Path: Pediatric GI Flashcards
What is the clinical presentation of esophageal atresia with tracheoesophageal fistula?
Aspiration, regurgitation and respiratory distress with initial feeds.
What is the most common variant of esophageal atresia?
A proximal segment of the esophagus that is blinded and a fistula joining the trachea with the distal end of the esophagus.
What karyotypic anomalies are frequently associated with esophageal atresia?
Trisomy 13, 18, 21.
What is the clinical presentation of duodenal stenosis?
Vomiting at birth; bilious vomiting if the stenosis is distal to the ampulla of Vater.
What is the typical cause of jejuno-ileal atresia?
Intrauterine vascular accidents or other vascular insults (volvulus, hernias, necrotizing enterocolitis).
What is the clinical presentation of jejuno-ileal atresia?
Proximal: vomiting
Distal: abdominal distension
How are anorectal atresias classified?
(1) low: below the levator sling and associated perineal fistulae
(2) high: above the levator sling and associated with fistulae to the genito-urinary tract
What are the consequences of short bowel syndrome?
(1) decreased fluid and electrolyte reabsorption
(2) nutrient and salt deficiency
(3) chronic diarrhea
How is short bowel syndrome treated?
(1) total parenteral nutrition
(2) bowel transplant
On what side of the bowel are GI duplications typically found? GI diverticula?
Duplications: mesenteric
Diverticula: anti-mesenteric
From what are diverticula derived?
Remnants of the vitelline duct.
Describe the formation of neurenteric remnants or cysts.
(1) They originate from the dorsal midline of the GI tract.
(2) They attach or pass through vertebrae and spinal cord, typically at the cervical or lumbar level.
Besides GI obstruction, what are frequent symptoms of neurenteric remnants or cysts?
(1) respiratory distress
(2) hypertrichosis or hyperpigmentation on dorsal cutaneous area
(3) paralysis
(4) infectious or chemical meningitis
What are the 4 main classes of congenital GI structural anomalies?
(1) atresia and stenosis
(2) abdominal wall defects
(3) duplications and diverticula
(4) malrotation
What are the 2 general classes of primary pseudoobstructions?
(1) enteric neuropathy
(2) visceral myopathy
What is the primary function of ganglion cells in the enteric nervous system?
Controlling relaxation of intestinal smooth muscle.
What are the 2 classes of plexuses in the GI tract?
(1) submucosal or Meissner’s plexus
(2) myenteric or Auerbach’s plexus
What is the typical clinical presentation of Hirschsprung’s disease?
Failure to pass meconium and/or abdominal distension.
What is a more technical name for Hirschsprung’s disease?
Aganglionosis.
What is the most common GI emergency in newborns?
Necrotizing enterocolitis.
What are the 4 most important contributing factors for necrotizing enterocolitis?
(1) intestinal ischemia
(2) intestinal immaturity
(3) bacterial colonization of gut
(4) enteral feeding
What is the typical clinical presentation for necrotizing enterocolitis?
Abdominal distension, bloody stools and apnea in the first 2 weeks of life. It may affect any segment of the bowel, but the distal ileum, cecum and ascending colon are most frequent.
J: This refers to the presence of gas in the wall of the bowel.
What is pneumotosis?
What is a notable bad outcome of necrotizing enterocolitis?
Fibrous strictures or atresia that require resection of the bowel, resulting in short bowel syndrome.
What is the most common cause of intestinal obstruction in childhood?
Intussusception.
What is intussusception?
The invagination of one intestinal segment into another.
What is the primary concern in the event of intussusception?
Ischemia of the intussuscepted segment due to compression of the mesentery.
How can cystic fibrosis affect the intestines?
(1) meconium ileus
(2) distal intestinal obstruction syndrome
(3) rectal prolapse
How can cystic fibrosis affect the pancreas?
(1) obstruction of ducts by viscid secretions
(2) acinar destruction, fibrosis, diffuse fatty replacement
(3) loss of exocrine function
How can cystic fibrosis affect the liver?
Chronic hepatic disease manifested by focal biliary cirrhosis or multilobular cirrhosis.
