1. Nephro-Urology Study Day 12th July 2018
Case 1
Nephro-Urology Study Day 12th July 2018
Presenter
Ravindar Anbarasan
Paediatric Urology Fellow

2. Oxygen at birth, then stable Urine output 3 ml/kg
35/40 boy
RDS - NICU admission
Oxygen at birth, then stable
Urine output 3 ml/kg
26 weeks - Mild unilateral HN
resolved on further scans
D2 Creatinine 102 and 126
D2 USS KUB - Bilateral HUN (R > L)
Rest U & E normal

3. What is your next step?
Read Regional guidelines as not sure what to do
Insert a urethral catheter
Discharge and book a Renal USS in 6 weeks
Commence on prophylactic antibiotics, discharge
and refer to urology

4. Advice over telephone...
Indwelling Urinary catheter and free bladder drainage
Look for post-obstructive diuresis
liaison with nephrology (Dr Nagra), bloods
Prophylactic TMP

5. MCUG (Day 7)
no PUV
Grade 5 VUR (Right)
Grade 4 VUR (Left)

6. PLAN No active urology intervention - follow up USS 8/52
No active urology intervention - follow up USS 8/52
Nephrology follow up for VUR with renal impairment
Due to significant bilateral reflux - at risk of incomplete bladder emptying (due to re-filling) - urinary stasis and UTI.
TMP prophylaxis 2mg /kg.
If breakthrough infection - low threshold for offering circumcision +/- STING.

7. USS 8/52 Lk normal - 5.6cm RK smaller - 3.9 cm Normal bladder
6mm right ureter behind bladder
Lk normal - 5.6cm
RK smaller cm
some cortical thinning
APD 7 mm
dilated ureter throughout its length
urothelial thickening

8. No breakthrough UTI
“Does he need any Intervention ??”

9. What management option would you choose?
Conservative management, serial USS, monitoring of renal function
Conservative management with antibiotic prophylaxis
Circumcision
Bilateral STING
Circumcision + STING
There is no ‘correct’ answer here. Interesting to see opinion!

10. Cystoscopy and endoscopic correction of bilateral vesicoureteric reflux (5 months age)
Bilateral gaping ureteric orifices, slightly cranial to respective outer ends of the transtrigonal bar. Right UO is grossly gaping and admits 9Fr. scope easily. left UO is also gaping but does not admit the scope
Deflux injection to right UO - HIT -0.5 ml and STING 0.4 ml, Left UO - STING -0.5ml. Satisfactory mounds raised both sides.

11. Mum and dad preferred to defer circumcision
Small scarred RK, reduced function
compensatory functional enlargement LK
Last clinic in July 2018 – Doing well, No UTIs, ongoing nephrology care

12. Nephro-Urology Study Day 12th July 2018
Case 2
Nephro-Urology Study Day 12th July 2018
Presenter
Ravindar Anbarasan
Paediatric Urology Fellow

13. 38/40, girl, maternal drug abuse
Foster care – awaiting adoption
Transfer to nephrology for urosepsis and AKI
Foster parents not aware of any antenatal concerns

14. on IV ceftriaxone
Urine culture:
enterococcus - resistant to ceftriaxone
sensitive to amoxicillin
Changed to PO amoxicillin
urinary retention
Difficult catheterisation by Urology Specialist Nurse - large volume in bladder
polyuric post catheterisation
several days of IV fluid support

15. What are the possible Differentials?
Ureterocoele
Neuropathic bladder
Urogenital sinus anomaly
Pre-sacral mass
Rhabdomyosarcoma
All of the above
Some of the above
Ans. F

16. U&E’s normalise what is your next step?

17. USS on arrival
Bilateral HUN
thick walled bladder

18. X-ray & USS Spine

19. What next ??

20. What’s Going On? What Next ?
Markedly trabeculated bladder
Grade 4 bilateral VUR
Normal AP imaging of the urethra
MCUG
What’s Going On? What Next ?

21. EUA cystovaginoscopy normal perineum, urethra, vagina, anus Cystoscopy
normal urethra, bladder neck
orthotopic bilateral UO but very gaping
trabeculated bladder with diverticulae
catheter related cystitis cystica

22. Functional BOO !!

23. How to facilitate urinary tract decompression?
Clean intermittent catheterisation
Insertion of suprapubic catheter
Vesicostomy
Ureterostomy
Bilateral percutaneous nephrostomy

24. Clean Intermittent Catheterisation ? Issues...
Drainage needed for long term
Difficulty in catheterisation
Foster care

25. laparoscopy assisted left loop ureterostomy

26. MRI Spine
tiny speck of intrathecal high signal – may represent a tiny filum lipoma
Otherwise, normal intraspinal appearances
conus terminates at L1
Posterior elements intact

27. Nephro-Urology Study Day 12th July 2018
Case 3
Nephro-Urology Study Day 12th July 2018
Presenter
Ravindar Anbarasan
Paediatric Urology Fellow

28. 20:45 hours Male baby Respiratory distress
Abdominal distension at birth
Resuscitated by aspiration of 250 ml straw coloured fluid from abdomen
PROM 34/40, Emergency CS not in labour Apgars at 1/5: 1 at 1 min : 7 at 5 min Birthweight: 2715g

29. Antenatal history Antenatal scan showing echogenic bowel
Counselled by paediatric surgeon
Normal liquor at 30 weeks
Sibling - chromosome 7 deletion and cardiac anomalies
to represent a blockage in the baby's bowel. Sometimes, these appearances are associated with cystic fibrosis but Alexandra has been screened and is negative for common mutations. It would therefore be more likely that the baby has a bowel blockage for some other, currently unknown reason

30. 00:20 hours
Gonads, phallus, anus, probing, anal skin tag, not passed urine or meconium

31. What are your thoughts about gender at this stage?
MALE
FEMALE
DSD

32. AXR
U+E CREAT 67

33. 02:30 AM Next most appropriate investigation ?
USS
Ascitic fluid biochemistry
Karyotype
Contrast study
Laparoscopy

35. Ascitic Fluid biochemistry
Fluid creatinine 445

36. Possible Diagnosis ? Posterior Urethral Valves
Congenital Adrenal Hyperplasia
Cloacal anomaly
Bilateral Vesicoureteric reflux

37. Reassessment 8AM
500 ml in the next 6 – 8 hours via left percutaneous drain
Increasing abdominal distension
No passage of urine or meconium
Laparotomy

38. Laparotomy
On unit, distended bladder, huge distended vagina occupying peritoneal cavity, decompressed, distended sigmoid, open bladder, passage of catheter preferentially into vagina, atypical cloacal anomaly, streak ovary

39. Classic Persistent Cloaca

40. Posterior Cloaca Variant

41. THANK YOU