Congenital abnormalities Flashcards
(272 cards)
1
Q
How does malrotation present on ct and urography?
A
Anteriorly facing renal pelvis on CT Urography will show the calyces to be medial to the pelvis
2
Q
What is the cause of renal ectopy?
A
Due to lack of kidney acquiring blood supply during its cranial migration or spinal abnormality preventing migration
3
Q
What is the most common form of renal ectopy?
A
Pelvic kidney - located anterior to sacrum
4
Q
What test is run before any sort of surgical procedure on an ectopic kidney?
A
CTA to determine blood supply
5
Q
Which side is the intrathoracic kidney often found? M or F?
A
Male Left sided usually
6
Q
In true renal agenesis, what is found on cystoscopy?
A
Abscence of the trigone and ureteral orifice in the bladder
7
Q
What is the % of adrenal absence with renal agenesis?
A
8-10%
8
Q
What are some associations with renal agenesis in the male? female?
A
Male - ipsilateral seminal vesicle cysts, absence of ipsilateral vas deferens, testicular hypoplasia/agenesis, hypospadias Female - uni/bicornuate uterus, absent/hypoplastic uterus, aplasia of vagina
9
Q
What is rokitansky-kuster-hauser syndrome?
A
Mullerian ductal anomalies Type a - absent uterus and upper vagina, normal ovaries Tybe b - ovarian/renal abnormalities
10
Q
What are the facial features of bilateral renal agenesis?
A
Low set ears and prominent palpebral fold
11
Q
How do you differentiate acquired from congenital renal hypoplasia?
A
Acquired will have normal number of calyces congenital will have less calyces
12
Q
Where is the supernumerary kidney usually located?
A
caudal may be connected to ipsilateral kidney with separate collecting system
13
Q
What are the 4 types of crossed renal ectopy? Which is most common?
A
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
14
Q
Which is more common ectopy - L-R or R-L?
A
L->R is 3x more common
15
Q
What is thought to cause crossed renal ectopy?
A
Abnormally positioned umbilical artery which forces caudal migration to flow to the other hemiabdomen
16
Q
What is the most common renal anomaly? M or F?
A
Horseshoe kidney M 2x more common
17
Q
What is thought to cause horseshoe kidney
A
Abberant umbilical artery obstructs normal cephalic migration
18
Q
What is the common associated renal problem with horseshoe kidney?
A
UPJ obstruction
19
Q
What are the three imaging features of horseshoe kidney aside from the connection
A
Lower poles are more medial than upper poles, causing a renal tilt Kidneys are more caudad Malrotation with anterior facing pelves
20
Q
What is a pancake kidney?
A
Fusion of the upper and lower poles results in a flat pancake like renal mass with 2 distinct ureters and anterior collecting systems
21
Q
How many calyces are usually in a kidney? What is the association with a unipapillary kidney
A
10-14 ipsilateral renal hypoplasia and contralateral abnormalities in contrast, polycalycosis is usually isolated finding
22
Q
How do you differentiate megacalycosis vs hydro?
A
Megacalycosis will have normal renal pelvis and ureter kidney is also usually normal in size
23
Q
Blunt ended calyx that is broader than it is tall suggests what
A
abortive calyx
24
Q
where is an abortive calyx usually found
A
upper pole infundibulum
25
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
26
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
27
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
28
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
29
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
30
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
31
What is congenital megaureter?
Functional obstruction of DISTAL ureter
32
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
33
where is an abortive calyx usually found
upper pole infundibulum
34
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
35
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
36
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
37
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
38
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
39
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
40
What is congenital megaureter?
Functional obstruction of DISTAL ureter
41
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
42
where is an abortive calyx usually found
upper pole infundibulum
43
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
44
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
45
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
46
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
47
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
48
Where do ectopic ureteroceles end?
50% in bladder, 50% prostatic urethra
49
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
50
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
51
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
52
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
53
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
54
What is prune belly/eagle barret syndrome? which sex? association?
Triad of absent abdominal musculature, undescended testicles, urinary tract abnormalities males only twins
55
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
56
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
57
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
58
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
59
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
60
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
61
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
62
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
63
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
64
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
65
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
66
where is an abortive calyx usually found
upper pole infundibulum
67
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
68
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
69
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
70
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
71
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
72
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
73
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
74
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
75
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
76
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
77
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
78
where is an abortive calyx usually found
upper pole infundibulum
79
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
80
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
81
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
82
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
83
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
84
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
85
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
86
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
87
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
88
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
89
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
90
where is an abortive calyx usually found
upper pole infundibulum
91
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
92
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
93
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
94
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
95
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
96
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
97
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
98
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
99
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
100
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
101
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
102
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
103
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
104
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
105
where is an abortive calyx usually found
upper pole infundibulum
106
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
107
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
108
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
109
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
110
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
111
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
112
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
113
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
114
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
115
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
116
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
117
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
118
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
119
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
120
where is an abortive calyx usually found
upper pole infundibulum
121
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
122
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
123
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
124
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
125
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
126
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
127
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
128
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
129
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
130
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
131
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
132
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
133
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
134
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
135
where is an abortive calyx usually found
upper pole infundibulum
136
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
137
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
138
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
139
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
140
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
141
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
142
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
143
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
144
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
145
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
146
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
147
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
148
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
149
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
150
where is an abortive calyx usually found
upper pole infundibulum
151
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
152
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
153
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
154
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
155
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
156
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
157
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
158
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
159
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
160
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
161
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
162
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
163
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
164
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
165
where is an abortive calyx usually found
upper pole infundibulum
166
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
167
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
168
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
169
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
170
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
171
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
172
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
173
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
174
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
175
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
176
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
177
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
178
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
179
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
180
where is an abortive calyx usually found
upper pole infundibulum
181
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
182
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
183
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
184
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
185
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
186
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
187
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
188
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
189
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
190
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
191
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
192
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
193
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
194
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
195
Which ureter in a duplicated collected system is prone to obstruction? which one refluxes?
Obstruction - upper Reflux - lower
196
Blunt ended calyx that is broader than it is tall suggests what
abortive calyx
197
where is an abortive calyx usually found
upper pole infundibulum
198
DDx for a smooth marginated and fixed filling defect in the collecting system
Stone Aberrant/ectopic papilla (smooth margins and fixed position are characteristic)
199
What is the most common cause of abdominal mass in a neonate?
Congenital UPJ obstruction
200
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
201
What is beer drinkers hydronephrosis?
Mild UPJ obstruction, the diuresis caused by the alcohol causes hydro
202
How to differentiate extrarenal pelvis from UPJ obstruction?
Extra renal pelvis will have normal calcyeal size
203
When thinking of UPJ obstruction, what other abnormality must be considered?
Aberrant vessel with mass effect
204
What is congenital megaureter?
Functional obstruction of DISTAL ureter, not anatomic
205
How does a circumcaval ureter form?
Forms when the infrarenal IVC arises from the subcardinal vein and not the supracardinal vein
206
Which side is a circumcaval ureter found?
Always right sided since IVC forms from right sided veins
207
What is the reverse J sign?
The medial course of a circumcaval right ureter has a reverse J shape
208
What is the most common anomaly involving the urinary tract?
Partially duplicated collecting system
209
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
210
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
211
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
212
Which ureter in a duplicated collected system is prone to obstruction? which one refluxes?
Obstruction - upper Reflux - lower
213
What is the faceless kidney?
Complete duplicated pelves will have absence of collecting system elements or renal sinus fat in between
214
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
215
How does ectopic insertion of the ureter present in males?
Chronic or recurrent epididymitis
216
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
217
How does an obstructed upper pole moiety present on US?
Echo free cystic lesion in medial upper pole with dilated ureter coursing to bladder
218
Where is the most common site for ectopic ureter insertion in duplicated collecting systems?
Male - prostatic urethra Female - vestibule
219
What is the association with ectopic ureterocele?
Ipsilateral complete duplication
220
Which ureter has the ectopic ureterocele?
Upper pole moiety
221
Where do ectopic ureteroceles end?
50% in bladder, 50% prostatic urethra
222
What is an orthotopic ureterocele?
Dilation of a normally coursing ureter at the trigone orifice
223
What percentage of ectopic ureters are associated with duplicated collecting systems? M or F?
80% 6x more common in females
224
T or F: the more distal the ectopic insertion of the ureter, the more dysplastic the ipsilateral kidney
True
225
In a nonduplicated ectopic ureter, what happens to the ipsilateral trigone?
Absent
226
What causes clubbed calyces and overlying parenchymal scarring?
Reflux nephropathy
227
What is prune belly/eagle barret syndrome? which sex?
Triad of absent abdominal musculature, undescended testicles, urinary tract abnormalities males only
228
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
229
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
230
What is a hurley stick sign?
Widening of the distal ureters with exstrophy
231
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
232
Where is a bladder diverticulum usually located?
Superolateral to the ureteral orifice
233
What is the pathology associated with a bladder diverticulum?
Reflux due to distortion of the valve mechanism
234
What are bladder ears?
Inferolateral protrusions of the bladder in young kids due to incomplete bladder filling
235
What is the allantois?
Attachment of the bladder dome to the umbilicus.
236
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/
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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
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What are the remnants of mullerian duct atrophy?
Prostatic utricle and appendix testes
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Nonatrophy of the mullerian duct results in what?
cystic dilations along the route of the vas deferens
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Where do mullerian duct cysts occur?
Midline just superior to the prostate Midline - Mullerian
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What is the association with seminal vesicle cysts?
Ipsilateral absence of the kidney and ureter
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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
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Who gets posterior urethral valves?
Young males
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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)
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test of choice for posterior urethra valves?
voiding cystourethrogram
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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
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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.
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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
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What is the easiest way to differentiate meatal stenosis vs posterior urethral valves?
Meatal stenosis will have much more distal obstruction of catheter insertion
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What is a lacuna magna?
Wide necked diverticulum in the dorsal urethra associated with meatal stenosis
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Where is the defect in hypospadias?
Ventral surface
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Which is more common, hypo or epi spadias?
Hypospadias are more common
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Who gets congenital urethral diverticulum? Where?
Males only - females get acquired version ventral surface of anterior urethra
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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
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What are the indications of removal of an anomalous urethra?
Urinary incontinence and urethritis
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What is the function of cowpers glands?
Secrete mucin to help lubricate semen and prevent coagulation of sperm
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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
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Major risk factor for cryptorchidism?
Prematurity
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What are the three most common complications of cryptorchidism?
Malignant degeneration, sterility, torsion
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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
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What are the three main catergories of female genital tract anomaly?
Agenesis, duplication, anomalies related to DES
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What is a class 1 female genital anomaly?
Mullerian agenesis or hypoplasia varies - hypoplasia of vagina, cervix, uterus, fallopian tubes
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What is mayer-rokitansky-kuster-hauser syndrome?
Absence, of uterus, cervix, and vagina - can also see renal and ovarian abnormalities
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What is a class 2 female genital anomaly?
Failure of formation of one mullerian duct Unicornuate uterus
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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
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What is the association with uterine didelphys?
Renal agenesis
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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
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What is a class 5 female genital anomaly?
Septate uterus External surface appears normal, but there is a septum that separates the fundus
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What is a class 6 female genital anomaly?
Arcuate uterus Slight bulging of fundal surface of endometrial cavity into endometrial space
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What are class 7 female genital anomalies?
Associated with DES morphologic changes, adenosis, ridges/hoods/stenosis of the cervix
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What is the appearance of a DES uterus? What is the risk?
T shaped uterus Clear cell carcinoma
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what are the uterine anomalies?
