Chapter 63: The Fetal Urogenital System

IPKD- (Infantile Polycystic Kidney Disease) Potter Type 1 Associated with:

-Meckel Gruber syndrome -large echogenic kidneys, polydactyly and encephalocele -Trisomy 13 -Poor prognosis

IPKD- (Infantile Polycystic Kidney Disease) Potter Type 1 sonographic findings:

-symmetric bilateral renal enlargement -secondary to renal collecting tubule dilation -echogenic kidneys -small or absent bladder -oligohydramnios

IPKD- (Infantile Polycystic Kidney Disease) Potter Type ___ -Autosomal ____ disorder -Affects both fetal ____ and _____ -small bilateral renal cysts and liver cysts -___% chance of recurrence -family _____

1, recessive, kidneys and liver, 25, history

Ureters and Urethra -Normal fetal ureters and urethra are not visualized -ureters measure less than ____mm in diameter -slight _____ of the ureter may be visible at the renal pelvic and urine jets may be seen in the bladder where the ureters enter

1-2, dilated

Urine formation begins the end of the 1st trimester (_____ weeks)

11-12 wks

External Genitalia Development -External genitalia is fully differentiated by the ____th week -Male -masculinization is produced by ____- from fetal tests -Phallus becomes ____ -Female -urogenital sinus becomes ____ and ____ -urogenital folds become the labia ____ -labioscrotal swellings become the labia ____ -phallus becomes the _____ Turtle Sign- ___ Hamburger Sign-____

12, androgens, penis, urethra, vagina, minora, majora, clitoris, boy, girl

Kidneys seen as early as _____ weeks assess presence, number, position, size and texture -appear as bilateral _____ structures ____ weeks- differentiate cortex from medulla

12-14, hyperechoic, 25

___ weeks- bladder may be seen -normal wall measurement is __mm or less -assess presence and size

13, 2

Kidneys should be sonographically documented in all fetuses beginning at ____ weeks gestation

18

Abnormal Renal Pelvis BOX 63-5 -Greater _____ mm (AP measurement) before the 3rd trimester is considered abnormal -greater than ___mm after the 3rd trimester is abnormal -greater than ___mm is considered mild hydro -____ mm is considered moderate hydro -greater than ____mm is considered severe hydro

4-4.5, 7, 7-15, 15

Permanent Kidneys are metanephros developed early in the ___th week

5

The kidneys originate within the pelvis and lie close together. They gradually migrate to the abdomen and become separate from one another -normally complete migration by ___ weeks

9

Characterized by multiple, smooth walled, non functioning, noncommunicating cysts of variable size and number which replace the normal renal tissue

Adult Multicystic Dysplastic Kidney MCKD- Potters Type II

Most common form of renal cystic disease in childhood

Adult Multicystic Dysplastic Kidney MCKD- Potters Type II

Urethra -Dilation of the posterior urethra is highly suspicious for an obstructive process such as ____, known as "key-hole sign" -PUVO occur only in ____ fetuses -manifested by the presence of a valve in the posterior _____

PUVO, males, urethra

term used to describe renal diseases other than renal agenesis that result in renal failure and facial or structural abnormalities caused by oligohydramnios

Potter's sequence

characterized by a group of findings associated with oligohydramnios and renal failure or bilateral renal agenesis; findings include abnormally positioned extremities, wide-set eyes, low-set ears, and broad nasal bridge

Potter's syndrome

External Genitalia -External Genitalia -gender should be determined in the presence of other ______ -Majority of fetuses the gender can be differentiated in the ___nd trimester -important to determine gender by what ___ imaged and not by what is ___ seen in the area of genitalia -Testes are not visible within scrotal sac until approximately ___ weeks

abnormalities, 2, IS, NOT, 28

Urachus Bladder is continuous with ___ in early development -Allantois regresses to become a fibrous cord known as the _____ -extends from apex of ___ to ____ -Urachal _____ -develops if the lumen of the allantois persists while the urachus forms -Urachal _____ -formed if only a small part of lumen persists -Urachal ____ (patent urachus) -formed if a larger portion of lumen persists

allantois, urachus, bladder, umbilicus, fistula, cyst, sinus

Hydronephrosis -Most common fetal ______ -Commonly occurs with ______ -unilaterally the most common site of hydronephrosis is _______ -usually end result of obstruction at lower level in urinary tract -bilateral hydronephrosis- bladder outlet _____ is the most likely cause -Sonographic Findings -______ renal pelvis - communicated with the calyces (caliectasis) centrally located -normal outer______ -AP diameter greater than ____mm

anomaly, obstruction , ureteropelvic

Anterior Urethral Valves -Congenital defect results in obstruction of the ____ urethral valve in _____ -An exact cause is unclear -Dilated anechoic urethra seen proximal to the ____

anterior, Males, valve

Prune Bell Syndrome (Eagly Barrett Syndrome) -Urethral Atresia -massively dilated ____ (prune belly) -dilated prostatic _____ and _____ -mimics _____ but not "keyhole sign" -Protruding fetal ____ filled with multiple cystic structures (dilated ureters and bladder) -_______, pulmonary ______, fetal _____, ...pg 1373 bottom

bladder, urethra, ureters, PUV, abdomen, oligo, hypoplasia, ascites

Adult Multicystic Dysplastic Kidney MCKD- Potters Type II -Unilateral multicystic -multiple non communicating cyst of variable size -no distinct renal _____, _____ and _____ -normal or enlarged ______ kidney -______ seen in presence of adequately functioning renal tissue -____ and ____ are usually normal with unilateral occurrence -good prognosis with ____ unilateral involvement -Bilateral Multicystic -very _____ and _____ -sonographically will have bilateral multiple noncommunicating cyst of variable size, ____ and ____

borders, pelvis and parenchyma, contralateral, bladder, bladder and AF, isolated, uncommon, lethal, oligo, absent bladder

complex malformation involving lower limb anomalies, spinal defect, anal atresia, and lower abdominal wall defect below the cord insertion involving exstrophy of the bladder and protrusion of the intestines

cloacal exstrophy

Abnormalities of the Urinary Tract -2 categories of renal malformations -_____ malformation -______ process -Important of detection in utero -recognition of ____ or _____ anomalies -insure proper clinical and therapeutic management -Assessment of ____ -abnormal amniotic fluid levels greater than ____ weeks would indicate renal anomalies

congenital, obstructive, lethal or treatable, AF, 14-16

failure of the testes to descend into the scrotum

cryptorchidism

Prune Belly Syndrome (Eagly Barrett Syndrome) -AKA urethral obstruction malformation is recognized by three features -______ (undescended testes) -______abdominal musculature -_______ and bladder outlet obstruction

cryptorchidism, absent or dysplastic, megaureters

Transverse images, the renal pelvis may be measured in their AP_____ when the fetal spine is towards the maternal _____ wall. -The upper limit of normal is ___mm up to the 3rd trimester -The upper limit of normal is ___mm from 3rd trimester until term

diameter, anterior, 4, 7

-Malformation of the uterus and vagina -uterus _____- complete duplication -_______ Uterus- duplication of the uterus -Unicornuate Uterus- _____ uterine tube and horn

didelphys, bicornuate, single

Sonographic Findings of PUVO -Severe bladder ____ -Massive ______ -dysplastic changes in the renal tissue -Megaureters/Hydroureters (tortuous) -______ -Thickening of the bladder _____ -Dilated posterior urethra -______ appearance

dilation, hydronephrosis, oligo, wall, keyhole

Adult Dominant PKD - Potters Type III -Autosomal ______ -most common of the _____ renal cystic diseases with a recurrence risk of ___% -Genetic link -usually develops in ______ -may be diagnosed family history or PCKD or liver or both -renal enlargement may prompt a kidney and liver workup in parents to rule out disorder -_____ (almost always), ______, symmetric renal enlargement -normal amount of _____ and _____

dominant, hereditary, 50, adulthood, bilateral, echogenic, AF and normal bladder

Ureterocele -cystic dilation of the intravesical (bladder) segment of the distal ureter -females are more likely to present with renal _____ -males more commonly with _____ renal collecting system Tumors -rare _____ of kidney will be distorted or replaced by a ____ and absent ______ echoes

duplication, normal, contour, mass, pelvicaliceal

abnormal congenital opening of the male urethra on the top side of the penis

epispadias

dilated renal pelvis in the fetus; most common fetal anomaly

fetal hydronephrosis

ovarian mass that results from maternal hormone stimulation and is usually benign

fetal ovarian cyst

Renal Cystic Disease -Group of ____ development and acquired disorders -Potter sequence is used to describe diseases that are associated with renal ____, ___, and Potters ____

heritable, oligo, renal failure, facies

condition in which both ovarian and testicular tissues are present

hermaphroditism

inability to determine fetal gender

hermaphroditism

Development of Bladder -Derived from the _____ -Known as _____ sinus -Caudal end open into the____ and parts are gradually absorbed into the wall of the urinary bladder -This development causes the ____ and the _____ ducts to enter the bladder separately

hindgut, urogenital, cloaca, ureters, mesonephric

forms when the inferior poles of the kidney fuse while they are in the pelvis

horseshoe kidney

collection of fluid in the vagina and uterus

hydrometrocolpos

Pelvic Masses -_______ -collection of fluid in vagina and uterus -_____ or _____ "cyst like" mass posterior to the bladder in the area of the uterus -Ovarian cyst -caused from maternal _____ stimulation and is usually benign -may be multiseptated and bilateral -may torse, rupture, and appear complex or solid or cause _____ obstruction

hydrometrocolpos, hypoechoic or echogenic, hormonal, intestine

_____ does not cause renal enlargement!

hydronephrosis

Sonographic Findings of Potter Type IV -Fetal kidneys -early enlarged with ____ or ____ -later, small ____ with cortical peripheral cysts -Unilateral -______ bladder and amniotic fluid -____ obstruction -Bilateral -early bladder outlet ______ -_____ bladder -bilateral ______ -thick walled bladder -severe ______

hydronephrosis, hydroureter, echogenic, normal, UPJ, obstruction, hydronephrosis, oligo

Posterior Urethral Valve Obstruction PUVO -PUV obstruction results in _____, _____, or dilation of the ____ and posterior _____ -Congenital folds of urethra act as _____ -urine unable to pass through urethra into __ -Most common cause of urinary obstruction in ____ (ONLY occurs in males) -May cause severe ____ to urinary system -depends on the severity of renal function and associated ______

hydronephrosis, hydroureters, bladder, urethra, valves, AF, males, damage, oligo

Ureters -Dilated Ureters -seen as tortuous cystic masses -______- abnormally dilated ureters that may be traced into the kidney and bladder

hydroureters

dilated ureters

hydroureters

abnormal congenital opening of the male urethra on the undersurface of the penis

hypospadias

autosomal recessive disease that affects the fetal kidneys and liver; the kidneys are enlarged and echogenic on ultrasound

infantile polycystic kidney disease (IPKD)

Ureterocele & Ectopic Ureter -Ureterocele is a cystic dilation of the ______ (bladder) segment of the distal ureter -lower end of ureter bulges into _____ -Ectopic ____ -does not insert near posterolateral angle of the trigone of the bladder -females- may insert in ____, ____ -males - may insert in ____, _____, _____ - in an ectopic location of the bladder

intravesical, bladder, ureter, vagina, uterus, seminal vesicle, vas deferens or ejaculatory ducts

Adrenals relatively ____ in fetus -seen lying superior to kidney

large

In sagittal images, the renal _____ and_____ measurement may be obtained

length, AP

Horseshoe Kidney -fusion of ____ poles of both kidneys -connecting ____- may be seen anterior to aorta with spine _____

lower, isthmus, down

Ureterovesical Junction Obstruction - UVJ -Commonly presents with dilation of the____ end of the ureter (megaureter) -results from primary defect (stenotic ureteral ____ or ____-) -secondary to an obstruction at another level (Causing ____ or _____ flow of urine) -Sonographic findings -dilation of the renal ____ and tortuous dilated ureter- almost always ____ -Double collecting system - common -dilated ____ pole and normal lower pole may result from ectopic ureterocele within bladder

lower, valve or fibrosis, reflux, backflow, unilateral, upper

the level of the urethra where the urinary tract may become obstructed

megacystis

dilation of the lower end of the ureter; the common presentation of ureterovesical junction obstruction

megaureter

Solid Masses -congenital ______ nephroma -_______ -most common renal tumor (____) -______ Tumor -malignant tumor renal tumor -_______ -An adrenal tumor which is the premalignant precursor of Wilms tumor

mesoblastic, hamartoma, benign, Wilms, neuroblastoma

Unilateral Renal Agenesis -____ common than bilateral agenesis -_____ survival rate -Occurs in ____ to ____ -male to female ratio is ____ -______ kidney may be enlarged to compensate for the absent kidney -Normal ___ may be present since one kidney is producing urine -Associated with ____ anomalies in females and _____ anomalies in males

more, excellent, 1 to 600 or 1 to 1000, 1:1, contralateral, AF, uterine, testicular

multiple cysts replace normal renal tissue throughout the kidney; usually causes renal obstruction

multicystic dysplastic kidney disease (MSCK)

Hydrocele -small amount of fluid in fetal scrotum is _____ -when fluid surrounds entire testicle a ______ is present -Male gender identified

normal, hydrocele

Obstructive Cystic Dysplasia Potter Type IV -Caused by early renal ______ -due to obstruction in ___ or early ___trimester -Unilateral -_____ (UPJ) or _______ (UVJ) junction obstruction -UPJ- renal pelvic -UVJ- at level of _____ -megacystis- level of ______ -Bilateral -severe bladder outlet _____ -_____ or urethral _____

obstruction, 1st, 2nd, pelviureteric, vesicoureteric, bladder, urethra, obstruction, PUVO, atresia

Collecting system -normal or dilated, if dilated look for level and cause of _____ and whether it is _____ or ______

obstruction, unilateral or bilateral

Potters Syndrome -Characterized -renal agenesis (bilateral), _______, ______ hypoplasia, abnormal facies (___nose, ____ chin, abnormal ____ and wide set ____) and abnormal or malpositioned _____ -Sonographic Findings

oligo, pulmonary, flat, recessed, ears, eyes, limbs -Sonographic Findings -oligohydramnios -absence or urine in bladder -small thorax-pulmonary hypoplasia

Sonographic Findings in Bilateral Renal Agenesis -Severe ______ after 13-15 menstrual weeks -Persistent absence of ____ in fetal bladder (observe 1 hour) -Failure to _____ kidneys or renal arteries (use color flow to outline renal arteries) -Abnormally small ______

oligo, urine, visualize, thorax

Crossed renal ectopia -Lies on _____ side of abdomen and occurs usually on the _____ and both kidneys may be fused together

opposite, right

Renal Ectopia -kidney lies ____ normal position -Usually in the area of the _____

outside, pelvis

______ -a kidney that remains within the pelvic cavity -may appear in females as a pelvic mass

pelvic kidney

occurs when the kidney does not migrate upward into the retroperitoneal space

pelvic kidney

Ureteropelvic Junction Obstruction (UPJ) -Occurs at the junction between the renal_____ and ____ -usually ____- and more common in _____ -Most common reason for ____ in neonate -Sporadic condition caused by -fibrous _____, kinks in ____, abnormal _____ valves etc -Sonographically there is a collection of urine within the pelvis- normal renal cortex, ureter, bladder and amniotic fluid

pelvis and ureter, unilateral, males, hydronephrosis, adhesions, ureter, ureteral

The kidneys do not function in utero because the ______ eliminates waste from the fetal blood; however the kidneys must be able to assume their waste excretion role after birth

placenta

occurs only in male fetuses; is manifested by the presence of a valve in the posterior urethra

posterior urethral valve

Developmnet of the Kidneys -Embryo has three sets of kidneys -______- rudimentary and nonfunctional -_______- function for a short while and they degenerate -_______- permanent kidneys

pronephros, mesonephros, metanephros

dilation of the fetal abdomen secondary to severe bilateral hydronephrosis and fetal ascites; fetus also has oligohydramnios and pulmonary hypoplasia

prune belly syndrome

dilated renal pelvis without involvement of the calyces

pyelectasis

renal system fails to develop

renal agenesis

-Amniotic Fluid -a critical marker in the assessment of ___ function -fetal kidneys are not a major contributor of fetal urine and amniotic fluid until ___ weeks -renal agenesis cannot be excluded prior to ____ weeks since the kidneys are not contributing to fetal urine or amniotic fluid

renal, 14-16, 14-16

Renal Agenesis -This condition occurs when the ____ system fails to develop -Bilateral (complete absence of kidneys) -______ disorder because or renal insufficiency and hypoplasia of the lungs -occurs in _____ to _____ -male to female ratio is ____ -adrenal glands may be large and may mimic the kidneys -associated anomalies -_______ defects, ______ disorders, ___ and ____ -most of these anomalies will not be detected prenatally due to the absence of amniotic fluid

renal, lethal, 1:3,000-1:10,000, 2.5:1, cardiac, musculoskeletal, CNS, GI

Exstrophy of the Bladder -Characterized by the protrusion of the posterior wall of the urinary bladder which contains the ___ of the bladder and ureteric ____ -Lack of _____ formation in the anterior abdominal wall to cover the bladder -Occurs primarily in _____ -Sonographically -____ filled bladder not identified -small irregular ____ in the lower abdomen below the umbilical insertion

trigone, orifices, muscle/tissue, males, fluid, mass

a small part of the lumen of the allantois that persists while the urachus forms

urachal cyst

Permanent kidneys develop from two different sources: -_______ -give rise to the ureter, renal pelvic, calyces, and collecting tubules -_______

ureteric bud, metanephric mesoderm

congenital outpouching of the distal ureter into the bladder

ureterocele

junction of the ureter entering the renal pelvis; most common site of obstruction

ureteropelvic obstruction (UPJ)

junction where the ureter enters the bladder

ureterovesical junction (UVJ)

condition that causes a massively distended bladder (prune belly)

urethral atresia