Module 6 : Congenital Flashcards
what is a variation
- variation of anatomy
is a varient pathological what could it interfere with
- no pathological
- could interfere with function
are variants and anomalies congenital or developmental
- both
can anomalies be major or minor
- yes
what are 4 different areas where there could be anomalies
- number
- size
- positon
- structure
are anomalies or variants more common to interfere with function
- anomalies
what are the 15 anomalies of the urinary tract
- hypertrophied column of Bertin
- junctional parenchymal defect
- extra renal pelvis
- dromedary hump
- fetal lobulation
- renal sinus lipomatosis
- duplicated collecting system
- ureteroceles
- horse shoe kidney
- ectopic kidney
- compensatory hypertrophy
- supernumerary kidney
- hypoplasia
- congenital megacalices
- congenital megaureter
- ureteropelvic obstruction
- ptosis
- multiple renal arteries
what is a hypertrophied column of Berlin (HCB)
- double layer of cortex between medullary pyramids
where is a HCB commonly located
- upper or middle portion of the kidney
how does a HCB indent the sinus
- laterally
what is the upper limit of normal for an HCB
< 3cm
US appearance of HCB
- isoechoic and continuous with renal cortex
what causes a junctional parenchymal defect
- incomplete embryological fusion
what kidney is junctional parenchymal defect more commonly identified
- right
- liver gives us a good window to see them
US look of junctional parenchymal defects
- hyperechoic wedge shape area
- can be confused with scarring
where is an extra Renal pelvis (ERP) located
- medial to renal sinus
what might an ERP mimic and what can patients be more prone to
- hydro
- hydro
can ERP decompress
- yes when patient in prone
where is a dromedary hump most commonly seen
- lateral aspect of left kidney
what is a dromedary hump
- thickening or bulging of the cortex
what is important to visualize to diagnose a dromedary hump vs a mass
- CM junction
- will have pyramids
what is fetal lobulation
- infolding of the cortex with no thinning
what will the outer contour of the kidney look like with fetal lobulation
- scalloped cortex
is fetal lobulation common or uncommon
- more common than not
what is renal sinus lipomatosis
- excessive fatty infiltration of the renal sinus
what 2 things is renal sinus lipomatosis associated with
- aging
- obesity
US look of renal sinus lipomatosis
- enlarged echogenic sinus
what is the most common anomaly of the UT
- duplicated collecting system
what is a duplicated collecting system `
- variable duplication of the renal sinus
what are the 2 types of duplicated collecting system
- complete
- incomplete
what is complete duplicated collecting system
- 2 collection systems
- 2 ureters
- superior ureter will have ECTOPIC bladder insertion
what is incomplete duplicated collecting system
- duplicated ureters join before inserting into the bladder
- Y shaped ureter
what 4 things is duplicated collecting system associated with
- UPJ obstruction
- uterus didelphys
- reflux
- ureterocele
US look of duplicated collecting system
- kidney longer than normal
- 2 separate sinus regions
- cortex through entire centre of the kidney
what is a ureterocele
- cystic dilation of th eternal ureter which protrudes in the urinary bladder
is ureteroceles congenital or acquired
- can be both
what other disorders will patients with ureteroceles commonly have
- UTIs
- obstruction
what is a horseshoe kidney
- fusion of the kidney at lower poles
what leads to an increased risk for infection and stone formation with horseshoe kidney
- malrotation
- associated UPJ obstruction
US look of horseshoe kidney
- difficult to identify lower pole
- lower position in abdomen
- inferior pole situated more medially
- renal tissue drapes ANTERIOR to great vessels (isthmus)
what is an ectopic kidney
- failure of kidney to ascend in utero
where is the kidney usually located with ectopic kidney
- pelvis and unilateral
what does the ectopic kidney usually look like
- small and abnormally rotated
what is crossed ectopia
- both kidneys located on one side
- upper pole of ectopic kidney usually fused to the lower pole of normal kidney
what is compensatory hypertrophy
- tissue/organ expands in volume
is compensatory hypertrophy diffuse or focal
- DIFFUSE sometimes focal
what 2 factors will cause a normal kidney to enlarge
- unilateral kidney agenisis
- nephrectomy
what is a supernumerary kidney
- very rare
- small extra kidney with its own blood supply
is a true hypoplastic kidney common or rare
- rare
what is a hypoplastic kidney
- contains two few nephrons
is hypoplastic kidney unilateral or bilateral
- both
US look of hypoplastic kidney
- small kidney
- difficult to visualize
what are the characteristics of congenital megacalices
- non obstructive
- unilateral
- normal function and parenchyma
- enlarged clubbed calyces
- increased risk of kidney stones
characteristics of congenital megaureter
- functional obstruction of ureter due to distal peristalsis
- men
- left
- may lead to hydro
- fusiform dilation of the distal third of the ureter
characteristics of ureteropelvic obstruction
- results in hydro
- males
- left
- caused by functional or anatomical anomaly
- ## increased incidence of MCKD and contralateral renal agenisis
what is the most common palpable mass of the neonatal abdomen
- UPJ obstruction
US look of UPJ obstruction
- ballooning of renal pelvis
- cortical atrophy
- ureter normal
characteristics of ptosis
- renal fascia tears
- prolapse of kidney
characteristics of multiple renal arteries
- developmental anomaly’s
- failure of regression of renal vessels during ascent
what are 3 bladder anomalies
- exstrophy
- hypospadia
- bladder outlet obstruction
is bladder exstrophy common or rare what gender is it more common in
- rare
- men
what is bladder exstrophy
- large anterior abdominal wall defect
what gender is effected by hypospadias
- males
what is hypospadias
- urethra opens posteriorly not centrally
- check for renal anomaly’s
what is bladder outlet obstruction and what does it cause
- obstruction of bladder outlet
- hydro
what are the 3 potential causes of bladder outlet obstruction
- neurogenic bladder
- tumors
- congenital malformations (posterior urethral valves)
what is a posterior urethral valve and who is it most common in
- flap of mucosa obstructs the bladder outlet
- males
what do patients with posterior urethral valves present with
- palpable flank mass and failure to thrive
US look of PUV
- thick walled bladder
- key hole appearance
- dilated ureters/hydro
what are the 4 urachal anomalies
- patent
- urachal cyst
- urachal sinus
- diverticulum
what is the most common urachal anomaly
- patent
- associated with urethral obstruction
which gender is affected more by urachal anomalies
- men
US look of urachal anomalies
- anechoic mass between the bladder and umbilicus
characteristics of situs inversus
- organ of abdominal cavity reversed
- liver located on the left
- spleen right
- orientation of heart reversed
what are the 5 vascular variations of the abdomen
- accessory hepatic vein
- variations in hepatic branching
- hepatic artery branching from (LGS SMA)
- extra renal arteries
what are the 3 types of organ agenisis
- complete
- partial
- biliary atresia
what three complete organ agenisis is incompatible with life
- liver
- adrenal (bilateral)
- kidney (bilateral)
is GB agenisis common or rare or life threatening
- rare
- not life threatening
is bladder agenisis rare and what is it associated with
- rare
- still born or multiple other abnormalities
with what organs partial agencies would compensatory hypertrophy show
- liver
- pancrease
- unilateral kidney or adrenal
what is biliary atresia
- bile ducts from hilum of liver enter to the duodenum are obliterated
- rare
- treated surgically
what 6 structures may be duplicated
- multiple hepatic ducts
- duplicated renal collecting system
- bladder
- gallbladder
- panc tail
- accessory adrenal glands
what 5 organs can be ectopic
- kidneys
- ureteral insertions
- GB
- panc tissue
- accessory spleens
what are the 2 pancreatic anomalies
- annular
- pancreatic divisum
characteristics of annular panc
- rare
- panc head surrounds second part of duodenum
- males
what is the most common panc variant
pancreatic divisum
characteristics of panc divisum
- dorsal and ventral buds do not fuse
- 2 separate ducts
- prone to pancreatitis
what are 2 adrenal gland abnormalities
- congenital hypoplasia
- congenital hyperplasia (adrenogenital syndrome)
characteristics of adrenal hypoplasia
- hormone production altered
- males
- hypogonadism
characteristics of adrenal hyperplasia
- autosomal recessive
- interferes with production of cortisol and aldosterone
- females results in vitalization (more male)
- precocious puberty
- adrenals diffusely enlarged
what are 3 splenic variants
- accessory spleen
- born again spleen
- wandering spleen
what is an accessory spleen
- splenunculi
- located at splenic hilum
- same texture and echogenicty as spleen
what is a born again spleen
- hypertrophy of accessory spleen post splenectomy
what is a wandering spleen
- long mesentery (point of attachment)
- prone to torsion
what are congenital splenic abnormalities a part of and what are the 2 types
- spectrum of anomalies known as visceral heterotaxy
- asplenia, polysplenia
what is asplenia
- dominant right side organs
- midline liver
- GU/GI tract anomalies
- compeer cardiac malformations
what is polysplenia
- dominant left side organs
- biliary atresia
- absence of GB
- GI abnormalities
what is hypertrophic pyloric stenosis
- congenital narrowing of pylorus
- hypertrophy of pyloric muscle
- new born males
clinical presentation of HPS
- projectile vomitting
- palpable abdominal mass
- dehydration
- weight loss, failure to thrive
US technique of HPS
- patient in supine or RLD
- obtain long and short of pylorus
- evaluate real time motion
US look of HPS
- doughnut sign
+ hypo echoic muscle mass with central hyperechoic lumen - thickness > 3mm HPS
- pyloric canal length > 15mm consistent with HPS
what is bezoars
- mass of foreign material in the stomach usually hair
