1.02 Developmental GI Anomalies Flashcards
What is the most common kind of TEF/EA?
Esophageal atresia + tracheoesophageal fistula
The most common TEF/EA complications will appear as what clinically?
Feeding intolerance = choking
Bronchitis/pneumonia = aspiration
GERD = spitting up
Strictures = increased secretions
What condition is associated with a 50% increased likelihood of additional abnormalities?
TEF/EA
If you clinically suspect TEF/EA what are the next steps?
Insert a NG/OG tube an order radiograph
(+) if tube in upper thorax
What prenatal finding hints at a possible duodenal or esophageal atresia?
Polyhydraminos
Down Syndrome is associated with what condition?
Duodenal atresia
What differentiates a duodenal atresia from an esophageal one on X-RAY?
Double bubble sign
What is the clinical presentation of newborn with duodenal atresia?
Bilious emesis in first two days
Distended abdomen
You notice a newborn with an umbilical hernia, next steps?
Watch, wait, restraint
Surgery IF still present at 5 years old
Where does an omphalocele originate?
Umbilical base/midline stalk
What should you consider in a child born with omphalocele that plays a part in prognosis?
Associated anomalies are common (50%) - prognosis based on it
What are risk factors for omphalocele?
Beckwith-Weidemann
Trisomy 13 & 18
How can you tell an omphalocele and gastroschisis apart?
Gastroschisis has no sac (no amnion, no jelly, no peritoneum)
Where does gastroschisis defect originate?
Right of umbilicus
What are the risk factors and prognosis related to gastroschisis?
Volvulus risk, intestinal atresia, and necrotizing enterocolitis (NEC)**
An omphalocele and gastroschisis are due to failure of what in embryonic development?
Lateral folding closure and return of gut to abdominal cavity
What is the plan if a child is born with omphalocele or gastroschisis?
- Cover exposed area with saline soaked dressing
- Gastric decompress, bowel bag, surgery
TEF/EA is due to a defect at what stage (when) in development?
Week 4-6
Duodenal atresia is due to a defect at what stage (when) in development?
Week 8-10 recanalization
Duodenal atresia is associated with what anomalies?
Trisomy 21, malrotation, congenital heart disease, renal anomaly
What is the preferred what to visualize a duodenal atresia?
Upper GI contrast radiography
Duodenal atresia plan?
- Nasogastric decompress
- Ped surgery consult
- IV hydrate +stabilize
- Surgery
What happens during intestinal malrotation?
It will rotate at most 180 (vs. 270), could wrap around the SMA, and be held back by Ladd bands
During a upper GI contrast series, what indicates a midgut volvulus?
“Corkscrew sign” of dilated bowel loops and duodenum doesn’t cross vertebrae
What causes a midgut volvulus?
Blood flow at SMA compromised leading to ischemia — bilious vomiting (clinical sign)
What is the plan for a midgut volvulus?
Urgent Surgery!!
Delay = necrosis, morbidity, mortality
What is the treatment plan for patient with intestinal malrotation and symptomatic/asymptomatic for less than 12months?
Ladd procedure
What is the treatment plan for a patient with intestinal malrotation if asymptomatic and OVER 12 months?
Surgical consult (+/-)
Heterotaxy syndrome present in 1% of the population leads to a high occurrence of what condition?
Intestinal malrotation; isomerism (left/right) vs. situ solitude (normal)
When do 95% of infants express meconium?
Within first 24 hours
Next step if newborn goes over 24 hours without meconium?
Plain abdominal radiograph
What is considered diagnostic and therapeutic for meconium ileus and meconium plug syndrome?
Contrast enema (barium)
What often causes meconium plug syndrome?
Maternal treatment of preeclampsia with magnesium-sulfate
What are indications for meconium plug syndrome?
No abdominal findings or gradual increase in girth with no other signs of illness, did not pass 24hr meconium, plain radiograph is nonspecific
Hirschsprung disease is a congenital defect where?
Parasympathetic postganglionic neuron cell bodies are not present (aganglionic section) leading to a proximal megacolon
The sensory neurons and Schwann cells of para nervous system and all enteric/autonomic ganglia are derived from what embryonic tissue?
Ectoderm - neural crest cells
What are some anomalies associated with Hirschsprung Disease?
RET protooncogene, trisomy 21, Waardenburg Syndrome
What section of the colon is most commonly aganglionic in Hirschsprung?
Rectosigmoid colon
What are the clinical features that differentiate Hirschsprung from a meconium plug?
Feeding intolerance, bilious vomit, severe constipation/obstruction, abdominal distention**
What is the gold standard diagnosis for Hirschsprung?
Suction rectal biopsy 2 cm above dentate line
You suspect Hirschsprung, next step?
Digital exam & evacuation -> large explosive could smelling bowel movement
What will you find in Hirschsprung histology?
Axon hyperplasia
What is the plan for Hirschsprung?
Surgical repair
If not.. toxic megacolon risk
What are possible complications following surgical repair of Hirschsprung disease?
Constipation, fecal soiling, prolapse, and stricture
Meconium ileus over 95% of teh time associated with what condition?
cystic fibrosis
What are signs that differentiate meconium ileus from some other conditions in assessment?
Rectal exam has narrow vault, radiograph has dilated bowel oops, and barium enema shows “soap bubble” in ilium and the entirety of colon = micro
What causes the meconium in meconium ileus to be thick, dry, and smelly?
CF = decreased pancreatic enzymes = not processed correctly
Malabsorption of carbs, fat, and protein
What increases the risk and is associated with neonatal small left colon syndrome?
Diabetic mothers
Small left colon syndrome affects what part of the colon?
Descending colon ONLY
