Ectopic Ureter: Symptoms, Types, Causes and Treatment

The ectopic ureter is one of the more intriguing and challenging congenital anomalies of the urinary tract. It refers to a ureter that drains somewhere other than its normal insertion point in the bladder. This condition can lead to a wide variety of symptoms, from incontinence in young girls to recurrent infections in boys and adults. Understanding the symptoms, types, causes, and treatments of ectopic ureter is crucial for timely diagnosis and effective management. In this comprehensive guide, we'll break down the key facts, provide insightful tables, and delve deeper into the clinical and anatomical considerations.

Symptoms of Ectopic Ureter

When it comes to ectopic ureter, symptoms can vary widely depending on age, sex, and the exact site of the abnormal ureteral insertion. Recognizing these symptoms is vital for early detection and intervention.

Symptom	Population/Age	Key Feature	Source(s)
Urinary Incontinence	Females, children	Continuous dribbling or wetting	2 3 5 8 12
Urinary Tract Infection (UTI)	All ages	Recurrent infections, fever	1 2 4 5 6 14
Voiding/Ejaculatory Symptoms	Males, adolescents/adults	Perineal/genital pain, ejaculatory issues	4 8
Pelvic/Abdominal Mass	Infants/children	Palpable mass, hydronephrosis	1 6 8
Lumbar/Abdominal Pain	All ages	Flank pain, discomfort	4 6 14
Epididymitis	Males	Testicular pain/swelling	1 5

Table 1: Key Symptoms

Understanding the Symptoms

Urinary Incontinence

• Most common in females, especially young girls, due to the ureter inserting below the urinary sphincter, resulting in continuous dribbling from birth or early childhood. Unlike typical incontinence, this is continuous and not just during the urge or stress events.
• In boys and men, incontinence is rare because the abnormal opening is often above the external sphincter, but it can occur in specific cases, especially if the ureter opens into the urethra distal to the sphincter or into reproductive organs 3 4 5 8.

Recurrent Urinary Tract Infections

• Both males and females may present with recurrent UTIs, sometimes associated with fever or systemic symptoms.
• Infections are often due to urinary stasis, poor drainage, and associated renal dysplasia or obstruction 1 2 4 5 14.

Voiding and Ejaculatory Symptoms

• In males, ectopic ureters that open into the seminal vesicle or other genital organs can cause voiding difficulties, ejaculatory pain, or perineal discomfort 4 8.
• Epididymitis may also be a presenting symptom in boys or men.

Pelvic/Abdominal Mass and Pain

• Especially in infants and young children, a palpable mass may be found, sometimes due to severe hydronephrosis or a massively dilated, dysplastic kidney 1 6 8.
• Lumbar or abdominal pain can also be a sign and is often due to obstruction or infection 4 6 14.

Additional Clues

• Delayed diagnosis is common if the kidney is nonfunctioning and mistaken for a solitary kidney 2.
• Symptoms may evolve over time or after surgical procedures or trauma 3.

Types of Ectopic Ureter

Ectopic ureters are not all the same. Their classification is based on anatomical considerations: whether they are associated with a single system or a duplex system, and where they insert.

Type	Description	Typical Insertion Sites	Source(s)
Single-System	Ureter drains a single (entire) kidney	Bladder neck, urethra, genital tract	2 4 7 8 10
Duplex-System	Ureter drains upper pole of a duplex kidney	Urethra, vagina (females); seminal vesicle, vas deferens (males)	2 5 6 14 16
Intravesical Ureterocele	Ureterocele entirely within bladder	Bladder	6 13 14
Ectopic Ureterocele	Ureterocele extends to bladder neck/urethra	Bladder neck, urethra	13 14 17

Table 2: Types of Ectopic Ureter

Breaking Down the Types

Single-System vs. Duplex-System Ectopic Ureter

• Single-System Ectopic Ureter (SSEU):

  • Involves a ureter draining an entire (often dysplastic) kidney.
  • More common in males; frequently associated with severe renal dysplasia or nonfunctioning kidneys 2 4 7 8 10.
  • Symptoms may include incontinence, infection, or mass effect.

• Duplex-System Ectopic Ureter:

  • Most common type overall.
  • The upper pole ureter of a duplex kidney opens ectopically; the lower pole drains normally.
  • Often present with ureterocele, hydronephrosis, or reflux 2 5 6 14 16.

Ureterocele Types

• Intravesical Ureterocele:

  • Cystic dilatation contained within the bladder.
  • Typically less severe and easier to treat 6 13 14.

• Ectopic Ureterocele:

  • Extends into the bladder neck or urethra.
  • More common in duplex systems.
  • Associated with a higher rate of infection, obstruction, and need for surgical intervention 13 14 17.

Anatomic Insertion Sites

• Females:
  • Ureter can insert into the bladder neck, urethra, vagina, or vestibule.
• Males:
  • Insertion can be into the prostatic urethra, seminal vesicle, vas deferens, epididymis.
  • Symptoms may relate more to reproductive tract involvement (pain, epididymitis) 4 8.

Causes of Ectopic Ureter

Ectopic ureter is fundamentally a developmental anomaly, but its underlying causes are rooted in embryology and genetics.

Cause	Mechanism/Explanation	Associated Findings	Source(s)
Embryologic Malformation	Abnormal separation of ureteric bud from mesonephric duct	Ureter duplication, dysplasia	5 10 12
Ureter Duplication	Early branching of ureteric bud	Duplex kidneys, ureterocele	2 5 6 10 12 14
Renal Dysgenesis	Poor kidney development due to abnormal ureteric insertion	Nonfunctioning/dysplastic kidney	4 6 8 9
Genetic/Unknown Factors	Multifactorial, possibly genetic	Variable	5 12

Table 3: Causes of Ectopic Ureter

Delving Into the Causes

Embryological Origins

• The ureteric bud arises from the mesonephric (Wolffian) duct during fetal development.
• If the ureteric bud separates abnormally or migrates incorrectly, it can implant in places other than the bladder trigone, creating an ectopic ureter 5 10 12.

Ureter Duplication

• Premature branching of the ureteric bud can lead to duplication of the ureter.
• The upper pole ureter is more prone to ectopic insertion, leading to duplex systems—this is the most common anatomical scenario for ectopic ureters 2 5 6 10 12 14.

Renal Dysgenesis and Associated Anomalies

• The more remote the ectopic ureteral orifice from its normal position, the more likely the kidney is to be dysplastic, hypoplastic, or even aplastic 4 6 8 9.
• This is especially true for single-system ectopic ureters.

Genetic and Syndromic Associations

• While most cases are sporadic, some are associated with congenital syndromes (e.g., Mayer–Rokitansky–Kuster–Hauser syndrome) 12.
• The precise genetic mechanisms remain unclear, but familial clustering has been described.

Treatment of Ectopic Ureter

Treatment strategies for ectopic ureter are highly individualized, taking into account the patient's anatomy, symptoms, kidney function, and associated anomalies.

Treatment	Indication	Key Considerations	Source(s)
Observation	Asymptomatic, non-obstructive	Regular monitoring	5
Nephroureterectomy	Nonfunctioning/dysplastic kidney	Most common for single-system cases	7 8 15
Ureteral Reimplantation	Functioning kidney/ureter	Preserves renal function	7 13 15
Upper Pole Heminephrectomy	Duplex systems with nonfunctioning upper pole	May require further bladder surgery	14 15 16
Endoscopic Ureterocele Puncture	Intravesical or obstructive ureterocele	Emergency or elective, high recurrence for ectopic	13 14 17
Ureteroureterostomy	Duplex systems, select cases	Preserves lower pole function	16

Table 4: Treatment Approaches

Exploring Treatment Options

Observation

• If the ectopic ureter is asymptomatic and renal function is preserved, regular monitoring may suffice 5.
• However, most cases eventually require intervention due to symptoms or risk to renal function.

Surgical Treatments

Nephroureterectomy

• Removal of the entire nonfunctioning kidney and ureter.
• Most common in single-system ectopic ureter with severe dysplasia 7 8 15.

Ureteral Reimplantation

• For a functioning kidney/ureter, the ureter is reimplanted into the bladder.
• Preserves renal function and resolves incontinence or infection 7 13 15.

Upper Pole Heminephrectomy

• In duplex systems where the upper pole is nonfunctioning.
• The upper pole and its ureter are removed; may require additional intervention if reflux or infection persists 14 15 16.

Endoscopic Ureterocele Puncture

• Minimally invasive; used for obstructive ureteroceles.
• High rate of additional surgery, especially in ectopic ureterocele or duplex systems 13 14 17.

Ureteroureterostomy

• Attaches the ectopic ureter to the normal ureter, allowing normal drainage.
• Useful in select duplex systems; preserves lower pole function 16.

Postoperative Outcomes and Considerations

• Residual symptoms (e.g., wetting) may persist for a time post-surgery but usually resolve 2 13.
• Reoperation is sometimes necessary, especially in duplex or ectopic ureterocele cases 14 17.
• Bladder function is generally preserved, especially with early intervention 7 13.
• Close follow-up is essential due to the risk of recurrent infection, obstruction, or reflux.

Conclusion

Ectopic ureter is a complex congenital anomaly with diverse presentations and a spectrum of management options. Early recognition and individualized treatment are key to preserving renal function and improving quality of life.

Summary of Main Points:

• Ectopic ureter most often presents with incontinence in girls and infections or genital symptoms in boys.
• Classification is based on the number of renal systems (single vs. duplex) and the insertion site.
• It arises from embryological malformations, often associated with other urinary tract anomalies.
• Treatment is tailored to the anatomical and functional scenario, with options ranging from observation to surgical reconstruction or nephroureterectomy.
• Prognosis is excellent with early and appropriate intervention, but ongoing monitoring is crucial due to possible complications.

Understanding ectopic ureter not only helps in managing a rare urological anomaly but also highlights the intricacies of embryonic development and the importance of coordinated multidisciplinary care.