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4/2/2015 1 Pediatric Genitourinary Imaging Sadaf T. Bhutta Radiology Review Course April 2 nd 2015 Case 1 What is the diagnosis? 1. Multicystic dysplastic disease. 2. Reflux nephropathy 3. Pyelonephritis 4. Autosomal recessive polycystic kidney disease

Pediatric Genitourinary Imaging - CloudCME

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Page 1: Pediatric Genitourinary Imaging - CloudCME

4/2/2015

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Pediatric Genitourinary Imaging

Sadaf T. BhuttaRadiology Review Course

April 2nd 2015

Case 1

What is the diagnosis?1. Multicystic dysplastic disease.2. Reflux nephropathy3. Pyelonephritis4. Autosomal recessive polycystic kidney disease

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8 month old with right sided VUR

International Reflux study committee grading system

I: Reflux into ureter not reaching renal pelvis

II: Reflux reaching pelvis but not blunting calyces

III: Mild Calyceal blunting

IV: Progressive calyceal and ureteral dilation

V: Very dilated and tortuous collecting system, intrarenalreflux

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SFU grading for hydronephrosis

15 yr old renal Tx pt with h/o fever and abdominal pain

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17 yr old with h/o persistent fevers and right flank pain

PEDIATRICS Volume 128, Number 3, September 2011

CLINICAL PRACTICE GUIDELINEUrinary Tract Infection: Clinical Practice Guideline for the Diagnosis and Management of the Initial UTI in Febrile Infants and Children 2 to 24 Months

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16 yr old with acute left flank pain

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Case 1

What is the diagnosis?1. Multicystic dysplastic disease.2. Reflux nephropathy3. Pyelonephritis4. Autosomal recessive polycystic kidney disease

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Case 2

What is the diagnosis:1- Chronic kidney disease2- Multicystic dysplastic kidneys3- Autosomal recessive polycystic kidney disease4- Autosomal dominant polycystic kidney disease

Liver US

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ARPKD

Single gene disorder characterized by bilateral, symmetrical cystic renal disease involving distal convoluted tubules and collecting ducts

Hyperechoic kidneys, poor cortico-medullary differentiation

Small cysts <1 cm in diameter, diffuse microcysts

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ADPKD

Multiple renal cysts > 1cm in diameter

Cystic organ involvement: kidneys (100%), liver (50%), pancreas, brain, gonads

Non-cystic organ involvement: cardiac valves (25%), hernias, colonic diverticula, cerebral aneurysms

Cysts start appearing within first decade of life, chronic renal insufficiency by 4th decade of life

4 month old with MCDK

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MCDK

Non-functioning kidney, dysplastic tissue

Multiple cysts ranging in size from 1- >10 cm

Second most common abdominal mass in neonate

Involutes with time

40% have contralateral renal abnormality:UPJO

VUR

Case 2

What is the diagnosis:1- Chronic kidney disease2- Multicystic dysplastic kidneys3- Autosomal recessive polycystic kidney disease4- Autosomal dominant polycystic kidney disease

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Case 3

What is the cause of the abnormality?1- Urethral injury2- Anterior urethral valves3- Posterior urethral valves4- Primary megaureter

Newborn with antenatal diagnosis of bilateral

hydronephrosis

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Posterior urethral valves

3 typesI: Most common, anterior fusion of plicae colliculiII: Rarest, longitudinal folds from verumontanum to bladder neckIII: windsock tissue distal to verumontanum

Distinct caliber change of urethra at level of valves

VCUG is still “gold standard”

US: angling the transducer towards the bladder neck may reveal dilated posterior urethra

“Key-hole” appearance is classic finding in PUV

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4 yr old with h/o unilateral hydroureteronephrosis

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Primary megaureter

Obstructive ureteral dilation above adynamic, normal caliber, short ureteral segment above ureterovesical junction (UVJ)

Diagnosis best on IVP or MAG3 renal diuretic scan

Etiology: Unknown, paucity of ganglion cells?, Hypoplasia of muscle fibers?

Case 3

What is the cause of the abnormality?1- Urethral injury2- Anterior urethral valves3- Posterior urethral valves4- Primary megaureter

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Case 4

What is the diagnosis?1- Testicular torsion2- Scrotal cellulitis3- Trauma4- Epididymo-orchitis

Epididymo-orchitis

Infectious inflammation of epididymis, testicle or both

Classic finding: Enlargement of the testes and/or epididymis with increased blood flow and small reactive hydroceles

Tx: Antibiotics

Recurrent episodes may lead to decreased fertility

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12 yr old with h/o acute scrotal pain

Testicular Torsion

Spontaneous or traumatic twist of testes and spermatic cord, resulting in occlusion/infarction

Usually testes twists medially, and can be manually de-torsed

Decreased or absent blood flow on color Doppler US

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Torsion of appendix epididymis

Case 4

What is the diagnosis?1- Testicular torsion2- Scrotal cellulitis3- Trauma4- Epididymo-orchitis

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Case 5

Most common solid abdominal mass?1- Neuroblastoma2- Hepatoblastoma3-Wilm’s tumor4- Multilocular cystic nephroma

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Wilm’s Tumor

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Wilm’s Tumor

Wilm’s tumor

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Wilm’s Tumor

Most common solid abdominal mass and most common renal tumor

Embryonic tumor with epithelial, blastemal and stromal elements

Peak incidence 2-3 years, African American at higher risk, 1% familial

Same incidence as Neuroblastoma

Wilm’s Tumor

Firm non-tender, painless mass in abdomen

Hematuria

Hypertension

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Wilm’s Tumor

Bulky and replaces most of the kidney

Usually solid with pseudocapsule, calcification rare

Local and regional extension to lymph nodes and extra-renal space

Renal vein/IVC, urothelial spread

Distant mets to lung and liver

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Neuroblastoma

10% of all childhood cancers

Most common extra-cranial solid malignancy of childhood

Second most abdominal cancer in older infant and child after Wilm’s tumor

Average age of diagnosis is 2 years

50 to 60% have metastases at time of diagnosis

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Neuroblastoma

Tumor of primitive neural crest cells (neuroblasts) that may arise anywhere in the sympathetic ganglion chin or adrenal medulla

Increased secretion of catecholamines and metabolites (HVA, VMA, norepinephrine or dopamine)

Usually silent till it invades adjacent structures, metastasizes or produce paraneoplastic syndromes (MEI, diarrhea with hypokalemia)

Mets to skeleton, bone marrow, liver, lymph nodes and skin

Neuroblastoma

Gross or microscopic Ca+2, well-demarcated but no capsule

Two-thirds of NB arise in abdomen, of which 2/3 arise in the adrenal gland

Differences from Wilm’s tumor

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Case 5

Most common abdominal solid abdominal mass?1- Neuroblastoma2- Rhabdomyosarcoma3-Wilm’s tumor4- Multilocular cystic nephroma

Case 6

Diagnosis?1- Sacrococcygeal teratoma2-Perforated appendicitis with appendicolith3-Ovarian teratoma4- Mesenteric cyst

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14 yr old with pelvic pain

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18 yr old with yolk sac tumor

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Case 6

Diagnosis?1- Sacrococcygeal teratoma2-Perforated appendicitis with appendicolith3-Ovarian teratoma4- Mesenteric cyst

Thank You!Questions?