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SONOGRAPHIC
APPEARANCE
 FETAL KIDNEYS:
 demonstrated at approximately 11 weeks transvaginally and
at 12 weeks using transabdominal probes.
 During the first trimester, the kidneys appear as hyper-echoic
oval structures at both sides of the spine
 This echogenicity will progressively decrease, and during the
third trimester, the cortical echogenicity will always be less
than that of the liver or spleen.
 Simultaneous to the decreased echogenicity, corticomedullary
(CMD) differentiation will appear at approximately 14 to 15
weeks.
 . Urine distending the renal pelvis may help in their
identification.
BLADDER
 Urine is first produced by the kidneys during the 9th
week of embryonic life.
 At this stage, the urine in the bladder can be
visualized as a fluid-filled structure within the fetal
pelvis.
 During the second and third trimester, the bladder will
empty and refill continuously every 25 to 30 minutes.
 The position of the fetal bladder can virtually always
be identified, because it lies between the umbilical
arteries within the fetal pelvis. These arteries are
readily seen with the use of color Doppler imaging.
URINARY TRACT
ABNORMALITIES
Systemic approach to assess
prenatal UT abnormalities include:
 Assessment of amniotic fluid index
 Localisation and characterisation of
urinary tract abnormalities
 Search for associated abnormalities
 Presence
 Number
 Position
 Appearance
 Unilateral or bilateral
BILATERAL
RENAL AGENESIS
 Ureteric buds fail to develop
 Lethal congenital anomaly
 1 in 4000 births and 2.5:1 male preponderance
 Associated with potter’s syndrome
 Pulmonary hypoplasia is the major cause of death
USG FINDINGS
 Severe oligohydraminos/anhydraminos
 Absent kidneys
 “LYING DOWN” adrenal sign
 Absent renal arteries on Color Doppler imaging
 Non visualisation of bladder (over 1 hour)
UNILATERAL
AGENESIS
 More common than B/L
 AFI, bladder- NORMAL
 Contralateral kidney shows compensatory
hypertrophy
 The ipsilateral adrenal gland is usually
present, and appears globular and should
not be mistaken for the kidney. Without the
adjacent kidney, the adrenal gland may be
elongated in appearance in what has been
called “the lying down adrenal sign”
 Contralateral kidney associated with
abnormalities ,most common—VUR
 One or both kidneys in abnormal position
 Pelvic kidney is the most common
 When ectopic kidney is located on opposite side of the abdomen
relative to its urethral insertion in to bladder is defined. CROSSED
FUSED ECTOPIA.
 Associated with abnormalities like VUR.
 Most common fusion anomaly
 On usg,
 Abnormal longitudinal axis of both
kidneys with bridge of renal tissue
connecting the lower poles
 Majority have anterior orientation of
pelvis B/L
 Associated with anomalies like turners
syndrome
 Higher prevalence of VUR, calculus ,UTI
Non – genetic cystic diseases
 Multi-cystic dysplastic kidney
(MCDK)
 Obstructive dysplasia
Genetic cystic diseases
 Autosomal recessive polycystic
kidney disease
 Autosomal dominant polycystic
kidney disease
RENAL CYSTIC DISEASE
 Most common renal cystic disease
 Kidney replaced by multiple cysts of varying sizes separated by dense
stromatolites and there is usually no renal parenchyma
 The cysts can be large, presenting as a large fetal abdominal mass, or on the
contrary, microcystic.
 Effect of urinary obstruction on subsequent renal development depends on the
time of onset and severity of obstruction
 Fetal urinary tract responds differently than adults to chronic obstruction by
possible development of macroscopic cysts
 Unilateral- UPJ OR UVJ obstruction
 Bilateral- Bladder outlet. Obstruction
AUTOSOMAL RECESSIVE
POLYCYSTIC KIDNEY
DISEASE
 ARPKD has an incidence of 1 in 20,000 live births and
often causes fetal and neonatal death.
 Renal failure and hepatic fibrosis develop in many
babies who survive the perinatal period
 The disease is characterised by marked elongation of
the collecting tubules that expand into multiple small
cysts. The cystic dilatation of the tubules is variable
and predominates in the medulla. The outer cortex is
spared because it contains no tubules. There is
associated biliary dysgenesis. Hepatic fibrosis has been
described on pathologic examination of affected
fetuses.
 The sonographic presentation varies according to the
severity of involvement. The most typical presentation
is that of markedly enlarged hyperechoic kidneys
without CMD).
 Another presentation of ARPKD can be reversed CMD with large kidneys.
 This finding is probably related to increased number of interfaces within the
medullae, and to inspissated material within the dilated tubules.
 It is an important observation because there are very few other causes of reversed
CMD.
AUTOSOMAL DOMINANT
POLYCYSTIC KIDNEY
DISEASE
 ADPKD is the most common form of polycystic
kidneys disease in humans , 1 in 800 live births.
 The disease is characterized by the presence of
multiple cysts that develop on the wall of collecting
tubules and nephrons. The cysts are scattered
within the cortex and medulla. These cysts enlarge
progressively and are typically identified in early
adulthood. Cysts may also develop in the liver,
spleen and pancreas in adults.
HYPERECHOGENIC
KIDNEYS
 Most commonly detected renal
abnormality
 Hydronephrosis refers to abnormal
dilatation of renal pelvis and calyces.
 Pyelectasis refers to milder form
with only dilatation of renal pelvis
 The severity of renal pelvic dilatation can be assessed using
different grading systems. Measuring the maximal antero-
posterior diameter of the renal pelvis (APPD) on a
transverse scan of the fetal abdomen is the generally
accepted method
 thresholds vary between 4 and 10 mm in the second
trimester, and between 7 and 10 mm in the third trimester.
Using a cut-off value of 4 mm in the second trimester,
results in a diagnosis of pyelectasis in 2% of pregnancies
(Adra et al., 1995; Corteville et al., 1991). A 7 mm cut-off in
the third trimester has a sensitivity of 100
 Obstruction at the UPJ is the most common cause of
non-physiologic neonatal hydronephrosis,
 It is also one of the most common reasons for postnatal
surgery of the UT.
 Most cases of UPJ obstruction are functional (caused by
a muscular abnormality) rather than the result of mixed
anatomic lesions such as fibrous adhesions, kinks,
valves, or aberrant vessels.
 On sonography, a dilated renal pelvis with or without
caliectasis is identified. he ureter and the bladder are
not dilated. Severe chronic obstruction leads to
effacement of the calyces and thinning of the renal
cortex (SFU grade 4)
 More common in males
 Primary megaureters- 3 types
LOWER URINARY
TRACT
OBSTRUCTION
 Fetal megacystis:
 10-14 weeks
 Longitudinal diameter of bladder- >7mm or
more
 2nd- 3rd trimester – enlarged bladder ,fails to
empty over 45 min of observation
 If >7-15mm mostly associated with
chromosomal abnormalities.
CAUSES OF
FETAL
MEGACYSTI
S
 Posterior urethral valve
 Urethral atresia or stricture
 Ureterocole
 Prune belly syndrome
 Megalourethra
 Cloacal malformation
 Megacystis microcolon intestinal
hypoperistalisis syndrome
 Most common cause
 Seen exclusively in males
 Infra vesicular pressure generated secondary to obstruction results in persistently
dilated UB with a dilated proximal dilated urethra giving KEY HOLE appearance
 Thickening of bladder wall,B/L tortuous hydroureters and hydronephrosis
 most severe, and earliest detected, form of
obstructive uropathy. he sonographic features
include a greatly distended bladder that may
ill the whole abdomen, and anhydramnios
after the first trimester
 . Urethral atresia is almost always fatal,
because of associated renal dysplasia and
pulmonary hypoplasia, and only case reports
of survivors following antenatal treatment
have been described.195,1
 characterized by a congenital deficiency of the
mesodermal tissues of the phallus, with dilation of the
penile urethra and enlargement of the penis.
 this condition has been classified into two types,
fusiform and scaphoid urethra, but it is preferable to
consider it as a spectrum.
 Urinary stasis in the dilated penile urethra results in
functional obstruction of the UT.
 The prenatal sonographic findings include those of
LUTO, with dilation and elongation of the penile
urethra
 . Associated malformations of the UT include urethral
atresia, posterior urethral valves, prune belly syndrome,
and horseshoe kidney. Abnormalities involving the GI
tract and spine and VACTERL association on have been
reported
 Deficiency of abdominal musculature and cryptorchidism
 Rarely in females
 Bladder is large thin walled
 PRESENCE
 APPEARANCE AND SIZE
FAILURE OF URINE
PRODUCTION
 Bilateral severe renal abnormalities
like renal agenesis, MCDK, ARPKD,
UPJ obstruction.
 Severe intrauterine growth restriction
or placental insufficiency
FAILURE TO STORE URINE
 Bladder exstrophy
 Cloacal exstrophy
 Cloacal malformation
 Bilateral ectopic ureter with drainage
outside of bladder
 Incomplete median closure of the inferior part
of the anterior abdominal wall and anterior
wall of urinary bladder
 On USG,
 AFI and kidneys are normal but bladder is not
identified
 Irregular mass may be seen arising from the
anterior abdominal wall inferior to umbilicus
 Low umbilical cord insertion
 Widening of pubic bones
 Rarely reported with OEIS complex
Longitudinal scan of the lower fetal abdomen in a 29-
week fetus shows an irregular mass on the anterior
abdominal wall (arrows), below the umbilical cord
insertion (U). A luid-illed bladder was not identiied.
Note normal amniotic luid volume. (B) Corresponding
photograph shows the exposed bladder (arrows) below
the umbilical cord (U).
 More than half of all congenital abdominal masses
found in the neonate originate in the kidney.
 In the fetus, the most common renal tumor is the
mesoblastic nephroma .
 It appears as a solid tumor that is sometimes difficult
to delineate from the adjacent renal parenchyma.
 The tumor can appear partially cystic.
 In utero, polyhydramnios is typically associated and
hypertension develops after birth.
 Cases of fetal renal Wilms’ tumor have been reported
as solid or partially cystic tumors. The prognosis is
good. Bilateral involvement suggests
nephroblastomatosis, which is a condition with
multiple benign nodular lesions.
 Whenever a mass is detected in the suprarenal
area, a neuroblastoma should be suspected first
regardless of the pattern of the mass
 Neuroblastoma may present as a cystic, a solid
or more complex appearance.
 Color Doppler imaging may demonstrate
increasing vascularization of the hyperechogenic
regions of the mass.
 The differential diagnosis should include adrenal
hemorrhage, associated or not with RVT, and
adrenal cysts.
 . Extra-adrenal causes, such as sub-
diaphragmatic pulmonary sequestration, should
also be included in the differential diagnosis

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Fetal genitourinary

  • 1.
  • 2.
  • 3. SONOGRAPHIC APPEARANCE  FETAL KIDNEYS:  demonstrated at approximately 11 weeks transvaginally and at 12 weeks using transabdominal probes.  During the first trimester, the kidneys appear as hyper-echoic oval structures at both sides of the spine  This echogenicity will progressively decrease, and during the third trimester, the cortical echogenicity will always be less than that of the liver or spleen.  Simultaneous to the decreased echogenicity, corticomedullary (CMD) differentiation will appear at approximately 14 to 15 weeks.  . Urine distending the renal pelvis may help in their identification.
  • 4. BLADDER  Urine is first produced by the kidneys during the 9th week of embryonic life.  At this stage, the urine in the bladder can be visualized as a fluid-filled structure within the fetal pelvis.  During the second and third trimester, the bladder will empty and refill continuously every 25 to 30 minutes.  The position of the fetal bladder can virtually always be identified, because it lies between the umbilical arteries within the fetal pelvis. These arteries are readily seen with the use of color Doppler imaging.
  • 5. URINARY TRACT ABNORMALITIES Systemic approach to assess prenatal UT abnormalities include:  Assessment of amniotic fluid index  Localisation and characterisation of urinary tract abnormalities  Search for associated abnormalities
  • 6.  Presence  Number  Position  Appearance  Unilateral or bilateral
  • 7. BILATERAL RENAL AGENESIS  Ureteric buds fail to develop  Lethal congenital anomaly  1 in 4000 births and 2.5:1 male preponderance  Associated with potter’s syndrome  Pulmonary hypoplasia is the major cause of death
  • 8. USG FINDINGS  Severe oligohydraminos/anhydraminos  Absent kidneys  “LYING DOWN” adrenal sign  Absent renal arteries on Color Doppler imaging  Non visualisation of bladder (over 1 hour)
  • 9. UNILATERAL AGENESIS  More common than B/L  AFI, bladder- NORMAL  Contralateral kidney shows compensatory hypertrophy  The ipsilateral adrenal gland is usually present, and appears globular and should not be mistaken for the kidney. Without the adjacent kidney, the adrenal gland may be elongated in appearance in what has been called “the lying down adrenal sign”  Contralateral kidney associated with abnormalities ,most common—VUR
  • 10.  One or both kidneys in abnormal position  Pelvic kidney is the most common  When ectopic kidney is located on opposite side of the abdomen relative to its urethral insertion in to bladder is defined. CROSSED FUSED ECTOPIA.  Associated with abnormalities like VUR.
  • 11.  Most common fusion anomaly  On usg,  Abnormal longitudinal axis of both kidneys with bridge of renal tissue connecting the lower poles  Majority have anterior orientation of pelvis B/L  Associated with anomalies like turners syndrome  Higher prevalence of VUR, calculus ,UTI
  • 12. Non – genetic cystic diseases  Multi-cystic dysplastic kidney (MCDK)  Obstructive dysplasia Genetic cystic diseases  Autosomal recessive polycystic kidney disease  Autosomal dominant polycystic kidney disease RENAL CYSTIC DISEASE
  • 13.  Most common renal cystic disease  Kidney replaced by multiple cysts of varying sizes separated by dense stromatolites and there is usually no renal parenchyma  The cysts can be large, presenting as a large fetal abdominal mass, or on the contrary, microcystic.
  • 14.  Effect of urinary obstruction on subsequent renal development depends on the time of onset and severity of obstruction  Fetal urinary tract responds differently than adults to chronic obstruction by possible development of macroscopic cysts  Unilateral- UPJ OR UVJ obstruction  Bilateral- Bladder outlet. Obstruction
  • 15. AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE  ARPKD has an incidence of 1 in 20,000 live births and often causes fetal and neonatal death.  Renal failure and hepatic fibrosis develop in many babies who survive the perinatal period  The disease is characterised by marked elongation of the collecting tubules that expand into multiple small cysts. The cystic dilatation of the tubules is variable and predominates in the medulla. The outer cortex is spared because it contains no tubules. There is associated biliary dysgenesis. Hepatic fibrosis has been described on pathologic examination of affected fetuses.  The sonographic presentation varies according to the severity of involvement. The most typical presentation is that of markedly enlarged hyperechoic kidneys without CMD).
  • 16.  Another presentation of ARPKD can be reversed CMD with large kidneys.  This finding is probably related to increased number of interfaces within the medullae, and to inspissated material within the dilated tubules.  It is an important observation because there are very few other causes of reversed CMD.
  • 17. AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE  ADPKD is the most common form of polycystic kidneys disease in humans , 1 in 800 live births.  The disease is characterized by the presence of multiple cysts that develop on the wall of collecting tubules and nephrons. The cysts are scattered within the cortex and medulla. These cysts enlarge progressively and are typically identified in early adulthood. Cysts may also develop in the liver, spleen and pancreas in adults.
  • 19.  Most commonly detected renal abnormality  Hydronephrosis refers to abnormal dilatation of renal pelvis and calyces.  Pyelectasis refers to milder form with only dilatation of renal pelvis
  • 20.  The severity of renal pelvic dilatation can be assessed using different grading systems. Measuring the maximal antero- posterior diameter of the renal pelvis (APPD) on a transverse scan of the fetal abdomen is the generally accepted method  thresholds vary between 4 and 10 mm in the second trimester, and between 7 and 10 mm in the third trimester. Using a cut-off value of 4 mm in the second trimester, results in a diagnosis of pyelectasis in 2% of pregnancies (Adra et al., 1995; Corteville et al., 1991). A 7 mm cut-off in the third trimester has a sensitivity of 100
  • 21.
  • 22.
  • 23.  Obstruction at the UPJ is the most common cause of non-physiologic neonatal hydronephrosis,  It is also one of the most common reasons for postnatal surgery of the UT.  Most cases of UPJ obstruction are functional (caused by a muscular abnormality) rather than the result of mixed anatomic lesions such as fibrous adhesions, kinks, valves, or aberrant vessels.  On sonography, a dilated renal pelvis with or without caliectasis is identified. he ureter and the bladder are not dilated. Severe chronic obstruction leads to effacement of the calyces and thinning of the renal cortex (SFU grade 4)
  • 24.  More common in males  Primary megaureters- 3 types
  • 25. LOWER URINARY TRACT OBSTRUCTION  Fetal megacystis:  10-14 weeks  Longitudinal diameter of bladder- >7mm or more  2nd- 3rd trimester – enlarged bladder ,fails to empty over 45 min of observation  If >7-15mm mostly associated with chromosomal abnormalities.
  • 26. CAUSES OF FETAL MEGACYSTI S  Posterior urethral valve  Urethral atresia or stricture  Ureterocole  Prune belly syndrome  Megalourethra  Cloacal malformation  Megacystis microcolon intestinal hypoperistalisis syndrome
  • 27.  Most common cause  Seen exclusively in males  Infra vesicular pressure generated secondary to obstruction results in persistently dilated UB with a dilated proximal dilated urethra giving KEY HOLE appearance  Thickening of bladder wall,B/L tortuous hydroureters and hydronephrosis
  • 28.  most severe, and earliest detected, form of obstructive uropathy. he sonographic features include a greatly distended bladder that may ill the whole abdomen, and anhydramnios after the first trimester  . Urethral atresia is almost always fatal, because of associated renal dysplasia and pulmonary hypoplasia, and only case reports of survivors following antenatal treatment have been described.195,1
  • 29.  characterized by a congenital deficiency of the mesodermal tissues of the phallus, with dilation of the penile urethra and enlargement of the penis.  this condition has been classified into two types, fusiform and scaphoid urethra, but it is preferable to consider it as a spectrum.  Urinary stasis in the dilated penile urethra results in functional obstruction of the UT.  The prenatal sonographic findings include those of LUTO, with dilation and elongation of the penile urethra  . Associated malformations of the UT include urethral atresia, posterior urethral valves, prune belly syndrome, and horseshoe kidney. Abnormalities involving the GI tract and spine and VACTERL association on have been reported
  • 30.  Deficiency of abdominal musculature and cryptorchidism  Rarely in females  Bladder is large thin walled
  • 31.
  • 33. FAILURE OF URINE PRODUCTION  Bilateral severe renal abnormalities like renal agenesis, MCDK, ARPKD, UPJ obstruction.  Severe intrauterine growth restriction or placental insufficiency FAILURE TO STORE URINE  Bladder exstrophy  Cloacal exstrophy  Cloacal malformation  Bilateral ectopic ureter with drainage outside of bladder
  • 34.  Incomplete median closure of the inferior part of the anterior abdominal wall and anterior wall of urinary bladder  On USG,  AFI and kidneys are normal but bladder is not identified  Irregular mass may be seen arising from the anterior abdominal wall inferior to umbilicus  Low umbilical cord insertion  Widening of pubic bones  Rarely reported with OEIS complex Longitudinal scan of the lower fetal abdomen in a 29- week fetus shows an irregular mass on the anterior abdominal wall (arrows), below the umbilical cord insertion (U). A luid-illed bladder was not identiied. Note normal amniotic luid volume. (B) Corresponding photograph shows the exposed bladder (arrows) below the umbilical cord (U).
  • 35.  More than half of all congenital abdominal masses found in the neonate originate in the kidney.  In the fetus, the most common renal tumor is the mesoblastic nephroma .  It appears as a solid tumor that is sometimes difficult to delineate from the adjacent renal parenchyma.  The tumor can appear partially cystic.  In utero, polyhydramnios is typically associated and hypertension develops after birth.  Cases of fetal renal Wilms’ tumor have been reported as solid or partially cystic tumors. The prognosis is good. Bilateral involvement suggests nephroblastomatosis, which is a condition with multiple benign nodular lesions.
  • 36.  Whenever a mass is detected in the suprarenal area, a neuroblastoma should be suspected first regardless of the pattern of the mass  Neuroblastoma may present as a cystic, a solid or more complex appearance.  Color Doppler imaging may demonstrate increasing vascularization of the hyperechogenic regions of the mass.  The differential diagnosis should include adrenal hemorrhage, associated or not with RVT, and adrenal cysts.  . Extra-adrenal causes, such as sub- diaphragmatic pulmonary sequestration, should also be included in the differential diagnosis

Editor's Notes

  1. he permanent kidney (metanephros) is the third in a series of excretory organs in the human embryo, forming ater the pronephros and mesonephros.3 In the seventh menstrual week, the metanephros begins to develop from two sources: the metanephric diverticulum (ureteric bud) and the metanephric mass of intermediate mesoderm (Fig. 39.1). he ureteric bud is an outgrowth from the mesonephric duct, near its entrance into the cloaca. It elongates and branches in a dichotomous pattern, giving rise to the ureter, renal pelvis, calycescollecting tubules. hrough interaction with the metanephric mesoderm, the ureteric bud induces the formation of nephrons. In early embryonic life, the kidneys are located in the pelvis, but they “ascend” to their adult position by the 11th menstrual week. At this gestation, the kidneys start to produce urine.By the ninth menstrual week, the cloaca (caudal part of hindgut) is divided by the urorectal septum into the rectum posteriorly and the urogenital sinus anteriorly (see Fig. 39.1). he urinary bladder, the female urethra, and most of the male urethra develop from the urogenital sinus and the surrounding splanchnic mesenchyme. Initially, the bladder is continuous with the allantois, but this structure soon constricts and becomes a ibrous cord, the urachus, which extends from the apex of the bladder to the umbilicus.
  2. heir hyperechogenicity can be compared to that of the liver or spleen)
  3. ITFALLS IN INTERPRETATIONAmniotic luid volume may be normal before 16 weeks’ gestationBowel or adrenal glands can be mistaken for kidneys Urachal diverticulum may mimic the bladderEmpty bladder may be caused by impaired renal function from other causes (e.g., intrauterine growth restriction)
  4. . An ectopic kidney is usually smaller and may be malrotated.  Other ectopic locations include horseshoe, crossed fused ectopia (both kidneys lie on the same side), and intrathoracic ectopia. Crossed ectopia should be differentiated from duplex kidneys. In crossed (fused) ectopia, there is an angulation between the two kidneys, whereas in duplication, the two renal moieties lie in the same continuous plane 
  5. Consists of heterogenous group of disorders with inherited and acquired causes Pottter classification is based on histology not considered
  6. Kidneys -non functional So prognosis depends on other kidney Affects whole kidney but also affects portion of the duplex kidney supplied by attic ureter The sonographic findings in patients with MDK are usually straightforward: unilateral involvement with noncommunicating cysts of variable size, variable amount of hyperechogenic stroma, no normal cortex or medulla, irregular renal contours, and no identifiable collecting system MDK may also develop in the upper part of a duplex system (see Fig. 16-29) or be located in an ectopic position (Fig. 16-41). The size of the cysts may decrease in utero or after birth (see Fig. 16-40). Therefore, the entire MDK may “disappear” and give the appearance of renal agenesis. Unilateral involvement carries a good prognosis, although there may be an anomaly affecting the contralateral kidney (i.e., UPJ obstruction). The condition can be associated with other system’s malformations (Fig. 16-42). When MDK is an isolated finding, there is no increased risk of chromosomal anomaly. Cases with bilateral involvement (1 out of 15 cases) are associated with severe oligohydramnios and pulmonary hypoplasia; they carry a poor progno
  7. This appearance can be observed in the second trimester. The patterns may evolve, and the size of the kidneys may continuously increase during the third trimester. Other findings include oligohydramnios and lung hypoplasia, and therefore, the prognosis is usually poor. There are no other malformations associated with ARPKD. The list of differential diagnoses includes glomerulocystic disease and Bardet-Biedl syndrome (BBS) (see later).79,80 In these two diseases, the parenchyma is more homogenous than in ARPKD. When the kidneys are not as enlarged (+2 → +6 SD), the diagnosis may be more difficult to ascertain. A hypoechoic outer cortical rim that is usually present in the recessive type may help in suggesting the diagnosis (see Fig. 16-30C). Cysts (greater than 3 mm) may be already apparent in the fetus in the third trimester; they may appear unilateral, and their number varies. This finding occurs in one third of diagnosed fetal cases but is not specific.81 Coronal and transverse scans of a 22-week fetus show bilateral large, diffusely hyperechoic kidneys (calipers) with loss of corticomedullary differentiation
  8. These patients have a better prognosis when the amniotic fluid volume is preserved. The differential diagnosis includes metabolic disorders and intrauterine growth restriction. The liver abnormalities, for example, hepatic fibrosis, are not demonstrated in utero Coronal scan of a 30-week fetus shows markedly enlarged kidneys (calipers), with a hypoechoic peripheral rim surrounding the centrally increased echogenicity. Right kidney measures 7.5 cm in length and left kidney 8 cm. (B) Coronal scan of a different fetus at 31 weeks’ gestation shows markedly enlarged kidneys (calipers), with hyperechoic pyramids (arrowheads), resulting in “reversed corticomedullary differentiation.”
  9. In the fetus, a pattern highly suggestive of the disease has been recently described by Brun et al90 and was encountered in more than 80% of affected patients in the third trimester. They found moderately enlarged kidneys (1-2 SD above the mean) which have hyperechoic cortices and relatively hypoechoic medullae For the authors, the hyperechoic cortex is probably related to the presence of multiple microcysts that predominate at this stage in the cortex. In most cases, the amniotic fluid volume was normal.90 Although the exact sensitivity of the pattern is unknown, its demonstration should prompt a familial inquiry including sonography of the parents and grandparents because 50% of cases with the dominant form are discovered following the sonographic detection. However, it is estimated that an unknown percentage of cases of ADPKD are the result of a new mutation. A list of differential diagnoses exists for this pattern and should be considered (Table 16-7 and Fig. 16-33). For some patients, only long-term follow-up will establish the final diagnosis. Coronal scan of 30-week fetus shows enlarged kidneys (calipers), measuring 5 cm in length (>3 SD), with increased corticomedullary differentiation due to increased echogenicity of the cortex.
  10. n type I or primary obstructed megaureter, there is proximal dilation due to relative obstruction by a short aperisaltic segment of the ureter near the vesicoureteral junction. he transvesical course and insertion of the ureter are normal. It is thought to be due to muscular derangement with increased connective tissue in the afected segment of the ureter. Type II or reluxing primary megaureter is usually the result of an abnormality involving or closely associated with the vesicoureteral junction including a short or absent intravesical ureter, a con- genital paraureteric diverticulum, or another abnormality of the vesicoureteral junction. Type III, or nonreluxing, nonobstructed primary megaureter, is the most common of the three types. As the name suggests, neither relux nor apparent obstruction of the distal ureter is found. he dilation starts just above the bladder, and the cause is unknow
  11. Coronal scan of a 17-week fetus shows a greatly distended bladder (B) that occupies the entire abdomen. The thorax (arrows) is compressed and bell shaped because of pulmonary hypoplasia. There is anhydramnios. P, Placenta.
  12. Longitudinal scan of the lower fetal abdomen in a 29-week fetus shows an irregular mass on the anterior abdominal wall (arrows), below the umbilical cord insertion (U). A luid-illed bladder was not identiied. Note normal amniotic luid volume. (B) Corresponding photograph shows the exposed bladder (arrows) below the umbilical cord (U).
  13. The prognosis of antenatal neuroblastoma is excellent even when hepatic metastases are present. Furthermore, some cases of spontaneous involution have been reported (Fig. 16-66). Bilateral calcifications are unusual and may suggest Wolman syndrome (Fig. 16-68). Isolated, uncomplicated small cysts may be observed at the level of the adrenals (see Fig. 16-67). Their size is usually small and they disappear spontaneously. Large cysts develop in association with the Beckwith-Wiedemann syndrome. These cysts may bleed, and their appearance becomes more complex