Paediatrics Flashcards
What is distal femoral cortical irregularity
Distal femoral cortical irregularity has also been referred to as “cortical desmoid”, “cortical irregularity syndrome” and “distal femoral defect”. The typical radiological appearance is a radiolucent cortical defect with adjacent sclerosis. It is often asymptomatic and is almost an incidental finding.
What is choanal atresia?
Congenital occlusion of the nasal cavity from the nasopharynx - the ‘choanae’
Maybe due to bone or soft tissue
What is CHARGE syndrome ?
Colombo - gap in retina
Heart defects
Atersia of the choanae
Retardation of development
Genitourinary anomalies
Ear anomalies
What are the key features of a pyriform aperture stenosis
Piriform aperture < 11mm
Hypoplasia of the hard palate
Medial displacement of the maxillary nasal process - i.e. it moving inwards to make the piriform aperture smaller
What virus causes croup
Parainfluenza virus
What virus causes epiglottitis ?
H.influenza virus
What is Lemierre syndrome ?
Post oral infection - such as tonsillitis. There is extension into the internal jugular vein. This then causes septic pulmonary emboli or mediastinitis - mediastinal extension via the danger space
What bug is responsible for exudative bacterial tracheitis ?
S.aureus
What is the most common congenital diaphragmatic hernia ?
Bochdalek - back on the left
Causing bowel to herniate
Can result in pulmonary hypoplasia
What is Congenital diaphragmatic hernia associated with ?
Bowel malrotation
Neural tube defects
Tuners syndrome
Trisomy 21
What is sequestration?
Aberrant lung tissue with systemic blood supply- usually arising from the thoracic aorta
Most common in the left lower lobe
What virus causes bronchiolitis
RSV
What typically causes round pneumonia ? what does it look like?
S. Pneumoniae - looks like a round pulmonary opacity with well-defined margins, mimicking a mass
What are the causes of bronchiolitis obliterans syndrome ?
Final common pathway of small airway obstruction:
Causes include allogenic lung and bone marrow transplant - post lung transplant
Post infectious
Related to toxins / drug exposures
Which infections are associated with swyer-James Macleod syndrome ?
Adnovirus and mycoplasma pneumoniae
What is crpytogenic organising pneumonia
Filling of the bronchioles and alveoli with granulation tissue polyps -
It is organising pneumonia of unknown cause
What is the atoll sign meant to be associated with ?
Cryptogenic organising pneumonia.
What is holt-oram syndrome
ASD + upper extremity bone deformities , including abscence or hypoplasia of the thumb
What is the most common ASD malformation ?
Ostium secundum - failure of closure of the Ostium secundum with the septum
What are the 4 components of Tetrology of fallot?
- Overriding Aorta
- VSD
- RV hypertrophy
- Pulmonary atresia
Which syndromes is TOF associated with ?
DiGeorge
VACERAL
Trisomy 21
What is the classic CXR finding of TOF and why?
Boot shaped - due to RV hypertrophy and reduced pulmonary vascularture.
No cardiomegaly
What is Ebstine anomaly ?
Where the right atrium is too low - causing a bigger RA and a smaller RV. This causes reduced pulmonary vasculatture and cyanosis and not enough blood can get too the lungs.
What is the classic CXR finding of Ebstine anomoly ?
HUGH box shaped Herat with MASSIVE right atrial enlargement.
What is the most common type of ASD ?
Ostium secundum - Caused by incomplete covering of the secundum by the septum.
This one might close without treatment
What are the two most common extracadial shunt lesions resulting in acyanotic congenital heart disease ?
Vein of Galen malformation and hepatic hemangioendothelioma.
In paediatric congenital heart disease what is aortic coarctation associated with ?
Biscupid aortic valve.
Turners syndrome
Shone syndrome
Intracerebral berry aneurysms
When to patients with Patent ductus arteriosus classically present ?
Infants develop CHF around day 7-10 due to vascular resistance falling.
What are the two sub-types of transposition of the great arteries ?
Dextrose - TGA
Levo TGA
What is the most common cause of cyanotic heart disease in newborns ?
TGA
What are the different subtypes of total anomalous pulmonary venous return ?
Supracardiac - pulmonary veins go to left subclavian vein
Cardiac - coronary sinus
Infracardiac - hepatic veins/ portal vein/ hepatic IVC
Mixed
What are the anterior mediastinal masses ?
The 4 T’s
- Terrible lymphoma
- Teratoma
- Thymus / Thymoma
- Germ cell tumor
What are the middle mediastinal masses ?
Foregut duplication cysts
Neuroenteic cysts
Lymphadenopathy - lymphoma or granulomatous disease.
What are the posterior mediastinum masses?
Neurogenic tumours :
neurobloastoma (malignant ) - > ganglioneurobloastoma ->
ganglioneuroma (benign)
What is dose the spinnaker sail sign represent ?
Pneumomediastinum
Where should the Umbilical artery catheter be positioned?
Terminates ideally at T6-T10 or L3-L5.
What syndrome is rhabdomyoma associated with ?
Tuberous sclerosis
Where do cardiac fibroma usually arise ? What is the pathognomic sign?
Intraventriclar septum
Central calcification - differentiating them from rhabdomyomas
What is the most common paediatric tumour arising from the cerebral hemisphere ?
Astrocymtoma
Where does NEC tend to occur ?
Distal ileum and right colon
WHO is at risk of NEC ?
Pre-term babies, congenital heart disease, hirschsprung disease and umbilical venous catheter or immunosupression
What is the most common delayed complication of NEC ?
Bowel stricture
What are the imaging criteria of pyloric stenosis ?
> 3mm thick
Longer than 15mm
No gastric contents passing through the pylorus
What age should you be concerned about a intersusspection ?
< 3 months or > 3 years
Need to look for a lead point
What pressure do you inflate to for trying to reduce an intersusseption ?
3x 80
3x 100
3 x 120mmHg
How many types of tracheoesophagel fistulas are there ? Which is the most common ?
5 types A - E
The most common is oesophageal atresia with distal trachooesophgeal fistula (87%)
Atresia - the oesophagus is just cut in half
B -proximal fistula
C - distal fistula ( most common)
D- Double fistulation ]
E- Just a joining between the two
What are the 3 causes of proximal bowel obstruction - neonate
- Malrotation mid gut volvulus - surgical emergency
- Duodenal artesia
- Jejunal atresia
What are the surgical causes of low bowel obstruction ?
Hirschsprung disease and distal atresias
What is a micro colon?
An abnormally small colon - < 1cm
What is secreted by the small bowel to prevent micro colon?
Sulcus entericus
What is one of the earliest manifestations of cystic fibrosis>
Meconium ileus - almost 100% of babies with meconium ileus have CF
Group A ependymoma tumours occur in which age group ?
Under 5 - group A epdnymoma
Over 5 - group B ependymoma
What is small left colon caused by ?
Temporary immaturity of the colonic ganglion cells - causing the distal colon to have abnormal motility right after birth
What are the most common causes of bowel obstruction in children ?
AAIIMM
Appendicitis
Adhesions
Inguinal/internal hernia
Meckles diverticulum
Malrotation
What percentage of meckles contains gastric mucosa?
25 %
What scan can be done to look for meckles ?
Tc-99m Pertechnetate scan
What sign on US might you see in biiliary atresia?
Triangular cord sign - echogenic fibrous tissue adjancent tot he portal vein representing abnormal bile duct
What is bilary atresia associated with ?
Polysplenia and trisomy 18
How does hepatoblastoma usually present ?
Painless abdominal mass with raised AFP
What is usually elevated in hepatblastoma ?
AFP > 1000ng/ml
What does a hepatoblastoma look like ?
Large, heterogenous, lobulated mass, that takes over the whole liver parenchyma, often cause IVC thrombosis
What age does HCC commonly occur in ?
Over 5s
What are the causes of VUR
Primary - Abnoramlly short intravesicular portion of the distal ureter. Or secondary due to distal obstruction - posterior urethral valves or neurogenic bladder.
How many grades are there when assessing VUR.
5
What are the two radionuclide scans that can be performed for VUR ?
- Radionuclide cystogram: inserting Tc99m into the bladder, assessing for reflex.
- Tc-99m DMSA renal scintigraphy scan - gold standard for detection of renal cortical scarring (seen as areas of relative photopenia
What does the dropping Lilly sign indicate ?
Duplex kidney - reduced number of calycs
- duplicated system with a functioning lower pole calycs which is displaced inferiorly by the hydronephrotic upper pole.
Renal Ptosis - normal number of calcyes
What are the common causes of uteropelvic junction obstruction?
Abnormal development of the upper segment of the ureter
Aberrant renal artery
Aperistaltic segment of the ureter
How would prune belly syndrome and posterior urethral valves differ on imaging?
Prune belly - the entire urethral system is dilatated
Posterior urethral valves - only from the prostatic urethra upwards is dilated.
What are the three major findings in prune belly syndrome ?
Lack of anterior abdominal walls
Dilataiton of the renal system
Cryptorchidism (bilateral undescended testes)
What are patients with horeshoe kidney at risk of ?
Damage from the vertebral body - so can’t play certain sports
Increased risk for stones, infection and cancer - Wilms, TCC and renal carcinoid.