Ectopiс ureter is a congenital anomaly of the topography of the ureter, consisting in an atypical intravesical or extra–vesical location of its mouth. With extra-bubble ectopia, involuntary leakage of urine is noted against the background of preserved urination; intravesical types of abnormalities are accompanied by the development of pyelonephritis and ureterohydronephrosis. Diagnostics may include ultrasound, cystoscopy and urethroscopy, excretory urography, cystography, ureteropyelography, renal angiography, gynecological examination in women, rectoromanoscopy. With preserved kidney function, ureteroureteroanastomosis or ureteropyeloanastomosis is performed. In case of loss of kidney function, partial or total nephroureterectomy is indicated.
ICD 10
Q62.6 Incorrect location of the ureter
Meaning
With ectopiс ureter, its mouth may open distal to the angle of the triangle of the Lieto or flow into neighboring organs (urethra, vagina, uterus, intestines, seminal vesicles). Pathology in most cases is combined with a complete doubling of the ureter and pelvis, while ectopic, as a rule, is the ureter connecting to the upper pelvis. Less common is the incorrect location of the base of the solitary or main ureter. Ectopia of the mouth is a congenital anomaly of the ureter, which manifests itself already in childhood. In practical urology, this malformation is detected 4 times more often in girls than in boys.
Causes
The development of the urogenital system during embryogenesis occurs between the 3rd and 8th weeks of gestation. Initially, the rudiments of the ureters are formed from mesodermal ducts proximal to the area of their future location. Gradually they migrate downwards to flow into the back wall of the bladder in a typical place. Under the influence of teratogens (somatic diseases of a pregnant woman, occupational hazards, medication, hereditary factors and other adverse effects), the mouths of the ureters are not implanted into the bladder in a timely manner, remaining in the rudiments of the mesonephral duct.
In the future, the vagina, uterus and fallopian tubes are formed from the paramesonephral ducts in girls, so the ureter turns out to open into one of these structures, or derivatives of the urogenital sinus – the bladder is lateral to the triangle of the Lieto, the urethra, the neck of the bladder, the paraurethral region. All variants of ectopia in women are found either outside the urinary system or below the level of the external sphincter of the bladder, which is clinically manifested by drip leakage of urine.
From the mesonephral rudiments, men develop appendages of the testicles, seminal vesicles and the vas deferens, and the ectopic ureter, as a rule, opens into these structures. However, since these anatomical formations are located proximal to the external bladder pulp, ectopiс ureter in men is never accompanied by urinary incontinence. Pathology is often combined with doubling of the kidney and ureter, ureterocele, kidney dystopia, hypoplasia and polycystic kidney, etc. congenital anomalies.
Classification
Ectopiс ureter base can be unilateral or bilateral, intravesical or extravesical.
- Intravesical-ectopic ureter opens in an atypical place of the bladder.
- The extra-bubble–ectopic ureter in women can flow into the vestibule of the vagina, urethra, vagina, cervix or uterus body; in men – into the posterior urethra, seminal vesicles, seminal tubercle, vas deferens, prostate or appendage of the testicle.
Depending on the localization of the ureteral mouth , there are:
- cervical-vesicular
- urethral
- paraurethral (vestibular)
- hymenal
- vaginal
- uterine
- intestinal and other types of ectopia.
The type of pathology determines the features of clinical manifestations, diagnosis and therapeutic tactics for this defect.
Symptoms
A pathognomonic sign of extrapubular ectopiс ureter base in girls and women is a constant uncontrolled drip of urine with a preserved normal act of urination. An increase in involuntary urine discharge is observed during movements, changing body position, and physical activity. In addition, there may be discharge from the genital tract, vulvitis and colpitis, abdominal and lower back pain, inflammation of the skin of the perineum due to constant contact with urine (urinary dermatitis).
In boys and men, ectopia in the prostatic urethra is manifested by recurrent pyelonephritis, dull pain in the lower back and abdomen, pyuria, urgent urges and frequent urination. When the ectopic ureter flows into the appendage of the testicle, an epididymitis clinic may develop. Also, the disease in men is characterized by pain of an indeterminate nature in the perineum and pelvic region, discomfort during ejaculation, constipation, infertility, asymmetry of the scrotum or abdomen, palpable formation in the abdomen or lumbar region. The presence of vesicoureteral reflux causes the gradual development of hydronephrosis.
Diagnostics
Examination in case of suspected ectopiс ureter base includes collection of complaints, examination of the patient, carrying out instrumental sonographic, endoscopic, X-ray diagnostic methods:
- Gynecological complex. Paraurethral ectopia is well accessible to external examination and presents no difficulties in diagnosis. The vaginal location of the mouth in women can be detected by gynecological examination and colposcopy with catheterization of the ectopic ureter. Uterine ectopia is more difficult to diagnose, requiring ultrasound of the pelvic organs, hysteroscopy, and sometimes diagnostic laparoscopy.
- Visualization of the urinary system: ultrasound of the kidneys and bladder, urethroscopy and cystoscopy, intravenous urography, cystography, ascending urethrography. Complex echographic, endoscopic, X–ray examination of the urinary system allows you to detect anatomical anomalies of the kidneys and ureters – dystopia, doubling, ectopia; assess the condition of the calyx-pelvic system. To identify complications and assess the functioning of the kidneys, scintigraphy, renal angiography, CT, MRI are required.
- Laboratory tests. Laboratory data for ectopia of the mouth are characterized by the typical detection of pyelonephritis in the general urine analysis of leukocytes and bacteria. To identify the pathogens that support inflammation, a bacteriological examination of urine and a smear from the urethra is carried out.
In men, a finger examination of the rectum and urethroscopy is mandatory. Rare cases of intestinal location of the base of the ureter can be detected by rectoromanoscopy.
Treatment
The treatment is operative, including two fundamentally different approaches – organ-preserving or organ-removing.
- Organ-sparing interventions. If the functional characteristics of the kidney are preserved, implantation of the ectopic ureter into the bladder – ureterocystoanastomosis can be undertaken. In the case of doubling of the ureters, good results are obtained by the imposition of pelvic and intervertebral anastomoses – ureteropyeloanastomosis and ureteroureteroanastamosis. With these interventions, the additional ureter, which has an ectopia of the mouth, is removed, and the part of the kidney that it drained is preserved.
- Radical operations. With extra-bubble ectopia in men, the kidney quickly loses its ability to function normally, so it is often necessary to resort to organ-removing interventions. In case of a decrease in the function of one half of the kidney, heminephrectomy with ureterectomy is indicated. With complete loss of kidney function, a nephrectomy is performed.
Prognosis and prevention
With the timely start of treatment and the absence of complications, the prognosis is usually favorable. In the early postoperative period, urinary tract infections and long-lasting hematuria may occur. In men after heminephrectomy, in rare cases, there are violations of the blood supply to the kidney, requiring complete removal of the organ. Patients of both sexes are shown the supervision of a urologist, blood pressure monitoring, periodic tests to exclude proteinuria. Preventive measures provide for the exclusion of teratogenic effects.