documented, see the variant table below

Cite this entry

Nicholas, F. W., Tammen, I., & Sydney Informatics Hub. (2022). OMIA:001891-9615: Online Mendelian Inheritance in Animals (OMIA) [dataset]. https://omia.org/. https://doi.org/10.25910/2AMR-PV70

References

Note: the references are listed in reverse chronological order (from the most recent year to the earliest year), and alphabetically by first author within a year.

2022 Dekerle, B., Maurice, E., Decambron, A., Viateau, V., Maurey, C., Manassero, M. :
Outcomes of 25 female dogs treated for ectopic ureters by open surgery or cystoscopic-guided laser ablation. Vet Surg 51:568-575, 2022. Pubmed reference: 35348227. DOI: 10.1111/vsu.13807.
Greenfield, Z.P., Berent, A.C., Weisse, C.W. :
Urinary incontinence in a dog with a duplex renal system and extramural ectopic ureter. J Small Anim Pract 63:234-238, 2022. Pubmed reference: 34263926. DOI: 10.1111/jsap.13399.
Hooi, K.S., Vachon, C., Martel, D.V., Dunn, M. :
Retrospective evaluation of cystoscopic-guided laser ablation of intramural ectopic ureters in female dogs. J Vet Intern Med 36:156-163, 2022. Pubmed reference: 34877712. DOI: 10.1111/jvim.16321.
Jacobson, E., Meler, E.N., Delisser, P.J., Thompson, A.L. :
Cystoscopic-guided scissor transection of intramural ectopic ureters as a novel alternate minimally invasive treatment option to laser ablation in female dogs: 8 cases (2011-2020). J Am Vet Med Assoc :1-9, 2022. Pubmed reference: 35290202. DOI: 10.2460/javma.21.03.0153.
Merz, F., Gallana, M., Hartnack, S., Del Chicca, F., Dolf, G., Hungerbühler, S., Hittmair, K.M., Dorsch, R., Zaal, M., Vink-Nooteboom, M., Hartmann, A., Pieńkowska-Schelling, A., Schelling, C., Reichler, I.M. :
[Outcome of selective mating in the Entlebucher Mountain Dog for reduction of ureteral ectopia]. Schweiz Arch Tierheilkd 164:535-546, 2022. Pubmed reference: 35791823. DOI: 10.17236/sat00363.
Taylor, O., Knight, R., Genain, M.A., Owen, L. :
Ultrasonography as a sensitive and specific diagnostic modality for the detection of ectopic ureters in urinary incontinent dogs. Vet Radiol Ultrasound 63:328-336, 2022. Pubmed reference: 35064712. DOI: 10.1111/vru.13055.
2021 Griffin, M.A., Culp, W.T.N., Munro, M.J.L., Palm, C.A., Wanamaker, M.W. :
Surgical treatment of retroiliac ectopic ureters with secondary hydronephrosis and hydroureter in a dog. J Am Vet Med Assoc 258:740-747, 2021. Pubmed reference: 33754813. DOI: 10.2460/javma.258.7.740.
Hoey, C.S.F.K., Friend, E., Meakin, L.B., Chanoit, G.P. :
Long-term outcome of female dogs treated for intramural ectopic ureters with cystoscopic-guided laser ablation. Vet Surg 50:1449-1462, 2021. Pubmed reference: 34350607. DOI: 10.1111/vsu.13702.
Schwarz, T., Bommer, N., Parys, M., Thierry, F., Bouvard, J., Pérez-Accino, J., Saunders, J., Longo, M. :
Four-dimensional CT excretory urography is an accurate technique for diagnosis of canine ureteral ectopia. Vet Radiol Ultrasound 62:190-198, 2021. Pubmed reference: 33350535. DOI: 10.1111/vru.12934.
Werner, M., Meyer-Lindenberg, A., Felten, S., Hartmann, K., Dorsch, R. :
[Cystoscopic-guided laser ablation for treatment of ectopic ureteroceles in 2 female dogs]. Tierarztl Prax Ausg K Kleintiere Heimtiere 49:310-316, 2021. Pubmed reference: 33890272. DOI: 10.1055/a-1428-7266.
2018 Gallana, M., Utsunomiya, Y.T., Dolf, G., Pintor Torrecilha, R.B., Falbo, A.K., Jagannathan, V., Leeb, T., Reichler, I., Sölkner, J., Schelling, C. :
Genome-wide association study and heritability estimate for ectopic ureters in Entlebucher mountain dogs. Anim Genet 49:645-650, 2018. Pubmed reference: 30276844. DOI: 10.1111/age.12728.
2016 Fox, A.J., Sharma, A., Secrest, S.A. :
Computed tomographic excretory urography features of intramural ectopic ureters in 10 dogs. J Small Anim Pract 57:210-3, 2016. Pubmed reference: 27000650. DOI: 10.1111/jsap.12460.
2014 Fritsche, R., Dolf, G., Schelling, C., Hungerbuehler, S.O., Hagen, R., Reichler, I.M. :
Inheritance of ectopic ureters in Entlebucher Mountain Dogs. J Anim Breed Genet 131:146-52, 2014. Pubmed reference: 24628724. DOI: 10.1111/jbg.12055.
Newman, M., Landon, B. :
Surgical treatment of a duplicated and ectopic ureter in a dog. J Small Anim Pract 55:475-8, 2014. Pubmed reference: 25168741. DOI: 10.1111/jsap.12227.
Volstad, N.J., Beck, J., Burgess, D.M. :
Correction of intramural ureteral ectopia by ureteral transection and neoureterostomy with the distal ureter left in situ. Aust Vet J 92:81-4, 2014. Pubmed reference: 24571343. DOI: 10.1111/avj.12147.
2013 Fritsche, R., Dolf, G., Schelling, C., Hungerbuehler, S.O., Hagen, R., Reichler, I.M. :
Inheritance of ectopic ureters in Entlebucher Mountain Dogs. Journal of Animal Breeding and Genetics 131:146-152, 2013. DOI: 10.1111/jbg.12055.
Novellas, R., Stone, J., Pratschke, K., Hammond, G. :
Duplicated ectopic ureter in a nine-year-old Labrador. J Small Anim Pract 54:386-9, 2013. Pubmed reference: 23551207. DOI: 10.1111/jsap.12056.
2012 Reichler, I.M., Eckrich Specker, C., Hubler, M., Boos, A., Haessig, M., Arnold, S. :
Ectopic ureters in dogs: clinical features, surgical techniques and outcome. Vet Surg 41:515-22, 2012. Pubmed reference: 22486391. DOI: 10.1111/j.1532-950X.2012.00952.x.
2011 Bitterli, F. :
Prävalenz und klinische Relevanz ektopischer Ureteren beim Entlebucher und Appenzeller Sennenhund. Dissertation, Vetsuisse Faculty, University of Zurich, Zurich , 2011.
2010 North, C., Kruger, J.M., Venta, P.J., Miller, J.M., Rosenstein, D.S., Randall, E.K., White, B., Fitzgerald, S.D. :
Congenital ureteral ectopia in continent and incontinent-related Entlebucher mountain dogs: 13 cases (2006-2009). J Vet Intern Med 24:1055-62, 2010. Pubmed reference: 20666980. DOI: 10.1111/j.1939-1676.2010.0562.x.
Smith, A.L., Radlinsky, M.G., Rawlings, C.A. :
Cystoscopic diagnosis and treatment of ectopic ureters in female dogs: 16 cases (2005-2008). J Am Vet Med Assoc 237:191-5, 2010. Pubmed reference: 20632793. DOI: 10.2460/javma.237.2.191.
Thomas, P.C., Yool, D.A. :
Delayed-onset urinary incontinence in five female dogs with ectopic ureters. J Small Anim Pract 51:224-6, 2010. Pubmed reference: 20214729. DOI: 10.1111/j.1748-5827.2009.00893.x.
Yoon, H.Y., Mann, F.A., Punke, J.P., Jeong, S.W. :
Bilateral ureteral ectopia with renal dysplasia and urolithiasis in a dog. J Am Anim Hosp Assoc 46:209-14, 2010. Pubmed reference: 20439946.
2006 Eckrich Specker, C. :
Ektopische Ureteren beim Hund: Eine retrospektive Analyse von 30 Fällen. Dissertation, Vetsuisse Faculty, University of Zurich, Zurich , 2006.
2004 Samii, V.F., McLoughlin, M.A., Mattoon, J.S., Drost, W.T., Chew, D.J., DiBartola, S.P., Hoshaw-Woodard, S. :
Digital fluoroscopic excretory urography, digital fluoroscopic urethrography, helical computed tomography, and cystoscopy in 24 dogs with suspected ureteral ectopia. J Vet Intern Med 18:271-81, 2004. Pubmed reference: 15188811.
2003 Cannizzo, K.L., McLoughlin, M.A., Mattoon, J.S., Samii, V.F., Chew, D.J., DiBartola, S.P. :
Evaluation of transurethral cystoscopy and excretory urography for diagnosis of ectopic ureters in female dogs: 25 cases (1992-2000). J Am Vet Med Assoc 223:475-81, 2003. Pubmed reference: 12930085.
Oglesby, P.A., Carter, A. :
Ultrasonographic diagnosis of unilateral ectopic ureter in a Labrador dog. J S Afr Vet Assoc 74:84-6, 2003. Pubmed reference: 15029953.
Taney, K.G., Moore, K.W., Carro, T., Spencer, C. :
Bilateral ectopic ureters in a male dog with unilateral renal agenesis. J Am Vet Med Assoc 223:817-20, 810, 2003. Pubmed reference: 14507098.
2002 Lautzenhiser, S.J., Bjorling, D.E. :
Urinary incontinence in a dog with an ectopic ureterocele. J Am Anim Hosp Assoc 38:29-32, 2002. Pubmed reference: 11804312.
2000 Holt, P.E., Thrusfield, M.V., Hotston Moore, A. :
Breed predisposition to ureteral ectopia in bitches in the UK. Veterinary Record 146:561 only, 2000.
Koie, H., Yamaya, Y., Sakai, T. :
Four cases of lowered urethral pressure in canine ectopic ureter. J Vet Med Sci 62:1221-2, 2000. Pubmed reference: 11129871.
1999 Anderson, C.C., Cook, C.R., Pope, E.R. :
What is your diagnosis? Ectopic ureter. J Am Vet Med Assoc 214:1321-2, 1999. Pubmed reference: 10319171.
1998 Lamb, C.R., Gregory, S.P. :
Ultrasonographic findings in 14 dogs with ectopic ureter. Vet Radiol Ultrasound 39:218-23, 1998. Pubmed reference: 9634190.
1995 Holt, P.E., Moore, A.H. :
Canine ureteral ectopia: an analysis of 175 cases and comparison of surgical treatments. Veterinary Record 136:345 only, 1995.
1988 Dean, P.W., Bojrab, M.J., Constantinescu, G.M. :
Canine ectopic ureter. Compend. Contin. Educ. Vet. 10:146-162, 1988.
1986 Hager, D.A., Blevins, W.E. :
Ectopic ureter in a dog: extension from the kidney to the urinary bladder and to the urethra. J Am Vet Med Assoc 189:309-10, 1986. Pubmed reference: 3744991.
1984 Hayes, H.M. :
Breed associations of canine ectopic ureter: a study of 217 female cases. J. Small Anim. Pract. 25:501–504, 1984.
1982 Holt, P.E., Gibbs, C., Pearson, H. :
Canine ectopic ureter – a review of twenty-nine cases. J. Small Anim. Pract. 23:195–208, 1982.
1981 Smith, C.W., Stowater, J.L., Kneller, S.K. :
Ectopic ureter in the dog: a review of cases. J. Am. Anim. Hosp. Assoc. 17:245-248, 1981.
1980 Smith, C.W., Stowater, J.L., Kneller, S.K. :
Bilateral ectopic ureter in a male dog with urinary incontinence. J Am Vet Med Assoc 177:1022-4, 1980. Pubmed reference: 7462081.
1977 Johnston, G.R., Osbourne, C.A., Wilson, J.W., Yano, B.L. :
Familial ureteral ectopia in the dog. J. Am. Anim. Hosp. Assoc. 13:168–170, 1977.
1976 Holt, P.E. :
Ectopic ureter in the bitch. Vet Rec 98:299-300, 1976. Pubmed reference: 1274140.
1975 Osborne, C.A., Dieterich, H.F., Hanlon, G.F., Anderson, L.D. :
Urinary incontinence due to ectopic ureter in a male dog. J Am Vet Med Assoc 166:911-4, 1975. Pubmed reference: 1168184.
1974 Hayes, H.M. :
Ectopic ureter in dogs: epidemiologic features. Teratology 10:129-32, 1974. Pubmed reference: 4428421. DOI: 10.1002/tera.1420100206.
1973 Lane, J.G. :
Canine ectopic ureter--two further case reports. J Small Anim Pract 14:555-60, 1973. Pubmed reference: 4789312.
1971 Seidenberg, L., Knecht, C.D. :
Ectopic ureter in the dog. J Am Vet Med Assoc 159:876-7, 1971. Pubmed reference: 5106547.
1969 Osborne, C.A., Perman, V. :
Ectopic ureter in a male dog. J Am Vet Med Assoc 154:273-8, 1969. Pubmed reference: 5812515.

Edit History


  • Created by Frank Nicholas on 25 Nov 2013
  • Changed by Frank Nicholas on 25 Nov 2013
  • Changed by Imke Tammen2 on 03 Jun 2022