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48 Cards in this Set

  • Front
  • Back
What are 2 causes of congenital intestinal obstruction (atresias and stenoses)?
1. failure in development

2. intrauterine vascular accidents
What is atresia?
Complete obstruction
What is stenosis?
Narrowing of intestine due to a diaphragm or web that partially obstructs
How does atresia present clinically?
vomiting, abdominal distention
What does esophageal atresia commonly present with?
Tracheo-esophageal fistula
What does esophageal atresia and fistula present with?
Aspiration, regurgitation, respiratory distress
Is duodenal stenosis more commonly proximal or distal?
Proximal
What is the major cause of duodenal stenosis?
Web- resulting from failure of recanalization
What is Annular Pancreas?
Another cause of duodenal stenosis due to surrounding of the duodenum by pancreas
What causes jejuno-ileal atresia?
Intrauterine vascular accidents
How are jejuno-ileal atresias classified?
1. Whether the blind ends are connected

2. Whether there is a corresponding defect in the mesentery
How are anorectal atresias classified?
High (above levator sling) or Low
How do anorectal atresias present?
Abnormal perineum, imperforate anus, and often fistula to bladder, urethra, vagina
What is an omphalocele?
Defect of the abdominal wall where the umbilical cord inserts - sac containing intestinal loops, liver
What is gastroschisis?
Defect in abdominal wall to the right of the umbilical cord - evisceration of bowel, stomach, gonads but NO SAC
What is short bowel syndrome?
Massive loss of bowel
Most children with short bowel syndrome require what?
Total parenteral nutrition

Most eventually need transplantation
What are duplications in the GI tract?
cystic or tubular outgrowths anywhere along GI tract
Do duplications communicate with the intestine?
NO! but may share wall
Where do neuroenteric remnants originate?
Dorsal midline GI
Where are neuroenteric remnants most common?
Cervical and lumbar regions
What are the clinical signs of neuroenteric remnants?
GI obstruction, respiratory distress, and CNS manifestations such as paralysis
What is a diverticulum?
remnant of the vitelline duct (most common- Meckel's)

Communication between yolk sac and endoderm in normal devo
What is the most common GI congenital anomaly?
Meckel's diverticulum
Where is a Meckel's diverticulum located?
antimesenteric border of terminal ileum
What causes most symptoms of Meckel's?
Gastric mucosa - pain, rectal bleeding, perforation
How many degrees and to what direction does the gut rotate in development?
270 degrees CCW
What does malrotation cause?
abnormal fixation

Sometimes, volvulus and internal strangulation
What is pseudobstruction?
Signs and Sx of obstruction without obstruction
What causes pseudobstruction?
Enteric neuropathy (Hirschsprung) (problem in nerves)

Visceral myopathy (problem in muscles)

Secondary causes
What gives rise to the enteric NS?
Vagal Neural crest cells
What does failure of migration of neural crest cells result in?
Aganglionosis (Hirschsprung)
Does aganglionosis result in loss of Meissner's plexus or Auerbach's?
Both.
What are 2 genetic susceptibilities to Hirschsprung?
1. Mutations in RET

2. Down's syndrome
How is Hirschsprung diagnosed?
Absence of ganglion cells on biopsy
What is the most common GI emergency in newborns?
Necrotizing enterocolitis
What is a common finding in newborns with NEC?
Low birthweight
What are the signs of NEC?
abdominal distention, bloody stools, apnea, gas in bowel on radiology
What is pneumatosis intestinalis?
Gas in the bowel wall (found in NEC)
What are long term results of NEC?
Strictures, atresia, Short Bowel Syndrome
What is intussusception?
invagination of one intestinal segment into another
What is the most common cause of intestinal obstruction?
Intussusception
Compression of the mesentery in intussusception results in what?
Ischemia
What is the primary defect in cystic fibrosis?
abnormal chloride channel protein coded by CFTR gene
What are the 4 areas of the GI tract that CF affects?
1. Intestinal

2. Pancreatic

3. Hepatic

4. Nutritional
What are CF's affects on the intestines?
Dense secretions

Meconium ileus (obstruction by viscid meconium)
What are CF's affects on the pancreas?
Duct obstruction

Acinar destruction and loss

Loss of exocrine function
What are CF's affect on the liver?
Elevated liver enzymes (ASx)

Steatosis

Cirrhosis

Microgallbladder

Gallstones