–Most of these cases are diagnosed using ultrasound.

–It may be asymptomatic or present with hematuria, recurrent urinary tract infection or abdominal pain.

–Symptomatic patients are treated with ureteral reimplantation with tapering of the dilated ureter.

Congenital Abnormalities of the Ureter

•Ureteral atresia

•Duplication of the ureter

•Ureterocele

•Ectopic ureteral orifice

•Obstruction of the ureteropelvic junction

•Obstructed mega-ureter

1.5Abnormalities of the Bladder

•Bladder Exstrophy (Figs. 1.45, 1.46, and 1.47)

–This is one of the most severe urological anomalies.

–It is characterized by absence of the anterior abdominal wall and the wall of the wall of the urinary bladder is open to the outside.

–It is associated with other abnormalities, especially epispadias.

–The pubic bones are widely separated.

–It requires surgical reconstruction.

•Cloacal exstrophy (Figs. 1.48, 1.49, 1.50, and 1.51):

Figs. 1.32, 1.33, 1.34, and 1.35 Intravenous urography and CT-urography showing left and right hydronephrosis. Note the thickness of the renal parenchyma in the CT-urography indicative of sever UPJ obstruction. The function of the kidney in these patients can be assessed

with an isoptoe renogram. A split renal function less than 40 % indicate a severe degree of obstruction. This as well as the size of the renal pelvis will determine the urgency of surgical intervention

Figs. 1.40 and 1.41 Intravenous urography showing right megaureter and intraoperative photograph showing reimplantation of a primary megaureter. Note the small

caliber of the distal part of the ureter followed by proximal dilatation of the ureter

–Cloacal exstrophy is a severe birth defect where the urinary bladder and pat of the intestines are exposed to the outside.

–Cloacal exstrophy is a major birth defect representing the severe end of the spectrum of the exstrophy-epispadias complex.

–It is characterized by the followings:

•Omphalocele

•Bladder extrophy (two exstrophied hemibladders separated by a foreshortened hindgut or cecum, often blind-end- ing resulting in an imperforate anus).

•Anterior lower abdominal wall defect

•Widened and separated pubic bones

•Splitting of both male (penis) and female (clitoris) external genitalia

•Anorectal agenesis or anteriorly placed anus

•Spinal defects

–Cloacal exstrophy is an extremely rare birth defect, present in 1/200,000 and 1/400,000 live births.

–The exact pathogenesis of cloacal exstrophy is not known.

•It is thought that it results from an abnormality during early embryologic development associated with rupture of the cloacal membrane before fusion with the urorectal septum.

•Genetic and environmental factors may play a role in the etiology.

–Prenatal diagnosis of cloacal exstrophy is possible from ultrasound findings:

•Non-visualization of the bladder

•Anterior abdominal wall defect

•Omphalocele

•Myelomeningocele

–Omphalocele is found in 88–100 % of patients.

–Gastrointestinal malrotation/duplication and short bowel syndrome are present in 46%, with absorptive dysfunction in some cases.

–The symphysis pubis is widely separated and the pelvis is often asymmetrically shaped.

–Duplication of the vagina and uterus, as well as vaginal agenesis, has also been reported.

Figs. 1.42, 1.43, and 1.44 Abdominal ultrasound showing a right megaureter and a micturating cystourethrogram showing no reflux. An intraoperative photograph

showing reimplantation of the right megaureter. Note the narrow distal ureteric segment and a more dilated proximal ureter

–Various urological malformations may also be present including:

•Ureteropelvic junction obstruction

•Ectopic pelvic kidney

•Horseshoe kidney

•Renal hypoor agenesis

•Megaureter

•Ureteral ectopy

•Ureterocele

–Spinal abnormalities ranging from hemivertebra to myelomeningocele occur in most patients and may be accompanied by skeletal and limb anomalies (clubfoot deformities, absence of feet, tibial/fibular deformities, and hip dislocation).

–The diagnosis of cloacal exstrophy is clear clinically and the management of these patients require multidisciplinary team approach.

•Persistent urachus (Figs. 1.52, 1.53, 1.54, 1.55, 1.56, 1.57, 1.58, 1.59, 1.60, and 1.61):

–The lumen of the allantois, which connects the bladder and the anterior abdominal wall closes normally.

–Persistent urachus results from failure of this connection to become obliterated.

–It may appear as a draining umbilical sinus and can become infected.

–It may appear as fistula connecting the urinary bladder to the umbilicus and the presence of outflow obstruction, urine will be seen coming through the umbilicus.

•Contracture of the bladder neck:

–This is a common cause of reflux, bladder diverticula and irritable bladder.

•Bladder diverticulum (Figs. 1.62, 1.63, 1.64, 1.65, 1.66, and 1.67):

Figs. 1.45, 1.46, and 1.47 Clinical photographs showing bladder exstrophy in a male and two females. Note the associated epispadias in the first one. Note also

the granular appearance of the bladder mucosa in the second one and also the associated prolapsed ovary in the third one

–A bladder diverticulum is an outpouching in the bladder wall. The bladder mucosa herniates through the muscular wall of the urinary bladder.

–It can be either congenital or acquired.

–Acquired bladder diverticula:

•These are often due to bladder outlet obstruction from an enlarged prostate, urethral stricture or neurologic disease.

•Acquired diverticula are most typically seen in elderly men and often associated with benign prostatic hyperplasia (BPH).

•Acquired diverticula are the result of obstruction, infections, or iatrogenic causes.

•The common causes include:

– Posterior urethral valves

–Anterior urethral valves

–Urethral strictures

–Neuropathic bladder

–External sphincter dyssynergy

–Herniation of the bladder mucosa through the ureteral hiatus after ureteral reimplantation is an iatrogenic cause of bladder diverticulum.

•They tend to be multiple and occur in trabeculated bladders.

•Many diverticula that are related to obstruction spontaneously resolve after relief or correction of the obstruction.

•In some cases, the diverticula that occur in response to obstruction serve a beneficial function by acting as pressure pop-off mechanism, protecting the kidney and ureters from high pressures.

Figs. 1.48, 1.49, 1.50, and 1.51 Clinical photographs showing cloacal exstrophy. Note the associated omphalocele in the first two and absence or very small omphalo-

cele in the second two pictures. Note also the associated club feet in the fourth picture

Figs. 1.54 and 1.55 Diagrammatic representation of patent urachus and a clinical photograph of a patient with patent urachus. Note the patent channel connecting the urinary bladder with the anterior abdominal wall at the

site of the umbilicus. Note also the feeding tube which was passed from the urethra and passed all the way to the umbilicus via a patent urachus

Figs. 1.56 and 1.57 Diagrammatic representation of an umbilical sinus where the distal part of the urachus remain patent and the rest becomes obliterated. The second dia-

gram represent an obliterated distal part of the urachus while the part attached to the bladder remain patent as a bladder diverticulum

–Congenital bladder diverticula:

•These are usually diagnosed in childhood or on prenatal ultrasound.

•Congenital bladder diverticula have a wide clinical spectrum and could lead to severe kidney damage. Urinary tract

infection and urinary retention are the most frequent presentation forms.

•Some children with diverticula have voiding dysfunction on urodynamic testing. Whether the voiding dysfunction leads to the formation of diverticula

Figs. 1.58 and 1.59 Clinical photographs showing an umbilical sinus. Note the opening in the umbilicus which is discharging. Note the probe which is passed into the

sinus for a small distance. Intraoperatively, this was found to be a urachal sinus with the distal part obliterated and connected to the bladder

Fig. 1.61 A diagrammatic representation of a patent urachus with prolapse of the mucosa

VUR

BLADDER

DIVERTICULUM

POSTERIOR

URETHRAL VALVE

Fig. 1.62 A micturating cystourethrogram showing posterior urethral valve. Note the associated VUR and bladder diverticulum

–With growth, the pelvis becomes more developed, and the bladder assumes a more pelvic position.

–Bladder ears are lateral protrusions of the bladder wall through the internal inguinal ring and into the inguinal canal.

–Bladder ears are rarely seen in adults.

–Bladder ears are diagnosed using:

•Voiding cystourethrography (VCUG)

•Intravenous pyelography (IVP)

•CT-scan

–Bladder ears are best seen when the bladder is filled and distended.

–It is important to recognize and diagnose bladder ears.

–This is specially so during the repair of inguinal hernia to avoid injury to the bladder which can be mistaken as a large hernia sac.

•Bladder agenesis:

–Bladder agenesis is a very rare congenital malformation.

–It is generally incompatible with life.

–All reported cases were seen in females

–This is because females have less outlet resistance than males and have preservation of renal function.

–In bladder agenesis, the ureters may enter into the urethra, vagina, Gartner duct cyst (female), prostatic urethra, rectum, or the patent urachus.

–Bladder agenesis are often associated with hydroureteronephrosis and renal dysplasia.

–Other associated anomalies include neurologic, orthopedic, hindgut, and other urogenital anomalies, such as renal agenesis and absence of the prostate, vagina, seminal vesicles, epididymis, or penis.

•Megacystis (Figs. 1.67, 1.68, 1.69, 1.70, and 1.71):

–Megacystis is an abnormally enlarged urinary bladder.

–This may result secondary to overfilling of the fetal bladder during development.

–This can be discovered antenatally on ultrasound or postnatally during evaluation of febrile urinary tract infection.

–Megacystis is associated with massive high-grade VUR.

–In the presence of massive reflux, a large percentage of the voiding bladder volume is refluxed into the upper tracts.

–This causes constant recycling of the urine between the bladder and ureters, resulting in progressive dilation of both.

–Megacystis can be seen in other conditions, such as:

•Posterior urethral valves

•Ehlers-Danlos syndrome

•Urethral diverticulum

•Microcolon hypoperistalsis syndrome

•Sacral meningomyelocele

•Sacrococcygeal teratoma

•Pelvic neuroblastoma

•Bladder duplication:

–Bladder duplication is a very rare malformation.

–It is divided into two types:

•Complete bladder duplication

•Partial bladder duplication

–Complete bladder duplication is more common than incomplete duplication.

–With complete bladder duplication, two urethras exist; with incomplete bladder duplication, the bladder joins distally into a single common urethra.

–The two halves of the bladder are on either side of the midline, with the corresponding ipsilateral ureter draining each bladder half.

–Associated anomalies occur more commonly in those with complete bladder duplication.

–These anomalies include:

•Duplication of the penis, vagina, uterus, lumbar vertebrae, and hindgut.

•In addition, fistulas may be present between the rectum, vagina, and urethra.

•Bladder septation:

–Bladder septation anomalies are rare.

–Fibromuscular or mucosa septations divide the bladder into equal or unequal portions.

–Septations may be complete or incomplete.

–The functioning of the associated renal units depends on the adequacy of upper tract drainage.