EMBRYOLOGY AND
ANOMALIES OF THE
GENITOURINARY
TRACT
Omar Alsoghier,MD
THE
KIDNEY
THE
CLOACA
Genital Development
Genital differentiation
Normal IVU
RENAL ANOMALIES
No.
Rotation
Ascent
Vasculature
Collecting system
Anomalies of Number
• 1- Bilateral renal agenesis:
incompatible with life (Potter
syndrome).
• 2- Unilateral agenesis: concomitant
absence of ureter and hemitrigone in
50%. Solitary kidney supports life.
Renal agenesis. Note the presence of a normal
right adrenal (arrow) in this patient with agenesis of
the right kidney
Anomaly of ASCENT
• 1- Ectopic pelvic kidney
• 2- Ectopic thoracic kidney
• 3- Crossed ectopia: both kidneys on
one side; S- or L-shaped kidney.
Anomaly of ASCENT
Cranial ectopia: lies immediately beneath
the hyperechoic diaphragm (large black arrows)
Horse-shoe kidney
• 1:400.
• Isthmus: point of fusion in the midline;
fibrous of parenchymatous. Hinged by the
inferior mesenteric artery.
• Diagnostics: low sited, Renal axes meet in
the pelvis, pelves and ureters face
anteriorly, a calyx is present medial to the
pelvis.
Horse-shoe kidney
Complications of renal anomalies
• Obstruction: at UPJ…. poor drainage.
• Infection.
• Stone formation.
• More liable to trauma.
• Obstructed labor, IVC obstruction: rare
Cystic disease of the kidney
•Congenital: Congenital cystic dysplasia (MCD)
•Genetic:
Autosomal recessive polycystic kidney disease (ARPKD),
Autosomal dominant polycystic kidney disease (ADPKD),
Medullary cystic kidney disease complex (NMCD)
•Acquired: Simple cysts, acquired cystic disease of
dialysis
•Cysts associated with systemic disease:VHLS, DS.
•Malignancy: cystic renal cell carcinoma (cRCC)
Adult Rt.
PCDK
Adult polycystic kidney disease
• 1:1250, represents 10% of ESRD.
• Autosomal dominant, bilateral.
• Failure of fusion between secreteory and
excretory portions of nephron.
• Age of presentation: 40-50: renal pain,
hypertension, progressive RF, hematuria,
resistent UTI., abdominal mass.
• Association: cystic disease liver &lung.
Cerebral aneurysm, cardiac valve prolapse.
UPJO
UPJO
UPJO; radio-isotope scan
URETERAL ANOMALIES
1) Ectopia
2) Ureteroceles
3) Ureteral Stenosis and Stricture (Obstructive
MU)
4) Ureteral Valves
5) Spiral Twists and Folds of the Ureter
6) Ureteral Diverticula
7) Ureteral Triplication and Supernumerary
Ureters
8) Vascular Anomalies Involving the Ureter :
RCU
Ureterocele
Prolapsed ureterocele
Megaureter; obstructive
Megaureter; obstructive
Epispadias
Urachus
Urachal anomalies
Bladder anomalies, exstrophy
• Muskloskeletal defects; bony, soft tissue
• Urinary defects;
• Genital defects; complete epispadias, dorsal
chordee, short broad phallus, + testicular
maldescent, in female: Vagina ant. disp.
• Anorectal defects; ant disp anus, broad
perineum, + rectal prolapse.
Management of Exstrophy
• Functional repair:
-Early single stage within 3 days.
-Late multistage if delayed; (1) abd wall &
bladder closure, osteotomy is needed
(2)Epspadias repair at 1.5 y (3) BN repair at
age of toilet training 5 ys.
• Excision of bladder patch and urinary
diversion in neglected cases.
Exstrophy closure
Male Exstrophy
Female Exstrophy