GI Flashcards
RF for NEC
- Pretrem
- Umbilical venous catheter
- CHD
- Immunosuppression
What is the diagnosis?
caterpillar sign: which describes the undulating contour of the gastric wall peristalsing against an obstructed pylorus
Pyloric stensosis
USS findings of Pyloric stenosis
- wall thickness > 4 mm measuring from echogenic mucosa to echogenic serosa and
- a channel length of more than 16 mm.
- pyloric volume: >1.5 cm3
The treatment of malrotation with volvulus is ….
Ladd procedure
colicky abdominal pain, “currant jelly stool,” and a palpable right lower quadrant abdominal mass. the diagnosis is …..
Intussusception
What is the diagnosis? target or pseudo kidney sign on USS
Intussusception
Ddx for bloody stool and thick-walled bowel on ultrasound includes :
- intussusception,
- coltis, and
- much less commonly intramural hematoma e.g., due to trauma or Henoch Schonlein purpura .
Treatment of intussuception
Air insufflation
air should be insufflated up to….
Air should be insufflated up to 120 mm Hg- bwn cries.
What is the rule of 3 re insufflation
The “rule of 3’s” applies to hydrostatic reduction:
- Up to 3 attempts can be performed,
- up to 3 minutes each.
- If unsuccessful –> surgery.
Contraindications to pneumatic reduction include :
- free air,
- peritoneal signs, and
- septic shock.
50 % of patients with TEF have associated anomalies, most commonly the VACTERL association:
- Vertebral segmenta on anomalies.
- Anal atresia.
- Cardiac anomalies.
- Tracheo-Esophageal Fistula.
- Renal anomalies.
- Limb (radial ray ) anomalies.
Classic type C/ n TEF
82%- TEF ( with proximal esophageal atresia and a distal tracheoesophageal Fistula) shows an Ng tube terminating in the mid-esophagus with air- filled bowel from a distal TEF.
Type A TEF
8% may present with a gasless abdomen, due to esophageal atresia without fistula.
Type H TEF
6 %- features a continuous esophagus without esophageal atresia, but with an upper esophageal TEF. This type may present later in childhood with recurrent aspiraton.
When is Esophageal atresia suspected in utero?
Polyhydramnios and lack of visualisation of stomach
What is TEF associated with?
Associated with tracheal anomalies including tracheomalacia and bronchus suis = right upper lobe bronchus arising directly from trachea .
A diagnostic imaging finding of gastric atresia
single bubble, with a large bubble of air ( or contrast in the proximal stomach).
the imaging test of choice for a distal obstruction is a ….
contrast enema
Associations of duodenal atresia
- Down’s syndrome
- VACTERL.
- Shunt vascularity
- cardiac lesions ASd, vSd, PdA, and endocardial cushion defect .
- malrotation.
- Annular pancreas, which is seen in 20 % of babies with duodenal atresia.
If distal bowel gas is present with a double bubble sign
- midgut volvulus,
- annular pancreas- pancreas wraps around the duodenum , and
- the less severe variants of duodenal atresia including duodenal stenosis and web.
A patient with a double bubble and no distal bowel gas can be presumed to have …..
duodenal atresia.
Triple bubble sign
jejunal atresia
Most common causes of microcolon
- meconium ileus and
- ileal atresia.
Microcolon with filling defects in bowel
Meconium ileus
Microcolon and abrupt cut-off at site of atresia
Ileal or colonic atresia
meconium ileus is the earliest manifestation of …..
CF
Classic radiographic appearances of meconium ileus
distal obstruction (multiple loops of dilated bowel) , with soap-bubble lucencies in the right lower quadrant.
Ca due to meconium peritonitis.
neonates who fail to pass meconium
Small left colon, also known as functional immaturity of the colon or meconium plug syndrome,
RF for small left colon:(3)
- preterm
- neonates born to mothers who received magnesium for pre eclampsia, and
- infants born to diabetic mothers.
T/F ? Small left colon is a cause of microcolon
False
T/F? Small left colon is associated with CF/ or meconium ileus
False
Appearances of small left colon on contrast enema
typically with a discrete transition in caliber at the splenic flexure. There may be filling defects within the small left colon representing meconium plugs.
Main DDX for small left colon;
Hirschsprung disease with transition at splenic flexure.
Hirschsprung vs small left colon
Hirschsprung disease would not feature a distensible rectum and would not resolve after an enema treatment.
Which part of bowel is almost always affected in Hirschsprung disease?
anus
Radiography of Hirschsprung disease shows a …..
distal bowel obstruction pattern.
Definitive diagnosis of Hirschsprung disease and management:
Biopsy Surgical
In contrast enema shows a cone shaped transition zone of narrowed distal colon and dilated proximal colon
Hirschsprung
megacystis microcolon intestinal hypo peristalsis syndrome findings (2)
rare congenital disorder characterised by a dilated non-obstructive urinary bladder and hypoperistalsis of the gastrointestinal tract and microcolon,
Imperforate anus is classified as high or low based on which anatomical location?
to the puborectalis sling.
Anomalies associated with High lesion in imperforate anus: (3)
- GU/GI fistula
- VACTREL
- L/S anomaly
Definitive Tx for high lesion imperforate anus
Colostomy and repair
Definitive Tx for low grade imperforate anus
Single stage perineal anoplasty
Clinical findings in high grade imperforate anus in boys and girls:
Boys: Meconium in urine (secondary to fistula between Anus and posterior urethra/ bladder
Girls : Meconium in vagina
Clinical findings in low grade imperforate anus in girls:
Distal vaginal fistula
Causes of conjugated hyperbilirubinemia: (6)
- biliary atresia,
- Alagille syndrome,
- bile acid synthetic defects,
- metabolic disease,
- alpha-1- antitrypsin deficiency, and
- infectous aetiologies.
to differentiate between biliary atresia (approximately 25 % of neonatal conjugated hyperbilirubinemia) and “neonatal hepatitis”
Tc-99m-HIDA hepatobiliary scintgraphy
Findings of biliary atresia on NM study:
normal hepatic tracer uptake and clearance but NO excretion into the small bowel.
USS: echogenic fibrous tissue anterior to the to the portal vein: triangular cord sign
T/F Lack of GB on uss is diagnostic of biliary atresia
False- GB is present in 20% of cases
What is affected in biliary atresia
affects the intra- and extra-hepatic bile ducts, leading to obstructive (conjugated) jaundice.
Biliary atresia leads to progressive cirrhosis and death in early childhood if untreated.
Findings of hepatitis on NM studies:
- poor hepatic excretion,
- delayed hepatic clearance beyond 12 hours , and
- variable bowel excretion
T/F? In mesenchymal tumours, the tumour marker are not elevated.
True
What is mesenchymal hamartoma?
is a benign, multi cystic, hamartomatous lesion, which contains malformed bile ducts, portal vein fragments, and often extramedullary hematopoiesis
***Swiss cheese ***
Non calcified
Which inflammatory process causes GB hydrous (distension)?
Kawasaki
Vascular hepatic tumour that may cause congestive cardiac failure
- Infantile haemangioma and
- haemangioendothelioma
hemangioendothelioma is usually benign, it has the potential to…….
metastasize
Infantile hemangioma is associated with ………..
kasabach merrit syndrome
kasabach merit syndrome is a syndrome of …
- vascular neoplasm,
- hemolytic anemia, and
- consumptive coagulopathy.
Hepatoblastoma is associated with ….. (4)
- Beckwith Wiedemann (Q6 month screening ultrasound screening is performed ).
- Familial adenomatous polyposis syndrome.
- Fetal alcohol syndrome.
- Wilms tumour
Which tumour marker is raised in hepatoblastoma?
AFP
RUQ Ca++
List the paediatric causes of liver cirrhosis:
- alpha-1-antitrypsin deficiency,
- glycogen storage disease,
- tyrosinemia,
- biliary atresia, and
- chronic viral hepatitis.
Which tumour marker is raised in HCC
AFP
What is the diagnosis?
highly aggressive mesenchymal neoplasm occurring in school-age children 6 -10 years old. AFP is negative.
Undifferentiated embryonal sarcoma
Common paediatric tumours metastasising to the liver: (2)
- Wilm’s
- Neuroblastoma
Meckel diverticulum can be diagnosed with….
Tc-99m- pertechnetate scan. (only +ve if it contains positive ectopic gastric mucosa)
What is meconium peritonitis?
It is caused by in-utero small bowel perforation and meconium spillage, resulting in peritonitis, secondary calcification, and meconium pseudocyst formation.
Causes of RUQ Calcification:
- gallstones,
- hepatoblastoma, and
- hepatic ToRCH infections e.g., Cmv and toxoplasmosis .
types of meckels mucosa
- gastric- take up pertechnetate
- pancreatic
patients at high risk of NEC are
- Low birth weight <1500g
- Perinatal asphyxia
- Hirschsprung
- Premature
- Cardiac patient
difference between gastroschisis and omphalocoele
gastroschistosis
Omphalocele
No surrounding membrane.
Do not have associated anomalies.
maternal AFP will be elevated higher
Gastro always on the right side
Omphalocele
covered by….. Associated with trisomy…. associated anomalies are: - - - - -
Which condition has a higher maternal AFP?
gastroschisis
Right sided heterotaxia
- left lung has 2 fissures
- asplenia
- cardiac malformations
- reversed aorta/ivc
left sided heterotaxia
- right lung has 1 fissure
- polysplenia
- less cardiac malformation
- azygos continuation of IVC
cystic mass with normal AFP, no ca++, there is a large PV branch feeding the tumour
mesenchymal hamartoma
child is less than 1 with skin lesions, CXR shows a large heart, doctors can feel a lump in the liver
haemangioendothelioma
this hepatic tumour can cause precocious puberty
hepatoblastoma
Caroli is associated with (2)
- polycystic kidney disease
- Medullary sponge kidney
Common causes of pancreatic insufficiency
- CF
- Shwachman diamond syndrome
Pancreatic insufficiency with diarrhea, short stature, and eczema- lipomatous pseudohypertrophy of the pancreas and cupping of the ribs
Shwachman diamond syndrome
Pancreatic mass in a 1 year old
Pancreatoblastoma
Pancreatic mass in a 6 year old
Adenocarcinoma
Pancreatic mass in a 15 year old
Solid Pseudopapillary tumour of the pancreas.
For antenatal hydronephrosis with hydroureter consider:
- Prune belly syndrome: tends to be associated with oligohydramnios
- posterior urethral valves: also tends to be associated with oligohydramnios
