Upper Tract Abnormalities

Cystic Renal Diseases

Disease Inherited? Renal Findings Non-renal Findings Treatment
ARPKD Yes
(chromosome 6)
Large homogenous echogenic kidneys Hepatic fibrosis
Biliary dysgenesis
Respiratory support
May require nephrectomy due to compression
Splenorenal shunt for portal HTN
ADPKD Yes
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
Yes
(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
VHL Yes
(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

UTD classification system system, from Campbell's

Antenatal UTD risk groupings, from Campbell's

Postnatal UTD risk groupings, 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

UTD class If... Then...
P1 Newly diagnosed Renal US in 1-6mo
P2 Renal US in 1-3mo
P3 Renal US + VCUG + renal scan in 1-3mo
P1 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
P2 Improving hydronephrosis Renal US q6-12mo until resolved
Stable hydronephrosis Renal US q3-12mo until resolved
Worsening hydronephrosis VCUG + renal scan
P3 Improving hydronephrosis Renal US q3-12mo until resolved
Stable hydronephrosis Renal US q3-6mo
Worsening hydronephrosis Pyeloplasty
Split function < 30-40%
Worsening renal function
UTIs
Stable hydronephrosis + renal function > 40% Pyeloplasty vs surveillance

Ureterocele management, from Campbell's

Ureteral anomalies

Ureteral duplication

Neonatal hydronephrosis

Megaureter

Ureterocele

Ectopic ureter

Ureteropelvic junction obstruction (UPJO)

Principles and presentation

Surgical management

Ureterocele management, from Campbell's

Kidney Transplants

Preparation

Procedural tips

Stone imaging algorithm, from Campbell's

Kidney Stones

Considerations

Urine stone workup findings

Prevention

Medications

Surgical management

References