GU embryology

• Weigert-Meyer - associated with upper pole, obstruction
• Can also be associated with reflux (less commonly) than obstruction
• Persistent Chwalla's membrane between ureteral bud (ureter) and UG sinus (bladder) (fails to obliterate - so can range from completely obstructive to not
• A/w defect in trigone formation, so also a/w dysfunctional bladder
• If was completely obstructed in utero then will lead to cystic dysplastic kidney pole
• Types
• Intravesical - contained entirely within bladder
• Ectopic - has some portion permanently situated at bladder neck or in urethra
• and a/w duplex vs single system

Presentation

• Urosepsis
• Antenatal diagnosis
• Palpable abdominal mass - hydronephrosis from obstructed renal unit
• Urinary retention - prolapse of ureterocele into urethra and occluding (rare!!)

Emergent indications for puncturing ureterocele

• Septic
• Prolapsed  - causing bilateral hydro/bladder outlet obstruction
• If visible, aspirate/reduce with 14 Fr Angiocath

Treatment

• General principles - if no lower pole VUR, start with endoscopic incision/watering can, if already has lower pole VUR then go ahead with reimplantation (?)
• Endoscopic incision
• Ideally within first 1-2 mo of life
• After ureterocele endoscopic treatment, can get de novo reflux on that side
• More likely to get reflux with complete resection > 'watering can' approach
• Kennedy does single puncture to avoid reflux; may need second puncture if obstruction doesn't resolve
• Excision and reimplantation
• May need bladder neck reconstruction to prevent incontinence
• Ureteroureterostomy of upper ureter to lower pole
• Risks - obstruction, damage to lower pole ureter
• Upper pole partial nephrectomy
• If duplex system and upper pole not functional
• Avoids surgery at bladder level