paeds cardio/gastro/surgery/neuro Flashcards
RF of PDA
high altitude, maternal rubella, prem
signs of PDA
heaving apex beat, subclavicular thrill, continuous machinery murmur
when does west syndrome (infantile spasm) present (progressive mental handicap/developmetal regression)
4-8 months of life
Tx with vigabatrin firstline then ACTH
what metabolic disturbance do you get with pyloric stenosis
hypocholoraemic, hypokalaemia metabolic alkalosis
what might you find O/E of intussusception
pain, baby (normally 5-7months) bringing knees up to chest, red current jelly stool, right upper quadrant mass (sausage shaped)
RF of intussusception
lead point (meckles), concurrent viral infection, boys, CF
what are the features of innocent murmurs
soft, systolic, no radiation, varies with posture, no heaves or thrill, come with viral infection, no symptoms
Investigations for TOF
ECG - right axis deviation
microarray - if genetic syndrome
CXR - boot shaped heart
Echo
O/E of TOF
central cyanosis, thrill or heave, pan systolic murmur heard best at the left sternal edge
complications of TOF
pulmonary regurg, SCD, infective endocarditis, arrhythmias
what’s the role of the umbilical vein
carries oxygenated blood from the placenta to the babies liver
what does the umbilical artery do
returns deoxygenated blood to the placenta
what do the ductus arterioles and the ductus venous become In the adult
ligamentum arteriosum and ligamentum venosum
what causes TGA
failure of the aorticopulmonary septum to spiral in septation
O/E of TGA
RV heave, loud S2 and centrally cyanosed
what does CXR of TGA show
egg on string
Mx of TGA
1) PGE2
2) emergency atrial balloon septostomy
3) surgery
what kind of VSD are most common
perimembranous
MX of eisenmengers
heart-lung transplant
what murmur do you get in VSD
pan systolic heard best on left lower sternal border
Mx of VSD
-if large –> need surgery
-medical Mx –> diuretics and ACEi to reduce after load (reduce pressure in L heart to prevent eisenmengers)
complications of VSD
endocarditis, growth failure and sudden death
is epstein anomaly cyanotic ?
it can be as often associated with a ASD and a R–>L shunt
O/E of Epstein anomaly
gallop rhythm, cyanosis, tachypnoea, signs of heart failure
O/E of ASD
ejection systolic murmur and fixed splitting of the S2
Mx of ASD
if <5mm –> should close by 12 months of birth
Surgery if >1cm
complications of ASD
arrhythmias, stroke if DVT (paradoxical embolism), eisenmengers if large
shunt in AVSD
large L to R
murmur in AVSD
mid-diastolic rumbling murmur
signs of coarctation of aorta
radio-femoral delay, weak peripheral pulses
Mx of coarctation of aorta
surgery
-if very bad may need prostaglandins to keep the ductus arteriosus open while awaiting surgery
what should pre and post ductal saturations be
difference of no more than 3%
explain the relevance of pre and post ductal saturations
pre ductal saturation - measured in right arm and measure saturations before the ductus arteriosus is
post ductal - measure in leg and measure saturations after ductus arteriosus.
If there is a difference of >3% this indicates mixing of the pulmonary circuit and a PDA dependent lesion eg severe coarctation of aorta / TGA
associations of pulmonary stenosis
TOF and Noonan
MX of pulmonary stenosis
balloon valvuloplasty
O/E of aortic stenosis
ejection systolic murmur, slow rising pulse, palpable thrill, narrow pulse pressure
Mx of aortic stenosis
percutaneous balloon aortic valvuloplasty or surgical aortic valvotomy
which type of ASD is most common
ostium secundum
what else is ASD associated with
fetal alcohol syndrome
a baby cyanosed at birth most likely to be
TGA
a baby cyanosed a 1-2 months after birth most likely to be
TOF
describe formation of the atrial septum
first septum primum forms (when looking at image, this is the septum closest to Left atrium). At bottom of here is the ostium primum. This then closes and the ostium secundum forms half way down the septum primum.
The septum secundum then forms (closest to the Right atrium) and a gap in this septum –> foramen ovale.
what is pulses parodoxus
in cardiac tamponade / severe asthma >10mmHg pressure fall in inspiration
what kind of pulse do you get in aortic regurg
collapsing
what is a bisferiens pulse
two peaks in the peripheral pulse in systole
what side is gastroschisis more common on
right
Rf of gastroschisis
maternal age <20, maternal smoking
worse prognosis of gastroschisis
intestinal atresia
presentation of NEC
feeding intolerance, abdo distension, bloody stools –> abdo discolouration and peripheral shut down
Ix for NEC
1) bloods (anaemia, thrombocytopenia and leucocytosis)
2) blood cultures
3) blood gas (metabolic acidosis)
4) US FIRSTLINE
5) Xray - pneumatosis intestinalis
complications of NEC
intestinal structure, short bowel syndrome, sepsis, perforation
give 5 causes of liver failure in paeds
1) Wilsons
2) alpha 1 antitrypsin deficiency
3) hepatitis
4) paracetamol OD
5) autoimmune
IX for alpha 1 antitrypsin deficiency
serum A1 antitrypsin and genetic testing
MX of alpha 1 antitrypsin deficiency
liver transplant and lung volume reduction surgery
who does autoimmune hepatitis normally affect
young females
how does autoimmune hepatitis present
fever, jaundice (in 25%), amenorrhoea
antibodies in autoimmune hepatitis
ANA and anti smooth muscle
what should happen when a baby is born to a mum who is infected with Hep B
complete course of Hep B vaccination and Hep B immunoglobulin on birth
conditions associated with omphalocele
downs and beckwith wiedemann
RF of omphalocele
maternal smoking and age >40
AFP is low in the quadruple test for DS, what conditions is it high in
gastroschisis, omphalocele and neural tube defects
term for faecal incontinence
encopresis
conditions which cause constipation in children
hirschsprungs, CF, hypothyroidism, anal stenois, coeliac
red flags of constipation in children
not passing meconium in 48 hours, ribbon stools, severe acute abdo pain, failure to thrive, any leg weakness or neurological abnormality
complications of chronic constipation
pain, reduced sensation, anal issues, haemorrhoids, UTI (bladder neck compressed)
disimpaction regime –> escalating dose of movicol. When is a stimulant laxative added?
if not disimpacted after two weeks
firstline laxative for children
a macrogol like movicol
what is sandifer syndrome
abnormal movements of the baby’s head/neck in response to reflux
IX of GORD
clinical Dx but can do a 24 hour oesophageal pH
when to suspect GORD
infant excessively crying, hoarseness of voice, unexplained pneumonia, child over 2 who has retrosternal pain
Mx of GORD in breastfed baby
1/2 week trial of gaviscon infant, if this does not work try 4 week trial omeprazole
complications of Hirschsprung disease
Hirschsprung associated enterocolitis (overgrowth of E coli and C diff)
Faecal incontinence
maintenance Tx for UC vs crohns
crohns - azathioprine / mercaptopurine ARE ALWAYS FIRSTLINE
UC - aminosalicylate firstline. Add azathioprine / mercaptopurine if had >2 relapses in one year.
can methotrexate be used in Tx of crohns or UC
crohns only
what’s a really important Mx point for crohns
STOP smoking
what is psoas sign
in appendicitis, RIF pain with extension of the right hip
how long to get back to normal activities after appendicitis
2-4 weeks
who might appendicitis have an atypical presentation in
old people, children and pregnant
Dx of biliary atresia
percutaneous liver biopsy with intraoperative cholangioscopy
surgery for biliary atresia
kasai portoenterostomy
(and then need bile enhancers and abx for the first year of life)
complications of biliary atresia
liver cirrhosis, ascending cholangitis, portal hypertension
Investigation for meckels
meckels scan using technetium
presentation of meckels
can be asymptomatic or can present similarly to appendicitis and can cause GI bleed!!!!
one undescended testis should be referred at what age
3 months
if bilaterally undescended testis, when should they be reviewed by a specialist
within 24 hours
Rf for undescended testis
prem, FHx
when is orchidopexy done for cryptorchidism
12 months
where is the deep inguinal ring found
midpoint of inguinal lig (half way between ASIS and pubic tubercle)
presentation of retinoblastoma
normally at 18 months, get absence of red reflex and it gets replaced by a white pupil, strabismus
if unilateral –> enucleation
if bilateral –> radiotherapy (and enucleation of the eye it is worse in)
VACTERAL associations
vertebral abnormalities
anal atresia
cardiovascular anomalies
TOF
oesophageal atresia
renal anomalies
limb anomalies
Ix for tracheooeophogeal fistula
AXR shows air in the stomach
causes for intestinal obstruction in paeds
anal atresia, Hirschsprung, volvulus, meconium ileus, strangulate hernia
what normally causes a hydrocele
patent processus vaginalis
what else does a patent processus vaginalis necessitate
inguinal hernia
how does an acute subdural haemorrhage present on CT
hyper dense (bright/white)
apart from hydrocephalus, what are some other causes for raised ICP
haemorrhage, brain tumour
CSF causes of a raised ICP
hydrocephalus –> due to aqueduct stenosis / problems draining
MX points for raised ICP
elevate head of bed, analgesics, anticonvulsants, controlled hyperventilation, sedation, manitol, shunts, invasive ICP monitoring, reverse any coagulopathies
what is the difference between cyanotic breath holding spells and pallid breath holding spells (AKA reflex anoxic)
cyanotic –> let out a long cry, then breath hold and become cyanotic / lose consciousness
pallid breath holding –> when child is startled and their vagus nerve is stimulated –> reduce CO and cause cerebral hypo perfusion
how motor neurones are affected in SMA
lower motor neurones (so get hypotonia, hyporeflexia, fasciculations, reduced muscle bulk and reduced power
-may have respiratory muscle involvement and swallow difficulties
what type of SMA is most common
type 1 (will nerve sit)
investigations for SMA
genetic testing for the SMN1 gene
Mx of SMA
MDT, physio, NIV, PEG
signs of hydrocephalus in child
increase in head circumference, sunsetting of eyes, bulging anterior fontanelle
both a craniopharyngioma and a pituitary adenoma cause bitemporal hemianopia - but what distinguishes them
craniopharyngioma - predominately inferior defect
pituitary macroadenoma - predominately superior defect
NOTE –> craniopharyngioma is a remnant of rathkes pouch and is most common in children
what’s a fluid challenge
drinking 0.2ml.kg every 10 minutes. Should be able to tolerate without vomiting. If they vomit give antiemetic (ondansetron is the antiemetic of choice in children)
Mx of PDA
if infant is PRETERM use NSAID (ibuprofen or indomethacin)
however, this does not work for TERM infants then have to use: transcatheter PDA closure
