Megaureter

Stephen Confer, M.D.

Pediatrics Didactic Presentation
July 25, 2006

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.
Megaureters
In one series, MGU comprised 20% of
antenatally diagnosed urologic anomalies,
much higher than in historical series b/c
most were discovered only after they
became symptomatic
If left undetected, many MGUs might never
become symptomatic
An observation that raises serious questions
with regard to treatment
Primary and Secondary
Refluxing Megaureter
Primary refluxing megaureters are associated with
congenital anomalies of the UVJ where a
deficiency of the longitudinal muscle of the
intravesical ureter results in an inadequate
valvular mechanism
Secondary refulxing megaureters are caused by
bladder obstruction and the elevated pressures that
accompany it
Examples include PUV (most common) as well as neurogenic
bladders and non-neurogenic neurogenic bladders
Primary Obstructive
Megaureter

The cause of 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, but histologic disorientation
of muscle, muscular hypoplasia, muscular
hypertrophy, mural fibrosis and excess collagen
deposition have been described


Primary Obstructive
Megaureter

Altered peristalsis prevents the free outflow of
urine
Retrograde regurgitation occurs as urine boluses are
unable to fully traverse the aberrant distal segment
Resulting ureteral dilatation depends on the
amount of urine that is forced to coalesce
proximally because of incomplete passage.
Other rare causes of primary obstructive MGU
include congenital ureteral strictures and ureteral
valves
Secondary Obstructive
Megaureter

Most commonly occurs with neurogenic and non-
neurogenic voiding dysfunction or infravesical
obstructions such as PUV
The ureter struggles with propulsion of urine
when pressure is > 40 cm H
2
O across the UVJ.
Ureteral dilatation, decompensation of the UVJ,
reflux, and renal damage result if pressures
continue unchecked
Dilatation largely resolves once the elevated
intravesical pressures are addressed
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

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 f/u 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
NRS offers objective, reproducible parameters of function
and obstruction

99m
Tc-DTPA and
99m
Tc-Mertiatide (MAG3) are most
commonly used assess function and clearance.
NRS 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
NRS can 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


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
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