Hirschsprung’s disease, Necrotizing enterocolitis, Imperforate anus
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What are the hallmarks of failure to pass meconium?
Failure to pass meconium in 24 hours, Abdominal distention, Reluctance to feed, Bilious vomiting
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What does bilious vomiting indicate in failure to pass meconium?
The obstruction was somewhere below the duodenum
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What is the differential for failure to pass meconium?
Duodenal atresia, Intestinal atresia, Malrotation and volvulus, Hirschsprung, Meconium ileus, Meconium Plug, Imperforate anus
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What is the first step in the workup for failure to pass meconium?
Rule out imperforate anus or any other anal abnormality
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What x‑ray finding suggests duodenal atresia or malrotation?
Double bubble
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What x‑ray finding suggests lower intestinal obstruction?
Multiple dilated loops of bowel
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What upper GI finding suggests malrotation?
Duodenal c‑loop does not cross midline
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What upper GI finding suggests malrotation with volvulus?
Bird’s beak sign
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What upper GI finding suggests jejunal/ileal atresia?
Transition zone in small bowel
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What contrast enema finding suggests meconium ileus?
Total microcolon
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What contrast enema finding suggests meconium plug syndrome?
Filling defects in the large bowel
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What contrast enema finding suggests Hirschsprung’s disease?
Transition zone
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What should be strongly considered in the workup of failure to pass meconium?
rectal manometry
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What condition is suspected when there's a double bubble finding on XRAY?
down syndrome.
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What other condition, besides malrotation with volvulus, would bird's beak be seen in?
achlasia.
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What is atresia?
closed or absent of a natural passage of the body
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What is stenosis?
narrowed
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What is volvulus?
an obstruction caused by twisting of the stomach or intestine
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What does VACTERL stand for?
Vertebral anomalies, Anus (imperforate), Cardiac defects, Trachea, Esophagus, Renal, Limbs
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What vertebral anomaly is associated with VACTERL?
Spina bifida
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What cardiac defects are associated with VACTERL?
VSD, ASD
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What is VACTERL?
Group of growth abnormalities that occur during fetal growth development. Need 3 (some sources say 2)
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What is the embryologic cause of esophageal atresia/TEF?
An interruption in the separation of the foregut into the trachea and esophagus during the fourth week of gestation
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What do all types of esophageal atresia/TEF involve?
incomplete formation of the esophagus
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How often is esophageal atresia associated with TEF?
90% of the time
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How often is EA associated with VACTERL?
10%
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Why is polyhydramnios seen in EA/TEF?
because the baby can’t swallow anything, so that fluid isn’t regulated
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What are the types of EA/TEF?
EA with distal TEF (87%), Isolated EA (8%), Isolated TEF (4%), EA with proximal TEF (1%), EA with double TEF (1%)
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What are symptoms of esophageal atresia/TEF?
Respiratory distress shortly after birth, worsening with feeding
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What is a prenatal sign of esophageal atresia/TEF?
polyhydramnios.
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What is the best initial test for esophageal atresia/TEF?
plain film (cxr/axr) which will show a blind upper pouch in about 92% of cases.
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What additional test should be ordered in EA/TEF?
Echocardiogram
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How many EA/TEF patients have congenital heart disease?
30–50%
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What is the management of esophageal atresia/TEF?
Surgical Peds Consult, NG Decompression, IV fluids, IV nutrition or gastrostomy, IV ABX.
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What is the definitive treatment for EA/TEF?
surgical repair.
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What is duodenal atresia?
Congenital absence or complete closure of part of the duodenal lumen. Defect occurs in early embryonic stages.
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What conditions are associated with duodenal atresia?
trisomy’s, cardiac lesion, and VACTERL
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What prenatal finding suggests duodenal atresia?
polyhydramnios
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Why does polyhydramnios occur in duodenal atresia?
The baby can take in fluid but only up until the duodenum, so then it becomes overflow incontinence and goes back up through the mouth, remaining with mom in the sac.
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What are symptoms of duodenal atresia?
bilious vomiting within hours after birth, failure to pass meconium, failure to thrive, scaphoid abdomen with epigastric distension
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What is the best initial diagnostic step for duodenal atresia?
abdominal xray → “double bubble sign”
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What is the management of duodenal atresia?
Peds surg consult, IV fluids and nutrition, May defer surgery, but if so get a contrast enema to r/o malrotation.
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What is the corrective surgery done for duodenal atresia?
duodenoduodenostomy.
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What post-op management is done for duodenal atresia?
obs, continue parental nutrition until stool passes, prophylactic IV abx.
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What is the best initial diagnostic step for duodenal atresia, and what sign is seen?
abdominal xray which shows double bubble sign.
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What are operative findings in duodenal atresia?
annular pancreas
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What is an annular pancreas?
Rare congenital condition where a ring of pancreatic tissue surrounds the second part of the duodenum causing obstruction of the duodenum.
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What must NEVER be done in annular pancreas?
NEVER DIVIDE the pancreas
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What causes intestinal atresia (in contrast to duodenal atresia)?
atresia elsewhere in the GI tract are typically caused by vascular accidents in utero.
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How often is intestinal atresia associated with cystic fibrosis?
10% will have cystic fibrosis.
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What are symptoms of intestinal atresia?
Similar to duodenal atresia
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What determines the severity of abdominal distension in intestinal atresia?
The severity of distension is proportional to how far the obstruction is down the GI tract.
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What is the best initial diagnostic test for intestinal atresia?
abdominal X-ray
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What radiographic signs are associated with intestinal atresia?
Triple bubble or apple peel
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What does triple bubble indicate?
Triple bubble in cases after the duodenum
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Where are the three bubbles in triple bubble found?
The bubbles are below the duodenum, the duodenum itself, and the stomach.
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What is apple peel associated with?
superior mesenteric artery disruption
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What additional diagnostic test can aid in intestinal atresia workup?
contrast enema
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What is Type I intestinal atresia?
short area of stenosis or a membranous web occluding the lumen
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What is type 1 atresia similar to?
Almost like ischemia, one part of the segment doesn’t enlarge like the other end of the intestine.