Ectopic ureter, Ureterocele
Dept Of Surgery
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Bilateral Renal Agenesis
? Bilateral Renal Agenesis was first recognized in 1671 by
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Wolfstrigel? Can occur secondary to a defect of the wolffian duct, ureteric
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bud, or metanephric blastema? Bilateral agenesis occurs in 1 of every 4000 births
? Male predominance
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? 40% of the affected infants are stillborn
? Children who are born alive do not survive beyond 48 hours
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because of respiratory distress associated with pulmonary
hypoplasia
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? The adrenal glands are usually normally positioned? Characteristic Potter facies and presence of oligohydramnios are
pathognomonic
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? Complete ureteral atresia is observed in slightly more than 50%
of affected individuals
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Potter's facial appearanceDiagnosis
? The characteristic Potter facies and the presence of
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oligohydramnios are pathognomonic and should alert forthis severe urinary malformation.
? Amnion nodosum--smal white, keratinized nodules found
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on the surface of the amniotic sac--may also suggest this
anomaly
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? Anuria after the first 24 hours without distention of thebladder should suggest renal agenesis
Diagnosis
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? BRA has been detected in higher proportion in cryptophthalmos
or Frazer's syndrome, Klinefelter's syndrome , Kallmann's
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syndrome, esophageal atresia.? Renal ultrasonography confirm the presence or absence of
urine within these structures.
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? Absence of uptake of the radionuclide in the renal fossa above
background activity confirms the diagnosis of BRA.
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? Umbilical artery catheterization and an aortogram defines theabsence of renal arteries and kidneys.
Unilateral Renal Agenesis
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? There are no tell tale signs (as with BRA) that suggest anabsent kidney .
? Diagnosis not suspected unless careful examination of the
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external and internal genitalia uncovers an abnormality
that is associated with renal agenesis or an imaging study is
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done.? Unilateral agenesis occurs once in 1100 births
? Males predominate in a ratio of 1.8:1
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? More frequent on the left side
? Ipsilateral ureter is completely absent in about half
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of the patients? Structures derived from the m?llerian or wolffian
duct are most often anomalous
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? Anomalies of other organ systems involve the
cardiovascular (30%), gastrointestinal (25%), and
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musculoskeletal (14%) systemsUnilateral renal agenesis to be associated with other
urologic abnormalities in 48% of patients
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? Primary vesicoureteral reflux (28%)? Obstructive megaureter (11%)
? Ureteropelvic junction obstruction (3%)
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Diagnosis
? No specific symptoms heralding an absent kidney
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? The diagnosis should be suspected during a physicalexamination when the vas deferens or body and tail of the
epididymis is missing or hypoplastic vagina is associated with a
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unicornuate or bicornuate uterus
? Radionuclide imaging
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? No clear-cut evidence that patients with a solitary kidneyhave an increased susceptibility to other diseases
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Horseshoe kidneys? Most common of all renal fusion anomalies
? Occurs in 0.25% of the population
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? fusion occurs before the kidneys have rotated on
their long axis
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The lower poles of the two kidneys touch and fuse as they cross the iliac arteries? In 95% of patients, the kidneys join at the lower pole; in a
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small number, an isthmus connects both upper poles instead? Calyces normal in number, are atypical in orientation
? Ureter may insert high on the renal pelvis and lie laterally
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? Blood supply to the horseshoe kidney can be quite variable
Arteriogram showing a multiplicity of arteries supplying kidney arising from
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aorta and common iliac arteries
? UPJ obstruction, causing hydronephrosis, occurs in
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one third of individuals? 60% patients remain asymptomatic for aprox. 10
years
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Associated Anomalies
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Diagnosis
? Excretory urogram
Prognosis
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? 13% have persistent urinary infection or pain
? 17% develop recurrent calculi
? Renal carcinoma has been reported within a horseshoe
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kidney in 123 patients? Incidence of Wilms' tumor in horseshoe kidneys is more
than twice
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Congenital cysts
? Kidney is one of the MC
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sites in body for cysts? Arise from the nephrons
and collecting ducts after
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they have formed
Cystic Diseases of the Kidney
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? Multicystic refers to a dysplastic entity
? Polycystic most inherited, all without dysplasia and all
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with nephrons throughout the kidney? Many of the polycystic kidney disease entities progress
to renal failure
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`Snowstorm' appearance of infantile polycystic
disease
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Ectopic Kidney
? Kidney not located in usual position
? 1 in 1,000 births, but only about one in 10 of these
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are ever diagnosed; up to 10% bilateral
Most common:
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? Horseshoe Kidney? Unilateral renal agenesis
? Pelvic kidney
(Left kidney more likely to be abnormal)
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Ectopic Kidney
? Function is general y normal initial y
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? Abnormal position leads to obstruction in 50% ofectopic kidneys
? Increased risk UTI, kidney stones, VUR
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? Frequently associated with abnormalities of otherorgan systems (uterine, cardiac, skeletal)
Ectopic Kidney Locations
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Ectopic Kidney
(simple renal ectopia)
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Mega ureter
? Ureters wider than 7 to 8 mm
? Normal ureteral diameter is rarely greater than 5 mm
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? Primary MGU is 2-4 times more common in boys than girls? Slight predilection (1.6 to 4.5 times) for the left side
? Bilateral in approximately 25% of patients
? In 10% to 15% of children contralateral kidney may be absent or
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dysplasticThree major classifications of megaureter based on primary and secondary causes
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Pathophysiology? Distal end of the ureter, as it becomes intramural and
subsequently submucosal, rearranges the muscular layers in its
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wall.
? All layers become longitudinally oriented
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? Ureteral adventitia fuses to the bladder trigone by attachment toWaldeyer's sheath
? Sympathetic and parasympathetic innervation to the distal ureter
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and UVJ area is believed to modulate primarily ureteral peristalsis
Diagnosis
Ultrasound
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? Distinguishes MGU from UPJ obstruction based on thepresence or absence of a dilated ureter
VCUG
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? to rule out refluxRenal scintigraphy
? Provides objective, reproducible parameters of function
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and obstructionWhitaker's perfusion test & ureteral opening pressure
? To evaluate obstruction, but their invasiveness and
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requirement for anaesthesia are drawbacks in childrenMagnetic resonance urography
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Magnetic resonance urogram showing obstruction at the right ureterovesicaljunction
Management
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Primary Refluxing Megaureter
? Medical management is often the initial approach
? Surgery
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? Endoscopic subureteric injection, is recommended for
persistent high-grade reflux in older children
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? Reconstructive surgery of a dilated ureter? distal ureterostomy for unilateral reflux
? vesicostomy for bilateral disease
? Secondary Refluxing or Obstructive Megaureter
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? Management of secondary MGUs is initially directed at their
root cause
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? Primary "Dilated" Nonrefluxing Megaureter:Nonobstructive versus Obstructive
? Expectant management is preferred
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? Antibiotic suppression & radiologic surveillance is appropriatein most cases
? Surgical correction
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? Surgical Options
? Plication or infolding for moderately dilated ureter
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ComplicationsPersistent reflux and obstruction
Postoperative VUR
Ectopic ureter
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? Ureter whose orifice terminates anywhere other
than the normal trigonal position
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? Lateral ectopia : an orifice more cranial and lateralthan normal
? Caudal ectopia : orifice is more medial and distal
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than the normal position
? 80% are associated with a duplicated collecting system
? Females :
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? More than 80% are duplicated
? Urethra and vestibule are the most common sites
? Males:
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? most ectopic ureters drain single systems
? posterior urethra is the most common site
? Drainage into the genital tract involves the seminal vesicle
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three times more often than the ejaculatory duct and vas
deferens combined
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Ureterocele(outpouching of ureter as it enters bladder)