Megaureter

Resident physician

Kyrillos M. Iskandar
Pediatric unit – Urology department
Tanta University
 Definition

• Normal ureteral diameter in children is rarely > 5 mm.

• Ureters > 7 mm are considered MGUs.
• The dilated ureter or MGU can be classified into one of four
groups based on the cause of the dilatation:
• (1) refluxing
• (2) obstructed
• (3) both refluxing and obstructed
• (4) both nonrefluxing and nonobstructed.
Demographics And Clinical Findings

• The incidence of obstructed megaureter is 1 per

10,000 population.
• The male-to-female ratio is 1.2-4.8:1.
• The left-to-right ratio is 1.7-4.5:1.
• Obstruction is bilateral in 10%-20% of obstructed
megaureter cases.
 Etiology
• Primary megaureter is caused by a structural
alteration in the muscular layers of the distal
ureter.
• diminished or absent longitudinal muscle fibers
• hypertrophied or hyperplastic circular muscle fibers
• increased connective-tissue deposition.
These changes are defined pathologically
 Pathophysiology

• Partial obstruction in the distal segment leads to progressive

dilatation to hydronephrosis when the ureter no longer
accommodates resistance to urinary drainage.
• Complete obstructions are rare and are associated with a
nonfunctioning renal unit at diagnosis.
• Primary obstructed megaureters enter the bladder in a normal
location on the trigone, with the ureteral orifice appearing
unaffected. This should not be confused with ectopic
megaureters that end in an abnormal location.
 Classification of megaureter

Refluxing Obstructed Nonrefluxing & nonobstructed

Primary Secondary Primary Secondary Primary Secondary

Primary reflux Neuropathic bladder Primary obstructed Neuropathic bladder Nonrefluxing &
Megacystic- megaureter Hinman syndrome megaureter Hinman syndrome nonobstructed
syndrome Posterior urethral valve Ureteral valve Posterior urethral valve
Ectopic ureter Bladder diverticulum Ectopic ureter Bladder diverticulum
Prune- belly syndrome Post opertave Ectopic uretocele Post opertave
 Refluxing Megaureter
• Primary refluxing
with congenital anomalies of the UVJ where a deficiency of the longitudinal
muscle of the intravesical ureter results in an inadequate valvular mechanism.

• Secondary refluxing
by bladder obstruction and the elevated pressures
 PUV (most common)
 neurogenic bladders and non-neurogenic neurogenic bladders
 Prune Belly Syndrome.
Primary Refluxing Megaureter
Prune Belly Syndrome
Obstructive Megaureter
• The primary obstructive MGU typically is an
aperistaltic juxtavesical segment 3 to 4 cm long that is
unable to propagate urine at acceptable rates of flow.

• True stenosis is rare.

• Other rare causes of primary obstructive MGU include

congenital ureteral strictures and ureteral valves.
Primary Obstructive Megaureter
 Presentation
• Children with an obstructed megaureter may present with vague
abdominal pain, hematuria, UTI, fever of unknown origin, or an
abdominal mass.
• The 1ry presentation, antenatal hydronephrosis, is diagnosed with
prenatal ultrasonography.
• Mostly is non symptomatic, discovered only with appearance of
symptoms or with associated abnormalities that may include the
following:
• Contralateral renal agenesis (9%-15%)
• Microcystitis in bilateral lesions
• Contralateral ureteroceles
• Renal dysplasia
• Ureteral duplication
 Work up – Lap. studies
• Creatinine and BUN to assess gross renal function.
• Levels of sodium, potassium, chloride, or bicarbonate.
• Urinalysis is recommended for infection, proteinuria, high
pH level, and low specific gravity that may reflect underlying
infection or renal dysplasia or damage.

• In neonates, wait 3-5 days to evaluate the baby’s kidney

function rather than the mother's kidney function.
• A creatinine level of 1 mg/dL or greater at age 1 year is
prognostic for subsequent renal failure.
 Work up –
• Ultrasonography:
imaging studies
- prenatal imaging reveals both hydroureter and
hydronephrosis.
- Repeat ultrasonography for hydronephrosis at age of 3-5
days
- Hydroureter and unilateral lesions may be evaluated
electively within the first 2 months of life.

the classic spindle configuration 

 Work up – imaging studies
• Ultrasonography:
• Postnatal imaging is used to define the degree of hydronephrosis
• Grade I - Splitting of the renal sinus more than 10 mm
• Grade II - Splitting of the renal sinus more than 10 mm in an
extrarenal or intrarenal pelvis not extending to the calyces
• Grade III - Dilatation extending into the calyces without cortical
thinning
• Grade IV - Dilatation extending into the calyces with cortical thinning

Postnatal imaging further defines the following anatomy:

•presence of collecting system duplication
•Presence or absence of ureterocele
•Bladder configuration, size, and thickening
 Work up – imaging studies

• Voiding cystourethrography (VCUG) is performed to

assess the presence of vesicoureteral reflux.

• In boys with bilateral megaureter, posterior urethral

valves must be excluded.
 Work up – imaging studies

• intravenous urography (IVU) can be used if renal function

is good
• Radionuclide renal scanning
• Magnetic resonance imaging
the capacity for functional recovery prior to surgery and
detailed anatomy
 Work up – imaging studies

N.B.
• Megaureters detected in neonates and infants may
require drainage for infections that do not respond to
antibiotics alone.
• Additionally, the massively dilated ureter may be
decompressed with nephrostomy drainage, which
often allows a decrease in ureteral size and greatly
reduces ureteral bulk during both tailoring and
reimplantation.
 Work up – imaging studies
N.B.
• Megaureters detected in neonates and infants may
require drainage for infections that do not respond to
antibiotics alone.
• Additionally, the massively dilated ureter may be
decompressed with nephrostomy drainage, which
often allows a decrease in ureteral size and greatly
reduces ureteral bulk during both tailoring and
reimplantation.
•  Antegrade pyelogram
• Conservative observation

• Depends on degree of hydronephrosis (Grades 1 to 3 

12, 23 and 34 months)
• Ureteral diameter
• Laterality
• Gender
• Surgical Options
• The ultimate goals
to relieve obstruction -
to return near-normal function to the collecting system -
to create a nonrefluxing vesicoureteral reimplantation -
 .to preserve renal development without long-term complications -

• Reconstruction vs. Removal

• Depends on initial function, bilaterality, reflux, drainage curve and
symptoms
• Surgical Options
• Intravesical
• Extravesical
• Laparoscopic
• Patients with voiding dysfunction or significant
reflux may benefit from an intravesical ureteral
reimplantation.

• Patients without voiding problems or reflux do

equally well from an extravesical approach.
Surgical Options
• Folding vs. Plication vs. Tapering

94.6% success 74 megaureters

Stent for 4-5 days

Ehrlich RM: The ureteral folding technique for megaureter surgery. J Urol
134:668, 1985
Surgical Options
• Folding vs. Plication vs. Tapering

Reduces lumen 50-60%

Severely tortuous ureter

Starr A: Ureteral plication. A new concept in ureteral tailoring for megaureter.

Invest Urol 17:153, 1979
Surgical Options
• Folding vs. Plication vs. Tapering

Lower versus total ureteral

reconstruction
41% re-operation

Hendren WH: Complications of megaureter repair in children. J Urol 113:238,

1975
Obstructive Megaureter

• The secondary Most commonly occurs with

neurogenic and non-neurogenic voiding
dysfunction or infravesical obstructions such as
PUV
Secondary Obstructive Megaureter

• Sometimes, the ureter remains dilated due to altered

compliance or a damaged peristaltic mechanisms.
• Transmural scarring from chronic infection is seen in some cases.
• Obstruction is not truly present but elevated intravesical pressures
are projected proximally as a noncompliant column
• Other obstructive causes of ureteral dilatation include
ureteroceles, ureteral ectopia, bladder diverticula,
periureteral postreimplantation fibrosis, neurogenic bladder,
and external compression by retroperitoneal tumors,
masses, or aberrant vessels
Primary Nonobstructive,
Nonrefluxing Megaureter
Once VUR, obstruction, and secondary causes of •
dilatation have been ruled out diagnosis of primary
nonrefluxing, nonobstructive MGU
Most newborn MGUs fall in this category •
Possible causes: increased fetal UOP, persistent •
fetal folds, delayed ureteral patency, immature
peristalsis, hyperreflexic bladder of infancy,
transient urethral obstruction
Primary Nonobstructive,
Nonrefluxing Megaureter

The newborn ureter is a more compliant conduit than that •

of the adult
The kidneys of newborns are probably better buffered •
from the pressures of any partial or transient obstructions
that might occur early in development than are kidneys
obstructed at more proximal levels (UPJ) or at a later age
Secondary Nonobstructive,
Nonrefluxing Megaureter
More common than originally thought, and often •
have an identifiable cause
Can result from acute UTI with bacterial •
endotoxins that inhibit peristalsis
Resolution with appropriate antibiotic therapy •
Nephropathies and other conditions lead to •
increased UOP that overwhelm max peristalsis
which leads to progressive dilatation
Secondary Nonobstructive,
Nonrefluxing Megaureter

These include lithium toxicity, diabetes insipidus or mellitus, •

sickle cell nephropathy, and psychogenic polydipsia
The most extreme examples of nonobstructed ureteral •
dilatations occur with the prune-belly syndrome
 Prevalence

Studies showed that clinically significant MGU accounted for 8% of •

children found to have hydronephrosis on imaging studies, preceded
.by UPJ obstruction 22%, PUV 19% andectopic ureterocele 14%
MGU are reported to occur in app 23% of neonates noted to have •
.antenatal hydroureteronephrosis
Ranking 2nd in D/D of neonates with hydronephrosis after UPJ •
.obstruction
.More often in boys and on left side •
 Evaluation
Ultrasound is the initial study obtained in any child with a •
suspected urinary abnormality
Usually distinguishes MGU from UPJ as the most common •
cause of hydronephrosis
Provides useful anatomic detail of the renal parenchyma, •
collecting system, and bladder
Baseline standard for the degree of hydroureteronephrosis •
for serial future studies
 Evaluation

The presence of ureteral dilatation •

VCUG to rule out reflux and assess the quality of the bladder •
and urethra
Neurogenic dysfunction or outlet obstruction are common •
causes of secondary MGU
Need to assess renal function •
 Evaluation
Renal scans offers objective, reproducible parameters of •
function and obstruction
99m
Tc-DTPA and 99m Tc-Mertiatide (MAG3) are most •
.commonly used assess function and clearance
Renal scan shortcomings: standardized tracer dosing, •
timing in diuretic dosing, and patient hydration ensure
valid comparison of results
should defer the study for 3 months for glomerular •
maturation
Scans that evaluate drainage (half-life) alone routinely •
yield values indicative of obstruction because of the
dilatation of the collecting system
 Evaluation
Renal scan estimate GFR and absolute renal function by •
measuring the uptake of radionuclide (DTPA) early after its
systemic administration
This may indicate the impact of megaureter on renal •
parenchymal level, rather than within the collecting system,
where slow rates of washout are to be expected because
of dilatation
Magnetic resonance urography has the capability of providing •
.greater anatomic abnormalities
Percutaneous perfusion studies (Whitaker perfusion test) can also •
.be used
 Recommendations: Primary Refluxing
Megaureter

Routinely recommending surgery in newborns and infants •

with grades IV-V reflux is not appropriate
Medical management is appropriate during infancy and is •
continued if a trend to resolution is noted
Surgery remains the recommendation for persistent high- •
grade reflux in older children and adults
In the rare infant for whom medical management has •
failed but who is considered too small for reconstructive
surgery, distal ureterostomy for unilateral reflux or
vesicostomy for bilateral disease provides an ideal
temporizing solution
Recommendations: Secondary
Refluxing or Obstructive Megaureter
Management of secondary MGUs is directed at their cause •
Reflux and dilatation improve with the ablation of PUV or •
medical management of neurogenic bladder
MGUs from prune-belly syndrome, diabetes insipidus, or •
infection, require no more than observation alone
Some degree of nonobstructed hydroureteronephrosis •
usually persists, even after primary or secondary causes
have been corrected
Re-evaluation is often necessary •
Recommendations: Primary
Nonobstructive, Nonrefluxing Megaureter

The complication rate of surgery is higher in infants •

repeat surgeries were required for 12% infants operated on before •
8 mo in one series
As long as renal function is stable and UTIs are not a •
problem, expectant management is preferred
Antibiotic suppression with close radiologic •
surveillance is appropriate in most cases
U/A and RUS every 3 to 6 months during the first year •
Recommendations: Primary
Nonobstructive, Nonrefluxing
Megaureter
Severe hydroureteronephrosis that shows no signs •
of improvement or the clinical status worsens,
correction is undertaken when it is technically
feasible, usually between the ages of 1 and 2
.years
For the occasional newborn who presents with •
massive ureteral dilatation or poor renal function
(which is rare with MGUs) or develops recurrent
infections, distal ureterostomy provides an effective
means for poor drainage until the child is old
.enough to undergo reimplantation
Surgical Options
Ureteral tailoring is usually necessary to achieve •
the proper length-to-diameter ratio
Narrowing of the ureter may enable the walls to •
coapt, leading to more effective peristalsis
Revising the distal segment intended for •
reimplantation is all that is usually required
The proximal segments regain tone once they are •
unobstructed. Kinking is usually nonobstructive
.and will resolve
Extended stent drainage after tapering •
decompresses the system
Leads to peristaltic recovery •
Surgical Options
Plication or infolding is useful for the moderately •
.dilated ureter
Ureteral vascularity is preserved, and the revision •
can be taken down and redone if vascular
compromise is suspected
Bulk is a problem with the extremely large ureter •
Excisional tapering is preferred for the more •
severely dilatated or thickened ureter
Plication of ureters greater than 1.75 cm in diameter •
experienced more complications in one series
 Surgical Options

Remodeled MGUs have been generally •

reimplanted with standard cross-trigonal or
Leadbetter-type techniques
Extravesical repairs can also be successfully done •
The success with reimplantation of remodeled •
MGUs is 90-95% regardless of technique
Compares to 95-99% of non-megaureter reimplants •
 Tapering •
Starr Plication •
Kalicinski Plication •

Techniques Of Ureter
Remodeling
Tapering
Folding (Kalicinski)
Tailoring
 Politano-Leadbetter Technique •
Cohen Cross-Trigonal Technique •
Glenn-Anderson Technique •
Gil-Vernet Technique •

Reimplantation
Techniques Of Ureter
 Results and Complications
The reimplantation of MGUs has the same •
complications (i.e., persistent reflux and
obstruction) as that of nondilated ureters, but at
increased rates
Complications can occur regardless of whether •
excisional tapering or a folding technique is used
Better results with obstructive MGU and higher •
rates of unresolved reflux after tailoring of
refluxing variants
Higher incidence of bladder dysfunction associated with •
the latter and more dramatic abnormalities of their
.musculature
Results and Complications
Increased collagen deposition in refluxing MGUs •
and altered smooth muscle ratios
In contrast, obstructive MGUs were not found to •
be statistically different from controls
Increased levels of type III collagen in refluxing •
MGUs
Leads to an intrinsically stiffer ureter that lessens the •
surgical success in reimplantation
Rarely, reflux persists despite adequate ureteral •
.tunnels in both tapered and normal-sized ureters
Leads to intrinsic ureteral dysfunction caused by •
transmural scarring