4.6 Flashcards
Hirschsprung disease is also called
congenital ganglionic megacolon
megacolon in Hirschsprung disease is due to
absence of ganglion cells in the mucosal and muscular layers of the colon –> functional obstruction (failure of colonic muscles to relax)
most common Hirschsprung disease
rectum and sigmoid colon involvement
risk factors for Hirschsprung disease
most common in males (4:1)
pathophys of Hirschsprung disease
failure of neural crest cells to migrate completely –> absence of enteric ganglion cells
what plexuses are affected in Hirschsprung disease
Auerbach and meissner
clinical manifestations of Hirschsprung disease
large bowel obstruction –> failure of meconium passage in first 48 H
Bilious vomiting
Progressive abdominal distention
Poor weight gain
FTT
Hirschsprung disease may also present with
enterocolitis –> sepsis like symptoms
PE in Hirschsprung disease
no stool in rectal vault : tight anal sphincter and the anal canal and rectum are devoid of fecal material
abdominal distention
what disease is associated with squirt/blast sign
Hirschsprung disease
what is the squirt/blast sign
explosive gush of flatus and liquid stool as the finger is withdrawn during rectal exam –> may relieve obstruction temporarily
what is the best initial test for Hirschsprung disease
contrast enema –> funnel shaped transition zone between normal and aganglionic bowel
when should you avoid contrast enema for Hirschsprung disease
if enterocolitis suspected
definitive diagnosis of Hirschsprung disease
rectal suction biopsy –> absence of ganglion cells in mucosal and submucosal layers of involved bowel
management for Hirschsprung disease
surgical resection of affected bowel
rectus abdominis muscles separate
diastasis recti
complete absence of closure of portion of the esophagus
esophageal atresia
esophageal atresia most commonly presents with
tracheoesophageal fistula
polyhydramnios
clinical manifestations of esophageal atresia
presents immediately after birth
excessive oral secretions
choking
drooling
inability to feed
respiratory distress
coughing
common complication/illness associated with esophageal atresia
aspiration pneumonia
diagnosis of esophageal atresia
inability to pass a nasogastric tube further than 10-15 cm
water-soluble contrast may reveal it in fluoroscopy
what type of contrast should be avoided in esophageal atresia and why?
barium; risk of aspiration pneumonia
management of esophageal atresia
surgical ligation with primary anastomosis of esophageal segments
complete absence or closure of a portion of the duodenum leading to gastric outlet obstruction
duodenal atresia
risk factors for duodenal atresia
polyhydramnios
down syndrome
clinical manifestation of duodenal atresia
presents shortly after birth (24-48 h)
bilious emesis
abdominal distention
what will you see on abdominal radiographs for duodenal atresia
double bubble sign
what is used preoperatively for duodenal atresia
upper GI series
definitive management of duodenal atresia
duodenoduodenostomy
twisting of any part of the bowel about its mesenteric attachment site and axis of its blood supply
volvulus
what is involved in up to 90% of cases of volvulus
sigmoid colon
in what type of patients does volvulus commonly present
patients who are less mobile, bed bound, and institutionalized usually with a background of chronic constipation
clinical manifestations of neonates with volvulus
bilious vomiting within the first week of life
colicky pain
what imaging will you get first for volvulus
abdominal X-ray with NG tube –> bent inner tube or coffee bean sign
what is the gold standard for diagnosing midgut volvulus
upper GI series with barium enema
treatment for midgut volvulus
emergency surgery –> counterclockwise unwinding
Ladd procedure
possible appendectomy
telescoping of an intestinal segment into the adjoining distal intestinal lumen, leading to bowel obstruction
intussusception
epidemiology of intussusception
most common cause of bowel obstruction in children 6 most - 4 years of age
when is intussusception commonly seen
after viral infection
classic triad of intussusception
vomiting - may be bilious
abdominal pain
passage of blood per rectum (currant jelly stools)
PE for intussusception
sausage shaped mass in the mid abdomen or RUQ + emptiness in RLQ (Dance’s sign)
emptiness in RLQ in intussusception
Dance’s sign
first line imaging for intussusception
US – will show donut, target, or bull’s eye sign
What type of enema is preferred for intussusception
air > barium
both diagnostic and therapeutic
treatment for intussusception
air enema is curative
observation for 48 hours after
when should you consider surgical intervention for intussusception
failed reduction
lead point presentation
