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Fetal Genitourinary System Flashcards

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1
Q

What are the sonographic characteristics of a normal fetal genitourinary system?

Normal Kidneys
A

Kidneys identified as early as 12-14 weeks gestation; echo-poor renal pyramids; small amount of fluid in renal pelvis; thin echogenic renal capsule.

Normal renal anatomy includes the renal sinus, renal pelvis, and renal pyramids.

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2
Q

What are the age-related renal pelvis measurements for normal fetal kidneys?

A

* **Normal:** 13 – 20 weeks: 5 mm
* **Probably normal,follow up:** 20 – 30 weeks: 8 mm
* **85% have anatomic anomaly:** > 30 weeks: 10 mm

## Footnote

Measurements help assess the risk of anatomical anomalies.

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3
Q

What are key sonographic features of the fetal urinary bladder?

A
  • Routinely visualized by 20 weeks, indicating renal function
  • Fills and empties in 30–45 minute cycles
  • Iliac arteries course laterally to the bladder

Identification of the bladder indicates renal function in the fetus.

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4
Q

How do fetal adrenal glands appear on ultrasound?

DO NOT mistake for kidneys. It can be difficult to differentiate when oligohydramnios due to agenesis of the kidney
A
  • Relatively large in the fetus
  • Seen superior to the kidneys on sagittal scans
  • Transverse view: appear as long, thin echogenic lines (medulla)
  • Surrounded by thicker sonolucent rims (cortex)
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5
Q

Why is evaluating fetal genitalia important on ultrasound?

A
  • Assists in differential diagnosis of genitourinary anomalies and/or chromosomal syndromes
  • Sex can be determined in virtually all normal pregnancies
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6
Q

What are the categories and examples of genitourinary abnormalities in fetal development?

A

Renal anomalies:
* Unilateral renal agenesis
* Bilateral renal agenesis (Potter sequence)

Excretory abnormalities:
* Renal ectopia
* Renal cystic disease

Obstructive uropathies:
* UPJ (ureteropelvic junction obstruction)
* UVJ (ureterovesical junction obstruction)
* PUV (posterior urethral valves)
Genitourinary neoplasms

Reproductive anomalies:
* Genital agenesis or dysmorphia
* Wolffian or Müllerian duct anomalies

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7
Q

What is renal agenesis and what are its key features?

A

Congenital absence of one or both kidneys
* Etiology: Unknown; likely multifactorial
* Cause: Failure of the metanephros to develop

Prevalence:
* Unilateral renal agenesis is 3–4 times more common than bilateral

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8
Q

What are the associated abnormalities of unilateral renal agenesis?

A
  • Trisomy 21 (Down Syndrome)
  • Turner Syndrome
  • Potter Syndrome/Sequence
  • Müllerian duct anomalies
  • Congenital heart disease

Obstructive uropathies:
* Posterior urethral valve (PUV) obstruction
* Ureterovesical junction (UVJ) obstruction
* Ureteropelvic junction (UPJ) obstruction

VACTERL association (vertebral, anal, cardiac, tracheoesophageal, renal, limb anomalies)
Sirenomelia (mermaid syndrome)

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9
Q

What are the sonographic findings for unilateral renal agenesis?

Arrow pointing to the adrenal gland
A
  • Absence of kidneys
  • Compensatory hypertrophy of the contralateral kidney
  • Normal filling of the urinary bladder
  • Normal amount of amniotic fluid
Hypertrophy of the other kidney

Hypertrophy may also be observed in the ipsilateral adrenal gland.

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10
Q

What is bilateral renal agenesis, and what are the key consequences?

A
  • A uniformly lethal condition due to absence of both kidneys
  • Results in no urine production and dramatically diminished amniotic fluid
  • May occur in isolation or as part of a syndrome
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11
Q

What are associated abnormalities seen with bilateral renal agenesis?

A
  • Potter sequence
  • Genital malformations
  • Sirenomelia (mermaid syndrome)
  • Cardiac anomalies (full range)

CNS anomalies:
* Hydrocephalus
* Microcephaly
* Meningocele
* Holoprosencephaly
* Iniencephaly

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12
Q

What gastrointestinal malformations are associated with bilateral renal agenesis?

A
  • Duodenal atresia
  • Imperforate anus
  • Tracheoesophageal fistula
  • Omphalocele
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13
Q

What are the key sonographic findings of bilateral renal agenesis?

A
  • Empty renal fossae bilaterally
  • Oligohydramnios
  • Long, thin “lying-down” adrenal glands
  • Absent renal arteries (on color Doppler)
  • Absent urinary bladder
  • Compression deformities from extreme oligohydramnios
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14
Q

What is Potter sequence and what causes it?

A
  • A constellation of abnormal facies, IUGR, and limb anomalies
  • Caused by chronic compression from severe oligohydramnios
  • Often linked to renal anomalies
  • May be caused by PROM or chronic leakage of amniotic fluid (AF)
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15
Q

What are the clinical findings associated with Potter Sequence?

A
  • Pulmonary hypoplasia (severe and incompatible with life)
  • Abnormal facies (Potter facies)
  • Low-set ears
  • Flattened nose
  • Wrinkled skin
  • Micrognathia

Intrauterine growth restriction (IUGR)
Limb abnormalities such as clubfoot (talipes equinovarus) and contractures

Other associations include obstructive uropathies and VACTERL association.

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16
Q

What is renal ectopia, and where are ectopic kidneys commonly located?

A
  • Renal ectopia is the presence of a kidney outside its usual location in the renal fossa

Most common locations include:
* Abdominal
* Pelvic
* Lumbar
* Thoracic

Occurs with a 2:1 male-to-female ratio

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17
Q

What are associated abnormalities with renal ectopia?

A
  • Multicystic dysplastic kidney
  • Ureterocele
  • Vesicoureteral reflux
  • Genital abnormalities
  • UPJ (ureteropelvic junction) obstructions
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18
Q

What is Potter Type Iand what is it also known as?

A
  • Potter Type I is Autosomal Recessive Polycystic Kidney Disease (ARPKD)
  • Also known as Infantile Polycystic Kidney Disease
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19
Q

What are some associated abnormalities with ARPKD?

A
  • Beckwith-Wiedemann syndrome
  • Meckel-Gruber syndrome
  • Trisomy 13 (Patau syndrome)
  • Glomerulocystic disease
  • Congenital hypernephroid nephromegaly
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20
Q

What are the clinical characteristics of Potter Type I (ARPKD)?

A
  • Bilateral, enlarged, and echogenic kidneys
  • Poor prognosis
  • 25% risk in subsequent pregnancies
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21
Q

What are the sonographic findings of ARPKD?

Study These Flashcards
A
  • Large and homogeneously hyperechoic kidneys
  • Increased kidney-to-abdominal circumference ratio
  • Loss of corticomedullary differentiation
  • Kidneys may appear normal early in pregnancy (14–16 weeks)
  • Presence or absence of fetal urinary bladder
  • Oligohydramnios after 20 weeks, though fluid may be normal earlier
22
Q

What is Potter Type II, and what is it also called?

Study These Flashcards
A
  • Potter Type II is Multicystic Renal Dysplasia, also known as Multicystic Dysplastic Kidney Disease (MCDK)
  • Can be unilateral or bilateral
  • Characterized by noncommunicating cysts of varying size
  • Generally has a good prognosis
23
Q

What are associated abnormalities with Multicystic Renal Dysplasia?

Study These Flashcards
A
  • Vesicoureteral reflux
  • Ureteropelvic junction obstruction
  • Ureterocele
  • Meckel-Gruber syndrome
  • Zellweger syndrome
24
Q

What causes the formation of a Multicystic Dysplastic Kidney (MCDK)?

Study These Flashcards
A

Obstruction in the first trimester leads to:
* Diminished kidneys
* Formation of randomly positioned cysts of varying sizes in the kidney parenchyma
* Increased echogenicity of the kidney parenchyma

25
What are the sonographic findings of Multicystic Renal Dysplasia?
* **Multiple noncommunicating cysts** * **Lobulated renal contour** * **Echogenic renal parenchyma** * **Urinary bladder** may be **present or absent** * **Oligohydramnios** if **renal function is impaired**
26
What is **Potter Type III**, and what is its inheritance pattern?
* **Potter Type III** is **Autosomal Dominant Polycystic Kidney Disease (ADPKD)** * Also called **adult polycystic kidney disease**
28
What are the clinical and sonographic features of ADPKD?
* **Does not manifest until adulthood** * **No in utero-associated abnormalities or sonographic findings** * **Very rare to diagnose in utero**
30
What is the inheritance risk of ADPKD (Potter Type III)?
* Inherited in an **autosomal dominant** manner * **50% risk** in **subsequent generations**
31
What is **Potter Type IV**, and what causes it?
* Also known as **Obstructive Cystic Renal Dysplasia** * Caused by **dysplastic cysts** in the **renal parenchyma** due to **chronic urinary outflow obstruction** * Often presents with **hydronephrosis with cortical cysts**
32
What are the associated abnormalities with Potter Type IV?
* **Posterior urethral valves** * **Duplex collecting system** * **Ureterovesical obstruction** * **VACTERL association** * **Congenital heart disease** * **CNS abnormalities** * **Gastrointestinal abnormalities**
33
What are the sonographic findings of Obstructive Cystic Renal Dysplasia?
* **Scattered, noncommunicating cysts** * **Echogenic renal cortex** **Evidence of obstructive uropathy** on the affected side: * **Dilated renal pelvis** * **Dilated collecting system** * **Dilated ureter** * **Bladder** may or may not be **dilated**, depending on **obstruction site**
34
What are the key characteristics of **obstructive uropathies**?
* Can **vary in location, severity, and chronicity** * **Unilateral** obstructions are **rarely associated with clinical sequelae** * **Bilateral** obstructions may cause **renal dysplasia, renal failure, or death** * **Sonographic appearance** depends on the **location and severity**
35
How are **obstructive uropathies** categorized by **level of obstruction**?
**Kidney:** * Hydronephrosis **Ureter:** * **Ureteropelvic junction (UPJ) obstruction** * Ectopic ureterocele * Congenital primary megaureter * Duplicated collecting system **Urethra:** * Bladder outlet obstruction * **Posterior urethral valves (PUV)** * Urethral atresia/stenosis * Prune Belly syndrome * **Ureterovesical Junction Obstruction (UVJ)**
36
What is hydronephrosis and how does it appear sonographically?
* **Dilatation of the renal collecting system** * **Dilated renal pelvis** is observed * **Variable caliectasis** may be present * In chronic cases, there is **thinning of the renal cortex** * There is **no associated hydroureter**
37
What are the renal pelvis diameter thresholds for diagnosing hydronephrosis in the 2nd and 3rd trimesters?
38
What is **Ureteropelvic Junction (UPJ)** Obstruction, and what does it lead to?
* Obstruction occurs at the **junction of the renal pelvis and ureter** * It is the **most common cause of hydronephrosis** * A **complete obstruction** can lead to **massive hydronephrosis** and **renal dysplasia (dysfunction)**
39
What is **Ureterovesical Junction (UVJ)** Obstruction, and what are its sonographic findings?
* Obstruction occurs at the **junction of the ureter and bladder** * **Hydronephrosis** and **hydroureter** are seen sonographically * Often associated with **duplicated renal anomalies** * The **abnormal ureter** may have a **stenotic opening** into the bladder and form a **ureterocele**, which appears as a **cystic structure** **within or adjacent to the bladder**
40
What is an **ectopic ureterocele**, and what does it cause?
* **Herniation of the distal ureter** into the **bladder** * Leads to **subsequent outflow obstruction**
41
What are the sonographic findings of an ectopic ureterocele?
* **Large, tortuous, fluid-filled ureter** filling the **abdomen** * **Cystic structure** projecting into the **bladder** * **Ipsilateral hydronephrosis**
42
What is congenital megaureter and what causes it?
* A **chronic dilatation of the ureter** * Caused by **abnormalities of the ureter itself**, **not due to distal obstruction**
43
What are the sonographic findings of congenital megaureter?
* **Large, tortuous ureter** filling the **fetal abdomen** * **Tapering of the dilated distal ureter** (**adynamic obstructive type**) * **Dilated distal ureter** at the **ureterovesical junction** * **Normal bladder filling and emptying**
44
What is renal duplication, and how common is it?
* A **complete or partial duplication** of the **renal collecting system** * Can be **unilateral or bilateral**
45
What are associated abnormalities seen with renal duplication?
* **Ectopic ureterocele** * **Vesicoureteral reflux** * **Hydronephrosis**
46
What are the sonographic findings of renal duplication?
* **Cystic dilatation of the upper-pole collecting system** * **Ipsilateral ureteral dilatation** down to the **bladder** * **Presence of ureterocele** (common) * **Nondilated lower-pole collecting system**
47
What are posterior urethral valves (PUV) and whom do they affect?
* A **bladder outlet obstruction** in **male fetuses** * Caused by **congenital folds in the urethra** acting as **valves**
48
What are the sonographic findings of PUV?
* **Massive distention** and **hypertrophy of the urinary bladder** * **Keyhole sign**: caused by **bladder distention** and a **dilated prostatic urethra** * **Hydroureter and/or hydronephrosis** in **90% of cases** * **Oligohydramnios** * Seen in **male fetuses only*
49
What is Prune Belly Syndrome and what are its key components?
Also called **Eagle-Barrett syndrome**, it includes: * **Anterior abdominal wall atrophy** **Genitourinary anomalies** such as: * **Megacystis** * **Megaureter** * **Dilated prostatic urethra** * **Undescended testes**
50
What are associated abnormalities with Prune Belly Syndrome?
* **Pulmonary hypoplasia** * **Trisomies 13 and 18** * **Congenital cardiac anomalies** * **Talipes equinovarus (clubfoot)**
51
What are the sonographic findings of Prune Belly Syndrome?
* **Hyperechoic renal parenchyma** * **Clubfoot** * **Dilated calyces** * **Dilated, tortuous ureters** * **Large, megacystic bladder**
52
What are the main types of genitourinary neoplasms detectable in utero?
**Congenital Mesoblastic Nephroma**: * **Rare renal tumor** * Appears as a **large, solid, well-circumscribed, highly vascular mass** * Can cause **cardiac overload** and **polyhydramnios** **Nephroblastoma (Wilms tumor)**: * The **most common kidney cancer** in **children** **Neuroblastoma**: * A **malignant tumor** typically found in the **adrenal gland** * Appears as an **echogenic, heterogeneous, suprarenal mass**
53
What are the sonographic findings of genitourinary neoplasms?
* **Solid mass** in the **renal fossa** * **Complex mass** with possible **cystic components** * **Hemorrhage** into the mass may vary echogenicity over time * **Distortion** of adjacent anatomy **Color Doppler** may show: * **Central feeding vessel** * **Diffuse vascularity** throughout the mass May be associated with **nonimmune hydrops fetalis** **Always turn color Doppler on!!**

OB 2 (16 decks)

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