Features of Potter’s Syndrome
Pulmonary Hypoplasia Low set ears Beaked Nose Prominent epicanthic folds Downwards slanting eyes Limb Deformity
Mechanism of Multicystic Dysplastic Kidney
Failure of union of the uterine bud with nephrogenic mesenchyme
Management of Multicystic Dysplastic Kidney
50% involuted by aged 2
Nephrectomy indications: very large/ hypertension develops
Causes of large Cystic Kidneys
Autosomal recessive/dominant polycystic Kidney disease
Multicystic Dysplastic Kidney
Tuberous Sclerosis
Presentation of Autosomal dominant polycystic kidney disease
Childhood hypertension
Renal failure in adulthood
Extra Renal feature of Autosomal Dominant Polycystic Kidneys
Cysts in liver and pancreas
Cerebral Aneurysm
Mitral Valve prolapse
Abnormal Caudal migration of the kidneys results in…
Horseshoe kidney or pelvic kidney
Duplex System is due to?
Premature division of the uterine bud
Complications of duplex system
Lower pole moiety- Reflux
Upper pole moiety- Ectopic drainage to urethra or vagina, prolapse into bladder, obstruction urging flow
Features of Absent Musculature Syndrome (Prune Belly)
Wrinkled appearance of abdomen
Large Bladder
Dilated Ureters
Crytorchidism
Site of urinary tract obstruction
Pelviuretric Junction
Vesicoureteric Junction
Bladder Neck
Urethra
Features of bilateral hydronehritis
Hydronephrosis
Hydroureters
Thickened bladder wall
Diverticula
Management of urinary tract anomalies
Start prophylactic antibiotics
US within 24hours, in males with bilateral hydronephrosis
-Normal: US repeat at 2-3 months, stop Abx
-Abnormal: MCUG and surgery
US within 4-6 weeks, in females or unilateral hydronephrosis
- Normal: US repeat at 2-3 months, stop Abx
-Abnormal: Further Ix
Renal Function Assessment in children
Plasma creatinine concentration: eGFR Insulin/EDTA GFR Creatinine Clearance Plasma Urea Concnetration
Radiological Investigation of Kidneys and Urinary Tract
Ultrasound: anatomy
DMSA scan: renal scarring (2 months after UTI- sensitive)
MCUG: detects reflux and urethral obstruction
MAG3 renogram (Children over 4 years): detects reflux, given with furosemide
Plain abdominal X-ray: spinal abnormalities
Features of UTI in infants
Offensive Urine Septicaemia Jaundice Poor Feeding Lethargy Irritability Vomitting Fever Febrile siezure
Features of UTI in children
Secondary Enuresis Dysuria Frequency Urgency Abdominal Pain Loin Tenderness Fever Anorexia Lethargy Vommitting and Diarrhoea Haematuria Cloudy Urine Febrile Seizure
dDx Dysuria
Girls- Cystitis or Vulvitis
Boys- Balanitis (Uncircumcised)
Collection of Urine Samples
Clean catch sample (recommended method) Adhesive plastic bag Urethral catheter Suprapubic aspiration (fine needle inserted above pubic symphysis under US guidance) Midstream sample- Older children
Dipstick Interpretation
Nitrate- positive indicates UTI
Leukocyte- present in fever without UTI, balanitis, and cystitis
Blood/Protein/Glucose- not UTI specific
Predisposing factors UTI
Renal or urinary tract abnormality
Incomplete Bladder emptying
Vesicoureteric reflux
Incomplete bladder emptying factors
Infrequent voiding Vulvitis Incomplete micturition Obstruction from constipation Neuropathic bladder Vesicoureteric reflux
Anatomy of vesicoureteric reflux
Anomaly of vesicoureteric junctions
Ureters displaced laterally
Ureters enter bladder directly
Shorted or absent intramural course
Complications of vesicoureteric reflux
Incomplete Bladder emptying
Increased risk of infection
Risk of pyelonephritis
Renal damage due to pressure transmission