Paeds - GI Flashcards
What is biliary atresia?
How to test for it?
What is diagnostic
Think of this in child with neonatal jaundice >2 weeks
It is scarring of intra and extrahepatic ducts
(Usually atresia just affects extra-hepatic ducts and they are absent)
This causes build up of bile in liver, causing scarring and loss of liver function eventually cirrhosis
Presents between 2 weeks and 2 months as obstructive jaundice
Ultrasound: triangular cord structure at porta is pathonomonic
HIDA scan: if no activity in the bowel at 24hours - this is diagnostic
***Associated with Downs and Polysplenia***
(Alagile is a rare form of biliary disease = congential cholestasis, liver biopsy needed to rule this out)
What are features of neonatal hepatitis?
When does it present?
Usually first month of life
Presents with jaundice
Causes
Hepatitis
CMV
Toxoplasmosis
Diagnosis
US can be normal or show increased echogenicity of liver
HIDA should show bowel activity (differentitates from biliary atresia)
Liver biopsy
What condition does Duodenal atresia have a strong assocaited with?
Downs Syndrome
How long and thick does pylorus have to be for pyloric stenosis?
Child presenting with RUQ pain, weight loss and high AFP
What is diagnosis?
Hepatoblastoma
Mostly found in kids under 3 but can be found at any point in childhood
CT: low attenuation mass with nodules
MRI: high T2 mass with fibrous bands running through
Can cause precocious puberty due to secretion of hormones
What are risk factors for necrotising enterocolitis?
Prematurity
Low birth weight
Congenital heart disease
Hirschprung disease
Perinatal asphyxia
Signs:
- portalvenous gas
- focal dilated bowel loop
- featureless bowel
- unchanging bowel gas pattern
What is an Omphalocele and Gastroschisis?
Omphalocele
__Midline defect
Intestines, liver, or other organs stick out through the belly button
Covered by peritoneal layer
Has multiple chromosomal anomalies associations
Heart abnormalities - VSD.
Neural tube defects
Beckwidth Wiedeman
IUGR
Gastroschisis
Intestines protruding out onto abdominal wall
Always right sided
No membrane/peritoneal layer covering
Maternal AFP raised
No associated anomalies
Associated with intestinal atresia
Distal intestinal obstruction syndrome
What is it?
What patients does it occur in?
Occurs in Cystic fibrosis patients usually in teens to 20’s where reduced compliance with enzyme supplements contributes
Bowel obstruction occurs usually in distal ileum due to dried up thick stool
In CF all of below contribute:
- thicker intestinal mucosa
- slower transit time
- pancreatic insufficiency
Child ages 3 presenting with a liver hilar mass causing biliary obstruction and lymphadenopathy
What is it?
Rhabdomyosarcoma
-Displaces local structures
Lymph node mets seen in 1/3 of cases
What is a meconium plug?
Does it cause obstruction? where
1/4 of patients have CF (some say its not assc with CF)
Also associated with diabetic mothers
- Failure to pass meconium in first 48 hrs*
- Causes large bowel obstruction at splenic flexure and small left colon*
- (meconium ileus causes SBO)*
- Tx: Water soluble contrast enema*
What is meconium ileus?
What are features on AXR?
Impaction of meconium at the terminal ileum/caecum causes small bowel obstruction
ONLY Occurs in children with Cystic fibrosis. Pancreatic insuff, thick mucosa
(NOT assc with prematurity)
Suspect if no meconium passed within 48hrs
AXR: multiple gas filled small bowel loops. Meconium can be seen as bubbly area in right lower quadrant
Calcification seen throughout abdomen on newborn AXR
What is cause?
Previous meconium peritonitis due to bowel perforation
This occurs antenatally and is a chemical peritonitis rather than a purulent one.
Necrotising enterocolitis
What age does this occur?
What group does it occur in?
Occurs in premature babies
Immature bowel mucosa
What is Hirschprungs
Due to failure of ganglion to migrate and innervate distal colon
The affected portions are small in calibre
A rare variant is Total Colonic Aganglionosis - can give microcolon and involve the terminal ileum
What is the most common liver tumour in infants under 5 years?
Hepatoblastoma is most common
Well circumscribed mass, right lobe
Serum AFP markedly elevated
- -calcifications often present*
- -associated with precocious puberty due to production of HCG*
Associated with syndromes such as Wilms, Beckwith Wiedmann
What are appearances of Mesenchymal hamartoma?
Under 5 years tumour
Cystic or multicystic liver mass
Enhancing septations present
Is a developmental anomaly
AFP negative
Large portal vein feeding vessel (however no significant doppler flow)
Calcifications UNCOMMON (helpful to separate from hepatoblastoma)
Wilms tumour
What age?
Appearances?
Most common abdo mass in children under 8
Solid renal tumour - can become necrotic
Invades
Differential for paediatric liver lesions with raised AFP
Which lesions are most common in >5 years?
What is most common type of tracehoesophageal fistula?
Blind ending proximal oesophagus with distal oesophagus connected to trachea
- This is a Type A or type C (sometimes known as N type)
- Will present very early with feeding difficulties
- If child is older e.g 3 years and presents with ?TOD then it is likely a Type E or H*
- Associated with Vacterl*
- -Most common type is Type C (blind ended oesophagus and distal oesophagus connected to trachea)*
NO AIR IN STOMACH = Oesophageal atresia of both ends (no fistula)
What does VACTERL stand for?
Phakomatoses
What is double bubble sign due to ?
What is double bubble sign seen in?
Double bubble of stomach and dilated proximal duodenum
Seen in:
Annular pancreas
Duodenal atresia
Duodenal stenosis
Midgut volvulus
What is most common cause of intussuception?
95% are idiopathic
Other causes:
- Meckels
- Henoch schonlein purpura
- Duplication cysts
Ileocolic most common type
What is VACTERL
Summary of bubble signs of obstruction
At what specific age range does Hypertrophic pyloric stenosis occur at?
From 2 weeks to 3 months
(DOES NOT occur at birth or after 3 months)
Criteria is 4mm single wall and 14mm length
Pylorus spasm is a differential
Patient with Downs syndrome presenting with bile stained vomit
Duodenal web (or intraluminal diverticulum)
Appears as ‘Windsock sign’
Also associated with malrotation
What is Stills disease?
Juvenile arthritis
Salmon rash
Hepatosplenomegaly
Fevers
Summary of Phakomatoses
Causes of intussuception in under 3 years?
