Congenital Anomalies and Trauma

CONGENITAL ANOMALIES
& TRAUMA OF THE
GENITOURINARY TRACT
DEPARTMENT OF UROLOGY IAŞI – 2010
CONGENITAL – KIDNEY
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more frequently than in any other organ
many cause impairment of renal function
association with other renal and extrarenal congenital anomalies
AGENESIS
bilateral – extremely rare; children do not survive; prenatal
suspicion – oligohydramnios (US)
unilateral – no symptoms; cystoscopy – ureteral ridge absent, no
orifice visualized; definitive diagnosis – isotope studies, US, CT
HYPOPLASIA
small kidney, smtm dysplastic; the other maybe larger than
normal
observed in infants with fetal alcohol syndrome or in utero
cocaine exposure
CONGENITAL – KIDNEY
differentiation – acquired atrophy (atrophic pyelonephritis, VUR,
stenosis of the renal artery)
 associated with hypertension  nephrectomy
ADULT POLYCYSTIC KIDNEY DISEASE
 autosomal dominant hereditary condition; almost always bilateral
(95%)
 in infants – autosomal recessive disease; short life expectancy
 cysts of the liver, spleen and pancreas
Etiology & Pathogenesis
 defects in the development of the collecting and uriniferous
tubules and in the mechanism of their joining  blind secretory
tubules connected to functioning glomeruli  cysts enlarge &
compress adjacent parenchyma  destroy it by ischemia and
occlude normal tubules  progressive functional impairment
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CONGENITAL – KIDNEY
Symptoms
 pain (drag on the vascular pedicles by the
heavy kidneys, obstruction, infection,
hemorrhage into a cyst)
 gross or microscopic total hematuria
 colic (blood clots or stones)
 abdominal mass
 infection (chills, fever, renal pain)
 renal insufficiency (headache, nausea,
vomiting, weakness, loss of weight)
Signs
 palpable kidneys (nodular, tender if infected)
 hypertension ( 60-70%)
 fever (pyelonephritis or infected cysts)
CONGENITAL – KIDNEY
uremia (anemia, loss of weight)
Laboratory
 anemia (chronic loss of blood or hematopoietic depression of
uremia)
 proteinuria and hematuria; pyuria and bacteriuria
 progressive  creatinine clearance
US – multiple transsonic images, large renal size
IVU
 enlarged renal shadows on a plain film
 bizarre (spider deformity) caliceal pattern (broadened, flattened,
enlarged, curved)
CT – extremely accurate (95%) for diagnosis – multiple thin-walled
cysts filled with fluid, large renal size
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CONGENITAL – KIDNEY
Differential Diagnosis
 bilateral hydronephrosis
 bilateral renal tumor
 von Hippel-Lindau disease (angiomatous cerebellar cyst, angiomatosis of
the retina, tumors or cysts of the pancreas, multiple bilateral cysts or
adenocarcinomas of both kidneys)
 tuberous sclerosis (convulsive seizures, mental retardation, adenoma
sebaceum, hamartomatous tumors of the skin, brain, retinas, bones, liver,
heart, and kidneys)
simple cyst
Complications
 pyelonephritis
 infection of cysts
 severe gross hematuria
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CONGENITAL – KIDNEY
Treatment
 General Measures: low-protein diet (0.5-0.75 g/kg/d), fluids (≥ 3 L/d),
reasonable physical activity, treatment of uremia (hemodialysis),
control of hypertension
 Surgery: a large cyst compressing the upper ureter (obstruction
and impairment of renal function)  resected or aspirated; renal
insufficiency  chronic dialysis or renal transplantation
 Treatment of Complications: pyelonephritis; infection of cysts 
surgical or percutaneous drainage; severe bleeding 
nephrectomy or selective embolization
Prognosis
 children – very poor
 clinical signs and symptoms after age 35-40 – more favorable
CONGENITAL – KIDNEY
SIMPLE (SOLITARY) CYST
 usually unilateral and single, but may be
multiple and multilocular; more rarely,
bilateral
Etiology & Pathogenesis
 origin may be similar to that of polycystic
kidneys, but the lesion can be acquired
(chronic dialysis)
 as it grows  compresses and may
destroy renal parenchyma; may compress
the ureter  progressive hydronephrosis
 spontaneous regression of cysts has occasionally been noted
Pathology
 5% contain hemorrhagic fluid (1/2 have papillary cancers on their walls)
CONGENITAL – KIDNEY
Clinical Findings
 Symptoms: pain in the flank or back, intermittent and dull; severe
pain (bleeding); mass in the abdomen; pain in the flank, malaise,
and fever (infected cyst)
 Signs: mass palpated or percussed; tenderness in the flank
(infected cyst)
 Laboratory: usually normal
 US – differentiates between a cyst and a solid mass; percutaneous
cyst aspiration
 CT – differentiates renal cyst and tumor (attenuation of cysts ≈ water,
density of tumors ≈ parenchyma; parenchyma more dense with contrast i.v.,
cyst unaffected)
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Percutaneous Cyst Aspiration With Cystography: differentiation
between cyst and tumor
CONGENITAL – KIDNEY
Differential Diagnosis
 carcinoma of the kidney (walls of a simple cyst – cancerous degeneration)
 polycystic kidney disease, renal carbuncle, hydronephrosis
 extrarenal tumor (adrenal, mixed retroperitoneal sarcoma)
 echinococcal (hydatid) cyst of the kidney
Complications (rare) – spontaneous infection, hemorrhage into the
cyst, hydronephrosis (cyst of the lower pole)
Treatment
 follow-up with US; if no definitive diagnosis with US, CT or MRI 
perc. needle aspiration (clear fluid  benign cyst), cytologic
evaluation, cystography; hemorrhagic fluid  surgical exploration
 complications: infected cyst  intensive antimicrobial therapy +
percutaneous or surgical drainage; hydronephrosis 
percutaneous puncture, aspiration & sclerotherapy
CONGENITAL – KIDNEY
RENAL FUSION
 ≈ 1‰, the most common – horseshoe kidney
Etiology & Pathogenesis
 fusion of the 2 metanephroi occurs early in embryologic life 
seldom ascend to the normal position (may remain in the pelvis);
may derive its blood supply from many vessels (aorta, iliacs)
Pathology
 normal rotation cannot occur  each pelvis lies on the anterior
surface  ureter must ride over the isthmus (horseshoe kidney)
or traverse the anterior surface (fused kidney)  ureteral
compression  hydronephrosis
 horseshoe kidney: isthmus joins the lower poles of each kidney;
each renal mass lies lower than normal; axes of the masses are
vertical or divergent upwards
CONGENITAL – KIDNEY
Clinical findings
 Symptoms: no symptoms; ureteral obstruction; infection; calculus
 Signs: usually negative; abnormally placed renal mass (horseshoe
kidney – palpable mass over the lower lumbar spine [isthmus],
crossed ectopy – mass in the flank or lower abdomen)
 Laboratory: normal
 X-Ray: horseshoe kidney – axes of the kidneys parallel to the spine
on a plain film, isthmus can be identified; large soft-tissue mass in
one flank w/o a renal shadow on the other side
 IVU: horseshoe kidney – renal pelves lie on the anterior surfaces
of the kidney masses, calyces in the lower pole that point
medially and lie medial to the ureter; crossed renal ectopy with
fusion – 2 pelves and 2 ureters (one ureter must cross the
midline); cake or lump kidney (fused pelvis kidney) – may
compress the dome of the bladder
CONGENITAL – KIDNEY
Clinical findings
 CT – outlines the renal mass
 Retrograde urograms: show the position of theureters and pelves
(pelvic fused kidney)
 Renal scanning: delineates the renal mass and its contour
Differential Diagnosis - separate kidneys that fail to undergo normal
rotation, other abdominal masses
Complications – ureteral obstruction, hydronephrosis, stone,
infection, dystocia due to a large fused kidney occupying the
concavity of the sacrum
Treatment – no treatment unless obstruction or infection; drainage
of a horseshoe kidney by dividing the isthmus; surgical resection
CONGENITAL – KIDNEY
ECTOPIC KIDNEY
Simple Ectopy
 low kidney on the proper side that failed to ascend normally (may
lie in the pelvis), blood supply from adjacent vessels, short ureter;
prone to ureteral obstruction and infection  pain or fever
 may be palpable - differential diagnosis (cancer of the bowel,
appendiceal abscess)
 IVU – position of the kidney; no redundancy of the ureter (as with
nephroptosis or acquired ectopy [displacement by large adrenal
tumor])
 treatment of obstruction and infection
Crossed Ectopy Without Fusion
 the kidney lies on the opposite side – US, angiography or CT for
diagnosis
CONGENITAL – KIDNEY
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ABNORMAL ROTATION
IVU – abnormal position
MEDULLARY SPONGE KIDNEY
congenital autosomal recessive defect – cystic dilatation of the
renal collecting tubules (usually bilateral, affecting all of the
papillae)
urinary stasis in the tubules  infection and calculi
symptoms – from infection and stone formation
diagnosis (IVU or CT): pelvis and calyces are normal, but dilated
(streaked) tubules (cystic dilatation); plain film – small, round
calculi in the pyramidal regions (just beyond the calyces)
differential diagnosis: tuberculosis, healed papillary necrosis,
nephrocalcinosis
terapy of the complications (pyelonephritis, renal calculi)
CONGENITAL – URETER
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are common, may cause severe obstruction or may be
asymptomatic and of no clinical significance
URETERAL ATRESIA
ureter may be absent entirely or may end blindly after extending
only part of the way to the flank
embryologically – failure of the ureteral bud to form from the
mesonephric duct or by an arrest in its development before it
comes in contact with the metanephric blastema
atretic ureteral bud  absent or multicystic, dysplastic kidney,
(rarely associated with hypertension, infection or tumor);
contralateral VUR is common (voiding cystourethrogram)
treatment – observation
CONGENITAL – URETER
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DUPLICATION OF THE URETER
one of the most common congenital malformations of the urinary
tract (0.9%); autosomal dominant; F>M; often bilateral
incomplete (Y) type – caused by branching of the ureteral bud
before it reaches the metanephric blastema; disorders of
peristalsis may occur near the point of union
complete type – 2 ureteral buds  2 totally separate ureters and 2
separate renal pelves; the ureter to the upper segment migrates
farther, ending medial and inferior to the ureter draining the
lower segment (Weigert-Meyer law)  the ureter draining the
upper segment may migrate too far caudally and become ectopic
and obstructed; the ureter draining the lower segment may end
laterally and have a short intravesical tunnel  VUR
CONGENITAL – URETER
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clinical findings – asymptomatic; persistent or recurrent
infections; F – ureter to the upper pole may be ectopic, distal to
the external sphincter or outside the urinary tract  true
incontinence + normal pattern of voiding; M – ectopic ureter is
always proximal to the external sphincter (w/o incontinence)
IVU – duplication; occasionally, one segment of the kidney is not
visualized, but there is displacement of the visualized calyces or
ureter or discrepancy between the amount of renal parenchyma
and the relatively small number of visualized calyces
voiding cystourethrogram – VUR or ureterocele
US – hydronephrotic upper pole and dilated distal ureter; evaluate
parenchymal thickness and the presence of bladder anomalies
CONGENITAL – URETER
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renal scanning (99mTc-DMSA) – estimating the degree of renal
function in each renal segment
treatment
 lower grades of reflux  medically
 higher grades of reflux  surgically
 very poor renal function in one segment  heminephrectomy
 to preserve renal parenchyma  pyeloureterostomy,
ureteroureterostomy or ureteral reimplantation
URETEROCELE
sacculation of the terminal portion of the ureter; it may be
intravesical or ectopic (bladder neck or urethra)
F:M = 7:1; ≈ 10% are bilateral
CONGENITAL – URETER
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delayed or incomplete canalization of the ureteral bud  early
prenatal obstruction and expansion of the ureteral bud; forms
between the superficial and deep muscle layers of the trigone
clinical findings – infection; bladder outlet obstruction or
incontinence; calculi can develop in the distal ureter
IVU, US – significant ureterohydronephrosis and a dysplastic
segment of the upper pole of the kidney; voiding
cystourethrography – reflux into the lower pole or contralateral
ureter; renal scanning – estimating renal function
treatment – transurethral incision; heminephrectomy and
ureterectomy, excision of the ureterocele, vesical reconstruction,
ureteral reimplantation
CONGENITAL – URETER
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ECTOPIC URETERAL ORIFICE
most commonly in association with duplication of the ureter
caused by a delay or failure of separation of the ureteral bud from
the mesonephric duct
clinical picture: M – urinary tract infection or epididymitis (ureter
may drain directly into the vas deferens or seminal vesicle);
F – continual dribbling despite normal voiding – pathognomonic
(ureteral orifice in the urethra, vagina or perineum), infection
(urgency and urge incontinence)
US, voiding cystourethrography, MRI, cystoscopy (hemitrigone,
ectopic orifice may be visualized or demonstrated by retrograde
catheterization) or laparoscopy
renal scanning – estimates relative renal function
CONGENITAL – URETER
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OBSTRUCTION OF THE URETEROPELVIC JUNCTION (UPJ)
the most common congenital abnormality of the ureter; M:F =
5:2; L:R = 5:2
bilateral obstruction – 10-15%, especially common in infants
cause of obstruction (intrinsic or extrinsic): ureteral polyps and
valves (very rare), angulation and kink at the junction, true
stenosis (rarely), thin-walled, hypoplastic proximal ureter
(frequently), histologic and ultrastructural changes  abnormal
peristalsis through the UPJ and interference with pelvic emptying,
high origin of the ureter from the renal pelvis, abnormal
relationship of the UPJ to a lower-pole renal artery
clinical findings - most common symptoms: pain and vomiting;
hematuria, urinary infection, calculi, trauma to the enlarged
kidney or (rarely) hypertension
CONGENITAL – URETER
US – diagnosis; prenatal US – diagnosis in utero; in equivocal
cases, diuretic renography or antegrade urography with pressureflow studies; voiding cystourethrogram – findings in VUR may be
similar
Therapy: symptomatic obstruction  treated surgically; early surgery
– kidneys with diminished function, massive hydronephrosis,
infection or stones; nonoperative surveillance with close followup
 procedures: dismembered pyeloureteroplasty (Anderson-Hynes),
Foley Y-V plasty, pelvic flap procedures (Culp, Scardino)
 endopyelotomy – antegrade, via a nephrostomy tract, or
retrograde, using either a ureteroscope (for direct vision) or an
Acucise balloon catheter with fluoroscopic visualization
 laparoscopic pyeloplasty
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CONGENITAL – URETHRA
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URETHRAL STRICTURE
uncommon in infant boys (fossa navicularis and membranous
urethra)
severe strictures may cause bladder damage
and hydronephrosis
symptoms of obstruction or urinary infection
POSTERIOR URETHRAL VALVES
the most common obstructive urethral lesions
in male infants and newborns
treatment – destruction of the valves –
transurethral fulguration of the valves
CONGENITAL – URETHRA
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HYPOSPADIAS
urethral meatus opens on the ventral side of the
penis proximal to the tip of the glans penis
repair with foreskin island flaps/buccal mucosa
grafts and incised urethral plate
EPISPADIAS
urethra opens on the dorsal aspect of the
penis; a distal groove extends from the
meatus through the glans
surgery is required to correct the
incontinence, remove the chordee to
straighten the penis, and extend the urethra
out onto the glans penis
CONGENITAL – TESTIS
CRYPTORCHIDISM
 incidence: 3.4-5.8% (at birth) & 1.8% (1 year – puberty)  after
the first year spontaneous descent is unlikely to occur
 ectopic – descend normally through the external inguinal ring,
then misdirected to an extrascrotal position (superficial inguinal,
perineal, prepenile, transverse scrotal, femoral or umbilical)
 true cryptorchid – arrested in their normal line of descent
(intraabdominal, intracanalicular or suprascrotal)
 1/3 of cryptorchid cases are bilateral  infertility
Histology and Endocrine Pathology
 reduced number of germ cells (2nd year, persists thereafter)
 impaired function of the hypothalamic-pituitary-gonadal axis 
 testosterone  failure of testicular descent + failure of
proliferation and maturation of germ cells
CONGENITAL – TESTIS
the higher the testis location  the worse its histology
 unilateral cryptorchidism is a bilateral disease  contralateral
descended testis is abnormal – similar abnormalities, but to a
lesser degree  subfertility
Diagnosis
 every general pediatric examination in boys – examination of the
testicular position!
 US – high false-negative rate! (testis present despite negative
ultrasound)
 CT (irradiation!) & MRI
 selective diagnostic laparoscopy  orchidopexy or orchiectomy
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CONGENITAL – TESTIS
Treatment
 early (8-12 months)   risk of infertility, malignancy (10-46)
and testicular torsion
 hormonal treatment  induction of testicular descent and
stimulation of germ cell maturation and proliferation
 hCG
 GnRH (Kryptokur) nasal spray 1.2 mg/d,28 d, followed by hCG
1,500 IU/wk, 3 wks
 LHRH analog (Buserelin) – nasal spray
 early orchiopexy (≤ 1 year)
 postpubertal – orchiectomy
INJURIES TO THE G-U TRACT
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EMERGENCY DIAGNOSIS & MANAGEMENT
10% of all injuries
initial assessment – control of hemorrhage and shock, along with
resuscitation (i.v. lines and urethral catheter – blood at the
urethral meatus?)
history – detailed description of the accident (? gunshot wounds –
type and caliber)
examination of abdomen and genitalia
 contusions or subcutaneous hematomas
 fractures of the lower ribs and/or the bony pelvis
 diffuse abdominal tenderness
INJURIES TO THE G-U TRACT
SPECIAL EXAMINATIONS
Catheterization and Assessment of Injury (Staging)
 catheterization – blood at the urethral meatus in men  urethral
injury  catheterization should not be attempted, but retrograde
urethrography; no blood  urethral catheter  ? microscopic or
gross hematuria
 abdominal CT with contrast media – retroperitoneal hematoma,
renal lacerations, urinary extravasation, renal arterial and venous
injuries, intra-abdominal injuries (liver, spleen, pancreas, bowel)
 retrograde cystography (≥ 300 mL) – ? bladder perforations
 retrograde urethrography (12 F/3 mL) – 20 mL
INJURIES TO THE G-U TRACT
Catheterization and Assessment of Injury (Staging)
 arteriography – renal parenchymal and vascular injuries,
persistent bleeding from pelvic fractures  embolization with
Gelfoam or autologous clot
 IVU – detect renal and ureteral injury
Cystoscopy and Retrograde Uretero-Pyelography
 seldom necessary
Abdominal Sonography
 if CT not available
INJURIES TO THE KIDNEY
the most common injuries of the urinary system
 pathologic kidneys (hydronephrosis, malignant tumors etc.) are
more readily ruptured from mild trauma
Etiology
 blunt trauma to the abdomen, flank or back (85%)
 rapid deceleration  can cause major vascular injury
 gunshot and knife wounds  penetrating injuries
 associated abdominal visceral injuries are common!
Pathology & Classification
 early pathologic findings – grade
 1 – renal contusion or bruising of the renal parenchyma 
microscopic hematuria
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INJURIES TO THE KIDNEY
2 – renal parenchymal laceration into the cortex  small
perirenal hematoma
 3 – renal parenchymal laceration extending through the
cortex and into the medulla  significant bleeding, large
retroperitoneal hematoma
 4 – renal parenchymal laceration extending into the collecting
system; main renal artery thrombosis from blunt trauma,
segmental renal vein or both; artery injury with contained
bleeding
 5 – multiple grade 4 parenchymal lacerations, renal pedicle
avulsion or both; main renal vein or artery injury from
penetrating trauma
late pathologic findings – urinoma, hydronephrosis, arteriovenous
fistula, renal vascular hypertension
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INJURIES TO THE KIDNEY
late pathologic findings – urinoma, hydronephrosis, arteriovenous
fistula, renal vascular hypertension
Clinical Findings & Indications for Studies
 some cases of renal vascular injury (deceleration) are not
associated with hematuria  imaging studies
 the degree of renal injury does not correspond to the degree of
hematuria
 gross hematuria or microscopic hematuria with shock (systolic BP
< 90 mm Hg)  imaging studies
 symptoms – pain (flank or over the abdomen), hematuria,
abdominal distention, ileus, nausea and vomiting (retroperitoneal
bleeding )
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INJURIES TO THE KIDNEY
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signs – shock or signs of a large loss of blood (heavy
retroperitoneal bleeding), ecchymosis in the flank or upper
quadrants of the abdomen, lower rib fractures, diffuse abdominal
tenderness
 "acute abdomen" – free blood in the peritoneal cavity
 palpable mass ≈ large retroperitoneal hematoma or urinary
extravasation (torn retroperitoneum  free blood in the
peritoneal cavity, but no palpable mass)
laboratory – microscopic or gross hematuria, anemia (acute or
chronic)
staging  systematic approach
 abdominal CT scan  parenchymal lacerations, urinary
extravasation, extent of the retroperitoneal hematoma,
nonviable tissue, injuries to surrounding organs
 arteriography – major arterial and parenchymal injuries
INJURIES TO THE KIDNEY
Complications
 early – hemorrhage (persistent retroperitoneal bleeding or heavy
gross hematuria), urinary extravasation (urinoma)  abscess
formation & sepsis
 late – hypertension, hydronephrosis, arteriovenous fistula,
calculus formation, pyelonephritis, heavy late bleeding !
Treatment
 emergency measures – prompt treatment of shock and
hemorrhage, complete resuscitation and evaluation of associated
injuries
 surgical measures
 blunt trauma (< 5%) – persistent retroperitoneal bleeding,
urinary extravasation, nonviable renal parenchyma and renal
pedicle injuries
 penetrating injuries – surgically explored (associated organ
injury)
INJURIES TO THE KIDNEY
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complications
 retroperitoneal urinoma or perinephric abscess  surgical
drainage
 malignant hypertension  vascular repair or nephrectomy
 hydronephrosis  surgical correction or nephrectomy
INJURIES TO THE URETER
rare, during difficult pelvic surgical procedures, gunshot wounds,
rapid deceleration accidents (avulsion of the ureter from the renal
pelvis), endoscopic procedures (ureteroscopies, basket
manipulation of ureteral calculi)
 flank & abdominal pain, nausea, vomiting, ileus; vaginal or
cutaneous fistula
 imaging: IVU, CT scan
Treatment – prompt repair (if stenting not feasible)
 immediate – ureteroureterostomy, transureteroureterostomy,
reimplantation into the bladder, autotransplantation of the
kidney, bowel replacement of the ureter + stenting
 late – percutaneous nephrostomy, then repair
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INJURIES TO THE BLADDER
occur most often from external force and are often associated
with pelvic fractures
 iatrogenic injury – gynecologic and surgical extensive pelvic
procedures and transurethral operations
Pathogenesis & Pathology
 extraperitoneal: pelvic fractures  extraperitoneal rupture 
urine extravasation  deep pelvic abscess
 intraperitoneal  acute abdomen (peritonitis)
Clinical findings
 KUB – pelvic fractures
 CT scan
 retrograde cystography
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INJURIES TO THE BLADDER
Treatment
 shock & hemorrhage treatment
 extraperitoneal – urethral catheter drainage (10 d); cystoraphy
 intraperitoneal – transperitoneal approach, cystoraphy, bladder &
peritoneal drainage
INJURIES TO THE URETHRA
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INJURIES TO THE POSTERIOR URETHRA
pelvic fractures from blunt trauma  the membranous urethra is
sheared from the prostatic apex  acute retention of urine
retrograde urethrogram
treatment – suprapubic cystostomy, delayed urethral
reconstruction
INJURIES TO THE ANTERIOR URETHRA
straddle injury, self- or iatrogenic instrumentation
urethral bleeding ± retention of urine; retrograde urethrogram
urethral contusion  no instrumentation or urethral catheter
urethral laceration  suprapubic cystostomy, delayed retrograde
urethrography, no further instrumentation or urethral catheter or
urethroplasty
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