Module 9: Fetal GI Pathology Flashcards
The ureteric buds become what? 4 (Anatomy)
- Ureters
- Renal Pelvis
- Calyces
- Collecting tubules
The metanephros gives origin to what?
Nephrons and the glomeruli and loop of henle
Fetal urine production begins around what week?
11 LMP
Early on, the primitive kidney (metanephros) is located where?
In the pelvis
The kidneys migrate where later on? What happens if this does not happen? 2
- To their normal paraspinal position
- Ectopic pregnancy
The cloaca is divided into what two parts? 2
- Urinary
- Rectal segments
The caudal end of the cloaca forms what? 2
- The urethra and bladder neck.
- The rest of the bladder is derived from the Allantois which ends at the urachal
The mesonephric ducts (ureters) enters what?
Posterior wall of the bladder, at the trigone
Prior to 8 weeks LMP< the fetal external genitals are in what state?
Ambiguous state
At 8-10 weeks LMP, with the presence of hormones (testosterone) what are formed?
Male genitalia is formed
What happens when male genitalia is formed?
Wolffian ducts grow and Müllerian ducts are inhibited
In females, with no Male hormones, the normal female genes develop due to what?
Maternal estrogen
What happens when female genitalia develop?
Wolffian ducts regress and Müllerian ducts are stimulated
Fetal kidneys are routinely imaged in what view?
Trans
If pathology is detected in fetal trans, what happens?
Sag views are included
What is the normal kidney length of the fetus?
Equal to the gestational age
20 weeks = 20mm
Mild fetal renal pelvis dilation can be up to what? 2
- 5mm up to 20 weeks gestation
- 8mm from 20 weeks to term
Bilateral renal agenesis is associated with what other anomalies? 5
- Other GU abnormalities
- GI abnormalities
- Cardiac abnormalities
- Sirenomelia (mermaid syndrome)
- Teratogens (Warfarin, Cocaine, maternal diabetes type 1)
Unilateral renal agenesis may be what? (Asymptomatic or symptomatic)
Asymptomatic and not noticed in utero
What is seen (or not seen) with bilateral renal agenesis? 5
- Absent kidneys
- Absent bladder
- Oligiohydramnios- anhydraminos
- Small abdominal circumference
- IUGR
With bilateral renal agenesis, adrenals assume what kind of shape?
Discoid shape and can be mistaken for kidneys
What position does the adrenal glands look like with agenesis of the kidneys?
Lying down
What should we do with a sonographic assessment with agenesis of the kidneys? 5
- Confirm presence or absence of kidneys
- Look for anechoic renal pelvises
- Look for renal cortex
- Use colour doppler to confirm presence of renal arteries
- Assess amniotic fluid
What does Potter’s syndrome refer to?
Bilateral renal agenesis
Potter’s sequence refers to what? (consequence)
The consequence of severe oligiohydramnios
What is the indicators of potter’s syndrome? 3
- Pulmonary hypoplasia
- Abnormal hand and foot positions
- Facial anomalies
What are abnormal of Potter’s syndrome? 3 (legs and hips)
- Bowed legs
- Clubbed feet
- Hip dislocation
With potter’s syndrome, malformations are caused by what?
Oligiohydramnios
Causes of oligiohydramnios are from what? 3
- Renal abnormalities
- Amniotic fluid leakage
- Placental abnormalities
What are some renal abnormalities that cause oligiohydramnios? 3
- Renal agenesis
- Multi cystic dysplastic kidneys
- Genitourinary obstruction
Oligiohydramnios impairs normal development in what? 3
- Extremities
- Facial features
- Lungs (pulmonary hypoplasia)
What are some facial features/ anomalies that appear in Potter’s sequence? 4
- Beaked nose
- Low set ears
- Prominent epicanthic folds
- Hypertelorism
What is hydronephrosis?
Distension of the renal pelvis and calyces with urine due to an obstruction
What percentage of renal abnormalities are hydronephrosis?
75%
What percentage of hydronephrosis is associated with syndromes?
20%
Hydronephrosis is typically bilateral or unilateral?
Typically unilateral
What are causes of hydronephrosis? 3
- Obstruction
- Reflux
- Ureterocele
What are causes of hydronephrosis at 3 possible levels?
- At the level of the renal pelvis (or along the ureter)
- At the level of the ureter insertion in to the bladder
- At the level of the bladder outlet/ urethra
What is reflux in terms of hydronephrosis?
Ureterovesical valves are not working properly
Ureterocole in terms of hydronephrosis is what?
Ectopic placement of the valves
What do we need to do to interrogate hydronephrosis? 4
- Determine if bilateral or unilateral
- Determine degree of hydronephrosis
- Determine level of obstruction
- Evaluate for other abnormalities
What are some things we see when determining level of obstruction in terms of hydronephrosis? 3
- Hydronephrosis only/ Only kidney are dilated
- Dilated ureter with hydronephrosis
- Dilated bladder and keyboard appearance
If we see hydronephrosis only/ only kidneys are dilated where do we see the obstruction?
- Ureteropelvic junction
- UPJ obstruction
If we see dilated ureters with hydronephrosis where do we see the obstructions?
- Ureterovesical junction
- UVJ
If we see dilated bladder and keyhole appearance, where is the obstruction?
- Urethral junction obstruction
- PUV - posterior urethra valve obstruction
What is the most common obstruction?
UPJ
Where are UPJ obstructions, most common seen??
At the level of the renal pelvis
If a ureteropelvic junction obstruction is unilateral what would we see?
Normal bladder and amniotic fluid
If bilateral what do we see with ureteropelvic junction obstruction? 2
- Oligiohydramnios
- Hydronephrosis of varying degrees
What is seen with severe UPJ obstruction?
Renal parenchyma may be destroyed
What are some pitfalls of determining UPJ obstructions? 3
- Be aware of the extra renal pelvis
- Prominent renal veins
- Reflux
Why should we be aware of the extra renal pelvis? 3
- Renal pelvis outside
- May mimic a small hydronephrosis
- Dilated calyces indicated hydronephrosis over extra renal pelvis
In terms of UPC, What do we do with the prominent renal viens?
Check with colour doppler
What do we see with reflux? 2
- Urine is moveing from the bladder up the ureter into the kidney
- This is transient meaning the kidney dilation
Where do UVJ obstructions insert. What is Ureterovesical obstruction due to?
- At the level of the ureter insertion into bladder
- Usually due to duplicated collection
What is a duplex kidney?
Upper pole ureter inserts Inferiorly in the bladder and will have a ureterocele protrued’
What is a ureterocele?
Cystic dilation of the intravesicular portion of the ureter
Ureteroceles are associated with dilation of which side of the kidney in a duplicated collecting system?
Upper pole
What does UVJ obstruction look like on u/s?
Dilated upper pole of the kidney with a dilate tourtous
With the UVJ, the ureter may mimic what? 2 why?
Bowel
1. Bowel is medial in the abdomen
2. Ureter would be lateral in the abdomen
The ectopic ureter is associated with what?
Ureterocele in bladder
Duplex kidney with hydronephrosis affects what pole?
Upper pole
What is UVJ obstruction associated with ?
Ureterocele
What is the abbreaviation of posterior urethra valves?
PUV
What is a PUV?
Obstructive membranes in the posterior urethra that obstruct the flow of urine at the level of the bladder outlet/ Urethra
What does a keyhole bladder look like? 3
- Large, distended bladder
- Dilated proximal urethra
- Bladder wall thickness
What symptoms do we see with PUV? 2
- Oligiohydramnios
- +/- Hydroneprhosis and hydroureter
Who is most likely affected by PUVs?
Males typically
PUV can be a precursor to what?
Prune belly syndrome (eagle- Barrett syndrome)
PUVs have a risk of what? What does this lead to?
- Bladder or renal forniceal rupture
- Ascites
What do we see with prune belly syndrome? 5 (3 main)
- Undescended testicles
- Very Large bladder, with abdominal distension
- Lack of abdominal musculature
- Ureters may be tortuous and dilated
- Kidneys can be normal, hydronephrotic, dysplastic
What are three types of renal cystic disease?
- Infantile Polycystic disease (ARPKD)
- Adult Polycystic disease (ADPKD)
- Multi cystic dysplastic kidney
Infantile Polycystic kidney disease is what kind of disease?
Autosomal recessive disorder (ARPKD)
What is the primary defect of Infantile Polycystic kidney disease? (what is primarily affected)
Collecting ducts (cortex)
What is seen with IPKD ARPKD? 5
- Multiple small cysts under 1-2 mm
- Grossly enlarged echogenic kidneys, but retain form
- Always bilateral
- Severe oligiohydramnios
- Absent bladder
What is the diagnostic criteria for IPKD- ARPKD? 3
- Bilateral enlarged echogenic kidneys
- Oligiohydramnios
- Absent bladder
What are four types of ARPKD based on the onset of the disease?
- Perinatal
- Neonatal
- Infantile
- Juvenile
IPKD is associated with disorders? 3
- Meckal- Bruner syndrome
- Robert’s syndrome
- T13
What is the DDX for IPKD? 3 (Infantile polycystic kidney disease)
Benign glomeruloscelerosis
1. Large
2. Echogenic kidneys but echopenic pyramids
3. Normal fluid
What kind of disorder is Adult Polycystic kidney disease?
Autosomal dominant disorder (ADPKD)
How common is ADPKD seen?
Rarely seen in antenatal sonography, however fetus assessed if a parent has the disease and vice versa if a parent has the disease
What is seen with APKD? 4
- Enlarged kidneys with cysts
- May be hyperechoic
- Bilateral - but ma be more prominent in one kidney
- Fetal bladder is present
What is the amniotic fluid like in APKD?
Normal
What are multicystic dysplastic kidneys?
Multiple cystic lesions which due not communicate
With MCDK, kidneys are shaped how?
Kidneys lose their reniform shape
In terms of MCDK, unilateral has what kind of prognosis? Why?
- Good prognosis
- Compensatory hypertrophy is often seen in unaffected kidney, and affected kidney is left alone
MCDK are rarely what?
Bilateral, however if so fatal due to lack of amniotic fluid.
Bilateral MCDK is associated with what anomaly?
Potter’s sequence
Is there a genetic predisposition of MCDK?
No
What would poor prognosis look like for GU? 6
- Bilateral renal agenesis
- Increased renal echogenicity
- Bilateral hydronephrosis
- Renal cysts (MCDK)
- Bladder obstruction
- Oligiohydramnios
What is the hydroneprhosis prognosis? 3
Depends on the timing of diagnosis
1. first trimester: severe pathology, poor outcome
2. 2nd trimester: most improve or resolve completely
3. 3rd trimester: higher incidence of post natal pathology requiring surgical intervention
What is the most common congenital renal tumor in utero?
Mesobalstic nephroma
IS mesoblastic nephroma benign or malignant?
Benign
What kind of mass is mesoblastic nephroma?
Solitary, solid mass arising from kidney area.
Mesoblastic nephroma may cause what to happen in terms of fluid?
Polyhydraminos
What kind of tumor is a neprhoblastoma? 3 (solid/ cystic, malignancy, occurance in utero)
- Solid mass
- Malignant
- Not commonly identified in utero
What is the most common abdominal tumor found in newborns?
Neuroblastoma s
What kind of mass is a neuroblastoma? 2 (location and misc)
- Mixed cystic/ solid mass near upper pole of kidney
- Calcifications may be present
What is the malignancy of neuroblastoma? 50% of individuals develop what at birth? Mortality depends on what?
- Malignant
- 50% have METS at birth
- Mortality depends on age at diagnosis and stage
Ambiguous genitalia may be associated with what? 2 (usually are what?)
- Adrenal hyperplasia or maternal ingestion of male hormones early in pregnancy
- Usually XX fetuses
Ambiguous genitalia may not be discovered until when? What happens then?
- The 16th week LMP
- By which time masculinization of affected female has already occurred
What is prenatal treatment of ambiguous genitalia?
Prenatal treatment includes steroid administration to the mother from 10 weeks LMP to delivery
What are X linked syndromes? 2
- Turners or Noonans’
- Hemophilia
What are hydrometrocolops?
Secretions in uterus and vagina
What is the occurrence rate of ovarian cysts?
- Rare
What is the prognosis of ovarian cysts?
Benign
What does ovarian cysts look like? How do they appear?
- Unilateral more common that bilateral
- Range in size from small cysts to structure filling the entire abdomen
- Cystic mass separate from urinary tract
What is this?
Fetal kidneys
Label
Urine filled renal pelvises
What is this?
Fetal kidneys
What is this?
Fetal kidneys in sag
Label
Kidneys and adrenals
What is the significance of this image?
Fetal bladder
What is going on here?
Sag Rt adrenals with agenesis NOT kidneys
Significance?
Missing kidneys
What does this mean?
Normal Renal Arteries
What’s going on here?
Normal and abnormal renal arteries
What’s happening here?
Unilateral Agenesis of the kidney
What does this image represent?
Unilateral agenesis of the kidney
What does this image represent?
Bilateral renal agenesis
What does this image represent?
Potter’s sequence
What does this image represent?
What’s going on here?
Unilateral hydronephrosis
What’s going on here?
Bilateral UPJ obstruction
What is going on here?
UVJ obstruction
What is this?
UVJ obstruction
What’s going on here?
Urethral obstruction
What’s going on here?
PUV obstruction
What’s going on here?
PUV obstruction
What’s going on here?
Prune belly syndrome
What’s going on here?
Prune belly syndrome
What’s going on here?
Infantile polycystic kidney disease
What’s going on here?
APKD
What’s going on here?
MCDK
What’s going on here?
Right multicystic dysplastic kidney
What does this image represent?
Mesoblastic Nephroma
What does this image represent?
Hydrocele and ovarian cyst