VARIOUS CASE SENERIOS

BY Dr CHARU KALRA Dr BHANU KUMAR BANSAL Dr NISHANT JAIN Dr ANUPAM CHATURVEDI Santokbha Durlabhji Memorial Hospital cum Research Institute, JAIPUR

OVER VIEW

INTRODUCTION

CASE 1 - PRUNE BELLY SYNDROME

CASE 2 - AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE

CASE 3 - POSTERIOR URETHERAL VALVE

TAKE HOME MESSAGE

INTRODUCTION

Hydronephrosis is the most common abnormality detected on prenatal USG.

It accounts for about 50% of all prenatally detected defects.

Fetal kidneys can be visualized by the 14th to 15th week of gestation.

By the 20th week of gestation, the internal architecture of the kidneys can be assessed.

SFU Grading for fetal hydronephrosis

ETIOLOGY OF FETAL HYDRONEPHROSIS

Physiological hydronephrosis :

Ureteropelvic junction obstruction:• Complete ureteral duplication• Ectopic ureterocoele• Congenital megaureter

Ureterovesical stenosis:

Bladder outlet obstruction:• Posterior urethral valve• Urethral atresia• Cloacal malformation• Prune belly syndrome• Megacystic-microcolon-intestinal hypoperistalsis syndrome

Case 1G1 P1 PT (36th wk) LSCS (gestational DM, pre-eclampcia with antenatal USG - b/l gross fetal hydronephrosis with oligohydramnios) with APGAR 2 (1”) & 5 (5”), admitted with respiratory distress.

Child required CPR and immediately ventilated.Child also had circulatory shock - inotropes started.

O/E Anasarca, cyanosis, BP = 40/14 (28). P/A: Grossly distended, flanks full, b/l kidneys palpable and enlarged, bladder distended & scrotal sacs – empty. R/S: b/l reduced air entry & signs of respiratory distress present.

Investigation:Na = 134, K = 5.3, BUN = 30, Cr = 1.2CXR : b/l lung hypoplasia & diaphragm elevated

USG abdomen: b/l gross hydronephrosis with very thin renal cortex & distended bladder with b/l PUJ obstruction

• Course during hospitalization:

Uretheral catheterisation done immediately with difficulty

USG guided b/l nephrostomy done, 80 ml urine drained & rt. kidney completely decompressed

Planned for definitive surgery

Patient deteriorated d/t pulmonary hypoplasia and expired at 36 hrs of life despite optimal management

• Probable diagnosis - Prune Belly Syndrome

• Proposed line of treatmentAntenatal:

• Fetal per-cutaneous puncture for gross hydronephrosis.

Post natal: • Adequate ventilatory support• Immediate decompression of kidney • Definitive surgical procedure for correction

of GU anomalies.

Case 2

G1P1 FTNVD, antenatal mild hydronephrosis, out-born, received on LD – 4 with c/o poor feeding and reduced urine out-put. No h/o perinatal depression.

O/E

– No edema, BP = 90/50 (64)

– P/A: soft, mild distention, b/l kidneys palpable, bladder distended, liver 2 cm BCM – soft.

• Investigation :

Hb = 14.6 TLC = 24000, N = 78, L = 14, Plt = 1.2 lac CRP = 3.4

Na = 134, K = 6.0, Cr = 7 BUN = 120

– CXR : normal study– USG Abdomen: multiple

hypoechoic cysts in b/l kidneys & solitary hypoechoic cyst in liver.

• RFT monitored 12 hrly for initial 2 days – gradually improved.

• Renal & Liver biopsy not done.

Treatment :

Furosemide

Reno-protective antibiotics.

Follow-up : thriving well.

Probable Diagnosis - Neonatal ARPKD

Proposed line of treatment :almost always symptomatic treatment of portal hypertension,UTI,cholangitis &rarely a liver transplant

Case 3

G2P1 FT, LSCS (NPOL), 2nd &3rd trimester USG - b/l moderate hydronephrosis, admitted on LD2 with hematuria with urethral catheter in situ.

O/E No oedema, BP = 60/40 (47)P/A - distended, flank fullness, bladder distended,

b/l kidneys palpable

Investigations :

Hb = 15.2, TLC = 14,000, Plt = 2,20,000 CRP = 0.6

Cr =1.2, BUN = 30, Na = 134, K = 4.2

Urine R/M - RBC = 6-7/hpf, WBC = Nil

USG Abdomen - b/l moderate hydro-ureteronephrosis with distended bladder & dilated posterior urethra s/o PUVVCUG

Treatment :Cystoscopic transurethral valve ablation (with electrocautry)

Out-come: Patient thriving well on follow-up.

Diagnosis - Posterior Urethral Valve

Proposed line of treatmentIf renal functions (S. Creatinine level) normal

• Transurethral valve ablation with

– Cold knife

– Electro-cautery – Laser energy

If renal functions not normalise on catherisation

• More proximal diversion (pyelostomy, ureterostomy)

If the urethra is too small to accept the small cystoscope

• Vesicostomy or more proximal upper-tract diversion (pyelostomy, cutaneous ureterostomy) depending on renal functions

TAKE HOME MESSAGE

Oligohydromnios – Best predictor of poor neonatal outcome.

Once the diagnosis of prenatal hydronephrosis is made, serial ultrasounds are often needed.

Not all cases of antenatal hydronephrosis requires aggressive antenatal invasive procedure.

Regular AN followup &Comprehensive team approach.