Paeds - GI/GU/Gynae Flashcards

1
Q

How to approach renal masses in kids

What is most common in Neonates?

What is most common in pre-school 6-12 years?

A

Approach by age

Neonates

1. Mesoblastic nephroma is most common solid mass

  • can have cystic areas due to necrosis
  • WONT invade collecting system or renal vein (Wilms will)

2. Nephroblastomatisis

3. Multicystic dysplastic kidney

-whole kidney is replaced by cysts with fibrous septations therefore they do not communicate

Overall apperance said to resemble bunch of grapes

(Hydronephrosis can be mimic however apparent cysts will communicate)

No renal function on MAG3

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2
Q

Most common renal masses in pre school aged 4 ish?

What is claw sign?

A

1. Wilms

Solid tumour that invades. Mets to lung

Enhances but less so than normal parenchyma

Claw sign is where some normal renal tissue stretches around mass

  • 10% bilateral*
  • 10% vascular invasion*
  • 10% calcification*

2. Lymphoma

3. Multilocular cystic nephroma

  • Mass of cysts but importantly they will be surrounded by a thick fibrous capsule.
  • Protrusion into the renal pelvis is also a key finding, but this is also seen in some Wilms tumours.
  • There shouldn’t be a solid component to this mass but enhancement of the septae separating the cysts may be seen on cross-sectional imaging.
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3
Q

Neuroblastoma

A

Most common abdominal mass in infants

Locations:

  • adrenals
  • retroperitoneum
  • posterior mediastinum

Presents with abdo pain, large mass and fevers

Most secrete catecholamies

-VIP (diarrhoea)

-VMA (leads to HTN)

  • Can have speckled calcification*
  • Encases IVC*
  • Can displace or invade the IVC*
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4
Q

Name the main 2 AFP secreting ovarian tumours in teenagers?

A

1. Leydig sertoli tumour

Tumours secrete oestrogen or testosterone or both therefore amenorrhoea

  • AFP elevated
  • Solid fibrous tumours = Low MR signal
  • Can have cystic elements

2. Yolk Sac tumour

Large mass with or without cystic areas

Does NOT produce oestrogen or progesterone

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5
Q

Gynae Summary

A
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6
Q

What age group in females do germ cell tumours occur in?

A

Children and young adults

Germ Cell tumours include:

  1. Teratomas
  2. Choriocarinomas
  3. Yolk sac tumours
  4. Ovarian embryonal carinoma

Dysgerminoma is the malignant once (accounts for 5% germ cell tumours)

  • Thick septa is a sign of malignant tumour*
  • Can tort
  • Can cause elevation in HCG
  • Menstrual irregularity common
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7
Q

What are granulosa cell tumours?

What age do they appear?

How to differentiate from mucinous cystadenoma?

A

Oestrogen secreting tumour (therefore can cause endometrial thickening and PMP bleeding)

Occur in women of ANY age (more common in adults though)

Large, multicystic with thick septations

Differentiate from mucinous cystademona as they dont contain intracystic papillary projections

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8
Q

Kids with recurrent UTI’s

What are investigations?

A
  1. US within 6 weeks
  2. DMSA 4 - 6 months to look for scaring
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9
Q

What is best radionuclide study for looking at Neuroblastoma?

A

MIBG with planar and SPECT imaging

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10
Q

What is screening frequency for patients at risk of Wilms (patients with Beckwith Wiedman)

A

3 monthly US kidneys

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11
Q

What is Prune belly triad?

A
  1. Undescended testes
  2. Abdominal wall muscle insifficiency
  3. Non obstructed distended ureters

Almost always in males

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12
Q

What is Beckwith Wiedmann syndrome?

(EMG Syndrome)

A

Congenital overgrowth syndrome (exomphalos, macroglossia, giagantism)

  • -Macroglossia*
  • -Omphalocele (same as Exomphalos)*
  • -Hemihypertrophy (increased size in one side of body)*
  • -Hepatosplenomegaly*

Increased risk of Wilms, Hepatoblastoma and Neuroblastoma

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13
Q

What is WAGR syndrome?

A

Wilms

Aniridia (partial or complete absence or iris - colour of eye)

GU abnormalities

Retardation

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14
Q

What condition predisposes to Chromophobe RCCs?

A

Burt Hogg dube

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15
Q

Neuroblastoma vs Wilms?

A

Regular and stippled calcification in Neuroblastoma

Less common in Wilms - which can have curvilinear calcs

Neuroblastomas secrete catecholamines - VMA and VIP

-they are assc with :

Opsomyoclonus: rapid jerky eye and leg movements

Hutchinsons: limping due to bony mets

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16
Q

What is staging in Neuroblastoma?

A

123 MIBG

17
Q

Posterior urethral valve

Features?

What does bladder look like?

What is first line investigation?

A

Seen in baby boys

Due to valve formation in prostatic and membranous urethra

Causes obstruction

Bladder trabeculation & hydronephrosis

Reflux through VUJ (more often left than right)

-Can progress to needing renal transplant due to kidney damage from reflux

First line: MCUG

18
Q

What is Page kidney?

A

Hypertension as a result of external compression on kidneys from capsular haematoma or other things

Activated RAAS system

Can see distortion of the kidneys with delayed arterial washout

19
Q

Congenital adrenal hyperplasia

Features of adrenals on US?

A

Diffuse enlargement and ‘cerebriform morphology’

HyPOechoic adrenal cortex

HypERechoic medulla

Males: Electrolyte disturbance

Females: ambiguous genitalia. ovaries can be hard to see on US

20
Q

What condition causes COMPLETE aplasia of the uterus?

A
  • Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is an extreme subtype where there is complete aplasia of the upper third of the vagina and uterus. The fallopian tubes and ovaries will be seen normally. Primary amenorrhoea is the usual presentation.
21
Q

Paeds followup for UTIs

Is there followup US or MCUG for acute infection which resolves within 48 hours?

A
22
Q

Testicular torsion

What are signs of missed torsion?

A

Heteogenous testis with increased PERI-testicular flow but absent testicular flow in a patient with symptoms longer than 24 hours

23
Q

What is Bell clapper deformity?

A

High insertion of the tunica vaginalis onto the spermatic cord

24
Q

What is used in staging of Wilms?

Which has the best prognosis?

A

123 MIBG

Stage IV disease actually has best prognosis (involving skin, bone marrow, liver)

25
Q

What is most severe form of ARPKD?

A

Perinatal form is most severe (24 weeks to first month of life)

Features:

  • bilateral enlarged kidneys
  • loss of corticomedullary differentiation
  • multiple radially orientated small cysts

Associated with congenital hepatic fibrosis

Causes renal failure oliguria, oligohydraminos and pulmonary hypoplasia

Severe resp compromise is cause of death

26
Q

Weigart Meyer

What are rules?

Where does upper moiety insert?

A

UUO

Upper moiety is associated with Ureterocele and Obstruction

Upper moiety inserts medial and inferior to the lower moiety insertion

Lower moiety associated with reflux

27
Q

Grading of reflux on MCUG?

What is grade IV?

A

Grade 4 is tortuous ureter with moderate dilatation of pelvicalyceal system

28
Q

What is Currarino triad?

Currid SAP

A
  1. Presacral mass
  2. Sacrococcygeus defect
  3. Anorectal malformation
29
Q

Neurogenic bladder

What are appearances?

A

Trabeculated bladder with reduced capacity

Can progress to atonic bladdder

Detrusor control is S2 - S4

Causes

  • Meningomyelocele
  • Diabetes
  • MS
  • Polio
30
Q

Hypoechoic low T1 and T2 lesion in kidney of 11 month old. Minimal enhancement

A

Nephroblastomatosis

can have plaque like or nodular appearance