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

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

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

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

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

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.

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 - 3.9 cm some cortical thinning APD 7 mm dilated ureter throughout its length urothelial thickening

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

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!

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.

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

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

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

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

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

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

USS on arrival Bilateral HUN thick walled bladder

X-ray & USS Spine

What next ??

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 ?

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

Functional BOO !!

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

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

laparoscopy assisted left loop ureterostomy

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

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

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

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

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

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

AXR U+E CREAT 67

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

Ascitic Fluid biochemistry Fluid creatinine 445

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

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

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

Classic Persistent Cloaca

Posterior Cloaca Variant

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