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Peds > Peds GU > Flashcards

Peds GU Flashcards

1
Q

Unilateral Renal Agenesis Associations

A

Females- Genital anomalies

  • Unicornuate uterus
  • Rudimentary horn

Male genital anomalies

  • missing epipdidymis and vas deferens on the side of missing kidney
  • may also have seminal vesicle cyst on that side
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2
Q

Mayer-Rokitansky-Kuster-Hauser Syndrome

A

Mullerian duct anomalies

  • absence or atresia of uterus or unicornuate uterus
  • Unilateral renal agenesis

70% of females with unilateral agenesis have genital anomalies

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3
Q

Zinner Syndrome

A

Males

Unilateral renal agenesis

Seminal vesicle cyst on side missing kidney

Absent epididymis and vas deferens on side missing kidney

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4
Q

Bilateral Renal Agenesis

A

Potter Sequence

Insult in utero (ACE INHIBITOR)

Kidneys don’t form –> no urine

Oligohydramnios

Pulmonary Hypoplasia

PANCAKE ADRENAL SIGN on imaging

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5
Q

Horseshoe Kidney Associations

A

Increased risk of stones and infection

INcreased risk of cancer from chronic inflammation - Wilm’s, TCC< and Renal Carcinoid

Turner’s Syndrome

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6
Q

Vessel involved in Horseshoe Kidney

A

Lower poles fuse and get hung up on INFERIOR MESENTERIC ARTERY

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7
Q

Crossed-fused Renal Ectopia

A

One kidney crosses midline and fuses with other

Each kidney has its own ureter

the ECTOPIC kidney is INFERIOR

Left often crosses over to the Right

Complications: stones, infection, and hydronephrosis

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8
Q

Congenital UPJ Obstruction

A

Most common GU tract anomaly in neonates

Have to look for a crossing vessel before Tx (Pyeloplasty)

Intrinsic defect in the circular muscle bundle of the renal pelvis

Teen with flank pain after drinking lots of fluids

No hydro-ureter

May progress to multicystic dysplastic kidney

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9
Q

Whitaker Test

A

Urodynamic study combined with antegrade pyelogram

Differentiate between prominant extrarenal pelvis and congenital UPJ obstruction

NO HYDRO-URETER

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10
Q

AR Polycystic Kidney DIsease Associations

A

HTN

Renal Failure

Abnormal bile ducts

Liver fibrosis

Inverse relationship b/w kidney and liver disease (more severe kidney disease = less severe liver disease and vice versa)

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11
Q

US Findings in ARPKD

A

Smoothly marginated

Enlarged

Echogenic

Loss of corticomedullary differentiation

In utero - may not see urine in bladder

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12
Q

XR findings in ARPKD

A

Wide Abdomen

Bilateral flank “masses”

Pulmonary hypoplasia

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13
Q

Prune Belly/Eagle Barrett Syndrome

A

Males

Triad

  1. Lack of Abd muscles (partial or complete)
  2. Hydroureteronephrosis
  3. Cryptorchidism (bladder distension interferes with descent of testes)
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14
Q

Neonatal Renal Vein Thrombosis

A

Maternal DM

Usually UNILATERAL (Left)

Starts peripherally and progresses toward hilum

Renal Atrophy over time

Renal enlargement in acute phase

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15
Q

Neonatal Renal Artery Thrombosis

A

Often 2/2 UACs

Severe hypertension

No enlargement of kidney

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16
Q

Cryptorchidism Locations and Complications

A

Undescended testes - 72% in IC, 20% prescrotal, 8% intra-abdominal

  1. Malignant degeneration (even if fixed- still inc risk of testicular cancer even on UNAFFECTED side)
  2. Infertility
  3. Torsion
  4. Bowel incarceration 2/2 associated indirect inguinal henia
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17
Q

Primary Megaureter

A

Enlargement of ureter NOT related to obstruction

Causes

  • distal adynamic segment (if no dilation of collecting system then it is NOT an obstruction)
  • Reflux at UVJ
  • Idiopathic
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18
Q

Retrocaval Ureter

A

Problem in development of the IVC – Ureter gets pinned behind IVC

Usually No Sx’s – but can get partial obstruction and recurrent UTIs

IV pyelogram –> reverse J aka fish-hook ureter

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19
Q

Ureterocele

A

ystic dilation of the intravesicular ureter 2/2 obstruction of the ureteral orifice

IVP or US - COBRA HEAD SIGN

Associated with duplicated collecting system (upper pole moiety)

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20
Q

Weigert Meyer Rule

A

Duplicated Collecting System

  • Upper Pole (vowels)
    • Ectopic insertion - Inferior and Medial to lower pole insertion
    • Tends to form Ureteroceles
    • Tends to Obstruct
  • Lower Pole (consonants)
    • Normal insertion Superior and Lateral
    • Prone to Reflux
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21
Q

Ectopic Ureter

A

Normal collecting system

Ureter inserts ectopically

More common in females

results in incontinence when ureter connects distal to the external sphincter in the vestibule

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22
Q

Posterior Urethral Valves

A

Fold in the posterior urethra resulting in outflow obstruction and hydronephrosis with eventual renal failure

Most common cause of urethral obstruction in male infants

Forniceal rupture –> Peri-renal fluid collections (urinary ascites)

Actually a good prognostic indicator because pressure is relieved

(can happen in any obstructive pathology)

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23
Q

Posterior uretheral valve findings on VCUG and fetal MRI

A

VCUG - abrupt caliber change between dilated posterior urethra and normal caliber anterior urethra

Fetal MR - keyhole bladder

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24
Q

Vesicoureteral Reflux

A

Ureter insets horizontally/vertically (not oblique)

50% of children with UTIs

recurrent/2nd UTI–> get VCUG to eval for reflux

Urologist can inject material at UVJ to prevent reflux “DEFLUX”

Usually resolves by age 5-6

Chronically –> scarring –> HTN or renal failure

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25
Q

Grading of VUR

A
  1. Into non-dilated ureter
  2. Into pelvis and calyces - NO dilation
  3. Mild-Mod dilation of ureter, renal pelvis, and calyces– with minimal blunting of fornices
  4. Moderate ureteral tortuosity and dilation of pelvis and calyces
  5. Gross dilation of ureter, pelvic and calyces + loss of papillary impressions + ureteral tortuosity
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26
Q

Bladder Exstrophy

A

Herniation of urinary bladder through hole in anterior infra-umbilical abdominal wall

Increased risk of malignancy in extruded bladder

MANTA RAY SIGN

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27
Q

Manta Ray Sign

A

Unfused pubic bones on AP pelvic XR

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28
Q

Urachus

A

Umbilical attachment to the bladder

Starts off as the Allantois –> then urachus –> atrophies –> median umbilical ligament

Urachus can persist to form a canal, a sinus, a diverticulum, or a cyst –> often results in infection

2:1 M:F

Can turn into Adenocarcinoma from constant inflammation

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29
Q

Renal Mass DDx Age 0-3 yo

A
  1. Nephroblastomatosis
  2. Multicystic Dysplastic KIdney
  3. Mesoblastic Nephroma
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30
Q

Renal Masses Over age 3 (around age 4)

A
  1. Wilm’s
  2. Multilocular Cystic Nephroma
  3. Lymphoma
  4. Wilm’s Variants (actually under 2-3 yo)
    1. Clear Cell - Wilms + bone mets
    2. Rhabdoid (wilm’s + brain mets
    3. Wilms with mets to lungs
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31
Q

Wilm’s Tumor

A

~ 4 yo — NOT IN NEWBORNS

Deletion in Chrom. 11 (W11ms)

Usually NO CALC’s (dist. from Neuroblastoma)

CLAW SIGN (normal renal tissue around the mass)

Spreads via direct invasion

Bilateral in <10%

32
Q

Renal Masses in Older Children

A

RCC - around age 9-10

  • Medullary renal carcinoma (sickle cell trait)

Lymphoma

33
Q

Nephroblastomatosis

A

Persistent Nephrogenic rests beyond normal 36 weeks –> precursor to Wilms tumor

  • Mut be followed q3 months till age 7
  • usually goes away on own

Wilms has necrosis, Nephroblastoma does not

Imaging = Hypodense rind, can be perilobar or intralobar

Sporadic

Can be assoc. with Beckwith-Wiedemann

34
Q

Wilm’s Tumor Associations

A

Beckwith-Wiedemann

WAGR (Wilms, Aniridia, Genitourinary abn’s, Retardation)

Sotos

Denysh-Drash

35
Q

Wilm’s “Nevers”

A

Never Biopsy (will seed tract)

Never before 2 months old (unlike Neuroblastoma)

36
Q

Beckwith-Wiedemann Associations

A

Wilm’s

Omphalocele

Hepatoblastoma

Pancreatoblastoma

37
Q

Sotos Syndrome

A

Overgrowth Syndrome like BWS

Macrocephaly

Retardation (CNS abn’s)

Ugly face

Assoc. with Wilm’s

38
Q

WAGR

A

Wilm’s

Aniridia

GU Abn’s (gonadoblastomas)

Retardation

(not an overgrowth syndrome)

39
Q

Denys-Drash Syndrome

A

Wilm’s

Pseudohermaphroditism

Progressive Glomerulonephritis

Gonadoblastoma

(not an overgrowth syndrome)

40
Q

Multilocular Cystic Nephroma

A

Non-communicating, fluid-filled locules with thick fibrous capsule

Arises from Renal Medulla

4yo boys and 40yo women (Michael Jackson Tumor)

Can be assoc. with pleuropulmonary blastoma

NO SOLID component, no necrosis

**Protrudes into renal pelvis**

Needs resection because cannot distinguish from cystic RCC or Wilm’s on imaging

41
Q

Wilm’s Variants

A

All under 2-3 years old

Clear Cell - mets to bones

Rabdoid - resembles muscle histology but no actual muscle tissue

  • mets to brain
  • prefers medulla/hilum of kidney
  • characteristic peripheral subcapsular fluid collection
  • asspciated with midline posterior fossa PNET
42
Q

Only cortically-based renal neoplasm

A

Wilm’s!

Rhabdoid, Clear Cell, Mesoblastic Nephroma are all MEDULLARY based

43
Q

Mesoblastic Nephroma

A

Benign

Fetal Hamartoma involving RENAL SINUS

MOST COMMON NEONATAL TUMOR (most in first 3 months of life)

Looks like Wilm’s but too young (under 1 year old)

SOLID AND CYSTIC (unlike ML cystic nephroma)

US- concentric echogenic and hypoechoic rings

Pseudocapsule- like Wilm’s

44
Q

Rhabdomyosarcoma

A

Bimodal (2-6 yo and then 14-16yo)

HEAD AND NECK (orbit and nasopharynx) most common

2nd most common - pelvis (scrotum, bladder trigone, vagina/cervix, uterus, prostate, pelvic wall)

LOWER GI TRACT TUMOR IN PEDS IS RHABDOMYOSARC TILL PROVEN OTHERWISE

**Paratesticular mass**

Mets to bone, lungs, LNs

45
Q

Rhabdomyosarcoma on CT and MRI

A

CT - enhancing ST mass, 20% have adjacent bone destruction

MR- heterogeneous (when necrotic), T1 iso to hypointense, T2 hyperintence, + Enhancement

46
Q

Neuroblastoma

A

VERY YOUNG kids (can be born with it unlike Wilms)

90% produce catecholamines (increased VMA and HVA levels)

VIP levels (Vasoactive pepride) - better prognosis

VMA:HVA >1 –> immature tumor –> worse prognosis

Crossing midline and/or contralateral LNs –> upstages tumor

47
Q

Neuroblastoma Locations

A

35 % Adrenal

30% RP

20% Post. mediastinum

5% neck

Thoracic = better prognosis

48
Q

Neuroblastoma associations

A

USUALLY SPORADIC but can be assoc with:

NF-1

Hirschsprung

BWS

49
Q

Orbital mets in Neuroblastoma

A

Raccoon Eyes

50
Q

How to eval for Neuroblastoma bone mets

A

MIBG is better than bone scan

bone mets are usually lucent metaphyseal lesions NOT sclerotic

51
Q

Vessel involvement in Wilm’s vs Neuroblastoma

A

Wilms INVADES vessels

Neuroblastoma ENCASES vessels

52
Q

Neuroblastoma appearance on MRI

A

T1 heterogeneous - iso to hypo

T2 heterogeneously hyperintense

Heterogeneous enhancement

53
Q

Neuroblastoma vs Wilms

A

Wilms

  • Age 4, never before 2 months
  • Rarely Calcifies
  • INVADES vessles
  • Well-circumscribed
  • Usually no bone mets (unless clear cell variant)
  • Mets to Lung

NB

  • Age < 2yo, can be in utero
  • 90% CALCIFY
  • Encases vessels, does not invade
  • Poorly marginated
  • Mets to bones
54
Q

Adrenal Masses

A

Neuroblastoma or Adrenal Hemorrhage

55
Q

Adrenal Hemorrhage appearance on US

A

Anechoic

Avascular (NB is hypervascular)

Will resolve (NB will get bigger)

Assoc. with scrotal hemorrhage

usually doesn’t cause adrenal insufficiency

56
Q

Normal appearance of Adrenals on US

A

V or Y shape

3mm thick, 15 mm long

Hypoechoic outer layers, hyperechoic inner (TRILAMINAR)

57
Q

Wolman Disease

A

Enlarged, Calcified Adrenals

(bilateral)

A Zebra

58
Q

Hydrometrocolpos Associations

A

Uterus didelphys

Can cause hydronephrosis by mass effect- get renal US

59
Q

Dermoid or Teratoma

A

2/3 of all ovarian neoplasms ( the other 1/3 are usually malignant germ cell tumors)

Mural nodules & thick seprations –> may be cancer

Contains fat +/- calc’s

Peritoneal implants, ascites, LAD – BAD signs

60
Q

Germ Cell Tumors

A

Seminomatous or NON-Seminomatous

Seminoma - white man, 20 yo or older

Non-seminomatous

  • Teratoma
  • Yolk Sac ( elevated AFP)
  • Embryonal Cell (elevated AFP)
  • Choriocarcinoma (elevated b-hCG)
  • Mixed germ cell
61
Q

Patent Processus Vaginalis

A

Hydrocele

Septations - hematocele or pyocele

62
Q

Isolated Right sided Varicocele

A

Concern for claudication 2/2:

  • RCC
  • RP fibrosis

GET CT A/P

63
Q

Henoch-Schonlein Purpura

A

Most common cause of idiopathic scrotal edema

64
Q

Appendiceal Torson

A

most common cause of acute scrotal pain in kids age 7-14 yo

Blue dot sign on physical exam

sudden onset pain

If testicular appendace is >5mm – best indicator of torsion

65
Q

Bell Clapper Deformity

A

Related to testicular torsion

Testis and Spermatic cord twisting 2/2 failure of tunica vaginalis and testis to connect

66
Q

Testicular torson

A

Venous flow lost first

Absence of flow or Asymmetric flow

Decreased echogenicity

Intravaginal - most torsions

Extravaginal - in babies

caused by failure of tunica vaginalis and testis to connect (Bell Clapper Deformity)

67
Q

INTRA-Testicular Mass DDx

A

Germ Cell tumors (90%)

  • Seminoma (40%, seen in 4th decade)
  • Non seminoma (60%)
    • Teratoma (under age 2)
    • Yolk Sac (under age 2)
    • Mixed GCT
    • Choriocarcinoma

Non GCT (10%)

  • Sertoli
  • Leydig

LYMPHOMA

68
Q

Yolk Sac Tumor

A

Heterogeneous mass

Under age 2

elevated AFP

69
Q

Testicular Teratoma

A

Pure testicular teratomas are in kids under 2 yo

Mixed teratomas in people around age 25

Aggressive (unlike ovarian teratomas)

70
Q

Choriocarcinoma

A

Agressive

Highly Vascular

Adolescents

71
Q

Sertoli Cell Tumor

A
  • Bilateral
  • “Burned out” tumors (dense echogenic foci from calcified scars)
  • Assoc with Peutz-Jeghers (hyperpigmented spots on lips, hamartomatous polyps)
72
Q

Testicular Lymphoma

A

Normal homogenous echotexture on US replaced focally or diffusely with hypoechoic vascular lymphomatous tissue

**MULTIPLE HYPOECHOIC MASSES IN TESTICLE**

73
Q

Extra-testicular mass DDX

A
  • Paratesticular Rhabdomyosarcoma - most common extra-testicular mass in young men (age 2-4 and 15-17)
    • US shows mass NEXT to testes
    • more commonly in orbits and nasopharynx
74
Q

Testicular Mass + Precocious Puberty

A

Think Sex Cord/Stroma tumors (non-GCTs)

SERTOLI OR LEYDIG CELL TUMORS

75
Q

Sacrococcygeal Teratoma

A

Most common tumor of fetus or infant

Solid and/or cystic

Often benign when found early (< 2months)

Deeper in pelvis = worse prognosis

Often arise from coccyx so coccyx needs to be removed with resection or will recur

Mass effect is the issue:

  • on GI system, hip dislocation, nerve compression leading to incompetence
76
Q

4 Types of Sacrococcygeal Teratomas

A
  1. External to Pelvis (47%)
  2. Mostly Extra-pelvic (some intra)
  3. Mostly Intra-pelvic
  4. Completely INTRA-pelvic – highest rate of malignancy

Peds (3 decks)

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