These points are important to consider when

characterized by the congenital absence of

surgical correction is planned. The abnormal

abdominal

musculature,

which generally

ureteral segment must be excised and confirmed

involves the lower and medial parts of the

by pathology to insure that normal ureter is

abdominal wall. The result is a protuberant,

implanted.

thin walled and creased abdomen which

gives rise to the classical name.

Megaureter-Megacystis Syndrome

The condition is seen almost exclusively in

males and is associated with serious genito-

In contrast to the pipestem ureter, the Megaureter-

urinary anomalies. These anomalies include a

Megacystis syndrome is characterized by a large

large capacity bladder which may be associ-

capacity bladder with dilated and refluxing ure-

ated with diverticula or a patent urachus. The

ters in the absence of vesical neck or urethral

ureters are almost always dilated and tortuous

obstruction. This condition is generally diag-

with associated reflux and poor contractions.

nosed during childhood, because of the associ-

The kidneys are either hydronephrotic or dis-

ated symptoms, has an equal sex incidence, and

plastic and the urethra may be obstructed.

there is no known cause. The ureter in this syn-

Testicular male-descent is common, penile

drome is not only dilated, but also lengthened

curvature may occur, and most patients are

and tortuous – and although peristalsis is active,

sterile.

the propulsive powers are very limited.

Associated abnormalities include intestinal

A second significant difference is that the ure-

malrotation, congenital cardiac abnormalities,

teral orifices are widely dilated and permit easy

and talipes. As in Megaurer-Megacystis syn-

reflux, whereas the orifices in the congenital

drome the treatment is dependent on the

megaureter are generally normal in appearance.

degree of renal deterioration and extent of

Thirdly, in this syndrome the bladder is charac-

urological

involvement

as demonstrated

teristically dilated – but without trabeculation

by extensive endoscopic and radiographic

or outlet obstruction.

evaluation.

As in congenital megaureter, there appears to

be no histological abnormality in the number

and distribution of the ganglion cells. The typi-

Vascular Ureteral Obstructions

cal clinical history is that of a child which pres-

ents with a history of recurrent urinary tract

The final topic in regard to congenital ure-

infections, a palpable bladder on physical exam-

ination, and evidence of bone changes or mani-

teral obstructions deals with vascular obstruc-

festations of renal failure if the condition has

tion of the ureter. These may occur at any

been present for some time. The diagnosis is

level, but the most common cause of such

made by urography, voiding cystourethrogra-

obstruction by far is the presence of a residual

phy, and cystoscopy.

obstructing lower pole renal vessel at the ure-

In the differential diagnosis we have to

teropelvic junction (UPJ) with secondary

consider:

hydronephrosis.

1. Nephrogenic diabetes insipidus.

Although the presence of a retroiliac ureter is

rare, it can occur and when present is seen at the

2. Neurogenic bladder (which is rare in children

L-5 level. The retrocaval ureter is more common,

and seldom has a capacity of over 500 ml).

and both of these are the result of an anomaly of

3. Urethral valves.

the vena cava rather than the ureter. These

4. Prune belly syndrome.

anomalies are thought to be due to persistence

of the subcardinal vein as the infra-renal segment

All these possible causes must be excluded and

of the vena cava rather than the supra cardinal

surgical correction usually entails tapering of

vein (see Fig. 1.11).

the ureters prior to re-implantation.

In this anomalous condition, the ureter

Prune Belly Syndrome

abruptly turns medially from a normal position

in its upper third and passes behind the vena

A third type of congenital ureteral dilation

cava in the region of the third and fourth lumbar

vertebra. From there it returns to its normal

is seen in the prune belly syndrome. It is

position lateral to the vena cava after making a

Normal

Abnormal

1.

Resection of the ureter with re-anastomosis.

2.

Resection of the ureter with excision of the

postcaval section – which may be stenotic –

Figure 1.12. X-ray of retrograde pyelogram showing retrocaval

and re-anastomosis.

ureter.