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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.