1 - pediatric abnormalities Flashcards
1
Q
most common cause of prenatal hydronephrosis
A
transient (50-70%)
2
Q
most common pathologic cause of prenatal hydro
A
UPJO (10-30%)
3
Q
% with upjo presenting bilaterally
A
10%
4
Q
most common side for UPJO
A
left > right
5
Q
muscle fiber type involved in intrinsic UPJO
A
CIRCULAR muscle fibers at UPJ
6
Q
how does secondary UPJO due to reflux happen
A
severe VUR may cause totuous ureter and kinking at UPJ
7
Q
mgmt of 2ndary UPJO
A
fix UPJO before reflux
8
Q
how does mag 3 work in the kidney
A
tubular secretion
9
Q
how does DTPA work in kidney
A
glomerular filtration
10
Q
how does DMSA work in kidney
A
proximal tubular binding
11
Q
purpose of mag 3
A
function and drainage, same as MAG 3
12
Q
stent effect on urine leak after pyeloplasty
A
reduces leakage
13
Q
endoscopic management for pyeloplasty failure in kids?
A
not used very often
14
Q
pro and con of nuclear cystogram in kids
A
less radiation, poor anatomy
15
Q
3 indications for surgical correction of VUR
A
- poor compliance with prophylaxis, 2. breakthrough pyelo on prophylaxis, 3. parental decision
16
Q
ratio of ureter to tunnel for reimplant
A
5:01
17
Q
success rate of reimplant
A
98%
18
Q
overall success rate of deflux
A
72%
19
Q
2 things that predict improved outcomes in deflux
A
low grade, multiple injections
20
Q
post-procedure VCUG for deflux vs reimplant
A
needed for deflux, not reimplant
21
Q
definition of megaureter
A
> 7 mm
22
Q
2 major classifications of megaureter
A
obstructed or refluxing
23
Q
when to observe obstructed megaureter
A
normally functioning kidney
24
Q
megaureter vs refluxing ureter - quality
A
megaureter»_space; refluxing
25
4 indications for megaureter surgery
1. uti, 2. pain, 3. stones, 4. progression
26
pathophysiology of obstructed megaureter
aperistaltic DISTAL segment of ureter
27
what makes distal ureter aeristaltic in megaureter
circular fibers on only
28
3 steps in mgmt of refluxing megaureter
1. prophylaxis during observation (1yr), 2. observation for 1st yr, 3. surgery
29
what kindof megaureter gets cutaneous ureterostomy
refluxing with recurrent pyelo
30
how to taper megaureter
excision of lateral wall to preserve medial blood supply
31
what ureter gets taper vs tailor
taper for ureter > 1.5 cm
32
mgmt of moderate VUR after megaureter surgery
1. r/o bladder problem, 2. consider surgical correction (deflux vs re-reimplant)
33
2 major complications of megaureter surgery
1. obstruction, 2. VUR
34
2 causes of obstruciton after megaureter surgery
1. edema, 2. ischemia
35
mgmt of obstruction after megaureter surgery - 2
1. postop edema - transient and resolves w/in 8 wks. may need stent, 2. related to ischemia of ureter - resect ischemic ureter and reimplant
36
mgmt of VUR after megaureter surg
r/o bladder dysfunction, consider surgical repair - deflux vs reimplant
37
6 genetic causes of renal cystic disease
1. ARPKD, 2. ADPKD, 3. medullary cystic disease, 4. congenital nephrosis, 5. familial hypoplastic glomerulocystic disease, 6. multiple malformation syndromes (VHL, TS, ect)
38
7 non-genetic cuses of renal cystic disease
1. MCDK, 2. benign multilocular cyst (cystic nephroma), 3. simple cyst, 4. medullary sponge kidney, 5. acquired renal cystic disease, 6. sporadic glomerulocystic kidney disease, 7. calyceal diverticulum
39
ADPKD vs ARPKD - liver
AD - liver cysts later in life, AR - liver fibrosis
40
ADPKD vs ARPKD - RCC
no increased incidence
41
ADPKD vs ARPKD - aneurism
ADPKD only - berry aneurism 10-30%
42
ARPKD and development
oligohydramnios common, pulmonary hypoplasia --> incompatible with life
43
2 contralateral findings in MCDK
1. UPJO (3-12%), 2. VUR (30%)
44
natural history of MCDK
invoute, no assn with HTN
45
histology of MCDK
heterogenious non-communicating cysts
46
most common type of posterior urethral valves
type 1
47
% with VUR at time of PUV dx
50-70%
48
outcome of VUR after PUV ablation
30% resolve
49
findings with PUV in utero
bilateral hydro and bladder distention
50
fetal urine sampling significance
hypertonic urine is poor prognosis
51
ascites in PUV due to
40% due to urologic condition
52
nadir serum creatinine predicting ESRD in newborn
> 1
53
4 predictos of poor renal function
1. renal dysplasia on us, 2. presentation < 1 yo, 3. bilateral VUR, 4. nadir cr > 1.0 in 1st yr of life
54
where on valves is ablation done
at 5 and 7 oclock
55
result of complete valve ablation
high risk of urethral and sphincter injuries with complete resection
56
3 major findings in VCUG of kid with PUV
1. trabeculated bladder, 2. hypertrophied bladder neck, 3. dilated and elongated posterior urethra
57
when to remove dysplastic kidney with high grade VUR inpt with PUV
only if recurrent infection. if no infection, leave it alone
58
if PUV ablation cant be done because urethra is too small
temporary vesicostomy
59
what does VURD stand for
valves, unilateral reflux, renal dysplasia
60
significance of VURD
reflux happens into dysplastic kidney functioning as pop-off valve and protecting other kidney
61
common bladder problem after PUV ablation
persistent VUR
62
mgmt of persistent VUR after PUV ablation
make sure bladder function is nl, otherwise make sure bladder is rehabilitated before re-implanting
63
non-refluxing hydronephrosis in PUV after ablation
1/2 resolve after ablation, if persistent make sure bladder ok
64
3 outcomes of PUV bladder
1. decreased compliance/small capacity, 2. DO, 3. myogenic failure
65
3 outcomes of PUV bladder and age of presentation
1. decreased compliance/small capacity (infants), 2. DO (older kids), 3. myogenic failure (post pubertal)
66
3 outcomes of PUV bladder and mgmt
1. decreased compliance/small capacity (anticholinergics) 2. DO (anticholinergics), 3. myogenic failure (double void, alpha blockers, +/-CIC)
67
3 predictors of poor outcome in PUV
1. nadir cr > 1 at 1 yo 2. renal dysplasia, 3. younger age at presentation
68
transplant outcome in PUV depends on
good bladder function
69
how is renal function maximized in PUV
good bladder control
70
what is weigert myer rule
upper pole oriface is caudal to lower pole oriface
71
reflux and obstruction in duplication
upper pole obstruction, lower pole reflux
72
most common location of insertion in males for ectopic ureter
posterior urethra
73
most common location of insertion in females for ectopic ureter
bladder neck --> incontinence if below sphincter
74
cause of ureterocele
failure of chwalla membrane to rupture in distal ureter
75
% ureterocele with reflux - ipsilateral/ contralateral
70% / 10%
76
vcug finding suggestive of duplication
drooping lilly of lower pole kidney
77
3 goals in treatment of duplication
1. preserve renal function, 2. prevent uti 3. maintain continence
78
ureterocele mgmt if sepsis
incise asap
79
ureterocele mgmt if it extends down bladder neck
incise below bladder neck to prevent flap-valve
80
ureterocele caveat with family
let family know all mgmt options may require additional future surgery
81
ectopic ureter - 3 mgmt options
1. UU or pyeloureterostomy, 2. upper pole hemi-nx, 3. common sheath reimplant
82
when to do UU/PU in ectopic ureter
when there is no ipsilateral reflux
83
when to do upper pole heminx in ectopic ureter
no upper pole function
84
when to do common sheath reimplant in ectopic ureter
if ipsilateral or contralateral reflux is present
85
prune belly syndrome aka
eagle-barrett
86
triad of prune belly
1. bilateral hydro, 2. bilateral intra-abdominal cryptorchidism, 3. abdominal wall laxity
87
4 associated urologic findings in prune belly
1. renal dysplasia (50%), 2. large bladder +/- urachal diverticulum, 3. dilated posterior urethra (from prostatic hypoplasia not valves), 4. megalourethra
88
biggest urologic problem in PBS
urine stasis
89
early orchiopexy in PBS?
by 6 mo
90
what is megacystis-megaureter
huge bladder with megaureter only, no other physical findings
91
mgmt of megacystis-megaureter
reimplant
92
where in the kidney does scarring usually happen after pyelo
polar regions of kidney
93
fetal ureter bud forms when
5th wk
94
when can fetal kidneys be visualixed
12-13 wk
95
when can fetal bladder be seen
14th wk
96
when is amniotic fluid volume dependent on kidneys
16th wk
97
what diameter (mm) is fetal hydro mild, moderate, severe
mild 7-9mm, moderate 9- 15 mm, severe > 15 mm
98
what makes hydro more likely to be pathological - 5
1. calyceal dilation, 2. ureteral dilation, 3. chromosomal abnormalities, 4. multiple malformations, 5. oligohydramios
99
% of the time is hydro transient
40-80%
100
elevated urine N-acetyl-β-D-glucosaminidase found in what condition
obstructed kidney
101
elevated urine TGF-β1 found in what condition
unilateral UPJO
102
hypothesized etiology of posterior urethral valve
terminal ends of wolfian ducts mismigrate and are integrated into urethral wall abnormally resulting in obliquely oriented ridges that act as one way alves
103
who gets admitted for pyelo for iv abx
1. pyelo in infants and "young kids", 2. renal abscess, 3. urosepsis
104
when does a UTI always get admitted for IV abx
always in infants < 1 mo old
105
when not to give bactrim or nitrofurantoin
bactrim < 2 mo, nitrofurantoin < 1 mo
106
options for antibiotic prophylaxis - 4
1. nitrofurantoin, 2. bactrim, 3. keflex, 4. amoxicillin
107
risk of VUR if parent, sibling, twin have it
parent 50%, sibling 50%, twin 80%
108
spontaneous resolution rate for grade 1 VUR
90%
109
spontaneous resolution rate for grade 2 VUR
60-80%
110
spontaneous resolution rate for grade 3 VUR
50%
111
spontaneous resolution rate for grade 4 VUR
25-40%
112
spontaneous resolution rate for grade 5 VUR
<20%
113
before observing grade 1-2 VUR without abx, need to confirm what
normal bladder/bowel function
114
most common renal function abnormality associated with PUV
urine concentration defect
