Upper Tract Abnormalities

Cystic Renal Diseases

Disease Inherited? Renal Findings Non-renal Findings Treatment
(chromosome 6)
Large homogenous echogenic kidneys Hepatic fibrosis
Biliary dysgenesis
Respiratory support
May require nephrectomy due to compression
Splenorenal shunt for portal HTN
PKD1: 16
PKD2: 4
PKD3: not mapped
Cysts throughout renal parenchyma Diverticulitis
Liver/spleen/pancreas cysts
Mitral valve prolapse
Intracranial aneurysms
Seminal vesicle cysts (40%)
Control HTN (ACE/ARB)
Consider cyst drainage
Juvenile nephronophthisis
Medullary cystic kidney disease
(chromosome 2)
Corticomedullary junction cysts Retinitis pigmentosa (16%)
Hepatic fibrosis
Sodium repletion
Tuberous sclerosis Yes
TSC1: 9
TSC2: 16
Cysts + AMLs
RCC (3%)
Epilepsy (80%)
Facial angiofibromata (75%)
Developmental delay (60%)
Consider AML removal if > 4cm
(chromosome 3)
Cysts (76%) + adenomas
RCC (35-38%)
Cerebellar hemangioblastomas
Retinal angiomas
Pheochromocytomas (10-17%)
Pancreas/epididymis cysts
Partial nephrectomy for cancer control
Conisder bilateral nephrectomy
Multicystic dysplastic kidney No Minimal nephron development
Diffuse cysts
Nephrectomy for pain/hemorrhage
Benign multilocular cyst No Normal kidney compromised by growing cystic mass Partial/radical nephrectomy
Simple cyst No Single/multiple cysts
More common with increasing age
Depends on Bosniak classification
Medullary sponge kidney No Ectatic collecting ducts
Normal nephrons
KCit for stones
Abx ppx for recurrent UTIs
Acquired renal cystic disease No Diffuse cysts
Occurs with ESRD
Excision if mass > 3cm

Von Hippel-Lindau classification, from Campbell's

VHL screening for patients and family, from Campbell's

Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Juvenile Nephronophthisis (NPH) and Medullary Cystic Kidney Disease (MCKD) Complex

Tuberous Sclerosis

Von Hippel-Lindau (VHL)

Multicystic Dysplastic Kidney (MCDK)

Medullary Sponge Kidney (MSK)

Benign multilocular cyst (Cystic nephroma)

Acquired Renal Cystic Disease

Congenital nephrosis

Other diseases

Variety of supernumerary kidneys with one ureter, from Campbell's

Variety of supernumerary kidneys with two ureters, from Campbell's

Variety of crossed ectopia, from Campbell's

Variety of cross fused ectopia, from Campbell's

Malrotation: A embryonic, B normal, C incomplete, D hyper, E hyper, F reverse, from Campbell's

Retrocaval ureter, from Campbell's

Anterior-posterior diameter determination of antenatal hydronephrosis, from Campbell's

SFU grading of antenatal hydronephrosis, from Campbell's

Renal Anomalies

Abnormal renal number

Renal dysplasia

Renal ascent and fusion anomalies

Calyceal abnormalities

Vascular anomalies

Prenatally diagnosed urinary tract dilation (UTD)

Postnatal hydronephrosis management

SFU Grade If... Then...
Grade 1-2 Newly diagnosed Renal US in 1-6mo
Grade 3 Renal US in 1-3mo
Grade 4 Renal US + VCUG + renal scan in 1-3mo
Grade 1-2 Hydronephrosis resolved Follow up PRN
Hydronephrosis stable Renal US q6-12mo until resolved or toilet trained
Hydronephrosis after toilet training Space out or stop renal US
Grade 3 Improving hydronephrosis Renal US q6-12mo until resolved
Stable hydronephrosis Renal US q3-12mo until resolved
Worsening hydronephrosis VCUG + renal scan
Grade 4 Improving hydronephrosis Renal US q3-12mo until resolved
Stable hydronephrosis Renal US q3-6mo
Worsening hydronephrosis Pyeloplasty
Split function < 30-40%
Worsening renal function
Stable hydronephrosis + renal function > 40% Pyeloplasty vs surveillance

Ureterocele management, from Campbell's

Ureteral anomalies

Ureteral duplication

Neonatal hydroureteronephrosis



Ectopic ureter

Ureteropelvic junction obstruction (UPJO)

Principles and presentation

Surgical management

Ureterocele management, from Campbell's

Kidney Transplants


Procedural tips

Stone imaging algorithm, from Campbell's

Kidney Stones


Urine stone workup findings



Surgical management

Acute and chronic kidney disease

Acute kidney injury (AKI)

Chronic kidney disease (CKD)