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Diagnostic ultrasound ( PDFDrive )

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1788 PART V Pediatric Sonography

is typically used to clarify whether the dilation is caused by

obstruction.

An obstructed ureter can result from many causes. Ectopic

insertion of a ureter can cause obstruction either with or without

a ureterocele and may result in dilation of the more proximal

collecting system and ureter, typically manifesting with ureteral

duplication. he ureter can also be obstructed by an intraluminal

abnormality, such as a stone, blood clot, or fungus ball. Increased

peristalsis in the ureter proximal to the obstruction may be

detected with real-time sonography. Doppler sonography may

show a diminished or abnormal ureteral jet on the side of

obstruction. 18,40,41 he ureter can also be obstructed anywhere

along its course by extrinsic compression from a mass, such

as a lymphoma or abscess. he exact site of obstruction may

be diicult to visualize by ultrasound because of overlying

bowel gas.

Bladder Outlet Obstruction

Bilateral hydronephrosis is frequently caused by obstruction at

the level of the bladder or bladder outlet. Congenital obstruction

due to posterior urethral valves (PUVs) results in an irregular,

thick-walled bladder. PUV can occasionally be diagnosed by

ultrasound, with demonstration of a dilated posterior urethra.

Transperineal contrast-enhanced ultrasound (CEUS) diagnosis

of PUV has also been described 42,43 (Fig. 52.19). However,

VCUG should be performed for optimal visualization of

the valves.

Patients with spinal dysraphism and hyperrelexive detrusor

muscle activity will have a functional obstruction due to a

neurogenic bladder that produces high bladder pressure during

voiding. hese patients may develop a thick-walled, trabeculated

bladder 44 (Fig. 52.20, Video 52.2, Video 52.3, Video 52.4).

Congenital anomalies of the spine should be sought with radiographs

and spinal ultrasound in neonates, if not obvious

clinically.

A pelvic mass that obstructs the bladder outlet, such as

rhabdomyosarcoma, or less commonly, a ibroepithelial polyp,

can also be associated with bilateral hydronephrosis and

hydroureter. 45

Vesicoureteral Relux

Dilation of the renal collecting system is not always caused by

obstruction, and other abnormalities, such as VUR, should be

considered. In a child with hydronephrosis detected by ultrasound,

the bladder size and contractility and the urethra should be

further evaluated with VCUG. In addition, VUR can be diagnosed

and may be the cause of the UTD.

Contrast-enhanced voiding urosonography is a safe, nonionizing,

radiation-free alternative to the luoroscopic VCUG that

is used by experienced investigators in some centers throughout

the world for the management of VUR. Its sensitivity is comparable

to or greater than that of VCUG or radionuclide cystography

in the diagnosis of VUR. 46 Published studies reporting use of

this technique in the United States to date have been limited. 47,48

Contrast-enhanced voiding urosonography is performed ater

bladder catheterization and intravesical infusion of normal saline

containing the ultrasound contrast agent. Sequential imaging of

the bladder, kidneys, and urethra is performed during bladder

illing and voiding. Ultrasound contrast-speciic settings are used

to image the microbubbles. Digital ultrasound still images as

well as image clips are used for documentation purposes (see

Fig. 52.20). he severity of VUR can be graded using a scale

similar to the International Grading System used for conventional

VCUG. 49 Patients with relux are oten treated endoscopically

with a submucosal, subureteric injection of a bulking agent.

Follow-up sonography of the bladder will depict the implants,

which over time may undergo changes in size and shape as well

as calciication 50,51 (Fig. 52.21). Paltiel and colleagues 52 showed

that nonvisualization of the implant or a multilobed contour is

associated with persistent relux.

Prune-Belly Syndrome

Prune-belly syndrome, also known as Eagle-Barrett syndrome,

is a rare congenital disorder that occurs almost exclusively in

males and is thought to result from urethral obstruction early

in development. Abnormalities include absence or deiciency of

the abdominal musculature; large, hypotonic, dilated and tortuous

ureters; a large bladder; a patent urachus bilateral cryptorchidism;

and a dilated prostatic urethra. here are decreased muscular

ibers throughout the urinary tract and prostate, resulting in

dilation and hypoperistalsis. Renal dysplasia and hydronephrosis

can occur to varying degrees. Associated malformations of the

cardiopulmonary, gastrointestinal, and skeletal systems are

common. 53

Megacystis-Microcolon-Malrotation–

Intestinal Hypoperistalsis Syndrome

Megacystis-microcolon-malrotation–intestinal hypoperistalsis

syndrome (MMIHS), a rare syndrome, is an inherited, autosomal

recessive disorder that falls on the spectrum of chronic intestinal

pseudo-obstructive disorders, which includes chronic adynamic

ileus, hypoganglionosis, Hirschsprung disease, intestinal

neuronal dysplasia, and total colonic aganglionosis. It is more

common in girls than boys. Presentation is usually in the neonatal

period, although the diagnosis can also be suspected on prenatal

sonography. 54 Typical clinical features include abdominal distention,

hypoactive or absent bowel sounds, and an inability to void

spontaneously. A markedly dilated, nonobstructed bladder, a

microcolon, and decreased or absent intestinal peristalsis are

characteristic indings. Other frequent abnormalities include

hydroureteronephrosis, VUR, intestinal malrotation, and a

shortened bowel. Although the exact etiology has not yet been

elucidated, smooth muscle myopathic changes have been demonstrated

at pathology. Marked abnormality of the detrusor

muscle of the bladder results in voiding dysfunction. 55 Most

patients with MMIHS eventually succumb to sepsis, malnutrition,

or multiorgan failure. Recurrent UTIs result from the need for

chronic bladder catheterization. Intestinal function is resistant

to surgical intervention. Children who survive beyond the

neonatal period require long-term total parenteral nutrition

(TPN), which eventually results in TPN-associated chronic liver

disease. Transplantation is currently the only successful management

option for those patients who cannot tolerate TPN. he

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