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Transcript
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Martha L. Decker, MD
Maternal Fetal Medicine
Wake Forest University
Fetal skin allows transudation of plasma into amniotic sac
ƒ Urine produced as early as 9 weeks gestation
ƒ Kidneys do not contribute significantly to amniotic fluid volume prior to 16 weeks gestation
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7 weeks gestation
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Kidneys
Ureters
Bladder
Urethra
Adrenal glands
Internal and external genitalia
Normal kidneys are identifiable transabdominally in most fetuses by 20 weeks gestation (as early as 10‐12 wks)
Normal kidneys generally span 4‐5 vertebral lkd
ll
b l
bodies
Length of normal kidney in mm is similar to gestational age in weeks
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Pyelectasis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
Absent kidneys Duplicated collecting system
Progesterone effect on the ureter leads to inefficient removal of urine from pelvis and to vesicoureteral reflux due to poor tone in ureter
Genetic disorder
Early UPJ obstruction or pathological reflux
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Graupe et al. Clinics in PerinatologyVolume 28 • Number 2 • June 2001
210 fetuses with pyelectasis (>=4mm dilatation in AP plane in 2nd trimester)
Likelihood ratio of 1.3 for trisomy 21
Pyelectasis, in conjunction with other clinical l
i
j
i
i h h li i l
or sonographic parameters, warrants consideration for amniocentesis
Benacerraf et al. Obstet Gynecol 76:58, 1990
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AP measurement:
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ƒ < 4 mm – normal
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ƒ GA < 33 weeks, 4 – 9 mm – follow‐up after 33 ƒ
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weeks
ƒ GA > 33 weeks, 7 – 9 mm – notify pediatricians
ƒ ~ 10 mm – most likely a structural abnormality –
follow with serial ultrasounds
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Pyelectasis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
Absent kidneys
Duplicated collecting system
UVJ obstruction
Ureterocele
Urethral obstruction
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Fetal bladder fills and empties every 30‐155 minutes
Fetal bladder wall not normally seen when normal bladder is distended
l bl dd
d
d d
Normal ureters not visible
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Bladder outlet obstruction
ƒ Posterior urethral valves (in male)
ƒ Urethral atresia
ƒ Other –
Other like ureteroceles
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Sonographic findings:
ƒ Bilateral hydronephrosis
ƒ Hydroureter
ƒ Distended bladder
ƒ Oligohydramnios
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ƒ Multicystic dysplastic kidney
ƒ Cystic dysplasia
ƒ Autosomal recessive polycystic kidney disease (ARPKD)
ƒ Autosomal dominant polycystic kidney disease (ADPKD)
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Dilatation of the renal pelvis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
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kd
Absent kidneys Duplicated collecting system
End result of early (first trimester) urinary tract obstruction
70‐80% unilateral
Cysts of varying size
f
No communication between cysts, no uniformity in configuration
Reniform contour is lost
Non‐functioning
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Occurs from obstructive uropathy in 2nd and 3rd
trimester
Common causes include
ƒ UPJ obstruction
ƒ Bladder outlet obstruction:
Bladder outlet obstruction
▪ Urethral atresia
▪ PUV
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Results in irreversible renal damage
Presence of cysts associated with dysplasia ie. echogenicity Usually few small cysts with small, bright kidney Right ureteral obstruction
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Autosomal recessive; gene locus 6p
Symmetrical enlargement of both kidneys with innumerable 1‐2 mm cysts
May have hepatic involvement
M h h
ti i
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Diagnosable by 24 weeks
Broad biologic variability, may not present until postnatal period
Sonographic findings progress with advancing gestational age
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Gene mutation (PKD1) in 85% of affected European families on chromosome 16
y p
p
Severity of parental disease not predictive of offspring disease
Cystic echogenic kidneys with normal amniotic fluid volume
Systemic disorder: liver/pancreas/intracranial aneurysms/colonic diverticulae
Difficult to differentiate from ARPKD
Assess family history
Renal ultrasound of both parents
R l lt
d f b th t
ƒ If normal, consider ARPKD
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Dilatation of the renal pelvis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
h
kd
Absent kidneys
Duplicated collecting system
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Differential diagnosis:
ƒ Normal variant
ƒ Cystic dysplastic renal disease
ƒ Finnish nephrosis
ƒ Meckel‐Gruber syndrome
ƒ Trisomy 13
ƒ Beckwith‐Wiedemann syndrome
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Dilatation of the renal pelvis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
h
kd
Absent kidneys
Duplicated collecting system
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M:F ratio 2.5:1
100% mortality
Sonographic findings:
ƒ Oligo/anhydramnios
ƒ Bladder not visible
ƒ Kidneys not visible
ƒ Other anomalies common
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Dilatation of the renal pelvis
Hydronephrosis
Cystic kidneys
Echogenic kidneys
Absent kidneys
Duplicated collecting system
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Kidneys
Ureters
Bladder
Urethra
h
Adrenal glands
Internal and external genitalia
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Kidneys
Ureters
Bladder
Urethra
Adrenal glands
Internal and external genitalia
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