Congenital Flashcards
What is a variant?
Non-pathological variation of anatomy
What is an anomaly?
Major or minor abnormality of anatomy that is more probable to interfere with function
What is an HCB?
Hypertrophied Column of Bertin
Which urinary tract variant consists of a double layer of cortex between medullary pyramids?
HCB
Where does a hypertrophied column of bertin indent the renal sinus and where is it located?
Indents Laterally
Located in the upper or middle portion of kidney
An HCB should not be greater than what measurement in any dimension?
> 3cm
A junctional parenchymal defect is:
- Due to what?
- How does it appear?
- Where is it seen more often?
Incomplete embryological fusion
Hyperechoic wedge that can be confused with scarring
Seen more on right only because we see the RT KID better
Where is an extra renal pelvis located?
Medial to the renal sinus
Patients with an extra renal pelvis may be more prone to what?
Hydronephrosis (that can decompress when put in prone position)
A dromedary hump involves which aspect of the left kidney?
Lateral aspect
What is important to visualize in order to differentiate a dromedary hump from a mass?
CM junction
What is fetal lobulation?
Infolding of the cortex with no thinning, scalloped contour
What percentage of adult kidneys have some persistent lobulation?
51%
What is renal sinus lipomatosis?
Excessive fatty infiltration of the renal sinus
What is the most common congenital anomaly of the UT?
Duplicated collecting system
What are the two kinds of duplicated collecting systems?
Complete and incomplete
With complete duplicated collecting systems the superior ureter will have what kind of insertion?
Ectopic bladder insertion
What happens with an incomplete duplicated collecting system?
Duplicated ureters join before inserting into the bladder
What is a duplicated collecting system associated with? (4)
UPJ obstruction, uterus didelphys, reflux, and ureterocele
What is a ureterocele?
Cystic dilatation of the terminal ureter which protrudes into the urinary bladder
What do patients with ureteroceles have a high incidence of?
UTI’s and obstruction
What is a horseshoe kidney?
Fusion of the kidneys at the lower poles
Horseshoe kidneys can lead to an increased risk for what?
Infection and stone formation
What is important to visualize in order to confirm the presence of a horseshoe kidney?
Renal tissue that connects the lower poles (Isthmus)
Why does an ectopic kidney occur?
Kidney fails to ascend in utero, typically located in the pelvis unilaterally
Is diffuse or focal compensatory hypertrophy typically seen?
Diffuse
What may an enlarged normal kidney be caused by?
Unilateral renal agenesis or nephrectomy
What is supernumerary kidney?
Very rare small extra kidney with its own blood supply
How is hypoplasia different than atrophy of the kidney?
Hypoplasia occurs from Day 1, whereas with atrophy the kidney begins normal and then ceases to function, thus atrophying
A true hypoplastic kidney is very rare and may be unilateral or bilateral. True or false?
TRUE
What are enlarged clubbed calyces of the kidney known as?
Congenital megacalices
What is a functional obstruction of the ureter due to distal aperistalsis?
Congenital megaureter
Who does congenital megaureter affect more and on what side?
And how does it appear on US?
Males, left
Fusiform dilation of distal third of ureter
What is a UPJ obstruction?
Ureteropelvic junction obstruction
What is the most common palpable mass of the neonatal abdomen?
UPJ obstruction
With UPJ obstruction there is increased incidence of which two things?
Contralateral MCKD and contralateral renal agenesis
What would you see sonographically with a UPJ obstruction?
Ballooning of renal pelvis
What can appear the same as an ectopic kidney although it isn’t congential? (And what is it due to?)
Ptosis of the kidney
Prolapse of kidney due to trauma that causes tearing
What is a developmental anomaly that is failure of regression of the renal vessels during ascent?
Multiple renal arteries
What bladder anomaly is more common in males and presents as a large anterior abdominal wall defect?
Exstrophy
What is it called when the urethra opens posteriorly, not centrally on the penis?
Hypospadia
What should you check for if your male patient has hypospadia?
Renal anomalies
What does bladder outlet obstruction cause?
Bilateral hydronephrosis
What are potential causes of bladder outlet obstruction? (3)
Neurogenic bladder, tumors, and congenital malformations
A flap of mucosa that obstructs the bladder outlet is a congenital malformation causing bladder outlet obstruction known as what? (And how does it appear on US?)
Posterior urethral valves
US = Dilated posterior urethra (keyhole sign) with dilated ureters, hydro and thick bladder wall
What are the 4 types of urachal anomalies?
Patent, urachal cyst, urachal sinus, and diverticulum
What is the most common type of urachal anomaly?
Patent
Who does urachal anomalies affect more?
Men
What is situs inversus?
Organs of the abdominal cavity are reversed
What would be an example of a replaced hepatic artery?
Hepatic artery branching from the left gastric artery or SMA instead of the celiac axis
The complete agenesis of which organs are not compatible with life?
Liver, bilateral adrenals and bilateral kidneys
If a fetus has agenesis of the bladder what may happen?
Stillborn or have multiple associated abnormalities
What will you often see with partial agenesis?
Compensatory hypertrophy
Bile ducts from the hilum of the liver to the duodenum being obliterated refers to what rare condition?
Biliary atresia
If the pancreatic tail seems longer than normal, what may the patient have?
Duplication of the pancreas
Where will an ectopic GB be seen?
Embedded in the liver tissue
What pancreatic anomaly is consistent with the pancreatic head surrounding the second part of the duodenum?
Annular
Who does annular pancreas affect more?
Males
What is the most common pancreatic variant?
Pancreatic divisum
Which pancreatic anomaly is prone to pancreatitis?
Pancreatic divisum
Which adrenal gland abnormality is associated with hypogonadism?
Congenital hypoplasia
Adrenogenital syndrome is also known as what?
Congenital hyperplasia
What is an autosomal recessive disorder that interferes with the production of cortisol and aldosterone?
Adrenogenital syndrome
What is the sonographic appearance of congenital hyperplasia?
Adrenals may appear normal or diffusely enlarged
What are three splenic variants?
Accessory spleen, born again spleen, and wandering spleen
Congenital splenic abnormalities are part of a spectrum of anomalies known as what?
Visceral heterotaxy
Asplenia refers to what?
Dominant right side organs
Polysplenia refers to what?
Dominant left side organs
What may also be present with asplenia?
Midline liver, GU/GI tract anomalies, complex cardiac malformations
What may also be present with polysplenia?
Biliary atresia, absence of the gallbladder, and GI abnormalities
What does HPS stand for?
Hypertrophic Pyloric Stenosis
What is the classic sonographic appearance of HPS?
Doughnut sign
Muscle thickness of what measurement is consistent with HPS?
3mm or greater
Pyloric canal length of what measurement is consistent with HPS?
15mm or greater
What are bezoars?
Mass of foreign material in the stomach, most commonly hair
What is another name for accessory spleen?
Splenunculi
What is a born again spleen?
Hypertrophy of an accessory spleen or other splenic tissue post splenectomy
What is a wandering spleen?
Spleen with long mesentery that is prone to torsion
