Examkin

Question

Difficulty: HardPediatric Gastrointestinal Disorders

Match each pediatric patient's clinical vignette (left) with the underlying pathophysiological or embryological mechanism responsible for the condition (right).

  • A 33-week-old term infant with a history of delayed meconium passage presents with abdominal distension and explosive expulsion of gas and stool following a digital rectal examination.Arrest of craniocaudal migration of neural crest cells
  • A 1414-month-old girl is brought to the clinic for painless, large-volume hematochezia. She is pale and tachycardic but has a soft, non-tender abdomen.Persistence of the omphalomesenteric duct
  • A 22-day-old premature infant born at 2828 weeks gestation develops feeding intolerance, abdominal distension, temperature instability, and grossly bloody stools.Ischemic mucosal necrosis associated with immature gut defenses
  • A 66-week-old infant presents with worsening jaundice, acholic stools, and hepatomegaly. Laboratory evaluation reveals a prominent direct hyperbilirubinemia.Progressive fibro-obliterative destruction of the extrahepatic biliary tree
  • A 33-day-old infant with trisomy 2121 develops recurrent bilious vomiting without abdominal distension. Abdominal radiography reveals a double-bubble sign.Failure of the intestinal lumen to recanalize

Answer

The infant with delayed meconium matches with neural crest cell migration arrest (Hirschsprung disease). The toddler with painless hematochezia matches with persistence of the omphalomesenteric duct (Meckel diverticulum). The premature infant with bloody stools matches with ischemic mucosal necrosis (necrotizing enterocolitis). The infant with acholic stools matches with fibro-obliterative destruction of the biliary tree (biliary atresia). The infant with Down syndrome and a double-bubble sign matches with failure of the intestinal lumen to recanalize (duodenal atresia).
Each clinical vignette carefully details classic epidemiological characteristics (e.g., age, prematurity, chromosomal associations) and clinical manifestations that point to a single gastrointestinal diagnosis, which in turn maps directly to a distinct embryonic or pathophysiologic mechanism.

Step-by-Step Solution

1
Analyze the first vignette to identify the delayed meconium and characteristic 'squirt sign' following a rectal exam.
Diagnosis is Hirschsprung disease.
Matching to the etiology requires identifying the failure of neural crest cell migration.
2
Analyze the second vignette focusing on a toddler with large-volume, painless gastrointestinal bleeding.
Diagnosis is Meckel diverticulum containing ectopic gastric mucosa.
This condition stems from the incomplete obliteration of the omphalomesenteric (vitelline) duct.
3
Analyze the third vignette detailing a premature neonate with systemic instability and bloody stools after feeding.
Diagnosis is necrotizing enterocolitis (NEC).
NEC is characterized pathologically by ischemic necrosis of the intestinal mucosa complicated by bacterial invasion.
4
Analyze the fourth vignette describing an infant with conjugated hyperbilirubinemia and acholic stools.
Diagnosis is biliary atresia.
This condition is driven by a progressive fibro-obliterative process destroying the extrahepatic biliary tree.
5
Analyze the fifth vignette featuring an infant with trisomy 21, early bilious emesis, and a double-bubble sign on x-ray.
Diagnosis is duodenal atresia.
Embryologically, this defect occurs when the intestinal lumen fails to recanalize following the solid phase of development.

Key Concept

Pathophysiology and embryology of common pediatric gastrointestinal emergencies
Estimated Time:2m 0s
Rate this question

Topics

Pediatric Gastrointestinal Disorders

More questions from this topic