Abnormal Development Of The Kidney And Ureters Flashcards
All the subtopics under Abnormal development of Kidneys and Ureters
1.Anomalies of Renal Volume and Structure
- Simple Cyst
- Polycystic Kidney
- Multicystic Kidney
- Medullary Sponge Kidney
2.Anomalies of number
- Renal agenesis
- Duplex Kidney
3.Anomalies of ascent or fusion
- Renal ectopia
- Horseshoe Kidney
4.Anomalies of Ureters
- Ectopic Ureter
- Ureterocele
- Ureteropelvic junction obstruction [UPJ]
- Vesicoureteral Reflux
- Megaureter
Simple Cyst
Definition: Oval to round cyst within or on surface of Kidney. It is bordered by cuboidal epithelium and is filled with transudate-like clear fluid. Cysts could be singular or multiple, unilateral or bilateral
Pathology: Fetal form of epidermal growth factor mitogens necessary for nephrogenesis. They induce cyst formation if not modulated
Incidence: 1-50% depending on age
Symptoms:
- Undiscovered or found incidentally, size of cysts remains unchanged and rarely call attention
- Can produce abdominal mass or pain
- Hematuria secondary to rupture into Pyelocalyceal system
- Hypertension secondary to segmental ischemia
Dx:
- Ultrasound
- CT for tumour suspection/needle aspiration/MRI
Treatment:
- Only when benign cyst causes Pyelocalceal obstruction
- Unroofind of cyst or percutaneous aspiration and injection of sclerosing agent
- Partial or radical nephrectomy for carcinoma suspection
Polycystic Kidney Disease
Definition: bilateral kidney are full of various sized cysts
Pathology: AR disease with generalized fusiform dilations of the collecting tubules representing cysts
Incidence: ~0,01%
Symptoms:
- Oligohydramnios, respiratory distress and hypoplasia in perinatal stage
- Renal failure and Hypertension in jeuvenile stage
- Associated cysts in liver is also a common finding
Dx:
- Ultrasound identifies PKD
- IV urography or CT with contrast show functioning capacity
Treatment: No cure has been found. Respiratory care in neonate and Renal transplantation most common choices
Multicystic Kidney
Definition: Unilateral kidney is full of variable sized and communicating cysts. Kidney has “grape” like appearance
Pathology: extreme form of Hydronephrosis secondary to Renal pelvis/Ureteropelvic junction/Ureteric obstruction
Incidence: most common type of renal multiplex cystic disease
Symptoms: most common cause of abdominal mass in infants. Can also cause abdominal pain, hematuria and hypertension
Dx:
- Ultrasound is first study performed
- IV pyelography and in difficult cases radioisotope studies with Tc-99 DMSA may be helpful
Treatment: Nephrectomy can be approved in abdominal pain, gross hematuria or hypertension cases
Medullary Sponge Kidney
Definition: radiographic features give cross-sectioned kidney appearance of a sponge
Pathology: Dilated intrapapillary collecting ducts and small medullary cysts
Incidence: ~0,02%
Symptoms: usually occurs after age of 20
- Renal colic
- UTIs
- Gross hematuria
- Hypertension
- Urinary stones
Dx:
- IV urography (more sensitive)
- CT or US (less sensitive)
- Enlarged kidneys with calcifications in the papillae, elongated papillary tubules that fill with contrast
Treatment: requires management
- Infections treated with antibiotics
- Stones removed by extracorporeal lithotripsy and percutaneous nephrolithotomy
- Thiazides to treat hypercalciuria, also limits stone formation
Renal Agenesis
Definition: Unilateral or bilateral complete absence of kidney
Pathology: absence of nephrogenic ridge or failure of a ureteral bud to develop from the Wolffian duct will lead to agenesis
Incidence: 0,025%
Symptoms: no specific symptoms. Bilateral in fetal life leads to oligohydramnios and pulmonary hypoplasia [failure to thrive post birth]
Dx:
- Renal US
- Excretory uropathy
- CT
- Radionuclide imaging
- Compensatory hyperplasia to the normal kidney
Treatment: children born cannot thrive beyong 2 days due to respiratory distress in bilateral agenesis. No treatment has been found for this disease
Duplex Kidneys
