Peds -- Gu

Front 1

1. Where do neuroblastomas arise from?
2. What is the difference b/w ganglioneuroma, ganglioNB, and NB?
3. Do majority of NB arise in the abdomen or elsewhere?

Back 1

1. Adrenal medulla and sympathetic ganglia.
2. Ganglioneuroma, ganglioNB, NB differ in the degree of cellular differentiation.
3. 70% arise in the abdomen; the remaining arise in the chest, head, and neck. Neurblastoma arising in the chest has a better prognosis.

Front 2

1. What is the blood supply to the ovary?
2. Why is the ovary enlarged in torsion?
3. Is arterial flow always absent in torsion?

Back 2

1. Uterine artery and ovarian artery
2. The low-pressure venous system is the first to occlude, causing edema and ovarian enlargement. As the vascular compromise grows, ischemic changes increase with transudative fluid leaking out of the dying cells. This creates prominent follicles as well as free fluid in the cul-de-sac.
3. Doppler ultrasound may demonstrate the presence of arterial flow but this may be secondary to recruited arteries from the uterine artery.

Front 3

1. What is the etiology of primary megaureter?
2. How do you differentiate primary megaureter from VUR?
3. How do you assess the functional signifance of the ureteral obstruction?

Back 3

1. Aperistaltic fibrotic segment just proximal to the ureterovesicular junction that causes obstruction. Think of it like Hirschsprung disease of the ureter.
2. There is absence of reflux in primary megaureter.
3. Nuclear renal scintigraphy or Whitaker test.

Front 4

1. What are findings associated with bilateral renal agenesis?
2. What is the appearance of adrenal gland in renal agenesis?
3. What GU malformations are associated with renal agenesis?

Back 4

1. Bilateral agenesis results in
- oligohydramnios
- prematurity
- Potter facies with low-set, floppy ears
- prominent epicanthal folds
- micrognathia
- pulmonary hypoplasia-pneumothorax
2. The adrenal gland is ENLARGED and has a DISCOID shape.
3. Renal agenesis may be associated with genital malformation.
- In girls: hydrometrocolpos, vaginal atresia, vaginal septum.
- In boys: cryptoorchidism, hypospadias, seminal vesicle cyst, and absent testes.

Front 5

What is DDX of enlarged kidneys?

Back 5

Nephroblastomatosis
Nephrotic syndrome
Polycystic kidney disease
Glycogen storage
Lymphoma/leukemia

Front 6

1. What is nephroblastomatosis?
2. What is the imaging appearance of nephroblastomatosis?
3. What findings suggest conversion of nephroblastomatosis into Wilms tumor?
3. What are some congenital abnormalities associated with Wilms tumor?

Back 6

1. Persistence of METANEPHRIC tissues in the kidneys. It is considered a precursor to Wilms tumor (1/3 of pts progress to Wilms tumor). US screening is performed every 3 months till the age of 7 to detect development of Wilms tumor. Wilms tumor tends to be heterogeneous, whereas nephroblastomatosis is homogeneous.
2. It can present as a rind of soft tissue around the kidneys or it may present as multiple masses in the kidneys. Found in nearly all bilateral Wilms tumor.
3. Rapid change in size and heterogeneous enhancement.
4. 15% of all children with Wilms' tumor have an associated congenital abnormality.
- 1% of patients have sporadic aniridia (absence of iris).
- Beckwith-Weidemann
- Hemihypertrophy
- WAGR

Front 7

1. What are bladder diverticula?
2. What is the etiology of bladder diverticula?
3. What is the sequelae of bladder diverticula?

Back 7

1. Mucosal outpouchings through the muscular layer of the bladder wall.
2.
- CONGENITAL: Located adjacent to or involving the UVJ (Hutch diverticula).
- ACQUIRED: result from outlet obstruction (posterior urethral valve) whereby the mucosa extrudes between hypertrophied muscular bundles.
3.
- Obstruction if they involve the ureters or produce a mass effect on the bladder.
- Diverticula lead to stasis, infection, and stone formation.

Front 8

1. What are the different types of renal hypoplasia?
2. What are the characteristics of simple hypoplasia?
3. What are the characteristics of segmental hypoplasia?
4. What are the characteristics of oligonephronic hypoplasia?

Back 8

1. 3 varieties of hypoplasia: simple hypoplasia, segmental hypoplasia, and oligonephronic hypoplasia.
2. Simple hypoplasia:
- congenitally small or miniature kidney.
- Ectopia or malrotation increase risk of recurrent infections and scarring.
3. Segmental hypoplasia:
- congenital or acquired process affecting a segment of the kidney resulting in cortical atrophy and dilation of the collecting system due to VUR and pyelonephritis. In the context of hypertension this finding is referred to as Ask-Upmark kidney.
4. Oligonephronic hypoplasia:
- bilateral extremely small kidneys with a reduced number of nephrons eventually leads to renal failure.

Front 9

1. What are the congenital causes of hydrometrocolpos?
2. What are the acquired causes of hydrometrocolpos?
3. What is hydrometrocolpos associated with?

Back 9

1. Imperforate hymen, vaginal septum, vaginal atresia, vaginal diaphragm, rudimentary uterine horn.
2. Endometrial tumors, cervical tumors, post-irradiation fibrosis.
3. Imperforate anus, dysraphic abnormalities of the vertebrae, urinary tract abnormalities.

Front 10

1. What are the imaging manifestations of multilocular cystic nephroma?
2. What is the demographics for MLCN?
3. What other tumors have similar imaging findings?

Back 10

1. Multiple noncommunicating cysts of variable size that tend to be herniated into the renal pelvis.
2. Young boys (<4 years old) and adult females.
3. Cystic Wilms tumor and multicystic dysplastic kidney. Because of the possibility of Wilms tumor, the kidney is resected.

Front 11

1. What is a posterior urethral valve?
2. What are the imaging findings in PUV?
3. What is included in the DDX?

Back 11

1. A posterior urethral valve is not a valve...it is abnormal fusion of normal mucosal folds within the urethra that obstructs urine.
2.
- Dilation and elongation of posterior urethra.
- Thickened trabeculated bladder
- Reflux
- Hydronephrosis
- Urinary ascites/urinoma.
- "Keyhole sign due to dilatation of posterior urethra.
3. Neurogenic bladder:
- bladder is trabeculated (Christmas tree appearance)
- however, posterior urethra is not dilated.

Front 12

1. What is the most common ovarian tumor in pediatrics?
2. What is another name for cystic teratoma?
3. What is a dermoid plug?

Back 12

1.
- Simple ovarian cyst: seen in the neonatal or premenarchal period 2/2 hormonal stimulation.
- Germ cell tumors: especially teratomas
2. Cystic teratomas are also known as dermoids due to their predominantly ectodermal make up (teeth, skin, hair, oil).
3. Mural nodule that is echogenic (tip of the iceberg) due to echogenic calcium and hair that prevents transmission of sound waves obscuring the posterior wall of the lesion.

Front 13

1. What are the imaging findings in ARPCKD?
2. What are some other associated abnormalities seen with ARPCKD?
3. What are the different classes of ARPCKD?

Back 13

1. Bilateral enlarged, hyperechoic kidneys. Cysts are not visualized as they are below the resolution of ultrasound.
2. Congenital hepatic fibrosis and pulmonary hypoplasia and PTX (oligohydramnios)
3. Perinatal, neonatal, infantile, juvenile.
- The perinatal form demonstrates severe involvement of the kidney with minimal hepatic involvement.
- The juvenile form demonstrates minimal renal involvement but extensive hepatic involvement.

Front 14

1. What is the most common renal tumor seen in the neonatal period?
2. What are the imaging features?
3. What is the treatment?

Back 14

1. Mesoblastic nephroma
2. It is a hamartoma of the kidney which presents as a large solid abdominal mass in neonates less than 1 year of age.
3. Imaging cannot differentiate Mesoblastic nephroma from the rare neonatal Wilms tumor. Therefore, these masses are resected.

Front 15

1. What is the etiology of multicystic dysplastic kidneys?
2. Why must the other kidney be scrutinized?
3. What is the imaging appearance of MCDK?
4. How is MCDK managed?

Back 15

1. Severe in-utero obstruction of the kidey.
2. 15% of pts have a contralateral UPJ obstruction.
3. Numerous cysts that do not communicate with each other.
4. Patients are followed up every 6 months for 3 years to ensure the diagnosis and monitor the decrease in size of the cysts. Surgical removal is necessary if growth occurs. A cystic form of Wilms' tumor will grow larger, rather than involute.

Front 16

1. What is the triad of prune belly syndrome/Eagle Barret syndrome?
2. Is there a gender predilection?
3. Why is a patent urachus seen?

Back 16

1.
- hypoplasia of the abdominal musculature
- cryptorchidism is thought to be related to an enlarged bladder which prevents the testicles from descending.
- marked dilatation of the urinary tract.
2. It is exclusively seen in males.
3. A patent urachus may be present allowing decompression of the urinary tract.

Front 17

1. Describe the grading of VUR.
2. Discuss the managemnt of VUR?

Back 17

1.
2.
- Medical management initially for VUR I, II, III.
- If no improvement in 2-3 years: surgery
- Grade V is unlikely to resolve: surgery after infancy.
- Siblings should be screened

Front 18

What are the possible predisposing factors for urolithiasis in children?

Back 18

1. Chronic infection
2. Metabolic disorders
- Hypercalcemia
- Hyperoxaluria
- Hyperuricemia
3. Congenital anomalies that cause obstruction.

Front 19

lf you see a renal cyst in child, what should you consider?

Back 19

1. Remember that renal cysts are unusual in a child. Therefore if a renal cyst is identified, consider TS, VHL, Caroli's disease, NF.

Front 20

1. What is the growth pattern of Wilms tumor?
2. What are the findings that can help differentiate Wilms tumor from renal medullary carcinoma?

Back 20

1.
- Wilms tumor tends to displace structures
- Can invade into renal vein and IVC
- MC mets to lungs
- Rarely calcifies
2. Renal Medullary Cancer
- Aggressive tumor
- Sickle cell trait
- Bone mets (NOTE: Wilms tumor does not metastasize to bones very frequently).

Front 21

Obstructive cystic dysplasia

Back 21

- hydronephrosis + renal parenchymal cysts.
- small peripheral cysts that do not communicate with other.
- usually results from a distal obstruction and the kidneys are generally small and echogenic with small cysts.

Front 22

Staging of Neuroblastoma:

Back 22

- Stage 1: Localized tumor confined to the area of origin.
- Stage 2: Unilateral tumor with positive ipsilateral nodes.

- Stage 3: Tumor infiltrating across midline or positive contralateral lymph nodes.

- Stage 4: Wide metastatic disease with dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.

- Stage 4S: Age <1 year old with localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow.

Front 23

Neonatal hydronephrosis

Back 23

Imaging of the kidneys within the first week of life can underestimate the degree of hydronephrosis because of dehydration. If the kidneys are normal on ultrasound within the first week of life, imaging should be repeated.

Front 24

1. What is the most common neoplasm of the lower urinary tract?

Back 24

1. Rhabdomyosarcoma
2. MC sites include the head and neck; in the pelvis, it may originate from the bladder, vagina, uterus, prostate, or paratesticular tissues.
- Typically are large cystic or solid tumors with significant mass effect.
- Prostate is the MC site of pelvic origin in boys.
- The botryoid subtype often presents as a polypoid mass resembling a cluster of grapes protruding from the vagina.

Front 25

Cystic mass in the kidney

Back 25

- Multicystic dysplastic kidney
- Hydronephrosis
- Multilocular cystic nephroma
- Cystic Wilms Tumor

NOTE: in a duplicated collecting system, a MCDK of a single pole can look like multilocular cystic nephroma.

Front 26

Enlarged bladder

Back 26

OBSTRUCTIVE CAUSES:
- posterior urethral valves
- prune belly syndrome
NON-OBSTRUCTIVE CAUSES
- VUR
- Megacystis-microcolon-intestinal hypoperistalsis syndrome

Front 27

Renal medullary carcinoma

Back 27

- massive retroperitoneal LAD lifts up the aorta from the spine.
- sickle cell trait
- tumor enhances poorly after iv contrast

Front 28

Bladder extrophy

Back 28

KUB shows widened pubic symphysis
IVP shows bladder exstrophy and epispadias

Front 29

Testicular adrenal rests

Back 29

- pts usually have a past medical history of removal of bilateral adrenal tumors that have been surgically removed. - pts are on daily steroids.
- NOTE: cortical adrenal tissue is attached to the testes as it descends in the fetus.
- Located in mediastinum testes.
- No tumor risk.
- Pain attributed to inconsistency in steroid meds.