4. Incidence
1 in 1250 births.
M:F = 2:1
Bilateral in 10-36%
Most common site of congenital ureteral
obstruction.
5. Etiology
Two types of PUJO – Koff
Intrinsic – most common (adynamic)
Extrinsic – aberrant vessels, adhesive bands,
AVMs and Ostling folds.
Intra luminal – ureteral valves, fibro-
epithelial polyps
6. Pathogenesis
Chang and colleagues – deleted
Calcineurin B type 1 (CnB1) gene –
aperistalsis.
Wang and colleagues – antagonized
BMP4 and Nogin protein – inhibition of
smooth muscle formation – adynamic
segment.
7. Clinical features
Antenatal USG
Postnatally
Episodic flank pain
Abdominal pain
UTI
Dietl crisis – cyclic abdominal pain with
vomiting.
8. Diagnosis
Ante-natal : sonographic features
Pelviectasis
Normal amniotic fluid volume
No ureteral dilatation
Normal thickness of the bladder wall
Normal cycling of the bladder
9. Postnatal diagnosis
USG Abdomen
Highly accurate in the diagnosis of
hydronephrosis.
Pelviectasis and caliectasis, absence of
ureterectasis, normal bladder cycling and normal
bladder thickness
15. Surgery...
Incision
Flank incision – tip of twelfth rib towards the
umbilicus
Extra-peritoneal approach.
External oblique, internal oblique, latissimus
dorsi and serratus posterior divided
16. Surgery...
Diaphragm released from the tip of 12th
rib
Peritoneum separated from Gerota’s fascia
Self retaining retractor
Kidney mobilized
Ureter identified, looped, traced towards
kidney.
21. Excision of PUJ and spatulation of the ureter
Ureteral stent/nephrostomy tube
Anastomosis – lower lip of renal pelvis to the
spatulated apex of ureter.
Posterior layer first - continuous or intermittent
6-0 or 5-0 sutures
Suturing continued until pelvis closed.
24. Laparoscopy
Infants vs older children
Pain
Length of hospital stay
Population based comparison of laparoscopic and open pyeloplasty in paediatric‐
pelvi ureretic junction obstruction - John Knoedler, Leona Han, Candace Granberg,‐
Stephen Kramer, George Chow, Matthew Gettman, Brittany Kimball, James Moriarty,
Simon Kim, Douglas Husmann