Congenital abnormalities Flashcards
How does malrotation present on ct and urography?
Anteriorly facing renal pelvis on CT Urography will show the calyces to be medial to the pelvis
What is the cause of renal ectopy?
Due to lack of kidney acquiring blood supply during its cranial migration or spinal abnormality preventing migration
What is the most common form of renal ectopy?
Pelvic kidney - located anterior to sacrum
What test is run before any sort of surgical procedure on an ectopic kidney?
CTA to determine blood supply
Which side is the intrathoracic kidney often found? M or F?
Male Left sided usually
In true renal agenesis, what is found on cystoscopy?
Abscence of the trigone and ureteral orifice in the bladder
What is the % of adrenal absence with renal agenesis?
8-10%
What are some associations with renal agenesis in the male? female?
Male - ipsilateral seminal vesicle cysts, absence of ipsilateral vas deferens, testicular hypoplasia/agenesis, hypospadias Female - uni/bicornuate uterus, absent/hypoplastic uterus, aplasia of vagina
What is rokitansky-kuster-hauser syndrome?
Mullerian ductal anomalies Type a - absent uterus and upper vagina, normal ovaries Tybe b - ovarian/renal abnormalities
What are the facial features of bilateral renal agenesis?
Low set ears and prominent palpebral fold
How do you differentiate acquired from congenital renal hypoplasia?
Acquired will have normal number of calyces congenital will have less calyces
Where is the supernumerary kidney usually located?
caudal may be connected to ipsilateral kidney with separate collecting system
What are the 4 types of crossed renal ectopy? Which is most common?
Crossed fused ectopy - both kidneys on one side of midline with normal ureteral insertion, most common (90%) Crossed non fused ectopy Solitary crossed Bilaterally crossed - kidneys in normal positions but ureters cross and enter bladder on opposite side
Which is more common ectopy - L-R or R-L?
L->R is 3x more common
What is thought to cause crossed renal ectopy?
Abnormally positioned umbilical artery which forces caudal migration to flow to the other hemiabdomen
What is the most common renal anomaly? M or F?
Horseshoe kidney M 2x more common
What is thought to cause horseshoe kidney
Abberant umbilical artery obstructs normal cephalic migration
What is the common associated renal problem with horseshoe kidney?
UPJ obstruction
What are the three imaging features of horseshoe kidney aside from the connection
Lower poles are more medial than upper poles, causing a renal tilt Kidneys are more caudad Malrotation with anterior facing pelves
What is a pancake kidney?
Fusion of the upper and lower poles results in a flat pancake like renal mass with 2 distinct ureters and anterior collecting systems
How many calyces are usually in a kidney? What is the association with a unipapillary kidney
10-14 ipsilateral renal hypoplasia and contralateral abnormalities in contrast, polycalycosis is usually isolated finding
How do you differentiate megacalycosis vs hydro?
Megacalycosis will have normal renal pelvis and ureter kidney is also usually normal in size
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter
What are the 3 main abnormalities involved with complete duplication?
Maldeveloped valve in lower pole ureter Ectopic ureteral insertion of upper pole ureter outside bladder Ectopic ureterocele of upper pole ureter
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
Where do ectopic ureteroceles end?
50% in bladder, 50% prostatic urethra
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
What is prune belly/eagle barret syndrome? which sex? association?
Triad of absent abdominal musculature, undescended testicles, urinary tract abnormalities males only twins
What are the urinary tract abnormalities with prune belly syndrome?
Can see renal dysplasia or hypoplasia or normal kidneys Tortuous and dilated ureters Enlarged bladder with/without patent urachus (tract to the umbilicus) Dilated and rapidly tapering prostatic urethra
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
What is the Meyer-Weigert law?
States that the ureter from the upper moiety will enter inferomedially (ectopic) States that upper moiety is predisposed to obstruction
Which ureter in a duplicated collected system is prone to obstruction? which one refluxes?
Obstruction - upper Reflux - lower
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
where is an abortive calyx usually found
upper pole infundibulum
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
What is the pathology behind UPJ obstruction and congenital megaureter?
Deficiency and derangement of ureteric smooth muscle fibers with associated fibrosis - results in abnormal peristalsis
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
What is a ureteral diverticulum?
When a branch of a partially duplicated collecting system fails to reach the metanephric blastema (kidney) and results in a blind ending ureteral stump
What is the embryologic difference between partial and complete duplicated collecting systems?
Partial is a single ureteral bud from the mesonephric duct that branches before it gets to the metanephric blastema Complete has two separate ureteral buds from the mesonephric duct
What is the Meyer-Weigert law?
States that the ureter from the upper moiety will enter inferomedially (ectopic) States that upper moiety is predisposed to obstruction
Which ureter in a duplicated collected system is prone to obstruction? which one refluxes?
Obstruction - upper Reflux - lower
What is the faceless kidney?
Complete duplicated pelves will have absence of collecting system elements or renal sinus fat in between
What are the 3 main abnormalities involved with complete duplication?
Maldeveloped valve in lower pole ureter Ectopic ureteral insertion of upper pole ureter outside bladder Ectopic ureterocele of upper pole ureter
How does ectopic insertion of the ureter present in males?
Chronic or recurrent epididymitis
What is the drooping lilly sign?
In a duplicated collecting system with obstruction of the upper pole moiety, the lower pole moiety will be displaced inferomedially with a smaller than usual number of calyces
How does an obstructed upper pole moiety present on US?
Echo free cystic lesion in medial upper pole with dilated ureter coursing to bladder
Where is the most common site for ectopic ureter insertion in duplicated collecting systems?
Male - prostatic urethra Female - vestibule
What is the association with ectopic ureterocele?
Ipsilateral complete duplication
Which ureter has the ectopic ureterocele?
Upper pole moiety
Where do ectopic ureteroceles end?
50% in bladder, 50% prostatic urethra
What is an orthotopic ureterocele?
Dilation of a normally coursing ureter at the trigone orifice
What percentage of ectopic ureters are associated with duplicated collecting systems? M or F?
80% 6x more common in females
T or F: the more distal the ectopic insertion of the ureter, the more dysplastic the ipsilateral kidney
True
In a nonduplicated ectopic ureter, what happens to the ipsilateral trigone?
Absent
What causes clubbed calyces and overlying parenchymal scarring?
Reflux nephropathy
What is prune belly/eagle barret syndrome? which sex?
Triad of absent abdominal musculature, undescended testicles, urinary tract abnormalities males only
What are the urinary tract abnormalities with prune belly syndrome?
Can see renal dysplasia or hypoplasia or normal kidneys Tortuous and dilated ureters Enlarged bladder with/without patent urachus Dilated and rapidly tapering prostatic urethra
What is the most common congenital bladder lesion? What is it? What are the associations?
Bladder exstrophy - deficiency in development of lower abdominal bladder muscles, bladder is open and mucosa is continuous with skin. Epispadias Skeletal - pubic symphysis widening GI - hernias
What is a hurley stick sign?
Widening of the distal ureters with exstrophy
What is the spectrum of bladder duplication?
Complete - Each bladder has normal musculature and mucosa. Can have separate urethral orifice that drains into single urethra or have separate urethra and penises Partial - incomplete septal division
Where is a bladder diverticulum usually located?
Superolateral to the ureteral orifice
What is the pathology associated with a bladder diverticulum?
Reflux due to distortion of the valve mechanism
What are bladder ears?
Inferolateral protrusions of the bladder in young kids due to incomplete bladder filling
What is the allantois?
Attachment of the bladder dome to the umbilicus.
What is the urachus?
As the bladder lowers into the pelvis from the abdomen during embryogenesis, the bladder dome narrows and forms the urachus which elongates with bladder descent/
What is the spectrum of urachal remnants? M or F?
M 3x Patent urachus Umbilical sinus - failure of closure at the umbilical attachement Urachocele - Failure of closure at the bladder dome Urachal cyst - failure of closure in any other site
What are the remnants of mullerian duct atrophy?
Prostatic utricle and appendix testes
Nonatrophy of the mullerian duct results in what?
cystic dilations along the route of the vas deferens
Where do mullerian duct cysts occur?
Midline just superior to the prostate Midline - Mullerian
What is the association with seminal vesicle cysts?
Ipsilateral absence of the kidney and ureter
What are the three types of posterior urethral valves?
1 - leaflets from distal verumontanum to urethral wall 2 - proximal leaflets 3 - iris diaphragm across distal prostatic urethra
Who gets posterior urethral valves?
Young males
What is the urethral crest?
Continuation of the inferior aspect of the verumontanum which is a mucosal fold that divides into two to four fins (plica colliculi)
test of choice for posterior urethra valves?
voiding cystourethrogram
What is a fibroepithelial polyp in the urethra?
A stalked polyp that arises from the prostate and connects to the verumontanum, and extends in the prostatic urethra or into the bladder When voiding, the polyp will extend distally into the bulbar urethra
What is atresia ani-urethralis
Associated with anal atresia, a fistulous tract can from between the bowel and posterior urethra. Tehre will be difficulty catheterizing the bladder.
What condition is associated with bowel calculi?
Anal atresia - the passage of urine from a fistulous tract between the posterior urethra and the bowel precipitates crystal formation with bowel mucous
What is the easiest way to differentiate meatal stenosis vs posterior urethral valves?
Meatal stenosis will have much more distal obstruction of catheter insertion
What is a lacuna magna?
Wide necked diverticulum in the dorsal urethra associated with meatal stenosis
Where is the defect in hypospadias?
Ventral surface
Which is more common, hypo or epi spadias?
Hypospadias are more common
Who gets congenital urethral diverticulum? Where?
Males only - females get acquired version ventral surface of anterior urethra
How do duplicated urethras lie in the male vs female? which is functional?
Males - one on top of another, ventral is more functional Female - side by side
What are the indications of removal of an anomalous urethra?
Urinary incontinence and urethritis
What is the function of cowpers glands?
Secrete mucin to help lubricate semen and prevent coagulation of sperm
What is the presentation of cowpers retention cysts?
Swell, causing ductal obstruction and mass effect on the bulbar urethra Will be seen as an indentation on the floor of the mid or proximal bulbar urethra
Major risk factor for cryptorchidism?
Prematurity
What are the three most common complications of cryptorchidism?
Malignant degeneration, sterility, torsion
Does location of the undescended teste have an affect on the risk of malignancy?
Yes - the more proximal to the abdomen, the higher the risk
What are the three main catergories of female genital tract anomaly?
Agenesis, duplication, anomalies related to DES
What is a class 1 female genital anomaly?
Mullerian agenesis or hypoplasia varies - hypoplasia of vagina, cervix, uterus, fallopian tubes
What is mayer-rokitansky-kuster-hauser syndrome?
Absence, of uterus, cervix, and vagina - can also see renal and ovarian abnormalities
What is a class 2 female genital anomaly?
Failure of formation of one mullerian duct Unicornuate uterus
What is class 3 female genital anomaly?
Uterine didelphys - two cervices, each with there own cornu and tube May have own vagina or there may be a vaginal septum Note on HSG, both endocervical canal must be opacified to show the anomaly
What is the association with uterine didelphys?
Renal agenesis
What is a class 4 female genital anomaly?
Bicornuate uterus one cervix with two upper uterine segments and two fundi, each with its own cornu
What is a class 5 female genital anomaly?
Septate uterus External surface appears normal, but there is a septum that separates the fundus
What is a class 6 female genital anomaly?
Arcuate uterus Slight bulging of fundal surface of endometrial cavity into endometrial space
What are class 7 female genital anomalies?
Associated with DES morphologic changes, adenosis, ridges/hoods/stenosis of the cervix
What is the appearance of a DES uterus? What is the risk?
T shaped uterus Clear cell carcinoma
what are the uterine anomalies?
